A case of spontaneous splenorenal shunt ligation during open laparotomy for treatment of hyperammonemia

患者は67歳女性.脳血管性認知症による幻視,記憶障害,意欲低下の治療中に血中アンモニア値の上昇を認めた.腹部造影CT 検査で脾静脈-左腎静脈シャントを認め,シャントによる高アンモニア血症が原因と考えられた.ラクツロース･分岐鎖アミノ酸製剤の内服では改善しないため,開腹下にシャント閉鎖術を施行した.術後の血中アンモニア値は正常化し意欲低下は改善したが,幻視,記憶障害は改善しなかった.頭部MRI検査の再検でCreutzfeldt-Jakob病が疑われた.先天性脾静脈-左腎静脈シャントによる高アンモニア血症に対しては,シャント血管の結紮･切離のみで良好な結果が得られる可能性がある.

Here we report on a 67-year-old woman with a splenorenal shunt. Her serum ammonia level was elevated during the treatment for hallucination, defect of memory and loss of motivation by vascular dementia. Enhanced computed tomography and angiography showed the presence of a splenorenal shunt. We considered that it was caused by hyperammonemia due to a splenorenal shunt. Firstly we chose in internal use of lactulos and branched chain amino acid to treat. However, it was not ineffective. On this account, we surgically ligated the splenorenal shunt during an open laparotomy. The preoperative high level of serum ammonia decreased to the normal range after the operation. Her loss of motivation was improved, but her hallucination and defect of memory were not improved. A Creutzfeldt-Jakob disease was suspected as her diagnosis by head MRI re-examination. Ligation a splenorenal shunt may be possible to give us good clinical course for hyperammonemia.

脾腎シャント

高アンモニア血症

シャント閉鎖術