目的 提高對兒童Abernethy畸形的認識。 方法 歸納總結2001年1月－2012年11月我國文獻報道的13例兒童Abernethy畸形(即先天性肝外門腔分流，臨床分為Ⅰa型、Ⅰb 型和Ⅱ型)的臨床表現和治療方法。 結果 13例兒童Abernethy畸形中，11例Ⅰ型（Ⅰa型4例、Ⅰb型7例）和2例Ⅱ型患兒，1歲內發病6例，確診前病程≥5年6例。在11例Ⅰ型患兒中，便血3例，嘔血4例，肝功能異常4例，肝硬化1例，肝性腦病2例，肝臟結節3例，脾臟腫大4例，脾功能亢進3例，門脈高壓3例，合并畸形3例。在2例Ⅱ型患兒中，便血1例，肝功能異常伴肝硬化、脾臟腫大和脾功能亢進1例，肝臟結節1例；無嘔血和合并畸形。門脈系統血液分流到下腔靜脈2例、髂內靜脈4例、左腎靜脈1例、奇靜脈1例、右心房2例和盆靜脈叢1例，其余2例不詳。患兒多采用保守治療，如有巨脾、便血或嘔血，脾臟切除和結扎乙狀結腸周圍血管手術也有采用。 結論 Abernethy畸形以Ⅰ型患兒為主，發病年齡較早，臨床表現不具特異性。診斷依賴于影像學檢查，螺旋CT血管造影對診斷有較高價值。個體化治療應根據畸形類型和病情而定。
目的 探討Abernethy畸形致下消化道出血的診斷及治療方法。方法 回顧1例Abernethy畸形患者的診治情況，并結合相關文獻進行分析。結果 血管造影確診后經積極術前準備，行剖腹探查、乙狀結腸造瘺術，術后3周開始給予中藥保留灌腸至術后7周，6個月關閉造瘺,3周后繼續輔以中藥保留灌腸4周。患者順利恢復出院，隨訪9個月未再次出血。結論 正確的診斷及合理的治療方案是救治的關鍵。
Objective To explore the clinical presentation and diagnosis and treatment of prehepatic portal hypertension （PPH） and discuss its surgical strategies. Methods Forty-six cases of PPH treated in the 2nd Artillery General Hospital and Peking Union Medical College Hospital from January 2000 to May 2009 were analyzed retrospectively, including 2 cases of Abernethy abnormality. All patients were evaluated by indirect portal vein angiography, CT angiography and (or) portal duplex system Doppler ultrasonography before treament. Surgical strategies included: 23 cases with meso-caval shunt, 8 cases with splenectomy and spleno-renal vein shunt, 1 case with porta-caval shunt, 2 cases with paraumbilical vein-jugular vein shunt, 3 cases with portal azygous disconnection, 1 cases with splenectomy and portal azygous disconnection, 1 case with sigmoidostomy and closed the fistula of sigmoid six months later, 1 case with resection of part of small intestine due to acute extensive thrombosis of portal vein system, 4 cases with selective superior mesenteric artery and (or) splenic artery thrombolytic infusion therapy, 2 cases remained no-surgical option and underwent conservative treatment. Results Forty-four patients were followed-up from 2 months to 5 years， average of 23.4 months, one patient without surgical treatment was lost. Satisfactory outcomes were obtained in 34 patients with various shunts, which expressed as a release of hypersplenism and gastrointestinal hemorrhage. Two cases were treated with meso-caval shunt because of rehemorrhage in month 13 and 24 and one died in month 8 after disconnection, one died on day 40 after thrombolytic therapy due to putrescence of intestines, one who remained no-surgical option underwent hemorrhage 4 months later, and then went well by conservative treatment. Conclusion The key of treatment of PPH is to reduce the pressure of hepatic portal vein. Surgical managements of shunt and selective superior mesenteric artery and (or) splenic artery thrombolytic infusion therapy are safe and effective, but individual treatment strategy should be performed.