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A four month old male infant was referred to the pediatric clinic of Afshar Hospital in Yazd city because of cyanosis. On physical examination, patient had central cyanosis, single second heart sound, A grade III/VI continuous murmur in left clavicular area, abdominal distension and large sized smooth and movable abdominal mass in right upper quadrant of abdomen. Transthoracic echocardiography showed VSDpulmonary atresia and small patent ductus arteriosis. Plain abdominal X Ray showed right sided abdominal mass. Ultra sonography showed a mass lesion in RT upper quadrant of abdomen with diameters of 5cmX12cm that was suggestive of fecaloma. Soap enema was performed and after that abdominal mass disappeared and abdominal distension was improved. Rectal biopsy 5cm from anal verge showed absence of ganglion cells in the intramural and sub mucosal plexuses. Ten day later, barium enema was performed. Barium enema showed dilated descending, transverse ascending colon and transition zone in recto sigmoid area. Therefore, definite diagnosis of the case was Hirschsprung disease with VSD- Pul. Atresia and small PDA. At first the patient underwent Right modified Blalock Taussig shunt (systemic to pulmonary shunt) and one month later underwent temporary colostomy. As no case of Hirschsprung disease with pulmonary Atresia- VSD and PDA has been reported to date, the above case has been reported.

Keywords: Hirschsprung Disease, VSD, Pulmonary Atresia, PDA, Systemic to pulmonary shunt, Colostomy

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