| Abstract | Noonan's syndrome exhibits Turner's syndrome-like symptoms, namely a short truncus, webbed neck, ocular hypertelorism, and ptosis. But, Noonan's syndrome has normal chromosomes(1). For associated cardiac anomalies, pulmonary stenosis, atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), and hypertrophic cardiomyopathy (HCM) have been reported(2). This syndrome is often associate with a risk of developing malignant hyperthermia. Recently we experienced anesthetic management in a patient with Noonan's syndrome with HCM who underwent orchiopexy. The details are described in the following paragraphs. uCase PresentationvA 6-year-old boy, height 110cm and weight 18kg, was scheduled for the surgery of bilateralorchiopexy. The patient was delivered at a gestational age of 36 weeks by forceps in an asphyctic state. A birth weight was 3,500g. An electroencephalographic examination revealed an abnormal spike. He had been under medication with anti-convulsant until the age of 3, but he has not had any obvious convulsive attacks. Developmental delays were evidentGhis neck became steady at 3 months and he learned to walk at 3 years. No chromosomal anomalies were recorded at a chromosomal analysis performed immediately after birth. Family history was unremarkable. |
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