Primary Lymphedema Multidisciplinary Approach (P-LYMA): Comprehensive Treatment and Preliminary Results in Patients with Primary Lower Extremity Lymphedema
Joseph M. Escandón1, Pedro Ciudad2, Lauren Escandón3, Juste Kaciulyte4, Oscar J. Manrique1
1University of Rochester Medical Center, Rochester, NY; 2Arzobispo Loayza National Hospital, Lima, Peru; 3Universidad El Bosque, Bogota DC, Colombia; 4Sapienza University of Rome, Italy
Purpose: Primary lymphedema is a chronic condition that results from developmental abnormalities and malfunction of the lymphatic system. Its worldwide prevalence is about 1 in 100.000 individuals. The natural history of the disease is variable as it depends on the penetrance and expression of underlying genetic anomalies. Primary lymphedema usually involves the lower limbs (LL), and rarely affects the genitalia and upper limbs. We present the outcomes of the surgical management of primary LL lymphedema (PLLL) following our algorithm.
Methods: We reviewed charts of patients with a diagnosis of PLLL made with lymphoscintigraphy and ICG-lymphography from 01/2017 to 06/2020. We implemented physiologic procedures such as lympho-venous bypass (LVB) and vascularized lymph node transplant (VLNT); and excisional procedures such as suction-assisted lipectomy (SAL), radical reduction with preservation of perforators (RRPP), and Charle’s and Homan’s procedure, independently or in combination, depending on the number of suitable lymphatics and lymphedema stage. All patients had postoperative complex decongestive therapy (CDT). Postoperative circumferential reduction rates (CRR) and Lymphedema Quality of Life Score (LeQOLiS) at 3, 6, 12, and 24-months were evaluated.
Results: Twenty-two LLs in eleven males (58%) and 8 females (42%) with PLLL were evaluated. The mean age and BMI were 28.8±11.1-years and 29.1±5.3-kg/m2. The average symptom duration was 13.41±7.97-years. According to ISL staging, two patients presented with stage IIA lymphedema, four patients with stage IIB, and thirteen with stage III. At 12 months postoperatively, the CRR in patients treated with LVB was 50% and in the patient treated with VLNT was 45%. The mean CRR in eight patients treated with SAL+LVB was 72.9%±26% and in five patients treated with SAL+VLNT was 73.4%±9.6%. In two patients treated with RRPP+LVB the CRR was 70% and in two patients treated with RRPP+VLNT the CRR was 72.5%. In three patients treated with CHAHOVA procedure, the mean CRR was 90%±8.7%. The average number of episodes of cellulitis per year decreased from 1.9±0.8 to 0.4±0.6 after surgery. The preoperative mean LeQOLiS was 70.4±11.6. LeQOLiS improved to 20.3±9.6, 23.5±17.4, and 24.2±13.9 at 3, 6, and 12 months after surgery, respectively. At a 25-month follow-up, the LeQOLiS score was 25.3±14.4 (12 patients).
Conclusion: The combination of different surgical techniques (physiologic and excisional) according to optimal patient stratification, as well as preoperative and postoperative concomitant CDT, improves patients’ quality of life, improves the CRR, and decreases the episodes of cellulitis in patients with PLLL on the long-term.
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