Purpose
Report of a nationwide survey on lymphangioma conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2019.

Methods
The authors reviewed and analyzed the clinical data of pediatric patients who started treatment for lymphangioma in hospitals of KAPS members from 2011 to 2013. Their follow-up data is also included in the study.

Results
A total of 532 patients with lymphangioma from 18 institutes were registered for the study. The results were discussed at the 35th annual meeting of KAPS, which was held in Gyeongju on June 13–14, 2019.

Conclusion
This study provides general information on lymphangioma and comprehensive treatment outcomes for this disease. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of lymphangioma.ope

안수민

오정탁

한석주

Advances in Pediatric Surgery

Yonsei University Medical Library Open Access Repository

1https://aps-journal.orgABSTRACTPurpose: Report of a nationwide survey on lymphangioma conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2019.Methods: The authors reviewed and analyzed the clinical data of pediatric patients who started treatment for lymphangioma in hospitals of KAPS members from 2011 to 2013. Their follow-up data is also included in the study.Results: A total of 532 patients with lymphangioma from 18 institutes were registered for the study. The results were discussed at the 35th annual meeting of KAPS, which was held in Gyeongju on June 13–14, 2019.Conclusion: This study provides general information on lymphangioma and comprehensive treatment outcomes for this disease. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of lymphangioma.Keywords: Lymphangioma; Pediatrics; Surveys and questionnairesINTRODUCTIONLymphangioma or lymphatic malformation includes a wide spectrum of abnormalities because it can be found in any region of the body and has a variety of appearances [1-4]. Although pediatric surgeons occasionally encounter this disease, the incidence of lymphangioma is relatively low. This means that it takes a long time for surgeons to accumulate experience in the treatment of lymphangioma. Therefore, a nationwide study is helpful for surgeons to understand this disease and choose optimal treatment methods. The 2019 annual nationwide survey of the Korean Association of Pediatric Surgeons (KAPS) was on the subject of lymphangioma, and the results were first discussed at the annual meeting of KAPS in Gyeongju on June 13–14, 2019.Received: Dec 7, 2021Accepted: Dec 8, 2021Published online: Jun 9, 2022Correspondence toJung-Tak OhDepartment of Pediatric Surgery, Severance Children’s Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Korea.Email: jtoh@yuhs.acCopyright © 2022 Korean Association of Pediatric SurgeonsThis is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.ORCID iDsJung-Tak Oh https://orcid.org/0000-0003-4860-7410Conflict of InterestNo potential conflicts of interest relevant to this article are reported.Original ArticleJung-Tak Oh , Soo Min Ahn, Yoon-Jung Boo, Eun Young Chang, Hye Kyung Chang,  Soo Jin Na Choi, Yoon Mi Choi, Jae Hee Chung, Sang Young Chung, Jeong Hong, Seok Joo Han, Yeon Jun Jeong, Eunyoung Jung, Sung Eun Jung, Dae Youn Kim, Hyun-Young Kim, In-Koo Kim, Seong Chul Kim, Ho-Kyun Lee, Ju Yeon Lee,  Nam-Hyuk Lee, Seong-Cheol Lee, Suk-Koo Lee, Jung-Man Namgoong, Chaeyoun Oh,  Jin Young Park, Kwi-Won Park, Jeong-Meen Seo, Jae Ho Shin, Jiyoung Sul The Korean Association of Pediatric Surgeons, Seoul, KoreaLymphangioma: A 2019 Survey Conducted by the Korean Association of Pediatric SurgeonsAdv Pediatr Surg. 2022 Jun;28(1):1-8https://doi.org/10.13029/aps.2022.28.1.1pISSN 2635-8778·eISSN 2635-8786METHODSThe authors reviewed and analyzed