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Resection, Rotationplasty, and Femoropelvic Arthrodesis in Severe Congenital Femoral Deficiency A Report of the Surgical Technique and Three Cases
Kenneth L.B. Brown, MD, MSc, FRCSC
View Disclosures and Other Information
Investigation performed at British Columbia's Children's Hospital, Vancouver, British Columbia, Canada
Kenneth L.B. Brown, MD, MSc, FRCSC British Columbia's Children's Hospital, Room A207, 4480 Oak Street, Vancouver, BC V6H 3V4, Canada. E-mail address: kbrown@interchange.ubc.ca
No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

The Journal of Bone & Joint Surgery.  2001; 83:78-78 
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Abstract

Background: The major problems associated with severe congenital deficiency of the femur are an unstable hip joint and a femur that is more than 50% shorter than the contralateral, normal femur. The usual treatment of these extreme cases of congenital femoral deficiency is a Syme or Boyd amputation when the child begins to walk. A knee fusion is done when the child is older, and the patient functions as an above-the-knee amputee. Rotationplasty has been described as an alternative treatment that allows the patient to function as a below-the-knee amputee. None of the currently described types of rotationplasty address the problem of the unstable hip.

Methods: Three patients with severe congenital femoral deficiency underwent a unique single-stage reconstruction. In this procedure, the limb is completely detached except for the sciatic nerve and the femoral vessels. The proximal part of the dysplastic femur and some muscles are resected. The residual limb is externally rotated 180° and the rotated distal part of the femur is fused to the pelvis. All of the muscles distal to the knee remain undisturbed.

Results: The anatomical knee in its rotated position functioned as a hip with flexion and extension, and the femoropelvic arthrodesis provided a more stable support. The rotated ankle acted as a knee, and the patients functioned as below-the-knee amputees. The duration of follow-up of these three patients was eight, six, and four years. Active hip flexion was 10° to 70°, 10° to 90°, and 0° to 80°, and active knee flexion was 90° and 95°. The patients' gait continued to improve as they matured.

Conclusions: The femoropelvic arthrodesis provides a stable hip. Since the muscles distal to the knee are not disturbed, the problem of derotation of the limb, which is often seen following the Van Nes rotationplasty, is not seen after this operation.

Figures in this Article
    Patients with severe congenital femoral deficiency have flexion and external rotation contractures of the limb, femoral shortening of more than 50% compared with the normal side, and instability of the knee and hip. The proximal thigh segment is shaped like a ship's funnel, and the hip joint is seen to be absent on radiographs. Classification of congenital femoral deficiencies with use of radiographs may be difficult since the appearance can change as the child grows and the proximal part of the femur becomes more ossified1,2. Severe congenital femoral deficiency with a deficient hip joint and marked limb-shortening is usually treated with a Boyd or Syme amputation followed by fitting with an above-the-knee prosthesis. However, the short bulbous shape of the thigh and the hip and knee contractures make prosthetic fitting difficult. The child often undergoes a knee arthrodesis to eliminate the hip and knee contractures, making the limb more vertical and narrowing the width of the perineum during stance, which improves prosthetic fitting3. Occasionally, epiphysiodesis of the residual limb may be necessary to shorten it sufficiently to accommodate a prosthetic knee. When the child reaches skeletal maturity, he or she functions as an above-the-knee amputee. Patients with severe femoral deficiency have a Trendelenburg lurch due to the instability of the proximal part of the femur4.
    Rotationplasty as described by Van Nes5 is an alternative reconstruction in which the limb is rotated 180° through a tibial osteotomy so that the ankle functions as a knee joint and the foot serves as a short below-the-knee limb segment, allowing the patient to function as a below-the-knee amputee. It is often difficult to achieve full rotation during one operation, and there is a high prevalence of derotation when the procedure is done in children younger than six years of age. To overcome some of these limitations of the Van Nes rotationplasty and to improve hip stability, a new form of rotationplasty for congenital femoral defects was developed. In this procedure, the limb rotation is performed through the residual thigh segment after excision of part of the abnormal femur and some of the thigh musculature. The distal part of the femur is fused to the ilium after the limb is rotated so the foot points backward. Thereafter the flexion-extension arc of the anatomical knee provides hip flexion and the rotated ankle functions as a knee joint, similar to the case with a Van Nes rotationplasty. The anatomy distal to the knee is not disturbed in this procedure, as the entire rotational repositioning of the limb is performed through the proximal femoral segment of the limb. This procedure provides a more stable hip so the lurch associated with severe forms of congenital femoral deficiency is eliminated. Prerequisites include a relatively normal knee, ankle, and foot.
    Three patients treated with the procedure since 1989 had sufficient follow-up to allow adequate clinical assessment of the functional result.
     
