Perforation of Malone antegrade continence enema: diagnosis and management

Severe bowel dysfunction often accompanies neurogenic bladder, and Malone antegrade continence enema (MACE) procedures can lead to improvement in the quality of life of these patients. However, complications such as catheter false passage with subsequent intraperitoneal instillation of irrigation can lead to significant morbidity. We present our experience with the diagnosis and management of this condition.

The records of all patients undergoing MACE procedures at pediatric institutions from 1989 to 2002 were retrospectively reviewed. The records of patients diagnosed with a perforation were assessed for presentation, initial imaging studies, management and outcomes.

Of 187 consecutive patients treated with MACE procedures we identified 6 females and 1 male (3.7%). Mean patient age at initial surgery was 11.3 years. Of the 7 patients presented within 3 months of the initial surgery, 6 presented with abdominal pain after irrigation and 4 reported traumatic catheterization. Six patients had extravasation of contrast material on imaging studies. Two patients presented with peritonitis and underwent immediate laparotomy. In 5 patients endoscopy was performed with catheter placement which was then maintained for 6 weeks. After a mean followup of 4.7 years 4 patients have complete continence, 2 have a MACE button in place and 1 has mild fecal leakage.

MACE procedures have a low incidence of conduit false passage and perforation. Prompt diagnosis and early intervention are crucial to management. Endoscopic evaluation with catheter placement can be helpful in preserving continence and decreasing morbidity.