Digestive tract disorders associated with asplenia/polysplenia syndrome
The authors reviewed experience gained over a 20-year period of asplenia or polysplenia syndrome, focusing on patients with associated digestive tract disorders (DTDs). Eleven of 27 patients (40%) with asplenia/polysplenia had associated DTDs. The DTDs comprised malrotation of the intestine in nine, both preduodenal portal vein and gastric volvulus in three, esophageal hiatal hernia in two, and biliary atresia in one. Laparotomy was carried out on four patients with symptoms of acute bowel obstruction and on one patient with biliary atresia. One patient with both malrotation and gastric volvulus, and another with only associated malrotation survived. Nine patients died, eight of cardiac insufficiency and one because of hepatic insufficiency. When infants are diagnosed with heterotaxia, they should be examined for other combined DTDs, because they may have a chance for survival if they undergo surgery when their condition is still stable.
Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis
The asplenia syndrome: a review of significant extracardiac structural abnormalities in 29 necropsied patients
Malrotation of the bowel in patients with congenital heart disease associated with splenic anomalies
Laparoscopic repair of gastric volvulus associated with wandering spleen in an adult: report of a case
Screening and Treatment of Intestinal Rotational Abnormalities in Heterotaxy: A Systematic Review and Meta-Analysis
Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation
Evaluation of the portal vein after duodenoduodenostomy for congenital duodenal stenosis associated with the preduodenal superior mesenteric vein, situs inversus, polysplenia, and malrotation
Abnormalities of intestinal rotation in patients with congenital heart disease and the heterotaxy syndrome
Heterotaxy and intestinal rotation anomalies: 20 years experience at a UK regional paediatric surgery centre
Single institution experience with the Ladd's procedure in patients with heterotaxy and stage I palliated single-ventricle
Fetal echocardiographic evaluation of atrial morphology and the prediction of laterality in cases of heterotaxy syndromes
Is Screening of Intestinal Foregut Anatomy in Heterotaxy Patients Really Necessary?: A Systematic Review in Search of the Evidence
Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.