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    +Fig. 1:Diagram illustrating severe congenital femoral deficiency and an abnormal proximal part of the femur in a child. The dotted lines indicate the incisions for the rotationplasty.
     
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    +Fig. 2:Intraoperative diagram illustrating the removed proximal part of the femur with its hypoplastic head as well as the surrounding thigh muscles.
     
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    +Fig. 3-A:Diagram showing attachment of the residual femur to the pelvis with staples crossing the growth plate. The fibula is now on the medial aspect of the limb following rotation.
     
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    +Fig. 3-B:Diagram illustrating an alternate method of femoral attachment through a window in the pelvis. Part of the resected proximal part of the femur is inserted as a bone graft to bridge the gap between the femur and the pelvis.
     
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    +Fig. 3-C:Diagram of the limb following surgery, with the limb rotated and the residual femur attached to the pelvis.
     
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    +Fig. 4-A:Figs. 4-A, 4-B, and 4-C: Case 1, a child with Pierre Robin syndrome.
    Fig. 4-A: Preoperative radiograph, made at the age of two years, showing a deficient acetabulum and no visible femoral head. There is no ossification of the contralateral femoral head.
     
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    +Fig. 4-B:Clinical photograph of the patient wearing the prosthesis six years following surgery.
     
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    +Fig. 4-C:Weight-bearing radiograph of the rotated limb six years following surgery, showing a solid iliofemoral arthrodesis and growth arrest of the distal part of the femur. The anatomical knee is just distal to the level of the acetabulum.
    The pelvis and both lower extremities should be prepared and draped to facilitate orientation of the rotated leg. The locations of the posterior tibial and dorsalis pedis pulses are found with use of an ultrasonic Doppler flow detector (model 811-AL; Parkes Medical Electronics, Aloha, Oregon) and are marked on the skin with indelible ink. A circumferential incision is made just proximal to the skin crease in the popliteal fossa of the affected limb (Fig. 1Fig. 1). A longitudinal extension perpendicular to this incision is made anterolaterally between the crest of the ilium and the greater trochanter. This provides access to the proximal part of the femur and the pelvis. If required, the incision may be extended down the anterior border of the tibia to improve knee-joint exposure, which will aid in the attachment of the limb to the pelvis following rotation. It is important to elevate the superficial fascia of the thigh along with the skin flaps to help to preserve their blood supply. The external table of the ilium is exposed by subperiosteal dissection. The femoral vessels are located at the brim of the pelvis and are dissected to the level of the knee. Side branches and profunda femoris vessels are ligated and cut. Cutting these branches helps to prevent tethering of the vascular bundle during rotation. The gastrocnemius origins are detached from the femur, with care being taken to protect their neurovascular bundles that enter the proximal part of the muscle. The femoral nerve supply to the proximal part of the quadriceps muscles is preserved, and the quadriceps muscles are cut proximal to the rectus femoris tendon. Most of the vastus lateralis and vastus medialis and all of the adductors, from origin to insertion, and the distal hamstrings are removed. The nerve supply to the hamstring muscles usually enters quite proximally, and their innervation can be easily preserved. The sciatic nerve is freed from the surrounding tissues and protected. It is isolated by first exposing and mobilizing the peroneal nerve beneath the biceps tendon near the neck of the fibula. The peroneal nerve is then followed proximally to the origin of the tibial nerve.
    If there is a fixed flexion contracture of the knee, a posterior capsulotomy is performed. In some cases, the cruciate ligaments must be released to achieve full knee extension. The joint capsule is left open. The distal femoral growth plate is identified, and epiphysiodesis is attempted by curetting the physis and stapling across it. Fluoroscopy helps to identify the physis and the placement of the staples.
