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Congenital Eyelid Colombia: Clinical Evaluation and Surgical Outcome- A Study of 40 Cases

Congenital Eyelid Colombia: Clinical Evaluation and Surgical Outcome- A Study of 40 Cases

Corresponding author

Dr. Golam Haider, Professor and Head, Department of Oculoplasty, National Institute of Ophthamology & Hospital, Dhaka, Bangladesh, Phone No: +8801718797246, E-mail: drgolamhaider@yahoo.com
Copyright: Bangladesh Ophthalmic Journal
This is an open-access article distributed under the terms of the Creative Common Attribution-Noncommercial-Share Alike 3. Ounporte permits unrestricted use, distribution, and reproduction in any medium, the original work is properly cited.
Abstract
Purpose: To evaluate the clinical presentation and surgical outcome in the management of congenital eyelid colombia.
Methods: All cases of congenital eyelid coloboma presenting between January 2005 and December 2013 were assessed for the extent of the eyelid defect, associated ocular anomalies, status of the cornea and any systemic anomalies. The surgical procedure was selected according to the size of defect and finally cosmetic outcome of surgical intervention was judged.
Results: Thirty-one patients (77.5%) presented with an upper eyelid coloboma and 9 patients (22.5%) had a lower eyelid coloboma. 12 (30%) presented with bilateral lid defect, 16 cases had right eyelid defect and 12 (30%) had left eyelid defect. Systemic associations (found in 40%) include Goldenhar syndrome and Treacher-Collins syndrome. The cosmetic outcome of surgical intervention was good in the majority of cases (75%).
Conclusion: Congenital colobomas of the eyelids, presenting in a wide array of clinical presentations, pose a major challenge to the oculoplastic surgeon in the attempt to provide good globe protection, appropriate long-term functional visual outcome, and reasonable cosmesis to the patient.
Keywords: Coloboma, Tenzen flap, Cutler Beard precedure

Introduction

The word coloboma is Greek (Koloboma) meaning mutilated of curtalied and refers to a congenital malformation that occurs in about 1:10000 briths, in which ocular structures are incompletely formed due to failure of the embryonic optic fissure to fuse1. Conggenital eyelid colobomas are a partial or total absence of eyelid structures and are caused by failure of fusion of the mesodermal lid folds. It was first noted by Jacques Guillemeau (1585) who termed it paupieres accurcies2. The fronto nasal and maxillary processes of an embruyo are formed at the 4th week of life and continue their structural differentiation until 8 to 9 weeks. Any form of disturbance that occurs during this embryological period (4 to 9 weeks) can affect the formation of normal eyelid1.
Although the exact cause of congenital eyelid coloboma remains uncertain, several theories have been proposed, likening it to a form of facial cleft1. Furthermore, intrauterine factors such as amniotic band, inflammation, decreased placental circulation, mechanical influences, or abnormal vascular system have been suggested1.
Upper lid colobomas are more common and are present at the junction of middle and medial third of lid and lower lid coloboma is at the lateral third of lid. Eyelid colobomas are most commonly tiangular in shape with the base at the eyelid margin and can vary in size from a small identation of the eyelid to near absence of the entire eyelid.
Eyelid coloboma may occurs as an isolated finding or associated with systemic conditions like Goldenhar syndrome, Treacher Collins syndrome, Fraser syndrome, frontonasal dysplasia, nasopalpebral lipoma coloboma syndromes3.
The eyelid coloboma may presents with exposure keratopathy due to poor Bell’s phenomenon, corneal opacity and visual deprivation leads to amblyopia1.
Management of these cases involves surgical repair of the eyelid defect and any associated ocular and systemic anomalies to provide corneal protection and monitoring of visual development2.

Material and methods

Forty consecutive patients of congential lid coloboma of any age range were selected from Oculoplasty department of National Institute of Opthalmology, Dhaka, Bangladesh, from January 2005 to December 2013. All patients underwent complete opthalmic and general medical examination. At the time of initial visit all patients/ parents were interviewed regarding the term of delivery, their exposure to drugs and infectious diseases during pregnancy and the heritance of the coloboma. During general opthalmic examination position of eye ball, Bell’s phenomenon and any facial deformity were noted. The site and size of the eyelid defect, conjunctival involvement, corneal status, and ocular motility were also assessed. Before repair of the eyelid coloboma, the defects were measuredas one third, half or more than half the eyelid size.

All surgeries were performed by a single surgeon. Defects upto one third were repaired by direct closure, with or without cantholysis. The edges of the defects were made raw with sharp incisions, following which precise anastomosis was performed by bringing lid margins together and by using two layer approximations of the tarsus and skin. Defects by Tenzel semicircular flap. When the defect involved more than half of the upper eyelid, the Cutler Beard procedure was employed. In this procedure, a full thickness lower eyelid flap was advanced into the defect of upper eyelid by passing it behind the remaining lower eyelid margin.

Surgical outcome were graded as poor, satisfactory and good on the basis of cosmesis, eyelid closure and exposure. Post-operative outcome was defined as “Good” if it was cosmetically acceptable with no complication, “Satisfactory” if it was adequate cosmetically with minor complications and “Poor” if it was inadequate cosmetically.

Results

In this study, the age of the patients ranged from 1 months to 35 years (Table 1). Out of these 40 patients 24 (60%) were male and 16 (40%) were female. There was no history of premature birth of any case.

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