Sustained hip flexion contracture after femoral lengthening in patients with achondroplasia.

BACKGROUND: Hip flexion contracture often occurs after femoral lengthening in patients with achondroplasia, but few studies have investigated its development in these patients. The purpose of this study was to analyze sustained hip flexion contracture in achondroplasia patients who underwent femoral lengthening and to identify contributing factors. METHODS: This study included 34 patients with achondroplasia who underwent femoral lengthening (mean age at operation, 11.1 years). Sustained hip flexion was defined as flexion contracture lasting > 6 months postoperatively despite physiotherapy. Demographic data, spinopelvic parameters (pelvic incidence, pelvic tilt, sacral slope, lumbar lordosis, and sagittal vertical axis), and quantitative assessments of femoral lengthening were investigated. The associations among these factors and the development of sustained hip flexion contracture were assessed. RESULTS: Sustained hip flexion contracture developed in 13 (38%) of 34 achondroplasia patients after femoral lengthening. Eight (62%) of these 13 patients concomitantly exhibited limitation of knee flexion. Excessive femoral lengthening (odds ratio [OR], 1.450; 95% confidence interval [CI], 1.064 to 1.975; p = 0.019) and forward sagittal vertical axis tilt (OR, 1.062; 95% CI, 1.001 to 1.127; p = 0.047) contributed to sustained hip flexion contracture. CONCLUSIONS: Sustained hip flexion contracture frequently occurs after femoral lengthening in achondroplasia patients. Both excessive femoral lengthening and preoperative forward SVA tilt may contribute to the development of sustained hip flexion contracture in these patients.

Lower extremity joint contracture according to ambulatory status in children with Duchenne muscular dystrophy.

BACKGROUND: Lower extremity joint contractures have negative effects on gait in children with Duchenne muscular dystrophy (DMD). Thus, contracture prevention is essential for maintaining a patient's functional ability and an acceptable quality of life. This study investigated hip flexion (HF), knee flexion (KF), and ankle joint plantar flexion (APF) contractures among male patients with DMD, based on the patients' ambulatory status. Differences in major joint contractures, based on passive stretching exercise participation, were also investigated. METHODS: A total of 128 boys with DMD, followed at the DMD clinic of a tertiary care hospital, were included in this cross-sectional study. The passive ranges-of-motion of the hip, knee, and ankle joints were measured, in the sagittal plane, using a goniometer. The Vignos Scale was used to grade ambulatory function. Boys with DMD who performed stretching exercises for more than 5 min/session, > 3 sessions/week, were classified into the stretching group. RESULTS: The HF (23.5o), KF (43.5o), and APF (34.5o) contracture angles in the non-ambulatory group were more severe than those in the ambulatory group. APF contractures (41 patients, 52.6%) were more frequently observed early, even within the ambulatory period, than were hip (8 patients, 10.3%), and knee joint (17 patients, 21.8%) contractures. Passive stretching exercises > 3 sessions/week were not associated with the degree of lower extremity joint contractures in the ambulatory or non-ambulatory group. CONCLUSION: HF, KF, and APF contractures are more common and severe when there is deterioration of ambulatory function. Stretching exercises alone are unlikely to prevent lower extremity joint contractures.

A three-year follow-up study of the development of joint contractures in 131 patients with systemic sclerosis.

OBJECTIVES: To analyse the correlation between the number of joint-contractures and other major clinical findings in a follow-up study of 131 patients with systemic sclerosis (SSc). METHODS: The range of motion of joints (ROM), HAQ-DI, and the major clinical characteristics were assessed. RESULTS: A high frequency of contractures (ROM<75% of the normal) were present at baseline in small joints of the hand (82%), wrists (75%), and shoulders (50%). ROM of the dominant side hand was significantly more decreased compared to the non-dominant side. The number of the upper extremity contractures correlated positively with ESR (p<0.01), CRP (p<0.01), HAQ-DI (p<0.01), and negatively with forced vital capacity (FVC) (p<0.05). The number of contractures was not significantly different in cases with early (≤ 4 years) and late disease duration in both the limited and diffuse subgroups. During the three-year follow-up period, an increase in the number of joint contractures (ROM<75%) was associated with an increase of ESR, modified Rodnan's skin score, and the European Scleroderma Study Group Activity Index by multiple linear regression analysis. Univariate analysis over a six-year period demonstrated poor outcome in patients with more than ten contractures, or more than four contractures of unilateral hand-joints. CONCLUSIONS: Contractures predominantly develop during the early years following disease onset in both SSc subgroups. Inflammation and skin-involvement are significant contributing factors for the development of contractures. The dominant hand may be more pronouncedly impaired compared to the non-dominant side. A high number of joint-contractures might be an unfavourable prognostic factor in SSc.

