Oliveira C, Sacher P, Meuli M
The Hospital for Sick Children, Department of General Surgery, Toronto, Canada.
Eur J Pediatr Surg. 2010 Sep;20(5):302-6. doi: 10.1055/s-0030-1254149. Epub 2010 Jun 24.
Abdominal lymphatic malformations (ALM) are rare congenital malformations that can regress spontaneously or lead to serious complications. Thus, the appropriate management may be challenging, particularly since pertinent literature is missing. We present our experience in the management of 5 patients with prenatally diagnosed ALM and their outcome and propose a decision-making algorithm.
We retrospectively reviewed the history, diagnostics, therapy, complications, and outcome of 5 patients with a prenatal diagnosis of ALM, referred to our department between January 2006 and February 2008.
ALM was prenatally diagnosed by ultrasound in all patients (gestational age 21, 23, 23, 32, and 34 weeks). MRI was performed pre- and postnatally in one patient and postnatally in another. Clinical symptoms ranged from none to respiratory distress and abdominal compartment syndrome. One ALM involuted. 2 patients underwent primary OK-432 treatment. This led to a 70% size reduction in one patient. The other developed massive intracystic bleeding and required emergency surgery. 2/3 patients with surgery needed segmental bowel resection and 3/3 stayed recurrence-free. Complications included one partial inferior vena cava thrombosis after surgery, one subileus, and one hemorrhage after OK-432 application.
Asymptomatic and regressing ALM are best managed conservatively ("watchful waiting") while symptomatic ALMs require surgery. Further studies are necessary to determine the ideal timepoint for intervention for non-regressing ALM.
腹部淋巴管畸形（ALM）是罕见的先天性畸形，可自发消退或导致严重并发症。因此，恰当的治疗可能具有挑战性，尤其是因为缺乏相关文献。我们介绍了我们对5例产前诊断为ALM的患者的治疗经验及其结果，并提出了一种决策算法。
我们回顾性分析了2006年1月至2008年2月间转诊至我科的5例产前诊断为ALM患者的病史、诊断、治疗、并发症及结果。
所有患者均通过超声在产前诊断出ALM（孕周分别为21、23、23、32和34周）。1例患者在产前和产后均进行了MRI检查，另1例仅在产后进行了检查。临床症状从无到呼吸窘迫和腹腔间隔室综合征不等。1例ALM自行消退。2例患者接受了OK-432的初次治疗。其中1例患者的病变大小缩小了70%。另1例发生了大量囊内出血，需要进行急诊手术。2/3接受手术的患者需要进行节段性肠切除，且3例均无复发。并发症包括1例术后部分下腔静脉血栓形成、1例肠梗阻和1例应用OK-432后的出血。
无症状且正在消退的ALM最好采用保守治疗（“密切观察”），而有症状的ALM则需要手术治疗。需要进一步研究以确定对无消退趋势的ALM进行干预的理想时间点。