    At this point, the limb is attached only by the femoral artery and vein and the sciatic nerve (Fig. 2Fig. 2). The limb is then externally rotated and moved proximally to where the acetabulum is normally located. The femur is attached to the pelvis in a position of neutral rotation and no abduction-adduction of the limb, with the knee in full extension. Arterial pulses in the foot should be checked with a sterile ultrasonic Doppler flow detector before and after rotation to be sure that blood flow is preserved and the vessels are not kinked. If the pulse disappears during rotation, the limb should be derotated and the vessels should be checked for kinking. If the thigh muscles are too bulky after rotation, more muscle has to be removed or the pelvic bone must be notched to provide a more direct line for the vessels as they exit the pelvis. In most cases, the thigh segment is already short so the vessels are not too redundant and additional shortening and anastomoses should not be necessary. The repositioned residual limb is perfused mainly by the femoral artery and vein, and these vessels must not be obstructed or kinked by the muscle reattachments. Following external rotation of the limb, the femoral vessels and the sciatic nerve lie anterior to the limb. Usually only a little more than 100° of rotation is required since the affected limb is already in marked external rotation in most patients with severe congenital femoral deficiency.
    The knee should be brought as close as possible to the normal site of the acetabulum without impairing the circulation to the limb. The femur is approximated temporarily to the pelvis by Kirschner wires. An intraoperative radiograph is obtained to check rotation and abduction-adduction of the limb. It is important to look at the plane of the distal part of the femur to be sure that it is in a neutral position. Radiographically, the plane of the distal femoral joint surface should be parallel to a line connecting the bottoms of the two ischia. The overall alignment of the rotated limb may spuriously appear to be proper as a result of mediolateral laxity of the knee even when the femur is actually malpositioned in abduction or adduction. Determination of the correct position is facilitated by including both lower limbs and the entire pelvis in the operative field. The limb is positioned with the knee in full extension. After attachment to the ilium, the part of the femur that is proximal to the site of the femoropelvic fixation is removed. The surfaces of the ilium and femur are not usually well coapted; therefore, supplemental bone graft should be inserted between them. A piece of the cortical bone from the resected femur can be turned on its side and shaped to serve this purpose. Fixation is achieved with AO screws and bolts (Fig. 3-AFig. 3-A) or with Kirschner wires. In patients with a longer proximal femoral segment, the rotated limb can be further stabilized by inserting the rotated femur into an oval-shaped opening in the ilium (Figs. 3-BFigs. 3-B and 3-C3-C). Excision of the redundant proximal part of the femur should only be done following stable fixation of the femur to the pelvis.
    Following bone fixation, the muscles of the leg are attached to the remaining stumps of the resected thigh muscles. The tendons of the gastrocnemius muscles are attached to the stump of the iliopsoas muscle and the proximal part of the quadriceps muscles. The hamstring muscles are attached to the anterior tibial and peroneal muscles of the leg and the quadriceps tendon of the rotated knee. Redundant thigh musculature is removed, if necessary, to prevent obstruction of the rotated femoral vessels. None of the thigh musculature is required for the ankle to function optimally as a knee joint because all of the motor power comes from the leg muscles, which are undisturbed by this procedure. Attachment of the quadriceps and hamstring muscles to the rotated leg should allow active flexion and extension of the anatomical knee, which serves functionally as a hip. A drain is left in place, and the subcutaneous tissues and skin are closed. The rotated limb is incorporated into a hip spica cast with the foot and ankle exposed.
    When the patient awakens from anesthesia, he or she can usually move the ankle and toes. Arterial pulses in the foot should be monitored frequently during the first two days to be sure that the circulation does not become obstructed by postoperative swelling. An ultrasonic Doppler flow detector is useful for this purpose. It is important to be aware that there is no collateral circulation to the lower limb following this procedure. Critical danger signs of impaired circulation include the loss or a change in the character of pulses detected with the Doppler flow detector, loss of prior movement, and decreased capillary flow. These signs of vascular compromise should be treated first by removal of the upper half of the hip spica and dressings. If these measures do not improve the circulation to the foot, prompt surgical exploration of the major vessels is required.
    Immediate ankle motion is encouraged. The spica cast is removed in six to eight weeks, and a prosthesis is fabricated. If the femoropelvic fusion is delayed or tenuous, the first prosthesis should include a pelvic extension to allow protected knee flexion and extension.
