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Rotationplasty for Severe Congenital Femoral Deficiency

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Rotationplasty for Severe Congenital Femoral Deficiency children Article Rotationplasty for Severe Congenital Femoral Deficiency Corey B. Fuller 1 , Craig H. Lichtblau 2 and Dror Paley 2,* 1 Department of Orthopedic Surgery, Loma Linda University, Loma Linda, CA 92354, USA; [email protected] 2 Paley Orthopedic and Spine Institute, St. Mary’s Hospital, West Palm Beach, FL 33407, USA; [email protected] * Correspondence: [email protected] Abstract: 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 suc- cessfully 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 Citation: Fuller, C.B.; Lichtblau, C.H.; revision with one osteotomy and a thigh compartment syndrome requiring debridement. Indication Paley, D. Rotationplasty for Severe specific rotationplasty successfully addresses the severe degree of femoral deficiency, deformity, and Congenital Femoral Deficiency. discrepancy in patients with CFD, despite high rates of wound complications. Children 2021, 8, 462. https:// doi.org/10.3390/children8060462 Keywords: rotationplasty; congenital femoral deficiency; deformity Academic Editor: Vito Pavone Received: 27 April 2021 1. Introduction Accepted: 26 May 2021 Congenital femoral deficiency (CFD) is a rare congenital disorder with a reported inci- Published: 1 June 2021 dence of 1:50,000 births [1–3]. It presents with a wide spectrum of severity from mild femoral hypoplasia to severe femoral deficiency with deformity and length discrepancy. The natural Publisher’s Note: MDPI stays neutral history of CFD is a progressive limb length difference, but the deformities and contractures with regard to jurisdictional claims in persist but do not progress [4]. Surgical management of CFD can be challenging and options published maps and institutional affil- include reconstruction with lengthening with reconstructable hip and knee joints versus iations. amputation or rotationplasty with prosthesis for more severe cases [5–8]. Rotationplasty (RP) is an old concept that has been used for congenital and acquired lower extremity bone loss. It is a limb-sparing procedure that rotates the involved extremity 180 degrees so that the ankle functions like a knee joint and can be fitted with a below-knee Copyright: © 2021 by the authors. type prosthesis onto the foot that functions as the tibia. Rotationplasty is a well-recognized Licensee MDPI, Basel, Switzerland. reconstructive option of treatment for congenital femoral deficiency (CFD) and limb salvage This article is an open access article for lower extremity sarcomas [9–14]. distributed under the terms and Rotationplasty was originally described for treatment of a patient with bone loss from conditions of the Creative Commons tuberculosis in 1927 [15] and was popularized by Van Nes for treatment of CFD in 1950 [16]. Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ This type of rotationplasty also converts the ankle to a knee, while the original knee is 4.0/). fused straight, and the hip is left free floating relative to the pelvis. Krajbich popularized Children 2021, 8, 462. https://doi.org/10.3390/children8060462 https://www.mdpi.com/journal/children Children 2021, 8, x FOR PEER REVIEW 2 of 24 Children 2021, 8, 462 2 of 23 (http://creativecommons.org/licenses knee is fused straight, and the hip is left free floating relative to the pelvis. Krajbich /by/4.0/). popularized this method for use in CFD and performed it through a long lateral S-shaped thisincision method [17] for. Since use inthe CFD proximal and performed end was not it through anchored, a long it had lateral a tendency S-shaped to incisionderotate, [17 ]. Sinceundoing the the proximal benefit endof the was procedure. not anchored, Brown described it had a tendencya new type to of derotate, rotationplasty undoing (first the benefitpresented of the in Dallas procedure. in 1996 Brown at a meeting described on CFD, a new then type published of rotationplasty in 1998 as (firsta book presented chapter in Dallasin an AAOS in 1996 monograph at a meeting from on CFD,that meeting then published [18], finally in 1998publishing as a book his case chapter series in in an 2001 AAOS monograph[19]) by using from a circumferential that meeting [ 18incision], finally and publishing fusing the hisdistal case femoral series remnant in 2001 [to19 ])the by side using aof circumferential the ilium, thus incisionalso converting and fusing