98th Annual Meeting DOG 2000

P 322

Systemic and ocular characteristics of Hallermann-Streiff-François Syndrome

C. Foja, P. Meier

Introduction: The Hallermann-Streiff-François-Syndrome (H.-S.-F.-Syndrome) is an autosomal-recessive hereditary complex of many systemic and ocular abnormalities: prominent thin, pointed nose, severe dental abnormalities, atrophy and thinness of the perioral skin, microphthalmus, congenital cataract.

Patient: We report on a male infant (age of 4 month) with classic phenotype of H.-S.-F.-Syndrome.

Results: Under general anesthesia we documented ophthalmological findings. Later cataract excision was performed on the left eye. At present it is not possible to precise the visual acuity and its prognosis.

Summary: The sporadically occuring H.-S.-F.-syndrome is seen very rarely and relates to the complex of progeria. With regard to clinical findings, clinical images and the material of 150 cases, which are reported in literature, we discuss the typical signs of H.-S.-F.-syndrome in particular consideration of the ocular symptoms.

Department of Ophthalmology, University of Leipzig, Liebigstraße 10-14, D-04103 Leipzig