    Case 1. A child with Pierre Robin syndrome and severe congenital femoral deficiency (Fig. 4-AFig. 4-A) began to walk at sixteen months of age with an extension-type prosthesis. Preoperatively, the lower extremity had a resting position of 60° of external rotation, with an additional 30° of passive external rotation possible. She had a fixed knee flexion contracture of 50° and laxity of the knee in the anteroposterior and mediolateral planes. Proximal femoral resection rotationplasty was performed in July 1989, when she was two and one-half years old. As the ilium was exposed, a small femoral head was found in a tiny acetabulum. During the operative procedure, the femoral vessels became kinked because of an inadequate muscle resection, resulting in severe arterial spasm. Several hours after surgery, the patient was taken back to the operating room for removal of a thrombosis in the artery. Wound-healing was impaired, and débridement and split-thickness skin-grafting was required two weeks after the initial procedure. The rest of the postoperative course was uneventful, although the patient was not able to move the foot and ankle for several weeks after the procedure. One year after surgery, the femoropelvic fusion was solid, but a progressive varus deformity was developing at the distal part of the femur as a result of only a partial epiphysiodesis. She also had a 15° equinus contracture of the anatomical ankle joint. The patient underwent a wedge osteotomy of the femur through the residual physis to correct the angular deformity and a percutaneous Achilles tendon lengthening.
    When last seen, eight years after the rotationplasty, the patient walked without an abductor lurch and had active knee motion during the gait cycle. The heel was slightly externally rotated (Fig. 4-BFig. 4-B), and the forefoot was in slight varus. The anatomical ankle, functioning as the knee, had a range of motion from full plantar flexion to 5° of dorsiflexion. Much of this movement took place in the forefoot, with less excursion of the hindfoot than of the forefoot. The strength of dorsiflexion and plantar flexion was grade 4 of 5. The sensation of the extremity was normal. At the hip, there was a 10° flexion contracture of the anatomical knee, with additional active flexion to 70°. The patient still had mediolateral laxity of the knee, which was unchanged. Radiographs revealed a solid fusion of the femur to the pelvis (Fig. 4-CFig. 4-C).
    Case 2. A four-year-old girl presented with a congenital femoral deficiency of the left lower limb. She had begun walking at fifteen months of age. Preoperative evaluation showed a shortened externally rotated thigh segment with a 60° knee flexion contracture. The foot ended at the proximal calf level of the normal limb. In 1991, the patient underwent an excision of the proximal part of the femur, rotationplasty, and arthrodesis of the distal part of the femur to the pelvis with an epiphysiodesis. At surgery, a hypoplastic femoral head was found in a small acetabulum. There were no perioperative complications but, after the drapes were removed, the extremity was found to be attached in a slightly internally rotated position. (This prompted us to prepare both limbs and the pelvis when subsequent patients were managed with this procedure.) Two years later, a varus deformity of the rotated limb was developing as a result of an incomplete growth arrest of the distal part of the femur. The epiphysiodesis was revised, and the limb was rotated externally through the physis. The limb was stabilized by an Ilizarov external fixator, which remained on for two months. Follow-up six years after the rotationplasty revealed a normal axial alignment of the limb. There was a fixed flexion deformity of the anatomical knee of 10°, with additional active flexion to 90°. There was no instability of the anatomical knee. The anatomical ankle, functioning as a knee, had a range of motion of 0° to 95°. The patient regularly used a prosthesis, and her gait had been continuously improving.
    Case 3. A three-year-old girl had a short limb with the heel at the level of the contralateral knee. The limb was externally rotated, and there was a 45° knee flexion contracture with mediolateral laxity. Radiographs showed an absent acetabulum and proximal part of the femur, but during surgery a small femoral head and ligamentum teres were found in a small acetabulum. After the operation, superficial necrosis of the wound developed; it healed in two weeks. The hip spica cast was removed seven weeks after the rotationplasty.
    Follow-up examination when the patient was four years of age revealed that the distal part of the femur had continued to grow, causing the anatomical knee to migrate distally from its original postoperative position at the level of the hip. An open epiphysiodesis was performed eighteen months after the rotationplasty, but the epiphysiodesis had to be repeated two and one-half years later. Follow-up at the age of seven years revealed a slightly internally rotated position of the lower limb. The femur was firmly attached to the pelvis, with good alignment of the leg. Active flexion of the anatomical knee, functioning as a hip, was 80° with additional passive flexion possible to 100°. With the prosthesis on, the patient had 0° to 90° of active ankle motion (functioning as knee motion), with an extension lag of 5°. She had a slight toe-toe gait because she was not utilizing the full flexion and extension capacity of the rotated ankle yet, but the hip was stable and she walked without a Trendelenburg lurch.
