Document Detail


Glaucoma and globe enlargement associated with neurofibromatosis type 1.
MedLine Citation:
PMID:  19729098     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To describe the features of glaucoma and globe enlargement sometimes associated with neurofibromatosis type 1 (NF1). DESIGN: Single institution, retrospective, and cross-sectional study. PARTICIPANTS: Eighty medical records of patients treated at King Khaled Eye Specialist Hospital in Saudi Arabia with NF1 were reviewed, and 46 patients were examined. METHODS: We reviewed the charts of patients with NF1 and examined available individuals, including gonioscopy, axial length, and ultrasound biomicroscopy in appropriate patients. MAIN OUTCOME MEASURES: Presence and type of glaucoma, anterior chamber angle abnormalities, globe axial length, ultrasound biomicroscopy, and visual outcome. RESULTS: Two patient groups were identified: Group 1 had 56 patients with orbito-facial NF1, and group 2 had 24 patients with NF1 but without orbito-facial involvement. Group 1 included 13 patients with glaucoma (23%), which occurred only ipsilateral to the orbito-facial involvement and generally presented before age 3 years. Glaucoma surgery was required in all of these patients, and visual prognosis was poor. In group 1, mean axial length on the side affected by NF1 was 29.8+/-4.1 mm in patients with glaucoma and 25.6+/-2.0 mm in patients without glaucoma. Patients with glaucoma (P<0.001) and without glaucoma (P<0.0001) in group 1 had significantly larger globes on the affected side. Group 2 patients had a mean axial length of 23.6+/-1.6 mm for both eyes without significant globe asymmetry. CONCLUSIONS: In this Arab population, glaucoma associated with orbito-facial NF1 occurred less often than the 50% rate that is typically cited. Glaucoma presented early in life and only in patients with ipsilateral orbito-facial involvement. Glaucoma in this setting was always associated with globe enlargement. Glaucoma required surgery, and visual prognosis was poor because of glaucoma and concurrent pathology. Globe enlargement was most severe when associated with glaucoma but also present on the side with orbito-facial involvement in patients without glaucoma. The presence of neurofibroma may induce globe enlargement on the affected side in orbito-facial NF1. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
Authors:
Jose Morales; Imtiaz A Chaudhry; Thomas M Bosley
Related Documents :
1867298 - Clinical patterns and associated conditions in chronic uveitis.
3857778 - Correlation of ocular rigidity and blue sclerae in osteogenesis imperfecta.
8841298 - Hla typing in patients with ocular manifestations of stevens-johnson syndrome.
2624728 - Outpatient penetrating keratoplasty.
9825198 - Prior revascularization increases the effectiveness of enhanced external counterpulsation.
23107148 - Iodine deficiency and subclinical hypothyroidism are common in cystic fibrosis patients.
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Ophthalmology     Volume:  116     ISSN:  1549-4713     ISO Abbreviation:  Ophthalmology     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-09-04     Completed Date:  2009-09-24     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7802443     Medline TA:  Ophthalmology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1725-30     Citation Subset:  IM    
Affiliation:
Glaucoma Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia. jmorales@kkesh.med.sa
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adolescent
Child
Child, Preschool
Cross-Sectional Studies
Eye / pathology*,  ultrasonography
Facial Neoplasms / complications*,  diagnosis
Female
Glaucoma / diagnosis,  etiology*,  surgery
Gonioscopy
Humans
Hypertrophy
Infant
Infant, Newborn
Intraocular Pressure
Male
Microscopy, Acoustic
Neurofibromatosis 1 / complications*,  diagnosis
Optic Nerve Diseases / diagnosis,  etiology*,  surgery
Orbital Neoplasms / complications*,  diagnosis
Prevalence
Retrospective Studies
Visual Acuity

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Ocular findings after allogeneic hematopoietic stem cell transplantation.
Next Document:  Homocysteine, methylenetetrahydrofolate reductase C677T polymorphism, and risk of retinal vein occlu...