Department of Surgery, Milton S. Hershey Medical Center, The Pennsylvania State University, 500 University Drive, P. O. Box 850, Hershey, PA, 17033-0850, USA.
Penn State College of Medicine, Hershey, PA, USA.
Pediatr Surg Int. 2021 Jun;37(6):783-790. doi: 10.1007/s00383-021-04859-5. Epub 2021 Feb 13.
Lymphatic malformations (LMs) are congenital abnormalities which result from disturbances in the embryologic development of the lymphatic system. We sought to determine the characteristics and treatment patterns for LMs in a rural setting, and the effect of a specialized vascular malformations clinic on triage and follow-up.
This is a retrospective cohort study at a single tertiary care institution. Sixty-two patients were identified; chart review was completed to obtain demographic, surgery/sclerotherapy session and follow-up information.
The head/neck region was the most predominant LM location (N = 26, 41.9%), followed by trunk (N = 16, 25.8%), extremity (N = 11, 17.7%), and intraabdominal/retroperitoneal (N = 7, 11.3%). Twenty-eight patients were managed non-surgically, while 21, 7 and 6 patients required surgery, sclerotherapy, or both. Head/neck LMs were the most likely to recur (73%, p = 0.028). Patients seen in specialty clinic had similar duration of follow-up and time to intervention, but were more often below 1 year of age (p = 0.030). Average LM volume among patients with available imaging was much larger in those referred to specialty clinic (73.2 cm versus 14.8 cm, p = 0.022).
Our experience reiterates not only the wide variety of clinical presentations of lymphatic malformations, but also demonstrates the necessity of multiple subspecialties and their collaboration to achieve prompt and efficacious treatment.
淋巴管畸形（LMs）是一种先天性异常，源于淋巴管系统胚胎发育过程中的紊乱。我们旨在确定农村地区 LMs 的特征和治疗模式，以及专门的血管畸形诊所对分诊和随访的影响。
这是一项在单一三级医疗机构进行的回顾性队列研究。共确定了 62 名患者；通过病历回顾获取了人口统计学、手术/硬化治疗疗程和随访信息。
头颈部是最常见的 LM 部位（N=26，41.9%），其次是躯干（N=16，25.8%）、四肢（N=11，17.7%）和腹腔/腹膜后（N=7，11.3%）。28 名患者接受非手术治疗，21 名、7 名和 6 名患者分别需要手术、硬化治疗或两者兼施。头颈部 LM 更易复发（73%，p=0.028）。在专科诊所就诊的患者随访时间和干预时间相似，但更年轻（p=0.030）。有影像学资料的患者中，专科诊所转诊患者的 LM 体积明显更大（73.2 cm 与 14.8 cm，p=0.022）。
我们的经验不仅重申了淋巴管畸形临床表现的多样性，还表明了多个亚专科及其协作的必要性，以实现快速有效的治疗。
