Anesthesia in a patient with dyskeratosis congenita presenting for urgent subtotal gastrectomy

•Dyskeratosis congenita is a rare disease with anesthetic implications.•Pulmonary fibrosis, dental disease, and stem cell failure complicate care.•Choice of anesthetic, monitoring, and pain management depends upon the disease state.•We discuss a successful anesthetic approach in a patient with dyskeratosis congenita.

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.