tethering the spinal cord is found in more than 50% of patients with anorectal, urogenital, or sacral malformations.73 Table 5 Urologic Anomalies in Spinal Dysraphism82,112–121 Prognostic Factors The overall medical and psychosocial prognosis of patients with spinal dysraphism depends on the extent of the neurologic deficits and associated congenital abnormalities, as well as the extent and sophistication Inhibitors,research,lifescience,medical of the treatment they receive. In general, the lower and less severe the spinal lesion, the higher the chance the patient will be ambulatory and not have hydrocephalus and, therefore, a better outcome. Children with spinal dysraphism seem to have a higher risk for exhibiting worse levels of internalizing symptoms and lower levels of self-esteem than normal children.74,75 They are also more likely to be interpersonally lonely and socially immature.76 Spinal dysraphic children, especially Inhibitors,research,lifescience,medical those with hydrocephalus, frequently have difficulties in certain academic areas, such as arithmetic,77,78 and they tend to score at the low end of the average range
of intelligence. They also tend to exhibit deficits in executive functioning, abstract reasoning, and the ability to focus attention.79 Parents who have a positive and hopeful attitude are able to improve the quality of life of their adolescents by up to 25% over that which would be predicted for the disability at birth.80 The status of the lower and upper Z-VAD-FMK nmr urinary Inhibitors,research,lifescience,medical tracts primarily depends on the individual patient’s neurologic condition.81 At birth, it is believed that 5% to 25% of children with spinal dysraphism will demonstrate an abnormal upper urinary tract (mostly mild reflux),82 with up to 3% having decreased Inhibitors,research,lifescience,medical renal function (significant hydronephrosis). In a series of 64 infants, 9 patients (14%) Inhibitors,research,lifescience,medical were born with abnormal upper urinary tracts, with an additional 6 (9%) subsequently developing upper tract deterioration within 3 years of life.83 If untreated, 10% to 50% of patients will develop not only abnormal upper tracts but also significantly decreased renal function. Therefore, appropriate management of these individuals may
prevent significant urologic morbidity and mortality others from taking place. The life expectancy of patients with significant neurologic lesions is shorter than that of the general population. It is estimated that approximately 40% to 50% of children with neural tube defects will die during infancy.81,84 In the past, prolonged life expectancy was almost exclusively achieved by ambulatory patients with sacral lesions and without hydrocephalus. If patients survived their neurologic problems, life span depended mostly on their subsequent renal function. Therapy for hydrocephalus and antibiotics, developed in the 1950s, had the most significant impact on patient survival, because hydrocephalus was the major source of infant mortality. At all ages, renal failure is the most common cause of death.