Factors contributing to skin breakdown include excessive pressure associated with limited mobility and overweight, infection, trauma, poor circulation, lack of sensation. and fecal and urinary incontinence. Prevention and management include [70,71]:

Careful inspection of the skin
Proper skin cleansing
Avoidance of occlusive clothing
Elimination of movements that cause friction
Proper fitting orthosis and wheelchairs
Symmetric weight bearing
Frequent weight shifts
Exposure of the affected skin to air
Prompt medical attention to an affected area
Protective skin lotions and ointments may reduce pain and erythema associated with perineal skin breakdown in incontinent patients. Although no studies are available in children, these preparations have been shown to be effective in incontinent elderly patients [72].

Latex allergy — Many children with myelomeningocele have allergic reactions to latex, ranging in severity from contact urticaria to anaphylactic shock [73]. In one review of 60 children with myelomeningocele, 48 percent were sensitized and 15 percent were allergic to latex [74]. In another review of 71 patients who were followed for 20 to 25 years, 33 percent were allergic to latex and 9 percent had experienced a life-threatening reaction [28]. The mechanism for development of allergy is thought to be repeated exposures to latex rubber during multiple surgical procedures, as well as daily bladder catheterization and bowel management, although there may be factors unique to the underlying condition [75]. Products containing latex should be avoided [76].

PROGNOSIS — The prognosis for patients with myelomeningocele depends upon decisions regarding their care, the level of the lesion, and the presence and severity of neurologic deficits, hydrocephalus, and other central nervous system anomalies, as illustrated below.

With aggressive treatment, the majority (approximately 85 percent) of patients survive the neonatal period [77,78]. In one review of 212 patients, 72 percent of survivors were ambulatory and 79 percent were considered to have normal cognitive development [77]. In another series of 200 patients, 74 percent were at least partially ambulatory and 87 percent had urinary continence [78]. There was a small, but statistically significant improvement in the first year survival rate of infants with spina bifida in the United States after the introduction of mandatory folic acid fortification of the grain supply (from 90.3 to 92.1 percent) [79]. (See "Prevention of neural tube defects", section on Relationship between folate and NTDS).

In children with myelomeningocele and hydrocephalus, high spinal cord lesions (T12 and above) are associated with more severe anomalous brain development. More severe anomalous brain development is associated with poor neurobehavioral outcomes on measures of intelligence, academic skills, and adaptive behavior [80].

The long-term outcome of myelomeningocele was outlined in a review of 118 children with myelomeningocele who were treated nonselectively [81]. Among the 71 patients who were available for follow-up at 20 to 25 years, the following findings were noted:

The overall mortality was 24 percent and continued to increase into young adulthood
86 percent of patients had undergone cerebrospinal fluid (CSF) diversion and 95 percent had undergone at least one shunt revision
32 percent had undergone release of tethered cord, after which 97 percent had improvement or stabilization in their preoperative symptoms
43 percent had undergone spinal fusion for scoliosis
23 percent had had at least one seizure
85 percent were attending or had graduated from high-school and/or college
Long-term survival may be related to the need for CSF diversion. In one review of 904 patients with myelomeningocele seen in a multidisciplinary clinic over 43 years, survival into adolescence was similar for patients with and without CSF diversion [28]. However, for patients alive at 16 years, survival after age 34 years was decreased for those with shunted hydrocephalus compared to those without a shunt.

FETAL SURGERY — In animals with a surgically created spinal defect, intrauterine closure of the exposed spinal cord tissue prevents secondary neurologic injury [82]. In one study in humans, intrauterine repair was performed at 24 to 30 weeks gestation in 29 patients with isolated fetal myelomeningocele [83]. The following results were reported:

Compared to matched controls, fewer infants in the treatment group required shunt placement for hydrocephalus at six months of age (59 versus 91 percent)
Compared to controls, the median age at shunt placement was later
(50 versus 5 days of age)

The incidence of hindbrain herniation was reduced (38 versus 95 percent).
The treatment group had a higher incidence of oligohydramnios (48 versus 4 percent) and preterm contractions (50 versus 9 percent) than the control group
The treatment group had lower mean gestational age (33.2 versus 37) and birth weight (2171 versus 3075 g) than the control group
In a subsequent report, the same group described 116 infants who had undergone intrauterine repair of spina bifida and had postnatal follow-up of at least 12 months [84]; 54 percent required the placement of a ventricluloperitoneal shunt by one year of age. Shunt placement was less likely to be necessary among fetuses who had a ventricular size of <14 mm at the time of surgery, who had surgery at 25 weeks gestation, and had defects located at or below L4 (all fetuses with defects at or above L1 required shunts).

In other reports, intrauterine repair did not improve lower extremity function [85] or affect the progression of ventriculomegaly [86]. This approach is not recommended until data on long-term follow-up and the results of an ongoing randomized trial are available [87].

PREVENTION — The prevention of neural tube defects is discussed separately. (See "Prevention of neural tube defects").