title: Anesthesia in a patient with dyskeratosis congenita presenting for urgent subtotal gastrectomy.
abstract: Dyskeratosis congenita is a rare and complex congenital disease that may complicate surgical treatment and impact anesthetic care. We present the perioperative management of a patient with severe pancytopenia, respiratory dysfunction, and oral leukoplakia who presented for urgent surgery for removal of a gastric hemorrhagic malignant tumor. Important issues in the management of this patient include choice of anesthetic technique, correction of pancytopenia (thrombocytopenia in particular), judicious perioperative fluid management to avoid dilutional coagulopathy, antibiotic prophylaxis, and strict aseptic technique. Careful management of a potentially difficult airway and a higher likelihood of respiratory insufficiency further complicate patient care. Knowledge of this rare disease process and its potential impact on anesthetic management is paramount for safe perioperative patient care.
authors.affiliation: University of Medicine and Pharmacy Iuliu Hatieganu Cluj-Napoca; Regional Institute of Gastroenterology and Hepatology "O. Fodor", Cluj-Napoca, Romania. Electronic address: cmitre2001@yahoo.com.
authors.forename: Calin Iosif
authors.initials: CI
authors.lastname: Mitre
authors.affiliation: Department of Anesthesiology, University of Florida College of Medicine, Gainesville, FL, USA.
authors.forename: David M
authors.initials: DM
authors.lastname: Corda
authors.affiliation: General Surgery, Regional Institute of Gastroenterology and Hepatology "O. Fodor", Cluj-Napoca, Romania.
authors.forename: Flavius
authors.initials: F
authors.lastname: Dunca
authors.affiliation: 3rd Department of Surgery, Regional Institute of Gastroenterology and Hepatology, "O. Fodor," Cluj-Napoca; Iuliu Hatieganu University of Medicine and Pharmacy, Romania.
authors.forename: Cornel
authors.initials: C
authors.lastname: Iancu
mesh_terms: D000230:Adenocarcinoma / Q000601:surgery; D000328:Adult; D000758:Anesthesia / Q000379:methods*; D019871:Dyskeratosis Congenita / Q000503:physiopathology*; D005743:Gastrectomy / Q000379:methods*; D006471:Gastrointestinal Hemorrhage / Q000209:etiology / Q000601:surgery; D006801:Humans; D008297:Male; D019990:Perioperative Care / Q000379:methods; D013274:Stomach Neoplasms / Q000601:surgery*
keywords: Dyskeratosis congenita; Gastric cancer; Perioperative management; Thrombocytopenia