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PubMed1985

[Steindler-plasty in paralysis of the elbow flexor muscles].

In 1918, Steindler described an operation technique in which the origins of the wrist flexors are displaced proximally from the ulnar epicondyle. In this way, these muscles attain a usable flexor function at the elbow joint. In the last seven years, we have carried out this surgical technique in nine patients. These comprised patients with incomplete upper arm plexus paresis, poliomyelitis and congenital multiple arthrogryposis. The active flexor function of the elbow joint differed in strength but always led to a marked improvement in use of the affected limb. The biomechanical problems of this surgical technique are commented on.

Adolescent↗
PubMed1985

A plan of management and treatment results in the arthrogrypotic hip.

In a retrospective review of 52 hips in 26 patients with arthrogryposis, 65% of all hips studied were affected by the disorder; 46.6% of the patients were independent ambulators, 33.3% required some type of bracing for ambulation, and 20% were confined to a wheelchair. Based on the results of the authors' treatment as determined by a functional hip score, the authors recommend a one-stage open reduction, varus shortening femoral derotational osteotomy for all unilateral hip dislocations and those bilateral hip dislocations that have a less severe generalized involvement. They also recommend an aggressive traction program with appropriate soft-tissue releases during the neonatal period for patients with subluxation and/or marked limitation of motion of the hip.

Adolescent↗
PubMed1978

Deformities of chick embryos in experimental Akabane virus infection.

A material containing 10(3.0) approximately 10(5.0) TCID50 of Akabane virus was inoculated into 6-day-old chick embryos by the yolk sac route. Death of embryos did not increase in the course of embryonic development till 18 days of age, as compared with control groups. Later than 18 days of age, however, the numbers of dead and peeping but unhatched embryos increased, making the hatching rate significantly low. Deformities, such as arthrogryposis and hydranencephaly, appeared in almost all the dead and unhatched embryos. They were severe in dead embryos, considerably severe in peeping unhatched embryos, and comparatively mild, though highly frequent, in affected hatched chicks. Many of the hatched chicks manifested ataxia, abnormal gait, astasia, or tremor of body or legs separately or together. Virus growth was demonstrated in chick embryos inoculated at 7 days of age. The virus titer was the highest (10(3.25 approximately 10(3.75) TCID50/O.1g) in head, trunk, and muscle, and the second highest (10(2.0) approximately 10(2.5) in brain, heart, and other visceral organs. It was the highest (10(4.0) approximately 10(4.5)) in muscle and a mixture of cerebellum and brain stem in embryos inoculated at 8 days of age.

Abnormalities, Multiple↗
PubMed1980

[Radiological appearances of congenital malformations of the hand (author's transl)].

Pre- and post-operative radiological appearances of various isolated congenital malformations of the hand are described, as observed in a personal series of 52 cases. Six groups of malformations can be distinguished, and are, in decreasing order of frequency: agenesias, syndactylies, duplications, amniotic bands, arthrogryposis, and macrodactyly.

Adult↗
PubMed1981

Teratogenic effects of Nicotiana glauca and concentration of anabasine, the suspect teratogen in plant parts.

Nicotiana glauca consumption by pregnant cows or sows produced arthrogryposis and other congenital deformities in their offspring. The plant material administered contained 0.11% anabasine, the suspect teratogen. But concentration varied among various plant parts; woody material was very low but leaves and bark were high. The anabasine was identified by mass spectral fragmentation analysis.

Anabasine↗
PubMed1981

Nicotiana glauca-induced congenital deformities in calves: clinical and pathologic aspects.

Seven calves born to 7 cows fed Nicotiana glauca during portions of the 1st trimester of gestation were deformed at birth. Deformities increased in severity as the calves aged. At birth, calves typically had arthrogryposis of the forelimbs or curvature of the spine; severity varied among calves. In 4 calves necropsied at about 15 months of age, there was general malpositioning and misalignment of the distal ends of the radius and ulna and the proximal ends of the metacarpal bones. Carpal joints were severely affected, fetlock joints were moderately affected, and pastern joints were slightly affected, with lateral rotation of forelimbs common; severity varied among calves. In 1 of the 4 calves, there was moderate torticollis and scoliosis resulting from wedging of some of the cervical and thoracic vertebrae and abnormal cranial curvature of the left thoracic ribs. Histologic changes were not noticed in muscle, brain, spinal cord, or endocrine organs of the 4 calves.

Abnormalities, Multiple↗
PubMed1995

Fetal hypokinesia syndrome in the monochorionic pair of a triplet pregnancy secondary to severe disruptive cerebral injury.

