[Management of gastrointestinal malformations in children with asplenia syndrome].

Asplenia syndrome (AS) is a complex disorder that includes a variety of cardiovascular and gastrointestinal malformations (GMs), and its prognosis remains poor. We treated 40 AS patients between 2000 and 2010, 10 of whom underwent surgery to correct GMs. These comprised 7 hiatus hernias (HH), 1 intestinal malrotation (IM), 1 stomach volvulus, and 1 ompalocele. In this review, we consider the optimal surgical timing and methods of management, especially for HH and IM. Surgery was performed in 6 of 7 HH patients. Four of those underwent laparotomy, 1 thoracotomy, and 1 laparoscopy. The Toupet method or fixation of the His angle or hiatus closure was selected in each case. Because the stomach intruding into the mediastinum may cause pulmonary venous obstruction and respiratory impairment, surgical repair is preferably performed prior to Glenn surgery. Among the 9 IMs, 8 were nonrotational and required no treatment, and only 1 required surgical treatment. GMs in AS are difficult to manage because of the unique anatomy, meaning there are no fixed surgical techniques. Each patient must be carefully examined preoperatively, and the optimum surgical method selected. Close collaboration with cardiovascular surgeons is vital when determining the timing and method of surgery.

Sonographic prenatal diagnosis of malpositioned stomach as a feature of uncomplicated intestinal malrotation.

Intestinal malrotation is a developmental anomaly affecting the position and peritoneal attachments of the small and large intestines during fetal life. Most often the diagnosis is established in the first year of life on the basis of abdominal pain and bile-stained vomiting secondary to bowel obstruction. The antenatal diagnosis can be suggested by identification of the complications such as bowel dilatation, ascites or meconium peritonitis. We describe two cases of isolated antenatal gastric malposition without any other associated anomaly that were confirmed after birth to be due to intestinal malrotation. We suggest that such an antenatal finding should alert the paediatrician to close clinical follow-up and prompt the diagnosis and surgical treatment in case of abdominal pain and/or bilious vomiting.

Prenatal diagnosis of intrathoracic stomach (gastric herniation).

Intrathoracic stomach is a rare and serious congenital abnormality. The anomaly may be complicated by gastric volvulus and can lead to ischemic gastric infarction in the neonate. If diagnosed antenatally, neonatal management can be planned in advance so as to reduce morbidity. This anomaly must be differentiated from the more common congenital diaphragmatic hernia, as associated pulmonary hypoplasia is common in the latter and rare with gastric herniation. We report an infant born to a mother with Marfan's syndrome with the antenatal diagnosis of intrathoracic stomach. The ultrasound and magnetic resonance imaging features of this congenital abnormality are described. A review of the literature would indicate that this is the first case report of gastric volvulus diagnosed in utero.

[Two rare complications of percutaneous endoscopic gastrostomy: obstruction of the pylorus and gastrocolic fistula occurring in one patient]. / Zwei seltene Komplikationen der perkutanen endoskopischen Gastrostomie: Pylorusobstruktion und gastrokolische Fistel bei einem Patienten.

UNLABELLED: We report an 10 month old male infant, who developed an obstruction of the pylorus by dislocation of the retention disk 5 months after percutaneous endoscopic gastrostomy (PEG). This could be corrected under endoscopic control. Two years after PEG insertion diarrhoe occurred immediately after feeding caused by a gastrocolic fistula with dislocation of the retention disk in the colon transversum. An excision of the fistula and a resection of the colon segment were performed successfully. CONCLUSION: In patients with PEG and unclear abdominal symptoms a tube dislocation has to be kept in mind at any time.

An unusual case of gastric volvulus after laparoscopic paraesophageal hernia repair.

Laparoscopic surgery for paraesophageal hernia is well accepted. However, the complications of this relatively new procedure have not been thoroughly investigated. Only four cases of recurrent volvulus after paraesophageal hernia repair have been reported. A 52-year-old man presented with a large right-side paraesophageal hernia. He experienced a retroperitoneal midgastric volvulus despite correct orientation of the stomach distally and proximally. We report an unusual complication that seems congenital in origin. Diagnostic and corrective measures are suggested.

Congenital diaphragmatic hernia with gastric volvulus presenting as an acute tension gastrothorax.

This report describes a 3-month-old infant with acute severe respiratory distress in whom a diagnosis of congenital diaphragmatic hernia with tension gastrothorax and gastric volvulus was made. A review of the pathophysiology, clinical presentation, differential diagnosis, diagnostic evaluation, and treatment of congenital diaphragmatic hernia is presented.

[Volvulus in childhood]. / Der Volvulus im Kindesalter.

Gastric volvulus or volvulus of the small-bowel can occasionally be found in neonates and small infants. Since volvulus is an emergency case, the radiologist must know the characteristic radiological findings and the ultrasound signs in correlation to the clinical symptoms. Two forms of gastric volvulus can be distinguished: the organoaxial type and a mesenterioaxial form. Besides an idiopathic etiology, diaphragmatic alterations can be observed in children with volvulus of the stomach. Volvulus of the small-bowel occurs in children with malrotation type I or II or with nonrotation. Bile-stained vomiting starts within the first days of life and is followed by the clinical signs of high bowel obstruction and peritonitis. Primarily in cases of gastric volvulus, an ultrasound examination can show the wrong position of the stomach or the pyloric region. In cases of small-bowel volvulus, abnormal localization of the superior mesenteric artery can be demonstrated. The plain film features an upper small-bowel obstruction. Upper intestinal contrast studies may reveal the level of small-intestine obstruction. A contrast enema can rule out a concomitant colon nonrotation or malrotation. A rare form which can be misdiagnosed easily, is volvulus of the sigmoid with pathological elongation and positioning of the sigma. It appears mostly in school children with less urgent symptoms and can disappear spontaneously. A typical feature is pain in the left lower abdomen and complete obstruction in an opaque enema.

Chronic gastric volvulus in children--a ten-year experience.

Gastric volvulus is a rare entity in children. During the last ten-year period of 1977 to 1987, 21 children were operated upon with the diagnosis of chronic gastric volvulus at The Department of Paediatric Surgery, Hacettepe University School of Medicine. This study includes retrospective analysis of these cases. The patients were investigated with respect to associated anomalies, radiological diagnosis, surgical treatment and prognosis. Results are presented and discussed.