TITLE

Heterochromia Iridis and Pertinent Clinical Findings in Patients With Glaucoma Associated With Sturge-Weber Syndrome

AUTHOR(S)
Aggarwal, Nalini K.; Gandham, Sai B.; Weinstein, Rebecca; Saltzmann, Robert; Walton, David S.
PUB. DATE
November 2010
SOURCE
Journal of Pediatric Ophthalmology & Strabismus;Nov/Dec2010, Vol. 47 Issue 6, p361
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Purpose: To examine the clinical and gonioscopic findings in patients with glaucoma associated with Sturge--Weber syndrome. Methods: Retrospective review of clinical findings of all patients with Sturge--Weber syndrome who presented between January 1978 and December 2003. Koeppe gonioscopy was performed under general anesthesia and findings were photographed when feasible. Iris color was documented by color sketch or photograph at initial presentation. Fisher exact test was used to determine whether the presence of heterochromia was statistically higher in the glaucomatous group.Results: Fifty-five patients who met inclusion criteria were identified, of whom 44 (80%) had glaucoma. Unilateral glaucoma was diagnosed in 30 eyes and bilateral glaucoma in 14 eyes, yielding 58 eyes with glaucoma and 52 eyes without. Gonioscopic examination revealed distinct anatomic abnormalities in the anterior chamber angle in 32 (55%) of the glaucomatous eyes in contrast to the normal fellow eyes in patients with unilateral glaucoma and to the patients without glaucoma. Heterochromia iridis with darker iris on the glaucomatous side was present in 11 of the 30 patients with unilateral glaucoma (37%, P < .001) but in none of the patients without glaucoma.Conclusion: The characteristics observed in the anterior chamber angle in this large, uniquely examined series are the first reported to the authors' knowledge and may support a role for neural crest derived anomalies in the pathogenesis of glaucoma in these infant eyes. Careful examination for heterochromia iridis may be judicious in these patients.
ACCESSION #
55545503

 

Related Articles

  • Periodontal management of gingival enlargement associated with Sturge-Weber syndrome. Elavarasu, Sugumari; Thanga Kumaran, S.; Sasi Kumar, P. K. // Journal of Indian Society of Periodontology;Mar-Apr2013, Vol. 17 Issue 2, p235 

    The Sturge-Weber syndrome is a rare uncommon neurocutaneous disorders with angiomas involving the leptomeninges (Leptomeningeal Angiomas) and skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. The cutaneous angioma is called a Port-Wine...

  • Diagnostic dilemma: Sturge-Weber syndrome, without facial nevus. Zanzmera, Paresh; Patel, Tinkal; Shah, Vinay // Journal of Neurosciences in Rural Practice;Jan-Mar2015, Vol. 6 Issue 1, p105 

    Sturge-Weber syndrome (SWS), a rare sporadic neurocutaneous disease, is characterized by a congenital unilateral port-wine nevus affecting the area innervated by V1, ipsilateral leptomeningeal angiomatosis, and calcification in the occipital or frontoparietal region and glaucoma/vascular eye...

  • Neurocutaneous vascular syndromes. Puttgen, Katherine B.; Lin, Doris D. M. // Child's Nervous System;Oct2010, Vol. 26 Issue 10, p1407 

    There have been significant recent advances in the past several years in the field of neurocutaneous vascular syndromes, including the development of more stringent diagnostic criteria for PHACE syndrome, the renaming of macrocephaly-cutis marmorata telangiectatica congenita to...

  • Periodontal Management of Sturge-Weber Syndrome. Kalakonda, Butchibabu; Pradeep, Koppolu; Mishra, Ashank; Reddy, Krishnanjaneya; Muralikrishna, Tupili; Lakshmi, Vijaya; Challa, Radhika // Case Reports in Dentistry;2013, p1 

    Sturge-Weber syndrome (SWS) is a sporadic disorder and is frequent among the neurocutaneous syndromes specifically with vascular predominance. This syndrome consists of constellation of clinical features like facial nevus, seizures, hemiparesis, intracranial calcifications, and mental...

  • port-wine stain. Peters, Michael // BMA A-Z Family Medical Encyclopedia;2004, p620 

    A definition of the term "port-wine stain" is presented. It refers to a purple-red birthmark which is permanent, non-raised type of haemangioma. It is a feature of Sturge-Weber syndrome.

  • Sturge-Weber syndrome with bilateral cerebral calcifications but without a facial nevus. Kumar, Surender; Anand, Sucharita; Ambesh, Paurush; Paliwal, Vimal Kumar // Neurology India;Nov/Dec2015, Vol. 63 Issue 6, p979 

    A letter to the editor focusing on Sturge-Weber syndrome, a sporadic-congenital syndrome characterized by capillary-venous angiomas is presented.

  • Left-Sided Facial Nevus With Contralateral Leptomeningeal Angiomatosis in a Child With Sturge-Weber Syndrome: Case Report. Widdess-Walsh, Peter; Friedman, Neil Roy // Journal of Child Neurology;Apr2003, Vol. 18 Issue 4, p304 

    Sturge-Weber syndrome is characterized by a facial vascular nevus associated with an ipsilateral leptomeningeal angioma. Variants of this classical presentation have been described in the literature, some of which have prognostic significance. We report a magnetic resonance imaging...

  • Sturge -- Weber syndrome: A case report. Gill, Namrata C.; Bhaskar, Nandini // Contemporary Clinical Dentistry;Jul-Sep2010, Vol. 1 Issue 3, p183 

    Sturge-Weber angiomatosis is a rare, nonhereditary developmental condition characterized by a hamartomatous vascular proliferation involving the tissues of brain and face. A report of a case with facial port wine stains, gingival overgrowth, and dilated ocular vessels is described.

  • Sturge-Weber syndrome presenting with intractable seizures. Roka, Y. B.; Puri, P. R.; Ahmad, W. S.; Bista, B. K. // Journal of Institute of Medicine;Apr2010, Vol. 32 Issue 1, p40 

    Sturge-Weber syndrome (SWS) also known as encephalotrigeminal angiomatosis is one of the many neurocutaneous syndromes consisting of port wine stain (facial nevus flammeus), congenital glaucoma and cortical calcification with anomalous leptomeningeal venous plexus. A child with intractable...

Share

Read the Article

Courtesy of THE LIBRARY OF VIRGINIA

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics