急性软头综合征（帽状腱膜下血肿）伴眶周水肿作为镰状细胞病的罕见表现
Acute Soft Head Syndrome (Subgaleal Haematoma) with Periorbital Oedema as a Rare Presentation in Sickle Cell Disease.
作者信息
Al-Ansari Rehab Y, Al Harbi Maan, Al-Jubair Nawaf, Abdalla Leena
机构信息
Adult Hematology Unit, Internal Medicine Department, KFMMC, Dhahran, Kingdom of Saudi Arabia.
Internal Medicine Department, KFMMC, Dhahran, Kingdom of Saudi Arabia.
出版信息
Eur J Case Rep Intern Med. 2020 Jul 30;7(10):001766. doi: 10.12890/2020_001766. eCollection 2020.
BACKGROUND
Sickle cell disease is a genetic condition frequently found in Africa and the Arabian Peninsula. Uncommon complications include subgaleal haematoma (soft head syndrome) and periorbital oedema.
CASE PRESENTATION
A 17-year-old male patient presented with body aches and progressive right parieto-temporal and frontal head swelling. Physical examination revealed puffiness of the right eye that progressed rapidly to reddish periorbital oedema sparing the extraocular muscle and pupil response to light. CT and MRI of the brain suggested multiple subgaleal haematomas (soft head syndrome) and right periorbital oedema.
CONCLUSION
Subgaleal haematoma (soft head syndrome) and periorbital oedema are uncommon complications of sickle cell disease. Management is conservative rather than surgical.
LEARNING POINTS
Subgaleal haematoma concurrently with periorbital oedema is a rare presentation of sickle cell disease.There are no guidelines on treatment, but the conditions in our patient resolved with conservative management.
背景
镰状细胞病是一种在非洲和阿拉伯半岛常见的遗传性疾病。罕见的并发症包括帽状腱膜下血肿（软头综合征）和眶周水肿。
病例报告
一名17岁男性患者出现全身疼痛，右侧顶颞部和额部头部肿胀进行性加重。体格检查发现右眼肿胀，迅速发展为眼眶周围红肿，不累及眼外肌和瞳孔对光反应。脑部CT和MRI提示多发帽状腱膜下血肿（软头综合征）和右侧眶周水肿。
结论
帽状腱膜下血肿（软头综合征）和眶周水肿是镰状细胞病的罕见并发症。治疗以保守治疗为主，而非手术治疗。
经验教训
帽状腱膜下血肿合并眶周水肿是镰状细胞病的罕见表现。目前尚无治疗指南，但我们患者的病情通过保守治疗得以缓解。
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