    Borggreve6 was the first to describe rotationplasty, to our knowledge. In 1927, he resected part of the femur of a twelve-year-old girl who had undergone multiple operations for tuberculosis infection. The residual limb was shortened through the femur and rotated 180° before reattachment. The ankle became the knee joint, and the patient functioned as a below-the-knee amputee. More recently, this type of rotationplasty has been utilized for limb reconstruction in patients following resection of malignant tumors of the distal part of the femur7-12, proximal part of the tibia13,14, proximal part of the femur15, and ilium16. The functional benefits are becoming recognized, especially in very young children17 and for the salvage of failed tumor endoprostheses7.
    Van Nes5 utilized rotationplasty for patients with congenital femoral deficiency; he performed the procedure either through a femoral pseudarthrosis or through the tibia in conjunction with a knee arthrodesis. Failures were due to progressive tibial derotation or to poor ankle strength and range of motion. Derotation usually occurred when the patients underwent Van Nes tibial rotationplasty before six to eight years of age18-20. Rotation of the limb through the tibia produces extreme tibial torsion, and often the tibia cannot be completely rotated in one stage because of vascular compromise21. Torode and Gillespie22 reported the results of Van Nes rotationplasty performed at the time of knee arthrodesis. In their technique, the leg muscles no longer spiraled around the tibia, resulting in fewer problems with derotation, but this procedure did not address the problem of an unstable hip.
    Steel et al.23 described a technique that they used in four patients with severe congenital femoral deficiency. The distal part of the femur was fused to the pelvis in 90° of flexion without rotation. The iliofemoral arthrodesis was combined with the Van Nes tibial rotationplasty in two patients, but gradually the tibiae derotated about 45°. The poor ambulatory function of these patients was attributed to the malrotation since the foot pointed to the side and the plane of flexion and extension was such that the rotated ankle could not function as a knee.
    Several authors have made reference to the cosmetic aspects of rotationplasty23,24 and the fact that patients have requested an ankle disarticulation because of the cosmetic appearance23,25. Some authors have suggested toe amputation to improve the cosmetic appearance, but experience has shown that this has a very deleterious effect on function and it is not recommended7,19,26. Kotz and Salzer10 stated that very often resistance to the idea of rotationplasty comes from physicians with little firsthand experience with the procedure. When patients who have had a rotationplasty are dressed and wearing the prosthesis, their appearance is quite normal.
    A functional rotationplasty with an ankle and foot acting as a short below-the-knee residual limb provides a patient with substantial functional advantages over an above-the-knee ampuation, which is the effect of a Syme or Boyd amputation. Patients who have had a rotationplasty are able to walk up and down stairs in the usual way. When they sit, the ankle, functioning as a knee, bends in a more natural way. Activities such as walking quickly, skiing, and bicycling are facilitated. Patients who have had a rotationplasty walk faster and have less oxygen consumption than those who have had an above-the-knee amputation27. One of the main disadvantages of above-the-knee amputation is that the patients have difficulty in making the mechanical knee joint change cadence and stride length during different activities28. All of the gait studies of which we are aware have included a small number of patients, and comparisons often have been made between adults and children7,24,29-32. It is known from analyses of the normal gait of children that they walk at a slower velocity than adults. The results of gait analysis of patients with congenital femoral deficiency cannot be compared with those of patients who have had a rotationplasty following tumor excision since patients with congenital femoral deficiency have substantial hip instability, which further increases energy expenditure and slows down gait velocity.
    An advantage of femoral resection rotationplasty with femoropelvic arthrodesis is that one major procedure is done when the child is young and the foot and ankle have a normal excursion. Thus, treatment can be completed and the patient can be a stable prosthesis wearer by school age. This procedure has some advantages over other types of rotationplasty because a stable hip is created and the leg does not derotate because of the absence of the abnormal pull of the muscles spiraled around the tibia. Hip arthrodesis improves gait by stabilizing the proximal part of the femur and narrowing the perineum, improving prosthetic fitting. In our patients, the repositioned knee acting as a hip improved gait by eliminating pistoning of the femur. Attention must be paid to reattaching the residual proximal hamstrings to the tibialis anterior and peroneal muscles in order to maximize extension power at the hip.
