January 2014

Evolution

patient who had unbearable diplopia in 1979. Fechner and 6. Bores LD, Myers W, Cowden J. Radial keratotomy: an analysis of the
Worst introduced a phakic myopia lens of iris claw design American experience. Ann Ophthalmol 1981;13(8):941-948.
in 1986.
Most refractive surgeons agree that safety and accuracy of 7. Grady FJ. Experience with radial keratotomy. Ophthalmic Surg
keratorefractive surgery declines in eyes having more than 1982;13(5):395-9.
10 diopters of myopia or 4 diopters of hyperopia. For these
patients, intraocular lens surgeries may be an option first 8. Hecht SD. Radial keratotomy: Is Professor Fyodorov correct? Ann
FDA approved in 2004, Phakic Intraocular Lenses (PIOLs) Ophthalmol 1982;14(4):313-314.
are available to treat myopic eyes up to 20 diopters.
9. Hoffer KJ, Darin JJ, Pettit TH, et al. UCLA clinical trial of radial
Two FDA approved phakic IOLs currently are keratotomy. Preliminary report. Ophthalmol 1981;88(8):729-736.
available
Visian ICL. The Visian ICL (Implantable Collamer Lens) 10. Hoffer KJ, Darin JJ, Pettit TH, et al. Three years experience with
marketed by Staar Surgical is a posterior chamber phakic radial keratotomy. The UCLA study. Ophthalmol 1983;90(6):627-
IOL. It received FDA approval in 2005 for correcting 636.
nearsightedness ranging from -3.00 to -20.00 D
Verisyse. The Verisyse (Abbott Medical Optics) is an 11. Nirankari VS, Katzen LE, Karesh JW, et al. Ongoing prospective
anterior chamber phakic IOL. In 2004, the Verisyse phakic clinical study of radial keratotomy. Ophthalmol 1983;90(6):637-
IOL received FDA approval for correcting moderate to 641.
severe nearsightedness within the range of -5.00 to -20.00
diopters 12. Nirankari VS, Katzen LE, Richards RD, et al. Prospective clinical
The Visian ICL and Verisyse phakic IOL are FDA approved to study of radial keratotomy. Ophthalmol 1982;89(6):677-683.
correct myopia. Clinical trials for both lenses are continuing
for potential FDA approval for treating hyperopia. FDA 13. Schachar RA. Indications, techniques, and complications of radial
approval for a toric version of the Visian ICL is awaited. keratotomy. Int Ophthalmol Clin 1983;23(3):119-128.
Also pending FDA approval is the AcrySof Cachet angle-
supported phakic IOL (Alcon). The Cachet is a soft acrylic 14. Stark WJ, Martin NF, Maumenee AE. Radial keratotomy. II. A
lens positioned in front of the iris and secured in the angle. risky procedure of unproven longterm success. Surv Ophthalmol
The Cachet IOL has been approved for use in Europe since 1983;28(2):101, 6-11.
2008. Clinical studies show mean refraction improved
from -10.00 D to -0.25 D (which has remained stable over 15. Steel DL. Radial keratotomy. Arch Ophthalmol 1983;101(1):134.
the five years of follow-up), and more than 96 percent of 16. Steele AD, Buckley RJ, Sherrard ES. Early experiences with radial
patients were satisfied with the results of the Cachet IOL
procedure at one and five years after surgery. keratotomy. Trans Ophthalmol Soc U K 1982;102 (Pt 1):35-41.
17. Waring GO. Radial keratotomy for myopia. South Med J
Conclusion
The field of refractive surgery have come a long way from 1981;74(1):1-3.
the era of Sato and has not just improved vision but also the 18. Waring et al. Ophthalmology 1991 Aug;98(8):1164-76
quality of life in millions of people. Extensive research and 19. Taboada J, Mikesell GW Jr, Reed RD. (1981)“Response of the corneal
technological advances have made these procedures safer,
providing much better visual results. epithelium to KrF excimer laser pulses”. Health Phys. 40: 677-83
20. Trokel SL, Srinivasan R, Braren B. (1983) “Excimer laser surgery of
References
the cornea”. Am J Ophthalmol. 96: 710-715
1. Snellen, H.A. (1869) “Die rishtunge des hauptmeridiane des 21. Srinivasan R. (1983) “Ablation of polymers and biological tissue by
astigmatischen auges”. Albrecht von Graefe’s Arch Klin. Exp.
Ophthalmol. 15: 199-207 ultraviolet lasers”. Science. 234: 559-565
22. C.R. Munnerlyn, S.J. Koons and J. Marshall.(1988) “Photorefractive
2. Akiyama K, Tanaka M, Kanai A, Nakajima A. Problems arising from
Sato’s radial keratotomy procedure in Japan. CLAO J 1984;10(2):179- keratectomy: a technique for laser refractive surgery”. J Cataract
184 Refract Surg. 14: 46–52
23. Pallikaris IG, Papatzanaki ME, Stathi EZ, et al. (1990) “Laser in situ
3. Anwar, M. and K. Teichmann. Cornea 2002;21(4):374–383 keratomileusis”. Lasers Surg Med. 10(5): 463-8.
4. National Advisory Eye Council: letter to the editor. Radial 24. Buratto L, Ferrari M, Rama P. (1992) “Excimer laser intrastromal
keratomileusis”. Am J Ophthalmol. 113: 291-5.
keratotomy. Am J Ophthalmol 1980;90(5):741-742. 25. Kurtz RM, Liu X, Elner VM, et al. (1997) “Photodisruption in the
5. Bores LD. Radial keratotomy. I. A safe, effective way to correct a human cornea as a function of laser pulse width”. J Refract Surg.
13:653–658.
handicap. Surv Ophthalmol 1983;28(2):101-105. 26. Burris TE. Intrastromal corneal ring technology: results and
indications. Curr Opin Ophthalmol 1998 Aug;9(4):9-14.
27. Sekundo et al. JCRS Volume 34, Issue 9, September 2008, Pages
1513–1520.
28. Sekundo et al. Br J Ophthalmol 2011;95:335-339 doi:10.1136/
bjo.2009.174284
29. Strampelli et al. Tolerance of acrylic lenses in the anterior chamberan
refraction disorders. Ann. Ottalmol. Clin. Ocul. 80(2), 75-82(1954).
30. Barraquer J. Anterior chamber plastic lenses. Trans. Ophthalmol.
Soc .UK, 393-424 (1959).
31. Worst JG. Iris claw lens. J Am. Intraocul. Implant. Soc 6(2), 166-
167(1980).

