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[心脏正常的儿童心力衰竭伴下肢复杂动静脉畸形:病例研究及文献综述]

[Heart failure with normal heart revealing complex arteriovenous malformation of the lower limb in a child: case study and literature review].

作者信息

Sow Ndèye Fatou, Lèye Mohamed, Basse Idrissa, Dieng Yaay Joor, Seck Mame Aïta, Cissé Djénéba Fafa, Sow Amadou, Fattah Mohamed, Kane Awa, Houngbadji Morgiane, Faye Papa Moctar, Fall Amadou Lamine, Guèye Ndèye Ramatoulaye Diagne, Ndiaye Ousmane

机构信息

Centre Hospitalier National d'Enfants Albert Royer, Avenue Cheikh Anta Diop, Dakar, Sénégal.

Clinique de Cardiologie, CHU de Fann, Dakar, Sénégal.

出版信息

Pan Afr Med J. 2018 Oct 22;31:131. doi: 10.11604/pamj.2018.31.131.17078. eCollection 2018.

Abstract

Arteriovenous malformations (AVM) are congenital high-flow vascular defects. They are very rare in children. Diagnosis and treatment are often delayed due to their atypical place of occurrence and to their variable clinical manifestations. We report the case of a child treated at the National Centre Hospitalier Albert Royer Children, Dakar. A boy aged 9 years was referred from a health facility in rural areas for the treatment of heart failure. Admission clinical examination showed impaired general condition, global heart failure syndrome and voluminous right warm inguinocrural mass extended to the right abdominal wall (iliac fossa and right flank), with poorly defined limits. Auscultation of this mass showed a thrill and diffuse murmur. Cardiac ultrasound showed severe pulmonary arterial hypertension (PAH) with extensive impairement of the heart cavities, without cardiac structural involvement. The diagnosis of AVM was confirmed by doppler ultrasound of the mass complemented by angiography scan. They showed multiple arteriovenous fistulas within the mass. The diagnosis of complex stage IV AVM (according to Schöbinger classification) of the root of the right thigh was retained. Clinical treatment of heart failure was based on furosemide, spironolactone and captopril to obtain hemodynamic stabilization before possible surgical procedure. Arteriovenous malformations of the limbs, in particular of the proximal end of the lower limb in children are still largely unknown, hence frequent diagnostic errors and delays. Their evolution is unpredictable requiring early diagnosis and careful monitoring involving multidisciplinary interaction between pediatrics, surgeons and radiologists.

摘要

动静脉畸形(AVM)是先天性高流量血管缺陷。它们在儿童中非常罕见。由于其非典型的发生部位和多变的临床表现,诊断和治疗往往会延迟。我们报告了一例在达喀尔阿尔贝·罗耶国家儿童医院接受治疗的儿童病例。一名9岁男孩从农村地区的一家医疗机构转诊来治疗心力衰竭。入院时的临床检查显示全身状况不佳、全身性心力衰竭综合征以及右侧腹股沟股部有巨大肿块延伸至右腹壁(髂窝和右腰侧),边界不清。对该肿块听诊可闻及震颤和弥漫性杂音。心脏超声显示严重的肺动脉高压(PAH),心脏腔室广泛受损,但无心脏结构受累。通过对肿块进行多普勒超声检查并辅以血管造影扫描,确诊为AVM。检查显示肿块内有多个动静脉瘘。最终确诊为右大腿根部复杂IV期AVM(根据舍宾格分类法)。心力衰竭的临床治疗基于使用呋塞米、螺内酯和卡托普利,以便在可能进行手术之前实现血流动力学稳定。肢体动静脉畸形,尤其是儿童下肢近端的动静脉畸形,在很大程度上仍不为人所知,因此经常出现诊断错误和延误。其病情发展不可预测,需要早期诊断并进行仔细监测,这需要儿科、外科医生和放射科医生之间的多学科协作。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a71c/6462351/32c79c57b2de/PAMJ-31-131-g001.jpg

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