Hallermann-Streiff Syndrome in Concordant Monozygotic Twins With Congenital Cataracts, Exudative Retinal Detachments, and One Case of Corneal Perforation Requiring Keratoplasty.

Abstract

PURPOSE:

We describe the management of Hallermann-Streiff syndrome in monozygotic female twins with congenital cataracts, exudative retinal detachments, and 1 case of corneal descemetocele with associated dellen and subsequent perforation.

METHODS:

This study was a case report and review of the literature.

RESULTS:

Twins 1 and 2 exhibited all 7 cardinal characteristics of Hallermann-Streiff syndrome, presenting with spontaneous lenticular resorption, anterior uveitis, and glaucoma. They underwent bilateral cataract extraction with near total capsulectomy. Both twins experienced recurrent glaucoma, for which twin 1 underwent successful endocyclophotocoagulation in both eyes and twin 2 in the left eye alone. The fellow eye developed 2 sites of perilimbal corneal descemetoceles with associated dellen at the inferotemporal limbal corneal junction leading to spontaneous perforation of 1 site, requiring a full-thickness corneal graft. Both twins developed recurrent bilateral exudative retinal detachments unresponsive to oral prednisolone. Twin 1's last best-corrected visual acuity with aphakic spectacles was 20/260 in the right eye and 20/130 in the left eye at age 4 years and 8 months. Twin 2's last best-corrected visual acuity was 20/130 in each eye at age 4 years and 11 months, over a year after right eye penetrating keratoplasty.

CONCLUSIONS:

We describe 2 rare cases of Hallermann-Streiff syndrome in monozygotic twins complicated by corneal perforation requiring penetrating keratoplasty in 1 eye of 1 twin. Although corneal opacities have been described in this condition, this is the first case of corneal descemetocele in Hallermann-Streiff syndrome. The cornea was stabilized with a relatively favorable visual outcome over 1 year later.

• Authors: Deion T Sims, Nicole R Mattson, Laura C Huang, Michele D Lee, Randall A Bly, Emily R Gallagher, Francine M Baran, Michelle T Cabrera
• Journal: Cornea (Cornea) Vol. 42 Issue 7 Pg. 899-902 (Jul 01 2023) ISSN: 1536-4798 [Electronic] United States
• PMID: 37088900 (Publication Type: Review, Case Reports, Journal Article)
• Copyright: Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.

Topics

• Humans
• Female
• Child, Preschool
• Hallermann's Syndrome (complications)
• Corneal Perforation
• Retinal Detachment
• Twins, Monozygotic
• Cataract (complications)
• Corneal Transplantation (adverse effects)
• Keratoplasty, Penetrating (adverse effects)
• Glaucoma (complications)