MRCS Applied Basic Science and Clinical Topics

Correctable congenital abnormalities 287

• Direct surgical closure to be flat and air entry is reduced on the
• Skin flap closure affected side. The heart sounds are often
displaced. A chest x-ray will confirm the
Small defects can usually be closed surgically presence of gastrointestinal loops in the
but closure of large defects may require staged chest. Occasionally, patients present later
procedures. Overzealous reduction can result in life with respiratory distress or intestinal
in caval compression. After conservative obstruction.
treatment, a ventral hernia repair may be
required at about 1 year of age. Management

Gastroschisis Respiratory support with intubation and
ventilation is usually required. A nasogastric
Gastroschisis also has a male preponderance. tube should be passed. Gas exchange
A gastroschisis never has a sac. The umbilical and acid–base status should be assessed.
cord arises from the normal place in the Any acidosis may need correction with a
abdominal wall, usually to the left of the bicarbonate infusion. Surgery should be
abdominal wall defect. The evisceration considered early after resuscitation. The
usually only contains intestinal loops. It is hernial contents are usually reduced via
rarely associated with other major congenital an abdominal approach. The hernial sac
anomalies, but may be associated with is excised and the diaphragm repaired
intestinal atresia. Infants have better a with non-absorbable suture or a Gortex
prognosis than those with an exomphalos. patch. A Ladd’s procedure may be required
Mortality is approximately 10%. for malrotation. A chest drain is usually
not required. Early respiratory failure is
Management associated with a poor prognosis.

A gastroschisis can often be treated by direct Oesophageal atresia
full-layer closure of the abdominal wall. If
direct closure is not possible, then staged Oesophageal atresia affects 1 in 3000
reduction within a silo maybe preferable. live births. The aetiology is unknown but
Surgery is often associated with postoperative the incidence is increased in first degree
gut dysfunction and patients usually require relatives. It is often associated with a trachea-
postoperative nutritional and ventilatory oesophageal fistula (TOF – Figure 19.2). The
support. various presentations include:

Congenital diaphragmatic • Oesophageal atresia with TOF – 85%
hernia • Isolated oesophageal atresia – 8%
• Isolated TOF – 4%
A congenital diaphragmatic hernia occurs • Oesophageal atresia with proximal and
in 1 in 4000 live births. It results from failure
of closure of the pleuro-peritoneal canals. distal TOF
Approximately 95% occur through the
posterior foreman of Bochdalek. Less than About 50% of patients have other congenital
5% occur through the anterior foreman of abnormalities usually involving the
Morgagni. About 90% occur on the left. The cardiovascular, urogenital or anorectal
midgut herniates into the chest impairing systems.
lung development. It is often associated with
gastrointestinal malrotation. Clinical features

Clinical features Prenatally, oesophageal atresia may be
diagnosed by the finding of polyhydramnios.
Patients often present with cyanosis and The stomach is empty on ultrasound.
respiratory distress soon after birth. The Postnatally, the diagnosis should be
prognosis is related to the time of onset suspected by the neonate drooling, being
and degree of respiratory impairment. unable to swallow and cyanosed during
Examination often shows the abdomen feeding. Unrecognised, the patient usually
develops aspiration pneumonia. A 10 Fr

288 Chapter 19  Paediatric surgery

Oesophageal atresia with trachea-oesophageal fistula Figure 19.2  Oesophageal atresia
with trachea-oesophageal fistula

Upper pouch Tracheo-
oesophageal
Oesophageal fistula
atresia

nasogastric tube can not be passed more than features are often similar. Bile-stained
10 cm. On a chest x-ray, if there is gas in the vomiting is never normal in a neonate and
stomach, there must be a distal TOF. implies obstruction. Approximately 95%
of normal babies pass meconium within
Management the first 24 hours of life. Failure to pass
meconium should be regarded also as a
Feeding should be withheld and suction feature of obstruction until proved otherwise.
applied to the oesophageal pouch. The The degree of abdominal distension is
patient should be nursed in an upright variable.
position. The presence of other associated
congenital abnormalities should be Duodenal atresia
assessed. Surgery is required within the
first 24 hours of life. The operation involves Duodenal atresia occurs in 1 in 10,000
a right thoracotomy and an extrapleural live births. The site of the obstruction is
approach. The azygos vein and any TOF are most commonly in the 2nd part of the
divided. The oesophagus is mobilised and duodenum. The proximal the duodenum
a primary anastomosis is usually achieved. become hypertrophied. About 50% cases
If an anastomosis is impossible, then a are associated with polyhydramnios and
staged procedure is required. With the latter 60% of such pregnancies are complicated
approach, a gastrostomy is performed and or end prematurely. Approximately 30% of
the fistula divided at the initial operation. babies with duodenal atresia have Down’s
The oesophagus can be replaced by colon or syndrome. Other associated abnormalities
stomach after a few months. Complications are cardiac anomalies, intestinal malrotation
following surgery for oesophageal atresia and biliary atresia. Duodenal atresia can
include: often be diagnosed by antenatal ultrasound.
Postnatally, it presents with bilious or non-
• Oesophageal dysfunction bile stained vomiting. An abdominal x-ray
• Dilated proximal pouch may show a ‘double-bubble’ and no gas
• Gastro-oesophageal reflux within the bowel distal to the obstruction
• Anastomotic stricture (Figure 19.3).
• Recurrent fistula
Management
Neonatal intestinal obstruction
A nasogastric tube should be passed.
Neonatal intestinal obstruction can be due Intravenous fluid resuscitation should
to a variety of causes. The presenting clinical

Correctable congenital abnormalities 289

A plain abdominal x-ray showing duodenal atresia Figure 19.3  A plain abdominal
x-ray showing duodenal atresia

be given and major cardiac and delayed. Meconium filled loops of bowel may
other defects should be excluded. A be palpable. An abdominal x-ray may show a
duodenoduodenostomy should be performed ‘ground-glass’ appearance, especially in the
once the patient is resuscitated. right upper quadrant.

Other atresias Management
The administration of a gastrografin enemas
Atresias of the small bowel and colon are may be successful in 50% of patients. If
less common but again, are often associated unsuccessful, surgery will be needed. A
with polyhydramnios. Bilious vomiting and limited resection and stomas may be required.
distension are key features. An abdominal
x-ray will show dilated bowel and a gas-free Malrotation
rectum. A nasogastric tube should be passed
and intravenous fluid resuscitation should be Between 4 and 10 weeks of development, the
given. At operation, dilated proximal bowel intestines herniate into the umbilical cord.
should be resected or tapered and a primary During normal development, when returned
anastomosis may be possible. to abdomen, they rotate 270° anticlockwise. As
a result, the duodenojejunal (DJ) flexure lies
Meconium ileus to the left of the midline and the caecum lies
in right iliac fossa. The transverse colon lies
Meconium ileus is the commonest cause of anterior to the small bowel mesentery. Partial
neonatal intraluminal intestinal obstruction. failure of rotation results in malrotation. The
About 80% cases are associated with cystic commonest abnormality results in the caecum
fibrosis. Cystic fibrosis occurs in 1 in 2000 lying close to DJ flexure. The resulting midgut
live births and is inherited as an autosomal mesentery is abnormally narrow and liable
recessive condition. Viscid pancreatic to undergo a volvulus. Fibrous bands may be
secretions cause autodigestion of pancreatic present between the caecum and DJ flexure
acinar cells. The resulting meconium is (Ladd bands).
abnormal and ‘putty-like’ in consistency.
Meconium becomes inspissated in the lower Clinical features
ileum. There is a microcolon. It presents with There are two principal clinical presentations.
bilious vomiting and distension usually on Malrotation can present early with collapse
first day of life. The passage of meconium is

290 Chapter 19  Paediatric surgery

and acidosis due to intestinal infarction. mucosa and increased unmyelinated nerves
It can present late with intermittent bile- in the bowel wall.
stained vomiting and distension. Radiological
investigations are often unhelpful. Management
An initial defunctioning stoma should be
Management fashioned to relieve the obstruction. Definitive
treatment involves either a bypass of the
After resuscitation, an early laparotomy is affected segment (Duhamel or Soave bypass)
required. Any volvulus should be reduced. or excision of the aganglionic segment and
Resection may be required if there has anastomosis (Swenson procedure).
been small bowel infarction. Any Ladd
bands should be divided. The base of the Anorectal anomalies
mesentery should be elongated. The colon
should be placed on the left of the abdomen. Anorectal malformations comprise a wide
The small bowel should be placed on the spectrum of abnormalities. They affect 1 in
right. An inversion appendicectomy should 5000 live births and are often associated with
be performed to prevent future diagnostic other congenital malformations. Prognosis
uncertainty. depends on the severity of the malformation
and the extent of other anomalies. Early
Hirschsprung’s disease treatment of neonates with anorectal
anomalies is required.
Hirschsprung’s disease is due to absence of
the autonomic ganglion cells in Auerbach’s Clinical assessment
plexus of the distal large intestine. It Clinical inspection of the perineum is
commences at the internal anal sphincter and important. In 80% of patients, clinical
progresses for a variable distance proximally. examination and urinalysis allows a decision
It affects 1 in 5000 live births and the to be made as to whether a colostomy is
male:female ratio is 4:1. It accounts for 10% required. A flat perineum and absence of
of cases of neonatal intestinal obstruction. anal dimple suggest poor perineal muscle
Some cases appear to be due to autosomal development. These features are associated
dominant inheritance. About 75% cases are with high anorectal malformations.
confined to the recto-sigmoid but 10% cases Meconium at the perineum and a skin tag
have total colonic involvement. associated with an anal dimple or membrane
suggests a low malformation. Even if a
Clinical features perineal fistula is present, meconium may not
be passed for 24 hours.
Approximately 80% of cases present in the
neonatal period with delayed passage of Approximately 50% of all patients with
meconium. This is followed by increasing anorectal malformations have an associated
abdominal distension and vomiting. The urogenital anomaly. Renal abnormalities
child is at increased risk of enterocolitis and include:
perforation. It occasionally presents with
chronic constipation in infancy. • Renal agenesis
• Vesicoureteral reflux
Diagnosis • Neurogenic bladder
• Renal dysplasia
A plain abdominal x-ray will confirm • Megaureter
intestinal obstruction. A contrast enema • Hydronephrosis
may show a contracted rectum, cone shaped • Ectopic ureter
transitional zone and proximal dilatation.
Anorectal manometry may show a recto- Investigation
sphincteric inhibition reflex on rectal If the clinical signs are unclear, than
distension. The diagnosis is confirmed by radiological investigations may be useful. A
a rectal biopsy which will show absence of cross-table lateral x-ray with the baby prone,
ganglion cells in the submucosa, increased
acetylcholinesterase cells in the muscularis

Correctable congenital abnormalities 291

with a marker on the perineum, is a useful Types of hypospadias
initial study. The presence of air in the distal
rectum, within 1 cm of the perineum, suggests Glandular
primary repair may be possible. Ultrasound Cornal
may show features of co-existent obstructive
uropathy. If a colostomy is fashioned, distal Penile
colography may demonstrate a fistula to the
urinary tract. A plain x-ray of the spine and Penile/
sacrum may show associated abnormalities. scrotal
Urinalysis may indicate the presence of a Scrotal
fistula to the urinary tract.
Figure 19.4  Types of hypospadias
Management
Postoperative constipation is common and
Important issues to consider in the if unrecognised can result in a megarectum.
management of anorectal anomalies are: About 25% of patients have faecal incontinence.
Urinary incontinence may also occur. Severe
• Is there any other associated life bowel disturbances and urinary incontinence
threatening abnormality? are often associated with sacral defects.

• Should the patient undergo primary repair Hypospadias
with covering colostomy?
Hypospadias affects approximately 1 in 500
• Should the patient undergo defunctioning boys (Figure 19.4). It is due to incomplete
colostomy and later definitive repair? fusion of genital folds and the glandular
urethra. The urethra is found on the ventral
Intravenous fluids are required. A nasogastric surface of penis and is replaced distally by
tube should be inserted. A colostomy should a fibrous chordee. The deformity consists
be considered if there is: of a malpositioned meatus, chordee and
abnormal foreskin. Hypospadias is classified
• Recto-bulbar urethral fistula as glandular, coronal, penile or scrotal. About
• Recto-prostatic urethral fistula 70% are glandular or coronal, 10% are penile
• Rectovesical fistula and 20% are scrotal. Perineal hypospadias
• Imperforate anus without fistula is associated with intersex conditions and
• Rectal atresia anorectal anomalies.

A posterior sagittal anoplasty may be Management
possible if there is a low abnormality with a If any degree of hypospadias is present,
rectoperineal fistula. This avoids the need for circumcision is contraindicated. Treatment is
colostomy. required to improve the urinary stream and
allow sexual intercourse. Surgery is usually
Surgery performed between 2 and 4 years of age.
Glandular hypospadias requires a glandular
Repair of any anorectal malformation meatotomy. Coronal hypospadias requires
requires a meticulous and delicate technique. a meatal advancement and glanduloplasty.
The posterior sagittal approach is an ideal
method of defining and repairing anorectal
anomalies. Patients are placed in the prone
position with the pelvis elevated. Anorectal
abnormalities in 90% of male patients can be
repaired with a posterior sagittal approach
alone. The external sphincter is mobilised
to bring the anus back to the center of the
external sphincter. This can be performed
in the neonatal period without a protective
colostomy. An electrical stimulator helps
identify the location of the sphincteric
mechanism and postoperative anal dilatation
is required.

292 Chapter 19  Paediatric surgery

Proximal hypospadias without a chordee clinical sign is often a tuft of hairs or skin
can be treated by a skin flap advancement. dimple at the site of the defect. Neurological
If a chordee is present it should be excised deficit is rare. It may present with subtle
and an island flap urethroplasty performed. neurological abnormalities such as enuresis
Complications of hypospadias surgery include or incontinence.
a urethral fistula and stricture formation.
Meningocele and myelomeningocele
Neural tube defects
If the meninges bulge through vertebral
Embryology defect, it results in either a meningocele or
myelomeningocele. A meningocele does not
The central nervous system develops from contain spinal cord elements (Figure 19.6).
the dorsal ectoderm. The lateral edges of the A myelomeningocele contains the spinal
neural plate fold to form the neural groove. cord and nerve routes and may be associated
Fusion of the edges of the neural groove with caudal displacement of medulla and
generates the neural tube. Fusion starts cerebellum. This can result in hydrocephalus
cranially and progresses caudally. Both the (Arnold–Chiari malformation). It may
caudal and cranial ends of the tube remain also be associated with other congenital
temporarily open. The anterior neuropore abnormalities.
usually closes at about 25 days. The posterior
neuropore usually closes at about 27 days. Clinical features

Spina bifida A myelomeningocele occurs in 2–3 per 1000
The term spina bifida covers a range of live births. It can be detected prenatally by
vertebral and neural tube defects. It results increased maternal serum a-fetoprotein.
from failure of the posterior vertebral arch The spinal defect is clinically obvious
to fuse and most commonly occur in lumbo- and can result in various degrees of limb
sacral region (Figure 19.5). weakness, sensory loss, joint dislocation and
contractures and urinary disorders. Of all
Spina bifida occulta patients with a myelomeningocele, one-third
Spina bifida occulta is the commonest have complete paralysis and loss of sensation
form of spina bifida. Its true prevalence is below the level of the defect, one-third have
unclear. Isolated laminar defects are seen preservation of distal segments below the
on about 5% of lumbar spine x-rays. The level of the defect and one-third have an
spinal cord is usually normal. The only incomplete lesion. About 90% of children
develop urinary problems.

