Khoshkhesal Mona, Pham Alan C G, Deshpande Aniruddh V
Department of Medicine, School of Medicine and Public Health, University of Newcastle, Newcastle, Australia.
Department of Paediatric Surgery, John Hunter Children's Hospital, Newcastle, Australia.
Urol Ann. 2022 Apr-Jun;14(2):183-185. doi: 10.4103/ua.ua_19_21. Epub 2022 Apr 18.
Hemophilia is an inherited clotting disorder that typically presents as spontaneous hemorrhage or prolonged, excessive bleeding following minor trauma. Abnormalities of the genitourinary tract are a rare manifestation of the disease. Here, we report a case of undiagnosed hemophilia B in a teenage boy presenting with worsening testicular pain and a dumbbell-shaped testis. Scrotal ultrasound identified the presence of a hypoechogenic band constricting the left testicle. Surgical exploration of the left testis was performed with release and sampling of the fibrous tunica vaginalis tissue responsible for the testicular deformity. Histopathological analysis revealed evidence of chronic interstitial inflammation with hypocellular keloid-like fiber formation suggestive of old hemorrhage. The procedure was complicated by a delayed scrotal hematoma. Coagulation studies confirmed a prolonged activated partial thromboplastin time and factor IX assay of 5%. Scrotal evacuation with the administration of intraoperative tranexamic acid achieved effective hemostasis. The patient is doing well with ongoing hematology review and prophylactic recombinant factor IX administration.
血友病是一种遗传性凝血障碍疾病，通常表现为自发性出血或轻微创伤后出血时间延长、出血过多。泌尿生殖道异常是该疾病的罕见表现。在此，我们报告一例青少年男性未确诊的乙型血友病病例，该患者表现为睾丸疼痛加重及哑铃状睾丸。阴囊超声检查发现有一条低回声带压迫左侧睾丸。对左侧睾丸进行了手术探查，松解并取了导致睾丸畸形的纤维鞘膜组织样本。组织病理学分析显示有慢性间质性炎症证据，伴有细胞减少的瘢痕疙瘩样纤维形成，提示陈旧性出血。该手术因阴囊血肿延迟而复杂化。凝血研究证实活化部分凝血活酶时间延长，因子IX测定为5%。术中给予氨甲环酸并行阴囊引流实现了有效止血。患者在持续的血液学检查及预防性重组因子IX给药情况下情况良好。
