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.

Congenital femoral deficiency (CFD) and congenital coxa vara (CCV) are rare conditions characterized by abnormal development of the femur and hip joint, respectively. This case report documents the rehabilitation journey of a seven-year-old child diagnosed with CFD and CCV, highlighting the efficacy of physical therapy interventions in enhancing strength, balance, normal gait patterns, confidence, and mobility. Through a comprehensive physiotherapy regimen tailored to the specific needs of the patient, significant improvements in muscle strength, joint stability, and functional mobility were observed over the course of treatment. Moreover, the implementation of targeted exercises and adaptive strategies not only facilitated physical gains but also contributed to bolstering the child\'s confidence and overall quality of life. This case underscores the pivotal role of physiotherapy in addressing the complex challenges associated with congenital orthopedic anomalies, ultimately fostering independence and well-being in pediatric patients.

UNASSIGNED: Congenital femoral deficiency is characterized by limb length discrepancy and genu valgum. Lengthening of the femur along its anatomic axis increases valgus alignment by medial knee translation. Pairing limb lengthening with simultaneous medial distal femoral hemiepiphysiodesis can simultaneously correct two limb deformities.
UNASSIGNED: All skeletally immature patients with congenital femoral deficiency who underwent antegrade femoral lengthening and concomitant guided growth over a 4-year period were reviewed. Length and alignment data were quantified during lengthening, consolidation, and for 1 year after guided growth implants were removed or the patient reached skeletal maturity. Digital simulation was performed for all lengthenings to assess the mechanical alignment that would have been achieved had lengthening been performed without medial distal femoral hemiepiphysiodesis.
UNASSIGNED: Nine patients (five males, four females, mean age = 12.3 ± 1.9 years) underwent 10 antegrade intramedullary femoral lengthenings with simultaneous medial distal femoral hemiepiphysiodesis. All had improvement in valgus alignment (average improvement in mechanical axis deviation was 18 ± 11 mm, average change in limb alignment was 6 ± 5°). In simulated lengthenings without guided growth, all limbs would have experienced increased lateral mechanical axis deviation of 5 ± 3 mm. The hemiepiphysiodesis implant and lengthening device were explanted simultaneously in 7 of 10 lengthenings.
UNASSIGNED: Simultaneous medial distal femoral hemiepiphysiodesis with antegrade femoral lengthening for ongenital femoral deficiency can minimize the number of surgical episodes for the skeletally immature patient. The lengthening device and guided growth construct can be removed simultaneously in a majority of cases, saving children one or two additional surgical treatments.

BACKGROUND: Congenital femoral deficiency (CFD) is a challenging and complex condition that causes limb lengthening. We focused on the clinical and radiographic results of SUPERhip procedures in patients with congenital femoral deficiency type 1b, according to Paley\'s classification, prior to femoral lengthening.
METHODS: We reviewed all records and radiographs of patients who underwent this procedure between 2005 and 2020. We included 26 patients, with clinical and radiographic assessments performed during pre- and post-operative evaluations.
RESULTS: There were twenty-six patients (15 right vs. 11 left), with a mean age of 7 years (1-18). Most of the patients were female (17 patients; 65.4%). Twenty (76.9%) patients were undergoing their first procedure and six (23.1%) had already undergone a previous surgery. There was a significant improvement in all radiographic parameters, with the mean preoperative and postoperative Neck-Shaft Angle (NSA) being 72.3 ± 7.1° vs. 133.1 ± 12.7°, the Center-Edge Angle (CEA) 16.8 ± 9.8° vs. 33.5 ± 14.1°, and the Acetabular Index (AI) 27.8 ± 6.9° vs. 16.4 ± 6.8°, respectively. The complication rate was 15.4%, predominantly affecting patients under 5 years old.
CONCLUSIONS: The SUPERhip procedure is an effective and reproducible technique for clinical and radiographic correction to a significant degree in patients with Paley\'s type 1b CFD, in preparation for bone lengthening surgery.

Congenital femoral deficiency (CFD) is often associated with cruciate ligament deficiency. The lateral scanogram may be a potential solution to some limitations for detecting instability associated with cruciate ligament deficiency. This qualitative case study identified two children with congenital femoral deficiency who were assessed with a lateral scanogram and had their results correlated to the clinical examination and MRI. Both cases identified a child with congenital femoral deficiency, one with a total leg length discrepancy (LLD) of 12 cm and the next with 6.5 cm. The weight-bearing lateral scanogram revealed anterior tibial translation, indicating knee instability. Both patients will undergo anterior cruciate ligament (ACL) reconstruction prior to limb lengthening. The lateral scanogram is a useful imaging modality that is capable of detecting anterior tibial translation, and thereby knee instability, in children with congenital femoral deficiency. Larger studies utilizing and evaluating the benefits of lateral scanograms are warranted.

