Surgical treatment for hip pain in the adult cerebral palsy patient

Transcription

1 DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY REVIEW Surgical treatment for hip pain in the adult cerebral palsy patient LEON ROOT MD Hospital for Special Surgery, New York, NY, USA. Correspondence to Leon Root at Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021, USA. LIST OF ABBREVIATIONS HSS Hospital for Special Surgery THA Total hip arthroplasty VRO Varus rotation osteotomy CONFLICTS OF INTEREST The author declares no conflicts of interest. Hip subluxation or dislocation in the cerebral palsy population is an acquired condition that can result in pain and limitation of function. The incidence is reported to be from 18 to 59%. Awareness of the factors that cause the problem are essential in order to prevent this condition. Early treatment consists of appropriate muscle lengthening or releases, varus rotation hip osteotomies and in some cases pelvic osteotomies to provide acetabular coverage for the femoral head. For painful hip subluxation or dislocation with arthrosis in the adolescent or adult salvage procedures such as hip arthrodesis, valgus osteotomy, proximal femoral resection, or total hip arthroplasty have all been done to relieve pain. The author recounts his experience of the surgical management of the hip in the individual with cerebral palsy. The ability to stand and walk or to sit comfortably in a wheelchair depends in large part on the stability and mobility of the hips. Hip subluxation in cerebral palsy (CP) is an acquired condition resulting from muscle imbalance, persistent femoral anteversion and coxa valga, and delayed weight bearing. The incidence of hip subluxation or dislocation in the CP population is reported to be from 18 to 59%. Approximately 50% of these hips become painful, which decreases the person s ability to walk or stand, and in the non-ambulatory patient, to sit comfortably or to stand for transfers. The more severely involved patients have a greater incidence of hip subluxation or dislocation. In a review of over 2000 patients at the Hospital for Special Surgery (HSS) the incidence of subluxation was 37% and dislocation was 8%. In the totally involved quadriplegic patient, subluxation occurred in 38% and dislocation in 15.5%. In the partially involved patient (hemiplegic, diplegic, monoplegic) subluxation occurred in 9.5% and dislocation occurred in 1%. Early treatment involves appropriate muscle releases before subluxation occurs and when subluxation is documented on radiographs, varus rotation hip osteotomies are necessary to redirect the femoral head into the acetabulum. If there is acetabular insufficiency, a pelvic osteotomy is added in order to obtain coverage for the femoral head. A review of the literature and recommendations are made for the surgical treatment of these patients. For the mature adolescent or adult in whom the hip cannot be reduced due to deformity of the femoral head or acetabulum, salvage procedures are utilized to relieve pain and restore function (i.e. the ability to stand walk, or to sit comfortably) and to allow for the ability of standing transfers. Among the procedures most commonly performed are proximal femoral resections, valgus osteotomies, hip arthrodesis or total hip arthroplasty. All these methods shall be reviewed with indications and contraindications. Another disabling hip disorder in the adult CP is persistent femoral anteversion in the ambulatory individual. Three cases are presented to illustrate this problem. SURGICAL TREATMENT FOR HIP SUBLUXATION AND DISLOCATION IN THE CHILD WITH CP Hip subluxation or dislocation is an acquired problem in the CP population. The incidence varies from 18 to 59%. 1 5 In my review of more than 2000 patients treated at the Hospital for Special Surgery (HSS) in New York City the incidence of dislocation and subluxation was 8% and 37% respectively. The incidence was much higher in the totally involved quadriplegic patient, with dislocation occurring in 15.5% of these patients and subluxation occurring in 38%. In the partially involved patient (hemiplegic diplegic monoplegic), dislocation was present in 1%.of patients and subluxation in 9.5 %. ª 2009 The Author Journal compilation ª Mac Keith Press 2009 Developmental Medicine & Child Neurology 2009, 51 (Suppl. 4): DOI: /j x

