OWL: OCOSH Classification/Bone Diseases/Spinal Diseases/Spinal Dysraphism/Spina Bifida Cystica

Associations between orthopaedic findings ambulation and health-related quality of life in children with myelomeningocele

Danielsson et al — 2009-11-13 07:00:47 GMT

PURPOSE: Modern principles for treatment of patients with myelomeningocele include early closure of the neural tube defect, neurosurgical treatment of hydrocephalus and treatment aimed at minimizing contractures and joint dislocations. The aim is to achieve a better survival rate and a better quality of life (QOL). Better ambulatory function is thought to improve the management of activities of daily living. This study focused on evaluating which factors might affect ambulation, function and health-related QOL in children with myelomeningocele. METHODS: Thirty-eight patients with neurological deficit from myelomeningocele were examined in an unbiased follow-up. This included a physical examination using validated methods for ambulatory function and neuromuscular status, chart reviews and evaluation of radiographs in terms of hip dislocation and spine deformity. The Pediatric Evaluation of Disability Inventory (PEDI) was used to measure mobility, self-care and social function, and the Child Health Questionnaire (CHQ-PF50) was used to measure QOL. RESULTS: Muscle function class, quadriceps strength, spasticity in hip and/or knee joint muscles and hip flexion contracture as well as the ambulatory level all affected functional mobility as well as self-care/PEDI. Patients with hip dislocation, spinal deformity or those who were mentally retarded also had significantly worse functional mobility. Besides being affected by the severity of the neurological lesion, self-care/PEDI was significantly impaired by hip flexion contracture and absence of functional ambulation. General health-related QOL was significantly lower in this patient group than for US norms. Nonambulatory and mentally retarded patients had a significantly lower physical function of their QOL (CHQ). CONCLUSIONS: The severity of the disease, i.e. reduced muscle strength and occurrence of spasticity around hip/knee, affected ambulation, functional mobility and self-care. Acquired deformities (hip dislocation and spine deformity) affected functional ambulation only. Patients with reduced functional mobility and self-care experienced lower physical QOL. Children with myelomeningocele had significantly reduced QOL compared to healthy individuals.
Associations between orthopaedic findings, ambulation and health-related quality of life in children with myelomeningocele. Danielsson AJ, Bartonek A, Levey E, McHale K, Sponseller P, Saraste H. J Child Orthop. 2008 Feb;2(1):45-54.

Congenital Kyphosis Myelomeningocele

Carlos Villanueva — 2005-07-17 07:00:47 GMT

Congenital Kyphosis Myelomeningocele

Carlos Villanueva Spain Web Archive copy last posted 2005

Meningomyelocele Part 1 Orthopedic management in children

Specht — 2007-10-28 07:00:47 GMT

Goals of orthopaedic management are

Straight spine, legs and feet
Mobile hips
Pull-to or swing through gait
Maximum possible sefl sufficiency

West J Med 121:281-291 1974

Meningomyelocoele LSBU

IDM — 2002-01-31 07:00:47 GMT

Radiology case 338-1452 Clinical presentation: Young adult female with a large head and in a wheel chair.
There are symmetrical defects in the ilia. The lumbar canal is widened at its lower end with the increased interpedicular distance just visible in this view. A wide defect visible in the bone density of the lumbar spine with an absence of normal posterior structures behind the 4th and 5th lumbar vertebrae. Both hips are deformed. The right hip is dislocated proximally with a valgus deformity and acetabular dysplasia. The left hip os deformed and fused. The patient is constipated and there is a well-defined soft-tissue density over the right ilium that has a clear inferior silhouette beyond the abdominal wall.

Myelomeningocele eMedicine PMR

Kolaski — 2007-10-28 07:00:47 GMT

Author: Kat Kolaski, MD, Assistant Professor, Departments of Orthopedics and Pediatrics, Wake Forest University School of Medicine
Myelomeningocele is a complex congenital spinal anomaly that causes varying degrees of spinal cord malformation, or myelodysplasia. It is commonly referred to as spina bifida and is classified as a defect of the neural tube (ie, the embryonic structure that develops into the spinal cord and brain). Patients with myelomeningocele present with a spectrum of impairments, but the primary functional deficits are lower limb paralysis and sensory loss, bladder and bowel dysfunction, and cognitive dysfunction. Medical, surgical, and rehabilitation issues arise in the patient with myelomeningocele from birth through adulthood.
Synonyms and related keywords: open spinal dysraphism, neural tube defect, myelodysplasia, spina bifida, spina bifida cystica, spina bifida aperta, open spina bifida