the clinical data of patients who started treatment for lymphangioma in KAPS members’ hospitals from 2011 to 2013. Their follow-up data were also included in the study. We used Microsoft Access 2016® (Microsoft, Redmond, WA, USA) for patient registry and data collection. All the data were analyzed using the IBM SPSS version 23 statistical software package (IBM Co., Armonk, NY, USA).RESULTS1. DemographicsA total of 532 patients with lymphangioma from 18 institutes were registered for the study (Fig. 1). Although 550 patients were reported, 18 patients were reported by 2 institutes. Therefore, we unified the data of these patients. The patients’ demographics and clinical characteristics are summarized in Tables 1 and 2. Age at diagnosis varied; 39.3% of patients (209/532) were diagnosed in the infant period, and 25% (133/532) were diagnosed after the age of 5. Only 12.8% of patients (68/532) were diagnosed postnatally. The majority of patients (81.0%) were treated by pediatric surgeons. Most patients (94.9%) had a lesion at a single site, but 27 patients had multiple lesions at more than one site. The most common symptom was mass, followed by pain. The most common imaging study was ultrasound, followed by magnetic resonance imaging. We classified lymphangioma with a diameter of more than 1 cm as macrocystic. Macrocystic lymphangioma was the most common type of lymphangioma, and the median diameter size was 4.8 cm.2. TreatmentWe grouped treatment methods into 5 categories: observation only; operation; sclerotherapy; operation and sclerotherapy; and other treatments (Fig. 2). Operation with observation and sclerotherapy with observation were classified as operation and sclerotherapy, respectively.2https://aps-journal.org https://doi.org/10.13029/aps.2022.28.1.1Lymphangioma334477771314182324466975112114A B C D E F G H I J K L M N O P Q RNo. of patientsHospitals020406080100120Fig. 1. Hospital distribution of patients with lymphangioma.Observation onlyPatients who underwent observation only are summarized in Table 3. A total of 114 patients (21.4%) did not undergo any specific treatment, and the median observation period was 316 days. Among the patients in this group, 67.9% (88/114) showed grossly normal or partially improved appearance.3Lymphangiomahttps://doi.org/10.13029/aps.2022.28.1.1https://aps-journal.orgTable 1. Patient demographicsCharacteristics Value (n=532)Sex M:F=1.18:1 (288:244)Mode of deliveryNormal spontaneous vaginal delivery 203C-section 128Unknown 201Age at diagnosis≤28 days 10628 days<Age≤6 months 536 months<Age≤1 year 501 year<Age≤3 years 1153 year<Age≤5 years 74>5 year 133Unknown 1Prenatal diagnosis 68 (12.8)Department of treatmentPediatric surgery 431Other department 101Pediatrics 13Plastic surgery 17Radiology 5Otolaryngology 30Dermatology 14Cardiothoracic surgery 5Orthopedic surgery 9Ophthalmology 2General surgery 2Neurosurgery 2Oral and maxillofacial surgery 2Values are presented as number (%).114163 166391139No. of patientsObservationonlyOperation Sclerotherapy Operation+SclerotherapyOthertreatmentLoss tofollow-up020406080100140120160180Fig. 2. Treatment methods.OperationA total of 163 patients (30.6%) underwent an operation. The predominant operation site was the head and neck, followed by the abdominal wall and flank. Characteristically, lesions in the intra-abdominal and pelvic cavity were more common in this group compared to 4https://aps-journal.org https://doi.org/10.13029/aps.2022.28.1.1LymphangiomaTable 2. Clinical characteristicsCharacteristics Total Observation (n=114)Operation  (n=163)Sclerotherapy (n=166)Operation+Sclerotherapy (n=39)Others  (n=50)LocationSingle lesion 505 (94.9)Head