We report a set of triplets, two of whom were monochorionic diamnionic and demonstrated cerebellar hypoplasia and progressive arthrogryposis on an antenatal sonogram. At delivery the infants exhibited a Pena-Shokier phenotype. At autopsy, the twins were concordant for severe disruptive lesions of the cerebrum. The mechanism resulting in the devastating symmetric lesions may have been a transient cerebral vascular compromise associated with placenta vascular anastomoses characteristic of monochorionic twinning. This report accentuates the vulnerability of the monochorionic twin for ischemic cerebral injury.

Abnormalities, Multiple↗
PubMed1994

Ambulatory monitoring of the sudden death of an adolescent with hypertrophic cardiomyopathy.

Sudden death is an important cause of mortality in hypertrophic cardiomyopathy. Several mechanisms have been proposed, but ambulatory monitoring of the event has rarely occurred. The case of an adolescent girl with congenital hypertrophic cardiomyopathy and arthrogryposis multiplex congenita is presented. Despite appropriate therapy and severe activity limitation, the patient's condition progressively worsened. The patient died suddenly in her sleep, one month after a myocardial myomectomy for subaortic stenosis. The fortuitous ambulatory electrocardiographic (ECG) monitoring of the event recorded polymorphic ventricular tachycardia degrading into ventricular fibrillation. Retrospective review of previous ambulatory ECG revealed the presence of nonsustained ventricular tachycardia, a predictive determinant for sudden death, and atrioventricular node dysfunction. However, the patient had never reported symptoms during the dysrhythmias. The pathological specimen showed an unexpected old apical infarction scar, suggesting that her preoperative cardiac status was worse than suspected. In summary, the mechanism of sudden death in this patient was revealed by ambulatory ECG monitoring, and the involvement of neural and myocardial factors are proposed.

Adolescent↗
PubMed1993

Congenital dislocation of the knee. Its pathologic features and treatment.

Arthrograms and operative findings of 19 patients with 26 congenital dislocations of the knee (CDK) were reviewed to clarify the pathologic features, methods of treatment, and the optimum treatment for the patient with multiple joint deformities. The fundamental pathologic features in CDK involved a shortening of the quadriceps femoris tendon, a tight anterior articular capsule, and hypoplasia of the suprapatellar bursa. Valgus deformity of the knee, which was observed in half of the patients, was caused by the fragility of the medial components of the knee. This fragility was attributable to the forward displacement of the tibial insertion of the medial components. Arthrograms served as effective indicators when evaluating the pathologic features and determining the applicability of surgical therapy. There were limits to conservative therapy that were dependent on the extent of the pathologic changes. In extremely refractory patients with arthrogryposis multiplex congenita (AMC), an elimination of genu recurvatum and an improved range of movement could be obtained by the use of surgery. The valgus deformity was significantly reduced by invasive treatment that reinforced the fragile inner components. Experience with nine patients with multiple lower-limb joint deformities indicates that the knee dislocation should be treated before any of the other deformities.

Abnormalities, Multiple↗
PubMed1996

[Permanent constriction of the jaws in children: 3 cases with extra-articular etiology].

Three cases of permanent constriction of the jaw are reported. One was a sequela after a noma, the second occurred in a child with arthrogryposis and the third was an inborn malformation. Each case raised specific problems for diagnosis and treatment. Mechanicotherapy is essential postoperatively and requires a specific electric apparatus to mobilize the joint.

Arthrogryposis↗
PubMed1997

A variant of Freeman-Sheldon syndrome maps to 11p15.5-pter.

Distal arthrogryposis type 1 (DA1) and Freeman-Sheldon syndrome (FSS) are the two most common known causes of inherited multiple congenital contractures. We recently have characterized a new disorder (DA2B) with a phenotype intermediate between DA1 and FSS. We report the mapping of a gene that causes DA2B to chromosome 11p15.5-pter. Linkage analysis in a single kindred generated a positive LOD score of 5.31 at theta = 0 with the marker D11S922, and recombinants localize the gene to an approximately 3.5-6.5-cM region between the marker TH and the telomere. Analysis of additional families improves the LOD score to 6.45 at theta = 0 and suggests linkage homogeneity for DA2B.

Abnormalities, Multiple↗
PubMed1997

Surgical treatment of congenital convex pes valgus.

