Report an error   


Submitted: 28 June 2018 Modified: 05 July 2018
HERDIN Record #: R07-VSMMC-18062815581110

Congenital bilateral upper eyelid coloboma with corneopalpebral adhesions and exposure keratopathy secondary to poor bell's phenomenon.

Juan Carlos  B. Marzan

See More

Congenital eyelid coloboma is part of a spectrum of various eye defect with a  deduced approximation of incidence that is between 1:12000 to 1:57000 live births.The exact disease burden of uncorrected eyelid coloboma remains to be unreported but worldwide, it is animportant cause of vision loss in children. We report a documented case of an 8 month old baby girl with congenital upper eyelid coloboma with corneopalpebral adhesions and exposure keratopathy secondary to poor bell's phenomenon. The coloboma defect size is more than half of the total length of the upper eyelid. Upon initial consult, patient is borderline malnourished with a Z score of -2. Surgery was delayed until nutritional status was improved (above Z score -2) . During the interim, ample lubrication of the exposed cornea was ensured using eyedrops / eye lubricants. Eyelid colobomas may be classified as small, moderate and large. In this case, the defect is classified as a large size upper eyelid coloboma, a lid sharing technique is  therefore recommended. Lid-sharing procedures are of two types - advancement flap (Cutler-Beard) and switch flap (Abbe-like Flap), we opted to do an Abbe-like flap procedure, which is a staged procedure, with 3 weeks flap division and insetting interval to minimize the risk of occlusion amblyopia. Postoperatively, patient was well and stable, with acceptable results. Hopefully, the reporting of this case will bring increased awareness of this condition and that reconstructive measures can be achieved in our institution.

Publication Type
Research Report
Date
LocationLocation CodeAvailable FormatAvailability
Vicente Sotto Memorial Medical Center Abstract Print Format (Ask the Information Officer)
1. Gregory-Evans, CY , Williams, MJ , Halfors, S , Gregory-Evans, K . "Ocular Coloboma: A Reassessment in the Age of Molecular Neuroscience." MedGenet 41, 881-891, 2004
2. Molina, JM, , Horna, ER , Miranda, AD , Rubio, R , de Larraya, AS , Casillas, G , Horna, ER , Miranda, AD , Rubio, R , de Larraya, AS , Casillas, G . Congenital Upper Eyelid Coloboma: Clinical and Surgical Management.. Case Reports in Ophthalmological medicine., 2015. 4 pages. Retrieved from: http://doi.org/10.1155/2015/286782
3. Smith, HB , Verity, DH , Collin, JRO . "The incidence, embryology, and oculofacial abnormalitiesassociated with eyelid colobomas" Eye 29, 492–498, 2015
4. Hoyama, E , Limawararut, V , Malhotra, R , Muecke, J , Selva, D . "Tarsomarginal graft in upper eyelidcoloboma repair." ,
5. Lodhi, AA , Junejo, SA , Khanzada, MA , Sahaf, IA , Siddique, ZK . "Surgical outcome of 21 patients with congenital upper eyelid coloboma." IntJ Ophthalmol. Vol 3 No 1, 2010
6. Tawk, HA , Abdulhafez, MH , Fouad, YA . "CongenitalUpper Eyelid Coloboma:Embrologic, Nomeclatorial, Nosolgic, Etilogoci,Pathogenetic, Epidemiologic, Clinical and Management Perspectives." ,
7. Lee, H , Takahashi, Y , Ichinose, A , Kakizaki, H . "Reconstruction of a Congenital Upper EyelidColoboma Using a Lamellar-Based Technique" ,
8. Chiummariello, S , Angelisanti, M , Arleo, S , Alfano, C . "Surgicaltreatment of upper eyelid coloboma, our exeprience." Ann, Ital. Chir. 83, 5, 379-383, 2012
9. Kenkel, JM . "Eyelid Reconstruction. Selected Readingsin Plastic Surgery." Volume 9, , 2000
10. Krachmer, J , Mannis, MJ , Holland, EJ , Mannis, MJ , Holland, EJ . "Cornea" Third edn,, 2011
11. Mustafa, T . "The Bell’s phenomenon in newborns. " Neurosciences Vol.10(1), 41-43, 2005
12. (). Geneticshome reference, US National Library of Medicine. . Retrieved from: https://ghr.nlm.nih.gov
13. Risma, JM , Syed, NA . Exposure Keratopathy in the Critically Ill: A Case Report,Discussion, and Systems-Based Intervention.
14. Witmer, MT , Slonium, C . "Repair of Bilateral Upper Eyelid Colobomas in Infants" ,
15. "Global Database on Child Growth andMalnutrition" World Health Organization  ,
16. Meurling, S . "The perioperative nutritional careof neonates and infants." Scandinavian Journal of Nutrition Vol44, 8-11, 2000
17. Alghoul, M , Pacella, S , Mc Cella, WT , Codner, MA . "Eyelid Reconstruction." PRS Journal. Volume 132, pages 288e,
18. Uemura, T , Yanai, T , Yasuta, M , Kawano, H , Isihara, Y , Kikuchi, M . "Switch Flap for Upper Eyelid Reconstruction - How Soon Should theFlap Be Divided?. " , . Retrieved from: http://www.PRSGlobalOpen.com
19. Shrestha , JB , Shresth, GS , Joshi, N , Karmacharya, PC . "Congenital isolated bilateral upper lid coloboma." Nepal Journal ofOphthalmology 4 (7), 194-196, 2012
20. Ankola, P , Abdel-Azim, H . "Congenital UpperEyelid Coloboma." Journal of Perinatology 23, 166-167, d, 2003
21. Collin, JRO . "Congenital Upper Lid Coloboma." Australian and New Zealand Journal of Ophthalmology 14, 313-317, 1986

Copyright © One Window Project 2018. All rights reserved.