Spina bifida and neural tube defects

Parents of a fetus with a prenatal diagnosis of a neural tube defect

The following should be discussed:[67]Douglas Wilson R, Van Mieghem T, Langlois S, et al. Guideline no. 410: prevention, screening, diagnosis, and pregnancy management for fetal neural tube defects. J Obstet Gynaecol Can. 2021 Jan;43(1):124-39.e8. http://www.ncbi.nlm.nih.gov/pubmed/33212246?tool=bestpractice.com [103]American College of Obstetricians and Gynecologists. Committee opinion no. 720: maternal-fetal surgery for myelomeningocele. 2017 Sep;130(3):e164-7. https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2017/09/maternal-fetal-surgery-for-myelomeningocele http://www.ncbi.nlm.nih.gov/pubmed/28832491?tool=bestpractice.com

• Diagnosis, prognosis, and available services and support

• Options regarding termination of the pregnancy, fetal surgery, and the birth (e.g., cesarean birth), including benefits and risks of each approach

• For women for whom fetal myelomeningocele repair is an option, detailed information about the surgery and the intensive care required

• Initial treatment of the baby in the neonatal intensive care unit

Patients with a neural tube defect

Patients are advised to keep reports and medical documents that summarize their baseline physical exam and baseline ancillary test findings. They should also keep a list of drugs they are taking and a list of providers and past surgical procedures. It is essential to have a well-documented neurologic exam. Patients with shunts should have ready access to their most recent magnetic resonance imaging or computed tomography head study and shunt series in order to document normal ventricle size and shunt status. They should be taught to advocate for low-radiation techniques and to request alternate imaging modalities when available and appropriate.

Image Gently: The Alliance for Radiation Safety in Pediatric Imaging Opens in new window

Patients should be familiar with signs and symptoms of shunt obstruction, Chiari malformation, and pyelonephritis, and should be instructed to seek prompt medical attention for any neurologic deterioration as this may be a sign of shunt malfunction or tethered cord. In addition, patients with shunts should be advised to avoid rapid acceleration/deceleration movements such as somersaults or riding roller coasters.

Nonambulatory patients should be taught proper transfer technique. A healthy diet and regular exercise should be encouraged from an early age. Latex avoidance is recommended for all patients with spina bifida. In addition, they should be taught how to recognize anaphylaxis and use an auto-injectable epinephrine if prescribed.

Spina Bifida Association: latex and latex allergy guideline Opens in new window

Patients should be advised to perform daily skin checks of insensate areas for pressure or friction sores at common pressure sites including areas overlying the ischial tuberosities, coccyx, calcaneus, and ankles, and between the toes; children should be taught at an early age to use a hand mirror for daily skin checks of the buttocks and feet. Pressure sores are uncommon in infancy and childhood, although burns and friction injuries to the dorsum of the feet are seen in children who crawl without protective footwear. Parents should be cautioned that most babies with spina bifida are likely to be insensate in a saddle distribution and along the bottom of their feet; therefore, their child's feet should be covered at all times.