Strabisme et syndrome de Williams-Beuren. Présentation de trois cas opérés [Strabismus and Williams-Beuren syndrome. Presentation of 3 operated cases]

Details

Serval ID
serval:BIB_5351
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Strabisme et syndrome de Williams-Beuren. Présentation de trois cas opérés [Strabismus and Williams-Beuren syndrome. Presentation of 3 operated cases]
Journal
Klinische Monatsblätter für Augenheilkunde
Author(s)
de Ancos E., Klainguti G.
ISSN
0023-2165
Publication state
Published
Issued date
1996
Volume
208
Number
5
Pages
340-342
Language
french
Notes
Publication types: Case Reports ; English Abstract ; Journal Article
Abstract
BACKGROUND: Williams-Beuren syndrome (WBS) is a developmental disorder of unknown etiology. The classical features include: 1) a supravalvular aortic stenosis, 2) "elfin" facies and 3) mental deficiency. Ocular findings can be irisabnormalities (stellate, pattern), tortuositas vasorum and, though not essential for diagnosis, strabismus is commonly found. Three cases of WBS with convergent squint which underwent surgical treatment are presented. Biopsy and histological studies were performed in two cases. MATERIAL AND METHODS: Strabismus onset was diagnosed before the second year of life in all three. Pre-operative esotropia (ET) was 45, 40 and 62 delta. Age at operation was respectively 52,67 and 87 months. Surgical treatment consisted in recess-resect procedures on horizontal muscles. Biopsy was performed in two cases. RESULTS: Three months after surgery, angular measurements were respectively: exotropia (XT) 6 delta, ET 2 delta, ET 10 delta. Anatomopathological findings showed normal tendon tissue. DISCUSSION: No particular motility disturbances or pattern have been found in our patients. Amount of surgery has been chosen according to routine. Amblyopia treatment could be performed as usual. Treatment in our patients with WBS has been similar to ordinary cases of strabismus. Medium-term results do not differ from other squinting patients treated with similar procedures.
Keywords
Child, Child, Preschool, Esotropia/surgery, Female, Follow-Up Studies, Humans, Male, Postoperative Complications/etiology, Strabismus/surgery, Treatment Outcome, Williams Syndrome/surgery
Pubmed
Web of science
Create date
19/11/2007 13:41
Last modification date
20/08/2019 15:08
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