2 The etiology of hip subluxation and dislocation in children is persistent fetal femoral anteversion and neck shaft valgus because of delayed weight bearing and abnormal muscle imbalance about the hip. The muscle imbalance occurs because strong hip flexors overpower weaker hip extensors and strong hip adductors overpower weaker abductors. As a result of these abnormal forces, the hip will subluxate, or even dislocate, over time. Subluxation and dislocation in the young child can lead to femoral head deformity and result in hip pain as well as significant limitation of motion. In spite of braces and physical therapy, the process tends to be progressive. Early treatment consists of releasing contracted hip adductors and hip flexors as well as varus rotation osteotomy (VRO) to correct excessive neck shaft valgus and femoral anteversion. 1,6,7 (Fig. 1a,b,c) When surgery is done at an early stage, the hips can be preserved and function maintained. However, when the subluxation is advanced with a shallow acetabulum, more extensive procedures are necessary. These procedures may include proximal femoral derotation osteotomies with femoral shortening and a pelvic procedure to provide coverage for the femoral head. Muscle tendon lengthening or releases are often necessary, usually involving adductors, hamstrings and or hip flexors. With pelvic obliquity or wind-swept deformity, the located or contralateral hip may require a VRO to improve pelvic obliquity and to maintain equal leg lengths. (Fig. 2a,b,c,d) In our study at HSS, and confirmed by a review of the literature, reduction of the subluxated dislocated hip in the CP patient can result in a painless and functional hip joint. For ambulatory patients, a stable painless hip ensures continued ability to walk. For wheelchair-bound patients, a stable hip is essential for comfortable and secure sitting. These complex procedures are best performed at an early age, before the onset of femoral head deformity. However, even in the young adolescent who is skeletally mature and who has mild-to-moderate femoral head deformity, the procedure can be a great benefit in providing painless hips and stable sitting. Highlights of the extensive experience with such surgery over the past 20 years are as follows: (1) Carr and Gage 8 reported on 36 patients (ages 4 16) who underwent unilateral hip surgery and found that nonambulatory patients were at greater risk of deterioration of the non-operated hip than ambulatory patients. They also found that those patients operated on before 9 years of age had a significant worsening of femoral head coverage of the non-operated hip in comparison with those over 9 years of age. (2) Jerosch et al. 9 reported on 11 patients (mean age was 14.4 SD 3.7y) who had combined VRO and triple pelvic osteotomy with good reduction, were pain-free on follow-up. (3) Root et al. 10 followed 31 patients (35 hips) with severely subluxated or dislocated hips. 10 The patients were treated with open reduction, femoral VRO, femoral shortening, or pelvic osteotomy. Average follow-up was 7 years, all patients had at least a 2-year follow-up. Mean age was 12 years, age range 4 to 23 years. Although some patients had pain for up to 8 months, by 1 year after surgery all were pain-free and remained so at last follow-up. Good femoral head coverage was obtained. One hip required a repeat VRO. Four hips resubluxated but were painless on follow-up. Eight hips developed a degree of avascular necrosis with subsequent premature closure of the femoral capital physis. In spite of the complications, the extensive reconstruction was justified for these patients. (4) Atar et al. 11 reported on 17 hips in 14 patients, age range: 2.5 to 17 years (average age 10), who had VRO, Figure 1: Management of early hip subluxation: (a) Pre-op: mild subluxation right and bilateral femoral anteversion (b) 18 month following bilateral VRO, and (c) 2 years post-op. Figure 2: Management of right dislocated hip with windswept deformity: (a) Pre-op (b) 10 weeks following Salter innominate osteotomy right, adductor tenotomies and bilateral VROs, (c) 10 months post-op, and (d) 9 years post-op. Surgical Treatment for Hip Pain in Adult CP Leon Root 85

