Popliteal Pterygium Syndrome: A Case Report Highlighting Challenges and Surgical Interventions in a Resource-limited Setting

Abstract

Summary: Popliteal pterygium syndrome is a rare congenital disorder characterized by facial, genitourinary, and musculoskeletal anomalies, with popliteal webbing being notably challenging. A 4-year-old boy presented with progressive limping, cleft palate, and genital malformations. He had no follow-up care after an intraoral band excision at 15 days old. The boy underwent surgery for left-sided popliteal webbing, followed by genital and cleft palate repair. A modified jumping man Z-plasty flap was used for the popliteal webbing, followed by splinting. Subsequent follow-ups showed no complications. Enhancing care in resource-constrained settings requires addressing challenges such as delayed interventions due to late follow-up, limited awareness between communities and healthcare professionals, social stigma, and inadequate healthcare understanding. These obstacles hinder timely diagnosis and intervention, underscoring the need for increased awareness and effective early intervention strategies. Early detection and parental counseling are critical in managing popliteal pterygium syndrome. Timely surgical planning, including addressing orofacial and genital deformities and using Z-plasty for webbing release, is essential. Postoperative splinting significantly improves outcomes.