Correspondence to: Mr J. E. Moore, Department of Ophthalmology, Royal Victoria Hospital, Grosvenor Road, Belfast, Northern Ireland. BT12 6BA.
Email: johnny_moore@tiscali.co.uk

Accepted for publication:  2nd February, 2004

The video demonstrates the use of amniotic membrane to reconstruct the left ocular surface of a child with LOGIC syndrome. First, the scar tissue causing ankyloblepharon and symblepharon was carefully divided allowing the eyelids to open. Then scar tissue was gently dissected and excised from the ocular surface. Finally, amniotic membrane was sutured basement membrane downwards onto the cornea and conjunctival surfaces.

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Introduction

Laryngeal and ocular granulation tissue in children from the Indian subcontinent (LOGIC) syndrome is a homozygous recessive condition, originally reported in 22 patients from the Lahore district in Pakistan.[1] The condition manifests itself in infancy affecting epithelial surfaces resulting in skin lesions, dystrophic finger and toenails, lateral forniceal conjunctival granulation tissue and laryngeal involvement. The cause of this condition is as yet unknown.

Ocular involvement in the UK tends to be severe and is characterized by fibrovascular proliferation on the ocular surface. This scar tissue initiates in the lateral conjunctival fornices and grows nasally [2] invading corneal tissue resulting in corneal opacification and blindness. In addition the scar tissue often leads to severe symblepharon and ankyloblepharon. Prior to 2001 no recognized treatment was available until Moore et al reported a relatively successful treatment to reconstruct the ocular surface using amniotic membrane.[3]

This video report demonstrates the use of amniotic membrane as part of an extensive operative procedure carried out to reconstruct the left ocular surface of a child with LOGIC syndrome. This child had been bilaterally blind prior to successful treatment of the right eye with amniotic membrane 18 months prior to this procedure.

 

Methods

The scar tissue causing ankyloblepharon and symblepharon was carefully divided allowing the eyelids to open. The scar tissue was gently dissected and excised from the ocular surface. Amniotic membrane was measured, cut to size and sutured basement membrane downwards, successively onto corneal surface conjunctival surface, reforming fornices by use of internal and external bolsters. Fibrin glue was injected subamnion as an adjunctive measure to assist the amnion to remain attached to corneal and subtarsal surfaces. A large bandage contact lens was fitted over the surface. Postoperative management included antibiotic and steroid drops.

 

Comment

The benefits of using amniotic membrane to reconstruct the ocular surface in a variety of conditions is now well recognized. This particular condition is rare with very few reports in the literature. However it, poses a severe dilemma to the clinicians who manage it. No other known treatment option is available to manage this form of ocular surface scarring, which usually results in blindness. The use of amniotic membrane does not however completely prevent recurrence. The right eye of this child treated operated on 18 months prior to this procedure but shown at the end of this video demonstrates limited recurrence in the temporal margin. To date amniotic membrane appears to be the only successful management option available to treat ocular scarring in this condition. If further studies demonstrate a genetic defect it may be possible to specifically engineer treatments to target and treat this defect thus preventing the relentless scarring process in these eyes.

 

Acknowledgements

The authors would like to thank Mr J Wallace, Computing Science University of Ulster, Mr J Mairs, and Mr P McMullan, School of Media and Performing arts, for their technical assistance with preparation of the digital video.

 

References

1. G. Shabbir, M. Hassan, A. Kazmi. (1986). Laryngo-onycho-cutaneous syndrome-a study of 22 cases. Biomedica 2:15-25.

2. Ainsworth JR., Spencer AF, Dudgeon J. (1991). Laryngeal and ocular granulation tissue formation in two Punjabi children: LOGIC syndrome. Eye 5:717-722

3. Moore. J., Dua HS., Page AB,. Irvine AD., Archer DB. (2001). Ocular Surface reconstruction in LOGIC syndrome by Amniotic Membrane Transplantation. Cornea 20 (7): 753-756.