A 2795-g infant was born to a 20-year-old gravida 3, para 0 mother at 40 weeks gestation by Caesarean section following failed induction. The child was noted to be profoundly cyanotic within minutes of birth, was started on Prostaglandin E1 and transferred to this hospital. An initial chest and abdominal radiograph were obtained (Figure 1). A cardiac catheterization demonstrated pulmonary atresia, double outlet right ventricle with a large VSD with single ventricle physiology, total anomalous pulmonary venous return to the inferior vena cava, bilateral superior venae cavae and pulmonary artery branch stenoses. Because of this, the child was taken to the operating room where a right modified Blalock Taussig shunt, left atrial reimplantation of total anomalous pulmonary veins and repair of bilateral branch pulmonary artery stenoses. Subsequently, an abdominal sonogram (Figure 2) and upper gastrointestinal series (Figure 3) were performed.
DENOUEMENT AND DISCUSSION
The abdominal sonogram and plain radiographs demonstrated a transverse liver. Additionally the sonogram showed a left upper quadrant gall bladder, normal intrahepatic portion of the inferior vena cava and no discernible spleen. Given the heart disease and sonographic findings, a diagnosis of asplenia syndrome was made. The upper gastrointestinal series demonstrated congenital microgastria with a tubular stomach with a short antrum, located in the midline, and intestinal malrotation. The child was taken to the operating room after having stabilized from the cardiac surgery. A Ladd's procedure with lysis of duodenal adhesions was performed. A gastrostomy tube was placed. The absence of the spleen and microgastria were confirmed. Because of the child's tenuous cardiac status, no further surgery was done.
Asplenia syndrome is a syndrome of right isomerism or attempted bilateral right sidedness.1 Bilateral right-sidedness is manifest as bilateral trilobed lungs, bilateral right atrial appendages, transverse liver and absence of the spleen.1,2 Asplenia syndrome is usually associated with severe congenital heart disease including complete atrioventricular canal, transposition of the great arteries, anomalous pulmonary venous drainage and pulmonic or less commonly aortic stenosis or atresia. Because of the severity of the heart disease and the immune defect from absence of the spleen, the mortality among patients with asplenia is high.3
Intestinal malrotation and malfixation are present in essentially all patients with asplenia syndrome.1,4 A prophylactic Ladd procedure is recommended to prevent the occurrence of midgut volvulus.1 In addition gastric volvulus5 and microgastria also have been described in patients with asplenia syndrome. The risk of gastric volvulus is unknown although increased over patients with normal atriovisceral situs presumably because of lack of normal gastrolienal ligaments.
Congenital microgastria is a rare anomaly usually associated with other malformations, especially VACTERL association, asplenia syndrome, and multiple limb anomalies.6,7,8 Rarely, congenital microgastria has been reported in patients without other anomaly.9 Congenital microgastria can be characterized as a small, tubular or saccular appearing stomach without definite less and greater curvature and a widely open, incompetent gastroesophageal junction, allowing free reflux. The small stomach is usually located near the midline.5 Clinically, patients with microgastria present because of failure to thrive, vomiting and malnutrition. The mortality untreated is very high.5 The esophagus is usually dilated with an incompetent lower esophageal sphincter. It is likely that the esophageal dilatation is a compensatory mechanism to increase the reservoir capacity of the small stomach.5
Severe microgastria requires surgical intervention. This is usually done in the form of a food reservoir called a Hunt–Lawrence pouch, a procedure originally designed for patients after total gastrectomy. A food pouch is created from side-to-side anastomosed jejunal loops.10
Children with asplenia and microgastria may require staged surgery as in this case because of the severity of the congenital heart disease. Initially, Ladd procedure and gastrostomy were performed in this patient following Blalock Taussig shunt for palliation of severe cyanotic heart disease.
Applegate KE, Goske MJ, Pierce G, Murphy D . Situs revised: imaging of the heterotaxy syndrome. Radiographics 1999;19:837–852.
Ho SY, Cook A, Anderson RH, Allan LD, Fagg N . Isomerism of the atrial appendages in the fetus. Pediatr Pathol 1991;11:589–608.
Wu MH, Wang JK, Lue HC . Sudden death in patients with right isomerism (asplenism) after palliation. J Pediatr 2002;140:93–96.
Chang J, Brueckner M, Touloukian RJ . Intestinal rotation and fixatin abnormalities in heterotaxia: early detection and management. J Pediatr Surg 1993;28:1281–1285.
Aoyama K, Tateishi K . Gastric volvulus in three children with asplenic syndrome. J Pediatr Surg 1986;21:307–310.
Shackelford GD, McAlister WH, Brodeur AE, Ragsdale EF . Congenital microgastria. AJR 1973;118:72–76.
Cunniff C, Williamson Kruse L, Olney AH . Congenital microgastria and limb reduction defects. Pediatrics 1993;91:1192–1194.
Hasegawa S, Kohno S, Tamura K, Urushihara N . Congenital microgastria in an infant with the VACTERL association. J Pediatr Surg 1993;28:782–784.
Ramos CT, Moss L, Musemeche CA . Microgastria as an isolated anomaly. J Pediatr Surg 1996;31:1445–1447.
Velasco AL, Holcomb GW, Templeton JM, Ziegler MM . Management of congenital microgastria. J Pediatr Surg 1990;25:192–197.
About this article
Cite this article
Herman, T., Siegel, M. Asplenia Syndrome with Congenital Microgastria and Malrotation. J Perinatol 24, 50–52 (2004). https://doi.org/10.1038/sj.jp.7211022