Diagnosis often occurs prenatally through the quad marker screening test and/or ultrasound. The quad marker screening test is 85 percent accurate for detecting open neural tube defects. If an abnormal result is found on the quad marker screening, an amniocentesis may be recommended to verify the results (Stoppler and Davis). Despite a high level of accuracy, some cases of myelomeningocele will go undetected until birth. Treatment for myelomeningocele and other severe cases of spina bifida typically involves surgery within first few days of life. Additional surgery for placement of a ventriculoperitoneal shunt for hydrocephalus may take place within the first few days or weeks of life as well.
Alternatively, some mothers and babies may undergo prenatal surgery to close the lesion between 19 and 25 weeks gestation. In this procedure, surgical incisions are made in the mother’s uterus and surgery is done on the fetus in utero. The incisions in the uterus are then closed up and the mother is placed on bed rest for the remainder of the pregnancy. Babies who undergo this procedure are usually born prematurely. All babies who undergo prenatal surgery for myelomeningocele are born via cesarean section because of the danger of uterine rupture from the surgical scars. (Adzick). Results of the prenatal surgery have been mixed with some children showing above average mobility and reduced need for shunts while others continue to have poor mobility and require shunts for hydrocephalus. The long-term prognosis for babies who undergo prenatal surgery is unknown as the number of children who have had the procedure is small and the procedure is still very new.
Though the level of function varies greatly from individual to individual, most people with spina bifida have normal IQ’s and can lead active lives with adaptations. The factors that cause the varying levels of function and ability in individuals with myelomeningocele and other severe forms of spina bifida are still not fully understood. Many doctors ascribe to a “two-hit hypothesis” that the first damage to the spinal column comes from the neural tube failing to close and subsequent damage occurs from exposure of the open lesion to amniotic fluid, direct trauma, hydrodynamic pressure, or a combination of factors (Adzick). However, the varying levels of function in individuals with myelomeningocele and the mixed results for prenatal surgery indicate that the factors contributing the severity of disability may be many and complex.
Research into treatment and outcomes for spina bifida is relatively new because of a lack of available data for several decades. Historically, treatment for babies with spina bifida was withheld because doctors believed that quality of life was too poor to allow them to survive. Until the 1960’s, it was standard practice among physicians to withhold surgery from all babies with spina bifida until the age of two because it was believed that only the strongest would survive and should receive treatment. Shunt placement surgery and clean intermittent catheterization became advanced enough during the 1970’s to allow individuals with spina bifida to not only survive but become more active, however, doctors continued to advocate for withholding treatment based on the philosophy that quality of life was too poor to allow these infants to live. In addition to withholding treatment, doctors often heavily sedated spina bifida newborns so they would not request feeding, ensuring death (Pruitt). Because of biases in administering care, having a surviving population of individuals with spina bifida large enough for research is a relatively new development and is only the beginning of understanding this condition and the possibilities available to individuals with spina bifida.
Despite the abundance of evidence that individuals with myelomeningocele can lead active and productive lives, many physicians remain ignorant of this fact. Parents who receive a prenatal diagnosis of myelomeningocele are frequently pressured by doctors to abort. Doctors will often mislead parents by describing a worst case scenario and telling them that it is a prognosis that can not be changed and that there is no hope for the child to lead a normal life. (Pruitt).
BIRTH AND ITS IMPACT ON BREASTFEEDING
There are differing opinions on whether babies undergoing postnatal surgery should be delivered vaginally or by cesarean section. For example, Lucile Packard Children’s Hospital Stanford recommends cesarean delivery for fear that vaginal birth will increase the risk of damage to the spinal cord (Stanford Medicine). But according to the University of North Carolina School of Medicine, the recommendation of cesarean delivery is based on older literature which may not account for improved neonatal surgical techniques, nor does this recommendation improve the outcome for babies in all cases. This older literature also demonstrated only modest gains in function for spina bifida babies delivered via cesarean. C.S. Mott Children’s Hospital recommends vaginal delivery whenever possible and states that current medical literature shows no improvement for spina bifida babies delivered by cesarean.
If hydrocephalus has advanced in the womb to the point where the head circumference has become very large, a cesarean delivery may be indicated. Another reason for cesarean delivery is breech presentation, which spina bifida babies are at an increased risk for (University of Michigan Health System). Many spina bifida babies do end up being delivered by cesarean. Doulas, lactation consultants and other birth and breastfeeding professionals should be prepared to help mothers of spina bifida babies with the breastfeeding complications that can accompany cesarean birth.