Background: Hip stability remains a major preoccupation during femoral lengthening in Congenital Femoral Deficiency (CFD). We aimed to review hip stability in Paley type 1a CFD patients undergoing femoral lengthening. Methods: A total of 33 patients with unilateral CFD, who were treated between 2014 and 2023, were retrospectively reviewed. In 20/33 cases (60.6%) the SUPERhip preparatory surgery was performed at a mean age of 4.3 years (range 2.7–8.1). The femoral lengthening using an external fixator was performed at a mean age of 7.8 years (range 4.3–14.3). Results: All patients presented with a stable hip joint after preparatory surgery and during femoral lengthening. Six cases of hip instability at a mean of 637 days after the external fixator removal were observed (range 127 to 1447 days). No significant differences between stable and unstable hips were noted for (1) Center-Edge Angle: 23.7 vs. 26.1 deg; (2) Acetabular Inclination: 12.8 vs. 11.7 deg; and (3) Ex-Fix Index: 35.6 days/cm vs. 42.4 days/cm; p > 0.05. Late hip instability was related to Coxa Vara and decreased femoral antetorsion before lengthening. Conclusions: Late hip joint instability in Paley type 1a CFD patients may occur long after femoral lengthening despite hip morphology appearing to be normal on radiograms before and at the end of femoral lengthening. Coxa Vara, femoral torsional deformity, and posterior acetabular deficiency might be risk factors for hip instability.

Introduction: Patients with fibular hemimelia (FH) require multistage treatment, which often includes repeated and extensive limb lengthening procedures. One of the complications of leg lengthening is tibia fracture at the level of the regenerate, which poses an even greater risk in the absence of the fibula. Possible solutions include cast immobilization or prophylactic intramedullary stabilization; however, it may be associated with a risk of infection. Methods: All patients treated with tibial lengthening for fibular hemimelia at a single institution between 2014 and 2024 were included in the study. In total, 48 tibial lengthening procedures using circular external fixators were performed in 41 patients. After completion of lengthening and at externa fixator removal, prophylactic intramedullary nailing (Rush rods or Titanium Elastic Nails) was used in 24 cases, while a patellar tendon-bearing (PTB) cast was applied in 24 cases. Following nail implantation, antibacterial prophylaxis was administered for 10 days. The mean follow-up period from the time of external fixator removal was 56.6 months (range 18 to 109). Tibial lengthening parameters (achieved lengthening, External Fixator Index) and radiologic parameters of the regenerate (number of visible cortical walls and the Ru–Li classification) were evaluated. The prevalence of fractures, infections, unplanned reoperations, or complications during and after the lengthening process was analyzed. The data were compared between the nailing group versus non-nailng group. Results: The groups were comparable in terms of sex distribution, regenerate length, regenerate quality, and External Fixator Index. The group without prophylactic intramedullary nailing was significantly older (mean 11.5 y, range 2.4 to 18.0) compared with the prophylactic nailing group (mean 7.5 y, range 2.5 to 16.6), P =0.002. No fractures occurred in patients who underwent prophylactic intramedullary nailing, whereas 6 fractures (25%) occurred in patients without prophylactic nailing (4 within the first month, 1 after 4 mo, and one 6 mo after removal of the external fixator), P =0.0229. No infections was observed in either group. Conclusions: Prophylactic intramedullary nailing performed at external fixator removal significantly reduced the risk of fractures after completion of tibial lengthening in patients with fibular hemimelia. With the use of antibiotic prophylaxis, no infections were observed following intramedullary nailing. Level of Evidence: Level III.