3 open reduction, innominate osteotomy, adductor release, and psoas recession. Most patients had spastic quadriplegia. Sixteen of the hips were stable at a minimum followup of 3 years. (5) McNerney et al. 12 reported one-stage correction of the dislocated hip with the San Diego arthroplasty combined with VRO and soft-tissue releases (age range y). Average follow-up was 6.7 years. Ninetynine of 104 hips were reduced at follow-up; there were no redislocations. Eight hips (8%) had avascular necrosis of the femoral head. These authors concluded that even hips with some deformity of the femoral head can be successfully treated with this combined approach. (6) Noonan et al. 13 reported the results of VRO in the treatment of subluxation or dislocation in 65 patients (age range y) who had 79 hip surgeries for incongruity and some deformity of the femoral head. At follow-up, 72% of the hips were stable. They found that subluxated hips were likely to remain stable more than dislocated hips, and that children under 7.2 years of age were significantly more likely to be stable on follow-up than those who were over 10 years of age. SURGICAL TREATMENT FOR HIP SUBLUXATION AND DISLOCATION IN THE SKELETALLY MATURE PATIENT Introduction Some have expressed concern that reduction of the hips in the skeletally mature patient would not be successful in relieving pain. Inan 14 in 2007 reported on performing 33 incomplete transiliac osteotomies on 27 skeletally mature patients that resulted in a painless and stable hip in 26 patients. In the adult CP person with a painful subluxated or dislocated hip, reconstruction procedures are not successful (Fig. 3). When conservative therapy no longer provides relief from pain or improvement in care, treatment consists of surgical salvage procedures. Hip pain has been reported to be as high as 50% in those adult patients with hip dysplasia, and generally those patients who are most severely involved have the most significant problems. 5,15 22 Surgical options for adult CP patients include head and neck resection; interposition arthroplasty (Castle procedure); valgus osteotomy as described by Schantz and Haas, 27,28 or the McHale modification 30 ; hip arthrodesis; and total hip replacement. Head and neck resection Head and neck resection, or the Girdlestone procedure (Fig. 4), has not been successful in relieving pain as reported by Hoffer 17 and Perlmutter et al. 22 Even with Figure 3: Dislocation with severe femoral head deformity. Figure 4: Head-neck resection (Girdlestone). (a) Pre-op and (b) 1y post-op. postoperative skeletal traction, these patients continued to have pain. Kalen and Gamble 23 reported their results of 18 hips in 15 non-ambulatory patients who had resection of the proximal femur at a level above the lesser trochanter. Thirteen patients had spastic dislocation due to CP and nine patients had painful hips. Seven of the hips had good pain relief, but three required reoperation for pain caused by proximal migration of the proximal femur. Twelve of the 15 patients developed heterotopic ossification. Koffman 19 reported on 10 proximal femoral resection in six non-ambulatory CP patients with painful dislocations. Most procedures were done at the level of the lesser trochanter. 19 Every patient continued to have pain. Orthopedic surgeons no longer use this procedure. 86 Developmental Medicine & Child Neurology 2009, 51 (Suppl. 4): 84 91

4 Figure 5: Painful windswept hips (a) Pre-op (b) post-op following bilateral proximal resections and muscle interposition. Interposition arthroplasty Interposition arthroplasty, or proximal femoral resection, has been a major salvage procedure for patients with painful hips who are wheelchair-bound and nonambulatory. Castle et al. 24 described a more radical approach to this problem. His technique involves excising the proximal femur below the level of the lesser trochanter (Fig. 5a,b). The vasti muscles are sewn over the proximal end of the femur, and the abductor and psoas muscles and hip joint capsule are sewn over the acetabulum. In this manner, a large mass of soft tissue is interposed between the proximal end of the femur and the pelvic area. In many cases, the femur migrates proximally. Widmann et al. 25 reported the results at HSS of resection arthroplasty in 13 patients (18 hips). Average age at surgery was 26.6 years, with an average follow-up of 7.4 years. All patients had significant improvement in subjective assessment of pain after surgery. Upright sitting tolerance improved in all patients. Heterotopic ossification was reduced with single-dose radiation therapy to the operative area. These patients were treated with postoperative skin traction and early mobilization. Gabos et al. 26 reported on using a prosthetic arthroplasty in 14 painful degenerative hips in 11 patients with an age range of 11 to 20 