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The aim was to validate the new Taiwan Lymphoscintigraphy Staging, correlate it with Cheng Lymphedema Grading (CLG) and evaluate the treatment outcomes of unilateral extremity lymphedema.No consensus has been reached for diagnosis and staging for patients with lymphedema among medical specialties.We included 285 patients with unilateral extremity lymphedema using lymphoscintigraphy. Lymphoscintigraphy was correlated to clinical symptoms and signs, and classified into normal lymphatic drainage, partial obstruction, and total obstruction. Inter- and intraobserver reliability of Taiwan Lymphoscintigraphy Staging, correlation between Taiwan Lymphoscintigraphy Staging and clinical findings were conducted. Patients were categorized in “surgical” (n = 154) or “nonsurgical” (n = 131) groups for outcome evaluation.Lymphoscintigraphy found 11 patients (3.9%) with normal lymphatic drainage, 128 (44.9%) with partial obstruction, and 146 (51.2%) with total obstruction. Taiwan Lymphoscintigraphy Staging showed high interobserver agreement [intraclass correlation coefficient: 0.89 (95% confidence interval, 0.82–0.94)], and significantly correlated to computed tomography volumetric difference (r = 0.66, P < 0.001) and CLG [intraclass correlation coefficient: 0.79 (95% confidence interval 0.72–0.84)]. At a mean follow-up of 31.2 ± 2.9 months, significant improvement in the circumferential difference (from 23.9% ± 17.6% to 14.6% ± 11.1%; P = 0.03) with a mean circumferential reduction rate of 40.4% ± 4.5% was found in surgical group. At a mean follow-up of 26.6 ± 8.7 months, the nonsurgical group had increase of mean circumferential difference from 24.0% ± 17.2% to 25.3% ± 19.0% (P = 0.09), with a mean circumferential reduction rate was −1.9% ± 13.0%.The Taiwan Lymphoscintigraphy Staging is a reliable diagnostic tool, correlated with clinical findings and CLG, aiding in the selection of the appropriate treatment to achieve favorable long-term outcomes in unilateral extremity lymphedema.