www. dosonline.org l 69

Preoperative Workup for PG PCGoCronrneer
LASIK and Phakic IOL
Vijay Kumar Sharma
Vijay Kumar Sharma MS, Tarun Arora MD MS
Dr. Rajendra Prasad Centre for Ophthalmic Sciences,

All India Institute of Medical Sciences, New Delhi

The term ‘refractive surgery’ describes various elective or the old spectacles themselves so that refractive stability
procedures that modify the refractive status of eye. may be assessed.
Procedures that involve altering the cornea are collectively Inclusion criteria- As a general guideline, LASIK is done for
referred to as keratorefractive surgery or corneal refractive patients with myopia up to -10 D, Hyperopia up to +4D
surgery. Other refractive surgery procedures include and astigmatism up to 4D (Figure 1&2).
placement of an intraocular lens (IOL) implant, either in
front of the crystalline lens (phakic IOL) or in place of Figure 1&2: Laser ablation in progress
crystalline lens (refractive lens exchange)1. Refractive
surgery may be considered when a patient wishes to be less
dependent on eyeglasses or contact lenses, due to cosmetic
or occupational reasons.
Refractive surgeon faces three major challenges: to select
the right patient, to select the right procedure and to
achieve the right outcome. To ensure a favourable outcome
preoperative workup is the key to success.
Preoperative evaluation for LASIK
Preoperative work-up consists of—
• Screening
• Preoperative examination
• Counselling
Screening
Purpose of screening is to eliminate patients who are clearly
not candidates for refractive surgery2. Areas of inquiry may
include age and the type and degree of refractive error.
Although all excimer lasers perform grossly the same tasks,
their approved ranges of photorefractive keratectomy and
laser in situ keratomileusis (LASIK) differ. Contact lenses
must be removed prior to the preoperative examination (4
weeks for hard or rigid gas-permeable lenses and 7–14 days
for soft contact lenses). In addition, patients must be asked
to bring copies of old spectacle prescriptions if available

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Exclusion criteria Table 1: Systemic Medication causing dry eye
• Ectatic corneal disease eg keratoconus, terrien or
Antihypertensives Clonidine, Methyldopa,
pellucid marginal degeneration etc. Prazosin, Propranolol,
• Thin corneas Reserpine
• Active ocular infection
• Ocular surface disorders including dry eye syndrome Antispasmodics Cyclobenzaprine,
• Glaucoma (especially if a large bleb is present) Methocarbamol
• Blepharophimosis
• Monocular patients Cardiac Medications Disopyramide, Mexiletine
• Systemic or retinal vascular disorder
• Autoimmune disease Decongestants Ephedrine, Pseudoephedrine
• Pregnancy
Preoperative Examination Dermatologic Isotretinoin9
The preoperative examination consists of— Medications
• History
• Examination Gastrointestinal Metoclopramide
Medications
History
Patient age- The youngest acceptable age is about 18 Oral Contraceptives
years as stable refraction is required for elective refractive
surgery. No biological upper age limit exists for excimer Parkinson Medications Benztropine, Biperiden,
laser corneal surgery but early cataractous lens changes Procyclidine,
make intraocular lens implantation a better choice. Trihexyphenidyl(benzhexol)
History of contact lens use- Patients who wear rigid
contact lenses should be advised that it can take weeks to Psychiatric Amitriptyline, Amoxapine,
months for their corneas to relax into their natural state and Medications Desipramine, Doxepin,
they should be kept on follow up till their eye examination Imipramine, Nortriptyline,
becomes stable. Similarly patients wearing spherical/toric Phenelzine, Tranylcypromine,
soft contact lenses should be advised that it may take few Trimipramine
weeks for their eye examination to become stable after they
stop wearing contact lens. Typically patients wearing toric Pregnancy- Pregnant patients, and those who are in early
soft contact lenses will require more time for their corneas postpartum period or even those who are breast feeding,
to adjust than patients who wear spherical soft contact should be warned that they are at higher risk of being
lenses. over or undercorrected with laser refractive surgery8. This
Ocular herpes simplex virus- Patients with ocular HSV, appears to be due to increased levels of various hormones,
even if inactive for long time, can have recurrence following which can affect the healing process of surgery as well
exposure to excimer laser3-7. Hence many surgeons advise as the stability of the refractive end point before surgery.
against LASIK in these patients. These patients should be advised to defer surgery.
Monocular patients- Patients with poor vision in one eye Participation in high risk activities- Patients who are active
should be discouraged from elective refractive surgery. in martial arts, contact sports and other high risk activities
History of strabismus- Patients with history of strabismus should be informed that with LASIK there is chance of
during childhood have developed a recurrence of flap injury. Therefore one might consider surface ablation
strabismus following LASIK and these patients should be procedures to avoid the potential of a traumatic flap injury.
warned that they are at increased risk of developing eye Medicines- There are variety of medications which
muscle problems after LASIK. may affect the outcome of refractive surgery. Systemic
Medications that May cause dry eye are depicted in
(Table 1).
Medical conditions- Certain medical conditions can affect
healing. Diabetes, active autoimmune diseases (lupus,
rheumatoid arthritis) and immunodeficiency states (HIV)
can all potentially affect the outcome of refractive surgery
as these conditions can alter wound healing.

