This case report highlights the perioperative anesthetic implications of LEOPARD syndrome, particularly concerning associated cardiovascular conditions like congenital heart disease and hypertrophic obstructive cardiomyopathy.
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May warrant attention to cardiac comorbidities in LEOPARD syndrome anesthesia; extends sparse case evidence but leaves open prospective validation.
LEOPARD syndrome is a neuroectodermal disorder presumed to result from an abnormality in neural crest cells. The acronym 'LEOPARD' is derived from the clinical features which include multiple lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormal genitalia, retarded growth, and deafness. Given the multisystem nature of the disease process, several issues may affect the perioperative care of these patients. Of primary importance are associated conditions of the cardiovascular system including congenital heart disease, conduction disturbances, and progressive hypertrophic obstructive cardiomyopathy. The authors present a 4-year old boy who presented for anaesthetic care for repair of a ventricular septal defect and pulmonary valvotomy for congenital pulmonary stenosis. The potential perioperative implications of LEOPARD syndrome are discussed.
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Torres-Borrego et al. (2004) studied this question.
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