Hip flexion contracture and diminished functional outcomes in cerebral palsy.

BACKGROUND: Hip flexion contracture (HFC) in the ambulatory child with cerebral palsy (CP) may impair function and lead to deteriorations in health-related quality of life. Furthermore, increasing HFC may lead to increasing disability. However, the association between passive range of motion and the measures of function and well-being is unclear. This study was designed to determine whether increasing HFC is associated with functional outcome. METHODS: A total of 181 children, with an average age of 14.0 ± 10.2 years, were evaluated as part of a multicenter prospective data collection of patients with ambulatory CP. Measurements of HFC were recorded, and patients were evaluated using walking score from Gillette Functional Assessment Questionnaire (FAQ), Gross Motor Function Measure (GMFM), and the Pediatric Outcome Data Collection Instrument (PODCI). Patients were grouped on the basis of severity of HFC: group A, 0 to 14 degrees; group B, 15 to 29 degrees; and group C ≥ 30 degrees. Associations were examined using the Spearman correlation. RESULTS: There was an inverse association between degree of HFC and FAQ walking score (P<0.01, ρ=-0.25). Similarly, there was an inverse association between the degree of HFC and GMFM parts D (P<0.001, ρ=-0.31) and E (P<0.001, ρ=-0.32). Lastly, the PODCI domains of global function, mobility, and physical function also showed an inverse association with degree of HFC (P<0.001, ρ=-0.24). CONCLUSIONS: As surgeons treating children with CP, we often rely on joint measurements as an indirect measure of function. This study of children with ambulatory CP suggests that increased HFC from the physician's perspective is associated with deterioration in function from a patient and a therapist's perspective. LEVEL OF EVIDENCE: Level II, prospective study.

Excessive hip flexion during gait in patients with static encephalopathy: an examination of contributing factors.

BACKGROUND: Excessive hip flexion in gait is thought to be associated with hip flexion contracture, but has also been associated with excessive anterior pelvic tilt, knee flexion, internal hip rotation, and muscular factors. The purpose of this study was to examine the contributors to excessive hip flexion during gait in children with cerebral palsy, with and without hip flexion contractures. METHODS: A retrospective chart review was conducted of 155 children with cerebral palsy. Potential contributors to excessive hip flexion in stance were evaluated, including static and dynamic range of motion, strength measurements, and patient factors including age, previous surgery, distribution of involvement (hemiplegia, diplegia, and quadriplegia), and Gross Motor Function Classification System level. Univariate analysis was performed using simple linear regression and analysis of variance, with appropriate post-hoc tests. All variables were then included in a stepwise linear regression using forward selection. RESULTS: Univariate analysis demonstrated a significant relationship (P<0.05) between excessive hip flexion in stance and all predictive variables except static dorsiflexion range of motion with the knee flexed and maximum dorsiflexion in stance. Results of stepwise regression revealed that 3 variables accounted for 65% of the variance: passive hip extension range of motion, average pelvic tilt during the gait cycle, and knee extension achieved in the stance phase of gait. Twenty-two of 45 (49%) exhibiting hip flexion contractures of greater than 10 degrees did not exhibit excessive hip flexion in stance phase. CONCLUSIONS: Hip extension in stance in children with static encephalopathy depends primarily on hip extension passive range of motion, the amount of pelvic tilt, and knee extension in stance phase. These 3 variables account for 65% of variance in these 155 patients, whereas other factors (age, dorsiflexion in stance, and hamstring range) each account for only 2% to 3% of the variance. Careful clinical examination, including computerized gait analysis when available, is recommended before surgical intervention to determine whether excessive hip flexion is a primary or compensatory deviation. LEVEL OF EVIDENCE: Level IV, case series.

