Bilateral wrist dislocation in trisomy 21: a case report.

Ligamentous laxity is common in Trisomy 21 (Down's syndrome). The literature has numerous reports of atlanto-axial instability, patellar instability, carpal instability and other manifestations of joint instability. To date, no report has been published in the English literature describing non-traumatic unilateral or bilateral wrist dislocation either in trisomy 21 or in any other condition.

[Cervical spine trauma in patients with trisomy 21].

Atlantoaxial subluxation is predominantly found in trisomy 21. While neurological symptoms occur less frequently, fatal atlantoaxial instability with spinal cord compression has been described in trisomy 21 after minor trauma of the cervical spine. Presenting a special case we could demonstrate that atlantoaxial instability has to be ruled out in patients with trisomy 21 suffering from acute cervical spine trauma.

Treatment of Down syndrome-associated craniovertebral junction abnormalities.

Object: Operative intervention for craniovertebral junction (CVJ) instability in patients with Down syndrome has become controversial, with reports of a low incidence of associated neurological dysfunction and high surgical morbidity rates. The authors analyzed their experience in light of these poor results and attempted to evaluate differences in management.

Methods: Medical and radiographic records of 36 consecutive patients with Down syndrome and CVJ abnormalities were reviewed.

[A radiological study of the cervical alterations in Down syndrome. New findings on computerized tomography and three dimensional reconstructions].

Objective: We studied a large proportion of the population in our health district who have Down's syndrome to determine the incidence and variety of changes in the spine and to define the guidelines for preventive diagnosis advisable in relation to atlanto-axial instability, a common disorder in these patients.

Patients And Methods: First phase: a plain X-ray of the cervical spine in a neutral lateral projection and in flexion in 188 patients, measuring the atlanto-odontoid distance. Second phase: computerized tomography (CT) studies and three dimensional reconstructions in 25 patients (13.

Down syndrome and craniovertebral instability. Topic review and treatment recommendations.

The diagnosis and management of occipital-atlantal and atlantoaxial instability in Down syndrome patients is a challenging problem in pediatric spine surgery. To date, no systematic review of this topic has been presented on this confusing and sometimes contentious issue. This topic review will focus on the biomechanical and radiographic foundations for which treatment recommendations in Down syndrome patients are made.

Ossification of the posterior longitudinal ligament in Down's syndrome.

Cervical myelopathy in patients with Down's syndrome is not uncommonly the result of atlanto-axial instability, a condition that is caused by ligamentous laxity and which may be associated with congenital osseous anomalies at the occipito-atlanto-axial axis. Ossification of the posterior longitudinal ligament (OPLL) is well described, particularly in the Japanese population, and may be associated with diffuse idiopathic skeletal hyperostosis. This is the first case, to our knowledge, of OPLL occurring in a person with Down's syndrome presenting with myelopathy.

Should children with Down syndrome be screened for atlantoaxial instability?

In 1995, the Committee on Sports Medicine and Fitness of the American Academy of Pediatrics (AAP) published a position paper on atlantoaxial instability in children with Down syndrome in which a previous statement on the same subject published in 1984 (Table) was retired. The 1995 statement includes several arguments that disfavor screening of children with Down syndrome for atlantoaxial instability. Whereas some of these arguments are well founded, other lack substantive evidence that would support the statement.

Upper cervical spine fusion in the pediatric population.

The outcomes of 25 pediatric patients who underwent upper cervical or occipitocervical fusion at the authors' institution since 1983 were reviewed. At a mean age of 9 years, the patients presented with spinal instability that was associated with os odontoideum in 11 cases, rotatory subluxation in five cases, odontoid fracture in two cases, atlantooccipital dislocation in two cases, and congenital atlantoaxial instability in five patients, four of whom had Down's syndrome (trisomy 21). Ten children had abnormal findings on neurological examination preoperatively; however, nine experienced improvement or resolution of deficits as of their latest follow-up evaluation (mean 17 months).

Primary care of adults with mental retardation living in the community.

An increased number of adults with mental retardation are living in the community and seeking health care from family physicians. When mentally retarded patients are enrolled in a medical practice, guardianship status should be determined, but these patients should be involved in their own care to as great an extent possible. Since a verbal history may be difficult to obtain, a systematic, thorough physical examination is important.

Screening of children with Down syndrome for atlantoaxial (C1-2) instability: another contentious health question.

Screening for atlantoaxial (C1-2) instability in children with Down syndrome before their participation in sport is called into serious question. Despite the lack of clear supporting data to justify screening, it has been widely recommended and enforced by some sporting organisations for several decades. Experience in New South Wales during the past 25 years strongly suggests that routine screening should be discontinued, and the existing literature supports this move.

