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PubMed1975

[Possibilities of treatment of arthrogryposis].

The results of the treatment of 90 patients with arthrogryposis are resumed. The possibilities of the conservative treatment are discussed and on the basis of their experiences and the data of the literature the details of the surgical technique are given for each articulation. Besides conservative therapy 86 different surgical interventions have been performed on 21 patients. The treatment necessitates utmost care and good collaboration is necessary between the doctor and the patient, or the patients relatives, resp. The therapeutical program yields often after several years satisfactory result.

Adolescent↗
PubMed1989

Arthrogryposis.

Arthrogryposis (multiple congenital contractures) has various causes and appears as many different syndromes. The basic classification for therapeutic and prognostic purposes divides affected children into those who have only limb involvement, those with limb and trunk, craniofacial or visceral involvement, and those with severe central nervous system dysfunction. Decreased fetal movement is an important etiologic factor. A multidisciplinary approach is required to evaluate these children, and early physical therapy is essential.

Arthrogryposis↗
PubMed1987

[Severe neonatal myasthenia with arthrogryposis].

Two children born to myasthenic mothers presented at birth with severe arthrogryposis, hypotonia and respiratory distress which followed a favourable course. A family history in one of these two cases and in another case reported in the literature raises the problem of a possible recurrence.

Adult↗
PubMed1988

Arthromyolysis of the elbow in arthrogryposis.

The authors discuss the indications and describe the technique of arthromyolysis of the elbow in arthrogryposis and report 17 operations performed on 10 patients followed up after an average of 4.5 years. Their results show that this type of operation considerably improves passive mobility of the elbow and is capable of conferring adequate function on the limb, even without recourse to subsequent muscle transposition into the paralysed biceps.

Activities of Daily Living↗
PubMedDate not supplied

Neuropathic arthrogryposis multiplex congenita and intrauterine ischemia of anterior horn cells: a hypothesis.

Three patients with multiple articular deformities due to arthrogryposis multiplex congenita (AMC) are presented. All had severe thalamic and tegmental degenerations with glial scars and shrunken ferruginated neurons; one had also unilateral porencephaly and polymicrogyri. The character and the pattern of the lesions were highly suggestive of in-utero hypotensive/ischemic insult. Marked attenuations of anterior spinal roots were mostly seen in the lumbosacral and cervical enlargements, where anterior horn cell loss and ferrugination were most severe. The changes in the anterior horns were situated peripherally, a distribution corresponding to the terminal ramifications of the sulcal (central) branches of the anterior spinal artery, an area known for its susceptibility to ischemia, especially in children. The changes in the brains and spinal cords could reasonably be explained on the basis of intra-uterine perfusion failure in early stages of fetal development. This assumption was supported by the fact that all three patients were subjected to unfavorable environmental factors during gestation. The first was the twin of a macerated infant; their entangled umbilical cords might have caused differential placental perfusion resulting in partial ischemia in the twin with AMC and severe ischemia in the other accounting for its early demise. The second patient had a single functional umbilical artery, a condition frequently associated with developmental anomalies. The third was born to a mother whose pregnancy was complicated by heavy substance abuse. Ischemic myelopathy during a critical phase of CNS developmental and muscle innervation is proposed as a cause of some cases of neuropathic AMC. Placental circulatory failure should be considered in the pathogenesis of AMC in such cases.

Arthrogryposis↗
PubMedDate not supplied

Management of hip dislocations in children with arthrogryposis.

From a review of 131 children with arthrogryposis, we studied 18 patients with hip dislocations. Fourteen patients (10 unilateral, four bilateral) were treated by open reduction. Mean age at surgery was 9.7 months, and follow-up ranged from 9 to 245 months. Range of motion of patients treated by open reduction following the medial approach was greater than in those treated by anterolateral incision and greater than in bilateral cases treated by closed reduction. Acetabular development was satisfactory following the medial approach and there were no re-dislocations, but one hip developed avascular necrosis.

Arthrogryposis↗
PubMed1985

Arthrogryposis. Pediatric review and habilitative aspects.

