Abstract
Purpose
Lymphatic malformations (LMs) are congenital abnormalities which result from disturbances in the embryologic development of the lymphatic system. We sought to determine the characteristics and treatment patterns for LMs in a rural setting, and the effect of a specialized vascular malformations clinic on triage and follow-up.
Methods
This is a retrospective cohort study at a single tertiary care institution. Sixty-two patients were identified; chart review was completed to obtain demographic, surgery/sclerotherapy session and follow-up information.
Results
The head/neck region was the most predominant LM location (N = 26, 41.9%), followed by trunk (N = 16, 25.8%), extremity (N = 11, 17.7%), and intraabdominal/retroperitoneal (N = 7, 11.3%). Twenty-eight patients were managed non-surgically, while 21, 7 and 6 patients required surgery, sclerotherapy, or both. Head/neck LMs were the most likely to recur (73%, p = 0.028). Patients seen in specialty clinic had similar duration of follow-up and time to intervention, but were more often below 1 year of age (p = 0.030). Average LM volume among patients with available imaging was much larger in those referred to specialty clinic (73.2 cm3 versus 14.8 cm3, p = 0.022).
Conclusion
Our experience reiterates not only the wide variety of clinical presentations of lymphatic malformations, but also demonstrates the necessity of multiple subspecialties and their collaboration to achieve prompt and efficacious treatment.
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References
Kennedy TL, Whitaker M, Pellitteri P, Wood WE (2001) Cystic hygroma/lymphangioma: a rational approach to management. Laryngoscope 111(11):1929–1937. https://doi.org/10.1097/00005537-200111000-00011
Fevurly RD, Fishman SJ (2012) Vascular anomalies in pediatrics. Surg Clin N Am 92(3):769–800. https://doi.org/10.1016/j.suc.2012.03.016
ISSVA Classification of Vascular Anomalies (2018) In: ISSVA Workshop
Ranieri M, Wohlgemuth W, Müller-Wille R et al (2017) Vascular malformations of upper and lower extremity - from radiological interventional therapy to surgical soft tissue reconstruction - an interdisciplinary treatment. Clin Hemorheol Microcirc 67(3–4):355–372. https://doi.org/10.3233/CH-179216
Wohlgemuth WA, Brill R, Dendl LM, Stangl F, Stoevesandt D, Schreyer AG (2018) Abdominal lymphatic malformations. Radiologe 58(May):29–33. https://doi.org/10.1007/s00117-017-0337-5
Hogeling M, Adams S, Law J, Wargon O (2011) Lymphatic malformations: clinical course and management in 64 cases. Australas J Dermatol 52(3):186–190. https://doi.org/10.1111/j.1440-0960.2011.00777.x
Elluru RG, Balakrishnan K, Padua HM (2014) Lymphatic malformations: diagnosis and management. Semin Pediatr Surg 23(4):178–185. https://doi.org/10.1053/j.sempedsurg.2014.07.002
De Maria L, De Sanctis P, Balakrishnan K, Tollefson M, Brinjikji W (2020) Sclerotherapy for lymphatic malformations of head and neck: systematic review and meta-analysis. J Vasc Surg Venous Lymphat Disord 8(1):154–164. https://doi.org/10.1016/j.jvsv.2019.09.007
Cheng J (2015) Doxycycline sclerotherapy in children with head and neck lymphatic malformations. J Pediatr Surg 50(12):2143–2146. https://doi.org/10.1016/j.jpedsurg.2015.08.051
Khanwalkar A, Carter J, Bhushan B, Rastatter J, Maddalozzo J (2018) Thirty-day perioperative outcomes in resection of cervical lymphatic malformations. Int J Pediatr Otorhinolaryngol 106:31–34. https://doi.org/10.1016/j.ijporl.2017.12.034
Markovic JN, Nag U, Shortell CK (2020) Safety and efficacy of foam sclerotherapy for treatment of low-flow vascular malformations in children. J Vasc Surg Venous Lymphat Disord. https://doi.org/10.1016/j.jvsv.2019.11.023
Lidsky ME, Markovic JN, Miller MJ, Shortell CK (2012) Analysis of the treatment of congenital vascular malformations using a multidisciplinary approach. J Vasc Surg 56(5):1355–1362. https://doi.org/10.1016/j.jvs.2012.04.066
