This report emphasizes that children born with myelomeningocele require ongoing medical care and lifelong commitment from both patients and their families, as well as their medical care providers, with the goal of maintaining stable neurological functioning throughout their lives. As a result, there is an increased need for coordinated health care efforts to maintain and enhance the quality of life for these patients.

A comprehensive 20–25-year follow-up after the initial surgical closure of the back of patients born between 1975 and 1979 with open spina bifida highlights a broad range of outcomes including their current motor level, CSF shunt status, education, employment, mobility, and other medical history. Earlier reports on the outcomes of this study group at 5, 10 and 15 years are also available and were published by the senior author. The original study group was comprised of 118 patients. 71 patients (38 female and 33 male) were available for this review, 19 patients were lost to follow-up, and 28 patients had passed away.

At the Children’s Memorial Hospital in Chicago, a patient’s myelomeningocele level is evaluated based on motor function using manual motor tests at different stages of the patient’s life. Patients were grouped into thoracic, L2/L3, L4, L5, sacral, and asymmetrical levels. 73% of the 71 patients reviewed showed stable motor levels over time. 11% showed improved motor levels while 16% showed worsened motor levels. The thoracic and sacral levels were most commonly affected among patients lost to follow-up while the thoracic and L5 levels were prominent among the patients that passed away.

Furthermore, 47% of patients who were lost to follow-up had Cerebrospinal Fluid (CSF) shunts. In comparison, 86% of the review group had shunts, predominantly ventriculoperitoneal shunts. Shunt rates similar to the review group were noted among deceased patients. Shunt revisions were common, with 95% of the review group having at least one revision, and 41% of patients with shunts having 2-3 revisions. Infections were observed in 22 patients with shunts. Additionally, seizures were observed in 16 patients within the review group, all on medication. Shunted hydrocephalus was observed in all 16 patients with 7 of them having shunt infections.

The investigators also assessed educational status. Approximately 86% of the review group were attending high school, or college, or had recently graduated. The majority of the review group (63%) attended regular classes, but 14% required additional help, and 23% were in special education classes. It was also noted that 55 patients from the review group still lived with their parents.

Mobility was assessed, as the authors observed a decline in the review group from early childhood to early teens. However, those remaining mobile during teenage continued to ambulate majority of the time in early adulthood. 46% of young adults currently continue to walk most of the time, while 13% are mobile 25-50% of the time, and 41% depend entirely on wheelchairs for mobility. Patients who have better motor function in their lower extremities exhibited higher rates of walking. Additionally, 85% of young adults were on clean intermittent catheterization (CIC) for their bladder, with 90% of them performing self-catheterization. 

The study also reviewed symptomatic tethering of the spinal cord, with 23 patients requiring tethered cord release (TCR) due to symptoms including worsening scoliosis, gait changes, and spasticity. It was also noted that among the review group, 6% of patients underwent posterior cervical decompression, with similar rates observed for tracheostomy and slightly lower rates (4%) observed in having a gastrostomy tube. Notably, patients who underwent an increased number of shunt revisions (5+) were most prone to latex allergies.

Based on the results, it was observed that the death rate continued to increase into early adulthood, with the most common cause being an unrecognized shunt malfunction. Although many children with open spina bifida reach early adulthood, CSF shunt malfunctions can contribute to a decline in neurologic functions in these patients. This highlights the importance of monitoring patients with shunts for complications/revision.

Additionally, it is important to highlight that since the publication of this paper, the Management of Myelomeningocele Study1 (MOMS) conducted by Adzick et al. has underscored the efficacy of prenatal repair of myelomeningocele compared to the standard postnatal surgical approach. The study found that compared to postnatal surgery, prenatal surgery for myelomeningocele performed before 26 weeks of gestation reduced the risk of death or the requirement for a CSF shunt by 12 months of age. It also improved the combined scores for cognitive development and motor function at 30 months of age (P=0.007). 

Secondary outcomes including hindbrain herniation degree, motor function and the ability to walk independently also showed improvement with prenatal surgery. However, the authors of the MOMS study also emphasized the importance of weighing the potential benefits of prenatal surgery for myelomeningocele against several risks, including preterm birth and maternal morbidity. Despite the prenatal surgery group having more severe lesions and a higher incidence of preterm delivery compared to the postnatal surgery group, their significantly better outcomes were attributed to the timing of the surgery, allowing for more normal nervous system development before birth. 

Over the past three decades, significant advancements have been achieved in the prognosis of patients with myelomeningocele. However, limited knowledge about their long-term prognosis and late complications means that physicians must be prepared to manage various medical issues in these unique young adults, including pressure sores, obesity, renal disease, hypertension, depression, and visual impairment. It is also essential for future physicians and legislators to provide adequate support and care for this growing population.

Citation:

Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg. 2001 Mar;34(3):114-20. doi: 10.1159/000056005. PMID: 11359098.

In text citation:

1. Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; MOMS Investigators. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011 Mar 17;364(11):993-1004. doi: 10.1056/NEJMoa1014379. Epub 2011 Feb 9. PMID: 21306277; PMCID: PMC3770179.