    One of the problems of the procedure described here is the difficulty in achieving a growth arrest of the distal part of the femur in a young child, where tremendous forces are exerted by a healthy growth plate33. In addition to stapling, destruction of the growth cartilage of the distal part of the femur by a drill or curets is recommended. In our patients in whom the growth arrest was only partially successful, a progressive angular deformity developed. Correction, though relatively easy, required another operative procedure.
    This procedure is more technically demanding than the Van Nes rotationplasty and its various modifications. Extreme caution must be employed to avoid obstructing the blood flow or the limb can be lost. In all three patients described here, the femur fused to the pelvis. Fixation of the femur to the pelvis is difficult since there is no stable coaptation of the bones, and positioning of the femur at the time of the arthrodesis is technically challenging23. Thus, nonunion at the site of the femoropelvic arthrodesis may occur when this procedure is performed on more patients. Bone contact could be improved if a Chiari osteotomy is done, as suggested by King3.
    It is important to rotate the limb completely since the rotated anatomical knee is essentially a hinge joint that cannot compensate for any malrotation. This requires a circumferential incision and virtually complete detachment of the limb with excision of an adequate length of bone and muscles. Pirani et al.34 showed, with magnetic resonance imaging, that all of the thigh muscles are present in patients with congenital femoral deficiency and they are all contained within the shortened thigh, thus making the thigh very bulky. These muscles have no essential function following rotationplasty, and resection of a large part of the adductor and quadriceps muscles is necessary to prevent kinking of the vessels and circulatory compromise. In the first patient (Case 1), an inadequate resection of muscles resulted in kinking of the vessels at the brim of the pelvis and impairment of the circulation. The below-the-knee segment retains its normal anatomy and muscular relationships, thereby preserving maximal foot and ankle motion and strength.
    All patients in this series underwent a complete release of a preoperative knee flexion contracture. This release facilitated more accurate determination of the alignment of the extremity at the time of the femoropelvic arthrodesis, but it may lead to increased instability of an already abnormal knee. Follow-up of these patients was too short for us to ascertain the ultimate stability of the knee in its new position.
    It is probably best to perform this procedure when a child is between two and four years of age, when the child begins to walk independently. At this age, the vessels are of a larger caliber and the mobility of the foot and ankle is good. In older patients who have worn an extension prosthesis with the ankle in equinus, the ankle is often stiff. This may prevent the successful conversion of the patient's status to a below-the-knee amputee following rotationplasty. Patients with a stable hip and mild shortening of the femur may be better managed by lengthening procedures. Rotationplasty probably should not be performed in patients with poor ankle function and absence of several rays of the foot. This combination is more commonly encountered in patients with fibular hemimelia associated with congenital femoral deficiency. These patients are probably better managed with Syme or Boyd amputation and early prosthetic fitting. Rotationplasty is currently not recommended for patients with bilateral congenital femoral deficiency1,25.
    We reported the intermediate clinical follow-up results of a new reconstructive procedure in three patients with congenital femoral deficiency. The patients had not reached skeletal maturity by the time of follow-up, and their gait was continuing to improve with age. It is necessary to follow more patients to skeletal maturity to determine how this procedure will fit into the armamentarium of treatment of severe congenital femoral deficiencies. The potential advantages of this proximal femoral rotationplasty-arthrodesis are hip stability combined with maximization of function of the residual limb to a greater degree than is afforded by other methods. The procedure is demanding to perform, and careful attention to surgical technique is required to prevent irreversible limb ischemia.
    Note: The author thanks Dr. John Birch for his constructive review of this manuscript.
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    Fowler E; Zernicke R; Setoguchi Y; and Oppenheim W: Energy expenditure during walking by children who have proximal femoral focal deficiency. J Bone Joint Surg Am,1996.78: 1857-62, 781857  1996  [PubMed]
     