Neural tube defects

Dura mater Subarachnoid Arachnoid
space mater

Spinal
cord

Spina bifida occulta Meningocele Myelomeningocele
Figure 19.5  Neural tube defects

Common paediatric surgical disorders 293

A lumbo-sacral meningocele Figure 19.6  A lumbo-sacral
meningocele

Management of pyloric sphincter in the neonatal period
and mainly affects the circular muscle
Management is complicated and should fibres. The pylorus becomes elongated and
involve a multidisciplinary team. The team thickened, possibly due to failure of nitric
should include a paediatrician, orthopaedic oxide synthesis. It results in gastric outflow
surgeon, neurologist and physiotherapists. obstruction, vomiting and dehydration.
Treatment depends on the level and severity It has a multifactorial inheritance with a
of the defect. Patients with high defects strong genetic factor. It affects 3 per 1000 live
and gross neurological defects many not births. The male:female ratio is 4:1. It is most
be candidates for surgery. If the prognosis common in first born males.
is good, the aim of treatment should be to
achieve skin closure with 48 hours of birth. Clinical features
Ventriculo-caval shunting may be required in
the first week. Early treatment of orthopaedic Infantile hypertrophic pyloric stenosis
abnormalities is by physiotherapy. Surgical usually presents between 3 and 6 weeks of
intervention (e.g. osteotomies) may be age but late presentation up to 6 months can
required in the first few years of life. occur. The main clinical feature is rapidly
progressive projectile vomiting without bile.
Common paediatric surgical The child is usually hungry and often feeds
disorders immediately after vomiting. Dehydration
Infantile hypertrophic and alkalosis are other clinical features. The
pyloric stenosis clinical signs of dehydration include:

Infantile hypertrophic pyloric stenosis is often • Sunken eyes
described as congenital hypertrophic pyloric • Depressed anterior fontanelle
stenosis but is not truly a congenital disorder. • Reduced skin turgor
It results from hypertrophy and hyperplasia • Dry mucous membranes
• Increased capillary refill time
• Lethargy

294 Chapter 19  Paediatric surgery

A palpable ‘tumour’ in right upper quadrant diverticulum, duplication cyst or tumour. The
of the abdomen is best felt from left during a commonest site involved is the ileocaecal
test feed. Visible peristalsis is often seen. junction.

Investigation Clinical features
Intussusception usually presents with
The diagnosis can be confirmed by intermittent colicky abdominal pain and
abdominal ultrasound by assessment of vomiting. Each episode classically last
the length, diameter and thickness of the 1–2 minutes and recurs every 15–20 minutes.
pylorus. A wall thickness of great than 3 mm There may be the passage of blood,
supports the diagnosis. Serum electrolytes described as ‘red currant jelly’, per rectum.
and capillary gases should be measured. A sausage-shaped abdominal mass may be
Biochemically a hypochloraemic alkalosis is palpable.
usually present.
Investigation
Management The diagnosis can be confirmed with water
soluble contrast enema or ultrasound.
Dehydration should be corrected over
a 24 to 72 hour period prior to surgery. Management
A nasogastric tube is often required. Resuscitation with intravenous fluids and
Ramstedt described his pyloromyotomy in nasogastric tube is required. In the absence
1911. The operation can be performed via of peritonitis, reduction with air or contrast
either a transverse right upper quadrant or enema under radiological guidance can be
circumumbilical incision. A longitudinal attempted. If there is clinical evidence of
incision should be made in the pylorus peritonitis, shock or a failed reduction then
down to the mucosa. The incision should surgery is required. The intussusception
extend from the duodenum proximally on should be reduced. If there is necrosis of
to the gastric antrum. Mucosal perforation the bowel, then resection with primary
should be avoided. Feeding is re- anastomosis should be performed.
established within 12–24 hours of surgery.
Recurrence rarely occurs. Complications Necrotising enterocolitis
are rare and mortality is negligible.
Persistent postoperative vomiting may be Necrotising enterocolitis affects about 1 per
due to delayed return of normal gastric 1000 live births. The aetiology is unknown
motility, gastro-oesophageal reflux or an but bacterial infection and hypoxia appear
inadequate pyloromyotomy. The operation to be important. It occurs in premature or
has been described using a laparoscopic low birth weight infants and is associated
approach but no clear benefit has been with premature rupture of membranes,
demonstrated over a circumumbilical prolonged labour and respiratory distress. It
approach. is also well recognised following umbilical
artery catheterisation. It usually affects the
Intussusception terminal ileum and colon to a variable extent
and is characterised by mucosal necrosis
Intussusception occurs when one part of the with progression to intestinal infarction and
bowel invaginates (the intussusceptum) into perforation.
an adjacent section (the intussuscipiens)
resulting in intestinal obstruction and venous Clinical features
compression. If uncorrected, it can progress Necrotising enterocolitis usually occurs in
to arterial insufficiency and necrosis. It is the first week of life. The child is lethargic
the commonest abdominal emergency in and apathetic with vomiting and increasing
infants between 3 months and 2 years. The abdominal distension. Bloody diarrhoea is
peak incidence is 6 to 9 months. Most are a late feature. Abdominal examination may
idiopathic with the lead point due to enlarged show peritonitis or a mass.
Peyer’s patches as a result of a viral infection.
About 5% of cases are due to a polyp, Meckel’s

Common paediatric surgical disorders 295

Investigation Even benign conditions can be serious and
warrant prompt evaluation and treatment.
An abdominal x-ray may show distended The assessment of a child with an abdominal
bowel with mucosal oedema, intramural mass involves a number of diagnostic
gas (pneumatosis intestinalis) and portal considerations dependent on the age and sex
venous or free intraperitoneal gas. The of the patient, the location of the mass, and
extent of pneumatosis is not proportional the presence or absence of other potentially
to the severity of the illness. The presence of related clinical features. The common causes
pneumatosis per se is not an indication for of abdominal masses in children is shown
surgical intervention. Portal venous gas is a Table 19.3.
poor prognostic sign.
Nephroblastoma
Treatment
A nephroblastoma (Wilms’ tumour) originates
Initial treatment involves vigorous from the embryonal kidney. Pathologically,
resuscitation and medical management. The it contains renal tissue with various degrees
placement of a nasogastric intubation and of differentiation. It affects about 1 in 10,000
use of antibiotics are important. Parenteral live births. Approximately, 60% present before
nutrition should be considered. Indications the age of 3 years and 10% of tumours are
for surgery include: bilateral. The presentation is with an:

• Increasing peritonitis • Abdominal mass (90%)
• Failure of stabilisation with medical • Abdominal pain (20%)
• Haematuria (30%)
treatment
• Development of an abdominal mass The diagnosis can be confirmed by an
• Persistent loop or free gas on an abdominal CT scan. About 40% of patients
have metastatic spread at presentation
abdominal x-ray but this does not prevent cure. Treatment
Surgical treatment will involve resection is with nephrectomy and postoperative
with possible primary anastomosis. Overall, chemotherapy and radiotherapy. Stage 1
the prognosis is poor. The mortality of those disease (localised to the kidney) has a 3-year
undergoing medical treatment is about 20%. survival of more than 80%. Stage 4 disease
The mortality of those coming to surgery is (haematogenous spread) has a 3-year survival
about 30%. Amongst survivors, about 30% less than 30%.
develop ischaemic colonic strictures.
Neuroblastoma
Abdominal masses in children
Neuroblastomas arises from neural crest
The identification of an abdominal mass tissue, usually the adrenal medulla or
in a child is a cause for concern because
of the possibility of malignant disease.

Common causes of abdominal masses in children

Gastrointestinal Liver Genitourinary Other

Congenital hypertrophic Biliary atresia Hydronephrosis Neuroblastoma
pyloric stenosis Choledochal cysts Nephroblastoma Splenomegaly
Hepatitis Urethral valves Retroperitoneal sarcoma
Crohn’s disease Hepatoblastoma Teratoma

Intussusception

Constipation

Table 19.3  Common causes of abdominal masses in children

296 Chapter 19  Paediatric surgery

sympathetic ganglia. They show a range of regional variation in incidence. They are
malignancy from benign ganglioneuroma most commonly seen in Japan (1 in 1000
to malignant neuroblastoma. They affects live births) and are relatively rare in Western
about 1 in 8000 live births and usually occur Europe (1 in 100,000 live births). The
in first 5 years of life. Tumours in children are male:female ratio is 1:4 and the aetiology is
usually malignant. About 75% are abdominal unknown.
and 25% arise in the thorax, pelvis or neck.
Clinical presentation depends on the site of Pathology
the tumour and the presence or absence of
metastases. Bone and pulmonary metastases The cyst wall consists of fibrous tissue
are relatively common. Symptoms are often without muscle and the cyst may contain
due to metastatic spread and include: a large volume of bile. The cyst is often
associated with distal common bile duct
• Pallor, weight loss, irritability (40%) stenosis. If undiagnosed, the condition can
• Limb pain and hypertension (15%) progress to biliary fibrosis, cirrhosis and liver
• Abdominal mass or pain (30%) failure. Three types of choledochal cysts are
described.
About 90% of patients have increased urinary
VMA and HVA levels. A plain abdominal x-ray Clinical features
often shows diffused speckled calcification. The
diagnosis can be confirmed by an abdominal Choledochal cysts may be diagnosed
CT scan. Treatment is with surgery and prenatally on ultrasound. About 25% present
postoperative radiotherapy. Prognosis is best in neonates with prolonged jaundice and
in children presenting before 2 years. Stage cholestasis. The other 75% present later in
1 disease (localised to kidney) has a 3-year childhood with the triad of abdominal pain,
survival of more than 90%. Stage 4 disease abdominal mass and intermittent jaundice.
(haematogenous spread) has a 3-year survival The differential diagnosis includes biliary
less than 30%. atresia and neonatal hepatitis. Complications
include:
Choledochal cysts
• Recurrent cholangitis
Choledochal cysts are localised cystic • Hepatic fibrosis
dilatations of all or part of the common bile • Biliary cirrhosis and portal hypertension
duct (Figure 19.7). About 80% of choledochal • Rupture with biliary peritonitis
cysts present in childhood. There is a large • Pancreatitis

Classification of choledochal cysts Figure 19.7  Classification of
choledochal cysts. 1 = Gallbladder;
2 = Choledochal cyst.

1 1 1
2 2
2
Type l Type ll Type lll

Common paediatric surgical disorders 297

• Hepatic abscess Anal fissures
• Gallstoness
• Carcinoma of the biliary tree Anal fissures can occur at any age. The child
may be constipated and the fissure is often
Investigation visible. They often settle with laxatives.
An abdominal ultrasound usually reveals Surgery is rarely required but the child may
the cyst. Cysts can be further imaged by need a ‘gentle’ anal stretch.
abdominal CT or ERCP. Radioisotope
scanning may show delayed biliary Large bowel polyps
excretion and accumulation of isotope
within the cyst. Large bowel polyps present with painless
bleeding in an otherwise fit and well child.
Management Juvenile polyps account for 80% of polyps in
Type I and II cysts should be resected and childhood and 70% of patients are less than 5
a hepaticojejunostomy performed. This years old. Polyps are usually solitary and are
prevents both an anastomotic stricture often found in the rectum.
and malignancy in the cyst. Postoperative
cholangitis is uncommon. Type III cysts Infective enteritis
should be treated by cholecystectomy
and choledochojejunostomy or Infective enteritis can occur at any age.
choledochoduodenostomy. If the diagnoses Causative agents include:
is made early, the liver fibrosis regresses and
normal hepatic function can be expected. • Viruses – rotavirus, cytomegalovirus
Prognosis is poor if the patient presents with • Bacteria – Campylobacter, Shigella,
advanced disease and portal hypertension.
Salmonella, Escherichia coli
Rectal bleeding in children • Protozoa – Amoeba, Giardia

Most rectal bleeding in children is benign. Fresh stool should be sent for microscopy,
However, is may signify a life-threatening virology and culture.
disease. The likely aetiology can be
determined by the patient’s age and other Meckel’s diverticulum
associated clinical features (Table 19.4).
A Meckel’s diverticulum is a remnant
of vitello-intestinal duct, found in 2% of
population. About 30% of diverticulae
have heterotopic gastric mucosa that can
result in ulceration and bleeding. It usually

Causes of rectal bleeding in children

Common Neonates Infants Children
Less common
Anal fissure Anal fissure Anal fissure
Necrotising enterocolitis Intussusception Juvenile polyp
Viral gastroenteritis Gastroenteritis Meckel’s diverticulum
Midgut volvulus Meckel’s diverticulum
Intussusception Upper GI haemorrhage Inflammatory bowel disease
Intussusception
Vascular malformations
Solitary rectal ulcer
Henoch–Schonlein purpura

Table 19.4  Causes of rectal bleeding in children

298 Chapter 19  Paediatric surgery

presents with painless rectal bleeding. or posterior triangle of the neck my give an
It is best diagnosed with a radioisotope indication of the underlying pathology.
scan. Other complications include
intussusception, small bowel obstruction, Neck lumps in the anterior triangle include:
Meckel’s diverticulitis or a gastrointestinal
perforation. • Lymphadenopathy – infective, neoplastic
• Thyroglossal cysts
Circumcision • Dermoid cyst
• Goitre
A circumcision was first performed on • Branchial cyst
Abraham at the age of 90 years! Several
other references to circumcision exist in Neck lumps in the posterior triangle include:
the Old Testament. It is also depicted on
Egyptian tombs and murals. Each year 21,000 • Lymph nodes
circumcisions are performed in the UK where • Cystic hygroma
4% of boys will have had a circumcision by • Sternomastoid tumours
the age of 15 years. In Scandinavia, only 2% • Parotid swellings
of boys are circumcised. Many operations are
probably unnecessary. Branchial remnants
Branchial cysts arise from the second branchial
At birth the prepuce is normally sinus and are found on the anterior border of
adherent to the glans penis. This produces sternomastoid. They are often bilateral and
a physiologically non-retractile foreskin. extend deep into the neck. An internal opening
Only 4% of boys have a retractile foreskin at is occasionally found in the tonsillar fossa.
birth. By 5 years, in 90% of boys the foreskin Treatment is by surgical excision.
will be retractile. Only 1% of boys have a
true phimosis and 3% of boys have recurrent Cystic hygroma
balanitis. Indications for circumcision are:
A cystic hygroma is a hamartomatous
• Social and cultural lymphatic malformation resulting in a multi-
• Phimosis due to balanitis xerotica cystic mass. About 60% are found in the
neck region and they often present in early
obliterans, lichen sclerosis or trauma childhood as an expanding mass. They contain
• Balanitis or posthitis clear fluid and transilluminate brightly. Large
• Ballooning of foreskin lesions can be diagnosed prenatally and can
• Paraphimosis result in obstructed labour. Surgical excision
is difficult and can result in a poor cosmetic
Technique result. The use of sclerosants may be useful.