Examination
Visual acuity- Uncorrected and best corrected visual acuity
is recorded for distance and near.
Refraction- A cycloplegic and manifest refraction is done in
all patients. A stable refraction is essential usually <0.50D

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Figure 3: Keratoconus transient, this distortion may persist for up to 5 months.
For this reason, it is advisable for a patient to discontinue
change over last 02 years is acceptable. Refraction need to contact lens wear before the preoperative examination—
be performed after adequate time after removal of contact for at least 4 weeks for rigid lenses and 2–14 days for soft
lenses, usually 04 wks after RGP lenses and 01 wk after soft lens wearers. If topographic examination is suggestive of
contact lenses. corneal moulding, a longer period of contact lens cessation
Contrast sensitivity- Contrast sensitivity can be used in is warranted until the topography stabilizes.
addition to Snellen visual acuity to characterize patient’s Posterior ectasia can be identified by posterior elevation
visual function. Many studies have shown that contrast map as in Orbscan and in general any area >40 microns
sensitivity can be reduced following LASIK and PRK. is suspicious and >50 microns is highly suspicious of
Keratometry- Power and axis of steepest and flattest posterior ectasia.
meridian is determined by keratometry. It is done to find out Pupil examination- The evaluation of pupil size in darkness
axis and magnitude of corneal component of astigmatism. is an important component of thorough preoperative
It can also detect irregular astigmatism which characterises examination. Patients with large pupils (>7mm) are
subclinical keratoconus. Possibility of a free flap increases potentially at a higher risk of postoperative signs of glare,
in flat cornea (K value<40D) and buttonhole in steep halos and other night time symptoms compared to patients
cornea (K value >46D). of similar refractive error but normal size pupils and may
Computerized Videokeratography - Computerized indicate need for larger treatment and transition zone.
videokeratography is absolutely essential in the preoperative Slit lamp evaluation- Particular attention should be given to
evaluation of patients for refractive surgery10-11. It is the only the health of the tear film and lid margin. Mild intolerance
way to uncover early or mild keratoconus. The population to contact lens wear caused by mild dry eye can be a
of patients who seek refractive surgery seems to carry an reason patient wanting refractive surgery. Cornea should
increased incidence of keratoconus (3–5%), perhaps as be examined for any anterior membrane dystrophy, stromal
much as 10 times that of an expected random sample of scarring or vascularisation that may influence the type of
myopes. These patients are probably a self-selected group surgery or whether it should be done at all. Any stromal
for both keratoconus and dry eye because both pathologies thinning as in keratoconus, pellucid marginal degeneration
manifest as contact lens intolerance. and terrien marginal degeneration should alert the
It is likely that many patients who exhibit keratoconus- surgeon. Descemets membrane haze or guttata suggest
like patterns that are undetectable by other means and an abnormality of endothelial dysfunction. Evaluation of
cause no symptoms (so-called keratoconus suspects) crystalline lens is critical as patients with cataracts should
remain subclinical (Figure 3). Only long-term follow- be advised that corneal surgery may not be their best
up differentiates these patients from those who go on to surgical option.
progressive ectasia. However, Seiler and Quurke12 reported Fundus examination- A complete dilated fundus
a case of a patient with forme fruste keratoconus progressing examination should be well documented.
to frank ectasia following LASIK. Therefore, these patients Tonometry- Preoperative intraocular pressure should be
should be avoided. recorded as success of refractive surgery can be influenced
Corneal moulding (warpage) is a transient corneal distortion by level of IOP.
produced primarily by rigid contact lens wear, but it may Pachymetry- Pachymetry must be performed prior to
result from soft contact lens wear as well13. Although LASIK14-15. It is mandatory that there be at least 250mm of
tissue in the bed after ablation. If this important parameter
is ignored, the patient is at risk for corneal ectasia and
an unstable refraction. Controversy exists as to whether
optical pachymetry as provided by the Orbscan device
may be substituted for ultrasonic pachymetry. Although
optical pachymetry has the advantage of providing an
array of corneal thickness measurements throughout the
cornea and therefore identifying areas of corneal thinning
outside the central cornea, recommendations for minimum
bed thickness have been based upon ultrasonic and not
optical devices. Furthermore, the Orbscan algorithm seems
to produce readings that are consistently higher than those
obtained by ultrasonic means.

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Endothelial cell evaluation- Routine endothelial cell Figure 4: Visian ICL –V4c
evaluation has not been found to be clinically relevant for
patients undergoing refractive surgery. Types of pIOLs in use presently are-
Dry eye testing- Patients to be evaluated for any dry eye 1. Anterior chamber angle supported e.g. Kelman duet,
problems preoperatively and should be treated for the
same as these are likely to worsen after LASIK16-18. Acrysof
Ocular dominance determination- Ocular dominance 2. Anterior chamber iris fixated e.g. Verisyse/Artisan,
determination is done preoperatively to choose the first eye
to be treated for the patients who will undergo refractive Veriflex/ Artiflex
surgery in one eye at a time. If both eyes are to be corrected, 3. Posterior chamber sulcus supported e.g. ICL (Figure-4),
it is prudent to begin with the non dominant eye to allow
any needed adjustments to the treatment parameters of the PRL
second eye. Inclusion and Exclusion Criteria- Generally recommended
Informed consent- Although there is high probability inclusion criteria include:
of successful outcome for keratorefractive surgery, care • Age >21 years
should be taken to emphasize potential adverse events or • Stable refraction for at least 1 year (less than 0.50 D
complications that may occur, explaining which may be
transient and which may be permanent. The patient should change)
be informed of the potential risks, benefits and alternatives • Ammetropia not correctable with excimer laser surgery
to and among the different refractive procedures before • Unsatisfactory vision with/ intolerance of contact
surgery. The informed consent process should be
documented and the patient should be given an opportunity lenses or spectacles
to have all questions answered before surgery. The surgeon • Irido-corneal angle >300
is responsible for obtaining the patient’s informed consent. • Central ECC >2300 cells/mm2 (>2500 cells/mm2 if
Counselling- Not all patients who meet medical and
ophthalmic criteria for refractive surgery are necessarily >21 years old,>2000 if >40 years old)
good candidates for the procedure. Patients must be told • No anomaly of iris or pupil function, mesopic pupil
that spectacles may be required for certain tasks such as
driving at night. After surgery, patients should not expect size <5.0–6.0 mm
to obtain perfect uncorrected distance acuity. Those who Generally recommended exclusion criteria include the
are dissatisfied with these projections are likely to be following:
dissatisfied after surgery. Presbyopic myopes must be made • Active disease in the anterior segment (recurrent or
aware that the removal of distance glasses to achieve a
near addition is no longer possible after refractive surgery. chronic uveitis, clinically significant cataract etc.)
However, if the patient’s lifestyle permits and the patient • Previous corneal or intraocular surgery (to be evaluated)
is motivated sufficiently, monovision may be a possibility. • IOP >21 mm Hg or glaucoma
The preoperative evaluation of the patient for refractive • Preexisting macular degeneration or macular pathology
surgery is lengthy and must be performed in an unrushed
manner. However, it is time well spent because the
best treatment for complications and disappointment is
avoidance.
Preoperative evaluation for phakic IOL
Although LASIK remains the dominant and preferred
refractive procedure, it is less desirable in highly myopic
eyes owing to concerns of ectasia and decrease in quality
of vision. Phakic IOLs (pIOLs) offer a promising alternative
for patients who are not ideal candidates for corneal
refractive surgery. This procedure offers excellent refractive
accuracy, reversibility and preservation of accommodation
without compromising visual quality due to alteration of
normal corneal aspheric contour.

