Management of children with holoprosencephaly.

Holoprosencephaly (HPE) is the most common malformation of the embryonic forebrain in humans. Although HPE occurs along a continuous spectrum, it has been categorized into four types from most severe to least severe: alobar, semilobar, lobar, and middle interhemispheric (MIH) variant. Facial malformations are often associated with HPE and usually correlate with the severity of brain malformation.

Health related quality of life in adolescents with abnormal bladder function: an assessment using the Child Health and Illness Profile-Adolescent Edition.

Purpose: We studied the impact of abnormal bladder function due to congenital urological disorders on health related quality of life in children. A reliable patient based method is needed to assess the impact of these conditions in children and the interventions used to treat them.

Materials And Methods: Participants 11 to 17 years old with bladder exstrophy-epispadias complex, spina bifida or other causes of abnormal bladder function self-administered the Child Health and Illness Profile-Adolescent Edition, a generic health related quality of life instrument.

Fractures in children with cerebral palsy.

Introduction: We studied the fracture history in a large population of patients with cerebral palsy to determine which children were at the highest risk for fracture.

Methods: The International Classification of Diseases (Ninth Revision) coding identified 763 children with cerebral palsy. Patients and caregivers were contacted for information about fracture history and risk factors for low bone density.

Postoperative gastric rupture in children with cerebral palsy.

Children with cerebral palsy (CP) develop difficulties with swallowing and bowel motility, although the underlying etiology of these disorders is unclear. The authors identified three children treated at their institution in the past 6 years who developed severe gastrointestinal dysfunction leading to gastric rupture after orthopaedic surgery; all three had a history that included CP (subtype spastic quadriparesis), low cognitive function, and gastrointestinal motility disorders. All three cases were fatal; in two patients the diagnosis was made at autopsy.

A retrospective survey of perinatal risk factors of 104 living children with holoprosencephaly.

Holoprosencephaly (HPE) is a brain malformation resulting from a primary defect in development of the basal forebrain during early gestation. Prenatal genetic and environmental factors and birth outcomes were described in a population of 104 children with holoprosencephaly referred to three clinical centers from 1998 through 2002. The mean child age was 4 years.

Oral baclofen in cerebral palsy: possible seizure potentiation?

Baclofen, a gamma-aminobutyric acid agonist, is widely used to treat spasticity of cerebral and spinal origin. Patients with both acute baclofen overdose and withdrawal have developed seizures. After several reports of new-onset seizures in children treated with oral baclofen at our institution, we reviewed our experience regarding possible effects of baclofen on seizure induction in a childhood movement disorders program over a 2-year period.