All preputial adhesions should be freed. It is Sternomastoid tumours
important to avoid excessive tension in order
to preserve skin. To avoid injury to the glans, A sternomastoid tumour is a mass in the
blind dissection of the foreskin is ill-advised. middle third of the sternomastoid muscle. It
Sutures or bipolar diathermy should be used results from muscle damage during labour
for haemostasis. Excessive numbers of skin and presents with a neck lump and torticollis
sutures should be avoided. Complications are away from the affected side. Treatment should
rare but include ulceration of the glans and involve physiotherapy to correct the torticollis.
urethral meatitis. About 1% need reoperation Surgery to remove the lump is rarely required.
for bleeding or haematoma.
Paediatric hernias
Neck lumps in children
Inguinal hernias
Neck lumps are common in children but
neoplasia is rare. Most head and neck lumps An inguinal hernia is the commonest surgical
result from congenital or inflammatory condition encountered in Childhood.
processes. The position in either the anterior Approximately, 2.5% of children require
an operation for a hernia. The incidence is
increased in premature and low birth weight

Common paediatric surgical disorders 299

infants. The male:female ratio is 9:1. About • Vas injury
5% of newborn males have an inguinal hernia • Undescended testis
– 70% are right-sided and 5% are bilateral. • Testicular atrophy
Almost all are indirect hernias. Approximately
30% of hernias present within the first year Irreducible hernias
of life and 15% present with incarceration or
strangulation. The initial management of an irreducible
hernia is to attempt reduction by taxis. It
Embryology requires gentle pressure usually without
sedation. Forcible reduction under general
The testis descends into the scrotum during anaesthesia is contraindicated. If the hernia
the 7th month of gestation. It is preceded by remains irreducible, then the child should be
the processus vaginalis – an outpouching of operated on within 24 hours.
peritoneum. The processus normally begins
to obliterate prior to birth and closure is Paediatric umbilical hernia
normally complete during the first year of life.
Persistence of all or part of the processus can Paediatric umbilical hernias are present in
result in an: about 10% of caucasian babies. They are seen
in 90% of babies of Afro-Caribbean descent.
• Inguinal hernia The incidence is increased in low birth weight
• Communicating hydrocele infants, Down’s syndrome and Beckwith–
• Hydrocele of the cord Wiedemann syndrome. The hernia is usually
symptomless and strangulation is extremely
Clinical features rare. About 95% close spontaneously by 2 years
of age. Surgical repair need only be considered
Inguinal hernias usually present with an if the hernia persists beyond this age.
intermittent groin lump. In girls, the lump is
in the upper part of the labia majora. Hernias Undescended testes
can be difficult to detect in a quiet child but
they increase in size with straining or crying. The testis undergoes intra-abdominal descent
They may reach into the scrotum. up to 28 weeks gestation. It is normally found
in the inguinal canal from 28 to 32 weeks
Management and it would be expected to be found in the
scrotum from about 30 weeks gestation.
Hernias presenting less than 1 year of age
should be operated on as urgent elective Cryptorchidism is the presence of a testis
cases. In the older child, the need for in an abnormal position. In full-term infants,
intervention is less pressing. Surgery can cryptorchidism is seen in 6% of boys. By 3
often be performed as a day case and inguinal months, the incidence has fallen to 2%. A
herniotomy is the operation of choice. A higher incidence of cryptorchidism is seen in
transverse incision is made in the inguinal premature infants. A ‘normal’ testis is scrotal
skin crease, Scarpa’s fascia is divided and or retractile testis. An ‘abnormal’ testis has
the external ring is identified. The sac is never been seen low in the scrotum and
dissected off the cord and divided. Dissection can not be manipulated to that position. An
is continued proximally until the peritoneal undescended testis is found in the normal
reflection is identified. The sac is then path of descent, in the inguinal canal or
transfixed and excised. The wound is closed abdomen. A maldescended testis has exited
and the testis pulled back into the scrotum. via the superficial inguinal ring but is now
About 20% of boys will develop a contralateral in an ectopic position. The usual sites are
hernia at some stage in their life. Controversy the femoral triangle or perineum. In 80% of
exists as to whether contralateral exploration patients with cryptorchidism, the testis is
should be performed. Complications of palpable. About 90% of impalpable testes are
herniotomy include: either high in the inguinal canal or abdomen.
True anorchidism is rare and is due to
• Wound infection primary agenesis or neonatal torsion.
• Recurrence

300 Chapter 19  Paediatric surgery

Cryptorchidism increases the risk of trauma or asphyxia or disease or injury in
testicular tumours by ten-fold and 10% of early life. It is essentially a motor disorder
patients with testicular tumours give a history that affects voluntary movements. It may also
of testicular maldescent. Cryptorchidism also be associated with other deficits including
increases the risk of infertility. Of patients blindness, sensory abnormalities, speech
with cryptorchidism, 30% have oligospermia defects and learning difficulties.
and 10% azospermia.
Clinical features
Management The motor defects takes several forms in
either isolation or combination and include:
If the testis is palpable in the inguinal canal
or high in scrotum, the patient requires an • Spasticity
orchidopexy, scheduled for during the second • Loss of coordination
year of life. It is usually performed via a ‘groin • Rigidity
and scrotum’ incision and the testis is placed • Hypotonic muscles
in a dartos pouch. Early orchidopexy may
improve fertility but there is no evidence that Developmental milestones are often delayed
it reduces the risk of malignancy. and the paralysis can be variable in extent
affecting:
If the testis is impalpable, laparoscopy
is the best means of identifying an intra- • Arm and leg on one side – hemiparesis
abdominal testis, vas and vessels. If no • One limb – monoparesis
vas, vessels or testis is present then there • Both legs – paraparesis
is primary agenesis. If vas and vessels are • All four limbs – quadriparesis
identified and no testis is seen, then neonatal
testicular torsion must have occurred. If Spasticity is of an upper motor neurone
an intra-abdominal testis is identified, type. The flexor muscles are often more
consideration needs to be given to a staged spastic than the extensors. Tendon reflexes
orchidopexy or microvascular transfer. If the are exaggerated and stretch reflexes are
vas and vessels are seen entering inguinal abnormally sensitive. Deformities develop
canal then the groin should be explored. A early due to muscle imbalance and several
Fowler–Stephen’s orchidopexy is a two-staged patterns are seen, including flexion of the
procedure. The gonadal vessels are divided elbow wrist and fingers and flexion and
at the first operation. This can be achieved adduction of the hips.
laparoscopically. This encourages a collateral
blood supply to develop via the cremasteric Management
and vassal vessels. Six months later the testis The clinical features are variable and complex
is mobilised on these vessels and delivered and patients should be managed by a
through the abdominal wall into the scrotum, multidisciplinary team including:
passing medial to the inferior epigastric
vessels. Following an orchiopexy, the testis is • Paediatricians
often smaller and higher in the scrotum than • Orthopaedic surgeons
normal testis. The testis may atrophy and • Physiotherapists
retract to a higher position. • Psychologists
• Speech therapists
Orthopaedic disorders of • Social workers
infancy and childhood
Cerebral palsy The mainstay of treatment is physiotherapy.
Physiotherapy aims are to assist in
Cerebral palsy is a disorder of movement assessment, prevent or attempt to correct
and posture due to a defect in the immature musculo-skeletal deformity, train the child in
brain. The underlying brain pathology is posture and movement and provide suitable
non-progressive. It often presents at birth sensory stimulation.
or in early childhood and is caused by birth
Surgery

Surgery aims to correct any established
deformity and involves the tendons, joint

Orthopaedic disorders of infancy and childhood 301

capsules, skin and bones. Muscle balance within the acetabulum and requires the
is restored and spasticity diminished by presence of a normal femoral head. The
tendon lengthening, tendon transfers, partial female:male ratio is 5:1. Ligamentous laxity
denervation and splintage. Surgery is most may be an important aetiological factor. Other
valuable in the lower limb and the timing of important factors include:
surgery is important.
• Family history
Hip disorders • Breech presentation
• Foot deformity
Accurate diagnosis and treatment of • Torticollis
paediatric hip disorders is important because • Neuromuscular disorders
of the potential for complications, which may • Skeletal dysplasias
lead to degenerative joint disease in adult life.
Different disorders have a propensity to occur Clinical features
at different ages (Table 19.5).
Developmental dysplasia of the hip can
Development dysplasia of the hip present as:

This condition is often described as congenital • A neonate with hip instability
dislocation. Developmental dysplasia of • An infant with limited hip abduction
the hip is a more accurate term as common • A toddler with a limp
features include dysplasia of the acetabulum • An adult with degenerative hip changes
and femoral neck anteversion. The apparent
incidence of developmental dysplasia depends All neonates should be screened for hip
on the age of the child. Approximately 20 per instability. The hips are flexed to 90° and
1000 neonates have clinical evidence of hip instability detected by reduction of the
instability but only 1 per 1000 have evidence of dislocation by abduction and forward
hip dislocation at 3 months. pressure (Ortolani’s test) and dislocation of
the hip by adduction and backward pressure
Pathophysiology (Barlow’s test). Examination may also show
The acetabulum develops from a triradiate limb shortening, an extra thigh skin crease
cartilage and three ossification centres. and hip external rotation. At an older age,
Normal growth is dependent on apposition Galeazzi’s sign may be elicited. It is a sign
of unilateral hip displacement. The child is
supine with hips and knees flexed and one

Diagnostic calendar of hip disorders

Age at onset (years) Probable diagnoses
Birth
0–5 Developmental dysplasia
Perthe’s disease
5–10 Late presentation of developmental dysplasia
10–15 Irritable hip
Perthe’s disease
Irritable hip
Slipped upper femoral epiphysis
Infection
Rheumatoid arthritis

Table 19.5  Diagnostic calendar of hip disorders

302 Chapter 19  Paediatric surgery

leg is shown to be shorter than the other. It Clinical features
is important to be aware that bilateral hip The median age of onset is 6 years and the
dislocation can be difficult to demonstrate. male:female ratio is approximately 4:1. The
patient presents with hip pain and a limp.
Investigation Examination will show reduced movement,
Hip ultrasound is useful, but with a high especially abduction and internal rotation.
sensitivity, can result in significant over About 10% of patients have a fixed deformity.
diagnosis. Plain x-rays are unreliable until
the child reaches 3 months of age. A hip x-ray Investigation
will show a shallow acetabulum with an A hip x-ray will show the capital femoral
underdeveloped femoral head. epiphysis to be smaller, denser and flattened.
The medial joint space is increased and the
Management ossific nucleus is fragmented. If performed, a
The aims of treatment are to reduce the bone scan will show a ‘cold’ femoral epiphysis.
dislocation by traction or open reduction
and to maintain reduction by a harness, cast, Management
soft tissue release or osteotomy. The ultimate The aims of treatment are to prevent
aim is to achieve stable congruant reduction deformation of the femoral head and
without damaging the growth plate. The prevent osteoarthritis. This can be achieved
above aims can be achieved by: by a period of bed rest and reduced weight
bearing. Surgical containment can be
• Pavlick hip harness or Von Rosen splint in achieved by a subtrochanteric or innominate
a neonate osteotomy.

• Traction in an infant Slipped upper femoral epiphysis
• Open reduction, osteotomy or
A slipped upper femoral epiphysis is the
acetabuloplasty in an older child commonest significant hip disorder of
adolescence. The femoral head ‘slips’
Irritable hip posteriorly and into varus. It tends to occur
in the obese and skeletally immature child. It
Causes of an ‘irritable hip’ in a child include: is a progressive disorder and therefore early
diagnosis is essential. The male:female ratio
• Perthe’s disease is 3:1 and approximately 20% of cases are
• Slipped upper femoral epiphysis bilateral. About 5% of patients have a family
• Juvenile chronic arthritis history.
• Septic arthritis
• Osteomyelitis Clinical features
• Rheumatic fever Slipped upper femoral epiphysis usually
presents with a gradual onset of hip or knee
Perthe’s disease pain. The initial clinical features can be
minimal and the diagnosis is often missed.
Perthe’s disease is a childhood It occasionally presents with sudden onset
osteochondrosis of the hip that occurs of pain after exercise. Examination shows an
secondary to avascular necrosis of the antalgic gait. The hip initially may have a full
capital femoral epiphysis. It is a self-limiting range of movement. A severe slip results in
disorder with revascularisation occurring fixed external rotation.
within 2 to 4 years. The femoral head may
however remain deformed resulting in Investigation
osteoarthritis. Four stages of the disease are A radiological diagnosis can be difficult. A
recognised: ‘frog lateral’ radiograph is probably best at
demonstrating the slipped epiphysis.
• Stage 1 – Avascular necrosis
• Stage 2 – Fragmentation of the femoral

epiphysis
• Stage 3 – Regeneration and

revascularisation
• Stage 4 – Healing

Orthopaedic disorders of infancy and childhood 303

Treatment Clinical features

The aims of treatment are to preserve the A scoliosis shows a typical deformity with a
blood supply to the femoral head, stabilise ‘skew back’ and ‘rib hump.’ The hip normally
the physis and prevent avascular necrosis protrudes on the concave side. The scapula
and chondrolysis. This is usually achieved by normally protrudes on the convex side.
in situ pinning of the epiphysis. Occasionally The level and direction of the major curve
a reconstructive subtrochanteric osteotomy convexity should be noted. Convexity of
is required. curvatures determines the nomenclature of
the lesion (e.g. a right thoracic scoliosis has the
Scoliosis thoracic spine convex to the right). A balanced
deformity keeps the occiput in midline. A fixed
A scoliosis is an apparent lateral curvature scoliosis become more obvious on flexion.
of the spine. It is a triplanar deformity with The younger the child and the greater the
lateral, anteroposterior and rotational curvature, the worse the prognosis.
components. A postural scoliosis is
secondary to pathology away from the Investigation
spine (e.g. short leg or pelvic tilt) and the
curvature disappears when patient sits down. Full length posterior–anterior and lateral films
A structural scoliosis is a non-correctable of the spine are required. The upper and lower
deformity. Vertebral rotation results in ends of the spinal curve can be identified and
spinal processes swinging to the concavity the angle of curvature (Cobb’s angle) can be
of curve and secondary changes occur to measured either directly or geometrically
counterbalance the primary deformity. Most (Figure 19.8). A lateral bending view can assess
cases of structural scoliosis are idiopathic but the degree of correctability. Assessment of the
it can also result from bone, neurological or degree of skeletal maturity is important as a
muscular disease. scoliosis can progress during skeletal growth.

