Myelodysplasia



- Discussion:
    - disorder of spinal cord development/closure or secondary rupture of developing cord secondary to hydrocephalus;
    - term myelodysplasia includes:
          - spina bifida occulta
          - meningocele: sac without neural elements protruding through defect;
          - myelomeningocele;
          - rachischisis (neural elements exposed without any covering);
    - in utero dx: increased alpha feto protein, w/ high risk infant;
    - risk factors:
          - folic acid antagonists:
                  - dihydrofolate reductase inhibitors: (displace folate from the enzyme and block the conversion of folate to active metabolites);
                          - aminopterin, methotrexate, sulfasalazine, pyrimethamine, triamterene, and trimethoprim;
                  - note that adequate intake of folate before and during pregnancy may prevent up to 85% of myelomenigocele deformities;
          - antiepileptic drugs: carbamazepine, phenytoin, primidone, and phenobarbital;

- Functional ability and evaluation:
    - related to level of defect & other assoc congenital abnormalities;
    - myelodysplasia level is based on the lowest functioning level;
    - L4 is key level because quads can function & allow community ambulation;
    - functional ability depends on:
          - quadriceps
          - scoliosis
          - pelvic obliquity
          - alignment of lower extremity and trunk
          - dislocated hips do not limit functio nor do they cause pain;
    - sudden changes:
          - changes in function, scoliotic progression,  or new neurologic deficit may be associated w/:
                  - hydrocephalus, (most common);
                          - consider head CT (70% of myelodysplastics have hydrocephalus);
                  - tethered cord;
                  - hydromyelia;


- Orthopaedic Manifestations of Myelomeningocele



Original Article: Influence of Prognosis on Decisions Regarding the Care of Newborns with Myelodysplasia.

Talectomy for equinovarus deformity in myelodysplasia.

Achilles tenodesis for calcaneus deformity in the myelodysplastic child.

Evaluation and Treatment of Diastematomyelia.

Diastematomyelia and structural spinal deformities.

Intraspinal rhizotomy and distal cordectomy in patients with myelomeningocele.

Congenital abnormalities of the urogenital tract in association with congenital vertebral malformations

Reduction in Neural-Tube Defects after Folic Acid Fortification in Canada




Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Wednesday, April 25, 2012 10:55 am