Surgical Management of Pediatric Lymphedema: A Systematic Review

Abstract

Background Lymphedema is a rare, progressive, and debilitating condition caused by failure of the lymphatic system to adequately drain the protein-rich fluid exiting the capillaries. Conservative management is often emphasized in pediatric patients, resulting in a paucity of literature describing surgical treatment in this population.

Methods A systematic review was performed using PubMed, ScienceDirect, and Google Scholar to identify all studies describing surgical management of lymphedema in the pediatric population.

Results Of the 343 relevant articles identified, 14 met the criteria for full review. Articles were divided into the following treatment categories: genital lymphedema, excisional procedures for extremity lymphedema, and physiologic procedures for extremity lymphedema. Outcomes for genital lymphedema were overall positive. For extremity lymphedema, excisional procedures yielded good results overall and included the majority of patients in this study. Physiologic procedures had mixed outcomes in the small population included in this study, but definitive conclusions cannot be drawn due to the paucity of existing data.

Conclusion While conservative management of pediatric lymphedema is well described, the literature remains sparse regarding surgical treatment. Further studies are needed to evaluate efficacy and outcomes in all three categories in this population.

Keywords

lymphedema - pediatric - surgical

• References

• 1 Watt H, Singh-Grewal D, Wargon O, Adams S. Paediatric lymphoedema: a retrospective chart review of 86 cases. J Paediatr Child Health 2017; 53 (01) 38-42
• 2 Carl HM, Walia G, Bello R. , et al. Systematic review of the surgical treatment of extremity lymphedema. J Reconstr Microsurg 2017; 33 (06) 412-425
• 3 Cheng MH, Chang DW, Patel KM. “Definition, Incidence, and Pathophysiology of Lymphedema.” Principles and Practice of Lymphedema Surgery. Philadelphia: Elsevier; 2016: 44-45
• 4 Phillips JJ, Gordon SJ. Conservative management of lymphoedema in children: a systematic review. J Pediatr Rehabil Med 2014; 7 (04) 361-372
• 5 Lang EM, Munder BI, Andree C. , et al. A modified therapeutic option for a long lasting success in the treatment of genital lymphedema. Eur J Plast Surg 2006; 29: 13
• 6 Bolt RJ, Peelen W, Nikkels PG, de Jong TP. Congenital lymphoedema of the genitalia. Eur J Pediatr 1998; 157 (11) 943-946
• 7 Mor Y, Zaidi SZ, Rose DS, Ransley PG, Mouriquand PD. Granulomatous lymphangitis of the penile skin as a cause of penile swelling in children. J Urol 1997; 158 (02) 591-592
• 8 Schook CC, Kulungowski AM, Greene AK, Fishman SJ. Male genital lymphedema: clinical features and management in 25 pediatric patients. J Pediatr Surg 2014; 49 (11) 1647-1651
• 9 Ross JH, Kay R, Yetman RJ, Angermeier K. Primary lymphedema of the genitalia in children and adolescents. J Urol 1998; 160 (04) 1485-1489
• 10 Tapper D, Eraklis AJ, Colodny AH, Schwartz M. Congenital lymphedema of the penis: a method of reconstruction. J Pediatr Surg 1980; 15 (04) 481-485
• 11 Onoda S, Yamada K, Matsumoto K, Kimata Y. A detailed examination of the characteristics and treatment in a series of 33 idiopathic lymphedema patients. J Reconstr Microsurg 2017; 33 (01) 19-25
• 12 Olszewski WL. Lymphovenous microsurgical shunts in treatment of lymphedema of lower limbs: a 45-year experience of one surgeon/one center. Eur J Vasc Endovasc Surg 2013; 45 (03) 282-290
• 13 Fonkalsrud EW. Surgical management of congenital lymphedema in infants and children. Arch Surg 1979; 114 (10) 1133-1136
• 14 Mousavi SR, Mehdikhah Z, Kavyani A, Saberi AR. Innovative procedure in surgical management of chronic lymphoedema. Surg Pract 2009; 13: 48-52
• 15 Feins NR, Rubin R, Crais T, O'Connor JF. Surgical management of thirty-nine children with lymphedema. J Pediatr Surg 1977; 12 (03) 471-476
• 16 Fonkalsrud EW. Congenital lymphedema of the extremities in infants and children. J Pediatr Surg 1969; 4 (02) 231-236
• 17 Smeltzer DM, Stickler GB, Schirger A. Primary lymphedema in children and adolescents: a follow-up study and review. Pediatrics 1985; 76 (02) 206-218
• 18 Schook CC, Mulliken JB, Fishman SJ, Grant FD, Zurakowski D, Greene AK. Primary lymphedema: clinical features and management in 138 pediatric patients. Plast Reconstr Surg 2011; 127 (06) 2419-2431
• 19 Szuba A, Shin WS, Strauss HW, Rockson S. The third circulation: radionuclide lymphoscintigraphy in the evaluation of lymphedema. J Nucl Med 2003; 44 (01) 43-57
• 20 Maclellan RA, Couto RA, Sullivan JE, Grant FD, Slavin SA, Greene AK. Management of primary and secondary lymphedema: analysis of 225 referrals to a center. Ann Plast Surg 2015; 75 (02) 197-200
• 21 Scaglioni MF, Fontein DBY, Arvanitakis M, Giovanoli P. Systematic review of lymphovenous anastomosis (LVA) for the treatment of lymphedema. Microsurgery 2017; 37 (08) 947-953
• 22 Hara H, Mihara M, Ohtsu H, Narushima M, Iida T, Koshima I. Indication of lymphaticovenous anastomosis for lower limb primary lymphedema. Plast Reconstr Surg 2015; 136 (04) 883-893
• 23 Venkatramani H, Kumaran S, Chethan S, Sabapathy SR. Vascularized lymph node transfer from thoracodorsal axis for congenital and post filarial lymphedema of the lower limb. J Surg Oncol 2017; 115 (01) 78-83
• 24 Schook CC, Mulliken JB, Fishman SJ, Alomari AI, Grant FD, Greene AK. Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema. Plast Reconstr Surg 2011; 127 (04) 1571-1581