Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome: A Case Report

Abstract

Megacystis Microcolon Intestinal Hypoperistalsis Syndrom (MMIHS) is a quite rare congenital and fatal disease which was firstly defined by Berdon and his colleagues. It appears through a widely enlarged bladder and microcolon and its cause is unknown (Berdon et al., 1976; Carmelo et al., 2005; Makhija et al., 1999; Loening-Baucke and Kimura 1999; Redman et al., 1984; Hsu et al., 2003; Yigit et al., 1996; Srikanth et al., 1993). The disease is found in females three or four times more than in males (Srikanth et al., 1993; Sen et al., 1993; Hirato et al., 2003). Most of the cases die within the early months of their lives (Yigit et al., 1996; Srikanth et al., 1993; Sen et al., 1993; Hirato et al., 2003). We present the case of a female newborn with antenatal ultrasound revealing intestinal mass and bilateral hydroureteronephrosis. The case was admitted for intestinal obstruction after birth.

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“…Firstly, intestinal conditions, such as malrotation and short bowel with the resulting functional obstruction of the bowel may cause bacterial overgrowth and translocation, hence, leads to bacteremia and subsequent sepsis. Moreover, urinary tract infections (UTI) as well as catheter-induced sepsis resulting from prolonged TPN can augment the sepsis (20). Lastly, in several cases, attempts to provide sufficient enteral feeding have resulted in fatal pneumonia (21).…”

Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome: A Case Report of an Uncommon Condition

“…It is characterized by gastrointestinal and genitourinary dysmotility, resulting in its main manifestations: megacystis, microcolon, and decreased peristalsis. Additionally, it is often accompanied by multiple alterations, especially gastrointestinal and genitourinary, such as esophageal dilatation, stomach dilatation, dilated loops of the small intestine, intestinal malrotation, urinary tract dilatation, and vesicoureteral reflux [4][5][6][7][8][9]. Most patients are diagnosed after birth, but prenatal diagnosis is possible in up to 25% of cases based on ultrasound findings [4,8].…”