Intractable Rare Dis Res. 2021;10(1):52-54. (DOI: 10.5582/irdr.2020.03116)

A rare challenge in general surgery: double surgical procedure for large and small bowel obstruction in a patient with Gerstmann- Sträussler-Scheinker syndrome

Costanzi A, Monteleone M, Berardi V, Miranda A, Mari G, Maggioni D


Gerstmann-Sträussler-Scheinker syndrome (GSS) is a rare, infectious syndrome related to a mutation in the prion protein gene. Described here are the challenges posed by surgery for a patient with GSS. A 61-yr-old woman with GSS was admitted to this department and underwent surgery twice for large and small bowel obstruction. This is the first report of two major surgical procedures in a patient with GSS. Experiences with this case and precautions when using a disposable device during endotracheal intubation and a surgical procedure to manage a patient with GSS are described.

KEYWORDS: Gerstmann-Sträussler-Scheinker syndrome, general surgery, bowel obstruction

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