25 Years' experience with lymphangiomas in children

• Published in: Journal of pediatric surgery 1999-07, Vol.34 (7), p.1164-1168
• Main Authors: Alqahtani, A, Nguyen, L.T, Flageole, H, Shaw, K, Laberge, J.-M
• Format: Article
• Language: English
• Subjects: Abdominal Neoplasms - diagnosis; Abdominal Neoplasms - epidemiology; Abdominal Neoplasms - therapy; Adolescent; Age Distribution; Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Child; Child, Preschool; Diseases of the lymphatic vessels; Evaluation Studies as Topic; Female; Head and Neck Neoplasms - diagnosis; Head and Neck Neoplasms - epidemiology; Head and Neck Neoplasms - therapy; Humans; Incidence; Infant, Newborn; Lymphangioma - diagnosis; Lymphangioma - epidemiology; Lymphangioma - therapy; Male; Mediastinal Neoplasms - diagnosis; Mediastinal Neoplasms - epidemiology; Mediastinal Neoplasms - therapy; Medical sciences; Neoplasm Recurrence, Local - epidemiology; Neoplasm Recurrence, Local - etiology; Pregnancy; Prognosis; Quebec - epidemiology; Retrospective Studies; Risk Factors; Sex Distribution; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Vascular surgery: aorta, extremities, vena cava. Surgery of the lymphatic vessels
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/S0022-3468(99)90590-0

Background/Purpose: The management of lymphangioma in children is challenging because complete resection is difficult to achieve in some cases, and recurrences are common. The authors reviewed their experience to assess the risk factors for recurrence and the role of nonoperative treatment. Methods: A retrospective study over a period of 25 years was carried out. One hundred eighty-six patients with 191 lesions (five patients with de novo lesions in different sites) were treated. There were 98 boys and 88 girls. The average age at diagnosis was 3.3 years (range, fetal life to 17 years) and the average size 8 cm in diameter. Histocytological confirmation was obtained in all patients. The involved sites were head and neck, 89 patients (48%); trunk and extremities, 78 patients (42%); internal or visceral locations (eg, abdominal and thorax), 19 patients (10%). The treatment consisted of macroscopically complete excision in 145 patients (150 lesions, of which five were recurrences in different sites), partial excision in 10 patients, aspiration in five patients, laser excision in 10 patients, biopsy only in four patients, drainage and biopsy in two patients, and injection of sclerosing agents in 10 patients. Results: There were 54 recurrences; 44 underwent excision (five of them more than once), and five regressed spontaneously on follow-up. Five other recurrences were stable and not progressing. Recurrences, (defined as clinically obvious disease), were found to be 100% after aspiration, 100% after injection, 40% after incomplete excision, 40% after laser excision, and 17% after macroscopically complete excision. The recurrence rate in the last group was the highest in the head (33%), the least in the internal locations (0%), and intermediate for the cervical location (13%). There were no significant differences, in terms of outcome, between those who had their surgery immediately at the time of diagnosis (n = 101) and those who had delayed surgery (n = 85). Conclusions: There were fewer recurrences after macroscopically complete excision. Aspiration and injection had the highest recurrence rate. Risk factors for recurrence included location, size, and complexity of lesions. A period of observation may be useful for infants to facilitate complete excision. In the present series, spontaneous regression was infrequent and was seen more often with recurrent lesions.