and neck 228 53 39 97 18 21Chest and mediastinum 52 12 19 12 4 5Abdominal wall and flank 56 10 37 6 2 1Axilla 40 11 13 10 2 4Back and extremities 103 22 23 34 8 16Intra-abdominal and pelvic cavity 24 1 22 1Other 2 1 1Multiple lesions 27 (5.1) 4 10 7 5 1Chest+xilla 6 2 2 2Chest+Extremities 2 2Chest+Abdominal wall 4 2 1 1Abdominal wall+Extremities 3 1 1 1Neck+Chest 4 1 1 2Neck+Extremities 2 1 1Generalized 2 1 1Other 4 1 1 1 1Symptomsa)Mass 476 (89.5) 100 147 147 36 46Pain 56 6 31 11 2 6Infection 11 2 7 1 1Bleeding 16 7 4 2 1 2Swelling 12 4 2 6Other 22 1 12 4 2 3Imaging studiesa)US 355 (66.7) 79 105 121 28 22MRI 239 (44.9) 44 63 90 24 18CT 153 (28.8) 25 73 37 9 9Other 11 5 3 1 0 2Type of appearanceb)Macrocystic 380 (71.4) 75 120 132 29 24Microcystic 67 21 18 16 6 6Cavernous 25 8 11 2 1 3Unclassified 60 10 14 16 3 17Size (cm)Measurement 492 103 153 162 37 37Median (range)c) 4.8 (0.6–26.0) 3.7 (0.7–10.0) 6.0 (0.6–26.0) 5.0 (1.2–15.0) 6.5 (1.6–20.0) 4.1 (0.7–14.0)Values are presented as number (%).a)Including multiple selection; b)Data of imaging study or histopathology; c)Measurement of the largest diameter.Table 3. Observation onlyCharacteristics Value (n=114)Length of observation (n=88)Median days (range) 316 (7–4,490)ResultsGrossly normal 43 (37.7)Partial improvement (residual lymphangioma) 45No improvement 2Loss to follow-up 24Values are presented as number (%).the other treatment method groups. The most common histopathological diagnosis was lymphangioma, followed by hemangiolymphangioma. Most patients underwent complete excision in a single operation. Only 8% (13/163) of patients required more than 1 operation. Three patients underwent sclerotherapy during operation. Postoperative complications were not common; only 7.4% of patients experienced complications, and complications were of various kinds. After operation, 84.5% (138/163) of patients showed grossly normal results or no residual lesions (Table 4).SclerotherapyA total of 166 patients (31.2%) underwent sclerotherapy, which is similar to the number of patients who underwent operation. The most common sclerosing agent was OK-432, followed by bleomycin and doxycycline. General anesthesia was required in the majority of patients, and more than one-third of patients required multiple sessions of sclerotherapy. Complications after sclerotherapy were extremely rare; only 3 patients experienced complications. After sclerotherapy, 57.2% (95/166) of patients showed grossly normal results (Table 5).5Lymphangiomahttps://doi.org/10.13029/aps.2022.28.1.1https://aps-journal.orgTable 4. Operationa)Characteristics Value (n=163)Age at time of surgery (median, yr) 3.4 (0.0–19.7)Duration from diagnosis to operation (median, days) 11 (0–2112)Body weight at time of surgery (mean, kg) 21.0±17.9Operation duration (mean, min) 113.5±82.0Co-operation with other department 6Emergency operation 8b)PathologyLymphangioma 141 (86.5)Hemangiolymphangioma 12Hematoma or Pseudocyst 5Others 5Type of operationComplete excision 140 (85.9)Incomplete excision 22Unknown 1Sclerotherapy during operation 3No. of operations1 150 (92.0)2 11≥3 2Postoperative complications 12 (7.4)Skin necrosis 1Hematoma 1Postoperative intestinal obstruction 2Lymphatic fluid collection 2Proptosis (orbital lymphangioma) 1Wound dehiscence 1Secondary lymphedema 1Postoperative ileus 1Drain site infection 1Ipsilateral ptosis 1Result of operationGrossly normal 138 (84.5)Residual lymphangioma 21Loss to follow-up 4Values are presented as number (%).a)In cases of multiple operation, the data were based on the first operation; b)Eight cases of intestinal obstruction.Operation and sclerotherapyThirty-nine patients (7.3%) underwent