Ismail K Jalili

FRCS, DO, FRCOphth

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  ABSTRACT
I K Jalili

Childhood Onset Visual Impairment in the West Bank and Gaza Strip

 

INTRODUCTION: This is a detailed study of childhood onset visual impairment in the West Bank (WB) and Gaza Strip (GS) that was conducted between October 1985 and September 1987 whilst on the staff of St John Ophthalmic Hospital, Jerusalem.

AIMS: To investigate the causes of childhood onset visual impairment, establish any emerging patterns in the target population and put forward recommendations to combat childhood blindness.

PATIENTS AND METHODS: Pupils from the 5 blind schools, 2 training centres and those on their waiting lists, incumbents of 3 residential homes, all cases on St Johnís Ophthalmic Hospital register, their relatives and others recruited from field work who fulfilled the study criteria, underwent full ophthalmic examination by the author including psychophysical and electrodiagnostic investigations.

RESULTS: Of the total 699 patients registered (WB 347, GS 289), 61.7% had SVI/BL (WB 66.6%, GS 56.4%) with 54.6% in the <16 (WB 60%, GS 47%) with a minimal prevalence of 0.28/100,000 in the latter cohorts in each of the two regions. Seventy seven percent of the conditions were hereditary (WB 76.4%, GS 84.4%); 89% of which were inherited in an AR manner. This reflects the high consanguinity rate of 88.6% in the hereditary cohorts (WB 82%, GS 90%), 70% of which were first cousin marriages.

Retinal diseases (47.1%), lens (21.7%), congenital glaucoma (CG) (10.2%) and small eyes (MC) (5.5%) were the commonest conditions. The latter were more predominant in the GS (WB 4.3%, GS 7.6%). Retinal dystrophies (RD) formed 78% of the retina and congenital cataract (CC)/aphakia 89.5% of lens cases. Both CG and CC had high ocular/visual morbidity as a consequence of a severe form of the disease in the former, and intractable amblyopia from neglect of aphakia in the latter, which was the only truly treatable condition found.

A male preponderance of 1.44:1 was present in the total series particularly in the GS (1.6:1), being higher in the non-hereditary conditions (WB 2:1, GS 1.5:1). This diminished in the <16 and in the hereditary conditions. Male predominance was also demonstrated in CC, CG, MC and optic nerve disorders whilst female preponderance was significant in achromatopsia. There was no culture of male favouring in education and almost all known visually impaired children were enrolled in the schools.

Wide regional differences existed in the RD types together with wider inter and intra familial differences in their phenotypes. Childhood onset rod cone dystrophies and Leberís congenital amaurosis were predominant in the WB whereas cone disorders were the hallmark of RD in the GS, frequently in syndromatic forms.

Prevention of blindness in this population is not an easy task considering the cultural acceptance of consanguinity, compounded by economic and geopolitical factors. This will require the joint efforts of governmental and non-governmental bodies in sustained long-term measures to include health education, premarital carrier and genetic counselling together with improved screening with a view to early referral of treatable conditions and dedicated paediatric services in both regions.

Ismail Jalili

November 2005


 

Ismail K Jalili 2000-2016