Case Report: Dental treatment under general anesthesia for a child with Ullrich congenital muscular dystrophy.

Abstract

Background: Ullrich congenital muscular dystrophy (UCMD) is a rare inherited myopathy caused by mutations in the COL6 gene and is frequently associated with multisystem complications affecting the respiratory and skeletal systems, which may increase airway and anesthetic risks during dental treatment.

Case report: This report describes a 4-year-old girl with UCMD who presented with pain in the left lower molar. After a comprehensive multidisciplinary evaluation by the Department of Stomatology, Anesthesiology, and Pediatrics, the patient was classified as ASA III and underwent dental treatment under total intravenous anesthesia, with tracheal intubation performed under fiberoptic bronchoscopic guidance. The procedures included pulpotomy, tooth extraction, resin restoration, placement of preformed metal crowns, and topical fluoride application. The perioperative course was uneventful and no respiratory, airway, or anesthesia-related adverse events were observed. The patient was followed up continuously for 6 to 12 months after dental treatment.

Conclusion: This report delineates the multidisciplinary perioperative management of comprehensive dental treatment in a 4-year-old patient with UCMD, thereby providing a clinical reference for oral healthcare in individuals with this rare disorder.