operation and sclerotherapy separately, not simultaneously. The majority of these patients underwent sclerotherapy either before or after operation, but 3 patients underwent sclerotherapy both before and after operation. Multiple sclerotherapy sessions were required in 51.3% (20/39) of patients. After treatment, 53.8% (21/39) showed grossly normal results (Table 6).Other treatmentsA total of 11 patients underwent other treatments. Of these, 4 had drug treatment (2 cases of steroid medication and 2 cases of antibiotic medication), 4 underwent ultrasound-guided aspiration, and 3 had CO2 laser abrasions. Only 2 patients showed grossly normal results at follow-up.DISCUSSIONAlthough lymphatic malformations can occur in any anatomic region, most clinical studies of lymphangioma, or lymphatic malformation, focus on lymphatic-rich areas, such as the head and neck, the axilla, and the mediastinum [3,5,6]. However, understanding the overall clinical characteristics of lymphangioma is important because the basic concepts in the treatment of lymphangioma are similar, regardless of the lesion site [2,4]. The significance of this study lies not only in the fact that it is the first nationwide survey on lymphangioma in Korea but also in the fact that it integrates clinical characteristics and treatment results of patients with lymphangioma.6https://aps-journal.org https://doi.org/10.13029/aps.2022.28.1.1LymphangiomaTable 5. SclerotherapyCharacteristics Value (n=166)Age at time of sclerotherapy (median, yr) 1.4 (0.0–18.9)Sclerosing agentsOK-432 131 (78.9)Bleomycin 16Doxycycline 13Ethanol 6Anesthesiaa)General 103 (62.0)Local 32None 31Radiologic interventionYes 23 (13.9)No 143Complication 3 (1.8)Skin necrosis 2Ecchymosis 1No. of sclerotherapy sessions1 102 (61.4)2 383 104 4≥5 12ResultsGrossly normal 95 (57.2)Residual lymphangioma 48Loss to follow-up 23Values are presented as number (%).a)Multiple injections with general anesthesia or other methods were classified as general anesthesia.The results of this study are consistent with those of previous studies with respect to the most common locations of lesions, the major symptoms, the kinds of imaging studies, the types of appearances [1,7], and favoring of OK-432 as a sclerosing agent [8-10]. However, in contrast with other studies, we included observation without other treatments as a method of treatment [6]. We believe that the benign characteristic of lymphangioma and the possibility of spontaneous regression makes observation a viable treatment option [11].Among the treatment methods considered, the best results were obtained from operation, followed by sclerotherapy, co-treatment with operation and sclerotherapy, and observation. 7Lymphangiomahttps://doi.org/10.13029/aps.2022.28.1.1https://aps-journal.orgTable 6. Operation and sclerotherapyCharacteristics (n=39) SclerotherapyBefore operation After operation Before and after operationNo. of patients 17 19 3Age at time of sclerotherapy (median, yr) 0.5 (0.0–8.3) 2.4 (2.1–19.2) 0.4 (0.0–0.6)No. of sclerotherapy sessions1 9 102 3 23 3 2 24 1≥5 2 4 1Sclerosing agentsOK-432 17 16 3Bleomycin 2Doxycycline 1AnesthesiaGeneral 10 11 3Local 4 3None 3 5Radiologic interventionYes 4 6 3No 13 13 0ComplicationsFever 1Bleeding 1Pain 1Age at time of surgery (median, yr) 2.0 (0.2–10.1) 1.4 (0.1–18.5) 1.1 (0.6–1.6)Duration from diagnosis to operation (median, days) 375 (14–1917) 25 (5–399) 403 (210.0–555)Body weight at time of surgery (mean, kg) 13.4±6.3 17.9±18.3 9.5±1.7Operation duration (mean, min) 152.8±168.1 229.3±173.8 81.3±31.5Co-operation with other department 3 7 0Emergency operation 0 0 0PathologyLymphangioma 12 17 3Hemangiolymphangioma 3 2Unknown 