Brazilian Journal of Anesthesiology
Brazilian Journal of Anesthesiology
Case Report

Anesthetic management of scoliosis operation in a pediatric patient with Frank-ter Haar syndrome: a case report

Cuidado anestésico em cirurgia de escoliose em paciente pediátrico com síndrome Frank-ter Haar: relato de caso

Irem Basaran, Ezgi Gozubuyuk, Nur Canbolat, Ipek S. Edipoglu, Mehmet I. Buget

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Frank-ter Haar syndrome is a rare disorder characterized by multiple skeletal, cardiovascular abnormalities and facial features. Some of these characteristic facial features are important for anesthesiologists to predict the difficult airway. We present the anesthesia management of an 8-year-old boy with Frank-ter Haar syndrome who underwent posterior spinal instrumentation operation for scoliosis. In these patients, it is vital to anticipate possible difficult intubation before surgery and make all necessary preparations.


Frank-ter Haar syndrome;  Airway management;  General anesthesia;  Scoliosis


[1] A. Zrhidri, I.C. Jaouad, J. Lyahyai, et al. Identification of two novel SH3PXD2B gene mutations in Frank-Ter Haar syndrome by exome sequencing: Case report and review of the literature Gene, 628 (2017), pp. 190-193

[2] Y. Frank, M. Ziprkowski, A. Romano, et al. Megalocornea associated with multiple skeletal anomalies: a new genetic syndrome? J Genet Hum, 21 (1973), pp. 67-72

[3] C. Tommasino, M. Albicini Anaesthesia and orphan diseases: difficult tracheal intubation in a child with Frank-ter Haar syndrome Eur J Anaesthesiol, 35 (2018), pp. 542-544

[4] DAS guidelines for management of unanticipated difficult intubation (2015)

[5] American Society of Anesthesiologists Task Force on Perioperative Blood Management Practice guidelines for perioperative blood management: an updated report by the American Society of Anesthesiologists Task Force on Perioperative Blood Management Anesthesiology, 122 (2015), pp. 241-275

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