Abstract
Asplenia syndrome has been known to be associated with multiple gastrointestinal anomalies, notable among them being situs inversus, malrotation and jejunal atresias. We report another rare association of Asplenia Syndrome with cyanotic congenital heart disease, hemifacial hypoplasia and colonic atresias.

Introduction
The classical asplenia syndrome or the Polhemus - Schafer - Ivemark syndrome includes congenital splenic agenesis, cardiac defects and partial situs inversus viscerum.¹ Asplenia syndrome with cardiac heterotaxy (situs indeterminus) has been associated with centrally located liver, absent spleen and two morphologic right lungs (right isomerism).² Association between asplenia with intestinal malrotation has been reported.^3,4 We present a case of asplenia associated with colonic atresias cardiac anomalies and hemifacial hypoplasia; and yet unreported association.

Case Report
Female child with birth weight of 2.5 kg, was brought 27 hours post-delivery (day 2 of life) with gross abdominal distension and respiratory distress. The child had not passed meconium since birth. She was a full term normal delivery, progeny of non-consanguineous marriage (2nd marriage) with elderly parents. The child had severe tachypnoea with respiratory rate approximately 80 per minute with pallor, gross abdominal distension (mainly upper abdominal) with shiny, stretched out skin. The facies were hypoplastic (Fig.1). The external genitals, spine were grossly normal. Child had severe acidosis and serum creatinine of 1.3 mg%.

Plain X-ray abdomen showed dilated, gas-filled bowel loop with soap-bubble appearance at both ends and paucity of gas in pelvis (Fig. 2).

Conray enema was done after acute fluid resuscitation in view of features suggestive of meconium ileus on plain X-ray. On exploration, grossly distended proximal colon with distal atresia (mucosal webs) were found, which was taken care of by double-barrel colostomy/distal enterostomy and evacuation of contents (Figs. 3 and 4).

Fig. 1 : Grossly distended abdomen with shiny, stretched out skin. Also note left hemifacial hypoplasia	Fig. 2 :Erect X-ray abdomen showing dilated bowel loop with soap bubble appearance. Liver is noted beneath both domes of diaphragm.
Fig. 3 : Distended, air/meconium filled proximal colon with collapsed small bowel.	Fig. 4 : Area of colonic atresia with sacculations.

Discussion
Asplenia syndrome with right isomerism (bilateral right-sidedness) has absent spleen, bilateral trilobed lungs, right sided stomach, symmetric liver occupying the entire upper abdomen with colonic atresias (at two places). The proximal colon was grossly distended with tricuspid regurgitation (TR).
Colonic atresias account for about 11.7% of all intestinal atresias5 and have an approximate incidence of 1:20000 live births.6 The association of colonic atresia with asplenia and hemifacial hypoplasia seems unreported.

Though primary resection and anastomosis can be attempted in patients with colonic atresias, proximal colostomy with staged reconstruction would be the preferred approach in severely distended, acidotic child like this one since it is the least morbid procedure and minimizes the risk of infection/anastomotic complications in view of asplenia with profoundly impaired reticuloendothelial clearance and decreased T-cell function.

References

1. Dorland’s illustrated Medical Dictionary, 27th edition : 1637.
2. Daniel Bernstein, Nelson textbook of pediatrics, 16th edition - Harcourt Asia/Saunders: 2000; 1404.
3. Paddock RJ, Arensman RM. Polysplenia Syndrome: Spectrum of gastrointestinal congenital anomalies. J Pediatr Surg 1982; 17 : 563-66.
4. Modad Schiller. Paediatric surgery, Mosby : 5th ed. 1998: 1546-47.
5. Gray SW, Skandalakis JE. Embryology for surgeons. The embryological basis for the treatment of congenital defects. Philadelphia, PA Saunders, 1972; 191.
6. Keith T Oldham. Paediatric Surgery, Mosby: 5th ed. 1998: 1361-3.