Shapiro-Shulman and Sturge-Weber Syndromes

Abstract

In 1976, Shapiro and Shulman described a condition consisting of bilateral facial nevi involving the lower face and upper cervical areas, macrocrania, cephalic venous hypertension, and anomalous intracranial venous return [ [1] Shapiro K. Shulman K. Facial nevi associated with anomalous venous return and hydrocephalus. J Neurosurg. 1976; 45: 20-25 Crossref PubMed Scopus (20) Google Scholar ]. We read with great interest the report by Prats Viñas et al. [ [2] Prats Viñas J.M. Suinaga E. Blanco Lago R. Shapiro Shulman syndrome: A forgotten condition. Pediatr Neurol. 2011; 44: 308-310 Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar ], who highlighted a controversial issue concerning the nature of this syndrome: is it a variant of Sturge-Weber syndrome, or a discrete entity? Based on clinical and magnetic resonance findings in one of their patients, the authors defend the second opinion. Shapiro Shulman Syndrome: A Forgotten ConditionPediatric NeurologyVol. 44Issue 4PreviewWe report on a girl with cutaneous angioma and hydrocephalus who presented the characteristics of the condition described by Shapiro and Shulman. At birth, she manifested extensive pink discoloration on her face, scalp, and back. During infancy, she developed hydrocephalus, which later stabilized. Magnetic resonance angiography revealed anomalous intracranial venous drainage, which led to development of her hydrocephalus. Various authors described such a process in the 1970s and 1980s, interpreting it as a variant of Sturge-Weber syndrome, and listing it with other neurocutaneous syndromes associated with vascular nevi, but few references to this condition appear in the recent literature. Full-Text PDF ResponsePediatric NeurologyVol. 47Issue 3PreviewWe appreciate the interest shown by Azar and Robert in our article [1] and in the suggestion that Shapiro-Shulman syndrome should not be considered a different entity from Sturge-Weber syndrome. Full-Text PDF