Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 118
Filter
1.
Acta Ortop Mex ; 38(3): 197-201, 2024.
Article in English | MEDLINE | ID: mdl-38862151

ABSTRACT

INTRODUCTION: severe, rigid hip abduction deformity in individuals with cerebral palsy (CP) is an exceptionally uncommon condition. This posture hinders the positioning in the wheelchair and the completion of basic activities of daily living (ADL). Addressing such severe deformities can be quite challenging. MATERIAL AND METHODS: a 14-year-old male, with spastic-dystonic quadriplegic CP, developed rigid and severe flexion-abduction contractures in both hips, characterized by 90 degrees of flexion and 100 degrees of abduction. These contractures severely impeded his ability to comfortably use a wheelchair and even pass through doorways. Performing basic ADLs became a significant challenge for both the patient and his caregivers. RESULTS: the treatment approach involved a two-stage surgical procedure, one for each hip, with a two-month interval between them. An extensive release of the fascia latae, gluteus maximus, external rotators, and hip flexors; in combination with a proximal femur osteotomy were performed. To maintain the corrections achieved, long-leg casts connected with two bars were employed, followed by orthotic support and physiotherapy. Following the procedure, lower limb adduction was achieved, and the patient and caregivers were highly satisfied, as ADLs and basic caregiving had been greatly facilitated. CONCLUSIONS: while the available literature on the management of severe rigid abduction hip contractures in non-ambulatory CP patients is limited, and treatment options are often complex, the present case underscores the effectiveness of a comprehensive approach involving soft tissue release and bone surgery. Achieving a more favorable wheelchair positioning and facilitating basic ADLs and care represents a significant success for patients and families.


INTRODUCCIÓN: la deformidad severa y rígida en abducción de cadera en individuos con parálisis cerebral (PC) es una condición infrecuente. Esta postura dificulta el posicionamiento en la silla de ruedas y la realización de actividades básicas de la vida diaria (AVD). El tratamiento de estas deformidades tan severas puede ser todo un reto. MATERIAL Y MÉTODOS: varón de 14 años, con PC tetrapléjica espástica-distónica, que desarrolló contracturas rígidas y severas de flexión-abducción en ambas caderas, caracterizadas por 90 grados de flexión y 100 grados de abducción. Estas contracturas impedían gravemente su capacidad para utilizar cómodamente una silla de ruedas e incluso pasar por las puertas. La realización de actividades básicas de la vida diaria se convirtió en un reto importante tanto para el paciente como para sus cuidadores. RESULTADOS: el tratamiento consistió en una intervención quirúrgica en dos fases, una para cada cadera, con un intervalo de dos meses entre ellas. Se realizó una amplia liberación de la fascia lata, el glúteo mayor, los rotadores externos y los flexores de la cadera; en combinación con una osteotomía proximal del fémur. Para mantener las correcciones conseguidas, se emplearon escayolas de pierna larga conectadas con dos barras, seguidas de soporte ortésico y fisioterapia. Tras la intervención, se consiguió la aducción de los miembros inferiores y el paciente y los cuidadores se mostraron muy satisfechos, ya que se habían facilitado en gran medida las AVD y los cuidados básicos. CONCLUSIONES: aunque la bibliografía disponible sobre el tratamiento de las contracturas rígidas graves de la cadera en abducción en pacientes no deambulantes con PC es limitada, y las opciones de tratamiento suelen ser complejas, el presente caso subraya la eficacia de un enfoque integral que incluye la liberación de los tejidos blandos y la cirugía ósea. Conseguir una posición más favorable en la silla de ruedas y facilitar las AVD básicas y los cuidados representa un éxito significativo para los pacientes y sus familias.


