Anesthesia of the Patient with Zhu-Tokita-Takenouchi-Kim (ZTTK) Syndrome: A Case Report

Abstract

Zhu-Tokita-Takenouchi-Kim (ZTTK) syndrome is an extremely rare multiorgan disorder, first described in 2015. Nowadays, about 50 patients with ZTTK syndrome have been reported, but there are no data about management during anesthesia. ZTTK syndrome patients can be indicated for surgery of musculoskeletal deformations and corrections of cardiovascular or urogenital malformations. This syndrome can be challenging for the anesthetic team based on the clinical manifestation of the syndrome. Because there are no recommendations for the management of these patients, anesthesiologists have to study typical symptoms, anatomy and possible expected changes in pathophysiology in perioperative period. One of the most dreaded anesthetic complications, the scenario “can not intubate, can not ventilate” could occur in these patients. The goal of this publication is to show options for anesthetic and perioperative management of this new rare syndrome with no published studies about management and approach in the perioperative period. The anesthetic team should choose the safest available approach. We present the first case report of anesthesia of a patient with ZTTK syndrome, a 7-year-old boy indicated for posterior neuromuscular scoliosis correction and fusion. This case describes the author’s experiences with anesthetic management and mentions possible early postoperative complications. Adequate understanding of this syndrome can reduce perioperative complications and improve patient outcomes after surgery.