小儿门静脉海绵样变性误诊原因及预防对策

Misdiagnosis causes and prevention countermeasures of cavernous transformation of portal vein in children

目的探讨小儿门静脉海绵样变性(CTPV)的误诊原因及预防对策。方法回顾性分析19例门静脉海绵样变性患儿的临床资料。结果 CTPV主要表现为呕血和或便血,脾肿大,血常规白细胞、血红蛋白、血小板均减少。6例误诊为嗜血细胞综合征,4例误诊为再生障碍性贫血,1例误诊为白血病;2例误诊为遗传代谢病,1例误诊为消化性溃疡,1例误诊为肝炎,1例误诊为麦克尔憩室,3例误诊为肝硬化。15例患儿接受了手术治疗,5例保守治疗。结论小儿门静脉海绵样变性以脾大、脾亢及消化道出血为主要临床表现;误诊率高;查体不仔细,对本病认识不足,过分看重化验检查是误诊的主要原因;仔细体格检查,提高对本病认识,及时做彩色多普勒超声检查可最大程度的避免误诊。

Objective To investigate the misdiagnosis causes of cavernous transformation of the portal vein （CTPV） in children and the prevention countermeasures. Methods A retrospective analysis of clinical data of 19 cases of children with cavernous transformation of the portal vein was made. Results The main performance of CTPV included he- matemesis and/or hemafecia , splenomegaly, routine blood leukocyte, hemoglobin and platelets were reduced. 6 cases were misdiagnosed as hemophagocytic lymphohistiocytosis, 4 misdiagnosed as aplastic anemia, 1 misdiagnosed as leukemia. 2 misdiagnosed as genetic metabolic disease, 1 misdiagnosed as peptic ulcer, 1 misdiagnosed as hepatitis, 1 misdiagnosed as Meckel＇s diverticulum, 3 misdiagnosed as hepatic cirrhosis. 15 cases were underwent surgery treatment, 5 cases of conservative treatment. Conclusion Gastrointestinal bleeding and splenomegaly, hypersplenism is the main clinical manifestations. Misdiagnosis rate is high; Incomplete physical examination, insufficient understanding of the disease, disdainful of laboratory tests are the main reasons of misdiagnosis; Careful physical examination, improving the understanding of the disease, in a timely manner to do color doppler ultrasound examination can avoid misdiagnosis to a great extent.