Myelomeningocele Scoliosis Wheeless

Wheeless — 2007-10-28 07:00:47 GMT

scoliosis in myelomenigocele may have a multifactorial etiology:
- idiopathic causes;
- myelomeningocele structural defect;
- congenital defects
- paralytic etiology;
- 15-20% of children who have myelomeningocele have congenital vertebral abnormalities in addition to congenital scoliosis;
- over-all incidence of scoliosis in pts who have thoracic-level lesion is 85%;
- all children who are born with an osseous dysraphism at level of T12 or higher, are at risk for the development of a severe scoliotic deformity for which arthrodesis is needed;

Orthopaedic Manifestations of Myelomeningocele Wheeless

Wheeless — 2007-10-28 07:00:47 GMT

- Discussion of Myelodysplasia
- Spinal Deformities
- Lower Extremity
- hip in myelomeningocele:
- fractures:
- myelodysplastic knee:
- foot deformities in myelomeningocele:

Spina Bifida Global-HELP

Not Available — 2008-08-21 07:00:47 GMT

Health Education using Low-cost Publications
Currently the publications in this category are in Turkish

Spina Bifida OrthoFracs

Not Available — 2009-09-28 07:00:47 GMT

Definition
Neural tube defects can be grouped under the following terms
Spina bifida - Congential spinal disorder where the two halves of posterior arches fail to fuse leading to bony abnormality
Spinal dysraphism - Failure of the neural tube to close
Myelodysplasia - Any developmental defect of the spinal cord

Spina Bifida Wheeless

C.R.Wheeless — 2002-02-04 07:00:47 GMT

- Spina Bifida refers to a local failure of primordia of the two laminae to unite leaves vertebral canal open dorsally (spinal rachischisis, or spina bifida);
- dorsal body wall may or may not be intact over open vertebral canal;
- sub-types:
- spina bifida occulta:
- defect involves primarily bone, but skin may be attached to Dura, spinal cord, or nerve roots by fibrous bands;
- spina bifida cystica:
- neural tissue may be exposed (myeloschisis)
- meninges may protrude (meningocele)
- spinal cord may protrude (myelomeningocele);
- diastematomyelia:
- related to spina bifida is condition known as diastematomyelia, in which the spinal cord is split by bony spicule or a fibrous band, w/ each portion surrounded by a dural sac;
- as child grows, a long spicule of bone or a fibrous band may tether spinal cord and produce neurologic deficits;
Wheeless' Textbook of Orthopaedics

Spinal Dysraphism Myelomeningocele eMedicine Radiology

Khan et al — 2007-10-28 07:00:47 GMT

Spinal dysraphism can be classified as closed forms or open forms, which include myelocele, meningocele, and myelomeningocele. These open forms are often associated with hydrocephalus and Arnold-Chiari malformation type II and may be classified as spina bifida aperta. Von Recklinghausen (1886) gave a detailed account of spina bifida cystica (aperta). Spina bifida is described in the medieval literature, although the condition was recognized even earlier.
Synonyms and related keywords: neural tube defects, NTD, open neural tube defects, ONTD, myelocele, meningocele, myelomeningocele, spina bifida cystica, closed neural tube defects, spina bifida occulta, tethered cord, filum terminal syndrome, cord traction syndrome, diastematomyelia, diplomyelia

Surgical Release of knee flexion contractures in myelomeningocele

Marshall et al — 2007-10-28 07:00:47 GMT

We report the results of a prospective study of the surgical release of 45 knee flexion contractures in 28 patients with myelomeningocele... We conclude that surgical release of knee flexion contractures in myelomeningocele improves gait in all children who walk, particularly those with low lumbar lesions. Recurrence of knee flexion contractures after surgical release is most common in those with thoracic lesions who do not achieve independent walking.
JBJS B 1996 78 B 912-6

Wheeless Myelomeningocele Club Foot

Wheeless — 2006-12-11 07:00:47 GMT

most common foot deformity in myelomeningocele;
- teratological club-foot deformity in children w/ myelomeningocele is often more severe and more rigid than seen in congenital club foot deformity;
- secondary foot deformities may also develop as a result of muscle imbalance and/or spasticity