A Case of Asplenia Syndrome With Microgastria and Hiatal Hernia Surgically Managed With Hunt-Lawrence Pouch

Abstract

Asplenia syndrome is one of the multiple congenital anomalies, which has right atrial isomerism and complex cardiovascular anomalies. To increase survival, the management of this syndrome is important for not only the cardiovascular anomalies but also the associated gastrointestinal anomalies. We report the case of a boy with asplenia syndrome who had hiatal hernia and microgastria, whose stomach was completely displaced to the mediastinum, which was surgically managed with a Hunt-Lawrence pouch. The patient underwent palliative surgery for the single ventricle at 1 month of age, and then underwent repair for hiatal hernia and gastrostomy at 3 months of age. Because of the small capacity of his stomach, he still had severe gastroesophageal reflux. After undergoing the Fontan procedure, he had cardiovascular radical surgery at 2 years of age. Gastric augmentation was accomplished at 2.5 years of age, with the construction of a Hunt-Lawrence pouch in order to increase the gastric volume. He is now making satisfactory progress. The associated anomalies in asplenia syndrome are generally complicated for both cardiovascular and gastrointestinal systems. In the management of this syndrome, it is important for each medical team to be in close collaboration with each other, select the best timing and method for the treatment, and plan a multidisciplinary therapeutic strategy.