Arthroscopic gluteal muscle contracture release with radiofrequency energy.

Gluteal muscle contracture is common after repeated intramuscular injections and sometimes is sufficiently debilitating to require open surgery. We asked whether arthroscopic release of gluteal muscle contracture using radiofrequency energy would decrease complications with clinically acceptable results. We retrospectively reviewed 108 patients with bilateral gluteal muscle contractures (57 males, 51 females; mean age, 23.7 years). We used inferior, anterosuperior, and posterosuperior portals. With the patient lying laterally, we developed and enlarged a potential space between the gluteal muscle group and the subcutaneous fat using blunt dissection. Under arthroscopic guidance through the inferior portal, we débrided and removed fatty tissue overlying the contractile band of the gluteal muscle group using a motorized shaver introduced through the superior portal. Radiofrequency then was introduced through the superior portal to gradually excise the contracted bands from superior to inferior. Finally, hemostasis was ensured using radiofrequency. Patients were followed a minimum of 7 months (mean, 17.4 months; range, 7-42 months). At last followup, the adduction and flexion ranges of the hip were 45.3 degrees +/- 8.7 degrees and 110.2 degrees +/- 11.9 degrees, compared with 10.4 degrees +/- 7.2 degrees and 44.8 degrees +/- 14.1 degrees before surgery. No hip abductor contracture recurred and no patient had residual hip pain or gluteal muscle wasting. We found gluteal muscle contracture could be released effectively with radiofrequency energy.

Classification of gluteal muscle contracture in children and outcome of different treatments.

BACKGROUND: Gluteal muscle contracture (GMC) is a clinical syndrome due to multiple etiologies in which hip movements may be severely limited. The aim of this study was to propose a detailed classification of GMC and evaluate the statistical association between outcomes of different management and patient conditions. METHODS: One hundred fifty-eight patients, who were treated between January 1995 and December 2004, were reviewed at a mean duration of follow-up of 4.8 years. Statistical analyses were performed using X2 and Fisher's exact tests. RESULTS: Non-operative management (NOM), as a primary treatment, was effective in 19 of 49 patients (38.8%), while operative management was effective in all 129 patients, with an excellence rating of 83.7% (108/129). The outcome of NOM in level I patients was significantly higher than in level II and III patients (P < 0.05). The results of NOM and operative management in the child group were better than the adolescent group (P < 0.05). Complications in level III were more than in level II. CONCLUSION: NOM was more effective in level I patients than in level II and III patients. Operative management was effective in patients at all levels, with no statistical differences between levels or types. We recommend NOM as primary treatment for level I patients and operative management for level II and III patients. Either NOM or operative management should be carried out as early as possible.

Postural asymmetry in non-ambulant adults with cerebral palsy: a scoping review.

PURPOSE: Non-ambulant adults with cerebral palsy are vulnerable to development of postural asymmetry and associated complications. The primary aim of this scoping review was to identify postural deformities in non-ambulant adults with cerebral palsy. MATERIALS AND METHODS: Comprehensive searches were undertaken in EMBASE, CINAHL, AMED, Cochrane, Psych INFO, and Joanna Briggs (1986-Jan 2017), supplemented by hand searching. Two reviewers independently extracted data using a customised tool focusing on study design, participant characteristics, postural descriptors, measurement tools, and interventions. RESULTS: From 2546 potential records, 17 studies were included. Variability in populations, reporting methodology, and measurement systems was evident. Data suggest more than 30% of this population have hip migration percentage in excess of 30%, more than 75% experience "scoliosis", and more than 40% demonstrate pelvic obliquity. Estimates ranged from 14% to 100% hip and 32% to 87% knee contracture incidence. Conservative interventions were infrequently and poorly described. CONCLUSION: Many non-ambulant adults with cerebral palsy experience postural asymmetry associated with windswept hips, scoliosis, pelvic obliquity, and limb contracture. Options for non-radiographic monitoring of postural asymmetry should be identified, and conservative interventions formally were evaluated in this population. Implications for rehabilitation The common postural asymmetries of windswept hips, scoliosis, pelvic obliquity, and limb contracture require standardised clinical measurement. Radiography is most commonly used to monitor postural asymmetry in this population, but standardised positioning is not applied and may not be feasible indicating a need for alternate methods and rigorous documentation. The Posture and Postural Ability Scale may be considered for use in the management of body shape in adults with CP.