Subject(s)
Cerebral Palsy , Hip Contracture , Humans , Cerebral Palsy/complications , Male , Adolescent , Hip Contracture/etiology , Hip Contracture/surgery , Severity of Illness Index , Activities of Daily Living , Contracture/surgery , Contracture/etiology
2.
Acta Paediatr ; 110(5): 1562-1568, 2021 05.
Article in English | MEDLINE | ID: mdl-33305389

ABSTRACT

AIM: The aim was to identify the prevalence of hip contractures and their association with gross motor function and pain in the lower extremities among Danish children with cerebral palsy (CP). METHODS: This cross-sectional study was based on data collected during regular clinical examinations in 2018-2019 and registered in the National Danish Clinical Quality Database of children with CP. The study population was 688 children (59% boys) aged five to 12 years across all Gross Motor Function Classification System (GMFCS) levels. Any associations between hip contracture and gross motor function and pain were investigated with logistic regression analysis and presented as odds ratios (OR) with 95% confidence intervals (95% CI). RESULTS: The prevalence of hip contracture was 22% across all five GMFCS levels, and the incidence varied across the five Danish regions. The odds ratios for hip contracture were significantly higher at GMFCS level IV (OR 1.99, 95% CI 1.10-3.62) and V (OR 5.49, 95% CI 3.33-9.07) compared with level I. Hip contractures were not significantly associated with pain in the lower extremities (OR 1.43, 95% CI 0.95-2.15). CONCLUSION: Hip contractures were frequent and associated with low gross motor function, but not lower extremity pain, in children with CP.


Subject(s)
Cerebral Palsy , Hip Contracture , Cerebral Palsy/complications , Cerebral Palsy/epidemiology , Child , Child, Preschool , Cross-Sectional Studies , Female , Hip Contracture/epidemiology , Hip Contracture/etiology , Humans , Male , Pain , Prevalence
3.
J Arthroplasty ; 34(1): 71-76, 2019 01.
Article in English | MEDLINE | ID: mdl-30342954

ABSTRACT

BACKGROUND: Despite significant pain relief following total hip arthroplasty (THA) in patients with ankylosing spondylitis, a small subset of patients presenting with extra-articular extension contracture of hips remains unsatisfied. METHODS: We retrospectively evaluated the patients with ankylosing spondylitis who underwent simultaneous bilateral THA and had extensor tightness of both hips preoperatively. They were managed with modified Z-plasty of iliotibial band. Patients with windswept deformity, commonly seen in bilateral hip arthritis caused by ankylosing spondylitis, were excluded. RESULTS: Between July 2011 and June 2015, out of 148 patients with bilateral hip involvement, 10 patients (20 hips) had extension contracture of both hips that was addressed during surgery. All patients were followed up for a minimum of 2 years. They could sit comfortably on a chair of height 18 inches with hips and knees flexed to at least 90°. The mean postoperative sum range of motion was 144.6° with an average hip flexion of 95° (range, 90°-105°). None of them had recurrence of extension contracture. There was significant improvement in range of motion and hence ambulation and function. No radiolucent lines exceeding 2 mm were seen in any of the zones around either of the components as evaluated in latest X-rays. CONCLUSION: Extension contracture of hip although rare is a noticeable problem and needs to be addressed during THA. Modified Z-plasty technique of iliotibial band is a reliable method in managing these patients.


Subject(s)
Arthroplasty, Replacement, Hip/methods , Hip Contracture/etiology , Hip Contracture/surgery , Spondylitis, Ankylosing/complications , Adult , Arthritis/surgery , Arthroplasty, Replacement, Hip/rehabilitation , Contracture , Female , Hip/surgery , Hip Joint , Hip Prosthesis , Humans , Male , Middle Aged , Postoperative Period , Radiography , Range of Motion, Articular , Retrospective Studies , Walking , Young Adult
4.
BMC Musculoskelet Disord ; 19(1): 417, 2018 Nov 29.
Article in English | MEDLINE | ID: mdl-30497473

ABSTRACT

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.


Subject(s)
Achondroplasia/surgery , Bone Lengthening/adverse effects , Femur/surgery , Hip Contracture/etiology , Postoperative Complications/etiology , Adolescent , Adult , Child , Female , Follow-Up Studies , Hip Contracture/epidemiology , Hip Contracture/physiopathology , Hip Joint/physiopathology , Humans , Male , Postoperative Complications/epidemiology , Postoperative Complications/physiopathology , Range of Motion, Articular , Treatment Outcome , Young Adult
5.
BMC Musculoskelet Disord ; 19(1): 287, 2018 Aug 16.
Article in English | MEDLINE | ID: mdl-30111310

ABSTRACT

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.


