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首页> 外文期刊>The Laryngoscope: A Medical Journal for Clinical and Research Contributions in Otolaryngology, Head and Neck Medicine and Surgery, Facial Plastic and Reconstructive Surgery .. >Pediatric hemophilic pseudotumor of the paranasal sinus.
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Pediatric hemophilic pseudotumor of the paranasal sinus.

机译：鼻旁窦的小儿血友病假瘤。

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OBJECTIVES/HYPOTHESIS: Hemophilic pseudotumors are rare clinical entities in otolaryngology. An unusual case of a pediatric hemophilic pseudotumor of the paranasal sinus in a previously undiagnosed hemophiliac is presented. STUDY DESIGN: Case report. METHODS: A 6-month-old, otherwise healthy boy was admitted for evaluation of a rapidly expanding left-sided cheek mass. The patient's initial presentation, imaging workup, and intraoperative and postoperative courses are discussed. RESULTS: Imaging workup revealed a large, heterogeneous, dense lesion in the patient's left-side maxillary sinus with distortion of the orbital floor and hard palate. Because of the concern for an aggressive malignancy, a biopsy was performed. After a Caldwell-Luc approach, a large amount of greenish fluid leaked from an otherwise empty maxillary sinus. The patient continued to bleed from his intraoral incision postoperatively and required two packed erythrocyte transfusions. Subsequent hematological testing led to a diagnosis of severe hemophilia type A with factor VIII levels less than 1% of normal. After treatment with recombinant coagulation factors hemostasis was achieved, and the patient clinically improved. CONCLUSION: The study presents the first report of a pediatric hemophilic pseudotumor in the paranasal sinuses leading to a diagnosis of hemophilia. It is a rare entity characterized by a cycle of repeated hemorrhage with subsequent encapsulation of the coagulum leading to pressure-induced distortion of adjacent bone. In the correct clinical setting, hemophilic pseudotumor should be considered in the differential diagnosis of a rapidly expanding mass in children.
机译：目的/假设：血友病假瘤是耳鼻喉科中罕见的临床实体。呈现了在先前未被诊断的血友病患者中鼻旁窦的小儿血友病假瘤的罕见病例。研究设计：病例报告。方法：一个6个月大的健康男孩入院，以评估其迅速扩大的左侧脸颊肿块。讨论了患者的最初表现，影像检查以及术中和术后过程。结果：影像学检查显示患者左侧上颌窦有大的，异质的，密集的病变，眼眶底和硬pa变形。由于担心侵袭性恶性肿瘤，因此进行了活检。经过Caldwell-Luc治疗后，大量绿色液体从原本为空的上颌窦漏出。病人术后继续从口腔内切开出血，需要输血两次。随后的血液学检查导致诊断出严重的A型血友病，其VIII因子水平低于正常水平的1％。用重组凝血因子治疗后，止血得以实现，患者的临床状况得到改善。结论：该研究首次报道了小儿鼻旁窦血友病假性肿瘤导致血友病的诊断。它是一种罕见的实体，其特征在于反复出血的周期以及随后的凝结物包封，导致压力引起的相邻骨变形。在正确的临床环境中，对儿童迅速扩大的肿块进行鉴别诊断时应考虑血友病假瘤。

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