Background: Currently, there is a deficit of questionnaires available in Polish for as-sessing the quality of life of children with limb deformities. The LD-SRS questionnaire consists of 30 questions in 5 domains: function, experienced pain, patient's own ap-pearance, mental health and satisfaction with management, scored on a 1–5 scale, where higher scores indicate better quality of life. The aim was to culturally adapt and validate the LD-SRS questionnaire into Polish. Methods: The questionnaire has been translated from English into Polish by two translators. An expert team composed of physicians, nurses and a psychologist analysed the translations and developed the initial Polish version. It was translated back into English and analysed for the differences from the original to work out the pre-final version. After approval by the local ethics committee, the pre-final version was applied to a group of 21 patients as a pilot study. Then, the final version of the form was developed and tested once on the group of 77 children, mean age 13.9 years (8-20 years), and twice on the group of 34 children, mean age 15.9 years (12-19 years). The retest was obtained within 7 days (4-15 days). Results: The Cronbach’s alpha was 0.92, showing excellent internal consistency. The ICC value for total LD-SRS score (0.99) signified excellent reliability. The overall total LD-SRS score was 3.8 (SD=0.6). The results in each category were: function/activity 3.4 (SD=0.7), pain 3.9 (SD=1.0), self-image 3.5 (SD=0.7), mental health 3.6 (SD=1.0), satisfaction with management 4.2 (SD=0.8). We found no floor or ceiling effect for the total LD-SRS score. Conclusions: The Polish version of the LD-SRS questionnaire was found to be a reliable instrument with excellent internal consistency and can be used in assessing the quality of life of children treated for limb deformities.

Background: Fracture of newly formed bone (the regenerate) after EF (External Fixator) removal is a serious complication of bone lengthening in Congenital Femoral Deficiency (CFD). This study aimed to determine whether prophylactic intramedullary nailing (IMN) at the time of EF removal reduces the risk of femoral fractures in CFD and to evaluate its safety. Methods: This retrospective comparative study included 53 CFD patients who underwent femoral lengthening (44 in the IMN group, 9 in the no-IMN group). We analyzed the incidence of fractures, infections, and unplanned surgeries. The predictive value of Fischgrund principles for fracture risk was evaluated. Results: The fracture rate was 11.3% (6/53). The IMN group had fewer fractures (6.8%) versus the no-IMN group (33.3%, p=0.054). Surgical treatment risk for femoral fracture was significantly lower in the IMN group (2.3% vs 33.3%, p<0.05). Despite complications in IMN group (4.5% infection rate and 4.5% nail malposition rate), the rate of unplanned surgery was lower versus the no-IMN group (11.4% vs 33.3%, p=0.12). Poor regenerate morphology (insufficient cortices) predicted femoral fractures after EF removal only in no-IMN group. Prophylactic nailing reduced the negative impact of poor regenerate morphology. Conclusions: Prophylactic intramedullary nailing is an effective strategy to prevent early femoral fractures and reduce the risk of unplanned surgery. Fischgrund principles are not useful to predict the risk of femoral fracture after prophylactic nailing.

Background: Fracture of newly formed bone (the regenerate) after EF (External Fixator) removal is a serious complication of bone lengthening in Congenital Femoral Deficiency (CFD). This study aimed to determine whether prophylactic intramedullary nailing (IMN) at the time of EF removal reduces the risk of femoral fractures in CFD and to evaluate its safety. Methods: This retrospective comparative study included 53 CFD patients who underwent femoral lengthening (44 in the IMN group, 9 in the historical no-IMN group). We analyzed the incidence of fractures, infections, and unplanned surgeries. The predictive value of Fischgrund principles and Li classification for fracture risk was evaluated. Results: The fracture rate was 11.3% (6/53). The IMN group had fewer fractures (6.8%) versus the no-IMN group (33.3%, p = 0.054). Surgical treatment risk for femoral fracture was significantly lower in the IMN group (2.3% vs. 33.3%, p < 0.05). Despite complications in the IMN group (4.5% infection rate, 4.5% nail malposition rate, 2.3% hip dislocation), the rate of unplanned surgery was lower than in the no-IMN group (11.4% vs. 33.3%, p = 0.12). Associations between regenerate morphology and subsequent fracture occurrence were observed only in the historical no-IMN group. Prophylactic nailing may provide mechanical support in patients with less mature regenerate. Conclusions: In this retrospective study with a historical comparison group (no-IMN), prophylactic intramedullary nailing was associated with fewer fractures requiring surgery after femoral lengthening with EF in CFD.