years. They combined proximal resection with insertion of a non-cemented shoulder prosthesis into the femoral canal. Seven of these patients also had a glenoid component inserted. Complete pain relief was achieved in 10 patients (13 hips). Sitting tolerance improved in every patient. Valgus support osteotomy Valgus support osteotomy was originally described for osteoarthritis, non-union fractures of the hip, or late congenital hip dysplasia Samilson et al. 1 described valgus osteotomy for painful subluxation dislocation of the hip in severely involved CP patients. McHale et al. 30 reported on their results of combining femoral head resection and subtrochanteric valgus osteotomy on six hips in five non-ambulatory adolescent CP Figure 6: Valgus osteotomy (McHale procedure). patients with painful dislocated hips (Fig. 6). They reported few complications and good pain relief. Leet et al. 31 compared the results of the McHale procedure with results of proximal femoral resection and traction. This was a retrospective study of 36 hips in 27 patients, all of whom except one had severe quadriplegia. Their goals were to obtain pain relief, improve sitting tolerance, and facilitate perineal hygiene care. Sixteen patients (23 hips) had femoral neck resection below the lesser trochanter with muscle interposition (FHRT), as described by McCarthy; patients (15 hips) had the McHale procedure. 33 Complications were higher for the FHRT group. Patients or caregivers were asked to evaluate their overall satisfaction with the surgery. Seven of the FHRT group and eight of the McHale group responded to the questionnaire. Pain was reduced in both groups, but more so in the FHRT group than in the McHale group. Patients in both groups were satisfied with the surgery. Both groups took a long time to be pain-free. Hospital stay and complications were less in the McHale group than the FHRT group, but end results were equally satisfactory. More recently, Hogan et al. 33 reported on their modified Haas valgus-subtrochanteric osteotomy in 31 hips in 24 patients. Although they had 15 complications, the majority of the patients were doing well at an average of 44 Surgical Treatment for Hip Pain in Adult CP Leon Root 87

5 months after surgery. Twenty of these patients were spastic quadriplegics. These authors did not recommend concurrent femoral head resection in combination with a valgus osteotomy. Sixteen caregivers of these patients responded to a questionnaire; 14 of those who responded were satisfied with the operative procedure. In all 16 patients improvement was noted in sitting tolerance. Twelve of the patients had no pain with transfers. Neither valgus osteotomies nor McHale procedures have been done at HSS. For wheelchair-bound patients who are non-ambulatory and cannot stand to transfer, we prefer a proximal femoral resection with interposition muscle, early radiation to the operative area, postoperative skin traction, and early mobilization. Postoperative pain is a major problem in all these patients but usually resolves in several months, after which time caregivers and patients are pleased with the surgical result. Hip arthrodesis Arthrodesis of the hip is a time-honored procedure in orthopedics. I performed my first hip arthrodesis on a CP patient in Over the years, I performed the procedure in eight patients (eight hips) between the ages of ages 13 to 34 years. 35 Follow-up was from 8 to 33 years. Two of the patients required revisions; one had one revision, and the other required two. Both patients ultimately received a total hip replacement. The basic technique for arthrodesis is an interarticular denuding of the acetabulum and femoral head cartilage and fixation of the femoral head into the acetabulum with large screws or blade plate. A subtrochanteric osteotomy is necessary to promote fusion. Copious iliac bone graft is utilized. Immobilization in a spica cast is necessary for 3 to 6 months (Fig. 7a,b,c) Figure 7: Painful left hip subluxation in ambulatory 26 yo male (a) Pre-op (b) 5 years post hip arthrodesis with solid fusion, no pain and walking without external support (c) 30 years post-op. Successful hip arthrodesis for the painful subluxated dislocated hip in CP was also reported by demoraes Barros Fucs et al. 35 They evaluated 14 arthrodesis patients with a mean age at surgery of 15.5 years of age and a mean follow-up of 5.3 years. Bone union was obtained in all cases, as was relief of pain and postural improvement. Hip arthrodesis can be successful for the younger adult who has a normal contralateral hip and a normal lumbosacral spine. Ambulatory patients with a hip fusion function very well, and wheelchair patients can sit and stand comfortably. Nevertheless, in today s world, it is difficult to convince someone to accept a fused hip over having the mobility of a total hip replacement. Total hip arthroplasty Total hip arthroplasty (THA) has changed the life of millions of people with painful hips since the early 1960s when Sir John Charlney reported on his outstanding results. 