دوره 13، شماره 4 - ( پاییز 1384 )
جلد 13 شماره 4 صفحات 85-82 |
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Behjati - Ardakani M. Hirschsprung Disease with Vsd-Pul.Atresia and PDA. JSSU 2005; 13 (4) :82-85
URL: http://jssu.ssu.ac.ir/article-1-709-fa.html
URL: http://jssu.ssu.ac.ir/article-1-709-fa.html
بحجتی اردکانی مصطفی. معرفی یک مورد بیماری هیرشپرونگ با بیماری مادرزادی قلب نقص بین بطنی، آترزی شریان ریوی و کانال شریانی باز. مجله علمي پژوهشي دانشگاه علوم پزشكي شهید صدوقی يزد. 1384; 13 (4) :82-85
چکیده: (7713 مشاهده)
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.
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