Reappraisal of Pediatric Normal-Pressure Hydrocephalus

Abstract

While normal-pressure hydrocephalus (NPH) is most commonly diagnosed in older adulthood, a significant body of literature has accumulated over half a century documenting the clinical phenomenon of an NPH-like syndrome in pediatric patients. As in adult NPH, it is likely that pediatric NPH occurs due to a heterogeneous array of developmental, structural, and neurodegenerative pathologies, ultimately resulting in aberrant cerebrospinal fluid (CSF) flow and distribution within and around the brain. In this review, we aimed to systematically survey the existing clinical evidence supporting the existence of a pediatric form of NPH, dating back to the original recognition of NPH as a clinically significant subtype of communicating hydrocephalus. Leveraging emergent trends from the old and more recent published literature, we then present a modern characterization of pediatric NPH as a disorder firmly within the same disease spectrum as adult NPH, likely with overlapping etiology and pathophysiological mechanisms. Exemplary cases consistent with the diagnosis of pediatric NPH selected from the senior author's neurosurgical practice are then presented alongside the systematic review to aid in discussion of the typical clinical and radiographic manifestations of pediatric NPH. Common co-morbidities and modern surgical treatment options are also described.

Keywords: cerebral palsy; cerebrospinal fluid; congenital malformation; neurodegenerative disease; neurosurgery; normal-pressure hydrocephalus; pediatric hydrocephalus; pediatrics; systematic review; ventriculoperitoneal shunting.

Figure 1

Results of systematic literature review. * Search criteria included ((Pediatric[Title/Abstract]) OR (Children[Title/Abstract]) OR (Child[Title/Abstract]) OR (Infant[Title/Abstract]) OR (Infants[Title/Abstract]) OR (Newborn[Title/Abstract]) OR (Newborns[Title/Abstract])) AND ((Normal Pressure Hydrocephalus[Title/Abstract]) OR (Idiopathic Hydrocephalus[Title/Abstract])).

Figure 2

6-year-old female patient with Chiari I malformation (case #1). Pre-operative (A,B) and 10 years post-operative (C,D) T1-weighted midsagittal and T2-weighted axial magnetic resonance imaging (MRI). Inferior displacement of the cerebellar tonsils below the foramen magnum consistent with Chiari malformation is indicated by the white arrows.

Figure 3

17-month-old male patient with cerebral palsy (case #2). Pre-operative (A,B) and 1 year post-operative (C,D) T1-weighted midsagittal and T2-weighted axial magnetic resonance imaging (MRI).

Figure 4

18-month-old female patient with premature birth and developmental delay (case #4). Pre-operative (A,B) and 8 years post-operative (C,D) T1-weighted mid-sagittal and T2-weighted axial magnetic resonance imaging (MRI).

Figure 5

10-year-old male patient with Hunter syndrome and hydrocephalus (case #8). Pre-operative T1-weighted mid-sagittal and T2-weighted axial magnetic resonance imaging (MRI, A,B) and 1 year post-operative computed tomography scan with ventricular catheter visualized in the dilated right lateral ventricle (C).

Figure 6

18-month-old male patient with left-sided porencephalic cyst secondary to intrauterine cerebral ischemia (case #9). Pre-operative (A,B) and 2 years post-operative (C,D) T1-weighted right-sided sagittal and T2-weighted axial magnetic resonance imaging (MRI), demonstrating ventriculomegaly as well as left-sided porencephalic cyst.