Rotationplasty for Severe Congenital Femoral Deficiency

Fuller, Corey B.; Lichtblau, Craig; Paley, Dror

doi:10.3390/children8060462

Cited by 6 publications

(8 citation statements)

References 28 publications

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“…10,11 There are many complications associated with this technique including skin flap necrosis with an incidence of over 50% in former studies. [12][13][14] Unlike most patients with CFD, our patient had an almost normally functioning hip. Therefore, rotationplasty would have decreased the range of motion of the hip joint.…”

Section: Discussionmentioning

confidence: 72%

Distal Femoral Focal Deficiency

Soini¹,

Haara²,

Raitio³

et al. 2022

JAAOS Glob Res Rev

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Distal femoral focal deficiency is an extremely rare type of congenital femoral deficiency that comprises hypoplasia of the distal femur, with a normally developed hip. We represent a unique case of distal femoral hypoplasia and deficiency of knee extensors, childhood follow-up and final treatment with exarticulation, and a comparison with previous literature. Congenital femoral deficiency (CFD) is a rare congenital anomaly with a reported incidence of 1:50,000 births. [1][2][3] CFD consists all the femoral deficiencies from mild focal hypoplasia to severe deformity. 4 Most of the patients with CFD have proximal focal femoral deficiency (PFFD). Distal femoral focal deficiency (DFFD) is a type of deficiency that encompasses hypoplasia of the distal femur, with a normally developed hip. To the best of the authors' knowledge, it has been described in English literature in only three case reports previously. [5][6][7] Distal femoral focal deficiencies have been previously represented in Pappas and Paley classification schemes for congenital femoral deficiencies. 3,8 Distal deficiencies are classified as Pappas type IX and/or Paley type 4. Taylor et al. also proposed a separate classification scheme for DFFD, presenting types A, B, and C based on the Aitken 9 classification. Type A has an irregular distal femoral epiphysis with a bony connection between femoral components. The femur may be shortened. In type B, the distal epiphysis is present, but there is no osseous connection between the diaphysis and the distal epiphysis. In addition, the femoral length is shortened. In type C deficiency, the epiphysis is absent and the femoral segment is shortened (Figure 1). 7 We represent a case of DFFD and combined deficiency of anterior tight muscles and comparison with the previous reports.

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Section: Discussionmentioning

confidence: 72%

Distal Femoral Focal Deficiency

Soini¹,

Haara²,

Raitio³

et al. 2022

JAAOS Glob Res Rev

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“…The reported complication rate for Van Nes rotationplasty ranges from 25% to 52%, including wound complications, delayed union, deep infection, and vascular compromise resulting in amputation; however, these cited complication rates include data from series including oncology patients which may carry different risk profiles for complication [9][10][11][12] . It should also be noted that patients treated with rotationplasty for other lower extremity pathologies exhibit altered gait mechanics at adulthood; however, the ramifications of these gait deviations later in life remain incompletely characterized 8 .…”

Section: Discussionmentioning

confidence: 99%

Congenital Ankylosis of the Knee Treated with Van Nes Rotationplasty

Siebert,

Roach,

Stotts

2023

JBJS Case Connector

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Case: This is a case of a female patient born with thrombocytopenia-absent radius syndrome, with bilateral upper extremity phocomelia, bilateral hip dislocations, and congenital fusion of the right knee with progressively worsening flexion contracture. At age 3 years and 5 months, the patient was treated with excision of the knee ankylosis and Van Nes rotationplasty. This proved durable at age 20 years (final follow-up) without any need for further surgery and without complication. Conclusion: This is the first known report of Van Nes rotationplasty as a durable treatment option in the management of congenital knee ankylosis, which may avoid reoperation and eliminate risk of recurrence.

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“…Small published case studies of iliofemoral fusion conclude that a stable hip can be produced. [23][24][25] However, there are several significant complications reported including femoral artery thrombosis, varus deformity, or overgrowth due to continued growth of the femoral physis, wound necrosis, and dehiscence (occurring in 52% in a study of 19 patients 25 ). More information is therefore required, such as QoL analysis and gait analysis, to quantify the functional benefit and allow more confidence in recommending such complex procedures.…”

Section: Discussionmentioning

confidence: 99%

“…31 Radiographs were classified according to Paley. 9,10,15,25 The aim of this study was to see whether functional and QoL scores differed between different severities of PFFD and after surgical intervention. The outcome scores were compared according to Paley radiological classification, age at assessment (children under 18 vs. adult), and nonoperative vs. operative groups.…”

Section: Methodsmentioning

confidence: 99%

“…21,22 In cases of subtotal absence of the proximal femur, the question remains whether an iliofemoral fusion is beneficial. [23][24][25] This is an observational descriptive study evaluating prosthetic outcome in patients with severe congenital femoral deficiency. The aim was to provide a commentary on the potential benefits of surgical intervention, specifically looking at the influence on prosthetic fitting, gait, and impact on quality of life (QoL) for these patients with a severe lower limb longitudinal deficiency.…”

Section: Introductionmentioning

confidence: 99%

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Management of severe congenital femoral deficiency: Does surgical intervention enhance prosthetic function?

Calder,

Elsheikh,

Cross

et al. 2023

Prosthetics &Amp; Orthotics International

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Background & Objective: The purpose of this study was to evaluate prosthetic outcome in patients with severe congenital femoral deficiency and the potential benefits of surgical intervention on prosthetic fitting and gait. Methods: A retrospective review identified 26 active case records with a proximal femoral focal deficiency using a prosthesis. Validated outcome measures evaluated comfort, function, and prosthetic use and quality-of-life assessment. Outcome compared age groups and surgical intervention. Gait analysis performed in 7 patients further evaluated hip and knee function. Results: Eleven male patients and 15 female patients, including 13 children (mean age 10 years, range 5–16) and 13 adults (mean age 36 years, range 23–63) were evaluated. Better prosthetic function and PedsQL scores were recorded in the pediatric group. There was a trend for better scores after surgery. Gait analysis demonstrated reduced hip extension compensated by knee flexion in 3 patients, 2 patients had hip extension with near normal kinematics, 1 untreated patient walked with an unsteady gait, and the remaining walked well using an ischial-bearing prosthesis with pelvic compensatory movements. Conclusion: The management strategy in severe proximal femoral focal deficiency remains a major challenge. Hip reconstruction seems to improve functional scores. Overall, the scores seem to decline into adulthood but not significantly. Gait analysis before further surgical intervention is recommended because compensatory knee flexion may improve step length in terminal stance. Limited numbers, with short follow-up, prevents clear guidance on the benefit of surgery.

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Rotationplasty for Severe Congenital Femoral Deficiency

Cited by 6 publications

References 28 publications

Distal Femoral Focal Deficiency

Distal Femoral Focal Deficiency

Congenital Ankylosis of the Knee Treated with Van Nes Rotationplasty

Management of severe congenital femoral deficiency: Does surgical intervention enhance prosthetic function?

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