Suspected malignant hyperthermia in a child with laminin alpha2 (merosin) deficiency in the absence of a triggering agent

• Published in: Pediatric anesthesia Vol. 16; no. 4; pp. 462 - 465
• Main Authors: Shukry, Mohanad, Guruli, Zurab V, Ramadhyani, Usha
• Format: Journal Article
• Language: English
• Published: France 01.04.2006
• Subjects: Anesthesia, Intravenous; Child; Dantrolene - therapeutic use; Dexmedetomidine - therapeutic use; Humans; Laminin - deficiency; Laminin - genetics; Male; Malignant Hyperthermia - drug therapy; Malignant Hyperthermia - physiopathology; Muscle Relaxants, Central - therapeutic use; Muscular Dystrophies - complications; Muscular Dystrophies - congenital; Phenotype
• ISSN: 1155-5645

Malignant hyperthermia (MH) is an inherited disorder of the skeletal muscles that can be triggered by many anesthetic agents. MH has different presentations and manifestations that makes it difficult to diagnose. Patients with laminin alpha2 deficiency have never been reported to be susceptible to MH. We present a suspected MH episode in the absence of classic triggering agents in a 7-year-old boy with laminin alpha2 (merosin) deficiency and congenital muscular dystrophy. The episode was diagnosed using the MH clinical grading scale and responded well to prompt management with dantrolene. We conclude that patients with laminin alpha2 deficiency may be susceptible to MH, and early suspicion and rapid treatment is vital in the management of MH. Anesthesiologists should be prepared to treat MH in susceptible patients even in the absence of a classical triggering agent.