Subject(s)
Ankle Joint/physiopathology , Contracture/etiology , Gait , Hip Joint/physiopathology , Knee Joint/physiopathology , Muscular Dystrophy, Duchenne/complications , Adolescent , Age Factors , Biomechanical Phenomena , Child , Child, Preschool , Contracture/diagnosis , Contracture/physiopathology , Contracture/prevention & control , Cross-Sectional Studies , Hip Contracture/etiology , Hip Contracture/physiopathology , Humans , Male , Mobility Limitation , Muscle Stretching Exercises , Muscular Dystrophy, Duchenne/diagnosis , Muscular Dystrophy, Duchenne/physiopathology , Muscular Dystrophy, Duchenne/therapy , Range of Motion, Articular , Retrospective Studies , Treatment Outcome
6.
Eur Spine J ; 27(8): 1671-1678, 2018 08.
Article in English | MEDLINE | ID: mdl-29167992

ABSTRACT

BACKGROUND: We describe a case of severe and progressive lumbar hyperlordosis (160°) in a 28-year-old female university student with cerebral palsy. Her main complaints were abdominal wall pain and increasing inability to sit in her custom wheelchair. METHOD: When deciding on our opinion about the most promising treatment strategy, we contemplated slow continued correction by means of percutaneously expandable magnetic rods (MAGEC) after the index surgery as a key component of a satisfactory correction in this severe and rigid curve. After an initial radical release and partial correction, a release and correction procedure was required for the bilateral hip flexion contracture. A final in situ posterior fusion was performed as a second spinal procedure, once the desired final correction at 66° of lumbar lordosis was achieved. RESULT: Three years after the completion of surgery, the patient has a stable clinical and radiological result as well as a solid posterior fusion on CT. CONCLUSION: This is the first case published in which percutaneous magnetic distraction was successfully used in an adult patient.


Subject(s)
Lordosis/therapy , Magnetic Field Therapy/methods , Muscle Spasticity/therapy , Spinal Fusion/methods , Adult , Cerebral Palsy/complications , Cerebral Palsy/surgery , Female , Hip Contracture/etiology , Hip Contracture/surgery , Humans , Lordosis/etiology , Magnetic Resonance Imaging , Muscle Spasticity/etiology , Spine/surgery , Tomography, X-Ray Computed , Treatment Outcome
7.
Ann Vasc Surg ; 47: 281.e1-281.e4, 2018 Feb.
Article in English | MEDLINE | ID: mdl-28893707

ABSTRACT

Treatment of chronic peripheral ischemic wounds in patients with spinal cord injury (SCI) can be technically challenging, especially if they have significant hip contracture. This article describes the endovascular aortoiliac revascularization of a paraplegic patient with hip contracture and a hostile abdomen. It also reviews the particularity of the peripheral arterial system in SCI patients.


Subject(s)
Angioplasty, Balloon , Aortic Diseases/therapy , Hip Contracture/etiology , Iliac Artery , Paraplegia/etiology , Peripheral Arterial Disease/therapy , Spinal Cord Injuries/complications , Angioplasty, Balloon/instrumentation , Angioplasty, Balloon/methods , Aortic Diseases/complications , Aortic Diseases/diagnostic imaging , Computed Tomography Angiography , Embolic Protection Devices , Hip Contracture/diagnosis , Humans , Iliac Artery/diagnostic imaging , Male , Middle Aged , Paraplegia/diagnosis , Peripheral Arterial Disease/complications , Peripheral Arterial Disease/diagnostic imaging , Skin Ulcer/etiology , Spinal Cord Injuries/diagnosis , Stents , Treatment Outcome , Wound Healing
8.
Orthop Surg ; 6(4): 274-9, 2014 Nov.
Article in English | MEDLINE | ID: mdl-25430710

