Neonatal gastric outlet obstruction by isolated pyloric atresia, an often forgotten diagnosis.

BACKGROUND: Pyloric atresia (PA) is a rare condition, and may be misdiagnosed and especially confused for duodenal atresia pre-operatively. We looked for clues to avoiding pre-operative misdiagnosis and hence allow the best neonatal medical and surgical management. METHODS: A retrospective case-note review was carried out of the five patients managed in four centres with the diagnosis of isolated PA. We focused on antenatal ultrasound findings, postnatal clinical and radiological features, operative findings, surgical procedures and outcomes. RESULTS: Four patients had polyhydramnios and one double bubble sign on antenatal ultrasound. After birth, non-bilious vomiting and upper abdominal distension were the main symptoms. Gastric decompression showed non-bilious gastric fluid. Radiological findings were a large gastric air bubble with no gas beyond in all cases. The diagnosis of duodenal atresia was postulated at first in all cases. The diagnosis of PA was established peroperatively. One patient referred late, died 13-day post-operatively of cardiopulmonary failure secondary to a severe pneumonia that may be related to aspiration syndrome. Outcomes were otherwise satisfactory. CONCLUSIONS: Even though it is a rare diagnosis, PA has a specific clinical and radiological presentation underlined here that should be kept in mind when managing a neonate with a gastric outlet obstruction.

An exceptional cause of intestinal obstruction in a 2-year-old boy: strangulated hernia of the ileum through Winslow's foramen.

The case of a 2-year-old boy suffering from a strangulated ileal herniation through Winslow's foramen into the lesser sac is reported. He presented an abdominal pain of sudden onset 16 hours previously. The clinical examination, abdominal x-ray, and sonography were not helpful in the assessment of the diagnosis of internal hernia, which was evoked by the computed tomography. At laparotomy, approximately 30 cm of the strangulated ileum was reduced through Winslow's foramen. Fifteen centimeters of the ileum was gangrenous and thus resected with an end-to-end anastomosis. Despite its congenital character, strangulated hernia of the ileum through Winslow's foramen is diagnosed more frequently in adulthood and exceptionally in childhood. Early diagnosis and treatment are crucial because of the high morbidity and mortality associated with strangulation.

An exceptional type of anorectal malformation: anorectal atresia with posterior fistula in a newborn male.

The authors report the case of a newborn male with an exceptional type of anorectal atresia with a fistula that passes posterior to the muscle complex and exits at a site just to the tip of the coccyx. This anorectal malformation was associated with other abnormalities including agenesis of the right kidney, pyelic dilatation of the left kidney, duplicity, and left megaureter. There was no defect in the spine. The anorectal malformation was managed successfully by a posterior sagittal approach according to the Pena procedure.

Conservative treatment of a mesenteric lymphangiomyomatosis in an 11-year-old girl with a long follow-up period.

The authors report the case of a 11-year-old girl admitted for a mesenteric tumor discovered during an appendicectomy. The abdominal Doppler echography and computed tomography (CT) scan showed a 8 x 6 x 7-cm multinodular mass involved in the mesenterium. Biological markers and iodine 131 meta-iodobenzylguanidine (MIBG) scintigraphy results were normal. Percutaneous biopsies were performed. Pathology results showed perilymphatic proliferation of smooth muscle cells leading to cystic lesions. No sign of malignancy was seen. Laparotomy showed a multinodular fixed mass involved from the root of the mesenterium. The superior mesenteric vessels were encircled by the tumor, which could not be resected completely. Partial resection was performed. The pathology and the immunohistochemical tests confirmed the diagnosis of lymphangiomyomatosis. At 21 years of age, she remains asymptomatic. An echography and CT scan showed the mass remaining unchanged in size and echopattern. Lymphangiomyomatosis (LMA) is thought to affect exclusively women in their reproductive life, and the steroid hormones are supposed to play an essential role in the pathology and in the behavior of this condition. Infantile LMA is exceptional. The patient reported is remarkable for her young age, the location of the tumor, her long history, and her benign course. Because the tumor is supposed to be hormone dependent, the surge of oestrogen in this 21-year-old woman may initiate the extensive form of lymphangiomyomatosis with pulmonary involvement, the prognosis of which is extremely poor.