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. 2024 Oct 10;10(1):64.
doi: 10.1186/s40981-024-00747-8.

Anesthetic management of a patient with mandibular hypoplasia, deafness, progeroid features, lipodystrophy syndrome: a case report

Ryo Sekiguchi  1 , Michiko Kinoshita  2 , Yoko Sakai  3 , Katsuya Tanaka  1   4
Affiliations

Anesthetic management of a patient with mandibular hypoplasia, deafness, progeroid features, lipodystrophy syndrome: a case report

Ryo Sekiguchi et al. JA Clin Rep. .

Abstract

Background: Mandibular hypoplasia, deafness, progeroid features, and lipodystrophy (MDPL) syndrome is a rare autosomal dominant disorder that presents unique challenges for anesthetic management due to its multisystemic manifestations. This report outlines the anesthetic considerations for MDPL patients based on our case experience.

Case presentation: A 15-year-old male with MDPL syndrome underwent testicular extraction under general anesthesia. Insertion of a peripheral venous catheter was challenging due to scleroderma-like skin. Although the facial features of MDPL syndrome suggested a difficult airway, intubation with a McGrath™ Mac video laryngoscope was successful. Despite MDPL syndrome's association with hypertriglyceridemia due to lipodystrophy, this patient's triglyceride levels were normal. Thiamylal and sevoflurane were used without issues such as delayed emergence from anesthesia.

Conclusions: MDPL syndrome requires careful preoperative assessment and tailored anesthetic management due to potential airway challenges arising from its distinctive facial features and the possibility of altered anesthetic pharmacokinetics associated with lipodystrophy.

Keywords: Case report; General anesthesia; Lipodystrophy; MDPL syndrome; Mandibular hypoplasia; Progeroid syndrome.

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Conflict of interest statement

The authors declare that they have no competing interests.

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