39,40 However, orthopaedic surgeons have been reluctant to recommend the procedure for a CP person with a painful subluxed or dislocated hip. The questions were: Would a hip replacement in a CP patient dislocate or loosen prematurely because of spasticity or athetosis? And would the procedure reduce pain and restore function in this group of patients? In 1971, PD Wilson, Jr., performed the first total hip replacement on a CP patient at HSS. The operation was successful in relieving hip pain and restoring function to a 57-year-old male who remained active in the community and pain-free until his death many years later. Weber and Cabarela 36 reported on 16 patients with 16 hip replacements. Eighty-seven percent of the patients had pain relief, and ambulatory function improved in 79%. They had no dislocations, and complications were rare. Schorle et al. 37 reported on 19 CP patients who had painful hips. Following THA, 84% were pain-free and all walked better. We published our results on THA in CP in and again in 1993 (Buly et al.). 38 In September 2007, we presented the last follow-up on 65 hip replacements in 62 patients at the annual meeting of the American Academy for Cerebral Palsy and Developmental Medicine in Vancouver Canada, and at the March 2008 meeting of the American Academy of Orthopedic Surgeons in San Francisco. The survivorship was 85% for 10 years and preoperative pain was relieved in all patients. (Figs 8a,b,c and 9a,b,c) These studies confirm that THA is a safe and successful procedure for painful hips in the cerebral palsy population, even in the younger age groups and even in those who function mainly in a wheelchair. 88 Developmental Medicine & Child Neurology 2009, 51 (Suppl. 4): 84 91

6 Figure 8: 27 yo male with spastic diplegia and painful left hip (a) Preop (b) 1 year post-op, femoral head used to augment acetabulum (c) 22 years post-op with no pain, walking without external support. SURGICAL TREATMENT OF FEMORAL ANTEVERSION IN THE ADULT CP PATIENT: THREE CASE STUDIES It is not just painful subluxated hips that cause difficulty for the adult CP patient. A significant number of ambulatory and high-functioning diplegic and hemiplegic adults have increasing difficulty in walking as a result of an internal rotation gait pattern and gradually increasing tightness or contracture of their hips, knees, or ankles. The primary problem is excessive anteversion, and the secondary problem is muscle contractures. To illustrate this problem, I present three adult patients who had stable hips but had increasing difficulty in walking because of an internally rotated and spastic gait pattern. KP was 41-year-old female with spastic diplegia who presented with increasing difficulty in walking and carrying out her work as a nurse. She had three children and had been totally independent. As a child, she had multiple soft-tissue procedures and a left hip osteotomy. Upon first presentation, radiographs revealed stable hips, and clinical examination revealed significant increased internal rotation of the hips with limited external rotation. She also had contractures of the Achilles tendons and over-pull of the posterior tibial muscles. A gait study was performed. She underwent bilateral VROs, heel cord lengthening, and split tendon transfers in her feet. Her postoperative rehabilitation was lengthy and difficult, but after 1 year, she had no pain and was able to walk erect, but she had significant muscle weakness. It took almost 2 years before she was able to resume most of her normal activities. At her 5 year follow-up, she continued to use a cane for long distances. (Fig. 10a,b) BK was a 49-year-old female with left hemiplegia. She was single and worked full-time as a secretary. She complained of knee pain and increased difficulty walking due to marked internal rotation of her left leg. As a child she had a left heel cord lengthening. X-rays