the knee the distaljoint to femoral a stable remnant hinge like to hip. the Anchoring side of the the ilium, thusfemur also to convertingthe pelvis eliminated the knee joint the toproblem a stable of hinge the leg like derotating. hip. Anchoring The lower the femurlimb was to the pelvisfrequently eliminated fused to the the problem ilium in of valgus the leg due derotating. to the inclination The lower of limbthe lateral was frequently ilium. It also fused toprotruded the ilium laterally in valgus a duelot since to the the inclination new hip of joint the was lateral laterally ilium. located It also protruded comparedlaterally to the a lotnormal since hip the joint. new hip joint was laterally located compared to the normal hip joint. PaleyPaley modifiedmodified thethe BrownBrown techniquetechnique in 1997 by by fusing fusing the the distal distal femoral femoral remnant remnant to to a Chiaria Chiari pelvic pelvic osteotomy osteotomy (Paley–Brown (Paley–Brown technique) technique) instead instead of theof lateralthe lateral ilium. ilium. The The advan- tageadva ofntage fusing of tofusing a Chiari to osteotomya Chiari osteotomy instead of instead the side of of the pelvisside of is the medializationpelvis is the of themedialization hip joint to of improve the hip hipjoint mechanics to improve and hip cosmesis. mechanics The and Paley–Brown cosmesis. The modification Paley–Brown also makesmodification it easier also to achieve makes it the easier correct to achieve frontal the plane correct alignment. frontal plane The fusion alignment. of cancellous The fusio bonen of cancellous bone of the distal femoral remnant with cancellous bone of the ilium at the of the distal femoral remnant with cancellous bone of the ilium at the Chiari osteotomy in Chiari osteotomy in an axially loaded transverse osteosynthesis line also increases fusion an axially loaded transverse osteosynthesis line also increases fusion potential compared potential compared to the vertically sheared Brown fusion line. Paley types 2 and 3 CFD to the vertically sheared Brown fusion line. Paley types 2 and 3 CFD (Figure1) are the best (Figure 1) are the best indications for the Brown (Figure 2a–f) and Paley–Brown (Figure indications for the Brown (Figure2a–f) and Paley–Brown (Figure3a–d) rotationplasties. 3a–d) rotationplasties. Figure 1. Paley CFD classification. Figure 1. Paley CFD classification. Children 2021, 8, 462 3 of 23 Children 2021, 8, x FOR PEER REVIEW 3 of 24 (a) (b) (c) (d) Figure 2. Conts. Children 2021, 8, 462 4 of 23 Children 2021, 8, x FOR PEER REVIEW 4 of 24 (e) (f) FigureFigure 2. (a )2. Illustration(a) Illustration of of CFD CFD Paley Paley type 3 3 (a (a oror b),b (),b) ( bIllustration) Illustration after afterBrown Brown rotationplasty rotationplasty for Paley type 3 (a or b), (c) AP pelvis x-ray of 14-year-old boy with Paley type 3b CFD, (d) Preop for Paleystanding type erect 3 (a legor bx),-ray (c )of AP same pelvis boy, x-rayshowing of 14-year-oldthat the ankle boy is at withthe level Paley of the type opposite 3b CFD, knee (d ) Preop standing(incidentally erect leg this x-ray patient of samealso has boy, multiple showing osteochondromas), that the ankle (e is) AP at pelvis the level radiograph of the oppositein same knee (incidentallyboy, 7 years this after patient healed also Brown has multiple rotationplasty, osteochondromas), (f) Sanding radiograph (e) AP pelvis with prosthetic radiograph 7 years in same boy, 7 yearsafter after Brown healed rotationplasty Brown rotationplasty, with supramalleolar (f) Sanding osteotomy radiograph for ankle with realignment. prosthetic Clinically 7 years after he has Brown excellent gait and function. rotationplasty with supramalleolar osteotomy for ankle realignment. Clinically he has excellent gait and function. In a subset of Paley type 3a or 3b or in Paley type 2a CFD patients, there is a mobile femoral head in the acetabulum. This can be determined before surgery using MRI. In these cases, the distal femoral remnant can be fused directly to the femoral head (Paley rotationplasty) (Figure4a–e). Paley started using this method for selective cases since 2012. This creates a more 3-dimensional hip motion flexing and extending through the rotated knee joint and abducting-adducting and axially rotating through the femuro-acetabular joint. Neither the Brown, Paley–Brown, nor Paley types of RP are indicated if there is complete absence or ankylosis of the knee joint with an absent femur (Paley type 3c).
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