The SUPERankle procedure in the treatment of foot and ankle deformities in fibular hemimelia

Objective: To review indications for limb amputation or reconstruction using the SUPERankle procedure in patients diagnosed with Paley type 3 or 4 fibular hemimelia; To evaluate the correction of deformities needed to achieve a stable plantigrade foot, review variations of the original technique and their applicability, and describe challenges encountered in their execution. Methods: Qualitative, retrospective, descriptive study of 4 patients who underwent the SUPERankle procedure between 2019 and 2020 for treatment of foot and ankle deformities in fibular hemimelia. Pre- and postoperative clinical and radiographic evaluations were performed to identify objective evidence of the correction of foot and ankle deformities. The operative techniques employed are described. Results: The included patients met anatomical and psychosocial criteria for reconstruction, as established in the literature. Clinical correction of foot and ankle deformities was achieved, but on radiographic evaluation, there was no statistical difference; this was likely due to the small number of patients, given the rarity of fibular hemimelia. Conclusion: The procedure is reproducible, but requires a knowledgeable, well-trained surgical team. Correction of foot and ankle deformities can be achieved. The choice between amputation or reconstruction should be the result of an informed decision by Family members, supported by a multidisciplinary team. Level of Evidence IV; Qualitative Study; Case Series.

Paediatric Orthopaedics

This chapter contains practice questions based on a broad range of orthopaedic topics that may be encountered within the ‘Hand & Paediatrics’ station of the FRCS (Trauma & Orthopaedics) viva examination. The paediatric questions include important causes of hip pain in children, such as developmental dysplasia of the hip (DDH), slip of the right upper femoral epiphysis (SUFE), Perthes’ disease, and septic arthritis. Trauma topics include supracondylar and femoral fractures, and the chapter also discusses congenital deformities including fibular hemimelia and talipes.

Lengthening Reconstruction Surgery for Fibular Hemimelia: A Review

Fibular hemimelia (FH) presents with foot and ankle deformity and leg length discrepancy. Many historic reconstructions have resulted in poor outcomes. This report reviews modern classification and reconstruction methods. The Paley SHORDT procedure (SHortening Osteotomy Realignment Distal Tibia) is designed to correct dynamic valgus deformity. The Paley SUPERankle procedure (Systematic Utilitarian Procedure for Extremity Reconstruction) is designed to correct fixed equino-valgus foot deformity. The leg length discrepancy in FH is successfully treated with serial lengthening and epiphysiodesis. Implantable intramedullary lengthening devices have led to all internal lengthenings. Recent advancements in techniques and implants in extramedullary implantable limb lengthening (EMILL) have allowed internal lengthenings in younger and smaller patients, who would traditionally require external fixation. These new internal techniques with lengthenings of up to 5 cm can be repeated more easily and frequently than external fixation, reducing the need to achieve larger single-stage lengthenings (e.g., 8 cm). Modern reconstruction methods with lengthening are able to achieve limb length equalization with a plantigrade-stable foot, resulting in excellent functional result comparable or better than a Syme’s amputation with prosthetic fitting.

Clinical outcomes in pediatric tibial lengthening and deformity correction: a comparison of the Taylor Spatial Frame with the Orthex Hexapod System

Purpose Comparison of two hexapod frame systems in paediatric tibial deformity correction; the Taylor Spatial Frame (TSF) and Orthex Hexapod System. Methods Paediatric patients with congenital and acquired tibial deformities treated with either TSF (between 2014 and 2016) or Orthex (between 2017 and 2019) frames were included in a retrospective comparative study. Outcome measures were healing index, pin infection rate, regenerate quality and density, software residual rate, deformity correction accuracy, strut exchanges and quality of life (QoL). Results The TSF group had 17 patients (18 frames) and the Orthex group had 21 patients (25 frames). The most common indications for tibial deformity correction were fibular hemimelia (14) and septic or traumatic growth arrest (8). The median time in frame was 230 days (TSF) versus 203 days (Orthex) (p= 0.06). The mean lengthening achieved was 54 mm (TSF) and 51 mm (Orthex) (p = 0.41). The healing index was 41 days/cm (TSF) versus 43 days/cm (Orthex) (p = 0.70). Pin site infections occurred more in the TSF cohort (40%) than in the Orthex cohort (18%) (p < 0.001). The regenerate in the Orthex group showed higher density at three months (p = 0.029) and was more homogenous (p = 0.023) at six months after frame application. Strut exchanges were less frequent with the Orthex system (p < 0.0001). QoL measures were similar in both cohorts (p = 0.92). Conclusions This is the first study to compare two hexapod designs in paediatric orthopaedics. The Orthex system showed superiority in regenerate quality and a significant reduction in pin site infection rates. Both systems delivered predictable and accurate limb deformity correction. Level of evidence III

Fibular Aplasia, Tibial Campomelia, Oligo-Syndactyly Syndrome and Probable Femur Fibula Ulna Syndrome- Case Reports

One in two thousand neonates suffer from congenital limb deficiencies. Fibular hemimelia, a birth defect, has an estimated incidence of 5.7 to 20 cases per 1 million births. Fibular Aplasia, Tibial Campomelia and Oligo-Syndactyly (FATCO) syndrome is one such which is a triad of fibular hemimelia (aplasia/hypoplasia of fibula), tibial campomelia (bending of tibial bone) and oligo syndactyly. It is a syndrome of unknown genetic basis and inheritance. Very few cases on this condition have been reported so far. This article reports two cases on this condition, wherein the babies had considerable variability of limb malformations. The first is a newborn with FATCO, and the second is a two-month-old male infant with FATCO associated with right focal femoral deficiency. In view of paucity of the cases, there is a need to report every case which may help in creating awareness and a standardised management approach.