Spinopelvic Compensatory Mechanisms for Reduced Hip Motion (ROM) in the Setting of Hip Osteoarthritis.

STUDY DESIGN: Retrospective review from a single institution. OBJECTIVE: To investigate the effect of hip osteoarthritis (OA) on spinopelvic compensatory mechanisms as a result of reduced hip range of motion (ROM) between sitting and standing. SUMMARY OF BACKGROUND DATA: Hip OA results in reduced hip ROM and contracture, causing pain during postural changes. Hip flexion contracture is known to reduce the ability to compensate for spinal deformity while standing; however, the effects of postural spinal alignment change between sitting and standing is not well understood. METHODS: Sit-stand radiographs of patients without prior spinal fusion or hip prosthesis were evaluated. Hip OA was graded by Kellgren-Lawrence grades and divided into low-grade (LOA; grade 0-2) and severe (SOA; grade 3 or 4) groups. Radiographic parameters evaluated were pelvic incidence (PI), pelvic tilt (PT), lumbar lordosis (LL), PI-LL, thoracic kyphosis (TK), SVA, T1-pelvic angle (TPA), T10-L2, proximal femoral shaft angle (PFSA), and hip flexion (PT change-PFSA change). Changes in sit-stand parameters were compared between LOA and SOA groups. RESULTS: 548 patients were included (LOA = 311; SOA = 237). After propensity score matching for age, body mass index, and PI, 183 LOA and 183 SOA patients were analyzed. Standing analysis demonstrated that SOA had higher SVA (31.1 vs. 21.7), lower TK (-36.2 vs. -41.1), and larger PFSA (9.1 vs. 7.4) (all p < .05). Sitting analysis demonstrated that SOA had higher PT (29.7 vs. 23.3), higher PI-LL (21.6 vs. 12.4), less LL (31.7 vs. 41.6), less TK (-33.2 vs. -38.6), and greater TPA (27.9 vs. 22.5) (all p < .05). SOA had less hip ROM from standing to sitting versus LOA (71.5 vs. 81.6) (p < .05). Therefore, SOA had more change in PT (15.2 vs. 7.3), PI-LL (20.6 vs. 13.7), LL (-21.4 vs. -13.1), and T10-L2 (-4.9 vs. -1.1) (all p < .001), allowing the femurs to change position despite reduced hip ROM. SOA had greater TPA reduction (15.1 vs. 9.6) and less PFSA change (86.7 vs. 88.8) compared with LOA (both p < .001). CONCLUSIONS: Spinopelvic compensatory mechanisms are adapted for reduced hip joint motion associated with hip OA in standing and sitting. LEVEL OF EVIDENCE: Level III.

[Release of gluteal muscle contracture by radiofrequency under arthroscopy].

OBJECTIVE: To evaluate the clinical therapeutic effect of the release of gluteal muscle contracture by radiofrequency under arthroscopy. METHODS: From January 2004 to April 2005, 86 patients with gluteal muscle contracture were treated by radiofrequency release under arthroscopy.The patients were followed-up for 6-18 months(mean=12.6 months). RESULTS: Getting carriage, squatting down while keeping their knees contacting, crossing leg test, and impact on movement were served as evaluation criteria. Of the 86 patients, 79 had excellent results, 5 good, and the rest 2 were acceptable. There was no recurrence at the last follow-up, and all the patients were satisfied. CONCLUSION: For gluteal muscle contracture, radiofrequency release under arthroscopy has minimally invasion, good results and quick recovery.

Prone Position-Induced Quadriceps Transcranial Motor Evoked Potentials Signal Loss-A Case Report.

