Palo Nishit, Chauhan Virendra S, Lakhanpal Mahima, Dey Paresh Chandra, Malik Chhavi
Associate Professor, Department of Orthopedics, Santosh Medical College and Hospital, Ghaziabad, Uttar Pradesh, India.
Consultant, Department of Radiodiagnosis, Saral Diagnostics, Noida, Uttar Pradesh, India.
J Orthop Case Rep. 2023 Jul;13(7):110-115. doi: 10.13107/jocr.2023.v13.i07.3774.
Venous malformations are rare lesions of unknown etiology, with a reported incidence of 0.8-1%. Patients with inexorable growth and expansion of vascular malformations, or" have an unpredictable clinical course and a wide range of presenting symptoms. Often, they are erroneously diagnosed and inadequately treated due to their rarity and lack of expertise among clinicians. To author's information this is the first report of diffuse venous malformations with multiple phleboliths involving various compartments of the upper extremity in children.
The uthors discuss the clinical presentation, evaluation, and treatment over 8 months of slow-flow venous malformations with phleboliths in an11-year-old girl presenting with multiple painful swellings throughout her right upper extremity. The right upper extremity had multiple swellings over the right hand, forearm, arm, and shoulder region involving multiple compartments. The digital swellings had bluish discoloration, indicating a vascular nature. Blood tests revealed a raised D-dimer level (2.42 mg/L). Radiographs, Ultrasound, Magnetic resonance imaging, and CT angiography suggested a slow-flow venous malformation. The excisional biopsy confirmed the diagnosis. Ultrasound-guided Sclerotherapy with the Sclerotherapy with Adjunctive Stasis of Efflux Technique was performed for other lesions. Sodium Tetradecyl Sulfate (60 mg/2 mL; 0.5mL) was used in each lesion. Post-intervention, at 6 months follow-up, cosmetic appearance improved drastically, with the hands benefitted most. Parents were satisfied with overall outcome. Sclerotherapy was stopped after 4 cycles.
Ultrasound-guided sclerotherapy is effective in treating venous malformations. The ideal result is seen after 4-5 sittings. Sclerotherapy must be performed in the operating theatre under sedation or appropriate anesthesia with resuscitation equipment at the ready disposal. Excision is reserved for bigger superficial lesions.
静脉畸形是病因不明的罕见病变,报告发病率为0.8 - 1%。血管畸形持续生长和扩张的患者,其临床病程不可预测,症状表现多样。由于其罕见性以及临床医生缺乏相关专业知识,它们常常被误诊且治疗不充分。据作者所知,这是首例关于儿童上肢多个腔室出现伴有多个静脉石的弥漫性静脉畸形的报告。
作者讨论了一名11岁女孩的临床表现、评估及治疗过程,该女孩右上肢多处疼痛性肿胀8个月,经诊断为伴有静脉石的缓慢流动型静脉畸形。右上肢的右手、前臂、上臂和肩部区域有多处肿胀,累及多个腔室。手指肿胀呈蓝色,表明具有血管性质。血液检查显示D - 二聚体水平升高(2.42mg/L)。X线片、超声、磁共振成像和CT血管造影提示为缓慢流动型静脉畸形。切除活检确诊了该诊断。对其他病变采用超声引导下硬化治疗并结合流出道辅助淤滞技术。每个病变使用十四烷基硫酸钠(60mg/2mL;0.5mL)。干预后,在6个月的随访中,外观有显著改善,手部受益最大。家长对总体结果满意。4个周期后停止硬化治疗。
超声引导下硬化治疗对静脉畸形有效。4 - 5次治疗后可看到理想效果。硬化治疗必须在手术室进行,在镇静或适当麻醉下进行,并随时准备好复苏设备。较大的浅表病变则采用手术切除。