revealed a stable hip, and clinical examination demonstrated an internal rotation pattern of the left leg with stiff knee gait and tightness of her hamstrings. Internal rotation of the left hip was 60 and external was 0. A gait study was performed, and she underwent a left VRO, adductor tenotomy, bilateral hamstring lengthening, and distal left rectus femoris transfer. Although her osteotomy was slow to heal, she was pain-free at 1 year after surgery. At 9 years after surgery, she had no pain and Figure 9: Painful right hip in 16 yo male previously able to stand for transfers (a) pre-op (b) 1 year post-op right THR and left VRO, no pain and able to stand for transfers (c) 8 years post-op, no pain, good sitting tolerance, continues to stand for transfers. Figure 10: Management of femoral anteversion Case KP: (a) pre-op (b) immediate post-op (c) 3 years post-op. Surgical Treatment for Hip Pain in Adult CP Leon Root 89

7 Figure 12: Case MH: (a) Pre-op (b) immediate post-op (c) 3 years post-op. Figure 11: Case BK: (a) Pre-op (b) immediate post-op (c) post-op 1 year with delayed union, (d) 9 years post-op. walked with straight hip knee foot progression, but she used a cane for outdoor stability. (Fig. 11a,b) At time of presentation, the third patient, MH, a 34-year-old female, had been experiencing increasing difficulty in walking and pain in her left knee and foot. She had mild spastic diplegia. She walked with a mild crouch gait and marked internal rotation of her left leg and foot. In the past she had multiple soft-tissue procedures. She was single and worked full-time as a librarian. X-rays revealed stable hips, and clinical examination revealed significant internal rotation of the left hip with bilateral contractures of the adductors and hamstring muscles as well as a contracture of the left heel cord and tendency for equinovarus of the left foot. Gait analysis was done. She subsequently had a left VRO, bilateral adductor tenotomies, bilateral hamstring lengthening, left tendo-achilles lengthening, left split anterior tibial tendon transfer, and left posterior tibial tendon recession. The postoperative rehabilitation was long and laborious, but she was able to return to work full-time by 18 months after surgery. At the latest follow up, 4 years after surgery, she had straight alignment of her left leg, no pain, but continued to use a cane for long distances. (Fig. 12a,b) SUMMARY My approach to the child with CP and hips at risk is to perform early adductor releases, hamstring lengthening, and psoas tenotomies over the brim of the pelvis. These procedures can be delayed until 3 to 4 years of age by the judicious use of botulinum toxin A into the muscle in order to relieve spasticity. Yearly hip radiographs in hips at risk, particularly in the quadriplegic patient, are essential. Once hip subluxation is documented in spite of muscle procedures and conservative care, VRO is indicated for reduction. Generally, I prefer to wait until the child is 5 or 6 years of age but if there is progression of the subluxation, VRO should be done at an earlier age. If acetabular insufficiency is present, a pelvic osteotomy (Dega, Pemberton, Salter or Chiari or Peri-acetabular osteotomy) must be added to obtain femoral head coverage and preserve hip stability. Even in the presence of femoral head deformity, as long as the femoral physis is open, reduction can result in a painless functional hip. If the child is followed closely, and hip subluxation is aggressively corrected, the problem of a painful hip in the adult CP can be successfully averted. For the older adolescent or adult patient who is wheelchair bound and is unable to walk or stand to transfer, we prefer the Castle interposition arthroplasty. For the ambulatory patient or the wheelchair patient who can at least stand to transfer, THA is safe and reliable. Hip arthrodesis in the younger population is a reasonable option. Persistent femoral anteversion in the adult ambulatory patient, even when the hips are stable, can lead to decreased function and pain. Although hip rotation osteotomies can improve walking ability and relieve pain in the adult patient, the recovery is difficult and prolonged. I strongly recommend that significant femoral anteversion that causes an internal rotation gait pattern be surgically corrected in the younger patient. 90 Developmental Medicine & Child Neurology 2009, 51 (Suppl. 4): 84 91

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