Objective To summarize and review the pathogenesis, clinical characteristics and iconography characteristics and management of congenital diaphragmatic hemangioma with pleural effusions. Methods The clinical data of an infant with congenital diaphragmatic hemangioma and massive pleural effusion diagnosed from Gynecology and Obstetrics Hospital, Fudan University in January 2012 were summarized. The clinical, radiographic features, treatment and prognosis of this case were discussed in the context of the literature review. Results The premature infant who presented with respiratory distress at birth was diagnosed as right pleural effusion and diaphragmatic hemangioma. A fetal right massive pleural effusion was presented in a short time in the third trimester of pregnancy and postnatal sonography showed a solid mass in the right thorax associated with a massive pleural effusion. Serial pleurocentesis installation of shunts was performed and re-accumulation of the pleural effusions was apparent after post-pleurocentesis. The aorta and portal vein enhanced CTA showed a right diaphragmatic hemangioma. After the DSA angiography and embolization closure no pleural effusion reappeared. Long-term follow up was continued. Conclusions Congenital diaphragmatic hemangioma is an extremely rare lesion. Being difficult to reach by prenatal diagnosis, the disease should be considered when the fetus was with prenatal pleural effusion. Interventional therapy or an operation of removing hemangioma is the key to solve the pleural effusion. Multidisciplinary treatment plays a key role in improving the prognosis.%目的 总结表现为胎儿胸腔积液的先天性膈肌血管瘤患儿的临床资料,提高对该病的认识.方法 报道1例表现为胎儿胸腔积液的先天性膈肌血管瘤患儿的临床资料,总结其产前及出生后的临床表现、影像学特征、治疗和预后等,并进行文献复习.结果 本例早产儿诊断为右侧胸腔积液、膈肌血管瘤,产前表现为孕后期短期内出现的胎儿右侧大量胸腔积液,生后呼吸困难.生后超声显示右侧胸腔内膈肌上方实质性占位,右侧胸腔积液.反复胸腔穿刺抽液减压及持续引流后,仍然出现反复的右侧大量胸腔积液,经大动脉门静脉CT血管造影显示为右侧膈肌血管瘤,经数字减影血管造影加栓塞术堵闭介入治疗后未再出现胸腔积液.结论 先天性膈肌血管瘤罕见.临床中遇到产前胸腔积液的胎儿应考虑本病的可能.介入或手术切除血管瘤是解决胸腔积液的主要手段,多学科诊治模式对改善其预后起关键作用.
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