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Figure 5: Correct vaulting of ICL as Intraocular Lens Power Calculation and Diameter
measured by ASOCT Selection
The theoretical basis of the power calculation for refractive
• Abnormal retinal condition, systemic diseases (eg, phakic iris-claw IOLs was proposed by Van der Heijde and
autoimmune disorder, connective tissue disease, Fechner. These principles are also transferable to angle-
diabetes mellitus) supported IOLs. To calculate IOL power, the patient’s
refraction, keratometric dioptric power at the corneal apex,
The preoperative evaluation of a patient for a pIOL is more and adjusted ultrasound central ACD are used. Based on
comprehensive than is required for keratorefractive surgery. the formula, the manufacturers provide nomograms or
The preoperative workup for pIOL should include manifest software to calculate the required pIOL power. Most users
refraction, cycloplegic refraction, Snellen uncorrected employ the formula proposed by Olsen et al for posterior
distance visual acuity (UDVA) and corrected distance visual chamber pIOLs power, which uses the patient’s refraction
acuity (CDVA), pupillometry, applanation tonometry, at the 12.0 mm spectacle plane or the vertex refraction,
ultrasound anterior chamber depth (ACD) measurement, the corneal keratometric dioptric power at its apex, and
corneal topography, pachymetry, central endothelial cell adjusted ultrasound central ACD, also known as the
count (ECC), and a fundus examination. effective lens position.
As with every operative procedure, the surgeon should The pIOL’s overall diameter depends on the ACD and
ensure that the patient receives proper informed consent. should provide perfect stability, with no unnecessary
Anterior-chamber depth compression forces on the angle that could damage the
The anterior-chamber depth (ACD) is a critical component angle structures or induce pupil ovalization.
to the safety of a pIOL procedure and should be assessed The white to white (WTW) distance can be measured
before surgery. A shallow anterior chamber can complicate manually (using the Holladay-Godwin gauge or a measuring
the insertion and placement of the pIOL as well as increase caliper) or by automated technology (IOL Master [Carl
the loss of endothelial cells. Zeiss Meditec], and Orbscan II topography system [Bausch
ACD Requirements (Measured from Endothelium) is as & Lomb]). Automated measurement of the WTW distance
under provides more precise results than manual methods. The
• ICL: 2.8 mm for myopia, 3.0 mm for hyperopia diameter of angle-supported pIOLs is oversized 0.5 mm to
• PRL: 2.5 mm 1.0 mm from the WTW measurement. Currently, with the
• AcrySof phakic: >2.7 mm advent of AS-OCT and UBM, the angle-to-angle distance
• Artisan-Verisyse/Artiflex-Veriflex: 2.7 mm and anterior chamber angle can be measured precisely20.
An assessment of the anterior - chamber angle configuration
is necessary for the placement of anterior-chamber lenses. Intraocular Lens Diameter Selection: ICL
Gonioscopy, ultrasound, or optical coherence tomography The overall diameter of ICL depends on the ciliary sulcus
can be used for this evaluation. In addition, a careful diameter and should provide perfect stability with no
examination of the iris should be a part of the preoperative excess of compression forces to the sulcus and allow
workup. correct vaulting19. Excessive vaulting (>750 microns)
due to an ICL that is too long may cause angle-closure
while insufficient vaulting <250 microns may lead to
cataractogenesis (Figure 5).
Before the development of UBM, no system that allowed
determination of the internal diameter of the ciliary sulcus
existed. This evaluation was approximated and depended
on a WTW measurement. The ICL’s diameter is oversized
0.5 to 1.0 mm from the WTW measurement in myopic eyes
and the same length or oversized 0.5 mm in hyperopic
eyes. Many recent studies have demonstrated that there
may be variations and no correspondence between WTW
measurements externally to internal dimensions and may
be the cause of some complications associated with pIOLs
and it needs further refinement.

References

1. Fernandez-Vega L, Alfonso JF, Villacampa T. Clear lens extraction for
the correction of high myopia. Ophthalmology 2003; 110:2349–54.

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2. Krachmer JH, Mannis MJ, Holland EJ. Decision making in refractive 11. Nesburn AB, Bahri S, Salz J, et al. Keratoconus detected by
surgery. Textbook of cornea, 3rd ed. 2011, p 1761-1779. videokeratography in candidates for photorefractive keratectomy. J
Refract Surg. 1995; 11:194–201.
3. Vrabec MP, Durrie DS, Chase DS. Recurrence of herpes simplex
after excimer laser keratectomy [letter]. Am J Ophthalmol. 1992; 12. Seiler T, Quurke AW. Iatrogenic keratectasia after LASIK in a case of
114:96–7. forme fruste keratoconus. J Cataract Refract Surg. 1998; 24:1007–9.

