Surgical management of unilateral lower limb lymphedema in combination of debulking and physiologic procedures: a case report

Gathariki Mukami1, Khainga Stanley1, Aluora Kenneth1, Malungo Nang’andu1, Ocholla Laura2, Amuti Thomas3

1Thematic Unit of Plastic Surgery, Department of Surgery, University of Nairobi, Nairobi, Kenya

2University of Nairobi, College of Health Sciences

3Department of Human Anatomy, University of Nairobi

Correspondence to: Mukami Gathariki; email: mukamigathariki.mg@gmail.com

Received: 19 Jun 2021; Revised: 27 Feb 2022; Accepted: 10 Mar 2022; Available online: 14 Apr 2022

Summary 

Lymphedema has a high incidence and various causes including filariasis in developing countries and as complications following oncological surgery in developed countries. It reduces patients’ quality of life, productivity and currently lacks a cure. Current management involves debulking or physiological methods, with debridement being the preferred debulking method and lymph node transfer, the preferred physiological option. Most reports on the management of lymphedema or its associated outcomes have reported the use of either of these modalities with favourable outcomes, albeit some complications. There is however paucity of documented cases where a patient underwent both debulking and physiological treatment in management of chronic lymphedema and our case study reports on the same, with favourable outcome and minimal complications. 

Our patient, a 30 year old female presented with an 8 year history of slowly progressive right lower limb oedema. Physical examination revealed marked right lower limb swelling that was non- pitting, non-tender, erythematous, with marked thickening of skin, yellowing of the nails and a positive stemmer sign. Lymphoscintigraphy revealed hypoplastic lymphatic channels and faulty valves. Debulking using modified Charles procedure and vascularised lymph node transfer were done within a one month interval. Ten months later, she is doing well with no relapse.

 

Keywords: Lymphedema, Charle's procedure, Lymph node transfer.

Ann Afr Surg. 2022; 19(4): 221-225

DOI: http://dx.doi.org/10.4314/aas.v19i4.11

Funding: None

© 2022 Author. This work is licensed under the Creative Commons Attribution 4.0 International License.

 

Introduction 

Lymphedema is a chronic progressive disorder, defined as an abnormal limb swelling caused by accumulation of increased amounts of high protein interstitial fluid (ISF) secondary to defective lymphatic drainage in the presence of near normal net capillary filtration (1). In developing countries, the common causes are podoconiosis and filariasis (2). In developed countries, lymphedema tends to occur secondary to oncologic surgery when draining lymph nodes have to be removed or irradiated to avoid tumour spread. Other identified causes include: burns, trauma, and infection. 

Lymphedema is categorized into primary and secondary. Primary lymphedema is caused by a dysfunction in the lymphatic system while secondary, results from insults following surgery, trauma or infection. (3). Chronic deposition of protein rich lymphatic fluid in the interstitial space stimulates inflammation, leading to fibrosis and further damage to the lymphatics with resultant cycle of deterioration (4,5,6). This results in a grossly enlarged extremity with increased susceptibility to infection, functional impairment and psychosocial morbidity (7,8,9).

Various treatment include non-surgical options like: compression garments, manual lymphatic drainage, exercise and skin care. Previously, either debulking or physiologic management has also been employed. Debulking involves excision of the excess skin and subcutaneous tissue down to the deep fascia with skin grafting of the defect or liposuction of the excess subcutaneous tissue with resultant immediate improvement in function. Physiologic procedures promote clearance of lymphatic fluid from the interstitial space and include lymphovenous anastomosis (LVA) and vascularized lymph node (VLN) transfer. However, since they do not directly eliminate the solid component, any surgical plan without an excisional component falls short of fully addressing the issue at hand. This is particularly true for the more advanced stages as seen in this case. Therefore, understanding the efficient and timely management of lymphedema is paramount. 

Case presentation

A 30 year old female teacher was referred to our centre in May 2019, with a long standing history of primary lymphedema affecting her right lower limb, since 2011, was gradual on onset and worsened over several months. It was non pitting, and associated with severe pain. The pain and associated skin changes were debilitating to the extent that she could not stand for long periods as her profession requires her to. She reported of no comorbidities or family history of the same. Prolonged history of travel as well as stigmata for infections (filariasis) and malignancy were also ruled out. She also did not have any previous surgeries done on the leg or any history of trauma. 

Physical examination revealed marked swelling of the right lower limb with non-tender, non-pitting oedema with erythema and marked thickening of the skin. elephantiasis nostra verrucosa, which is an area of cobble-stoned, hyperkeratotic, papillomatous plaques most commonly seen on the shins. The patient was also noted to have yellowing of the nails. Patient was also noted to have a positive Stemmer sign, which is inability to pinch the dorsal aspect of skin between the first and second toes. Based on these findings, the lymphedema was at stage four.

Routine exami