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一名镰状细胞病患儿以双侧眶周蜂窝织炎为表现的颌面骨外造血。

Maxillofacial extramedullary hematopoiesis in a child with sickle cell presenting as bilateral periorbital cellulitis.

作者信息

Reiersen David A, Mandava Mamatha, Jeroudi Majed, Gungor Anil

机构信息

LSU-Shreveport HSC Department of Otolaryngology/Head & Neck Surgery, United States.

LSU-Shreveport HSC Department of Pediatrics, United States.

出版信息

Int J Pediatr Otorhinolaryngol. 2014 Jul;78(7):1173-5. doi: 10.1016/j.ijporl.2014.03.028. Epub 2014 Apr 6.

Abstract

Review of a case of paraosseous extramedullary hematopoiesis (EMH) affecting the maxillary sinuses and retro-orbital spaces imitating bilateral orbital cellulitis. Maxillofacial EMH causes diagnostic/therapeutic challenges. This case report describes a 4-year-old African American male with sickle cell disease (HbSS) who presented with bilateral orbitofacial swelling. Diagnosis was made with imaging and confirmed with tissue sampling. Partial exchange transfusion was utilized to stop the progression of maxillofacial EMH and to treat the patient's chronic anemia. Follow-up MRI showed regression of orbital and retro-orbital involvement. Early treatment with conservative modalities and close observation may prevent need for more invasive treatments.

摘要

回顾一例影响上颌窦和眶后间隙、酷似双侧眼眶蜂窝织炎的骨旁髓外造血(EMH)病例。颌面EMH引发诊断/治疗难题。本病例报告描述了一名患有镰状细胞病(HbSS)的4岁非裔美国男性,其表现为双侧眶面部肿胀。通过影像学检查做出诊断,并经组织取样得以证实。采用部分换血疗法来阻止颌面EMH的进展并治疗患者的慢性贫血。随访磁共振成像显示眼眶和眶后受累情况消退。采用保守方式早期治疗并密切观察可避免更具侵入性治疗的需求。

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