Occipitoatlantal and occipitoaxial hypermobility in Down syndrome.

Study Design: In this study, the authors evaluated upper cervical spine in 75 children and adolescents with Down syndrome on the basis of lateral flexion-extension radiographs.

Objective: To assess occipitoatlantal motion and occipitoaxial motion in children and adolescents with Down syndrome compared with age-matched control subjects.

Summary Of Background Data: Although previous studies have described a high prevalence of occipitoatlantal hypermobility in Down syndrome, there have been no comparisons with age-matched control subjects.

Complications and long-term outcome of upper cervical spine arthrodesis in patients with Down syndrome.

Study Design: A retrospective review of 15 patients with Down syndrome who had undergone arthrodesis of the upper cervical spine for instability.

Objectives: To determine the complication rate and long-term outcome after posterior cervical arthrodesis for upper cervical instability in patients with Down syndrome.

Summary Of Background Data: Atlantoaxial instability is common in patients with Down syndrome, and fusion of the upper cervical spine has been recommended for patients who have instability, with or without myelopathy.

Posterior spinal arthrodesis for atlantoaxial instability in Down syndrome.

Nine children with Down syndrome who had atlantoaxial instability underwent posterior spinal fusion. At follow-up, all patients had stabilization or improvement of their neurologic symptoms. Evaluation of the spine using flexion and lateral radiographs, as well as selective cineradiography, showed no instability over the fused area or adjacent motion segments.

Clubfoot deformity in Down's syndrome.

The association of Down's syndrome (trisomy 21) with clubfeet has not previously been elaborated. Eight patients with a total of 15 clubfeet were identified for review. Five of these had trisomy 21 noted by chromosomal analysis, and 1 had a mosaic pattern.

Atlantoaxial instability in Down's syndrome: a five year follow up study.

In 1986 all 90 children aged 4-19 years with Down's syndrome attending school in the area served by the Southern Derbyshire Health Authority underwent radiography to identify atlantoaxial instability (AAI). This study details repeat observations five years later. Full results were available on 67 (74%), information on health status was available on the remaining 19 (21%); four (4%) were untraced.

Preoperative evaluation of the cervical spine in children with trisomy-21: results of a questionnaire study.

Atlantoaxial subluxation (AAS) is a form of cervical spine instability predominantly found in persons with Trisomy-21. Several case reports describe the occurrence of acute AAS in the perioperative period. Some authors have recommended cervical spine radiographs prior to elective surgery in all children with Trisomy-21.

Neurologic sequelae secondary to atlantoaxial instability in Down syndrome. Implications in otolaryngologic surgery.

Objective: A comprehensive overview of atlantoaxial instability in Down syndrome as it relates to head and neck surgery and recommendations as derived from a review of the literature.

Data Sources: English-language literature: otolaryngologic problems in Down syndrome and atlantoaxial biomechanics.

Data Synthesis: Children affected with Down syndrome frequently require otolaryngologic procedures such as myringotomy with ventilation tubes or adenotonsillar surgery.

Radiological assessment of the atlantoaxial distance in Down's syndrome.

People with Down's syndrome are pre-disposed to atlantoaxial instability. As part of a study to determine whether those with Down's syndrome should be screened for atlantoaxial instability before they participate in sport, a series of 279 children, aged 6 to 17 years was investigated radiologically. Lateral radiographs of the cervical spine were taken in neutral position and in flexion.

Risk of sports activities in children with Down's syndrome and atlantoaxial instability.

10-40% of children with Down's syndrome have atlantoaxial instability. These children might run the risk of spinal cord compression if they play sport. The aim of our study was to assess this presumed risk.

[A girl with Down syndrome complicated by moyamoya disease and symptomatic atlanto-axial instability].

We reported a girl of Down syndrome with both moyamoya disease and spinal cord compression due to atlanto-axial instability. At the age of 3 years, she presented with muscle weakness of gradual onset, and could not walk without support. On admission, at the age of 6, she presented with spastic paraparesis with predominance on the right side.

No relation between general laxity and atlantoaxial instability in children with Down syndrome.

Atlantoaxial instability in children with Down syndrome (DS) may be caused by laxity of the transverse ligament. We tested the hypothesis that general laxity might predict atlantoaxial instability. General laxity and the atlantoaxial distance were assessed in 172 children with DS (aged 6-17 years; 45% girls, 55% boys).

Atlantoaxial subluxation and syringomyelia in Down syndrome: report of one case.

Atlantoaxial instability is a relatively frequent finding in Down syndrome, but syringomyelia is a rare symptom. A four-year-old girl with Down syndrome was noted to have one year history of torticollis and progressive motor deterioration presenting with abnormal gait, ataxia or quadriparesis for the past three months. She was admitted because of acute urinary retention.