A 9 days newborn has been operated on for necrotic perforation of the small intestine and a double-barreled ileostomy was carried out. Postoperatively the child develops ascites and chronic jaundice. Liver ultrasonography revealed hepatic fibrosis. At the second month after resolution of symptoms the echocardiography establishes a congenital superior vena cava stenosis. A second stage surgery is foreseen - namely a closing of the ileostomy. What next?
Firstly, is it realy congenital or acquired (e.g. protrombotic state)? Secondly, is it hemodynamically significant SVC stenosis? Thirtly, is there any relationship between former events like bowel perforation,ascites and chronic jaundice (inferior vena cava syndrome?) and SVC stenosis? It seems that this late diagnosis is incidentally. If it is symtomatic, endovascular appoach is best choice. Finally, are the studies intended for thrombosis done?
Thank you Dr.Kohli and Dr.Aydin,
Well,both perforations of the distal small intestine were not of the type of NEC. I presume that the cause of the ischemic perforation was a primary stenosis of the superior vena cava. Similar evidence present also the finding by the echocardiography. Transient thrombocytopenia was observed, probably due to liver stagnation. Currently, the baby’s status is stabilized. Our radiologists consider endovascular dilatation.
Do you think it is appropriate to carry out a MRI with vascular contrast program?
Dear Brankov,
These intestinal perfoations may be spontanous intestinal perforations (SIP). Transient thrombocytopenia may also be due to thrombo-embolic processes. I think contrast MRI is reasonable, and my opinion endovascular dilatation should be performed only if it is hemodinamically significant.
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