Anesthetic Considerations in Jansen’s Metaphyseal Chondrodysplasia: A Case Report of a Pediatric Difficult Intubation in a Rare Type of Dwarfism

Authors: Jorge-Reynolds L M, Meyerson P, Rodriguez L I, Del Rio I, Mavarez A C*

DOI: 10.23880/accmj-16000166

Volume 4, Issue 4

Abstract

Metaphyseal Chondrodysplasia Type Jansen (MChTJ) is a very rare autosomal dominant skeletal disorder characterized by abnormal cartilage formation in the metaphysis, leading to progressive growth plate abnormalities and a characteristic short limb dwarfism. Skull and facial deformities, renal impairment, hypercalcemia, and hypophosphatemia are seen in most of these patients. We report a case of a 6-year-old male child with diagnosed MChTJ undergoing multiple teeth extraction under general anesthesia. Our intent is to elucidate possible problems that may be encountered in the anesthesia management of patients with MChTJ and discuss the periopertive measures to take when these arise.

Keywords: Jansen’s metaphyseal chondrodysplasia; General anesthesia: Difficult intubation; Congenital abnormalities; Dwarfism

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