Hirschsprung Disease with Vsd-Pul.Atresia and PDA

[摘要] A four month old male infant was referred to the pediatric clinic of Afshar Hospital in Yazd city becauseof cyanosis. On physical examination, patient had central cyanosis, single second heart sound, A grade III/VIcontinuous murmur in left clavicular area, abdominal distension and large sized smooth and movableabdominal mass in right upper quadrant of abdomen. Transthoracic echocardiography showed VSDpulmonaryatresia and small patent ductus arteriosis. Plain abdominal X Ray showed right sided abdominalmass. Ultra sonography showed a mass lesion in RT upper quadrant of abdomen with diameters of5cmX12cm that was suggestive of fecaloma. Soap enema was performed and after that abdominal massdisappeared and abdominal distension was improved. Rectal biopsy 5cm from anal verge showed absence ofganglion 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 sigmoidarea. Therefore, definite diagnosis of the case was Hirschsprung disease with VSD- Pul. Atresia and smallPDA. 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 pulmonaryAtresia- VSD and PDA has been reported to date, the above case has been reported.

[学科分类] 药学、药理学、毒理学（综合）

[关键词] demosponge;spiculite;turbidite;Silesian Basin;Outer Carpathians;Cenomanian