Shanko Nagasa Wirtu, Eshete Mekonen, Hussen Reyad Adem, Chafamo Eyob Zergaw, Keno Senayad Banti
From the Plastic and Reconstructive Surgery Department, Addis Ababa University, Addis Ababa, Ethiopia.
Plastic and Reconstructive Surgery Department, Haramaya University, Dire Dawa, Ethiopia.
Plast Reconstr Surg Glob Open. 2024 Nov 21;12(11):e6332. doi: 10.1097/GOX.0000000000006332. eCollection 2024 Nov.
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.
腘窝翼状胬肉综合征是一种罕见的先天性疾病,其特征为面部、泌尿生殖系统和肌肉骨骼系统的异常,其中腘窝蹼尤其具有挑战性。一名4岁男孩出现进行性跛行、腭裂和生殖器畸形。他在15天大时接受口腔内束带切除术后没有接受后续护理。该男孩接受了左侧腘窝蹼手术,随后进行了生殖器和腭裂修复。采用改良的跳跃人Z形成形皮瓣修复腘窝蹼,术后进行夹板固定。随后的随访显示无并发症。在资源有限的环境中加强护理需要应对一些挑战,如因随访延迟导致的干预延迟、社区与医疗保健专业人员之间的认识有限、社会耻辱感以及对医疗保健的理解不足。这些障碍阻碍了及时诊断和干预,凸显了提高认识和有效的早期干预策略的必要性。早期发现和对家长的咨询对于管理腘窝翼状胬肉综合征至关重要。及时进行手术规划,包括处理口面部和生殖器畸形以及使用Z形成形术松解蹼,至关重要。术后夹板固定可显著改善治疗效果。