A precise diagnosis among the types of arthrogryposis must be made. For amyoplasia, the recurrence risk is nonexistent, the natural history favorable. During the first weeks, frequent stretching supported by maintenance casting and splinting of severe or generalized contractures is indicated in a residential multidisciplinary center. The key to deformity is the rigidity of the periarticular structures. With limb growth, contractures progress or recur. The growth years must therefore be dominated by physical management, which consists of stretching, casting, and bracing interspersed by staged soft-tissue procedures. At completion of limb growth, final corrections should be attained by osteotomies. Adolescence should be a time to follow social and prevocational pursuits in preparation for a place in society.

Adolescent↗
PubMed1985

Arthrogryposis multiplex congenita. A long-term follow-up study.

Most patients with arthrogryposis function well in adult life; however, many remain partially dependent upon others (parents, relatives, government subsidy). This dependency was more closely related to personality, education, and overall coping skills than to degree of physical deformity. Properly sequenced corrective surgical procedures are required to maximize musculoskeletal function. In addition to appropriate surgical correction, good family support, a proper educational environment, and promotion of independence at an early age are required to achieve maximal adult function.

Activities of Daily Living↗
PubMed1985

Genetic aspects of arthrogryposis.

Multiple congenital contractures or arthrogryposis is a birth defect that occurs in approximately one in 3000 births. It can be seen in isolation or in association with other abnormalities. The etiologic and genetic basis of multiple congenital contractures is very heterogeneous. In order to understand the genetic basis and natural history of a specific case, a specific diagnosis must be made. Over 150 conditions are known in which multiple congenital contractures are a predominant sign. In this chapter, the emphasis is on a systematic differential diagnosis and consideration of empiric recurrent risk figures if a specific diagnosis cannot be reached.

Abnormalities, Multiple↗
PubMed1985

Passive motion therapy for infants with arthrogryposis.

A comprehensive program for treatment of arthrogryposis multiplex congenita has been developed based on experiences with 95 infants and young children with arthrogrypotic deformities. The regimen emphasizes early passive stretching and serial splinting to improve joint motion. Ninety percent of the patients had contractures of all extremities; 40% had multiple congenital anomalies. Daily intensive passive stretching of joints and serial splinting have substantially increased patient function in this population. Surgical procedures have been used only in cases in which persistent deformities require correction.

Arthrogryposis↗
PubMed1985

Comprehensive management of arthrogryposis multiplex congenita.

Arthrogryposis multiplex congenita syndrome, characterized by multiple congenital joint contractures, is a manifestation of a neurogenic or myopathic disorder of unknown etiology. An accurate diagnosis is critical in management strategy. Muscle biopsy is usually the most important diagnostic procedure. Once the diagnosis is established, a team approach to comprehensive care is instituted, and the child is assisted in achieving maximum cognitive, physical, and social development. Physicians, nurse clinicians, therapists, social workers, and others constitute the team and work in coordination to develop an individual habilitation plan. The treatment plan is continually updated and modified as needed. Because the musculoskeletal deformities are generally the most prominent abnormality, the orthopedist usually has the major role in management. The orthopedist may serve as coordinator of the comprehensive care team and therefore must have a fundamental understanding of and global approach to management.

Arthrogryposis↗
PubMed1985

Management of upper limb problems in arthrogryposis.

The management of upper limb problems in arthrogryposis multiplex congenita (AMC) is described on the basis of the author's own experience gained from the care of a large number of affected children. Emphasis is placed on evaluation of the disability by repeated testing and observation. In many cases, the deformities will be accepted and improvements in function will be gained by orthoses or attention to details of seating, dressing, and toileting. Surgery is indicated in some cases, especially at the elbow, to obtain mobility, flexor power, or both. Arthrodesing the wrist is also useful in some cases. When surgery is indicated, it is often desirable to rearrange all three levels--shoulder, elbow, and wrist.

Arm↗
PubMed1985

The hip in arthrogryposis multiplex congenita.

The hip is involved in up to 80% of individuals with a diagnosis of arthrogryposis multiplex congenita. The hip deformity consists of contracture with or without dislocation. Isolated contracture can usually be treated conservatively by manipulation and splinting, only occasionally requiring operative intervention. Dislocation is as frequently bilateral as unilateral. Bilateral hip dislocations are best left unreduced; only accompanying contractures should be treated. The unilateral dislocation should be treated aggressively, because persistent dislocation will give rise to pelvic obliquity and scoliosis. Open reduction is always necessary.

