Use of Paley Classification and SUPERankle Procedure in the Management of Fibular Hemimelia

J Pediatr Orthop. 2019 Oct;39(9):e708-e717. doi: 10.1097/BPO.0000000000001012.

Abstract

Background: Fibular hemimelia is the most common deficiency involving the long bones. Paley classification is based on the ankle joint morphology, identifies the basic pathology, and helps in planning the surgical management. Reconstruction surgery encompasses foot deformity correction and limb length equalization. The SUPERankle procedure is a combination of bone and soft tissue procedures that stabilizes the foot and addresses all deformities.

Methods: We retrospectively reviewed 29 consecutive patients (29 limb segments), surgically treated between December 2000 and December 2014. Among the 29 patients, 27 were treated with reconstructive procedures. Type 1 (8 patients) cases were treated with only limb lengthening, and correction of tibial deformities. Type 2 (7 patients) cases were treated by distal tibial medial hemiepiphysiodesis or supramalleolar varus osteotomy. In type 3 (10 patients) cases, the foot deformity was corrected using the SUPERankle procedure. Type 4 (2 patients) cases were treated with supramalleolar osteotomy along with posteromedial release and lateral column shortening. In a second stage, limb lengthening was performed, using the Ilizarov technique. In the remaining 2 patients (type 3A and type 3C), amputation was performed using Syme technique as a first choice of treatment.

Results: The results were evaluated using Association for the Study and Application of Methods of Ilizarov scoring. Excellent results were obtained in 15 of 27 (55%) patients. Six (22%) patients had good results, 4 (14.8%) had fair results, and 2 (7%) had poor results. Mean limb length discrepancy at initial presentation was 3.55 cm (range: 2 to 5.5 cm) which significantly improved to 1.01 cm (range: 0 to 3 cm) after treatment (P=0.015).

Conclusions: Our results and a review of the literature clearly suggest that limb reconstruction according to Paley classification, is an excellent option in the management of fibular hemimelia. Our 2-staged procedure (SUPERankle procedure followed by limb lengthening) helps in reducing the complications of limb lengthening and incidence of ankle stiffness. Performing the first surgery at an earlier age (below 5 y) plays a significant role in preventing recurrent foot deformities.

Level of evidence: Level IV.

MeSH terms

  • Amputation, Surgical
  • Ankle Joint / surgery*
  • Bone Lengthening
  • Child
  • Child, Preschool
  • Ectromelia / classification*
  • Ectromelia / complications
  • Ectromelia / surgery*
  • Female
  • Fibula / surgery*
  • Humans
  • Ilizarov Technique
  • Infant
  • Leg Length Inequality / etiology
  • Leg Length Inequality / surgery*
  • Male
  • Osteotomy
  • Plastic Surgery Procedures / methods*
  • Retrospective Studies
  • Tibia / surgery