4. Bialasiewicz AA, Schaudig U, Draeger J, et al. Descemetocele after 13. Wilson SE, Lin DT, Klyce SD, et al. Topographic changes in contact
excimer laser phototherapeutic keratectomy in herpes simplex virus– lens–induced corneal warpage. Ophthalmology. 1990; 97:734–44.
induced keratitis: a clinico-pathologic correlation. Klin Monatsbl
Augenheilkd. 1996; 208:120–3. 14. Marsich MW, Bullimore MA. The repeatability of corneal thickness
measures. Cornea. 2000; 19:792–5.
5. Pepose JS, Laycock KA, Miller JK, et al. Reactivation of latent herpes
simplex virus by excimer laser photokeratectomy. Am J Ophthalmol. 15. Yaylali V, Kaufman SC, Thompson HW. Corneal thickness
1992; 114:45–50. measurements with the Orbscan Topography System and ultrasonic
pachymetry. J Cataract Refract Surg. 1997; 23:1345–50.
6. Tervo T, Tuunanen T. Excimer laser and reactivation of herpes
simplex keratitis [letter; comment]. CLAO J. 1994;20:152–3, 157. 16. Tervo T, Mustonen R, Tarkkanen A. Management of dry eye may
reduce haze after excimer laser photorefractive keratectomy [letter].
7. Dhaliwal DK, Romanowski EG, Yates KA, et al. Experimental laser- Refract Corneal Surg. 1993; 9:306.
assisted in situ keratomileusis induces the reactivation of latent
herpes simplex virus. Am J Ophthalmol. 2001;131:506–7. 17. Nelson JD. Diagnosis of keratoconjunctivitis sicca. Int Ophthalmol
Clin. 1994; 34:37–56.
8. Hefetz L, Gershevich A, Haviv D, et al. Influence of pregnancy and
labour on outcome of photorefractive keratectomy. J Refract Surg. 18. Fox RI. Systemic diseases associated with dry eye. Int Ophthalmol
1996; 12:511–12. Clin. 1994; 34:71–87.

9. Fraunfelder FT, LaBraico JM, Meyer SM. Adverse ocular reactions 19. Baikoff G, Lutun E, Ferraz C, Wei J. Static and dynamic analysis of
possibly associated with isotretinoin. Am J Ophthalmol. 1985; the anterior segment with optical coherence tomography. J Cataract
100:534–7. Refract Surg 2004; 30:1843–50.

10. Wilson SE, Klyce SD. Screening for corneal topographic abnormalities 20. Kojima T, Maeda M, Yoshida Y, Ito M, Nakamura T, Hara S, Ichikawa
before refractive surgery. Ophthalmology. 1994; 101:147–52. K. Posterior chamber phakic implantable collamer lens: changes in
vault during 1 year. J Refract Surg 2010; 26(5):327-32.

76 l DOS Times - Vol. 19, No. 7 January, 2014

MonthlyMoMntheleytMineegtinCgoCronrneerr

An Unusual Case of Anisha Seth
Choroidal Folds MS, DNB

Anisha Seth MS, DNB, Usha K. Raina MD FRCS, Gauri Bhushan MS, Neha Goel MS, DNB, FRCS,
Meenakshi Thakar MS, Basudeb Ghosh MD, DNB, MNAMS

Guru Nanak Eye Centre, New Delhi

Parry-Romberg syndrome is a rare disorder characterised Figure 1: Hemifacial atrophy at
by progressive hemifacial atrophy involving the skin, presentation with severe enophthalmos
subcutaneous tissue, muscles, cartilage and bone1, that
usually begins in the second decade of life2. This disease anterior chamber or vitreous cells in either eye. The pupils
progresses through an active phase of decompensation, were normal in size, shape and reaction and the ocular
generally lasting 2 to 10 years, followed by a quiescent phase movements were full and free. There was no strabismus.
without continued atrophy. The cause and pathophysiology The right fundus examination was unremarkable while the
of the syndrome are unknown. Ophthalmic involvement is left fundus examination showed choroidal folds (Figure 2).
seen in the form of progressive enophthalmos3, restrictive Intraocular pressure measured in both eyes with Goldmann
strabismus, heterochromia, pupillary disturbances, third applanation tonometer was 18mm of Hg. Axial length
nerve palsies and papillitis2,4. Other rare ocular features measurement of the eyes by A-scan biometry was 23.50
reported are scleral melting5, band shaped keratopathy6, mm in the right eye and 22.76 mm in the left eye. Hertel’s
primary corneal endothelial failure7, anisometropia, Exopthalmometry revealed a reading of 22mm in the right
ipsilateral cornea, iris, angle abnormalities, and juvenile eye and 16 mm in the left eye at 98 mm bar reading.
glaucoma8. Several retinal alterations leading to visual loss, Flourescein angiography (FA) of both eyes revealed a
like venous dilatation, disc edema, retinal folds and retinal normal arterio-venous filling with no leakage at the disc
edema has been described on clinical examination3,4 or macula (Figure 3). Spectral domain optical coherence
as well as on optical coherence tomography (OCT) and tomography (OCT) performed at the fovea with six radial
flourescein angiography (FA)9. We describe a case of Parry- scans revealed a normal study pattern with no intra retinal
Romberg syndrome with severe enophthalmos leading
to retinal alterations but with preserved visual acuity and
normal OCT and FA features.
Case Report
A 23 year old man presented with decrease in vision
in the left eye for 8 months. The general physical
examination revealed left sided enophthalmos (Figure 1)
that the patient noted 2 years back and was progressively
increasing. The patient did not give history of taking any
treatment for the same. There was no history of trauma,
any proceeding fever, associated headache, use of glasses,
previous treatment, ocular surgery or systemic illness On
ophthalmic examination, vision was 20/20 in the right eye
and 20/200 in the left eye that improved with +2.75DS to
20/20. The cornea was clear in both eyes and there was no

