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自发性闭孔内肌血肿:血友病性髂骨盆疼痛的一种新的未发表病因。

Spontaneous obturator internus muscle haematoma: a new unpublished cause of ilioipelvic pain in haemophilia.

机构信息

Center for Haemophilia Care and Department of Haematology, Hôpital Necker Enfants-Malades, Université Paris-Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.

出版信息

Haemophilia. 2013 Jan;19(1):157-60. doi: 10.1111/j.1365-2516.2012.02929.x. Epub 2012 Aug 14.

Abstract

Obturator muscles haematoma are rarely reported. The most often reported cases are primary pyomyositis or posttraumatic haematomas occurring during pelvic fractures. We firstly report herein two cases of spontaneous obturator internus haematoma (OIH) in two haemophiliacs with inhibitor. Clinical data and imaging of two patients treated in our clinic are reported here according to previously defined criteria of OIH in posttraumatic situation. Both patients were children suffering from severe and moderate haemophilia A, respectively, with an inhibitor at the time of the event. The clinical feature was marked by an iliopelvic pain letting discussing hip haemarthrosis, appendicitis or iliopsoas haematoma. For both patients ultrasonography (US) failed to provide the diagnosis. Careful and repeated clinical examinations eventually lead to suspect obturator haematoma which was confirmed by abdominopelvic computed tomography (CT) and magnetic resonance imaging (MRI). Respectively, high dose of FVIII or rFVIIa regimen allowed a rapid control of the muscular bleeding in the low and high responder inhibitor patients. Spontaneous OIH may be added to the differential diagnosis of iliopelvic pain in severe forms of haemophilia. US still often performed at first in such case remains unhelpful; abdominopelvic CT or MRI should be performed to discriminate among different diagnoses, including OIH which stays probably undiagnosed.

摘要

闭孔肌血肿很少见报道。最常报道的病例是原发性肌炎或骨盆骨折时发生的创伤后血肿。我们首次在此报告 2 例血友病伴抑制剂患者的自发性闭孔内肌血肿(OIH)。根据先前报道的创伤后 OIH 的标准,报道了 2 例在我们诊所治疗的患者的临床数据和影像学资料。这 2 名患者均为患有严重和中度血友病 A 的儿童,分别在发生时患有抑制剂。临床特征表现为严重的骨盆疼痛,易与髋关节积血、阑尾炎或髂腰肌血肿相混淆。对于这 2 名患者,超声(US)未能提供诊断。仔细且重复的临床检查最终怀疑是闭孔血肿,通过腹部骨盆计算机断层扫描(CT)和磁共振成像(MRI)得到证实。分别给予高剂量的 FVIII 或 rFVIIa 方案,允许低反应性和高反应性抑制剂患者的肌肉出血迅速得到控制。自发性 OIH 可能被添加到严重血友病患者骨盆疼痛的鉴别诊断中。在这种情况下,通常首先进行超声检查,但仍无帮助;应进行腹部骨盆 CT 或 MRI 以区分不同的诊断,包括可能未被诊断的 OIH。

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