Case Reports

Complex cases in paediatric cataract surgery: Hallerman-Streiff Syndrome

Case Report Details

First Author: M.Quiroz Quiroga SPAIN

Co Author(s):    M. Morales Ballús   G. Benejam Torrent                 

Abstract Details

Purpose:

The objective of this report is to highlight the difficulties in cataract surgery in complex paediatric cases such as Hallerman- Streiff syndrome (HSS).

Setting:

HSS is a rare genetic disorder characterized by ocular and craniofacial anomalies. Ocular findings reported include microphthalmia, microcornea, strabismus, vitreoretinal anomalies and congenital cataract.

Report of Case:

We report the clinical features, procedures and outcomes of 4 eyes from 2 confirmed cases with HSS and bilateral congenital cataracts. The four eyes from 2 months old infants presented microcornea, microphthalmia, narrow anterior chamber and dense cataract that can not allowed to explore the fundus. Surgery consisted in anterior vitrectorhexis, cortical aspiration, posterior vitrectorhexis, anterior vitrectomy by clear corneal approach, and peripheral iridectomy (patient 2), without intraocular lens (IOL) implantation. Intraoperative fundus exploration showed a retinal fold in both eyes from the second patient. This finding was previously reported in subsequent follow-up of the first patient. Refractive defect due to aphakia was promptly corrected with glasses. Severe microcornea does not allow adaptation to contact lenses. During the 3 years follow up patient 1 presented an iris bombe solved with surgical iridectomy in the right eye (RE) and exudative detachment in the left eye (LE). With best-corrected visual acuity (BCVA) RE 0.5, LE 0.2 and binocular 0.5. Patient 2 presents unremarkable 6 months follow up.

Conclusion/Take Home Message:

HSS is usually associated with complex cataracts in which anterior and posterior capsules may be in close approximation with each other and it may be difficult to perform an anterior capsulorrhexis or to implant the IOL in the bag. Additionally, microcornea and microphthalmos may thus precluding IOL implantation. Finally, glaucoma and vitreoretinal complications could determine visual prognosis.

Financial Disclosure:

None