    Catani F; Capanna R; Benedetti MG; Battistini A; Leardini A; Cinque G; and Giannini S: Gait analysis in patients after Van Nes rotationplasty. Clin Orthop,1993.296: 270-7, 296270  1993  [PubMed]
     
    McClenaghan BA; Krajbich JI; Pirone AM; Koheil R,; and Longmuir P: Comparative assessment of gait after limb-salvage procedures. J Bone Joint Surg Am,1989.71: 1178-82, 711178  1989  [PubMed]
     
    Steenhoff JRM; Daanen HAM; and Taminiau AHM: Functional analysis of patients who have had a modified Van Nes rotationplasty. J Bone Joint Surg Am,1993.75: 1451-6, 751451  1993  [PubMed]
     
    van der Windt DA; Pieterson I; van der Eijken JW; Hollander AP; Dahmen R; and de Jong BA: Energy expenditure during walking in subjects with tibial rotationplasty, above-knee amputation, or hip disarticulation. Arch Phys Med Rehab,1992.73: 1174-80, 731174  1992 
     
    Murray MP; Jacobs PA; Gore DR; Gardner GM; and Mollinger LA: Functional performance after tibial rotationplasty. J Bone Joint Surg Am,1985.67: 392-9, 67392  1985  [PubMed]
     
    Safran MR; Eckardt JJ; Kabo JM; and Oppenheim WL: Continued growth of the proximal part of the tibia after prosthetic reconstruction of the skeletally immature knee. Estimation of the minimum growth force in vivo in humans. J Bone Joint Surg Am,1992.74: 1172-9, 741172  1992  [PubMed]
     
    Pirani S; Beauchamp RD; Li D; and Sawatzky B: Soft tissue anatomy of proximal femoral focal deficiency. J Pediatr Orthop,1991.11: 563-70, 11563  1991  [PubMed]
     

