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Roth, Christian

Journal of Pediatric Endocrinology and Metabolism

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Sturge-Weber syndrome: presentation with partial hypopituitarism

Sunil Kumar Kota
  • Corresponding author
  • Department of Endocrinology, Medwin Hospital, Hyderabad, Andhra Pradesh, India
  • Email:
/ Lalit Kumar Meher
  • Department of Medicine, MKCG Medical College, Berhampur, Orissa, India
/ Siva Krishna Kota
  • Department of Anesthesia, Central Security Hospital, Riyadh, Saudi Arabia
/ Sruti Jammula
  • Department of Pharmaceutics, Roland Institute of Pharmaceutical Sciences, Berhampur, Orissa, India
/ Surabhi Venkata Satya Krishna
  • Department of Endocrinology, Medwin Hospital, Hyderabad, Andhra Pradesh, India
/ Kirtikumar D. Modi
  • Department of Endocrinology, Medwin Hospital, Hyderabad, Andhra Pradesh, India
Published Online: 2012-06-29 | DOI: https://doi.org/10.1515/jpem-2012-0056


Sturge–Weber syndrome (SWS) is a rare disorder involving central nervous system abnormalities. It manifests with a facial port-wine birthmark and a vascular malformation of the brain. Infants and children present with seizures and stroke-like episodes with focal neurologic deficits. Patients with Sturge-Weber syndrome carry the additional risk of developing hypothalamic- pituitary dysfunction, secondary to their central nervous system dysfunction. Although one would suspect the hypothalamic-–pituitary axis would be at risk for impairment given the abnormalities that often occur in Sturge-Weber syndrome, they are not commonly recognized by clinicians. Increased awareness of this potential complication in patients with this rare disease of Sturge-Weber syndrome is needed. We hereby report a case of SWS with partial hypopituitarism and consider it to be important that these patients should undergo routine evaluation of pituitary function in the face of any relevant clinical manifestations.

Keywords: hypothalamic-pituitary dysfunction; port-wine stain; stroke; Sturge-Weber syndrome

About the article

Corresponding author: Dr. Sunil Kumar Kota, Department of Endocrinology, Medwin Hospitals, Chiragh Ali Lane, Nampally, Hyderabad 500001, Andhra Pradesh, India Phone: +91-8008408648, Fax: +9140-66623441

Received: 2012-02-23

Accepted: 2012-05-11

Published Online: 2012-06-29

Published in Print: 2012-08-01

Citation Information: Journal of Pediatric Endocrinology and Metabolism, ISSN (Online) 2191-0251, ISSN (Print) 0334-018X, DOI: https://doi.org/10.1515/jpem-2012-0056. Export Citation

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