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Abstract：
Nodular regenerative hyperplasia (NRH) of the liver may lead to noncirrhotic portal hypertension with subsequent development of portosystemic shunts. While extrahepatic and macrovascular shunts are readily visualized with imaging or endoscopy, there is no standard technique to detect intrahepatic microvascular portosystemic shunting and quantitatively assess shunt burden. We present a case of a 53-year-old female with suspected NRH and hepatopulmonary syndrome with inconclusive liver biopsies and absent portosystemic shunts per abdominal imaging. A percutaneous transportal infusion of Technetium-99m labeled macroaggregated albumin (99mTc-MAA) successfully identified intrahepatic microvascular portosystemic shunting and quantified a lung shunt fraction of more than 30%. NRH was subsequently confirmed with a surgical wedge biopsy and the patient was successfuly treated with a liver transplant. Transportal 99mTc-MAA could be used to both identify and quantify otherwise occult microvascular portosystemic shunts in patients with clinical sequelae of portal hypertension.
摘要：
肝脏的结节性再生增生（NRH）可能导致非肝硬化门脉高压，随后发生门体分流。虽然肝外和大血管分流很容易通过成像或内窥镜观察，目前尚无检测肝内微血管门体分流和定量评估分流负荷的标准技术。我们介绍了一例53岁女性，疑似NRH和肝肺综合征，肝活检不确定，每次腹部成像均无门体分流。Tech-99m标记的大聚集白蛋白（99mTc-MAA）的经皮门静脉输注成功地鉴定了肝内微血管门体分流，并量化了30％以上的肺分流率。随后通过手术楔形活检证实了NRH，并成功进行了肝移植治疗。跨门性99mTc-MAA可用于识别和量化门脉高压临床后遗症患者的隐匿性微血管门体分流。
