Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
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Aplasia cutis congenita is a rare congenital absence of skin most commonly affecting the scalp. Although most defects are small and superficial, approximately 20% of cases involve absence of the skull. Such defects expose the brain and sagital sinus, with concomitant risk of fatal hemorrage, infection, or both. This anomaly most commonly presents as a solitary defect, but sometimes it may occur as multiple lesions. The lesions are noninflammatory and well demarcated, and range is variable from 0.5 cm to 10 cm or more. Although the majority of these scalp defects occurs sporadically, many family cases have been reported. Multiple causes have been suggested for aplasia cutis: genetic causes, syndromes and teratogens, intrauterine infection -varicella zoster virus, herpes simplex virus-, fetal exposure to cocaine, heroin, alcohol or antithyroid drugs. ⋯ In view of our experience and results, we believe that early surgery prevents vital risks, reduces local complications and makes easier the final reconstruction.