ABSTRACT

OBJECTIVE: Accurate understanding of the cause of the underlying pathology in children with diastrophic dysplasia would help in designing targeted management of their locomotion. METHODS: Diastrophic dysplasia was diagnosed in twelve patients (nine girls and three boys; age range 1-14 years), all of whom presented with small stature and apparent short extremities. Club foot (mostly talipes equinovarus) was the most frequent and consistent abnormality. Concomitant abnormalities such as hip flexion contracture, flexion contractures of the knees with excessive valgus deformity and lateral patellar subluxation, were also encountered. Muscle ultrasound and muscle magnetic resonance imaging imaging showed no myopathic changes and muscle biopsies and the respiratory chain were normal. Serum choline kinase and plasma lactate concentrations were normal. RESULTS: Surgical correction of the foot and ankle in patients with diastrophic dysplasia is extremely difficult because of the markedly distorted anatomy. In all of these children, plantigrade foot was achieved along with the improved function of the locomotor system. Mutations of the diastrophic dysplasia sulfate transporter (also known as solute carrier family 26 member 2) were encountered. CONCLUSION: Arthrogryposis multiplex is the usual terminology used to describe the abnormality in infants with multiple contractures. Diligent orthopaedic care should be provided based on an accurate understanding of the associated syndromes in such children.


Subject(s)
Arthrogryposis/surgery , Clubfoot/surgery , Coxa Valga/surgery , Dwarfism/complications , Orthopedic Procedures , Patellar Dislocation/surgery , Adolescent , Arthrogryposis/diagnosis , Arthrogryposis/etiology , Child , Child, Preschool , Clubfoot/diagnosis , Clubfoot/etiology , Contracture/diagnosis , Contracture/etiology , Contracture/surgery , Coxa Valga/diagnosis , Coxa Valga/etiology , Female , Hip Contracture/diagnosis , Hip Contracture/etiology , Hip Contracture/surgery , Humans , Infant , Knee Joint/surgery , Male , Patellar Dislocation/diagnosis , Patellar Dislocation/etiology , Treatment Outcome
9.
Am J Sports Med ; 42(11): 2654-8, 2014 Nov.
Article in English | MEDLINE | ID: mdl-25214532

ABSTRACT

BACKGROUND: Adequate hip range of motion is required for the transfer of energy from the lower to the upper extremity along the kinetic chain. Repetitive rotational stresses in the lower extremities during tennis may lead to sport-specific range of motion adaptations, which may increase the risk of injury to other joints along the kinetic chain. PURPOSE: To assess whether such range of motion adaptations occur in the hip, and if so, to identify whether they are associated with injury. STUDY DESIGN: Cross-sectional study; Level of evidence, 3. METHODS: A total of 125 female professional tennis players, the majority of whom were ranked in the top 200 World Tennis Association singles rankings, underwent a comprehensive preparticipation physical health status examination. Hip range of motion was assessed using a digital inclinometer and side-to-side differences in rotational parameters calculated, and associations with previous injuries were identified. RESULTS: A history of an abdominal strain was reported by 10% of players, and there was an association between abdominal strains and the presence of hip flexion contractures (odds ratio, 6.1; P = .006). Hip flexion contractures were bilateral in 85% of those found, affected only the nondominant side in 9%, and affected only the dominant side in 6%. We were unable to identify any specific side-to-side rotational adaptations in the dominant or nondominant hips, and no association between loss of hip range of motion and shoulder, lower back, hip, knee, or ankle injuries was found. CONCLUSION: We report an association in female professional tennis players between abdominal strains and flexion contractures of the hip with iliopsoas tightness. We did not find evidence of specific hip adaptations in rotational range of motion. If hip flexion contractures are found on clinical examination, a stretching program may be indicated. Further studies are required to assess whether such a program can reduce the risk of abdominal injury.


Subject(s)
Adaptation, Physiological/physiology , Hip Contracture/etiology , Hip Joint/physiology , Range of Motion, Articular , Sprains and Strains/etiology , Tennis/injuries , Abdominal Muscles/injuries , Adolescent , Adult , Cross-Sectional Studies , Energy Transfer/physiology , Female , Functional Laterality , Humans , Rotation/adverse effects , Young Adult
10.
Clin Exp Rheumatol ; 32(6 Suppl 86): S-68-74, 2014.
Article in English | MEDLINE | ID: mdl-25152080

ABSTRACT

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.


