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Published by irianewsletter, 2021-03-21 07:50:45

22nd Mar 2021

case of the week

Keywords: Case,week,IRIA,ICRI

ICRI CASE OF THE WEEK

Contributed By :

Dr M K Pooja, Dr. Sanjay P, Dr. Pradeep H N, Dr Hemanth Kumar G S,
Dept of Radiodiagnosis, Mysore Medical College & Research Institute, Mysore

Copyright of the Case belongs to : Dr Sanjay P.

Clinical Details

• 18 year old male patient
• c/o Pain abdomen and vomiting for 2 days
• Fever for 2 days
• No h/o TB, diabetes mellitus
• No h/o previous surgery

Ultrasound Abdomen

Midline transverse liver

Splenunculus in right hypochondriac region

SMA SMV

Reversal of SMA and Midline tubular aperistaltic
SMV relation non-compressible structure

Barium Meal Follow Through and Barium Enema

Right sided Small bowel loops clustered
stomach in left quadrants and large
bowel loops on right side

Non-rotation of bowel

CECT Abdomen

Midline
transverse
liver

Midline
appendix with
appendicolith

Right sided
stomach

Polysplenia

Diagnosis

LEFT ISOMERISM - SITUS AMBIGUUS WITH POLYSPLENIA
& ACUTE APPENDICITIS

Discussion

HETEROTAXY SYNDROME

Heterotaxy is a laterality defect characterized by isomerism of the thoracic organs and
random arrangement of the abdominal organs.
Historically, on the basis of splenic anatomy classified into

Asplenia Polysplenia

Now, better segregated on the basis of isomerism of the atrial appendages rather than
splenic anatomy as it is the atrial appendage morphology that is the most constant feature
and offers the most consistent “syndromic clustering”.

➢Normal situs : Aortic arch, cardiac apex, and stomach bubble are all
located on the left (A)

➢Situs inversus: When these structures are positioned on the right or
reversed.(B)

➢Indeterminate situs or heterotaxy: When any other situation is seen
(B)

Characteristics

• Right isomerism : Asplenia syndrome Drawing shows the typical
-More complex cardiovascular malformations anatomic features of classic
-Absence of the spleen Asplenia: trilobed lungs with
-Bilateral trilobed lungs bilateral minor fissures and
-Total anomalous pulmonary venous connections eparterial bronchi, bilateral
-Intestinal malrotation systemic atria, midline liver,
absent spleen, and variable
• Left isomerism: Polysplenia syndrome location of the stomach
-Less complex cardiovascular malformations
-Multiple spleens Drawing shows the typical
-Bilateral bilobed lungs anatomic features of classic
-Interruption of the inferior caval vein Polysplenia: bilobed lungs with
bilateral hyparterial bronchi,
Arrhythmias Either bilateral pulmonary atria, midline
Thrombocytosis liver, and multiple spleens located
along the greater curvature of the
stomach, which occurs in variable
locations.

Either

HEART

• Asplenia-associated Heterotaxy syndrome :
-Very high mortality rate due to frequent and severe
associated cardiac malformations
-Common atrioventricular canal
-Univentricular heart
-Transposition of large vessels
All of them tend to present with Anomalous pulmonary
venous drainage

• Polysplenia associated Heterotaxy syndrome
-Cardiac malformations are less frequent and milder
-Patients with left isomerism present with morphologically left atrial appendages (long
and narrow) and receive pulmonary veins
-Aortic stenosis

SPLEEN

Absence of spleen in the syndrome of heterotaxy with asplenia causes high mortality
before the first year of life due to its greater association with severe congenital heart
disease and decreased immunological activity

AORTA

• Normal position in most of the cases
• In some cases, located to the right of the midline when it coexists

with a duplicated IVC

Duplication of IVC in a
case of Situs ambiguuus
with Polysplenia

LIVER

• As much in polysplenia or asplenia, midline liver is a very common
finding. However, it appears more frequently in patients with
asplenia.

• We found midline liver in our patient, and hepatic artery originating
from the superior mesenteric artery as a variant of normal.

CT scan of upper abdomen reveals a midline liver

GASTROINTESTINAL TRACT

• Intestinal malrotation is common in heterotaxy syndrome. The stomach may be
in a normal or mirror location

• Regarding the small bowel and colon, different degrees of intestinal
malrotation may be observed. The most common finding described is the small
bowel to the right of the midline and the colon to the left

• The preduodenal position of the portal vein has been described in the literature,
which might cause intestinal obstruction

Inversion of the superior
mesenteric artery (SMA) and
superior mesenteric vein
(SMV)

Treatment and Prognosis

• The management of adults with polysplenia is generally palliative and
symptom-specific. Most adult patients with heterotaxy syndrome with
polysplenia do not present with any specific symptoms and are
incidentally diagnosed.

• The existence of associated anomalies such as a preduodenal portal vein,
intestinal malrotation or vascular anomalies can condition the surgery.
Its awareness is important to avoid complications in interventional
procedures.

Conclusion

• So-called “Heterotaxy” involves multiple systems.
• Terms, such as “Asplenia” or “Polysplenia”, are no longer adequate to

account for the overall constellation of findings, nor is labeling in terms of
“Situs ambiguus”.
• It is better simply to describe each system in explicit detail. This allows for
anticipation of functional abnormalities, as well as any interventions that may
be deemed necessary.
• From the stance of intracardiac lesions, the distinction of right as opposed to
left isomerism will permit better comparison between patients, and will point
to the need specifically to address the morphology of the atrial appendages.
Such a distinction is most important when assessing genotypic variations.

References

• Heterotaxy syndrome in the adult: a review of its radiological findings and
associated genetic alterations-ECR 2019 / C-273

• Radiologic Considerations in Heterotaxy: The Need for Detailed Anatomic
Evaluation, doi: 10.7759/cureus.470

• Situs Revisited: Imaging of the Heterotaxy Syndrome,
https://doi.org/10.1148/radiographics.19.4.g99jl31837


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