Management of Children with Megafoot Secondary to Proteus Syndrome: A Report of Three Cases with Long-Term Follow-Up

DEMİREL M., Koyuncu D., SAĞLAM Y., Bilgili F., Kılıçoğlu Ö. İ., DURMAZ H.

Journal of the American Podiatric Medical Association, cilt.114, sa.5, 2024 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 114 Sayı: 5
  • Basım Tarihi: 2024
  • Doi Numarası: 10.7547/22-130
  • Dergi Adı: Journal of the American Podiatric Medical Association
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, CINAHL, MEDLINE
  • İstanbul Üniversitesi Adresli: Evet

Özet

BACKGROUND: Proteus syndrome is a rare disorder characterized by overgrowth of limbs and organs and neurocutaneous findings. METHODS: We examined three Proteus syndrome cases with unilateral foot enlargement, megafoot. The patients had ambulatory and cosmetic difficulties. Debulking surgery was performed. RESULTS: After the surgeries, patient mobility and functional scores were analyzed and found to be improved; only one patient had recurrence after 5 years. In the literature, there is no consensus about the treatment and the timing of treatment of Proteus syndrome. The literature suggests that early surgery for megafoot leads to better functional results. Debulking surgery and amputation are the two options mentioned. In the present cases, soft-tissue debulking was performed. CONCLUSIONS: In patients with megafoot caused by Proteus syndrome, the functional results were better for those with early debulking surgery compared with those who did not undergo surgery. Patients were able to fit into shoes and walk without difficulty.