Anesthetic management in an infant with tetra‐amelia syndrome with congenital maxillomandibular fusion: A case report

Abstract

A 10-month-old girl who had tetra-amelia syndrome and congenital maxillomandibular fusion (syngnathia) was scheduled for the surgical fusion separation. Anesthetic management for this case was considerably challenging. Standard monitoring was still applied to the patient's extremities. IV access was suspected to be difficult but firmly needed before intubation to provide resuscitation during an emergency. Connecting anesthetic circuit with nasopharyngeal airway was the preferred technique due to its benefits such as maintaining spontaneous ventilation, providing inhaled anesthetic, as well as monitoring oxygenation and ventilation. Importantly, the cornerstones for handling such complicated cases are multidisciplinary approach and teamwork.

Early Surgical Intervention of Congenital Syngnathia

“…A total of 70 cases have been reported up to date after reviewing the literature on bony syngnathia case reports by Kumar et al15 and case reports published from 2019 to 2022,16–21 including the present report. Expression patterns or sex distribution for syngnathia were variously observed (Supplemental Digital Content, Table 3, http://links.lww.com/SCS/E634).…”