BACKGROUND: Transcranial motor evoked potential (TcMEP) is widely used intraoperatively to monitor spinal cord and nerve root function. To our knowledge, there is no report regarding TcMEP signal loss purely caused by patient positioning during the spinal procedure. PURPOSE: The objective of this article is to report an intraoperative TcMEP signal loss of a patient with fixed sagittal imbalance posture along with mild hip contractures. STUDY DESIGN: A retrospective case report. METHODS: A 57-year-old man had fixed sagittal imbalance and flexed hip contractures. For a reconstruction surgery of T10 to the sacrum/ilium and L5 pedicle subtraction osteotomy (PSO), he was put in a prone position on a Jackson table. In order to accommodate his fixed hip flexion contracture, thigh pads were not used and pillows were placed under his bilateral thighs for cushioning. TcMEPs were used to assess lumbar nerve root function. Ten minutes after incision, bilateral vastus medialis TcMEPs were lost during spine exposure whereas all other data remained normal at baseline. The bilateral lower extremities were repositioned, with the knees flexed into a sling position to increase hip flexion. Five minutes after repositioning, the bilateral vastus medialis TcMEPs gradually improved and maintained baseline amplitude during the remainder of the surgery. RESULTS: No muscle weakness was detected immediately after surgery. The patient was discharged day 6 postoperatively with markedly improved posture and alignment. CONCLUSION: Insufficient hip flexion in patients with fixed sagittal imbalance and hip flexion contractures may cause TcMEP signal changes in the quadriceps response. TcMEP monitoring of bilateral lower extremities is highly recommended for patients with sagittal imbalance and hip contractures, with consideration for lower extremity repositioning when data degradation does not correlate with the actual spinal procedure being performed.

Reorientational Proximal Femoral Osteotomies for Arthrogrypotic Hip Contractures.

BACKGROUND: Severe hip contractures in arthrogrypsosis are multiplanar, which can preclude or can greatly complicate sitting and ambulation. The reorientational osteotomy at the intertrochanteric level preserves preoperative hip motion but moves it to a more functional domain. We retrospectively compared preoperative and postoperative hip motion and evaluated the ambulatory abilities of patients who underwent the procedure. METHODS: Since 2008, 65 patients with arthrogryposis had 119 reorientational proximal femoral osteotomies with a minimum follow-up of 2 years. The mean patient age at the time of the surgical procedure was 48 months. An intertrochanteric wedge osteotomy aligned the femoral shaft with the body axis, leaving the hip joint in its preexisting position. A cannulated hip blade plate was used for fixation. Hip motions were recorded preoperatively, at implant removal, and at the time of the latest follow-up, as was ambulatory ability. RESULTS: Eighty-one hips had a mean flexion contracture of 52° preoperatively, which improved by 35°; 84 hips with a mean preoperative adduction of -20° improved by 42°; 101 hips with a mean preoperative internal rotation of -16° improved by 35° (p < 0.0001 for all). The flexion-extension total arc of motion for the 119 hips improved by 13° (p < 0.0001). Only 11 of 94 hips that preoperatively flexed ≥90° did not do so postoperatively, but none of the patients reported seating difficulties and one of the patients had already regained hip flexion of >90° by a soft-tissue release. At a mean follow-up of 40 months, 36 patients were independently ambulatory and 20 patients were walker-dependent. CONCLUSIONS: Children with arthrogryposis often have the potential for ambulation if the limb positioning can be optimized. The reorientational hip osteotomy corrects the hip contractures by altering the range of motion but not the total arc of motion. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Windswept hip deformity in children with cerebral palsy.

Windswept hip deformity describes an abduction and external rotation position of one hip with the opposite hip in adduction and internal rotation. Windswept hip deformity may occur in association with hip dislocation and scoliosis. We analysed the prevalence of this deformity in a total population of children with cerebral palsy, and the impact of hip prevention and early treatment of contractures on the prevalence and severity of windswept hip deformity. The frequency of windswept hip deformity was 12% in the control group and 7% in the study group, comprising children in the hip prevention programme. The children with this deformity in the study group had a lower frequency of scoliosis and none had hip dislocation. It thus seems that the hip prevention programme results in a decrease in the number of children with windswept hip deformity, and a decrease in the severity of the deformity.

"Syndrome of contractures" (according to Mau) with the abduction contracture of the right hip as causative factor for development of the so-called idiopathic scoliosis.

