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.

Delayed motor, mental and speech development and congenital brain malformations: the first description of Zhu–Tokita–Takenouchi–Kim syndrome in Russia

“…Назначена противоэпилептическая терапия: вальпроевая кислота (Депакин Хроносфера) Мы представляем наиболее актуальную и подробную характеристику клинической картины синдрома ZTTK (90 пациентов, включая наших) (табл. 2) [3,[14][15][16][17][18][19][20]. Из источников литературы известно, что клиническая картина пациентов с миссенс-мутациями гена SON легче (отсутствуют пороки развития), чем у пациентов, имеющих мутации, приводящие к преждевременной терминации трансляции.…”

Anesthesia and Perioperative Management for Surgical Correction of Neuromuscular Scoliosis in Children: A Narrative Review

“…Difficult airway management should be expected with a higher rate in patients with neuromuscular disorders as some syndromes are associated with facial dysmorphism, abnormal habitus, or limited mobility, especially limited neck mobility [17,19]. Anesthesiologists should search for risk factors for difficult airway management during the preanesthesia visit and prepare for airway securing, including advanced airway techniques such as video laryngoscopy or awake fiber optic intubation [20][21][22].…”

“…Careful cardiologist examination with echocardiography is recommended in patients with myocardial dysfunction, because many neuromuscular syndromes are associated with heart disease, such as cardiomyopathy, valve disease, congenital heart disease, or limited stress tolerance. Preoperative cardiologic evaluation should be suggested in patients without myocardial dysfunction if pulmonary hypertension is suspected [10,20,26,27].…”

“…The indication of a central venous catheter should be considered individually according to the comorbidities and expectation of the need for vasopressors [10,27]. Ultrasound-guided cannulation can reduce the incidence of unsuccessful attempts and also increases the safety of the procedure [10,20]. Depth of anesthesia monitoring is recommended for TIVA to prevent overdosing and shorten the time of awakening after the surgery [10,18,27].…”