Nam So-Hyun, Kwon Kyoung-Ah
Division of Pediatric Surgery, Department of Surgery, Dong-A University College of Medicine, Dong-A Medical Center, 26 Daesingongwon-Ro, Seo-Gu, Busan, 49201, South Korea.
Department of Pediatrics, Dong-A University College of Medicine, Dong-A Medical Center, Busan, South Korea.
J Med Case Rep. 2018 Jun 15;12(1):167. doi: 10.1186/s13256-018-1705-0.
Lymphatic malformations are histologically benign vascular structures that vary in anatomic lesion and size. Extensive head and neck lymphatic malformations may be life-threatening. In the present study, we described three difficult-to-treat infants with giant cervico-mediastinal lymphatic malformations accompanied by severe respiratory distress.
Case 1. A Korean girl born at a gestational age of 37 weeks and weighing 2920 g had a large cervical mass compressing the trachea. Despite initial OK-432 sclerotherapy, the mass extended over the contralateral retropharyngeal space and mediastinum. Although the cervical mass was completely excised, our patient was not weaned off the ventilator. The mediastinal lymphatic malformation was excised, and our patient underwent continued intensive respiratory care with nasal continuous positive airway pressure for 6 months. She is now 5 years old and doing well without any sequelae. Case 2. A 5-month-old Korean boy showed respiratory difficulty with feeding intolerance after partial excision of a neck lymphatic malformation. We found that the remnant cervical mass had grown into the retropharyngeal space and mediastinum. After a second operation for the cervico-mediastinal mass, he experienced severe respiratory difficulty requiring ventilator care for 6 months. However, he died from central-line fungal sepsis. Case 3. A 30-day-old Korean girl was referred for remnant lymphatic malformation after partial excision. The cervical mass extended to the mediastinum and occupied half of the thoracic cavity, encasing all of the major vessels. After surgical excision, she underwent ventilator care and pleurodesis three times with doxycycline for recurrent pleural effusion. At the age of 26 months, she was weaned off supplementary oxygen and she showed normal development without any sequelae.
Despite difficulties in the treatment process, combinations of delicate surgical treatment, appropriate adjuvant sclerotherapy, and intensive respiratory supportive care could result in a good outcome. However, complications due to long-term intensive care could still be considered.
淋巴管畸形是组织学上良性的血管结构，其解剖病变和大小各不相同。广泛的头颈部淋巴管畸形可能危及生命。在本研究中，我们描述了三名患有巨大颈纵隔淋巴管畸形并伴有严重呼吸窘迫的难治性婴儿。
病例1。一名孕37周出生、体重2920克的韩国女孩，颈部有一个巨大肿块压迫气管。尽管最初进行了OK-432硬化治疗，但肿块扩展到对侧咽后间隙和纵隔。尽管颈部肿块被完全切除，但我们的患者仍未脱机。纵隔淋巴管畸形被切除，我们的患者接受了持续6个月的鼻持续气道正压通气强化呼吸护理。她现在5岁，情况良好，没有任何后遗症。病例2。一名5个月大的韩国男孩在颈部淋巴管畸形部分切除后出现呼吸困难和喂养不耐受。我们发现残留的颈部肿块已长入咽后间隙和纵隔。在对颈纵隔肿块进行第二次手术后，他出现了严重的呼吸困难，需要呼吸机护理6个月。然而，他死于中心静脉导管相关真菌败血症。病例3。一名30天大的韩国女孩因部分切除后残留淋巴管畸形前来就诊。颈部肿块延伸至纵隔，占据胸腔的一半，包绕所有主要血管。手术切除后，她因复发性胸腔积液接受了三次呼吸机护理和强力霉素胸膜固定术。在26个月大时，她停用了补充氧气，发育正常，没有任何后遗症。
尽管治疗过程存在困难，但精细的手术治疗、适当的辅助硬化治疗和强化的呼吸支持护理相结合可能会取得良好的效果。然而，仍需考虑长期强化护理引起的并发症。