Congenital convex pes valgus is a rare, complex and heterogeneous anomaly of the foot, which is difficult to treat. From 1985 to 1994, we treated 14 patients (20 feet) with this deformity. There were six boys and eight girls. Six patients had bilateral involvement. Their ages at the time of surgery ranged from 4 to 32 months (mean, 13 mo). In eight patients (10 feet), the etiology was arthrogryposis multiplex congenita. The eitiology was unknown in six patients (10 feet). Associated problems included hip dislocation in seven patients, flexion or extension contractures of the knee in six patients, and clubfoot deformity in three patients. One patient had undergone previous surgery for release of a knee contracture. To achieve a plantigrade and balanced foot, all patients had one-stage surgical open reduction and circumferential release for the correction of deformities. At a minimum of 2 years following surgery, 11 patients (16 feet) had satisfactory results determined by a 10-point evaluation system based on both clinical and radiographic features. Satisfactory results and ambulatory prognosis were related to the etiology and severity of each patient's condition. Two patients (two feet) had scars with poor cosmetic appearance and two patients (three feet) had inadequate correction. We concluded that with adequate soft tissue release and complete talocalcaneonavicular joint reduction in one-stage surgery, proper postoperative maintenance, and physical therapy, satisfactory results in the treatment of congenital convex pes valgus can be achieved.

Arthrogryposis↗
PubMed1998

Disorders of the upper extremity in children.

This article presents a brief overview of the indications of MR imaging in a variety of disorders of the upper extremity of the pediatric patient. This covers congenital anomalies: Sprengel shoulder, Poland sequence, arthrogryposis; posttraumatic lesions of cartilage, bone, tendon, muscle and nerve including the brachial plexus injury; inflammatory arthritis and synovitis; bone and joint infection; osteochondritis dissecans, bone necrosis and infarcts in sickle cell anemia and juvenile Gaucher disease, as well as tumors. In this last category, the authors briefly describe the appearances of cysts and tumors of bones and soft tissues of the upper extremity. Indications for the intravenous administration of Gadolinium are given throughout the article with emphasis on the synovial enhancement seen in active arthritis and synovitis.

Adolescent↗
PubMed1998

Teratogenic and fetotoxic effects of two piperidine alkaloid-containing lupines (L. formosus and L. arbustus) in cows.

Cleft palate and minor front limb contractures were induced in calves by maternal ingestion of the piperidine alkaloid-containing lupines, Lupinus formosus and L. arbustus. Crooked calf disease, which includes an occasional cleft palate, is a congenital condition of widespread occurrence in cattle in the western U.S. and Canada. It is known to occur after maternal ingestion of certain species of Lupinus during specific gestational periods. Although many lupine species contain quinolizidine alkaloids including the teratogenic alkaloid anagyrine, L. formosus and L. arbustus produce piperidine alkaloids including the reported teratogen ammodendrine. In addition to ammodendrine, L. formosus contains both N-acetyl hystrine and N-methyl ammodendrine, whereas L. arbustus contains ammodendrine, trace amounts of N-methyl ammodendrine, and no N-acetyl hystrine. L. formosus and L. arbustus were fed to pregnant cows at equivalent ammodendrine doses during a 10-day period from days 40-50 of gestation. One calf from a cow fed L. formosus had a full cleft palate. Embryonic death and resorption of one fetus and minor front limb contractures (arthrogryposis) in another calf occurred with two cows fed L. arbustus. Alkaloid analysis of blood samples taken during the feeding period, and up to and including 48 hours after the last dose, demonstrated comparative plasma elimination times with N-methyl ammodendrine > ammodendrine > N-acetyl hystrine. The objectives of this experiment were to: 1) determine if N-acetyl hystrine is a potential teratogen; and 2) define the narrow cleft palate induction period in cows.

Alkaloids↗
PubMed2005

Restrictive dermopathy associated with transposition of the great arteries and microcolon: a rare neonatal entity with new symptoms.

BACKGROUND: Restrictive dermopathy is a very rare autosomal recessive skin disorder. The typical pathologic findings are striking: microstomia, micrognathia, thin but very tight translucent skin that tears spontaneously, and arthrogryposis multiplex. The mechanisms behind this disease are unknown. OBSERVATIONS: We describe for the first time a newborn girl with restrictive dermopathy, transposition of the great vessels, and microcolon. She had thin shiny skin with nearly no compliance indicating restrictive dermopathy. Additional dysmorphic findings included enlarged fontanelle, hypertelorism, absent eyelashes, small pinched nose, microstomia, micrognathia, dysplastic ears, pterygium colli, dyplastic fingers and toes with upper- and partial lower-limb flexion contractures, dysplastic genitalia, and muscular hypotonia. She also had left transposition of the great artery with small atrial septal defect, bilateral hypoplasia of the first rib, and congenital stenosis of the small bowel with microcolon. CONCLUSIONS: The pathogonomic diagnostic features remain reduced dermal thickness and nearly complete absence of elastic fibers in the dermis. In mice, a defective fatty acid transport protein 4 gene (Fatp4) leads to clear signs of restrictive dermopathy by influencing the arrangement of the lipids in the epidermis. Whether the left transposition of the great artery is associated with restrictive dermopathy or represents an additional malformation of multifactorial, polygenetic, or monogenetic cause remains open.

Abnormalities, Multiple↗