背景:先天性脂肪瘤过度生长,血管畸形,表皮痣,脊柱/骨骼异常,和/或脊柱侧凸(CLOVES)综合征是最近描述的以脂肪组织先天性过度生长为特征的联合血管异常,血管畸形,表皮痣,和骨骼畸形。这种情况表现出临床表现的显着变异性和快速进展的趋势,并影响广泛的解剖区域。关于硬膜外脂肪瘤病与低流量静脉淋巴管畸形的相关性的信息很少见,文献中很少有报道。
方法:作者介绍了一个6岁女孩的案例,该女孩入院急诊科,抱怨她的下肢无力迅速发展,腹股沟区感觉部分丧失。放射学上,在T2-6水平确定了硬膜外肿块,导致急性脊髓压迫.对肿块进行紧急减压和部分切除。尽管术中止血和淋巴止血令人满意,术后淋巴漏/血清瘤漏作为一种延迟性并发症,并予以保守治疗.
结论:CLOVES综合征的特征是各种临床症状的组合,并非所有这些都包含在缩写中,以及逐渐恶化的过程,新症状的出现,以及患者一生中的并发症。这就需要持续监测这些患者。
BACKGROUND: Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies, and/or scoliosis (CLOVES) syndrome is the most recently described combined vascular anomaly characterized by congenital excessive growth of adipose tissue, vascular malformations, epidermal nevi, and skeletal deformities. This condition exhibits a significant variability in clinical manifestations and a tendency for rapid progression and affects extensive anatomical regions. Information regarding the association of epidural lipomatosis with low-flow venous lymphatic malformations is rare, with few
reports in the literature.
METHODS: The authors present a
case of a 6-year-old girl who was admitted to the emergency department complaining of rapidly progressing weakness in her lower extremities and partial loss of sensation in the inguinal area. Radiologically, an extradural mass was identified at the T2-6 level, causing acute spinal cord compression. Urgent decompression and partial resection of the mass were performed. Despite satisfactory intraoperative hemo- and lymphostasis, postoperative lymphorrhea/seroma leakage was encountered as a delayed complication and was managed conservatively.
CONCLUSIONS: CLOVES syndrome is characterized by the combination of various clinical symptoms, not all of which are included in the abbreviation, as well as a progressively deteriorating course, the emergence of new symptoms, and complications throughout the patient\'s life. This necessitates ongoing monitoring of such patients.