RESUMO
We present an unusual case of Currarino syndrome with a mucosa-lined deep perineal fissure extending to the sacrum, penoscrotal transposition, perineal hypospadias, and a penile ventral skin defect. The child had a sigmoid diverting colostomy because of high anal atresia. Magnetic resonance imaging illustrated absence of the levator ani and muscle complex in the pelvis. At 15 months, perianal examination pointed out a fistula orifice and a sac related to the fistula at the left side of the perineal fissure. The fistula, a fluid-filled sac extending to the sacrum, and mucosa overlying the perineal fissure were removed en bloc. The neck of the sac was ligated and divided at the level of the distal sacrum. In the same session, a Glenn-Anderson procedure was performed for penoscrotal transposition, and the penile chordee was released. X-ray showed a bony deformity of the sacrococcygeal region in the shape of a scimitar. Histopathological examination demonstrated that the sac contained glial neuronal islands and nerve fibers. The boy has no neurologic deficits and seems to be well. To our knowledge, these associated malformations are extremely rare.
Assuntos
Anormalidades Múltiplas , Fissura Anal/congênito , Pênis/anormalidades , Sacro/anormalidades , Escroto/anormalidades , Fissura Anal/cirurgia , Seguimentos , Humanos , Recém-Nascido , Masculino , Pênis/cirurgia , Procedimentos de Cirurgia Plástica , Escroto/cirurgia , SíndromeRESUMO
In three infants with clinically mild necrotizing enterocolitis, heme-positive stools were initially attributed to anal fissures, delaying diagnosis and treatment of the enterocolitis for 4-10 days. Radiographically, pneumatosis intestinalis involved the entire colon and rectum. A causal relation may exist between necrotizing enterocolitis and anal fissure.