The article provides basic explanation of "syndrome of contractures" (Mau) at newborns and babies and it's conjunction with biomechanical etiology of the so-called idiopathic scoliosis (Karski 1995-2006). The authors analyzed children with "syndrome of contractures" and noted its relevance to some clinical symptoms at children with scoliosis. Newborns and babies with clinical signs of "syndrome of contractures" require further spine examination already at age of 3-4 in order to detect "danger of oncoming scoliosis" and to introduce neo-prophylaxis. The research based on "syndrome of contractures" can explain predominance of female gender of patients with scoliosis, sides of curves, side of rib hump, progression and sensibility to new rehabilitation exercises.

Paraplegia and congenital contractures as a consequence of intrauterine trauma.

We present a newborn infant with paraplegia and contractures of the lower limbs, consistent with neurologic injury rather than malformation. The mother was involved in a severe motor vehicle accident during the sixth month of pregnancy. We propose that this infant's injuries are a result of that accident.

Myelodysplasia. The influence of the quadriceps and hip abductor muscles on ambulatory function and stability of the hip.

In an analysis of motor function, ambulatory function, and hip stability in sixty-five patients with myelodysplasia, four motor-function groups based on the strength of the quadriceps and hip abductor muscles were identified. Retrospectively, it was evident that based on these groups, it would have been possible to predict which hips would remain stable, what level of ambulatory function the patients could achieve, and whether treatment to reduce and stabilize the hips was indicated. Fifty-seven of fifty-eight hips in the twenty-nine patients with functioning quadriceps muscles but non-functioning hip-abductor muscles were either subluxated or dislocated. Thirty-nine of the remaining forty-six patients with functioning quadriceps muscles could walk. In this series, three operative procedures were used to treat hip subluxation: varus osteotomy, varus osteotomy combined with iliopsoas transfer, and iliopsoas transfer alone. One shelf procedure was also done. Varus osteotomy was the best procedure for hip subluxation while posterior iliopsoas transfer, either alone or in combination with a varus osteotomy, was of questionable value. Treatment of hip instability (subluxation or dislocation) in patients without quadriceps function was not necessary.

Contracture of the hip secondary to fibrosis of the gluteus maximus muscle.

Twenty-eight children were treated who had limited flexion of the hips and various degrees of contracture of the abductor and external rotator muscles because of fibrosis of the gluteus maximus muscle. Although the lesions could be classified as those associated with poliomyelitis, infection of the gluteus maximus muscle, and fibrosis of unknown etiology, all forty-five hips had a typical restriction of motion such that an affected hip could not be flexed in the usual sagittal plane, but had to be flexed in abduction. Poliomyelitis may have been adjunctive to the causative factor of the lesion in some cases but the probable primary etiology was multiple intramuscular injections. Excellent correction of the hip contracture was achieved in all patients by division of the fibrotic bands.

Electromyography before and after surgery for hip deformity in children with cerebral palsy. A comparison of clinical and electromyographic findings.

Twenty-three ambulatory children with spastic diplegic cerebral palsy were evaluated clinically and by electromyography before and after hip-muscle surgery. The stretch tests originally designed to distinguish specific muscle tightness and spasticity were found to be non-specific when tested by electromyography. Ambulatory electromyograms using needle electrodes and telemetry generally showed decreased activity in the released muscles and, on occasion, changes in activity in muscles not operated on. These unanticipated changes after release may explain some of the unpredictability of results of such procedures in cerebral palsy.

A new case of Ullrich's disease.

A new case of congenital, hypotonic-sclerotic muscular dystrophy is presented. The patient showed congenital hyperlaxity and looseness of distal joints, muscle weakness, and spur-like protrusion of the calcaneus. Afterwards rapid progressive contractures of both knees and hip joints developed. Muscle biopsies revealed unequivocal dystrophic abnormalities and small atrophic fibers with numerous foldings of basal lamina suggestive of a neurogenic lesion. The disease presents clinical variability but the diagnosis is possible when a newborn shows: no dominant family history, slender body, marked distal joint laxity and hyperflexibility, proximal joint contractures and normal or slightly increased serum enzymes.

Evaluation of the hip.

This paper deals with clinical evaluation of the hip. Methods of testing for mobility in the hip joint are discussed. Analysis of gait and functional activities as related to muscle imbalance is presented. Gross techniques of manual muscle testing and basic principles of specific manual muscle testing are presented.