Serviço de Cirurgia Pediátrica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE
4th World Congress of Pediatric Surgery, 13 a 16 de Outubro 2013, Berlim.
Resumo:
Currarino´s triad (CT) is a rare combination of anorectal malformation, sacral defect and a pressacral mass. Clinical variability may lead to delayed diagnosis. Several urogenital abnormalities and obstipation have been associated with CT and may lead to worse long term prognosis. We report three cases of CT.
We retrospectively reviewed 3 patients with CT. Clinical and imagiological data, urinary and fecal symptoms before and after surgery are presented.
Patient 1 presented with anal stenosis, sacro-coccigeous teratoma and a sacral malformation in the neonatal period. A posterior sagittal anorectoplasty (PSARP) and pressacral mass excision were performed. No spinal cord dethethering was done. During follow up, patient reported recurrent urinary tract infections and bilateral vesicoureteral reflux with nephropathy that led to renal failure and left nephrectomy. Neurogenic bladder and refractory obstipation are also present.
Patient 2 presented with non-corrected anorectal malformation with perineal fistula. Subsequently, at 3 years of age, developed obstipation and soiling refractory to laxative therapy. Underwent x-ray that revealed scimitar sacrum, computerized tomography scan showed a pre-sacral mass, hemi-sacrum and anal canal stenosis. Magnetic resonance (MRI) showed a cystic pre-sacral lesion and megacolon. Patient was treated with a PSARP and excision of the mass of the filum terminale. After surgery, patient was continent for urine and feces.
Patient 3 was diagnosed at 13 months presenting with an infected sacro-coccigeous abscess and anal stenosis associated with obstipation. MRI showed global dilatation of the recto-sigmoid colon, defect of the distal sacral fusion and an anterior myelomeningocele. This patient is waiting for definitive surgery. Patient's mother also has a history of CT.
Clinical variability may lead to late diagnosis in CT cases. Urinary and colo-rectal tract function must be strictly monitored as it could be altered throughout follow up.