www. dosonline.org l 77

Monthly Meeting Corner

Figure 2: Fundus photograph showing Figure 3: Flourecein angiography of left eye
choroidal folds showing a normal arterio-venous filling with

edema or retinal folds (Figure 4). Ultrasonography of the no leakage at the disc or macula
globe and orbit did not reveal any orbital/scleral/choroidal Figure 4: Spectral domain OCT of left
masses or tumors or muscle thickening. The X-ray of the eye showed a normal study pattern with no
orbits and face did not show any orbital wall fractures or
sinusitis. The thyroid function tests were normal. intraretinal edema or retinal folds
After reviewing the literature for causes of enophthalmos
and choroidal folds, we examined the patient further and
found that he also had a left sided facial hemiatrophy.
To investigate the causes of hemifacial atrophy with
enophthalmos, we sent the patient to our dermatology
department where a skin biopsy was done that did not show
any dermal sclerosis and serum anti-nuclear antibodies
were tested that were also negative. Hence, they ruled out
scleroderma.
The patient was also sent to ENT department, where
silent sinus syndrome was ruled out. It is a spontaneous,
asymptomatic collapse of the maxillary sinus and orbital
floor associated with negative sinus pressures and presents
with enophthalmos and hemifacial atrophy. Magnetic
resonance imaging (MRI) of the face and orbits revealed left
sided facial and orbital soft tissue atrophy but a normal left
globe (Figure 5). Hence, a final diagnosis of Parry-Romberg
syndrome with enophthalmos was made.
We referred the patient to our plastic surgery department
where his facial atrophy was managed with autologous
fat infiltration (Figure 6). We also prescribed glasses to the
patient for visual rehabilitation.
Discussion
Parry Romberg syndrome is a progressive hemifacial
atrophy involving the skin, subcutaneous tissue, muscles,

78 l DOS Times - Vol. 19, No. 7 January, 2014

Monthly Meeting Corner

Figure 5: Magnetic resonance imaging (MRI) of the face and orbits revealed left sided
facial and orbital soft tissue atrophy but a normal left globe (as indicated by the red arrow)

cartilage and bone. It usually begins in the second decade of Figure 6: Photograph of the patient after
life. It is more common in females than in males. A cerebral autologous fat infiltration
disturbance on fat metabolism has been proposed as a
primary cause. Other than facial and ophthalmic features, while others recommend early intervention.Although there
neurological and oral abnormalities have also been seen. are many case reports describing ophthalmic findings in
Several retinal alterations associated with visual loss such Parry Romberg syndrome, no treatment has been described
as dilated retinal veins, optic disc edema, cotton wool for them. Also, there is no case report for orbital volume
spots, retinal folds, retinal edema, stellate exudative replacement or fat transfer into orbit for enophthalmos
neuroretinopathy and exudative retinal detachment10 have in Parry-Romberg syndrome. There are however,isolated
been described in Parry-Romberg syndrome. Theodossiadis case reports of fat infiltration into the orbit (Intraconal
PG et al have reported a case of Parry-Romberg syndrome compartment) in post-traumatic enophthalmos with intact
with retinal nerve fibre layer edema, thickened hyper- seeing globe11,12. Our patient was also given the option for
reflective vitreoretinal interface, diffuse retinal edema fat infiltration into the globe but he refused it due to high
and retinal folds on OCT; with leakage of the optic disc risk of complications
vessels on FFA and visual loss9. However, to the best of There was an apparent increase in the enophthalmos
our knowledge, no case of Parry-Romberg syndrome with after the facial atrophy was managed with fat infiltration.
significant enophthalmos has been described with retinal However, the magnification effect of the convex glasses
changes on fundus examination but normal vision and compensated partly for it. The patient did not show any
unremarkable OCT and FA. The retinal changes in our change in the and ophthalmic features during the 2 years
patient may be due to the severe orbital fat atrophy leading of follow up.
to enophthalmos and hence causing choroidal folds.
However, no structural retinal damage has yet been done
to cause a vision loss. A long term follow up of the patient
may help in knowing the course of retinal changes that
might eventually cause a decrease in the vision.
Regarding the management of the disease,
immunosuppressive drugs have been tried but have not been
found to be effective. Surgical management is the mainstay
of treatment and provides cosmesis to the face. Autologous
fat transfer, fasciocutaneous flap and bone grafts are done
depending upon the severity of the disease. The timing
of surgical intervention is however controversial. Some
surgeons prefer to wait until the disease has run its course

www. dosonline.org l 79

Monthly Meeting Corner

References 7. Ford JG, Busbee B, Reed JW, Yu D.Hemifacial atrophy and primary
corneal endothelial failure. Arch Ophthalmol. 1998. 9: 1246-8.
1. Parry CH. Collections From the Unpublished Medical Writings of
the Late Caleb I Lillier Parry. London, UK: Underwoods; 1825:8 8. Cohen JS. Congenital nonprogressive facial hemiatrophy with
ipsilateral eye abnormalities and juvenile glaucoma. Ann
2. Miller MT, Spencer MA. Progressive hemifacial atrophy: A natural Ophthalmol. 1979. 11(3):413-6.
history study. Trans Am Ophthalmol Soc. 1995; 93: 203–7.
9. Theodossiadis PG, Grigoropoulos VG, Emfietzoglou I, et al. Parry-
3. Muchnick RS, Aston SJ, Rees TD. Ocular manifestations and Romberg syndrome studied by optical coherence tomography.
treatment of hemi-facial atrophy. Am J Ophthalmol. 1979;88: 889- Ophthalmic Surg Lasers Imaging. 2008. 39(1):78-80.
97
10. Gass JDM, Harbin TS Jr, Del Piero EJ. Exudative stellate
4. Rudolph G, Haritoglou C, Kalpadakis P, et al. Hemifacial atrophy neuroretinopathy and Coats’ syndrome in patients with progressive
(Parry-Romberg syndrome, with papillitis, retinal alterations, and hemifacial atrophy. Eur J Ophthalmol. 1991. 1:2-10
restriction of motility. J AAPOS. 2002. 6(2):126-9.
11. Hunter PD, Baker SS. The treatment of enophthalmos by orbital
5. Hoang-Xuan T, Foster CS, Jakobiec FA, et al. Romberg’s progressive injection of fat autograft. Arch Otolaryngol Head Neck Surg. 1994.
hemifacial atrophy: an association with scleral melting. Cornea. 120(8): 835-9.
1991. 10(4): 361-6.
12. Cakir B, Aygit AC, Omur-Okten O et al. Retro-orbital intraconal fat
6. Grayson M, Pieroni D. Progressive facial hemi-atrophy with bullous injection: an experimental study in rabbits.J Oral Maxillofac Surg.
and band-shaped keratopathy. Am J Ophthalmol. 1970. 70: 42-44. 2012. 70(1):242-50.

