小儿门静脉海绵样变性的诊断及治疗

Diagnosis and Treatment of Cavernous Transformation of Portal Vein in Children

目的 探讨小儿门静脉海绵样变性的临床表现、诊断方法、治疗及预后等问题。 方法 16例门静脉海绵样变性患儿 ,14例首发症状表现为呕血、便血 ,2例表现为顽固性贫血 ,脾脏肿大是其重要体征 ,均经彩色多普勒获得明确诊断。均行脾切除、食道下端胃底静脉离断术 ,14例施行了不同方法的分流手术。 结果 均获随访 ,时间平均 3 (8月～ 7年 )。彩色多普勒显示分流处血管吻合口通畅 ,贫血症状明显好转。 3例患儿再次出现上消化道大出血症状 ,经内科保守治疗缓解。 结论 该病可能与先天因素有关。诊断主要根据影像学检查 ,其中彩色多普勒由于无创、可动态观察血流动力学、价格低廉等优点而被列为首选。治疗以手术治疗为主 。

Objective To discuss clinical manifestation,diagnosis,treatment and prognosis of cavernous transformation of portal vein. Methods 16 children of carvernons transformation of portal vein were retrospectively analyzed. The primary symptom was hematemesis or hematochezia in 14 children, refractory anemia in 2 children. Splenomegaly was the cardinal sign. All patients were diagnosed by multicolor doppler and performed splenectomy and ligation of inferior esophayus-fudus stomachal vein. 14 patients received different portosystemic shunts. Results All patients were followed up for 8 months to 7 years (average 3.5 years). Doppler showed blood stream smoothy at anastomotic stoma of the shunt place. The symptoms of anemia improved obviously. 3 patients occurred hemorrhage of digestive tract and got remission after conservative treatment. Conclusion The disease may be associated with congenital factors. Diagnosis is mostly depended on image examinations. Multicolor doppler is the first choice because of no trauma, low cost, and dynamic observation of blood stream. The basic methods of treatment is operation, portosystemic shunt can gain good effects.