In this issue of Pediatrics, Alabi et al1 describe surgical procedures and health outcomes by age in persons enrolled in the National Spina Bifida Patient Registry. Results from this descriptive study reveal that the incidence of 6 surgical procedure groups and 4 specific designated health outcomes vary by age and type of spina bifida (SB). This data-rich article contains clinically applicable information for pediatricians within the medical home model.
There are 2 general observations to be made before looking at the data in more detail. First, the type of SB matters. Myelomeningocele is the most complex form of SB; nonmyelomeningocele forms (lipomyelomeningocele, meningocele, and fatty filum) are less severe and less common. In lipomyelomeningocele, hydrocephalus and Chiari malformation are rare. Most lipomyelomeningoceles occur in the lumbosacral region, which increases the likelihood of independent community ambulation. Although neurogenic bladder and bowel can occur, more patients with nonmyelomeningocele SB achieve continence. This is an important message that primary care pediatricians (PCPs) can share with families as they work together to establish appropriate expectations and interventions.
Second, although >80% of participants reported having undergone no additional surgeries after the first year of life,1 the cumulative data suggest that as a group, individuals with myelomeningocele will still go through a number of later surgeries. These surgeries are used to address anatomic or physiologic issues, modify associated secondary complications, and promote functional status. PCPs need to be aware of the types and timing of these surgeries (Table 1 from source article). Most importantly, PCPs should recognize that these surgeries, although necessary, exact a toll on the family, child, and siblings, including psychosocial2 and financial stress and school absences in children who are already at risk for specific learning disabilities.3 Pediatricians provide an important service to support the family by acknowledging these stresses, linking families to appropriate community support agencies, and advocating with schools as needed.
Next, it is important to look at these detailed data in context, focusing on myelomeningocele. Table 3 in the source article includes the percentage of surgical procedure groups that occur at different ages. Approximately 90% of neurosurgical and orthopedic procedures occur by age 12 years, although neurosurgical procedures to address shunt malfunction or symptomatic tethered cord remain stable at 1% per year after age 12 years and increase again moving to adulthood. In contrast, only 73% of gastrointestinal procedures, 74% of urologic procedures (usually focused on promoting continence), and 64% of skin procedures occur by age 12 years.
Table 5 in the source article includes the percentage of individuals undergoing a specific surgical procedure at a given age and present a complementary picture. Although the largest percentage of individuals with SB undergo neurosurgical procedures in the first year of life, 5% to 9% of individuals with meningomyelocele require some form of neurosurgical intervention each year. Likewise, most orthopedic surgeries occur at younger ages. However, most urologic and gastrointestinal procedures do not occur until after the age of 4 years. Finally, the percentage of skin procedures increases with age.
What Does This All Mean?
When outcome measures (Figure 1 in the source article) are evaluated, urinary and fecal continence only occur in ∼50% of individuals by adulthood, which is a gradual increase from childhood rates. This reveals that numerous factors likely affect achieving bladder and bowel continence. Evidence from other National Spina Bifida Patient Registry articles4,5 reveal that the timing and frequency of surgeries that might impact continence vary among registry sites. Another confounding factor regarding continence may be a philosophy of waiting until a person with SB is “ready” to be continent rather than proactively addressing this issue. Family readiness and individual self-management skills may also impact continence rates. Because recent research reveals that continence (particularly bowel) might affect quality of life,6 ongoing discussions between the family and the PCP can help identify and clarify family goals and facilitate communication with subspecialty providers to achieve improved continence.
Most procedures that are performed to address alignment or improve function and that impact mobility occur in childhood. Ambulation status and mobility decrease with increasing age; only one-third of individuals with myelomeningocele are characterized as community ambulators by their late teenage years. Although providers must always be aware of the possibility of symptomatic tethered cord, a decline in independent mobility should also be viewed as part of SB’s natural history in the face of some degree of motor paralysis. It is harder and less efficient to walk with crutches and braces than to use a wheelchair when weight, energy expenditure, and the functional burden of keeping up with peers are considered. The PCP can support patients and families during these changes with the goal of promoting acceptance, participation, and inclusion in school, social, and community activities.
Skin breakdown occurs more frequently as age increases and is a potentially preventable cause of morbidity and mortality in SB.7,8 Being aware of this risk, educating patients and families about preventive strategies, and routinely monitoring for skin breakdown is an important task for the PCP.
Although subspecialty care is necessary for individuals with SB, optimal health outcomes cannot be achieved without PCP involvement. Data from this article can be used to support pediatricians in that important role.
FUNDING: No external funding.
COMPANION PAPER: A companion to this article can be found online at www.pediatrics.org/cgi/doi/10.1542/peds.2017-3730.
POTENTIAL CONFLICT OF INTEREST: Dr Brei serves as a site investigator for the National Spina Bifida Patient Registry, and Dr Walker is a site principal investigator for the National Spina Bifida Patient Registry.
FINANCIAL DISCLOSURE: Dr Brei receives financial support to serve as the Spina Bifida Association’s medical director through a contractual agreement between the Spina Bifida Association and the Department of Pediatrics at the University of Washington School of Medicine; and Dr Walker has indicated he has no financial relationships relevant to this article to disclose.