Indirect geometric measurement of Cobb’s angle Figure 19.8  Indirect geometric
measurement of Cobb’s angle

Most tilted vertebra
90°

Cobb’s angle Apex
90° Most tilted vertebra

304 Chapter 19  Paediatric surgery

Idiopathic scoliosis Neuropathic/myopathic scoliosis

Approximately 80% of scoliosies are Neuropathic/myopathic scoliosis is
idiopathic. Patients often have a family associated with polio, cerebral palsy and
history. Many patients have a trivial curvature muscular dystrophy. The scoliosis is typically
and only about 0.2% of the population have long and convex towards of muscle weakness.
a scoliosis with greater than 30° of curvature. X-rays with traction will assess the degree of
The age of onset defines three groups as correctability.
adolescent, juvenile and infantile.
Clubfoot
Adolescent idiopathic scoliosis
Adolescent idiopathic scoliosis occurs with Clubfoot or congenital talipes equinovarus is
an onset older than 10 years. About 90% of the commonest congenital ankle deformity in
patients are female and progression is not the UK. It affects about 3 per 1000 live births
inevitable. With a curvature of less than 20°, and the male:female ratio is 3:1. About 40%
spontaneous resolution can occur. Predictors of cases are bilateral. There is a low incidence
of progression include young age, marked in far east Asians and Polynesians and high
curvature and skeletal immaturity. The main incidence in black South Africans. Genetic
aim of treatment is to prevent mild deformity factors seem to important in its aetiology but
becoming severe. If there is mild scoliosis most cases are idiopathic. It is occasionally
with progression, consideration should be associated with neuromuscular disorders.
given to the use of a brace. If there is greater Aetiological factors include:
than 30° of curvature and progression occurs,
then operative intervention may be required. • Developmental arrest or delay
Harrington rods are used to reduce the • Intrauterine moulding
rotational deformity and lateral curvature. • Retracting fibrosis
• Neuromuscular factors
Juvenile idiopathic scoliosis
Juvenile idiopathic scoliosis occurs with an Clinical features
onset between 4 and 9 years. It is a relatively
uncommon condition. The prognosis is worse Many cases are diagnosed with prenatal
than in the adolescent group. Spinal fusion ultrasound but ultrasound can not assess
may be necessary before puberty. the severity of the condition. The anatomical
features of talipes equinovarus include:
Infantile idiopathic scoliosis
Infantile idiopathic scoliosis occurs with • Equinus deviation in the sagittal plane
an onset less than 3 years. It is a rare • Varus deviation in the frontal plane
condition and 60% of patients are boys. • Adduction of the forefoot in the horizontal
In 90% of patients, the deformity resolves
spontaneously. In those in whom progression plane
occurs the curvature can be severe. It • Deviation of the whole foot with respect to
is associated with a high incidence of
cardiopulmonary dysfunction. the talus
The severity of the deformity can be graded:
Osteopathic scoliosis
Osteopathic scoliosis is associated with • Grade 1 – foot can be held in neutral
hemivertebrae, wedged and fused vertebrae. position
The overlying tissue often shows angiomas,
naevi and skin dimples. The degree of • Grade 2 – fixed equinus or varus deformity
scoliosis is usually mild. Before considering of less then 20°
surgery it is necessary to exclude a
meningomyelocele. • Grade 3 – fixed equinus or varus deformity
of more than 20°

Management

Treatment should begin within the first week
of life. Initial management should be with
conservative therapy involving manipulation
and serial casting and continuous physical
therapy. With both methods, the forefoot

Orthopaedic disorders of infancy and childhood 305

adduction and cavus deformity, then the • Posterior part of deltoid ligament
whole foot varus deformity and finally the • Posterior part of ankle joint and subtalar
equinus deformity are addressed, in that
order. False correction of the deformity can joint capsules
occur leading to a ‘rocker-bottom foot’. • Fibulocalcaneal and fibulotalar ligaments
• Peroneal tendon sheaths
Surgery • Talonavicular joint capsule

Surgery is required in 10%, 50% and 90% of Tendons that may require lengthening include:
patients with Grade 1, 2 and 3 deformity,
respectively. By 3 months of age, it is usually • Achilles tendon
clear whether conservative management will be • Tendon of flexor digitorum longus
effective. The timing of surgery is controversial. • Tendon of flexor hallucis longus
Most surgeons recommend surgery between • Tendon tibialis posterior
9 and 12 months of age. Plaster can then be
removed at the time the child begins to walk. Percutaneous wires may be need to
The aim of surgery is release all the tight maintain correction and the foot is kept
structures and lengthen muscles. in plaster for about 6 weeks. Following
plaster removal, a splint is required until the
Structures that may need to be divided child is walking normally. Late problems
include the: following surgery include dynamic forefoot
adduction and varus, recurrent deformity and
• Plantar fascia overcorrection. Most children undergoing
• Tendon sheaths of tibialis posterior, flexor surgery achieve a plantigrade foot and most
can wear normal shoes.
hallucis longus, flexor digitorum longus



Chapter 20 Plastic and
reconstructive
surgery

Applied basic sciences The hypodermis
Anatomy of the skin
The hypodermis is the subcutaneous layer
The skin consists of three major layers: deep to the skin composed of adipose and
areolar connective tissue.
• Epidermis – outer superficial layer
• Dermis – middle layer Sweat glands
• Hypodermis – deepest layer
Sweat glands are found all over the body.
The epidermis They have different functions at different sites.
Eccrine sweat glands are found in the palms
The epidermis is composed of keratinised of the hand, soles of the feet and forehead.
stratified squamous epithelium and is made Apocrine sweat glands are found in axillary
up of four distinct cell types and five layers. and anogenital areas. Ceruminous glands
The cells of the epidermis are: are modified apocrine glands in external ear
canal and secrete cerumen. The mammary
• Keratinocytes – produce keratin glands are specialised sweat glands that
• Melanocytes – produce melanin secrete milk.
• Langerhans’ cells – epidermal
Functions of skin
macrophages
• Merkel cells – touch receptors in The functions of the skin include:

association with sensory nerve endings • Protection – chemical, physical and
mechanical barrier
The layers of the epidermis are:
• Temperature regulation
• Stratum basale (basal layer) • Cutaneous sensation – receptors sense
• Stratum spinosum (prickly layer)
• Stratum granulosum (granular layer) touch and pain
• Stratum lucidium (clear layer) • Metabolic functions – synthesis of vitamin
• Stratum corneum (horny layer)
D
The dermis • Blood reservoir – skin blood vessels store

The dermis is the second major skin layer up to 5% of the body’s blood volume
containing strong, flexible connective tissue • Excretion – limited amounts of
including fibroblasts, macrophages, mast
cells and white blood cells. It is composed of nitrogenous wastes are eliminated in sweat
two layers called the papillary and reticular
dermis. The papillary dermis is areolar Inflammation
connective tissue with collagen and elastic
fibres. Its superficial surface contains peg- Inflammation is a dynamic process that
like projections called dermal papillae. The occurs in response to tissue injury. The causes
dermal papillae contain capillary loops, of inflammation include:
Meissner’s corpuscles and free nerve
endings. The reticular dermis and accounts • Physical injury
for approximately 80% of the thickness of the • Chemical injury
skin. Collagen fibres in this layer add strength • Infection
and resilience to the skin. Elastin fibres • Immunological disorders
provide stretch and recoil properties.
Inflammation can be acute or chronic and
different mechanisms are involved in the
two processes. The clinical signs of acute
inflammation are:

308 Chapter 20  Plastic and reconstructive surgery

• Redness – rubor inflammatory exudate, polymorphs and cell
• Heat – calor fragments. If pus accumulates an abscess may
• Swelling – tumour form. Organisation occurs when during the
• Pain – dolor acute inflammatory process there is excessive
• Loss of function exudation or necrosis. Local conditions
prevent removal of exudate or debris. New
Acute inflammation vessel formation occurs and macrophage and
fibroblast proliferation results in fibrosis.
The clinical features of acute inflammation
can be explained by changes at the Chronic inflammation
microscopic level. Three processes are seen in
acute inflammation: Chronic inflammation occurs if the causal
agent is not removed. The cell population in
• Hyperaemia the damaged tissue changes. Polymorphs
• Exudation are replaced by lymphocytes and plasma
• Migration of leukocytes cells. Macrophages may form giant cells.
New capillaries are formed. Fibroblasts
Hyperaemia is associated with a vascular deposit collagen resulting in fibrosis. Chronic
response. Following injury, initial inflammation can be primary with no prior
vasoconstriction occurs followed by acute inflammatory response. This occurs
arteriolar dilatation This occurs in response in tuberculosis and sarcoidosis and is
to direct vascular injury, chemical mediators characterised by the formation of granulomas.
causing vasodilatation and an autonomic
neurological response. This explains the Wound healing
redness and heat that occurs during acute
inflammation. Exudation is the passage of The four main stages of wound healing are:
protein rich fluid into the interstitial tissue
resulting in protective antibodies entering • Haemostasis
the damaged tissue. It occurs as a result • Inflammation
of increases capillary permeability and • Regeneration
increased capillary pressure. This explains • Repair
the swelling that occurs during acute
inflammation. Increased blood viscosity These stages are not discrete but overlap.
reduces capillary blood flow. The loss of axial
stream results in margination of polymorphs Haemostasis
which adhere to the endothelium. These Damaged endothelium exposes platelets to
then pass between endothelial cells into sub-endothelial collagen and releases von
interstitium with the process facilitated by Willebrand factor and tissue thromboplastin.
chemotaxis. von Willebrand factor facilitates platelet
adhesion to sub-endothelial collagen which
Sequels of acute inflammation release ADP and thromboxane A2. This
leads to further platelet aggregation. Tissue
There are four possible outcomes of acute thromboplastin activates the coagulation
inflammation: pathways. Fibrin is the end product of the
coagulation pathways which forms a plug
• Resolution in which platelets and red blood cells are
• Suppuration trapped. This results in clot formation.
• Repair and organisation
• Chronic inflammation Inflammation
Platelets release platelet derived growth
Resolution is the restoration of normal factor (PDGF) and transformation growth
conditions and occurs if there is minimal cell factor β (TGFβ). These are chemotactic to
death and tissue damage, rapid elimination of neutrophils and monocytes. Neutrophils and
the causal agent and local conditions favour macrophages migrate to the site of injury and
removal of fluid and debris. Suppuration phagocytose foreign material and bacteria.
is the formation of pus formed by the

Plastic surgery trauma 309

Regeneration and repair A keloid scar

PDGF and TGFβ are also mitogenic to Figure 20.1  A keloid scar
epithelial cells and fibroblasts. This leads to
proliferation of epithelial cells and fibroblasts. children. It usually subsides with time and
Fibroblasts produce collagen. Vascular may even regress. It is more common over
endothelial growth factor is also mitogenic to flexor surfaces and after burns.
endothelial cells. It is released by monocytes
in response to hypoxia and promotes Plastic surgery trauma
angiogenesis. Wound strength by the end of Burns
the 1st week is 10% of the original. By the end
of 3rd month it reaches 70% and thereafter Pathophysiology
plateaus.
A burn is defined as coagulative destruction
Time line of events of the skin or mucous membrane caused
by heat, chemical or irradiation. Thermal
Within the first 24 hours of injury, neutrophils damage occurs to the skin when the
are the predominant cell type. This is the temperature is above 48ºC. The extent of
phase of acute inflammation. Epithelial necrosis is related to both the temperature
cells start proliferating and migrating into and duration of contact. Burns can result in:
the wound cavity. By the 2nd and 3rd day, • Increased capillary permeability and fluid
macrophage and fibroblasts are the dominant
cell types. Epithelial cell proliferation and loss
migration continues. Angiogenesis begins • Hypovolaemia and shock
and granulation tissue starts to appear. • Increased plasma viscosity and
Collagen fibres are present but these are
vertical. They do not bridge the wound microthrombosis formation
gap. By the end of 5th day, fibroblasts are • Haemoglobinuria and renal damage
the predominant cell type. They synthesise • Increased metabolic rate and energy
collagen. Collagen now bridges the wound
edges – bridging collagen. Epidermal cells metabolism
continue to divide and the epidermis is
now multi-layered. Abundant granulation
tissue is present. During the 2nd week acute
inflammation begins to reduce and collagen
continues to accumulate.

Aberrations in wound healing

Keloid scar

A keloid is an overgrowth of scar tissue
(Figure 20.1). The scar tissue extends beyond
the wound margin. It is more common in
coloured races and is uncommon in children.
Typical sites are the sternum, back and ear
lobes. Triamcinolone injections directly
into the tissue is the initial treatment. Other
options include excision of the overgrowth but
following surgery keloid scars tend to recur.

