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| CASE REPORT |
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| Year : 1988 | Volume
: 36
| Issue : 1 | Page : 29-31 |
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Congenital colobomas of the eyelid
SM Betharia, Sushil Kumar
, Dr. RP. Centre, AI.LM.S., Ansari Nagar, New Delhi-110 029, India
Correspondence Address:
S M Betharia
, Dr. RP. Centre, AI.LM.S., Ansari Nagar, New Delhi-110 029
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 3253195 
5 cases of congenital coloboma of the lids which is rare anamoly are described, the unusual features noted in 4 cases was the presence of nystagmus and abnormal eye brow deformity in 1 case. The aetiopathogenesis and surgical management is highlighted.
How to cite this article:
Betharia S M, Kumar S. Congenital colobomas of the eyelid. Indian J Ophthalmol 1988;36:29-31 |
| Introduction | |  |
The congenital coloboma of the lid was first noted by Jacques Guillemeau in 1585 who called it paupieres accurcies. A coloboma of the lid is a defect affecting primarily the lid margin where all portions of the lid structure are absent, It is usually seen in the upper lid in its medial one third but occasionally it maybe present in the lower lid as is seen in cases of mandibulofacial dysostosis Various associated findings which have been described are the presence of dermoids or dermolipomata, conjunctival chondroma, symblepharon, corneal opacities, cutaneous bridging, coloboma of the fundus and facial abnormalities [1]. We are describing 5 cases of congenital colobomas with varied clinical presentation along with their surgical management and aetiopathogenesis
| Case Reports | | |
Five cases of congenital colobomas of both the upper lids in the age group of 5 days to 20 years were studied. The extent of the coloboma was more than half of the upper lid and the location was on the medial side. In one case there was an unusual abnormality of the eye brow which was vertically placed Another interesting finding was the presence of mystagmus in 4 out of these 5 cases. The type of surgery done for repairing these colobomas included direct suturing after canthotomy and cantholysis in one case and Cutler Beard technique in the other 4 cases [Figure - 1],[Figure - 2],[Figure - 3]. In case No. 1 a tarsorrhapy was performed in one eye and the Cutler Beard operation in the other eye. Brow-plasty was also done in case No. 4 [Figure - 4][Figure - 5]. Details of the clinical presentation are given in [Table - 1].
| Discussion | | |
As compared to upper lid colobomas lower lid defects are encountered less frequently but when they occur, they are seen in the lateral half of the lid in association with mandibulofacial dysostosis. They may also occur medially where they are associated with the abnormalities of the lacrimal drainage channels [2].
The role of heredity in the transmission of the defect especially in the upperlid is not clear. The lower lid colobomas seen in mandibulofacial dysostosis exhibit a clear, irregular, dominant hereditary pattern [3].
There are variable views regarding the normal development of the lids [4],[5],[6].However, it is believed that the normal development is the result of complex interactions and the union of mesodermal sheets of the frontonasal processes in the case of upper lid and similar maxillary processes for the lower lids. Any delay or interference in the interrelationship of developmental components can produce a lid defect
In the management of congenital coloboma, the presence of symblepharon or dermolipomata need initial treatment in the form of excision and mucous membrane grafting. The eyelid defect is repaired subsequently. Regarding the repair of such defects there seems to be some difference from the post-traumatic colobomas and eyelid defects following tumour excision. Since there is a clear and regular line of demarcation between skin & mucosa and absence of surrounding scar tissue in cases of congenital colobomas, the excision of the margin of the coloboma is unnecessary. Only proper freshening of the edges of the coloboma is required.
Congenital colobomas involving one third of the lid can easily be repaired by direct suturing after proper freshening of the edges of the defect instead of one fourth of the eye lid defect due to other causes [7]. A defect upto one half or more will require canthotomy and cantholysis in addition to direct suturing. Colobomas involving more than half of the lid requires lid sharing procedures [8],[9] e. g. Cutler Beard operation and Hughes procedure, or lid sliding techniques [10],[11] e.g. Mustarde s rotation flap repair and Tenzel's semicircular flap from the lateral canthal region.
One of the important findings in 4 of our cases was the presence of nystagmus which is not previously reported in literature. Other important findings in case no. 4 was the absence of the eyebrow and hairy growth which was vertically placed on the forehead for which a browplasty was done successfully. Temporary tarsorrhaphy in one eye can be done while carrying out the Cutler Beard operation in the other eye. Alf possible precautions should be taken to save the lacrimal puncta. while repairing the upper lid colobomas as they are usually present on the medical side.
These cases should be taken up for surgical management at the earliest to give good functional and cosmetic results.
| References | | |
| 1. | Duke Elder, S. 1964: System of Ophthalmology, vol. 3 part 2 Congenital deformities : coloboma of the lid Henry Kinspton, London, p. 836.  |
| 2. | Roper Hall, M : 1973 : Congenital colobomas of the Lids, in Krizek,T.J. Hoopes, J. E., (eds) Symposium on basic science. Plastic surgery, New York, CV. Mosby Co. |
| 3. | Poswillo, D. 1976: Trans. OphthalmoL Soc. U.K 96-69. |
| 4. | Mann LC, 1957: Developmental abnormalities of the eye. Philadelphia, J. R Lippincott Co. p. |
| 5. | Mann LC., 1943: Brit J. Ophthalmol, 27:13. |
| 6. | Guibor, P. 1974: Surgical repair of congenital colobomas, in Guibor P, Smith B (eds) Contemporary Oculoplastic Surgery, New York, Stratton Intercontinental, p. 15. |
| 7. | Fox S 1963 : Ophthalmic Plastic Surgery, 3rd EdL Grune and Stratton, New York |
| 8. | Cutler, N.L : and Beard C. 1955: Am Journal Ophthalmol, 30: 1. |
| 9. | Beyer-Machule, CK, 1983: Plastic and Reconstructive Surgery of the eye lids, Thieme-Stratton, New York p. 1 |
| 10. | Mustarde, JC, 1966: Repair and reconstructions of the orbital region, Baltimore, Williams and Wilkins Co. p. 13 |
| 11. | Kidwell, E. D.R, Tensel, R 1979: Arch Ophthalmol 97: 1931. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]
[Table - 1]
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