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e-Article

A Case of Generalized Lymphatic Anomaly After 17 Years of Sclerotherapy for Lymphatic Malformation / 頸部囊胞性リンパ管奇形に対する硬化療法17年後に全身の骨病変を認めリンパ管腫症と診断した1例
Document Type
Journal Article
Source
日本小児外科学会雑誌 / Journal of the Japanese Society of Pediatric Surgeons. 2019, 55(5):988
Subject
cystic lymphatic malformation
generalized lymphatic anomaly
リンパ管腫症
囊胞状リンパ管奇形
Language
Japanese
ISSN
0288-609X
2187-4247
Abstract
The patient was a 17-year-old male. A congenital giant cystic lesion on the neck was detected prenatally, and he was diagnosed as having cervical cystic lymphatic malformation postnatally. Two rounds of sclerotherapy were performed during infancy. The cyst size was markedly reduced. At 4 years of age, he underwent surgical removal of the excess skin at the Department of Plastic Surgery, and the swelling in the neck improved. Thereafter, he discontinued outpatient visits on his own and no follow-up was conducted. When he had a fracture of the cervical spine during baseball practice at 17 years of age, multiple cystic lesions in the bone were detected. MRI indicated no recurrence of the cervical cystic lesion but multiple cystic lesions in the cervical spine, thorax, ribs, iliac bone, and spleen were detected; thus, he was diagnosed as having generalized lymphatic anomaly (GLA). Bone union occurred later at the fracture site of the cervical spine. No pleural effusion, cardiac effusion, or ascites were noted, and blood analysis showed no abnormality. Therefore, he is currently under follow-up with no treatment. Cystic lymphangioma is currently defined as lymphatic malformation due to developmental disorders of lymphatic vessels. The present case was classified as a macrocystic-type lymphatic malformation and considered to belong to a disease group different from GLA, which is a systemic disease. However, GLA may develop during the course, as in this case, and long-term follow-up in addition to diagnosis and treatment may be warranted.