Hypertrophic scar

Hypertrophy is overgrowth of scar tissue but
it does not extend beyond wound edge. It can
an occur in any race. It is more common in

310 Chapter 20  Plastic and reconstructive surgery

The ability of the skin to repair depends on of body surface area (BSA) involved. This can
the depth of the burn. Burns can be classified be calculated from either a Lund and Browder
as: Chart or by the Wallace ‘Rule of Nines’ (Table
20.1). Also, the surface area covered by the
• Superficial burns patient’s hand with fingers closed is about 1%
• Partial thickness burns the BSA. In a child, the body proportions are
• Full-thickness burns different. The head is relatively larger (19%
vs. 9%) and each lower limb relative smaller
Superficial burns (13% vs. 18%). The other body proportions are
similar to adults.
Superficial burns need to be differentiated
from simple skin erythema. In a superficial Fluid replacement
burn, only the epidermis and papillae are
involved. This results in red serum-filled Patients with extensive burns require
blisters. The skin blanches on pressure. The significant fluid resuscitation. To assess fluid
burn is painful and sensitive. Healing occurs replacement it is necessary to know:
in about 10 days without scarring.
• Time of injury
Partial-thickness burns • Patient’s weight
• Percentage BSA involved
In partial-thickness burn, the epidermis is
lost with varying degrees of dermis. The burn Intravenous fluid replacement needs to be
is usually coloured pink and white and may given for burns affecting more than 10%
or may not blanche on pressure. Variable BSA in a child and 15% BSA in adult. Many
degrees of reduced sensation may be present. formulas have been devised to estimate the
Epithelial cells are present in hair follicles fluid requirement. The most widely used
and sweat glands resulting in regeneration are the Muir and Barclay formula and the
and spread. Healing occurs in about 14 Parkland formula.
days. Some depigmentation of the scar may
occur. A partial-thickness burn requires skin The Muir and Barclay formula provides the
grafting. volume (in mL) of colloid to be given in the
first 4 hours. This volume should be repeated
Full-thickness burns every 4 hours for the first 12 hours, every 6
hours between 12 and 24 hours and once
In a full-thickness burn, both the epidermis between 24 and 36 hours.
and dermis are destroyed. The burn appears
white and does not blanche. Sensation is /Weight (kg) × % BSA 2 per period
absent and without grafting healing occurs
from the edge of the wound. The ATLS formula gives the total volume
(in mL) to be infused in the first 24 hours. Half
Assessment
The Wallace ‘Rule of Nines’
Initial assessment of a patient with burns
should be with ATLS principles. Good early Body area Percentage (%)
management is required to prevent morbidity
or mortality. In the airways assessment it is Head 9
important to look for signs of inhalation injury Each upper limb 9
including facial burns and soot in nostrils Each lower limb 18
or sputum. When assessing breathing, there Front of trunk 18
needs to be an awareness of carbon monoxide Back of trunk 18
poisoning. The patient may appear ‘pink’ with Perineum 1
a normal pulse oximeter reading. The fluid
loss from a burn is significant and can result Table 20.1  The Wallace ‘Rule of Nines’
in hypovolaemic shock and acute renal failure.

Assessment of the extent of the burn needs
an accurate measurement of the percentage

Plastic surgery trauma 311

the volume should be given in the first Electrical burns
8 hours.
Most electrical burns are flash burns and are
Weight (Kg) × % BSA× 4 superficial. They do not occur by electrical
conduction. A flash from an electrical burn
Metabolic water should be given in addition can reach 4000ºC. Low-tension burns are
to resuscitation fluid. In the adult, this usually small but full thickness. High-tension
amounts to about 2 litres of 5% dextrose. In a burns usually have an entry and exit wound.
child this amounts to 100 mL/Kg for the first The current passes along the path of least
10 kg and 50 mL/Kg for each subsequent Kg resistance (e.g. blood vessels, fascia, muscle)
of body weight. and the extent of tissue destruction can often
be underestimated. High-tension burns
The patient should be monitored to assess the can be associated with cardiac arrhythmias.
adequacy of resuscitation by: Myonecrosis and myoglobinuria can also
occur.
• Clinical assessment
• Vital signs – pulse, blood pressure Chemical burns
• Urine output (>50 mL/ hour in adult)
• Haematocrit (aim for 0.35) The commonest acids involved in chemical
burns are hydrochloric, hydrofluoric and
Criteria for referral to burns unit sulphuric. Acid burns may penetrate deeply
down to bone. First aid treatment involves
Patients should be referred to a burns unit if: liberal irrigation with running water. Calcium
gluconate may be useful in hydrofluoric acid
• Greater than 10% BSA in a child burns. The commonest alkalis involved in
• Greater than 15% BSA in an adult chemical burns are sodium hydroxide and
• Inhalation injuries cement and these also can cause deep-
• Burns involving the airway dermal or full-thickness burns.
• Electrical burns
• Chemical burns Skin grafts
• Specials areas – eyes, face, hand, perineum
A skin graft is an autograft from one part of
Escharotomy the body to another and can be partial or
full thickness depending on the amount of
Deep circumferential burns of the torso can dermis taken.
impair respiration. In a limb, they can impair
the distal circulation. In both situations, Partial-thickness skin grafts
escharotomies should be considered. No
anaesthetic is required. The burn should be Partial-thickness skin grafts contains
incised into subcutaneous fat and release of epidermis and the superficial part of dermis.
the underlying soft tissue should be ensured. It is usually taken from the donor site with a
On the chest wall, escharotomies should be dermatome or Humby knife. The donor site
performed bilaterally in the anterior axillary epithelium grows back from sweat glands
lines. Bleeding may be significant and a blood and hair follicles. Grafts can be ‘meshed’ to
transfusion may be required. increase the area that can be covered. Excess
skin can be stored in a fridge and reused for
Respiratory burns up to 3 weeks. Partial-thickness grafts can
not be used on infected wounds and are
Smoke inhalations should be suspected if not suitable for covering bone, tendon or
there is: cartilage. The cosmetic result is often poor
(Figure 20.2).
• Explosion in an enclosed environment
• Flame burns to the face Full-thickness skin grafts
• Soot in mouth or nostrils
• Hoarseness or stridor Full-thickness skin grafts contains epidermis
and all of the dermis. They can only be used
Intubation may be required. Blood
carboxyhaemoglobin levels can give an
indication of the extent of lung injury.

312 Chapter 20  Plastic and reconstructive surgery Figure 20.2  A partial thickness
A partial thickness skin graft skin graft

to cover small defects. Good cosmetic results the use of ‘delaying’ techniques. The flap is
can be obtained. The donor site needs to partially raised and replaced prior to use.
be closed with primary suture or a partial This encourages the flap to increase its blood
thickness graft. Common donor sites include supply through the pedicle.
the postauricular skin and supraclavicular
fossa. Tube pedicle grafts

Skin flaps Tube pedicle grafts are frequently raised
from the abdomen or inner arm. Parallel skin
Skin flaps are classified according to their incisions allow a tube of skin to be formed.
blood supply. The skin defect is then closed. The length of
the tube should not be greater than twice
Random pattern grafts the base and the long axis of the tube should
parallel to the direction of the cutaneous
A random pattern graft receives its blood blood vessels. It is a good means of delaying
supply from a segmental anastomotic or tissue transfer over a long distance and can
axial artery. Examples include advancement produce a good cosmetic result.
(Figure 20.3) and rotation flaps (Figure 20.4).
Myocutaneous flaps
Axial pattern grafts
In most parts of the body, the skin receives
An axial pattern graft receives its blood supply its blood supply from the underlying muscle.
from a direct cutaneous arteries. Examples The muscle, fascia and overlying skin can
include: therefore be moved as one unit and they
survive on the major blood vessel supplying
• Iliofemoral island flap supplied by the the muscle. Examples include:
superficial circumflex iliac artery
• Latissimus dorsi flap supplied by the
• Lateral forehead flap supplied the thoracodoral artery
superficial temporal artery
• Transverse rectus abdominis supplied by
• Deltopectoral island flap supplied by the superior epigastric artery
perforating branches of internal mammary
artery Myocutaneous flaps allow tissue transfer to
poorly vascularised areas. Bone can also be
Survival of all flaps depends on it receiving transferred for osseous reconstruction. Flaps
an adequate blood supply. This depends usually have no sensation.
on the length of flap in relationship to its
base. The blood supply can be improved by

An advancement flap Plastic surgery trauma 313

Figure 20.3  An advancement flap

A rotation flap Figure 20.4  A rotation flap

Free myocutaneous flaps Hand tendon injuries

Microvascular techniques allow the Hand flexor and extensor tendon injuries are
anastomosis of arteries and veins. common. Flexor tendon injuries are often
Myocutaneous flaps can therefore be detached associated with neurovascular damage.
from their blood supply and can be transferred Extensor tendon injuries are often associated
to other parts of body. Examples, include the with articular damage. Hand injuries require
free transverse rectus abdominis flap. careful assessment and management.
Assessment should be based on knowledge
Tissue expansion of tendon anatomy. Accurate surgical repair
requires meticulous surgical technique. If
Skin can be gradually stretched to poorly managed, tendon injuries can lead to
accommodate a greater area. If skin loss is significant functional disability.
anticipated, it is possible to expand adjacent
skin prior to surgery. Tissue expanders can Anatomy of hand tendons
be placed subcutaneously in collapsed state.
Over several weeks they can be inflated The flexor tendons of the hand run in
with saline through a subcutaneous port. fibro-osseous canals. The flexor digitorum
Expanded skin can then be used to cover the superficialis inserts into the middle phalanx.
defect and the tissue expander removed. The flexor digitorum profundus inserts into

314 Chapter 20  Plastic and reconstructive surgery

the distal phalanx. The metacarpal bones 2. From insertion of flexor digitorum
and phalanges form the dorsal wall. Synovial superficialis to the proximal edge of the A1
sheaths form the volar and lateral walls. pulley
The synovial sheath for the index to ring
finger begins at the neck of the metacarpals. 3. From the proximal edge of the A1 pulley to
The synovial sheath of the little finger is the distal edge of the carpal tunnel
continuous with the ulna bursa. Extensor
tendons are extra-synovial, except at the 4. Within the carpal tunnel
wrist. They are surrounded by extensive 5. Proximal to the carpal tunnel
paratenon with segmental arterial input. The
extensor retinaculum prevents bowstringing Extensor tendons are divided into eight
of the extensors. The main action is extension zones. Zones 1, 3 and 5 lie over the
of the metacarpophalangeal (MCP) joints. distal interphalangeal (DIP), proximal
interphalangeal (PIP) and MCP joints
Zones of injury respectively.

Flexor tendons are divided into five zones Flexor tendon injuries
(Figure 20.5). Zone 1 is distal and Zone 5 is
proximal. The five zones are: Assessment
An accurate history is essential. It is important
1. Distal to the insertion of flexor digitorum to know the handedness of the patient and his
superficialis or her occupation. Observation of the hand at
rest may indicate which tendons are involved.

Flexor zones of the hand Figure 20.5  Flexor zones of the
hand. (Reproduced courtesy of
Sam Scott-Hunter, London.)

Zone TII Zone TII
Zone TIII
Zone TIII Zone TIV
Zone TIV
Zone TV Zone TV

Pigmented skin lesions 315

The level of tendon injury may corresponds should be elevated to reduce swelling. Early
to the site of any laceration – but not always. mobilisation is required to reduce adhesion
If both flexor tendons are divided the finger formation and improve tendon healing and
will be extended. If the profundus tendon the final functional outcome. This requires
alone is divided then only the DIP joint will close supervision by a hand physiotherapist.
be extended. Further assessment should Mobilisation can begin as early as the first
involve testing of individual tendons of flexor postoperative day. Passive extension should
digitorum superficialis and flexor digitorum be avoided.
profundus. Neurovascular assessment is also
required. Extensor tendon injuries

Management For extensor tendon injuries, open
exploration and repair is required. They can
Early exploration and repair is required and often be repaired under local anaesthetic.
ideally surgery should be performed within Management depends on the zone of
24 hours of injury. Primary repair is the gold the injury. Proximal injuries require
standard but may not be possible if there has immobilisation with the wrist extended and
been delayed presentation or if the tendons the MCP joint flexed. Active movement can
have retracted. Antibiotic prophylaxis is begin after 3 weeks. Distal injuries require
required if presentation has been delayed longer periods of immobilisation.
or there is wound contamination. The ideal
tendon repair requires: Pigmented skin lesions
Malignant melanoma
• Sutures easily placed in the tendon
• Secure suture knots Epidemiology
• Smooth junction of the tendon ends
• Minimal gapping at the repair site Malignant melanoma is a tumour of
• Minimal interference with tendon epidermal melanocytes. It accounts for less
than 5% of skin cancers but more than 75%
vascularity of skin-cancer related deaths. The incidence
• Sufficient repair strength of melanoma is doubling every 10 years,
especially in sunny climates. Approximately
Many techniques of tendon repair have been 40 and 4 cases per 100,000 population are
described. They invariably involve a core seen each year in Queensland, Australia
suture and epitendinous suture. For Zone 1 and the UK, respectively. In the UK it
injuries, direct repair is usually possible. A accounts for about 1000 deaths. Patient
periosteal flap can be raised and the tendon education is increasing the number of thin
anchored with a core suture. For Zone 2 to tumours detected but the number of thick
5 injuries, wounds should be excised and tumours detected remains constant. It is the
irrigated. They may need to be extended to commonest cancer seen in 20 to 40-year-olds
retrieve and repair tendons. Incisions should and is more common in women. Risk factors
be avoided that cross skin creases. Careful include:
planning of incisions is required to prevent
skin necrosis or contracture. Incisions may be • Giant melanocytic naevus
required in tendon sheaths between the main • Total number of naevi
pulleys. The neurovascular bundles should • Dysplastic naevus syndrome
be identified and repaired is necessary. • History of recurrent sunburn
Tendons should be repaired using a standard • Skin type
technique. • Family history

Postoperative management Pathology

After repair, the hand should be placed in a About 20% of melanomas arise in pre-existing
back-slab with the wrist at 0–30° of flexion, naevi. Tumours have an initial radial and
the MCP joints at 60–90° of flexion and the followed by a vertical growth phase. These
PIP and DIP joints in full extension. The hand

316 Chapter 20  Plastic and reconstructive surgery

determine the growth characteristics of the A superficial spreading melanoma
tumour. Malignant melanomas are classified
as: Figure 20.6  A superficial spreading melanoma

• Superficial spreading (65%) to the skin to directly measure the depth to
• Nodular (20%) which tumour cells have invaded the skin.
• Lentigo maligna It is measured from the granular layer of
• Acral lentiginous the epidermis down to the deepest point of
invasion.
Superficial spreading melanoma occurs in Clinical features
middle age (Figure 20.6). The female:male Superficial spreading melanomas usually
ratio is 2:1. The commonest sites are the present with slightly elevated lesion with
lower leg in women and trunk in man. variable colour. Nodular melanomas are
Nodular melanomas are a more aggressive usually uniform in colour and present with
tumour that occur in a younger age group early ulceration and bleeding. Lentigo
(Figure 20.7). They have an early vertical maligna melanoma presents as flat light
growth phase. Lentigo maligna melanomas brown macules. A lesion is unlikely to be a
are the least malignant form and are usually melanoma without at least one major sign
found on the face of the elderly. They have a (Table 20.2). When assessing a potential
long radial growth phase. Acral lentiginous melanoma it is necessary to assess:
melanomas are an aggressive tumour. They • A = Asymmetry
are the commonest form seen in Afro- • B = Border irregularity
Caribbeans and Orientals. They occur on the • C = Colour variegation
soles of feet and palms of hand (Figure 20.8). • D = Diameter
Subungual melanomas are included in this • E = Evolution
group (Figure 20.9). Prognosis depends on: Suspicious skin lesions should be referred
for an excision biopsy. Incisional biopsy may
• Tumour thickness lead to inadequate histological assessment
• Growth phase and should be avoided.
• Epidermal ulceration Management
• Regression In the absence of metastatic disease, excision
• Lymphovascular invasion with wide margins and skin grafting, as
required, is the treatment of choice. The
In 5% of patients no primary tumour can
be identified and the disease presents with
regional or distant metastases.

