Introduction: Lymphedema, a debilitating characterized by localized fluid retention and tissue swelling, results from abnormalities in the lymphatic system. In the case of primary lymphedema, this condition is attributed to malformations in lymphatic vessels or nodes, and it is marked by a relentless progression leading to irreversible tissue fibrosis after repetitive inflammation. Many questions regarding its treatment, such as the choice of the type of intervention and the timing, still remain unanswered. This study aims to present our comprehensive approach to treat this challenging condition.
Methods: To elucidate our approach, we conducted a retrospective chart review of 42 patients treated for primary lymphedema at 3 hospitals between July 2010 and December 2022. The study included two patient groups, those with early-stage disease (20) and those in the advanced stages (22). We outline our algorithm, based on our clinical experience in Taiwan. Patients were followed for at least 12 months post-treatment, and assessments were made, including photographic evidence.
Results: A total of 42 patients participated in our study: 20 in the early stage and 22 in the late stage. Our approach yielded significant functional improvements and symptom regression in both groups. In the early-stage cohort, all 20 patients underwent VLNT procedures and SAL, with 15 (75%) undergoing unilateral procedures and 5 (25%) bilateral. Among the advanced-stage patients, 12 (54.5%) were treated with the modified Charles' procedure, and 10 (45.5%) with RRPP. The outcomes showed an average circumference reduction of 4.1 cm (2.9-5.3) after VLNT and liposuction. Reductions were noted at various levels: 5.7 cm (4.6-6.8) at mid-thigh, 4.3 cm (2.5-6.1) at mid-calf, 3.5 cm (2.7-4.3) at the ankle, and 1.4 cm (0.7-2.1) at mid-foot. Tonicity decreased by 5.9% (5.2-6.6), indicating significant tissue softening. Tissue removal averaged 3.7 kg (2.9-4.5) after the modified Charles' procedure and 2.6 kg (2.3-2.9) after RRPP. Patients experienced a mean of 3 (2-4) episodes of cellulitis per year, with no cellulitis in early-stage treated limbs during the follow-up period. Complications were minimal, including 4 partial skin graft losses that healed with conservative treatment and 3 postoperative infections after the modified Charles' procedure, treated successfully with antibiotics. No major complications were reported at the lymph node flap donor site.
Conclusion: Primary lymphedema poses a considerable challenge, primarily due to its relentless progression if left untreated. The existing literature offers limited guidance on its management. Our algorithm, developed over years of experience, aims to fill this gap. By integrating surgical and conservative interventions, as well as individualized patient follow-up, we provide a comprehensive framework for managing both early and late- stage cases.
Keywords: extensive therapeutic lipectomy; lymph node transfer; modified Charles procedure; primary lymphedema; radical reduction of lymphedema with preservation of perforators.
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