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儿童巨大肝血管瘤的诊断与管理

Diagnosis and management of massive hepatic hemangiomas in childhood.

作者信息

Cohen R C, Myers N A

出版信息

J Pediatr Surg. 1986 Jan;21(1):6-9. doi: 10.1016/s0022-3468(86)80640-6.

DOI:10.1016/s0022-3468(86)80640-6
PMID:3511216
Abstract

Twelve patients diagnosed as having hepatic hemangiomas during 1960 to 1982 at the Royal Children's Hospital, Melbourne are reviewed. This rare benign vascular tumor can present considerable problems in both diagnosis and management and has a high reported mortality. Our experience over this 23-year period demonstrates some of the difficulties in diagnosis and suggests a plan of management. Eight patients were diagnosed when five weeks of age or less, the youngest was 24 hours old. One was diagnosed at four months of age. In the remaining three patients, the diagnosis was made at postmortem. Ten patients had prominent hepatomegaly, nine had congestive cardiac failure and in one of these the onset was delayed four weeks. Thrombocytopenia was present in five and jaundice in four patients. Four patients had associated cutaneous or visceral hemangiomas. Seven patients underwent selective hepatic arteriography, and two of these had prior ultrasound examinations of the liver. Management of congestive cardiac failure included steroids, radiotherapy, hepatic resection, and in one patient, hepatic artery ligation. One patient with diffuse hepatic hemangiomas did not require any specific therapy and resolved spontaneously over two years. The four patients in whom hepatic resection was performed survived.

摘要

回顾了1960年至1982年期间在墨尔本皇家儿童医院被诊断为患有肝血管瘤的12名患者。这种罕见的良性血管肿瘤在诊断和治疗方面都可能带来相当大的问题,并且据报道死亡率很高。我们在这23年期间的经验展示了诊断中的一些困难,并提出了一个治疗方案。8名患者在5周龄及以下时被诊断,最年幼的为24小时大。1名患者在4个月龄时被诊断。其余3名患者在尸检时被诊断。10名患者有明显的肝肿大,9名有充血性心力衰竭,其中1名的发病延迟了4周。5名患者有血小板减少症,4名有黄疸。4名患者伴有皮肤或内脏血管瘤。7名患者接受了选择性肝动脉造影,其中2名之前做过肝脏超声检查。充血性心力衰竭的治疗包括使用类固醇、放疗、肝切除,还有1名患者接受了肝动脉结扎。1名患有弥漫性肝血管瘤的患者不需要任何特殊治疗,在两年内自行痊愈。接受肝切除的4名患者存活了下来。

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