Pediatric orthopedic cases 35

CASE                               35                               

 

A couple comes to you for counseling regarding their unborn child. They have been told that the child has spina bifida.

What should the family be told?

1. Your child will unlikely walk

2. Your child will not be of normal intelligence

3. You should have a natural birth

4. You should have a cesarean section

 

Discussion

The correct answer is (D). Myelomeningocele is a failure of closure of the spinal elements at 26 to 28 days of development or a re-opening of the spinal elements. Children with exposed neural elements should be born via cesarean section to avoid injury to the sac. Children with lumbar lesions usually can walk and those without hydrocephalus have normal intelligence. Also, outcomes are better if the defect is closed early within 24 hours of life.

Supplementation with what has decreased the incidence of myelomeningocele in the United States?

1. Vitamin A

2. Vitamin D

3. Folic acid

4. Vitamin B12

 

Discussion

The correct answer is (C). Myelomeningocele is associated with folate deficiency. There has been a 23% decrease in spina bifida since the supplementation of grains in the United States in 1998. There is also an increased incidence in first-degree relatives.

The child is born with a thoracic myelomeningocele and clubfoot deformity. What is the recurrence rate after Ponseti casting?

1. 10%

2. 26%

3. 44%

4. 68%

 

Discussion

The correct answer is (D). Children with high-level lesions often have clubfoot. Sixty-eight percent of patients recur with approximately one-third of those requiring a posteromedial release. Other foot conditions associated with myelomeningocele are calcaneovarus, calcaneovalgus, and vertical talus. The goal of treatment is a braceable foot that is plantigrade. This is critical due to their decreased sensation on their feet and tendency to ulcerate.

At the age of 4, she is not walking. Both hips are dislocated. What should be done?

1. Observation

2. Closed reduction in the OR with spica cast

3. Open reduction in the OR with spica cast

4. Dega osteotomy, proximal femoral osteotomy, and spica cast

 

Discussion

The correct answer is (A). In this case, the child has a high-level myelomeningocele, does not walk, and has bilateral hip dislocations. The best treatment is observation because the hips will not stay in due to the abnormal muscle

forces across the joint.

 

At age 10 the child has had a rapidly progressing scoliosis of over 15 degrees in 6 months to a curve magnitude of 30 degrees. What should be done?

1. Observation to 50 degrees

2. MRI

3. Posterior spinal fusion

4. Anterior spinal fusion

 

Discussion

The correct answer is (B). In myelomeningocele, a rapidly progressing curve should be concerning for a tethered cord and should undergo neurosurgical evaluation for detethering. Curves greater than 20 degrees usually progress in myelomeningocele patients and warrant surgical evaluation. In children who are ambulatory, however, spinal fusion should be approached with caution as many use their lumbar spine to assist with ambulation due to weak abductors.

At the age of 12 the child is unable to complete her schoolwork, her grades are suffering and she is not able to perform tasks she was previously able to. What is the most likely etiology?

1. Natural history of the condition

2. Too many surgeries at a young age and exposure to anesthesia

3. Shunt malfunction

4. Depression

 

Discussion

The correct answer is (C). This is a common scenario for patients with myelomeningocele, especially after spinal fusion. A deterioration in cognitive status is most commonly associated with shunt malfunction. During their lifetime, 86% require a shunt with 95% needing a revision due to increasing hydrocephalus.

 

Objectives: Did you learn...?

 

 

Early treatment of myelomeningocele? Prevention of myelomeningocele?

 

Associated conditions with myelomeningocele?

 

Treatment and outcomes of thoracic level myelomeningocele orthopaedic

conditions?