Subject(s)
Contracture/physiopathology , Range of Motion, Articular/physiology , Scleroderma, Diffuse/physiopathology , Scleroderma, Limited/physiopathology , Adult , Aged , Ankle Joint/physiopathology , Blood Sedimentation , C-Reactive Protein/metabolism , Cohort Studies , Contracture/etiology , Echocardiography , Electrocardiography , Female , Follow-Up Studies , Hand Joints/physiopathology , Hip Contracture/etiology , Hip Contracture/physiopathology , Humans , Knee Joint/physiopathology , Linear Models , Lung Diseases, Interstitial/etiology , Lung Diseases, Interstitial/physiopathology , Male , Middle Aged , Scleroderma, Diffuse/complications , Scleroderma, Diffuse/metabolism , Scleroderma, Limited/complications , Scleroderma, Limited/metabolism , Scleroderma, Systemic/complications , Scleroderma, Systemic/metabolism , Scleroderma, Systemic/physiopathology , Shoulder Joint/physiopathology , Vital Capacity
11.
Orthopedics ; 37(4): e398-402, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24762848

ABSTRACT

Osteochondroma (or osteocartilaginous exostosis) is the most common bone tumor of childhood, with an incidence ranging from 1 to 1.4 per 1,000,000. In the lumbar spine, osteochondromata usually arise from the posterior column at the secondary ossification center and grow away from the spinal canal without causing neurologic deficits. This article reports a rare intraspinal lumbar osteochondroma that compressed the thecal sac, resulting in a hip flexion contracture in an 11-year-old boy. This lumbar, intraspinal, extradural exostosis was confluent with the L3 inferior articular process and compressed the L3 nerve root and thecal sac severely. The patient underwent an en bloc resection of the tumor with a right-sided hemilaminectomy of L3 and L4, a right-sided partial facetectomy at L3 to L4, and an extended resection from the pars intra-articularis of the L2 to the L5 vertebrae. The tumor specimen measured 4.8×3.7×2.5 cm with clear margins. Instrumented posterolateral fusion was completed from L2 to L5 due to iatrogenic instability from the resection. The patient had an uneventful recovery and returned to his normal activities of daily living, including sports. He remains asymptomatic at 54-month follow-up. A solitary lumbar osteochondroma that compresses the spinal cord, resulting in a motor neurological deficit, has not been reported in a pediatric patient. Orthopedic surgeons should be aware of potential intraspinal presentation of osteochondromas. Magnetic resonance imaging is the modality of choice in diagnosing and screening for spinal osteochondromas. These cases can be treated with resection surgery.


Subject(s)
Hip Contracture/etiology , Lumbar Vertebrae , Osteochondroma/diagnosis , Spinal Neoplasms/diagnosis , Humans , Male , Osteochondroma/complications , Osteochondroma/surgery , Spinal Neoplasms/complications , Spinal Neoplasms/surgery
12.
JNMA J Nepal Med Assoc ; 52(193): 702-6, 2014.
Article in English | MEDLINE | ID: mdl-26905552

ABSTRACT

INTRODUCTION: Adductor spasticity at hips is the main barrier in functional activities and rehabilitation of spastic cerebral palsy patients. The aim of this study is to evaluate the results of percutaneous adductor release under general anaesthesia. METHODS: From July 2005 to July 2010, 64 hips in 32 patients (19 males and 13 females) were recruited from outpatient department having adductor contracture at hips in cerebral palsy children. All children were operated under general anaesthesia. All children were followed for twenty-four months. The clinical results were evaluated radiologically, including measurement of CE- angle, AC-index and femoral head coverage and in terms of activity level of children. RESULTS: Of the thirty-two children, twenty-eight showed marked and immediate improvement. None of our children was functionally worse at follow-up. The CE-angle and femoral head coverage did not change significantly. The AC-index improved significantly (P = 0.01).The results were excellent in 12.5% children, good in 50%, fair in 25% and poor in 12.5%. CONCLUSIONS: Bilateral mini-invasive adductor release can be an effective treatment for children suffering from adductor contracture refractory to nonoperative management and early adductor release can prevent subluxation and possibly the need for future bony procedure on the proximal femur and pelvis.