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Note:* last date for receiving application is: 05th of July 2014
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80 l DOS Times - Vol. 19, No. 7 January, 2014

MonthlyMoMntheleytMineegtinCgoCronrneerr

An Atypical Motility Shagun Sood
Presentation MS

Shagun Sood MS
Guru Nanak Eye Centre, New Delhi

A35 year old female presented with outward deviation Comment- DRS is the commonest CCDD, usually involves
of right eye & inability to move either eye fully since 6th cranial nerve nucleus. DRS is mostly sporadic, about
birth, abnormal head posture which was mainly face turn 10% of cases are heritable and many genetic loci have
to the left since early childhood and discomfort and pain been identified.
due to AHP. Her unaided visual acuity was 6/6 and near DRS enjoys great phenotypic heterogeneity with diverse
vision was N.6 both eyes. She had a face turn to left (30 systemic and ocular associations including cardiovascular
degrees) with slight left sided head tilt. Pupil was semi- anomalies as well as vascular arterio-venous malformations
dilated and fixed REand NSNR left eye. No ptosis or upper/ and vertebro-basilar artery aneurysms. Holt Oram syndrome
lower lid retraction either eye. Her fixation preference was may be associated with DRS. Heritable disorders, Bosley
RE. In Forced PP, she had a right eye hypertropia>20 PD
and exotropia>50 PD. There was reversal of hypertropia (a)
from elevation to depression and narrowing of palp. fissure
on abduction and widening on adduction OD. Fusion on (b)
distance worth four dot and no subjective torsion on DMR.
Park’s three step testwas inconclusive for any isolated Figure 1(a): Showing left face turn and left head
cyclovertical muscle palsy (Figure 1) Abduction limitation tilt 1(b):Montage showing motility in 9 cardinal
4+, adduction limited 2+ OS. No globe retraction / shoots
on adductionos. Fundus photo showed Right eye- intorsion gazes with Park’s three step test findings
4+ and left eye- extorsion 3+.
Findings were consistent with pupil involving partial
peripheral congenital 3rd nerve palsy with aberrant
innervation OD, type 3 exo DRS with retraction escape
with class1 congenital superior oblique palsy OS. Magnetic
resonance imaging of the brain and orbits revealed an
uncal hemangioma in right temporal lobe that compressed
right 3rd cranial nerve (Figure 2). Forced duction test was
strongly positive for right superior rectus, lateral rectus and
for left lateral rectus. Forced generation test was nil for left
lateral rectus. OD superior and lateral recti were recessed
by 10 mm and 14 mm respectively with former transposed
along medial rectus. OS lateral rectus and inferior oblique
were recessed by 10 and 8 mm, subsequently medial rectus
was resected 8 mm. Post- operative results were satisfactory
with elimination of face turn and improvement in primary
position alignment to within 8 PD and in conjugate torsion
(Figure 3).

www. dosonline.org l 81

Monthly Meeting Corner (a)
(a)

(b)
(b)

Figure 2(a): Pre-op conjugate torsion on grade 4 abduction limitation, absence of convergence and
fundus photograph 2(b): MRI brain showing inability to correct exotropia despite extensive surgery
uncalhemangioma along the course of 3rd and 4th betrayed diagnosis of DRS.
cranial nerve causing mass effect on 3rd nerve Though uncal herniation due to any cause is known to
cause rapidly evolving pupil involving 3rd cranial nerve
Salih Alorainy and Athabascan brainstem dysgenesis palsy and connotes an emergency, it’s causation by uncal
syndromes share abnormalities of internal carotid arteries, hemangioma remains unreported. Uncal hemangiomas are
common cavity disorders leading to deafness and invariably not reported and neither their association with DRS. Both
sport bilateral DRS. are likely conterminous in evolution as embryogenesis
Present case has many atypical features impacting diagnosis of cranial somatic motor nerve nuclei and angiogenesis
and treatment. Fixation preference with right eye helped of cerebral vessels share 4-8 week window of embryonic
her avoid diplopia achieve fusion by adopting face turn development and both uncus and pons derive blood
and head tilt also enabling her to escape amblyopia despite supply mainly from vertebro- basilar arterial system. It is
3rd cranial nerve palsy. Absence of globe retraction on likely that both resulted from a common insult sharing a
adduction was likely a retraction escape due to limitation vascular event. Neuro-imaging may be indicated in such
of adduction, other features like marked exotropia despite cases and surgical outcomes are modest.
References

1. Engle EC. Genetic Basis of Congenital Strabismus. Arch Ophthalmol.
2007;125; 189-95

2. DeRESPINIS PA, Caputo AR, Wagner RS, Guo S. Duane’s retraction
syndrome. Surv. Ophthalmol. 1993; 38: 257-88.

3. Chung M, Stout T, Borchert MS. Clinical Diversity of Hereditary
Duane’s Retraction Syndrome. Ophthalmology 2000; 107:500-3.

82 l DOS Times - Vol. 19, No. 7 January, 2014

DOS Times Quiz Delhi
Ophthalmological
Society

Instructions:

1. Please return your answers to [email protected] or mail them to “The Quizmaster, DOS Times Quiz, Dr. Rajesh Sinha,

Room No. 479, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi -

110029”. Please write your DOS membership number along with your answers.

