Undetected central core disease myopathy in an infant presenting for clubfoot surgery.
A 1-year-old child was scheduled for two stage bilateral clubfoot surgery. Preoperative evaluation was normal and total intravenous anesthesia with a continuous sciatic nerve block was performed. Two months later, before the second clubfoot correction, a hip subluxation was evident suggesting a provisional diagnosis of neuromuscular disease. Anesthesia was identical, except that a femoral nerve block, necessary to permit a diagnostic muscle biopsy was performed. The perioperative course was uneventful but result of the muscular biopsy was surprising in that central core disease was diagnosed. Although congenital myopathies of all grades and severity exist, they are often mild and underestimated. Patients affected by central core disease are considered susceptible to malignant hyperthermia. Because a high prevalence of myopathic changes is reported in children undergoing clubfoot surgery, anesthesiologists must take precautions including a hightened awareness of these events and a high index of suspicion.
Zanette
G
G
Department of Medico-Surgical Specialties, Section of General and Special Anaesthesiology, University of Padua, Padova, Italy. gastone.zanette@unipd.it
Robb
N
N
Zadra
N
N
Zanette
L
L
Manani
G
G
D001706:Biopsy / Q000379:methods; D003025:Clubfoot / Q000150:complications* / Q000601:surgery*; D003937:Diagnosis, Differential; D006615:Hip / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology; D020512:Myopathy, Central Core / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D035583:Rare Diseases