Yang Kaiying, Peng Suhua, Chen Linwen, Chen Siyuan, Ji Yi
Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China.
College of Clinical Medicine, North Sichuan Medical College, Nanchong, China.
J Paediatr Child Health. 2019 Oct;55(10):1194-1200. doi: 10.1111/jpc.14375. Epub 2019 Jan 9.
The aim of this study was to assess the efficacy of propranolol treatment in multifocal and diffuse infantile hepatic haemangioma (IHH).
A retrospective study of symptomatic or potentially symptomatic IHH was performed in our hospital between 2011 and 2016.
Thirteen patients were identified: 2 patients had diffuse lesions, and 11 patients had multifocal lesions, including 2 patients who had combined lesions that shared features of both multifocal and diffuse lesion patterns. Eleven (84.6%) patients had cutaneous infantile haemangioma. Hepatomegaly was the predominant clinical presentation. Hypothyroidism was identified in three patients, including one patient who had documented congestive heart failure (CHF). The median age at diagnosis and the median duration of treatment were 2.0 months (range 1.2-26.0) and 24.0 months (range 4.0-30.0). The median duration of follow-up was 30.0 months (range 3.0-48.0). For patients with hypothyroidism, the thyroid hormone level was normal after 4 weeks of propranolol and levothyroxine treatment. All but one patient responded well to propranolol treatment. The patient who failed to respond to treatment died of CHF and abdominal compartment syndrome induced by hepatomegaly. No significant side effects of propranolol were observed during follow-up.
Most multifocal and diffuse IHH respond well to propranolol. However, progressive cases may be fatal despite aggressive treatments. Our data suggest that propranolol may be considered the first-line treatment for multifocal and diffuse IHH due to its efficacy.
本研究旨在评估普萘洛尔治疗多灶性和弥漫性婴儿肝血管瘤(IHH)的疗效。
对2011年至2016年期间在我院就诊的有症状或潜在症状的IHH患者进行回顾性研究。
共纳入13例患者:2例为弥漫性病变,11例为多灶性病变,其中2例为合并病变,兼具多灶性和弥漫性病变特征。11例(84.6%)患者有皮肤婴儿血管瘤。肝肿大是主要临床表现。3例患者出现甲状腺功能减退,其中1例有充血性心力衰竭(CHF)记录。诊断时的中位年龄和治疗的中位持续时间分别为2.0个月(范围1.2 - 26.0个月)和24.0个月(范围4.0 - 30.0个月)。中位随访时间为30.0个月(范围3.0 - 48.0个月)。对于甲状腺功能减退的患者,普萘洛尔和左甲状腺素治疗4周后甲状腺激素水平恢复正常。除1例患者外,所有患者对普萘洛尔治疗反应良好。治疗无效的患者死于CHF和肝肿大引起的腹腔间隔室综合征。随访期间未观察到普萘洛尔有明显副作用。
大多数多灶性和弥漫性IHH对普萘洛尔反应良好。然而,尽管积极治疗,进展性病例可能致命。我们的数据表明,由于普萘洛尔的疗效,可将其视为多灶性和弥漫性IHH的一线治疗药物。