Subject(s)
Cerebral Palsy/surgery , Hip Contracture/surgery , Mobility Limitation , Muscle, Skeletal/surgery , Thigh/surgery , Adolescent , Cerebral Palsy/complications , Cerebral Palsy/physiopathology , Child , Child, Preschool , Female , Hip Contracture/etiology , Hip Contracture/physiopathology , Humans , Male , Prospective Studies , Treatment Outcome
14.
Rheum Dis Clin North Am ; 39(2): 431-55, 2013 May.
Article in English | MEDLINE | ID: mdl-23597973

ABSTRACT

Mucopolysaccharidosis and other lysosomal storage diseases are rare, chronic, and progressive inherited diseases caused by a deficit of lysosomal enzymes. Patients are affected by a wide variety of symptoms. For some lysosomal storage diseases, effective treatments to arrest disease progression, or slow the pathologic process, and increase patient life expectancy are available or being developed. Timely diagnosis is crucial. Rheumatologists, orthopedics, and neurologists are commonly consulted due to unspecific musculoskeletal signs and symptoms. Pain, stiffness, contractures of joints in absence of clinical signs of inflammation, bone pain or abnormalities, osteopenia, osteonecrosis, secondary osteoarthritis or hip dysplasia are the alerting symptoms that should induce suspicion of a lysosomal storage disease.


Subject(s)
Lysosomes/enzymology , Mucopolysaccharidoses/diagnosis , Mucopolysaccharidoses/enzymology , Bone Diseases, Metabolic/etiology , Bone Diseases, Metabolic/pathology , Bone Diseases, Metabolic/physiopathology , Early Diagnosis , Hip Contracture/etiology , Hip Contracture/pathology , Hip Contracture/physiopathology , Hip Dislocation, Congenital , Hip Joint/pathology , Hip Joint/physiopathology , Humans , Joint Diseases/congenital , Joint Diseases/etiology , Joint Diseases/pathology , Joint Diseases/physiopathology , Joints/pathology , Joints/physiopathology , Mucopolysaccharidoses/complications , Mucopolysaccharidoses/physiopathology , Musculoskeletal Diseases/diagnosis , Musculoskeletal Diseases/enzymology , Musculoskeletal Diseases/etiology , Musculoskeletal Diseases/physiopathology , Osteochondrodysplasias/etiology , Osteochondrodysplasias/pathology , Osteochondrodysplasias/physiopathology , Osteonecrosis/etiology , Osteonecrosis/pathology , Osteonecrosis/physiopathology , Pain/etiology , Pain/pathology , Pain/physiopathology , Prognosis
15.
Gait Posture ; 37(4): 473-9, 2013 Apr.
Article in English | MEDLINE | ID: mdl-23079586

ABSTRACT

This study used the random forest algorithm to predict outcomes of intramuscular psoas lengthening as part of a single event multi-level surgery in patients with cerebral palsy. Data related to preoperative medical history, physical exam, and instrumented three-dimensional gait analysis were extracted from a historic database in a motion analysis center. Data from 800 limbs of patients with diplegic cerebral palsy were analyzed. An index quantifying the overall deviation in pelvic tilt and hip flexion was used to define outcome categories. The random forest algorithm was used to derive criteria that predicted the outcome of a limb. The criteria were applied to limbs that underwent psoas lengthening with outstanding results (accuracy=.78, sensitivity=.82, specificity=.73). The criteria were then validated using an extended retrospective case-control design. Case limbs met the criteria and underwent psoas lengthening. Control limbs met the criteria, but did not undergo psoas lengthening. Over-treated limbs failed the criteria and underwent psoas lengthening. Other-treated limbs failed the criteria and did not undergo psoas lengthening. The rate of good outcomes among Cases exceeded that observed among controls (82% vs. 60%, relative risk=1.37), and far exceeded that observed in Over-treated limbs (27%). Other-treated limbs had good outcomes 52% of the time. Application of the criteria in the future is estimated to increase the overall rate of good pelvis-hip outcomes from 58% to 72% among children with diplegia who undergo single-event multi-level surgery (SEMLS).