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    +Fig. 4-C:Weight-bearing radiograph of the rotated limb six years following surgery, showing a solid iliofemoral arthrodesis and growth arrest of the distal part of the femur. The anatomical knee is just distal to the level of the acetabulum.
    Anchor for JumpAnchor for Jump
    +Fig. 4-B:Clinical photograph of the patient wearing the prosthesis six years following surgery.
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    +Fig. 4-A:Figs. 4-A, 4-B, and 4-C: Case 1, a child with Pierre Robin syndrome.
    Fig. 4-A: Preoperative radiograph, made at the age of two years, showing a deficient acetabulum and no visible femoral head. There is no ossification of the contralateral femoral head.
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    +Fig. 3-C:Diagram of the limb following surgery, with the limb rotated and the residual femur attached to the pelvis.
    Anchor for JumpAnchor for Jump
    +Fig. 3-B:Diagram illustrating an alternate method of femoral attachment through a window in the pelvis. Part of the resected proximal part of the femur is inserted as a bone graft to bridge the gap between the femur and the pelvis.
    Anchor for JumpAnchor for Jump
    +Fig. 3-A:Diagram showing attachment of the residual femur to the pelvis with staples crossing the growth plate. The fibula is now on the medial aspect of the limb following rotation.
    Anchor for JumpAnchor for Jump
    +Fig. 2:Intraoperative diagram illustrating the removed proximal part of the femur with its hypoplastic head as well as the surrounding thigh muscles.
    Anchor for JumpAnchor for Jump
    +Fig. 1:Diagram illustrating severe congenital femoral deficiency and an abnormal proximal part of the femur in a child. The dotted lines indicate the incisions for the rotationplasty.
    Aitken GT. Proximal femoral focal deficiency-definition, classification, and management. In: Aitken GT, editor. Proximal Femoral Focal Deficiency. A Congenital Anomaly. Washington, DC: National Academy of Sciences; 1969. p 1-22 
     
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    Fowler E; Zernicke R; Setoguchi Y; and Oppenheim W: Energy expenditure during walking by children who have proximal femoral focal deficiency. J Bone Joint Surg Am,1996.78: 1857-62, 781857  1996  [PubMed]
     
    Catani F; Capanna R; Benedetti MG; Battistini A; Leardini A; Cinque G; and Giannini S: Gait analysis in patients after Van Nes rotationplasty. Clin Orthop,1993.296: 270-7, 296270  1993  [PubMed]
     
    McClenaghan BA; Krajbich JI; Pirone AM; Koheil R,; and Longmuir P: Comparative assessment of gait after limb-salvage procedures. J Bone Joint Surg Am,1989.71: 1178-82, 711178  1989  [PubMed]
     
    Steenhoff JRM; Daanen HAM; and Taminiau AHM: Functional analysis of patients who have had a modified Van Nes rotationplasty. J Bone Joint Surg Am,1993.75: 1451-6, 751451  1993  [PubMed]
     
    van der Windt DA; Pieterson I; van der Eijken JW; Hollander AP; Dahmen R; and de Jong BA: Energy expenditure during walking in subjects with tibial rotationplasty, above-knee amputation, or hip disarticulation. Arch Phys Med Rehab,1992.73: 1174-80, 731174  1992 
     
    Murray MP; Jacobs PA; Gore DR; Gardner GM; and Mollinger LA: Functional performance after tibial rotationplasty. J Bone Joint Surg Am,1985.67: 392-9, 67392  1985  [PubMed]
     
    Safran MR; Eckardt JJ; Kabo JM; and Oppenheim WL: Continued growth of the proximal part of the tibia after prosthetic reconstruction of the skeletally immature knee. Estimation of the minimum growth force in vivo in humans. J Bone Joint Surg Am,1992.74: 1172-9, 741172  1992  [PubMed]
     
    Pirani S; Beauchamp RD; Li D; and Sawatzky B: Soft tissue anatomy of proximal femoral focal deficiency. J Pediatr Orthop,1991.11: 563-70, 11563  1991  [PubMed]
     
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