Arthrogryposis↗
PubMed1985

The knee in arthrogryposis.

Of 104 patients with arthrogryposis multiplex congenita treated between 1952 and 1982, 74 had significant knee contractures, instability, or recurvatum. The minimum follow-up period was two years. Nonoperative treatment modalities included physical therapy, bracing, and serial casting in 43 patients (78 knees). Thirty-one other patients (46 knees) had 62 operative procedures, including hamstring and posterior capsular release, epiphysiodesis, distal femoral or proximal tibial osteotomy, arthrodesis, and knee disarticulation. Surgery was reserved for the more resistant causes and was recommended only as part of an overall treatment plan including the correction of foot and hip deformities. The average follow-up period for the surgically treated patients was 11 years, with a range of two to 20 years. Recurrence of deformity was frequent, with no one procedure being clearly favored. Physiotherapy alone rarely resulted in clinically significant improvement unless accompanied by prolonged casting and bracing. The most useful surgical procedure in the growing child was posterior capsular release performed in conjunction with hamstring tenotomy. Contractures treated by osteotomies before the completion of growth had a high rate of recurrence.

Arthrodesis↗
PubMed1985

Foot and ankle deformities in arthrogryposis multiplex congenita.

When 51 patients with arthrogryposis multiplex congenita followed an average of 12 years were reviewed, talipes equinovarus was the most common foot and ankle deformity. The best results were obtained by talectomy. The majority of tendo Achillis lengthenings, posteromedial releases, and triple arthrodeses were unsuccessful and were associated with a high complication rate. Congenital convex pes valgus was the second most common deformity. Surgical treatment by open reduction generally resulted in unsuccessful outcomes. Ambulation was achieved in 46 patients. Residual foot deformity was the primary reason for persistent difficulties in walking.

Achilles Tendon↗
PubMed1985

Coincidence of paternal 13pYq translocation and maternal increased 13p NOR activity in a child with arthrogryposis and other malformations.

Cytogenetic examination of a boy with congenital multiple arthrogryposis, VSD and dysmorphic facies revealed a probable t(Y; 13)(q?; p1) translocation and three NOR-positive dots on one of the chromosomes 13. The latter variant could be followed in the family of the mother, the 13/Y translocation was found in the relatives of the father. Since all the family members affected by one or the other cytogenetic anomaly were healthy, the abnormal phenotype of the propositus was interpreted as coincidence by chance.

Adult↗
PubMed1979

Feeding and toileting devices for a child with arthrogryposis.

Patients with arthrogryposis multiplex congenita have many problems with activities of daily living because of limited upper and lower extremity joint motion and muscle strength. Feeding and toileting are two activities frequently performed at home and at school that may require one or two people to assist the child. A feeding device and a toileting ramp made from readily available materials have improved a child's independence at home and at school, have been of cosmetic and psychological value, and have minimized the need for assistance by one or more people. The feeding device may also be used for writing and art activities. No future dental problems are anticipated with the use of the feeding device over a period of time. Fabricating and training time with the feeding device is about one hour for each process. The fabricating and training time with the toileting ramp is lengthier with several hours required for each process. Patients with extensive involvement from burns or muscular dystrophy can also benefit from use of the feeding device.

Arthrogryposis↗
PubMed1971

The syndrome of arthrogryposis multiplex congenita.

Arthrogryposis Multiplex Congenita (AMC) is a syndrome characterized by confenital rigidity or "clubbing" of multiple joints. Soft-tissue ankylosis restricts the motion of the joints, which are fixed in postures dictated by the position of the embryo during development. The joint malformations result from relatively brief immobilization of the developing embryo due to a variety of factors. This concept of pathogenesis derives support from the experimental reproduction of AMC in chick embryos paralyzed by a variety of pharmacologic agents. In man, prenatal disorders which are capable of immolilizing the embryo give rise to AMC. These disorders may affect the brain, spinal cord, peripheral nerves, or muscles. Various mechanical factors that restrict the embryo's movements can also produce joint malformations. Thus, AMC should be regarded as a syndrome of articular malformations which may stem from many different causes rather than as a disease entity.

Animals↗