Rotationplasty is a reconstructive option for severe congenital femoral deficiency (CFD). The senior author (D.P.) developed five new rotationplasty techniques for use in CFD based on the Paley classification, including the Paley-Brown (fusion femur to pelvis), Paley (fusion femur to femoral head), Paley-Winkelman (insertion tibial condyle to acetabulum), PaleySUPERhip-Van Nes (hip osteotomy with knee fusion) and PaleySling-Van Nes (hip reconstruction with knee fusion revision) rotationplasty techniques. The purpose of this study is to retrospectively evaluate the complications, radiographic outcomes and need for secondary surgery in 19 rotationplasty cases performed by the senior author (D.P.) for severe CFD from 2009 to 2019. Rotationplasty comprised only 2% of the authors treated CFD cases during this period. Average age at surgery was 8.6 years old. Average follow-up was 3.3 years. Sixteen concomitant procedures were performed including temporary arthrodesis, tibial osteotomy and SUPERhip procedure. The most common complication was wound necrosis/dehiscence, which occurred in 52% of the cases related to the circumferential incision and required a total of 31 additional debridements. Additional complications were successfully treated and included sciatic nerve palsy decompressed by abducting the femur, a tibial delayed union that underwent bone grafting, two distal femur failed epiphysiodesis treated by revision with one osteotomy and a thigh compartment syndrome requiring debridement. Indication specific rotationplasty successfully addresses the severe degree of femoral deficiency, deformity, and discrepancy in patients with CFD, despite high rates of wound complications.

UNASSIGNED: The primary objective of this study was to evaluate the quality of life following limb lengthening surgery in patients with achondroplasia. The complications and different lengthening techniques have and effects on mid-term results were also investigated.
UNASSIGNED: We performed a retrospective, multicenter study by evaluating the records of patients with achondroplasia operated in our clinic between 1999 and 2014 for limb lengthening with a minimum follow-up of 3 years. Forty nine patients were underwent bilateral lower limb lengthening surgery and 21 of 49 patients underwent bilateral humerus lengthening surgery. Patients were evaluated by the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales to assess the health-related quality of life (HRQOL) of children. Twenty patients with achondroplasia who had no lengthening surgery history were also evaluated with the PedsQL score as the control group.
UNASSIGNED: The average age at the time of first surgery was 6.17 years. The average follow-up period was 100.2 months. The average age at the time of study 14.70 ± 2.44 (11-18) years. There were significant differences between the patients with humeral lengthening and patients who did not undergo humeral lengthening in all scores. Transient complications had minimal effects on scores. Although all scores in the operated group were higher than non-operated patients with achondroplasia, there were no significant differences.
UNASSIGNED: Quality of life was significantly improved as a result of humerus lengthening surgery of patients with achondroplasia, despite minor complications compared with Lower limb lengthening surgery.
UNASSIGNED: Level III, case control study.

Congenital femoral deficiency (CFD) is one of the most challenging and complex conditions for limb lengthening. We focused on the problem of hip instability during femoral lengthening because subluxation and dislocation are potentially catastrophic for hip function.
We assessed for hip stability in 69 children (91 femoral lengthenings) who had CFD Paley type 1a (43 children) and 1b (26 children). The mean age at first lengthening was 6.4 years.
Hip subluxation/dislocation occurred during 14 (15 %) of 91 lengthenings. Thirty-three pelvic osteotomies were performed before lengthening in an attempt to stabilize hips. Thirteen patients (type 1a, eight; type 1b, five) had acetabular dysplasia at initiation of lengthening. One of the eight with type 1a experienced mild femoral head subluxation; four of the five with type 1b experienced three dislocations and one subluxation. Eight patients (type 1b) experienced hip instability although they had pelvic osteotomies. Proximal femoral lengthening was a significant factor for hip subluxation. Patients with hip subluxation more likely underwent monolateral fixation and the original superhip procedure. Age ±six years was not a contributing factor for hip instability.
Important risk factors for hip instability during femoral lengthening are severity of CFD, residual acetabular dysplasia, and proximal femoral lengthening. We recommend routine performance of pelvic osteotomy for patients with Paley type 1b CFD and distal lengthening.
Therapeutic Level IV.

The PRECICE(®) Intramedullary Limb Lengthening System (Ellipse Technologies Inc., CA, USA) is a remotely controlled, magnetically driven, implantable limb lengthening intramedullary nail system. It has both CE mark and US FDA clearance for its first- (2011) and second-generation (2013) implants. It is indicated for the treatment of limb length discrepancy and short stature. It has been used worldwide in over 1000 cases. Its reported and published results in over 250 cases has been excellent with less pain and lower complication rates than with external fixation methods or previous implantable nail systems.

Abnormalities that affect the developing human limb range from subtle differences that are of no functional or cosmetic consequence, to complete absence of all limbs. Advances in non-invasive imaging have improved antenatal detection of these conditions and has broadened the options available to prospective parents. This paper considers congenital femoral deficiency and includes an overview of limb bud development and the mechanisms involved in normal growth. The use of ultrasound in antenatal screening and the clinical and radiological features in childhood will be discussed in addition to management including surgical reconstruction and prosthetic use. This should be one component of a multidisciplinary approach to provide the parents of an affected child with balanced information on functional prognosis, management choices and implications for future pregnancies.