2Type of operationComplete excision 10 7 1Incomplete excision 7 12 2Sclerotherapy during operation 1 1 1No. of operation1 16 14 12 1 3 1≥3 2 1Postoperative complications 1 (swelling)ResultGrossly normal (21/39, 53.8%) 11 9 1Residual lymphangioma 5 9 2Loss to follow-up 1 1However, the correlation between the choice of treatment method and the difficulty of treatment should be considered when interpreting these results. The difficulty of treating lymphangioma generally varies significantly depending on whether it takes a simple or complex form [5,12]. We think it was possible that we avoid the operation in complex form of lymphangioma, and non-operative treatment methods were selected as the preferred treatment method over operation. In this study, we did not distinguish between simple and complex forms of lymphangioma, which is one of the limitations of this study.Other limitations are that we did not include long-term follow-up results, and we did not compare results according to the area where the lesion occurred. These issues should be addressed in future studies. Despite its limitations, this study provides general information on lymphangioma and comprehensive treatment outcomes. Therefore, we expect it will be an important reference for pediatric surgeons seeking to better understand lymphangioma and treatment options for this disease and that it will ultimately lead to better outcomes.REFERENCES 1. Elluru RG, Balakrishnan K, Padua HM. Lymphatic malformations: diagnosis and management. Semin Pediatr Surg 2014;23:178-85. PUBMED | CROSSREF 2. Kang KS, Jung PM. Clinical manifestation and treatment of lymphangioma in children: a review of 117 cases. J Korean Assoc Pediatr Surg 2002;8:95-100. CROSSREF 3. Lee JH, Choi SO, Park WH. Abdominal cystic lymphangioma. J Korean Assoc Pediatr Surg 2006;12:232-7. CROSSREF 4. Okazaki T, Iwatani S, Yanai T, Kobayashi H, Kato Y, Marusasa T, et al. Treatment of lymphangioma in children: our experience of 128 cases. J Pediatr Surg 2007;42:386-9. PUBMED | CROSSREF 5. Hwang D, Lee S, Lim SY, Lee SK, Seo JM. Surgical treatment of difficult cervicofacial lymphangioma in children. J Korean Assoc Pediatr Surg 2015;21:17-23. CROSSREF 6. Perkins JA, Manning SC, Tempero RM, Cunningham MJ, Edmonds JL Jr, Hoffer FA, et al. Lymphatic malformations: review of current treatment. Otolaryngol Head Neck Surg 2010;142:795-803, 803.e1. PUBMED | CROSSREF 7. Alqahtani A, Nguyen LT, Flageole H, Shaw K, Laberge JM. 25 years’ experience with lymphangiomas in children. J Pediatr Surg 1999;34:1164-8. PUBMED | CROSSREF 8. Won JH. Sclerotherapy of cystic lymphangioma. J Korean Med Assoc 2014;57:343-7. CROSSREF 9. Jung SE, Kim DY, Shim IB, Lee SC, Park KW, Kim WK. Therapeutic effect of OK-432 for lymphangioma in children. J Korean Assoc Pediatr Surg 1998;4:131-6. CROSSREF 10. Ogita S, Tsuto T, Nakamura K, Deguchi E, Tokiwa K, Iwai N. OK-432 therapy for lymphangioma in children: Why and how does it work? J Pediatr Surg 1996;31:477-80. PUBMED | CROSSREF 11. Kato M, Watanabe S, Kato R, Kawashima H, Iida T, Watanabe A. Spontaneous regression of lymphangiomas in a single center over 34 years. Plast Reconstr Surg Glob Open 2017;5:e1501. PUBMED | CROSSREF 12. Trenor CC 3rd, Chaudry G. Complex lymphatic anomalies. Semin Pediatr Surg 2014;23:186-90. PUBMED | CROSSREF8https://aps-journal.org https://doi.org/10.13029/aps.2022.28.1.1Lymphangioma

Lymphangioma: A 2019 Survey Conducted by the Korean Association of Pediatric Surgeons

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Lymphangioma: A 2019 Survey Conducted by the Korean Association of Pediatric Surgeons

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