Department of Radiology, Perelman School of Medicine at the University of Pennsylvania, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
J Pediatr Surg. 2011 Nov;46(11):2083-95. doi: 10.1016/j.jpedsurg.2011.07.004.
To evaluate the clinical outcomes of percutaneous sclerotherapy for congenital head and neck lymphatic malformations in our institution.
Over a 7-year period, 17 children (10 M, 7 F) mean age 5.8 months (5 days to 13 months) underwent 49 sclerotherapy procedures for congenital head and neck malformations. The imaging and clinical records were reviewed for each patient. Ten of 17 had macrocystic disease; 7 of 17 had microcystic disease. Imaging response was categorized by volume reductions of 0% to 25%, 25% to 50%, 50% to 75%, or 75% to 100%. A concentration of 10 mg/mL doxycycline was used routinely via catheter in 3 instillations with a dose range of 50 to 500 mg per session as per our standard protocol in 17 of 17 patients. In more recent patients, systemic doxycycline levels were obtained after instillations. Additional treatments included direct injection doxycycline (10/17), instillation of absolute ethanol (7/17) or sodium tetradecyl sulfate (4/17), or a combination of these methods.
Imaging improvement of ≥ 76% was noted in 11 of 17. Of these, 8 of 11 had macrocystic disease. Four of 17 had 51% to 75% resolution, of which 3/4 were mixed. Two of 17 children had 25% to 50% resolution with a mixed lesion. Seven of 49 peri-procedural complications: hemolytic anemia in 2 infants, hypoglycemic and metabolic acidosis in 3 neonates aged 7 to 10 days, transient hypotension during absolute alcohol instillation in 1 neonate, and self-limiting skin excoriation secondary to peri-catheter leakage of doxycycline in one neonate. Neonates prone to these systemic complications had doxycycline doses of greater than 250 mg and resulted in serum levels of >5 μg/mL but as high as 21 μg/mL. Delayed neural complications occurred in 7 of 49 procedures, Horner's syndromes in 4 of 49 procedures, transient left lip weakness in 1 of 49 procedures, right facial nerve palsy in 1 of 49 procedures, and transient left hemidiaphragm paralysis in 1/49 procedures.
Our experience with catheter directed doxycycline sclerotherapy provides excellent results for large macrocystic head and neck lymphatic malformations. Microcystic and mixed lesions continue to provide a therapeutic challenge.
评估我院经皮硬化治疗先天性头颈部淋巴管畸形的临床疗效。
在 7 年期间，17 名儿童（男 10 例，女 7 例），平均年龄 5.8 个月（5 天至 13 个月），因先天性头颈部畸形接受了 49 次硬化治疗。对每位患者的影像学和临床记录进行了回顾。17 例中 10 例为巨囊型病变，7 例为微囊型病变。根据体积减少 0%至 25%、25%至 50%、50%至 75%或 75%至 100%，对影像学反应进行分类。17 例患者中有 17 例常规使用浓度为 10mg/ml 的强力霉素经导管分 3 次注入，每次剂量为 50 至 500mg，这是我们根据标准方案进行的。在最近的患者中，在注射后获得了系统强力霉素水平。其他治疗方法包括直接注射强力霉素（17/17）、注入无水乙醇（17/17）或十四烷基硫酸钠（17/17），或联合使用这些方法。
17 例中，11 例影像学改善≥76%。其中 8 例为巨囊型病变。17 例中有 4 例为 51%至 75%缓解，其中 3/4 为混合性病变。2 例儿童有 25%至 50%的缓解，为混合性病变。49 次围手术期并发症中，7 例：2 例婴儿出现溶血性贫血，3 例 7 至 10 天大的新生儿出现低血糖和代谢性酸中毒，1 例新生儿在注入无水乙醇时出现短暂低血压，1 例新生儿因强力霉素经导管周围泄漏导致皮肤擦伤，1 例新生儿出现短暂性左膈肌麻痹。易发生这些全身并发症的新生儿强力霉素剂量大于 250mg，导致血清水平>5μg/ml，但高达 21μg/ml。49 次手术中有 7 次出现迟发性神经并发症，49 次手术中有 4 次出现霍纳综合征，49 次手术中有 1 次出现左侧唇肌无力，49 次手术中有 1 次出现右侧面神经瘫痪，49 次手术中有 1 次出现左侧膈肌短暂瘫痪。
我们经皮强力霉素硬化治疗的经验为大的巨囊型头颈部淋巴管畸形提供了良好的效果。微囊型和混合性病变仍具有治疗挑战性。
