[Popliteal Pterygium Syndrome (PPS): A Multimodal Treatment Approach and Long-Term Outcomes in 4 Cases].

Abstract

Background: Popliteal pterygium syndrome (PPS) is a rare, autosomal dominant congenital disorder characterized by the presence of a skin and soft-tissue web in the popliteal region. Functional impairment due to knee flexion contractures, equinus deformity, and hip malalignment is common. The existing literature is dominated by isolated case reports, while robust long-term outcome data are lacking.

Patients and methods: In a retrospective case series, four patients with popliteal pterygium and functionally relevant knee flexion contractures were treated surgically. The operative approach included complete resection of the fibrous band, multiple Z-plasties, microsurgical neurolysis of the sciatic nerve, and Achilles tendon lengthening. The mean follow-up period was 16 years (range: 5-23 years). Postoperatively, individualized orthoses were used, and in two cases custom-built stretching devices were applied.

Results: Knee extension improved by a mean of 30° (from 45° preoperatively to 15° postoperatively). At final follow-up, all patients were able to ambulate without assistive devices. A growth-related recurrence occurred in one case. No neuropathic pain or secondary paresis was observed.

Conclusion: In functionally relevant popliteal pterygia, a single-stage surgical approach consisting of complete band resection, neurolysis, and multiple Z-plasties, combined with consistent postoperative stretching and orthotic management, can achieve sustained functional improvement. This case series presents long-term outcomes extending over more than two decades for the first time.