Abstract

DOMINGUEZ SAMANIEGO, Sandra Violeta  and  VEGA, Osvaldo Guzmán. Gorham Stout Syndrome. A Case Report. Pediatr. (Asunción) [online]. 2020, vol.47, n.3, pp.175-179. ISSN 1683-9803.  https://doi.org/10.31698/ped.47032020010.

Gorham Stout Syndrome (GSS) is a bone disease of unknown etiology, characterized by idiopathic osteolysis and progressive absorption. It occurs at any age, without predilection for gender or race; it can affect any part of the skeleton, the diagnosis is clinical, radiological and histopathological and by exclusion of other common conditions. Spinal involvement is extremely rare and has a poor prognosis. It also affects the viscera, a common complication is the development of chylothorax, which occurs in approximately 20% of patients and carries a high mortality rate. There are no standard diagnostic methods or treatments for the disease. It poses a diagnostic and therapeutic challenge for clinicians. We present the case of a 5-year-old patient, diagnosed and managed by a multidisciplinary team at the Pediatric General Hospital (HGP), with a review of the literature on the topic.

Keywords : Gorham Stout syndrome; pediatrics; osteolysis.

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