Tumour thickness

Tumour thickness is the most important
prognostic factor for local, distant recurrence
and survival. Tumour thickness can be
measured as the Clarke level or Breslow depth
(Figure 20.10). Five anatomical or Clarke
levels are recognised as follows:

• Level 1 – Melanoma confined to the
epidermis (melanoma in situ)

• Level 2 – Invasion into the papillary dermis
• Level 3 – Invasion to the junction of the

papillary and reticular dermis
• Level 4 – Invasion into the reticular

dermis
• Level 5 – Invasion into the subcutaneous fat

The Breslow depth is determined by
using an ocular micrometer at a right angle

An acral lentiginous melanoma Pigmented skin lesions 317

Figure 20.7  An acral lentiginous
melanoma

A nodular melanoma Figure 20.8  A nodular melanoma

A subungual melanoma generally accepted resection margins based
Figure 20.9  A subungual melanoma on clinical appearance are:

• Impalpable lesion – 1 cm margin
• Palpable lesion – 2 cm margin
• Nodular lesion – 3 cm margin

Regional lymphadenectomy

In patients with malignant melanoma,
about 20% of clinically palpable nodes
are histologically negative. About 20%
of palpably normal nodes have occult
metastases. Patients with palpable nodes
should undergo fine needle aspiration
cytology. Confirmed nodal metastases in the

318 Chapter 20  Plastic and reconstructive surgery

Clark level versus Breslow depth of malignant melanoma Figure 20.10  Clark level versus
Breslow depth of malignant
melanoma

Level I 0
Level II 1
Level III 2
3
Level IV 4

Level V

Major and minor signs of • Lymphoedema
malignant melanoma • Seroma
• ‘Functional deficit’
Major signs Minor signs • Wound infection
Change in size Inflammation • Persistent pain
Change in shape Bleeding
Change in colour Sensory changes Adjuvant therapy
Diameter >7 mm
Patients at high-risk of recurrence should be
Table 20.2  Major and minor signs of malignant considered for systemic adjuvant therapy.
melanoma High-risk patients include those with a
primary tumour more than 4 mm thick
absence of systemic spread is an indication and resectable positive locoregional lymph
for regional lymph node dissection. Sentinel nodes. No standard adjuvant therapy exists
lymph node biopsy should be considered for but the use of interferon a2b has shown
those with impalpable nodes. promising results. It has been shown to
increase disease-free and overall survival.
Therapeutic lymph node dissection
provides regional control and prognostic Isolated limb perfusion
information but produces no improvement
in survival. For tumours less than Isolated limb perfusion (ILP) is the use of
0.75 mm thick about 90% are cured by intra-arterial chemotherapy administered
local excision alone. For tumours more into the limb isolated from the remainder
than 4.0 mm thick about 70% have distant of the circulation. Commonly used
metastases at presentation. For these agents include melphalan +/– TNF-a. It is
two groups, lymphadenectomy provides administered with hyperoxygenation and
no added survival benefit. The role of hyperthermia at temperature of 41–42°C.
lymphadenectomy for ‘intermediate’ Perfusion generally lasts about 1 hour.
thickness tumours remains controversial. Indications for ILP include:

The complications of lymphadenectomy • In-transit metastases
include: • Irresectable local recurrence
• Adjuvant therapy for poor prognosis

tumours
• Palliation to maintain limb function

The complications of ILP include:

• Mortality

Pigmented skin lesions 319

• Limb oedema a 45° angle with subsequent identification

• Persistent pain of residual tumour using light microscopy.

• Neuropathy This method provides histological control of

• Venous thrombosis the surgical margins. It achieves the lowest

• Septicaemia and thrombocytopenia recurrence rate with maximal preservation of

Other skin cancers normal tissue. The cure rate is more than 95%.

Basal cell carcinoma Squamous cell carcinoma

Basal cell carcinoma is the commonest skin, Squamous cell carcinoma is the second
malignancy. It occurs on sun exposed skin commonest cutaneous malignancy. The
particularly the face above a line from the commonest sites involved are the face and
angle of mouth to the ear (Figure 20.11). the hands (Figure 20.12). Initially, it usually
presents a red plaque. With time it invariable
.ir/It usually presents as a pearl-coloured ulcerates and has hard and irregular edges.
Predisposing factors include solar keratosis,
nodule with rolled edges. As it enlarges, Bowen’s disease, viral warts and chronic
central ulceration can occur. Predisposing ulceration or sinuses (Marjolin ulcers). The
factors include xeroderma pigmentosa and differential diagnosis includes:

sradiotherapy. They are locally invasive and • Keratoacanthoma
• Basal cell carcinoma
rarely metastasise. • Amelanotic melanomas
• Skin adnexal tumours
sClinical features
nThe clinical types of a basal cell carcinoma are:Treatment is by wide local excision, skin
ia• Nodular or ulcerative grafting and possible elective lymph node
dissection.
• Cystic
Benign skin lesions and
rs• Pigmented manifestations of systemic
disease
• Sclerosing
Peutz–Jeghers syndrome
e• Cicatrical
Peutz–Jeghers syndrome is inherited as
• Superficial autosomal dominant disorder. It presents
with circumoral mucocutaneous pigment
.pManagement
ipTreatment is by local excision with 0.5 cm

margins and may require a full thickness graft.
Other treatments modalities are radiotherapy

://vand Mohs micrographic surgery. This is a

procedure in which the tumour is excised at
httpA basal cell carcinoma
A squamous cell carcinoma

Figure 20.11 A basal cell carcinoma Figure 20.12 A squamous cell carcinoma
  

320 Chapter 20 Plastic and reconstructive surgery 

lesions on the lower lip, buccal mucosa and they often involute over 6 months leaving an

palate. It is associated with hamartomatous irregular pitted scar. The differential diagnosis

polyps in the small intestine. The polyps have is a squamous cell carcinoma. Treatment is by

fibrous and smooth muscle core with normal excision biopsy.

epithelium and may presents in childhood Pigmented skin lesions
with gastrointestinal bleeding, anaemia or

intussusception. The polyps are pre-malignant. Melanocytes are of neuroendocrine origin

External angular dermoid and migrate to the skin during the first three
months of intrauterine development. They
An external angular dermoid is a
produce melanin from tyrosine. Melanin is
sequestration dermoid lined by epidermis.
They occur on face at lines of fusion of stored in melanosomes before being exported
embryonic processes. The commonest site is to keratinocytes. A freckle is increased
melanin by a normal number of melanocytes.
.ir/around eye at the site of fusion of the frontalA naevus is a pigmented lesion due to an
increased number of melanocytes. Naevi
and maxillary processes. They occasionally are believed to evolve from junctional via
extend into the orbit and skull. compound to intradermal lesion.

sTricholemmal cyst Junctional, compound and
sTricholemmal cysts are inherited as intradermal naevi

autosomal dominant condition. About 90% A junctional naevus usually presents as
a small flat macule and often appears in
noccur on scalp and are often multiple. They childhood as a homogenous brown or black
iaare derived from hair follicles and should not skin lesion with increased melanocytes in
the rete pegs. A compound naevus presents
be regarded as simple epidermoid cysts. as raised papule. It is often pale brown with a
junctional component and nest of cells in the
rsPyogenic granuloma dermis. An intradermal naevus appears as a
flesh coloured papule. They have an increased
Pyogenic granulomas are neither pyogenic prevalence in middle age with no junctional
activity and only intradermal nests.
eor granulomas. They are capillary
Blue naevus
haemangiomas often with a traumatic
A blue naevus is a dome-shaped blue or
.paetiology. They present as bright red, friable black papule seen in middle-age. They are
more common in women. The commonest
nodules characterised by contact bleeding. site is the scalp and face. They have a dermal
collection of spindle melanocytes with
ipThe commonest site affected is the hands. melanin in dendritic cells. They possibly
arise as a result of incomplete migration of
Treatment is by curettage or diathermy. melanocytes to the epidermis.

://vCylindroma

Cylindromas are benign tumour of eccrine
sweat glands. They occur on the scalp and

ttpcan be solitary or multiple. Multiple tumours

are often referred to as ‘Turban tumours’.
Treatment is by local excision.

hDermatofibroma
Dermatofibromas or benign histiocytomas
are more common in women than men. They Halo naevus
present with small firm pigmented nodules,
usually pink or brown. The commonest site is A halo naevus is a benign naevus with pale
rim. It needs to be differentiated form a
the leg. Treatment is by local excision.
melanoma with regression. It results from

Keratoacanthoma a naevus with lymphocytic invasion and
melanocyte destruction.
Keratoacanthomas are more common in men
than women. They present as rapidly growing Juvenile melanoma
skin lesions over 6–8 weeks. They are usually
dome-shaped with a keratin filled crater and A juvenile melanoma or Spitz naevus is a
benign tumour that histologically mimics a
may be up to 3 cm in diameter. If untreated,
melanoma. It has regular melanocytes with a

Pigmented skin lesions 321

vascular stroma and epidermal hypertrophy. shoes, poor foot care and inappropriate nail

It is most commonly seen in young adults cutting.

and is more common in women. It presents Management
as a single pink domed-shaped nodule and

is most frequently seen on the head and In the early stages, conservative management

neck. should be attempted. This includes regular

Nail disorders soaking and washing of the feet, careful
drying after washing and the wearing of
Ingrowing toenails properly fitting shoes. Patients should be

Ingrowing toenails are a common problem in taught to cut their nails transversely. The use
adolescents and young adults. They usually
affects the hallux but other nails may also be  of pledgets of cotton wool placed under the
affected. It is due to the lateral edge of the nail nail may encourage it to grow out.

.ir/growing into the adjacent soft tissue (Figure Surgery may be required if conservative
measures fail. The nail can be removed by
20.13). Bacterial or fungal infection may be avulsion or wedge resection. Recurrence is
superimposed. Attempted healing may result common. If simple avulsion fails, ablation of
in over-granulation of the nail bed. Possible the nail bed should be considered. This can
be achieved either chemically or surgically.
saetiological factors include poorly fitting Chemical ablation can be achieved with
iansBilateral ingrowing toe nails phenol. Surgical removal usually involves a
Zadek procedure. Avulsion and phenolisation
http://vip.persFigure20.13 Bilateralingrowingtoenailsis more effective than surgical procedures.

Subungual haematoma

Subungual haematoma results from blunt
trauma to the hallux and nail bed. Blood
collects under the nail. Increased pressure
causes severe pain. The nail initially appears
red but becomes purple as the blood
coagulates. The differential diagnosis includes
a subungual melanoma, a glomus tumour
or Kaposi’s sarcoma. The haematoma can
be evacuated by nail trephine with a heated
needle or drill. Blood under pressure is
released and the symptoms immediately settle.



Chapter 21 Neurosurgery

Applied basic sciences a protective vault surrounding the brain.
Fourteen bones form the face. The skull is a
Neuroanatomy protector of the brain. The bones of the skull
have three distinct layers with hard internal
The scalp and external tables and a thin cancellous
middle layer, or diploë. The thickest area is
The scalp consist of five different anatomical usually the occipital bone and the thinnest is
layers that including the skin, the the temporal bone. The cranium is covered
subcutaneous tissue, the galea aponeurotica, by periosteum on both the outer and inner
loose areolar tissue and the skull periosteum. surfaces. On the inner surface, it fuses with
The subcutaneous layer possesses a rich the dura to become the outer layer of the
vascular supply that contains an abundant dura.
communication of vessels that can result
in a significant blood loss when the scalp The meninges
is lacerated. The relatively poor fixation of
the galea to the underlying periosteum of The meninges are the protective coverings of
the skull provides little resistance to shear the brain and spinal cord consisting of three
injuries that can result in large scalp flap or layers of membranous connective tissue
scalping injuries. This layer also provides (Figure 21.1) The dura mater is the tough
little resistance to haematomas and extensive outer layer lying just inside the skull and
fluid collections related to the scalp tend to vertebrae. In the skull, there are channels
accumulate in the subgaleal plane. within the dura mater, the dural sinuses,
which contain venous blood. In the spinal
The skull cord, the dura mater is often referred to as the
dural sheath. A fat-filled space between the
The adult skull is normally made up of 22 dura mater and the vertebrae, the epidural
bones. Except for the mandible, all of the space, acts as a protective cushion to the
bones of the skull are joined together by spinal cord. The arachnoid mater is the
sutures with little movement. Eight bones middle layer. Projections from the arachnoid,
including one frontal, two parietal, one called arachnoid villi, protrude through
occipital bone, one sphenoid, two temporal one layer of the dura mater into the dural
and one ethmoid form the neurocranium,

