Lymphedema Microsurgery Improved the Outcomes of Congenital Limb Lymphedema

Introduction

Congenital lymphedema is a rare disease (1 in 100,000 individuals) that causes discomfort, functional impairment, recurrent infections, and psychosocial maladjustment. Complex decongestive physiotherapy provides certain benefit. Physiological lymphedema microsurgeries include lymphovenous anastomosis (LVA) for patients with short symptom duration and partial lymphatic obstruction and vascularized lymph node transfer (VLNT) for patients with prolonged symptoms and total lymphatic obstruction. This study investigates the outcome of microsurgical procedures for congenital lymphedema.

Methods

Nine pediatric patients (7 females, 77.8%), with a mean age of 9.2 ± 5.9 years (ranged, 2- 18 years), underwent surgical intervention for congenital lymphedema between 2013 and 2017. Preoperative assessments included measurements of limb circumference (to measure treatment effectiveness), Tc-99 lymphoscintigraphy (to assess lymphedema severity), sonography (to rule out proximal venous insufficiency and assess donor site lymph node quantity), Magnetic resonance (MR) lymphography (to rule out vascular lesions), MR angiography (to ascertain donor site vessel course and lymph nodes), and any other physical conditions (e.g., cellulitis). After surgical treatment, they received regular follow-up assessments including episodes of cellulitis, limb circumference measurement and ultrasoud Doppler for the patency of pedicle and number of trasnferred lymph nodes. The Wilcoxon signed-rank and Spearman's rank correlation tests were performed for statistical analyses.

Results

A total of 13 lymphedematous limbs (11 lower limbs and 2 upper limbs) underwent surgical treatment (10 submental VLNT, 2 LVA, 1 omental VLNT). Cheng’s Lymphedema Grading ranged from 0 to 4 (2.6±1.6). The VLNT flap success rate was 100%, and the two LVA were patent. At a follow-up of 32.2 ± 15.2 months, mean body weight was increased 6.9 ± 3.7 kg, from 38.7±21.7 kg (range 12 to 71 kg) preoperatively to 42.8 ± 17.3 kg (range 18 to 71kg). The limb circumference was reduced by 0.9 ± 4.0 cm for AK/AE (range: -3.5 to 8.5 cm, p=0.5), 1.2 ± 4.8 cm for BK/BE (range: -3.5 to 9.5 cm, p=0.7) and 4.9± 6.5 cm for AA (ranged -1.5 to 16.5, p<0.05) without wearing compression garments. Among 9 limbs with cellulitis preoperatively, the frequency of cellulitis decreased from 2.3±2.1 times/year preoperatively to 0.5±0.5 times/year postoperatively (p<0.05), and showed moderate positive correlation to preoperative Cheng's Lymphedema Grading (ρ=0.503, p=0.2).

Conclusions

Pediatric patients with congenital limb lymphedema may have reduced limb circumference and decreased episodes of cellulitis without wearing compression garments from the approaches of LVA and VLNT.