CASE REPORTS Case 1A 28-year-old gravida 3, para 2 woman, was referred to our unit at 30 weeks' gestation with suspected fetal bowel obstruction. The patient and her husband were first cousins. Their medical histories were unremarkable and the woman had two healthy girls. Ultrasonography showed severe polyhydramnios with a deepest amniotic fluid pocket of 19 cm and multiple loops of dilated small bowel with visible peristalsis and no ascites or bowel-wall thickening. The scan was otherwise normal. Small bowel obstruction was suspected and the patient was counseled accordingly. She was offered and declined screening for cystic fibrosis. At 36 weeks' gestation she was admitted in labor, and a Cesarean section was performed on maternal request; the female neonate weighed 3145 g.The neonate had a severely distended abdomen with multiple dilated loops of small bowel on plain abdominal X-ray. An explorative laparotomy was performed with a preoperative diagnosis of small bowel obstruction. On laparotomy, multiple dilated loops of small bowel were observed with no mechanical obstruction. Appendectomy and rectal biopsy were performed. These were both normal, with normal ganglion cells on pathological examination. During the immediate postoperative period the patient remained obstructed, the urinary bladder was persistently enlarged and a neurogenic bladder was suspected. No other urinary tract abnormalities were observed on postnatal investigation. With a diagnosis of primary congenital intestinal pseudo-obstruction (CIPO) and possibly megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) the patient was put on total parenteral nutrition and gastrointestinal prokinetic medication. The obstruction gradually subsided, as did the bladder dysfunction. Within a month the infant was discharged requiring no medication and is currently 7 years old and in excellent health.
Case 2A 31-year-old gravida 5, para 4 woman -sister to the first patient -was referred to our unit at 30 weeks' gestation with polyhydramnios. Like her sister, she was married to a first cousin. She had previously given birth to four healthy children. On admission the ultrasound scan showed moderate polyhydramnios, the deepest amniotic fluid pocket being 13 cm. A detailed anatomy sonogram performed at this time revealed a normal stomach bubble and multiple hypoechogenic dilated small bowel loops (Figure 1). The dilated loops had a maximal diameter of 18 mm and visible peristaltic waves were recorded. Additionally, right pyelectasis was observed