Subject(s)
Algorithms , Cerebral Palsy/physiopathology , Gait/physiology , Hip Contracture/surgery , Psoas Muscles/surgery , Case-Control Studies , Cerebral Palsy/complications , Child , Hip Contracture/etiology , Hip Contracture/physiopathology , Humans , Psoas Muscles/physiopathology , Retrospective Studies , Treatment Outcome
16.
Musculoskelet Surg ; 96(1): 17-21, 2012 Jun.
Article in English | MEDLINE | ID: mdl-22278604

ABSTRACT

Arthrogryposis represents a group of heterogeneous disorders, characterized by contractures of multiple joints at birth. Involvement of the hip is very common (55-90% of patients) ranging from soft tissue contractures to subluxation and dislocation. Isolated contracture of the hip can usually be managed conservatively: compensative mechanisms and contractures of other joints should be evaluated before planning surgery. For unilateral dislocations, open reduction is indicated to provide a level pelvis, even though the risk of stiffness, avascular necrosis and redislocation is significant. Indications for surgical reduction of bilateral dislocations are more controversial: an adequate evaluation of ambulatory potential of the child (muscular weakness, involvement of upper extremities, etc.) and of stiffness of the hips is suggested. An extensive and long-lasting programme of bracing, physiotherapy and multiple surgery may lead to some measure of functional ambulation in most cases, but high need for surgery and considerable risk of complications must be considered. This paper summarizes problems and principles of treatment for hip contractures and dislocations in arthrogryposis and provides a review of the current literature.


Subject(s)
Arthrogryposis/complications , Hip Contracture/therapy , Hip Dislocation, Congenital/therapy , Braces , Clubfoot/surgery , Femur Head Necrosis/etiology , Hip Contracture/congenital , Hip Contracture/etiology , Hip Contracture/surgery , Hip Dislocation, Congenital/etiology , Hip Dislocation, Congenital/surgery , Humans , Infant , Infant, Newborn , Physical Therapy Modalities , Treatment Outcome
17.
Musculoskelet Surg ; 96(2): 141-7, 2012 Aug.
Article in English | MEDLINE | ID: mdl-22237840

ABSTRACT

Treatment of chronic hip instability in children and young adults is challenging. Proximal femoral osteotomy had been suggested to provide pelvic stability and improved abductor function. Total hip replacement after pelvic support osteotomy can be challenging due to altered anatomy due to angulation of the proximal femur in both frontal and sagittal planes. This is a 29-year-old woman who had total hip replacement after pelvic support osteotomy. The patient had pelvic support at the age of 14 years. Pelvic support osteotomy delayed the need for total hip replacement for 15 years. Preoperative planning for total hip replacement with model was used for proper understanding of the anatomy of the proximal femur. Revision of femoral component was necessary due to penetration of the proximal femur. In conclusion, total hip replacement after pelvic support osteotomy is a technically demanding procedure, and careful attention to surgical details is necessary for successful outcome.


Subject(s)
Arthroplasty, Replacement, Hip , Hip Joint/surgery , Joint Instability/surgery , Osteotomy , Adult , Computer-Aided Design , Female , Hip Contracture/etiology , Hip Contracture/surgery , Humans , Ilizarov Technique , Leg Length Inequality/etiology , Leg Length Inequality/surgery , Locomotion , Models, Anatomic , Osteoarthritis, Hip/etiology , Osteoarthritis, Hip/surgery , Postoperative Complications/etiology , Postoperative Complications/surgery , Range of Motion, Articular , Recovery of Function , Time Factors
18.
J Bone Joint Surg Am ; 93(2): 150-8, 2011 Jan 19.
Article in English | MEDLINE | ID: mdl-21248212

ABSTRACT

BACKGROUND: this study was undertaken to determine the validity and reliability of the physical examination tests commonly used to measure hip flexion contracture in patients with cerebral palsy who are able to walk. METHODS: thirty-six consecutive patients (twenty-two male and fourteen female patients), with a mean age (and standard deviation) of 9.8 ± 3.9 years, who had cerebral palsy (level I, II, or III on the Gross Motor Function Classification System) and thirty-seven children without cerebral palsy (nineteen male and eighteen female subjects), with a mean age of 10.0 ± 3.0 years, were enrolled prospectively for this study. Hip flexion contracture was determined by three physical examination tests: the Thomas test, the prone hip extension test (the Staheli test), and the hamstring shift test. Three-dimensional gait analysis was performed in all subjects. The interobserver reliabilities of the three physical examination tests were determined with use of three observers. Convergent validity was assessed by evaluating the relationships between the findings on physical examination and kinematic and kinetic gait variables (maximum hip extension during stance and hip flexor index) and three-dimensional modeled psoas lengths. RESULTS: the Thomas test showed the highest intraclass correlation coefficient (0.501 in patients and 0.207 in controls) and the smallest mean absolute difference (5.8° in patients and 1.2° in controls). The Staheli test was found to be the most valid method in the patient group (r = 0.568 with hip flexor index), whereas the Thomas test was the most valid in the control group (r = 0.526 with maximum hip extension in stance, and r = 0.532 with the hip flexor index). The hamstring shift test had the lowest intraclass correlation coefficient and the lowest convergent validity. CONCLUSIONS: while the Thomas test showed the highest intraclass correlation coefficient and the smallest mean absolute difference, the Staheli test was the most valid method for detecting hip flexion contractures in patients with cerebral palsy. Although the Staheli test cannot be used for intraoperative assessment, we recommend that this test be included in preoperative physical examinations to determine the role of a hip flexion contracture in the abnormal gait of patients with cerebral palsy.


