The intelligence of 200 shunted hydrocephalic children was studied prospectively.The following diagnostic categories were used: (1) simple internal hydrocephalus; (2) internal hydrocephalus in the child with meningomyelocele; (3) internal hydrocephalus associated with porencephaly; (4) external hydrocephalus; (5) the Dandy\ x=req-\ Walker cyst.The group with meningomyelocele was brightest and the group with porencephaly the dullest; whites scored higher than blacks.The following groups were found to be of normal intelligence: white patients with internal hydrocephalusand with meningomyelocele, and black patients with meningomyelocele and with external hydrocephalus. Intelligence quotient was found to be unrelated either to number of shunt revisions or severity of hydrocephalus prior to initial surgery, but was related to age at initial shunt placement and shunt function. Children with internal hydrocephalus and hydrocephalus with meningomyelocele whose shunts were kept functioning were found to be of normal intelligence. Many studies have been pub¬ lished concerning the intelli¬ gence of hydrocéphalie children. These studies have included those evaluating shunted hydrocéphalies and those evaluating nonoperatively treated hydrocéphalies. Most studies have been purely descriptive; only a few have compared treated and nontreated groups. Though cerebrospinal fluid (CSF) has long been shunted from ventricu¬ lar or lumbar subarachnoid spaces in other body cavities, it was not until 195 that valves were used to regu¬ late the flow of CSF from the central nervous system. Since that time, shunting procedures of one sort or an¬ other have represented the main form of treatment for congenital hydro¬ cephalus.In essence, the purpose of the shunt is to direct the CSF from either the ventricular system or the lumbar subarachnoid space into another body cavity from whence it may be ab¬ sorbed. This results in less pressure, volume, and flow of CSF through the distending ventricular system. If the shunt is effective, the ventricles be¬ come either normotensive or hypotensive and diminish in size. At times they actually become smaller than normal. The shunted hydrocéphalie child becomes "shunt dependent," that is, intolerant of an obstructed shunting system.It is the purpose of this paper to give a critical analysis of those publi¬ cations discussing intellectual func¬ tion in shunted and untreated hydrocephalic children, and to present the results of our research on the intellec¬ tual development of shunted hydrocephalic children. Review of Literature and Statement of Clinical HypothesesIt is impossible to evaluate the lit¬ erature regarding intellectual func¬ tion in children with treated and untreated congenital hydrocephalus unless one knows the exact criteria on which the diagnosis of this congenital disease was based. Progressive in¬ crease in head size, bulging fontanel, split sutures, transillumination, posi¬ tive Macewen sign, "setting sun" phenomenon, shiny scalp, and dis¬ tended scalp veins, are all indicative of an in...

\s=b\We studied 173 children with myelomeningocele, 133 of whom developed hydrocephalus (and had shunt procedures) and 40 of whom did not. Eighty siblings were tested as a control group. Sixty-three percent of children with hydrocephalus had IQs above 80; 87% of those without hydrocephalus had IQs above 80. Children who had associated hydrocephalus were significantly less intelligent than their siblings, whereas those without hydrocephalus were not. When patients and siblings were matched by age and IQ, the former scored significantly lower on a perceptual-motor functioning test. When patients with and without hydrocephalus were similarly matched, those with hydrocephalus scored lower. Inverse relationships between sac location and IQ, and sensory level and IQ, were found to be dependent on the association of higher sac levels and of sensory loss with hydrocephalus. Patient IQ was related to family income and education. (Am J Dis Child 131:199-204, 1977) With few exceptions,'-7 published reports of the intelligence of myelomeningocele children with (MM/H) and without (MM) hydro¬ cephalus have been limited to analyz¬ ing the natural history of nonoperatively treated hydrocephalus. By and large, these studies have been retro¬ spective, based on an accumulation of varying numbers of survivors. Simi¬ larly, the appraisals of survival, quali¬ ty of survival, and impact of the affected child on his home and enviFrom the Division of Neurological Surgery, IL 60614 (Dr Raimondi). ronment have been made from anal¬ yses of groups of children whose management has not been adequately described. METHODAs a first step in studying these children we established the criteria for diagnosing hydrocephalus in the child with myelomeningocele, determining precisely when a shunt was no longer functioning, compar¬ ing intellectual quotients with the presence or absence of hydrocephalus (treated, in the former event), using the siblings as the norm, and identifying (neuroradiologically) the nature and degree of brain dyspla¬ sia."'3 The two groups studied were myelomeningocele (MM) and shunted hy¬ drocéphalie myelomeningocele (MM/HS) children. The abbreviation MM/H refers to children who did not undergo a shunt procedure.There is, at the present time, consid¬ erable interest in the question of whether to treat such children at all, or whether treatment should be offered only to those children who may be expected to "live a meaningful life." Criteria on which such a decision may be based are lacking, though some arbitrary criteria (such as level of the myelomeningocele sac at birth, paraplegia, and hydrocephalus) have been suggested.In fact, one author has carried prediction to an extreme,' -reporting such a poor prog¬ nosis for myelomeningocele children with a thoracolumbar lesion, for example, irre¬ spective of the presence of hydrocephalus, that he recommends withholding all forms of treatment. This article presents the results of our study of the intellectual development of 173 children with myelomeningocele, 133 who had a shunt for...