Thomas DM, Wieck MM, Grant CN et al (2016) Doxycycline sclerotherapy is superior in the treatment of pediatric lymphatic malformations. J Vasc Interv Radiol 27(12):1846–1856. https://doi.org/10.1016/j.jvir.2016.08.012
Randolph GD, Pathman DE (2001) Trends in the rural-urban distribution of general pediatricians. Pediatrics 107(2):e18
Statement P, Basco W, Rimszam M, Workforce. AA of PC on P (2013) Pediatrician workforce policy statement. Pediatrics 132(2):390–397. https://doi.org/10.1542/peds.2013-1517
Skinner AC, Slifkin RT (2007) Rural/urban differences in barriers to and burden of care for children with special health care needs. J Rural Health 23(2):150–157. https://doi.org/10.1111/j.1748-0361.2007.00082.x
Pletcher BA, Rimsza ME, Cull WL, Shipman SA, Shugerman RP, O’Connor KG (2010) Primary care pediatricians’ satisfaction with subspecialty care, perceived supply, and barriers to care. J Pediatr 156(6):1011-1015.e1. https://doi.org/10.1016/j.jpeds.2009.12.032
Cheng J, Liu B, Farjat AE, Routh J (2018) National characteristics of lymphatic malformations in children: inpatient estimates and trends in the United States, 2000 to 2009. J Pediatr Hematol Oncol 40(3):221–223. https://doi.org/10.1097/MPH.0000000000001078
Mayer ML, Beil HA, von Allmen D (2009) Distance to care and relative supply among pediatric surgical subspecialties. J Pediatr Surg 44(3):483–495. https://doi.org/10.1016/j.jpedsurg.2008.08.015
Ray KN, Bogen DL, Bertolet M, Forrest CB, Mehrotra A (2014) Supply and utilization of pediatric subspecialists in the United States. Pediatrics 133(6):1061–1069. https://doi.org/10.1542/peds.2013-3466
Bagrodia N, Defnet AM, Kandel JJ (2015) Management of lymphatic malformations in children. Curr Opin Pediatr 27(3):356–363. https://doi.org/10.1097/MOP.0000000000000209
Churchill P, Otal D, Pemberton J, Ali A, Flageole H, Walton JM (2011) Sclerotherapy for lymphatic malformations in children: a scoping review. J Pediatr Surg 46(5):912–922. https://doi.org/10.1016/j.jpedsurg.2011.02.027
Chung CM, Tak SW (2019) Congenital cutaneous pedicled macrocystic lymphatic malformaiton on the upper extremity: a rare case report and review of the literature. Arch Aesthet Plast Surg 25(4):163–166
Drobot A, Ganam S, Karra N, Bickel A, Shakra IA, Kakiashvili E (2020) Resection of an axillary macrocystic lymphatic malformation in a 14-year old female using intraoperative indocyanine green lymphography. J Vasc Surg Venous Lymphat Disord. https://doi.org/10.1016/j.jvsv.2020.04.003
Rochon PJ (2017) Importance of multidisciplinary approach to vascular malformation management. Semin Interv Radiol 34(3):301–302. https://doi.org/10.1055/s-0037-1604302
Straughan AJ, Mudd PA, Silva AL, Callicott SS, Krakovsky G, Bauman NM (2019) Cost analysis of a multidisciplinary vascular anomaly clinic. Ann Otol Rhinol Laryngol 128(5):401–405. https://doi.org/10.1177/0003489419826135
Acknowledgements
This study was conducted under the supervision and sponsorship of Christa N. Grant, MD. Dr. Grant was involved in the study design, the analysis and interpretation of data, the writing of the report, and the decision to submit the manuscript for publication.
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383_2021_4859_MOESM1_ESM.tif
Supplementary file1. Supplementary Figure 1: Statewide distribution of patients seen for lymphatic malformations (LM), represented in light grey, based on zip code. (TIF 24656 KB)
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Kronfli, A.P., McLaughlin, C.J., Moroco, A.E. et al. Lymphatic malformations: a 20-year single institution experience. Pediatr Surg Int 37, 783–790 (2021). https://doi.org/10.1007/s00383-021-04859-5
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DOI: https://doi.org/10.1007/s00383-021-04859-5