Currarino syndrome associated with penoscrotal inversion and perineal fissure.
Pediatr Surg Int
; 21(9): 733-5, 2005 Sep.
Article
en En
| MEDLINE
| ID: mdl-16025272
ABSTRACT
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.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Pene
/
Sacro
/
Escroto
/
Anomalías Múltiples
/
Fisura Anal
Tipo de estudio:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Humans
/
Male
/
Newborn
Idioma:
En
Revista:
Pediatr Surg Int
Asunto de la revista:
PEDIATRIA
Año:
2005
Tipo del documento:
Article
País de afiliación:
Turquía