The layers of the meninges Figure 21.1  The layers of the
meninges

Skull
Dura
Arachnoid mater
Pia mater
Grey matter

White matter

324 Chapter 21 Neurosurgery

sinuses. The arachnoid villi transport the CSF cerebrospinal fluid (CSF) which absorbs
from the subarachnoid space to the dural physical shocks to the brain, distributes
sinuses. Two cavities border the arachnoid: nutrients to and removes wastes from the
the subdural space is outside the arachnoid nervous tissue. It provides a chemically stable
and the subarachnoid space is within the environment. The two lateral ventricles are
arachnoid. This space contains blood vessels found in the cerebral hemispheres. The
and circulates CSF. The pia mater is the third ventricle is connected by a passage
innermost layer. It tightly covers the brain and (interventricular foramen) to each of the
carries blood vessels that provide the nervous two lateral ventricles. The fourth ventricle
tissues. is connected to the third ventricle (via the
cerebral aqueduct) and to the central canal
The brain of the spinal cord. Additional openings in the
fourth ventricle allow CSF to flow into the
There are three major divisions of the brain. subarachnoid space.
They are the forebrain, the midbrain, and the
hindbrain. The forebrain is responsible for a A network of capillaries called the
variety of functions including receiving and choroid plexus projects into each ventricle.
processing sensory information, thinking, Ependymal cells surround these capillaries.
perceiving, producing and understanding Blood plasma entering the ependymal
language, and controlling motor function. cells from the capillaries is filtered as it
There are two major divisions of forebrain: passes into the ventricle, forming CSF. Any
the diencephalon and the telencephalon. material passing from the capillaries to the
The diencephalon contains structures such ventricles of the brain must do so through
as the thalamus and hypothalamus which the ependymal cells. Tight junctions linking
are responsible for such functions as motor these cells prevent the passage of plasma
control, relaying sensory information, between them. Thus, the ependymal cells
and controlling autonomic functions. The maintain a blood–CSF barrier, controlling the
telencephalon contains the largest part of composition of the CSF.
the brain, the cerebrum. Most of the actual
information processing in the brain takes Intracranial pressure
place in the cerebral cortex. The midbrain
and the hindbrain together make up the As the cranial vault is essentially a closed,
brainstem. The midbrain is the portion of fixed bony structure, its volume is constant.
the brainstem that connects the hindbrain The contents are the brain, CSF and blood.
and the forebrain. This region of the brain is All these components are non-compressible
involved in auditory and visual responses as and as a result any increase in one of the
well as motor function. The hindbrain extends components must be at the expense of the
from the spinal cord and is composed of the other two. This relationship is known as the
metencephalon and myelencephalon. The Monro–Kellie doctrine. Once the cranial vault
metencephalon contains structures such as is filled, pressure rises dramatically.
the pons and cerebellum. These regions assist
in maintaining balance and equilibrium, Normal intracranial pressure (ICP) is
movement coordination, and the conduction 5–10 mmHg. Due to autoregulation, cerebral
of sensory information. The myelencephalon perfusion pressure (CPP), the pressure of
is composed of the medulla oblongata which blood flowing to the brain, is normally fairly
is responsible for controlling autonomic constant. A rise in ICP reduces cerebral blood
functions. flow by reducing the cerebral perfusion
pressure. Some compensation is possible as
Neurophysiology CSF and blood move into the spinal canal and
extracranial vasculature, respectively. There is
Cerebrospinal fluid however a point where further compensation
is impossible and ICP rises dramatically
There are four cavities in the brain, called (Figure 21.2), CPP falls and cerebral ischaemia
ventricles. The ventricles are filled with occurs. Common causes of raised ICP include:

Neurosurgical trauma 325

The relationship between intracranial volume and pressure Figure 21.2  The relationship
between intracranial volume and
Critical volume pressure

Intracranial Pressure

Intracranial volume

• Head injury haematoma or hypertension. The early
• Intracranial haematoma treatment of head injured patients is aimed
• Subarachnoid hemorrhage at the detection and prevention of secondary
• Brain tumour brain injury. Following major head injuries,
• Encephalitis autoregulation of cerebral blood flow is lost.
• Hydrocephalus (increased fluid around
Clinical features
the brain)
• Cerebrovascular accident Patients with head injuries should be managed
according to ATLS protocols to prevent
Neurosurgical trauma secondary brain injury. All patients presenting
Head injuries to an emergency department with a head injury
should be assessed by a trained member of staff
Epidemiology within a maximum of 15 minutes of arrival.
Approximately, 1 million patients attend This assessment should establish whether they
emergency departments each year in the UK are at high or low-risk for clinically important
with a head injury. brain injury. Full assessment requires:

• 80% are minor (GCS 13–15) • Glasgow Coma Scale (Table 21.1)
• 10% are moderate (GCS 9–12) • Pulse, blood pressure
• 10% are severe (GCS less than 8) • Assessment of pupil diameter and

Severe head injuries account for 50% of response
trauma-related deaths. • Assessment of limb movement

Pathophysiology Depressed conscious level should be ascribed
Primary brain injury is damage caused at to intoxication only after a significant brain
time of impact. It can be focal or diffuse. injury has been excluded.
Diffuse axonal injury is due to deceleration
and shearing forces. It is dependent on the Signs of a basal skull fracture are:
extent of the initial injury and is not amenable
to treatment. Secondary brain injury is • Blood or cerebrospinal fluid from nose or ear
any neurological insult imposed after the • Periorbital haematoma
primary injury. It can be due to hypoxaemia, • Mastoid haematoma (Battle sign)
hypercapnia, hypotension and intracranial • Haemotympanum
• Radiological evidence of intra-cranial air
• Radiological evidence of fluid levels in the

sinuses

326 Chapter 21 Neurosurgery

Glasgow Coma Scale

Eye opening Motor response Verbal response 5
4
Spontaneous 4 Obeys 6 Orientated 3
To speech 3 Localises 5 Confused 2
To pain 2 Withdraws 4 Inappropriate 1
None 1 Abnormal flexion 3 Incomprehensible
2 None
Extensor response 1
None

Table 21.1  Glasgow Coma Scale

About 5% patients with a severe head injury • Amnesia (antegrade or retrograde) lasting
have a cervical spine injury. Patients who more than 5 minutes
have sustained a head injury and present with
any of the following risk factors should have • Abnormal drowsiness
full cervical spine immobilisation: • Three or more discrete episodes of

• GCS less than 15 on initial assessment by vomiting
the healthcare professional • Clinical suspicion of non-accidental injury
• Post-traumatic seizure but no history of
• Neck pain or tenderness
• Focal neurological deficit epilepsy
• Paraesthesia in the extremities • GCS less than 14, or for a baby under 1
• Any other clinical suspicion of cervical
year GCS less than 15
spine injury • Suspicion of open or depressed skull injury

Investigation or tense fontanelle
• Any sign of a basal skull fracture
CT is the primary investigation of choice for • Focal neurological deficit
clinically significant brain injury. Indications • Dangerous mechanism of injury
for an immediate CT in an adult are:
Plain x-rays of the skull should not be used
• GCS less than 13 at any point since the to diagnose significant brain injury without
injury prior discussion with a neuroscience unit.
Skull x-rays have a role in the detection of
• GCS equal to 13 or 14 at 2 hours after the non-accidental injuries in children.
injury
Management
• Suspected open or depressed skull fracture
• Any sign of a basal skull fracture Criteria for admission and observation after a
• Post-traumatic seizure head injury are:
• Focal neurological deficit
• More than one episode of vomiting • Altered level of consciousness
• Amnesia for greater than 30 minutes of • Skull fracture
• Neurological symptoms or signs
events before impact • Difficult assessment – drugs or alcohol
• If LOC in patients older then 65 years, • No responsible carer

coagulopathy or dangerous mechanism of Observations should be performed and
injury recorded on a half-hourly basis until a
Indications for an immediate CT in a child GCS equal to 15 has been achieved. The
are: minimum frequency of observations for
patients with GCS equal to 15 should be as
• Loss of consciousness lasting more than 5 follows:
minutes

Neurosurgical trauma 327

• Half-hourly for 2 hours • Spontaneous hyperventilation
• Then 1-hourly for 4 hours • Respiratory arrhythmia
• Then 2-hourly thereafter • Bilateral fractured mandible
• Copious bleeding into mouth
Should a patient with GCS equal to 15 • Seizures
deteriorate at any time after the initial 2-hour
period, observations should revert to half- Antibiotics and anticonvulsants are of no
hourly and follow the original frequency benefit in uncomplicated head injuries.
schedule. Both may be used in compound depressed
fractures and penetrating brain injury.
Indications for referral to neurosurgeon are:
Complications of head injuries
• Persistent coma (GCS <8) after initial
resuscitation Acute extradural haematoma
An extradural haematoma is a complication of
• Unexplained confusion persisting for more low velocity injuries. The classic presentation
than 4 hours is with transient loss of consciousness with
rapid recovery followed by a lucid interval
• Deterioration in GCS after admission and then rapid deterioration in the level
• A seizure without full recovery of consciousness. This may be associated
• Progressive focal neurological signs with an increased blood pressure, falling
• Definite or suspected penetrating injury pulse rate, contralateral limb weakness and
• CSF leak ipsilateral pupillary dilatation. Treatment is by
emergency burr holes (Figure 21.3).
Indications for intubation and ventilation
following a head injury are: Acute subdural haematoma
A subdural haematoma is a complication
• GCS less than or equal to 8 of high velocity injury (Figure 21.4).
• Loss of protective laryngeal reflexes
• Ventilatory insufficiency as judged by

blood gases

–– PaO2 less than 9 kPa
–– PaCO2 greater than 6 kPa

Position of emergency burr holes Figure 21.3  Position of
emergency burr holes. A =
Temporal; B = Frontal; C = Parietal
burr holes.

B

4 cm A C
2 cm
External
auditory
meatus

Zygomatic
arch

328 Chapter 21 Neurosurgery

CT appearance of an acute right content that leads to osmotic swelling. The
subdural haematoma haematoma may reach a size to cause and
mass effect and raised intracranial pressure.
Figure 21.4  CT appearance of an acute right Clinical features
subdural haematoma The classical presentation is of an elderly
patient with a history of falls who presents
The patient is usually unconscious from with deteriorating neurological function. The
the time of injury with deteriorating patient often has headaches, confusion or
level of consciousness. Treatment is by focal mass effect – hemiparesis or dysphasia.
decompressive craniotomy. Investigation
A CT scan may show a crescent-shaped mass
Chronic subdural haematoma between the inner table of the cranium and
the outer surface of the cortex (Figure 21.5).
Chronic subdural haematoma is one of the Most haematomas are located in the fronto-
commonest neurosurgical emergencies. It parietal region. The natural history if for the
occurs when blood expands in the subdural clot to be initially hyperdense early after injury
space over a period of weeks. The incidence and then to become hypodense with time.
increases with age and is more common Bilateral isodense subdural haematomas can
in men. The majority occur after an initial easily be missed.
minor head injury. About 40% of patients Management
are unable to recall the injury and in 30% of Small haematomas without significant mass
patients the haematomas are bilateral. Risk effect can be managed conservatively. If the
factors include cerebral atrophy, coagulation
disorders and arachnoid cysts. The bleeding CT appearance of a chronic right
results from rupture of the small bridging subdural haematoma
veins between the dura and cranium. The
subdural space is progressively filled by Figure 21.5  CT appearance of a chronic right
extravasated blood. With time, the liquefying subdural haematoma
haematoma contains a high protein

Neurosurgical trauma 329

haematomas are large and have significant Patients with a partial lesion may regain
mass effect, then burr holes should be substantial or even normal neurological
considered. Craniotomy is indicated if the function even though the initial neurological
clot does not completely liquify or becomes deficit may be severe. The presence of the
loculated. Over 90% of operated cases show bulbocavernous reflex or anal–cutaneous
residual blood on early postoperative imaging reflex indicates sacral sparing and a more
and as many as 20% of cases require further favourable prognosis.
surgery.
Patients may develop respiratory failure due
Spinal cord injuries to:

All patients with multiple trauma should be • Intercostal paralysis
suspected of having a spinal injury. Failure to • Partial phrenic nerve palsy
detect usually results from failure to suspect. • Impaired ability to cough
The cervical spine and thoraco-lumbar • Ventilation–perfusion mismatch
junction are the commonest site of injury. The • Variable intercostal nerve paralysis
percentage of nerve injuries seen in patients • Associated chest injuries
with spinal fractures are:
Respiratory failure may develop as a late
• Cervical spine – 40% feature due to ascending oedema in the
• Thoracic spine – 10% cervical cord. The abdomen may be flaccid
• Thoraco-lumbar junction – 35% with absent sensation. Features of peritonism
• Lumbar spine – 5% may be absent. Priapism may develop.

All injuries should be assumed to be unstable Partial cord lesions
until proved otherwise.
With partial cord lesions, function may be
Initial assessment preserved distal to the level of cord injury.
The diagnosis may be missed if it does not
At the scene of the accident, it is vital to fit the classical injury pattern. The clinical
maintain in-line spinal immobilisation. This features of partial cord lesions are as follows:
requires supporting of the neck with a stiff
collar and sandbags. The patient should be • Central cord lesion – flaccid paralysis of
transported on a spinal board. During the the upper limbs
primary survey, if intubation is required in-
line immobilisation should be maintained. • Anterior cord lesion – loss of temperature
Cervical spine injuries reduce sympathetic and sensation
outflow. Therefore, in patients with spinal
cord injuries, pharyngeal stimulation with an • Posterior cord lesion – loss of vibration
airway or endotracheal tube can cause vagal sensation and proprioception
discharge and cardiac arrest. Patients may
be both hypotensive and bradycardic and • Brown–Sequard syndrome – loss of
may require the use of atropine and inotropic ipsilateral power and contralateral pain
support. Aggressive fluid resuscitation can and temperature
induce pulmonary oedema.
Radiological assessment
Clinical features
Approximately 20% patients with spinal cord
The level of the cord lesion is conventionally injury have no radiological evidence of bony
defined as the most caudal location with injury. Lateral cervical spine x-rays were until
normal motor and sensory function (Table recently the commonest means of assessing
21.2). Spinal shock may mimic a complete the cervical spine. With the increasing
cord lesion with total loss of motor and availability of CT scanning, this is being more
sensory function distal to the injury. However, commonly used to assess possible cervical
if the lesion is incomplete some function will spine injuries.
return. Almost all patients with a complete
cord lesion will not show functional recovery. Lateral cervical spine x-ray should be
taken during the primary survey. It should
ensure that the junction between C7 and T1
is seen. Anterio-posterior and odontoid peg
views should be taken during the secondary

330 Chapter 21 Neurosurgery

Assessment of level of spinal injury

Muscle group Nerve supply Reflex
Biceps jerk
Diaphragm C3, C4, C5 Supinator jerk
Shoulder abductors C5 Triceps jerk
Elbow flexors C5, C6
Supinators/pronators C6 Knee jerk
Wrist extensors C6
Wrist flexors C7 Ankle jerk
Elbow extensors C7 Bulbocavernosus reflex
Finger extensors C7 Anal reflex
Finger flexors C8
Intrinsic hand muscles T1
Hip flexors L1, L2
Hip adductors L2, L3
Knee extensors L3, L4
Ankle dorsiflexors L4, L5
Toe extensors L5
Knee flexors L4, L5, S1
Ankle plantar flexors S1, S2
Toe flexors S1, S2
Anal sphincter S2, S3, S4

Table 21.2  Assessment of level of spinal injury

survey. If unable to see the C7/T1 junction • Compression of a vertebral body more
consider a ‘swimmer’s view’. On a lateral than 25%
cervical spine film it is necessary to assess:
• Kyphotic angle of more than 10%
• Anterior vertebral alignment • Facet joint widening
• Posterior vertebral alignment • Teardrop fracture
• Posterior facet joint margins • Base of odontoid peg fracture
• Anterior border of spinous processes • Atlanto-axial gap more than 3 mm
• Posterior border of spinous processes • Atlanto-occipital dislocation
• Integrity of vertebral bodies, laminae,
Various reports confirm a higher sensitivity and
pedicles and arches specificity of CT sacnning versus conventional
• Pre-vertebral space plain films in cervical spine injury The chance
• Retropharyngeal space should be less than of finding additional information, like bony
ligamentous avulsion or dorsal arch fractures is
6 mm substantially higher with CT.
• Retrotracheal space should be less than
Management
22 mm
• Interspinous gaps A spinal cord injury is a medical emergency
requiring immediate treatment to reduce
Radiological signs of spinal instability
include:

Neurosurgical disorders 331

the long-term effects. The time between • Posterior communicating artery (30%)
the injury and treatment is a critical factor • Anterior communicating artery (25%)
affecting the eventual outcome. Steroids • Middle cerebral artery (25%)
may be used to reduce the swelling of the
spinal cord. Decompressive surgery may Most aneurysms remain asymptomatic but
be necessary to remove bony fragments or they are a common cause of sudden death.
stabilise the spine. About 15% of aneurysms are multiple.

