[Megacystis-microcolon-intestinal hypoperistalsis syndrome: a case of prolonged survival].
Megacystis-microcolon-intestinal hypoperistalsis syndrome is a multisystemic disorder in which impaired intestinal motor activity causes recurrent symptoms of intestinal obstruction in the absence of mechanical occlusion, associated with bladder distention without distal obstruction of the urinary tract. It is an uncommon disorder with high morbidity and mortality, and long-term total parenteral nutrition may be required. We describe an 18-year-old girl with long-term survival, followed-up by our service since birth. With medical, surgical and nutritional management she currently presents a good oral tolerance and good quality of life, only requiring parenteral nutrition during periods of decompensation.
Jiménez Gil de Muro
S T
ST
Unidad de Gastroenterología y Nutrición Pediátrica, Hospital Infantil Universitario Miguel Servet, Zaragoza, Spain. silviajgdm@yahoo.es
Moros Peña
M
M
Gimeno Pita
P
P
Castejón Ponce
E
E
Ros Mar
L
L
D000293:Adolescent; D003106:Colon / Q000002:abnormalities* / Q000000981:diagnostic imaging; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007410:Intestinal Diseases / Q000000981:diagnostic imaging; D010528:Peristalsis; D011859:Radiography; D017741:Survivors; D013577:Syndrome; D001745:Urinary Bladder Diseases / Q000000981:diagnostic imaging