Management and Delivery of a Patient with Maternal Myelomeningocele: A Case Report

Abstract

Objective: To report and discuss the pregnancy course and delivery of a patient with maternal myelomeningocele at term Background: Spina bifida is a congenital malformation which results from incomplete closure of the neural tube. Open neural tube defects (NTD) comprise 80% of all NTDs. The most common open NTD is myelomeningocele (MMC), a form of spina bifida characterized by herniation of both the meninges and spinal cord. The incidence rate of MMC is approximately 1/1,000 live births worldwide, and is inversely related to socioeconomic status. There is a paucity of data on pregnant women with spina bifida, however a study by Shepard et al. investigated deliveries among 10,000 women with spina bifida and 42 million women without. This study revealed that women with spina bifida were significantly more likely to be delivered via cesarean section, even when vaginal delivery was possible. Also, pregnancy rates in spina bifida patients have risen over time. Case Description: A 29-year-old gravida 1 female at 12 weeks gestation presented to the Maternal Fetal Medicine (MFM) clinic due to maternal MMC. Past medical history was significant for MMC, Chiari malformation type II, neurogenic bowel, and neurogenic bladder. Past surgical history was significant for MMC repair, ventriculoperitoneal shunt, spine-to-abdomen shunt, cervical spine fusion, spinal cord untethering, and club foot correction. Patient ambulated using a walker. Noninvasive prenatal testing was low-risk. The alpha-fetoprotein was unremarkable at 22 ng/ml. Ultrasound revealed unilateral fetal pyelectasis. Fetal echocardiogram showed normal anatomy and function. The patient was admitted for 4 days at 33 weeks for a unilateral vulvar infection and hematoma that developed from repeated self-catheterization. The patient was admitted out of precaution due to past history of labial Fournier’s gangrene. Wound cultures grew prevotella bivia. Infectious disease recommended outpatient oral antibiotics at discharge. Anesthesiology recommended maternal MRI prior to delivery planning. MRI showed lumbar lordosis and severe canal compromise in the lumbosacral region. The low-lying conus terminated at L3-L4. Cauda equina nerve roots extended to the sacral region. Morphology of the sacrum and coccyx was abnormal, consistent with MMC. Imaging determined epidural and spinal anesthesia carried a high likelihood of analgesic failure from scar tissue due to prior surgeries. The patient ultimately agreed to general anesthesia. A primary low-transverse cesarean section was performed at 39 weeks 0 days. The baby was delivered without delayed cord clamping and had 1- and 5-minute APGAR scores of 6 and 9. Dense adhesions were present throughout the abdomen, which prevented uterine exteriorization during hysterotomy repair. The remainder of the surgery was uncomplicated and indwelling foley was kept in place postpartum. At 6-week postpartum visit, patient was fully healed and had resumed self-catheterization. Discussion: Pregnant women with spina bifida require complex multi-disciplinary co-management. This patient received care from MFM, neurology, urology, anesthesiology, infectious disease, and neurosurgery. Currently, there are no universally established guidelines on the management of pregnant women with spina bifida, but general principles of management exist. In the pre-conception period, it is recommended that patients at high-risk for fetal spina bifida, including type 1 diabetics, take 4mg of folate daily. High-risk patients should take folate for at least 3 months prior to conception and up until 12 weeks’ gestation. Since 9-12% of this population is affected by epilepsy, optimizing seizure medications is prudent. Lamotrigine or Carbamazepine monotherapy is preferred, as they pose the least risk of congenital malformations. In the antepartum period, providers should anticipate treating common comorbidities. Bladder dysfunction includes either hyper-reflexive or areflexive bladders. Intermittent self-catheterization is a viable option for those with incomplete bladder emptying. Pregnant patients with ventriculoperitoneal shunts are susceptible to displacement from the gravid uterus. Since limited mobility is prevalent in this population, risk assessments for venous thromboembolism should be completed, and prophylactic anticoagulation may be considered. Vaginal delivery is possible in some patients. However, epidural or spinal anesthesia can increase the risk of spinal cord injury in those with tethered cords or prior surgery. Patients with limited mobility may require cesarean section due to inability to position for labor. In conclusion, MMC is rarely encountered in obstetrical contexts but is becoming increasingly more commonplace. MMC requires co-management by multiple specialties to surveil and optimize common comorbidities. In this case, the patient’s pregnancy course was mainly complicated by her pre-existing neurogenic bladder and resulting vulvar wound secondary to self-catheterization. For MMC patients who are interested in pregnancy, preparations should be started in the pre-conception period, with close monitoring throughout the antepartum period to follow. Currently, there are generally accepted guidelines for managing MMC patients in pregnancy, but there is a lack of global standardization. The development of standardized treatment guidelines may be possible as more of these cases are discussed and appear in the literature