2. The answers should reach not later than 30th April, 2014.

The quiz can also be viewed and directly answered on our website www.dosonline.org

3. The results will be announced at the DOS monthly clinical meeting on July 2014. The correct entry will be given a prize of

Rs. 2,500. If there are more than one correct entries, the winner of the prize will be decided by draw of lots.



Quiz compiled by Dr. Digvijay Singh Quiz Prizes Sponsored by

M/s. Syntho Pharmaceuticals Ltd.

Refractive in Sights Answer for December issue of DOS Times
1. A corneal inlay for presbyopia
e
e

2. The ‘L’ in ‘ReLEX’ i ue
e

3. Verisyse phakic IOL is made from this material.
ee

4. I designed the first posterior chamber phakic IOL
ooo

##

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Answer to DOS Times Quiz January 2014 B. ___________________________________________
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Tearsheet

LASIK Complications

Intraop. Etiology Clinical Features Prevention Management
Complication
of FS Lasik Laser shock wave pushing Gas bubbles can be No specific measures Proceed with excimer ablation
Gas Bubbles the bubbles posteriorly visualized in the anterior known without eye tracking after
in AC through weak stromal chamber either before Can make canal or thoroughly explaining the
Suction Loss lamellae; Retrograde or after flap lift after the pocket for FS gas of patient to maintain fixation
passage of bubbles creation of flap using FS adequate size and Alternatively can wait for
Opaque from the perilimbal area It will interfere with pupil width resolution of bubbles (30min)
Bubble Layer through the trabecular tracking where patient co operation
(DBL) meshwork into the anterior seems poor
chamber

Small palpebral fissures With suction loss, the eye Appropriate head Suction loss during lamellar
Excessive lid squeezing pulls away from the suction positioning with dissection not in the pupillary
Small corneal diameter cone and the laser does not patient being axis: reapply suction at
Conjunctival chemosis work explained about the the same place with the
Sub Epithelium Continued laser pulse procedure same parameters, same flap
breakthrough delivery after suction loss Careful patient diameter, same suction ring
and before foot pedal selection and applanation cone within
release causes stromal 10 mins to increase the chance
dissection in an anterior of having the new cut in the
plane primary dissection plane
Suction loss in central cornea:
second pass on the same day
not recommended. Potential for
irregular interface
Suction loss during side
cut: recut using smaller flap
diameters by 0.5 to 1 mm
Repeat procedure: create flap
thicker by 40µ recommended
or surface ablation

Hard docking Early (hard) OBL appears To bring the canal Diffuse OBL clears after the
Narrow short canal when the gas bubbles length much beyond flap lift
Thicker corneas spread into the tissue the applanation edge Hard OBL difficult to clear with
Small flaps anterior or posterior to the Alter the setting for a weckcell sponge – may not
plane at which the laser wide canal from 1.3 to affect the may not affect the
places additional pulses, 1.7 mm( in Wavelight excimer ablation nor the visual
mainly concentrated in the Refractive suite) outcome significantly
upper central cornea
Late diffuse OBL occurs
when gas travels into
the intralamellar spaces
after the raster pattern
has passed through the
particular portion, looks
more transparent and patchy
and builds as more gas is
introduced into the corneal
tissue

www. dosonline.org l 85

Tearsheet

Intraop. Etiology Clinical Features Prevention Management
Complication Dispersion of gas bubbles Oblong shaped black wet Careful patient VGB identified – stop laser,
of FS Lasik from the interface through spot seen as an island selection reset depth by 40µ and restart
Vertical Gas the anterior stroma via surrounded by chalky Anticipation of the If pachymetry precludes
Breakthrough defects in the Bowman’s white moat; seen within the problem in suspected creation of thicker flap –
(VGB) membrane into the sub normal homogenous pattern cases surface ablation in the same
epithelial space interfering Uncut stromal area noticed Most importantly it sitting or subsequently
with photodisruption of during flap lift and on must be recognized If VGB noticed after complete
the stroma in a small area vigorous attempts can lead immediately and flap creation – DO NOT
Bowman membrane to flap tear or pseudohole procedure halted attempt to lift the flap,
abnormalities as in post management as mentioned
trauma, post pterygium above along with flap diameter
surgery or in EBMD decreased by 0.5 mm in a
In thinner flaps second sitting

Post Op LASIK Interface Complications*

Clinical DLK CTK PISK Infection Epithelial ingrowth
Parameters
Etiology Inflammatory Toxic Fluid (steroid Bacteria – Epithelial cells in
response) Mycobacteria, Fungi interface
Typical onset 1 to 5 days 2 to 7 days 10 to 21 days 3 to 21 days 14 days or later
Initial effect on Minimal Pronounced Pronounced Variable Minimal
visual acuity
Pain Foreign body None Minimal Moderate to severe None
sensation
Location Diffuse Focal, well defined Diffuse Focal, less defined At flap edge
Corneal haze Granular until Dense focally, clear Diffuse Dense at infiltrate, Focal at cells
pattern stage 4 surrounding variable surrounding
Conjunctival None None None Moderate to severe None
erythema
IOP (measured) Normal Normal Normal or low Normal Normal
IOP (Real) Normal Normal High Normal Normal
Interface fluid None None Yes (may not be None None
apparent)
Response to Good/ excellent Minimal / none Poor with worsening Variable None
topical steroids
Response to oral Good/ excellent Minimal / none None with worsening Variable None
steroids
Response to flap Good Poor None Variable, depending Good, may require
lift on flap integrity flap sutures

DLK – diffuse lamellar keratitis CTK – central toxic keratopathy PISK- pressure-induced stromal keratopathy IOP – intraocular Pressure.
*Randleman JB, Shah RD.J Refract Surg. 2012 Aug;28(8):575-86

Srilathaa Gunasekaran MD, FAICO Srilathaa Gunasekaran MD
Dr. R.P. Centre, AIIMS, Delhi

86 l DOS Times - Vol. 19, No. 7 January, 2014