BACKGROUND: We describe our experience in Currarino syndrome (CS) and our clinical findings. METHODS: Case 1: Newborn female with prenatal diagnosis of myelomeningocele which was confirmed at birth as a lipomyelomeningocele, associated with partial sacral agenesis, horseshoe kidney and complex anorectal malformation with colonic vascular anomaly. Case 2: A 14-month-old male with constipation. The physical examination detected an anal stricture and radiological findings of a presacral lipomeningocele plus teratoma and a partial sacral agenesis. Case 3: An 8-month-old male with recurrent meningitis associated with anterior sacral meningocele and rectal fistula. An anal stricture and hemisacrum were also demonstrated. RESULTS: In case 1, an abdomino-perineal pull-through was performed but it failed because of her colonic vascular anomaly. Nowadays she is 3 years old and has a cecostomy, controls her micturition and is able to walk. Case 2 is two years old with satisfactory flow-up-after the teratoma and meningocele removal plus anal dilation. In case 3 a colostomy was necessary because after mass excision a recto-cutaneous fistula appeared during postoperative period. A ventriculoperitoneal valve implantation due to his hydrocephaly was also performed. CONCLUSIONS: The constipation is the most common symptom in the CS. Recurrent meningitis has a high mortality rate (56%) due to rectomeningeal fistula. The most common anorectal malformation is the anal stricture, and the more complex the malformation is, higher will the risk of incontinence be. The success of the management and follow-up is a multidisciplinary responsability.
BACKGROUND: We describe our experience in Currarino syndrome (CS) and our clinical findings. METHODS: Case 1: Newborn female with prenatal diagnosis of myelomeningocele which was confirmed at birth as a lipomyelomeningocele, associated with partial sacral agenesis, horseshoe kidney and complex anorectal malformation with colonic vascular anomaly. Case 2: A 14-month-old male with constipation. The physical examination detected an anal stricture and radiological findings of a presacral lipomeningocele plus teratoma and a partial sacral agenesis. Case 3: An 8-month-old male with recurrent meningitis associated with anterior sacral meningocele and rectal fistula. An anal stricture and hemisacrum were also demonstrated. RESULTS: In case 1, an abdomino-perineal pull-through was performed but it failed because of her colonic vascular anomaly. Nowadays she is 3 years old and has a cecostomy, controls her micturition and is able to walk. Case 2 is two years old with satisfactory flow-up-after the teratoma and meningocele removal plus anal dilation. In case 3 a colostomy was necessary because after mass excision a recto-cutaneous fistula appeared during postoperative period. A ventriculoperitoneal valve implantation due to his hydrocephaly was also performed. CONCLUSIONS: The constipation is the most common symptom in the CS. Recurrent meningitis has a high mortality rate (56%) due to rectomeningeal fistula. The most common anorectal malformation is the anal stricture, and the more complex the malformation is, higher will the risk of incontinence be. The success of the management and follow-up is a multidisciplinary responsability.