Bhatnagar Ankur, Upadhyaya Vijai Datta, Yadav Rajnikant, Kumar Basant
Department of Plastic Surgery, SGPGIMS, Lucknow, Uttar Pradesh, India.
Department of Pediatric Surgery, SGPGIMS, Lucknow, Uttar Pradesh, India.
Natl J Maxillofac Surg. 2020 Jul-Dec;11(2):193-198. doi: 10.4103/njms.NJMS_48_20. Epub 2020 Dec 16.
Lymphangioma are rare vascular malformation that results from maldevelopment of primitive lymphatic sacs. They are most frequently found in the neck and axilla, while intra-abdominal and mediastinal lymphangiomas are uncommon. Atypical site of cystic hygroma in pediatric age group are usually difficult to diagnose clinically but can be diagnosed easily by ultrasound. The aim of the study was to evaluate the result of the intralesional bleomycin for macrocystic lymphatic malformation (LM) presenting at atypical site.
All patients of LM of other than head& neck, axilla and abdomen presenting in pediatric age group were included in the study. Mainstay of diagnosis was ultrasound and was supplemented by CT scan wherever required. All patients were managed with intralesional bleomycin (ILB) and surgical excision was done only if primary therapy failed.
Total 15 cases of LM presenting at atypical sites were included in the study. Series include two case of cystic hygroma of breast, 4 cases of cystic hygroma of anterior chest wall, two case of substernal LM, three cases of LM of parotid gland, one case of inguinal region cystic hygroma and 4 cases involving submandicular area. Complete resolution was observed in 13 out of 15 cases, and two cases had less than 50% reduction in size and were managed with surgical excision after second session of ILB.
Aqueous Intralesional bleomycin is a cost effective alternative to surgery even at rare sites of LM which provide better aesthetic outcome, and avoids complication associated with surgery.
淋巴管瘤是一种罕见的血管畸形，由原始淋巴囊发育异常引起。它们最常出现在颈部和腋窝，而腹腔内和纵隔淋巴管瘤并不常见。儿童年龄组中囊状水瘤的非典型部位在临床上通常难以诊断，但通过超声检查很容易诊断。本研究的目的是评估病灶内注射博来霉素治疗非典型部位的大囊型淋巴管畸形（LM）的效果。
本研究纳入了所有儿童年龄组中出现在头颈部、腋窝和腹部以外部位的淋巴管瘤患者。诊断的主要方法是超声检查，必要时辅以CT扫描。所有患者均采用病灶内注射博来霉素（ILB）治疗，只有在初始治疗失败时才进行手术切除。
本研究共纳入15例非典型部位的淋巴管瘤病例。病例系列包括2例乳房囊状水瘤、4例前胸壁囊状水瘤、2例胸骨后淋巴管瘤、3例腮腺淋巴管瘤、1例腹股沟区囊状水瘤和4例累及颌下区的病例。15例中有13例观察到完全消退，2例体积缩小不到50%，在第二次ILB治疗后接受了手术切除。
病灶内注射博来霉素水剂即使在淋巴管瘤的罕见部位也是一种经济有效的替代手术的方法，能提供更好的美学效果，并避免与手术相关的并发症。
