Hypothyroidism and Sturge-Weber Syndrome associated with Bilateral Port-wine Nevus

JOURNAL TITLE: International Journal of Clinical Pediatric Dentistry

Author
1. Anshul Gangwar
2. Gyanendra Saroj
ISSN
0974-7052
DOI
10.5005/jp-journals-10005-1339
Volume
9
Issue
1
Publishing Year
2016
Pages
4
Author Affiliations
    1. Department of Pedodontics and Preventive Dentistry Maulana Azad Institute of Dental Sciences, New Delhi, India
    1. Department of Pedodontics and Preventive Dentistry, Institute of Dental Sciences, Bareilly, Uttar Pradesh, India
  • Article keywords
    Hypothyroidism, PWSs, Sturge-Weber syndrome

    Abstract

    Sturge-Weber syndrome (SWS) is a rare, nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation of the brain, resulting in multiple angiomas that occur on the same side due to arteriovenous malformations. It is believed to be caused by persistence of a vascular plexus around the cephalic portion of the neural tube and is present at birth in about 1 in every 50,000 babies. It is one of the phakomatoses which is often associated with port-wine stains (PWSs) of the face, glaucoma, seizures, mental retardation and ipsilateral leptomeningeal angioma. Many people with SWS probably never know they have it. Hypothyroidism is a condition that arises from inadequate release of thyroid-stimulating hormone to stimulate an otherwise normal thyroid gland. This condition is often associated with a deficient secretion of other pituitary hormone, and growth hormone deficiency occurs with an increased prevalence in SWS, presumably secondary to involvement of the hypothalamic-pituitary axis. Diagnosis is made by the presence of a facial PWS and evidence of leptomeningeal angioma either by skull X-ray or computed tomography scan that shows intracranial calcifications. Presently, there is no specific treatment for SWS, and the management of the clinical manifestations and complications is still far from adequate. Here, we report the case of hypothyroidism associated with SWS with oral and facial manifestations in an 11-year-old boy. How to cite this article: Saroj G, Gangwar A, Dhillon JK. Hypothyroidism and Sturge-Weber Syndrome associated with Bilateral Port-wine Nevus. Int J Clin Pediatr Dent 2016;9(1): 82-85.

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