The rehabilitation process following a Clinical features
spinal cord injury should begin in the acute
care setting. Physiotherapists, occupational The classic presentation of a subarachnoid
therapists, social workers, psychologists and haemorrhage is with a sudden onset of
other healthcare professionals work as a team a severe headache often associated with
to determine an individualised management nausea, vomiting, photophobia and neck
plan. In the acute phase, management stiffness. Neurological symptoms and signs
focuses on the patient’s respiratory status, may be present. The level of consciousness
prevention of complications, maintaining the may be reduced. Fundoscopy may show
range of motion and muscle bulk. subhyoid haemorrhages. The clinical
course is unpredictable. Overall mortality is
Complications of a spinal cord injury approximately 40%. Many patients die before
include: reaching hospital.

• Deranged blood pressure control Investigation
• Chronic kidney disease
• Deep vein thrombosis The diagnosis can often be confirmed by an
• Pulmonary infections early CT or MRI. CT has a sensitivity of 90%
• Pressure sores if performed within the first 24 hours. The
• Contractures sensitivity is reduced to 50% by 72 hours
• Urinary tract infections as blood is reabsorbed. The CT may also
• Incontinence identify the source of haemorrhage. Fluid-
• Loss of sexual function attenuated inversion recovery (FLAIR) is
• Muscle spasticity the most sensitive MRI pulse sequence for
the detection of subarachnoid hemorrhage.
Neurosurgical disorders On FLAIR images, blood appears as high
Subarachnoid haemorrhage signal-intensity in normally low signal-
intensity CSF spaces. MRA may be useful for
Subarachnoid haemorrhage accounts evaluating aneurysms and other vascular
for approximately 5% of cerebrovascular lesions. If the diagnosis is in doubt then
accidents. The outcome depends on lumbar puncture may be indicated and will
the degree of neurological deficit. The show uniform blood-staining of CSF and
lower the GCS on presentation, the xanthochromia.
worse the prognosis. About 70% are due
to berry aneurysms. The remainder are Complications
due to arteriovenous malformations and
hypertension. The major complications of a subarachnoid
haemorrhage are:
Pathology
• Rebleed
Berry aneurysms are found in 8% of • Delayed ischaemic neurological deficit
individuals at post mortem. They are thin • Hydrocephalus
walled saccular aneurysms found at arterial
bifurcations on the Circle of Willis. They The risk of rebleed is 4% at 24 hours, 25% at
occur due to turbulent flow and damage to 2 weeks and 60% at 6 months. Rebleeding
the internal elastic lamina of the intracranial is associated with a 60% mortality. Delayed
arteries. The commonest site of aneurysms are: ischaemic neurological deficit (DIND) is
due to intense vasospasm. Treatment is
by maintaining cerebral perfusion with

332 Chapter 21 Neurosurgery

adequate hydration. Hydrocephalus results Clinical tests
from impaired CSF reabsorption through
arachnoid villi and 10% of patients will • Confirmation of absent brainstem reflexes
require CSF diversion or shunting. • Confirmation of persistent apnoea

Management Clinical tests should be performed by two
experienced practitioners, at least one of
In patients fit for surgery, the aneurysm whom should be a consultant. Neither
should be clipped at craniotomy. The aim should be part of the transplant team. The
is to clip the neck of the aneurysm whilst tests should be performed on two separate
maintaining flow in the native vessel. It occasions. There is no prescribed time
may also be embolised endovascularly with interval between the two tests.
platinum coils. The timing of intervention
is controversial. Vasospasm is usually Clinical tests for absent brainstem reflexes
greatest at 5 days after the bleed. Surgery
has traditionally been deferred until 10 days • No pupillary response to light
after the initial bleed but patients may die • Absent corneal reflex
as a result of rebleed during this period. • No motor response within cranial nerve
Early surgery or coiling may be associated
with reduced mortality and no increased distribution
morbidity. • Absent gag reflex
• Absent cough reflex
Brainstem death • Absent vestibulo-ocular reflex

Brainstem death is a clinical concept, Test for confirmation of persistent apnoea
implying an unconscious patient, with
irreversible apnea and loss of brainstem • Preoxygenation with 100% oxygen for 10
reflexes. The concept defines the core minutes
physiological basis for the neurological
diagnosis of death in the UK. All modern • Allow PaCO2 to rise above 5.0 kPa before
neurological criteria of death are primarily test
met through clinical tests of brainstem
function. These tests seek to ascertain • Disconnect from ventilator
that brainstem reflexes, motor responses, • Maintain adequate oxygenation during test
and respiratory drive are absent in a • Allow PaCO2 to climb above 6.65 kPa
normothermic comatose patient with a • Confirm no spontaneous respiration
known irreversible massive brain lesion and • Reconnect ventilator
no contributing metabolic derangements.
Central nervous
Preconditions system infections

• Diagnosis compatible with brainstem Intracranial abscess
death
Intracranial abscesses are rare but require
• Presence of irreversible structural brain prompt recognition and treatment is
damage required. Delayed diagnosis often results
in disability or death. Abscesses can be
• Presence of apnoeic coma extradural, subdural or intracerebral. Most
are bacterial infections that reach the central
Exclusions nervous system by:

• Therapeutic drug effects (sedatives, • Inoculation from a penetrating wound
hypnotics, muscle relaxants) • Spread from adjacent infective focus (e.g.

• Hypothermia (Temp >35°C) otitis media, sinusitis)
• Metabolic abnormalities • Blood-borne spread from distant focus
• Endocrine abnormalities
• Intoxication (e.g. endocarditis, lung abscess)

In 20% of patients, no source of infection
is identified. In the UK, otitis media and
sinusitis are the commonest causes of
intracranial abscesses.

Neurosurgical disorders 333

Pathology Management

From a local focus of infection, bacteria can The principles of treatment of intracranial
penetrate the skull through the diploeic veins. abscess are:
Also, local osteomyelitis may result in venous
sinus thrombosis. As the dura is normally • Drain intracranial collection
a good barrier to the intracranial spread of • Administer effective antibiotic therapy
infection, pus in the extradural space usually • Eliminate primary source of infection
causes an extradural empyema. Subdural
sepsis causes oedema and cortical venous Supratentorial abscesses can be drained via
thrombosis. Brain penetration causes an a burr hole. Pus should be aspirated and
early diffuse cerebritis. A localised abscess sent for culture. Clinical progress can be
may develop with oedema and increased monitored by serial CT scans. Stereotactic
intracranial pressure. The abscess usually drainage may be required for multiple
forms in the subcortical white matter near to or multiloculated abscesses. Cerebellar
the septic focus. Haematogenous abscesses abscesses may require a suboccipital
may be multiple. craniectomy and open drainage. Subdural
empyemas are often diffuse and difficult
Clinical features to drain and may require craniectomy and
open drainage. Parenteral antibiotic should
Intracranial abscesses can affect any be administered for at least 2 weeks. The
age or sex. The systemic upset is often choice of antibiotics depends on the primary
mild. Symptoms of increased intracranial pathology and antibiotic sensitivities.
pressure include headache and vomiting,
often associated with progressive clouding Prompt treatment results in a mortality
of consciousness. As the abscess develops, of less than 10%. Delayed treatment results
focal neurological symptoms may evolve. in mortality greater than 50%. About 50% of
As a result, symptoms of increased survivors have neurological sequelae including
intracranial pressure associated with hemiparesis, visual field losses and epilepsy.
focal neurological signs requires urgent
neurosurgical assessment. The differential Spinal abscess
diagnosis includes meningitis or an
intracranial tumour. Osteomyelitis due to Spinal abscesses are usually bacterial.
frontal sinusitis or middle ear disease may Infection arises in adjacent bone or by
produces localised swelling (Pott puffy haematogenous spread. The commonest
tumour). organisms are staphylococcal and
streptococcal species. Pus is usually
Investigation confined to extradural space. Subdural and
intramedullary infections are rare.
MRI, with and without gadolinium
enhancement, is the investigation of Clinical features
choice. It is more specific than CT scanning
in differentiating a cerebral tumour, The patient is often systemically unwell and
haematoma and abscess. On contrast may present with severe thoracic or lumbar
enhanced CT scanning, a cerebral abscess back pain at the level of the abscess. The
appears as radiolucent space occupying pain is worse on movement and associated
lesion with ring enhancement of the capsule. with marked muscle spasm and vertebral
It is often surrounded by considerable tenderness. Radicular signs are often present
cerebral oedema. The position, size and at the level of the lesion. Cord compression
number of abscesses may suggest the results in long tract signs. Thrombophlebitis
underlying pathology. Lumbar puncture is can cause cord vessel thrombosis and
contraindicated as in the presence of raised cord infarction, presenting with complete
intracranial pressure it can precipitate paralysis, sensory and sphincter loss.
tentorial or tonsillar herniation.
Investigation

Serum white cell count, ESR and CRP are
invariably raised. X-rays are often normal but

334 Chapter 21 Neurosurgery

may show soft tissue swelling or vertebral neurosurgical assessment is required. If
collapse. MRI is the investigation of choice. vertebral body collapse occurs, consideration
should be given to anterior decompression
Management and stabilisation. If no vertebral collapse
A high index of suspicion is required to is seen then laminectomy or CT-guided
make the diagnosis. Once identified, prompt aspiration may be appropriate.

Chapter 22 Trauma and
orthopaedic
surgery

Applied basic sciences Through these cellular extensions, nutrients
Bone and wastes are exchanged between the
osteocytes and the blood vessels. Perforating
Bone has several functions including: or Volkmann’s canals provide channels that
allow the blood vessels, that run through the
• Calcium homeostasis central canals, to connect to the blood vessels
• Haemopoesis in the periosteum that surrounds the bone.
• Structure and protection of internal organs
• Movement Trabecular bone

Compact bone Trabecular or spongy bone consists of thin,
irregularly shaped plates called trabeculae.
Compact bone is found in the shafts of long These are arranged in a latticework network.
bones and the outside surfaces of some other Trabeculae are similar to osteons in that both
bones. It makes up 80% of the total body have osteocytes in lacunae. As in compact
mass. It consists of cylindrical units called bone, the canaliculi present in trabeculae,
osteons (Figure 22.1). Each osteon contains provide connections between osteocytes.
concentric lamellae of a calcified matrix with Each trabecula is only a few cell layers thick
osteocytes lodged in lacunae between the and each osteocyte is able to exchange
lamellae. Smaller canals, or canaliculi, radiate nutrients with nearby blood vessels.
outward from a central canal – Haversian
canals. These contains blood vessels and Microstructure of bone
nerve fibres. Osteocytes within an osteon
are connected to each other and to the Bone matrix or osteoid has both organic
central canal by fine cellular extensions. and inorganic components. The organic

Structure of compact bone Figure 22.1  Structure of compact
bone

Lacunae Lamellae
Haversian canal Canaliculi

Osteon

Osteon of
compact bone

Periosteum Trabeculae of
spongy bone

Haversian canel

Volkmann’s canal

336 Chapter 22  Trauma and orthopaedic surgery

component is composed of Type 1 collagen cartilage in synovial joints. It is also found in
and proteoglycans. The inorganic matrix is the centres of ossification in growing bone.
mainly made up of calcium and phosphate Elastic cartilage is similar to hyaline cartilage
salts. Osteoblasts secrete organic bone matrix but contains more elastin bundles. It is found
or osteoid. They become osteocytes when in the pinna, eustachian tube and epiglottis.
they are surrounded by the bone matrix. Fibrocartilage is a tougher form of cartilage
Osteoclasts are multinucleate cells derived found in intervertebral discs and symphyses.
from monocytes. They actively resorb bone.
Periosteum covers bones and contains blood Nerves and action potential
vessels, nerves and lymphatics. Its inner
surface is osteogenic and contains both Structure of neurones
osteoblasts and osteoclasts.
Nerve cells are designed to respond to stimuli
Ossification and transmit information over long distances.
They have three parts (Figure 22.2). The cell
Bone undergoes either intramembranous body has a single nucleus and is responsible
or endochondral ossification. Both result in for most of nerve cell metabolism, especially
the same microstructure. Intramembranous protein synthesis. Proteins made in the cell
ossification is directly from mesenchyme body must be delivered to other parts of the
and occurs in flat bones such as the skull. nerve by an axonal transport system. The
Endochondral ossification is the development axon is designed to transmit an electrical
of bone through an intermediate cartilage impulse and can be several meters long.
stage and occurs in long bone. Dendrites receive impulses from other
nerves.
Cartilage
The action potential
Cartilaginous matrix makes up over 80%
of cartilage and is primarily composed of Neurones transmit information as action
elastin, collagen and proteoglycans. These potentials. An action potential is a temporary
are large molecules with a protein backbone change in the membrane potential, usually
and glycosaminoglycan side chains. initiated in the cell body. It normally travels
Chondrocytes are mature cartilage cells in one direction and is conducted in an ‘all-
and are scattered throughout the cartilage or-nothing’ fashion. If the stimulus is too low,
matrix. Hyaline cartilage is made up of mainly there is no action potential. If the stimulus
Type 2 collagen and functions as articular is above a threshold, the action potential,
is always the same size. During an action

Structure of a neurone

Dendrites Schwann cell nucleus
Cell body

Axon terminal

Nucleus

Axon Myelin sheath Node of Ranvier

Figure 22.2  Structure of a neurone