Subject(s)
Cerebral Palsy/diagnosis , Gait/physiology , Hip Contracture/diagnosis , Physical Examination/methods , Range of Motion, Articular/physiology , Adolescent , Case-Control Studies , Cerebral Palsy/complications , Cerebral Palsy/surgery , Child , Female , Hip Contracture/etiology , Hip Contracture/surgery , Humans , Male , Observer Variation , Prospective Studies , Reference Values , Reproducibility of Results , Severity of Illness Index
19.
J Pediatr Orthop ; 30(6): 562-7, 2010 Sep.
Article in English | MEDLINE | ID: mdl-20733421

ABSTRACT

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.


Subject(s)
Cerebral Palsy/complications , Gait Disorders, Neurologic/physiopathology , Hip Joint/physiopathology , Adolescent , Analysis of Variance , Child , Child, Preschool , Female , Gait Disorders, Neurologic/etiology , Hip Contracture/etiology , Hip Contracture/physiopathology , Humans , Knee Joint/physiopathology , Linear Models , Male , Range of Motion, Articular , Retrospective Studies , Young Adult
20.
Orthopedics ; 32(10)2009 Oct.
Article in English | MEDLINE | ID: mdl-19824593

ABSTRACT

Painful adductor muscle contracture is an important cause of failure during rehabilitation following total hip arthroplasty (THA). Adductor muscle contracture may be caused by postoperative muscle retractions, adhesive capsulitis, postoperative leg-length inequalities caused by implant failure, or preexisting hip pathologies. A 34-year-old woman experienced a persistent painful contracture into the left adductor magnus muscle after THA. She had no leg-length inequalities and, according to the Medical Research Council scale (grades 0-5), muscle strength of the quadriceps was 5/5 for the right side and 3/5 for the left. The degree of functionality according to the Harris hip score (HHS) was 16/100 in the left hip. The pain level, measured with the visual analog scale (VAS), was 7/10. The patient was unable to fully adhere to the rehabilitation program and walked with a limp during the stance phase of gait. After 7 days of treatment with injections of botulinum toxin type A into the left adductor magnus muscle (dose, 150 UM) and subsequent rehabilitation, a great reduction of painful contracture was observed (VAS score, 2/10). The procedure was well tolerated and no adverse effects were noted. After 20 days, hip articular range of motion and gait had improved (HHS score, 75/100). The clinical effects of botulinum toxin type A were present at 2-month follow-up. This treatment may be a viable alternative for the management of painful adductor muscle contracture after THA, without significant side effects.


Subject(s)
Arthroplasty, Replacement, Knee/adverse effects , Botulinum Toxins, Type A/therapeutic use , Hip Contracture/drug therapy , Hip Joint/drug effects , Neuromuscular Agents/therapeutic use , Postoperative Complications/drug therapy , Adult , Female , Hip Contracture/etiology , Hip Contracture/physiopathology , Hip Joint/diagnostic imaging , Hip Joint/physiopathology , Humans , Muscle, Skeletal/drug effects , Muscle, Skeletal/physiopathology , Pain/drug therapy , Pain/etiology , Pain/physiopathology , Postoperative Complications/etiology , Postoperative Complications/physiopathology , Radiography , Range of Motion, Articular , Severity of Illness Index , Treatment Outcome
SELECTION OF CITATIONS
SEARCH DETAIL
...