megacystismicrocolonintestinal

Megacystismicrocolonintestinal (MCMI) is a rare congenital disorder characterized by a triad of findings: bladder exstrophy, a very small (microcolon) large intestine, and intestinal malrotation. The bladder exstrophy involves the bladder being exposed on the outside of the abdomen, with the pubic bones often separated. The microcolon aspect refers to a significantly underdeveloped and shortened colon. Intestinal malrotation is a condition where the intestines do not form in their normal position within the abdomen during fetal development.

This condition typically presents at birth with the visible bladder abnormality. The severity of the intestinal issues can vary. Infants with MCMI often have difficulty feeding and may experience intestinal obstruction due to the malrotation and the small size of the colon. Diagnosis is usually made shortly after birth based on physical examination and imaging studies such as ultrasound, X-rays, and contrast studies of the gastrointestinal tract.

The management of MCMI is complex and multidisciplinary, requiring the involvement of pediatric surgeons, urologists, and gastroenterologists. Surgical intervention is necessary to address the bladder exstrophy, reconstruct the gastrointestinal tract, and correct the intestinal malrotation. Prognosis depends on the severity of the intestinal involvement and the success of surgical repairs. Long-term follow-up is crucial to monitor for complications and manage ongoing feeding and growth issues.