Prophylactic gastropexy in the asplenia syndrome

Abstract

The asplenia [Ivemark] syndrome (AS) is the association of congenital absence of the spleen with a variety of visceral abnormalities, predominantly of the cardiovascular system. Varying degrees of malrotation and malfixation of the bowel are common in this condition, and the occurrence of catastrophic gastric volvulus due to malfixation of the bowel has been reported. With the improvement in long-term outlook for these patients with modern cardiac surgery and prophylactic antibiotics, the intra-abdominal anomalies have become increasingly significant. This paper draws attention to the prophylactic treatment of gastric malfixation in the AS through the presentation of two cases in which gastropexy was performed.

Prenatal diagnosis of cardiosplenic syndromes: a 10‐year experience

“…Varying degrees of malrotation and malfixation of the bowel, preduodenal portal vein, gastric volvulus, esophageal hiatal hernia and biliary atresia are common in both left and right isomerism, with a predominance in left isomerism. With the improvement in long‐term outlook for these patients with modern cardiac surgery the intra‐abdominal anomalies have become increasingly significant38, 39. Children with right isomerism and asplenia who survive cardiac palliation are at great risk of dying from sepsis40–42.…”

Fetal echocardiographic evaluation of atrial morphology and the prediction of laterality in cases of heterotaxy syndromes

“…Varying degrees of malrotation and malfixation of the bowel, preduodenal portal vein, gastric volvulus, esophageal hiatal hernia and biliary atresia are predominant in left isomerism, while children with right isomerism and asplenia who survive cardiac palliation are at great risk of dying from sepsis17, 25–27. With the improvement in long‐term outcome for these patients with modern cardiac surgery, the intra‐abdominal anomalies have become increasingly significant28, 29. Accurate prenatal diagnosis of these syndromes prompts a thorough evaluation for digestive tract or splenic disorders in the neonatal period, with the use of prophylactic antibiotics and vaccination in preventing possible non‐cardiac complications.…”

Clinical implication of hiatal hernia in patients with right isomerism

“…GI tract anomalies associated with heterotaxy syndrome, including malrotation, malposition and malfixation of the abdominal organs, have been reported in the literature [1][2][3]9,10,16,17]. Due to lateralization failure in the embryo stage of heterotaxy syndrome, the ligaments that fix the GI tracts are usually defective, and this leads to malrotation, or even volvulus of the gut.…”