Long-term health status in benign external hydrocephalus

Area of Science:

• Pediatric Neurology
• Neurodevelopmental Disorders
• Pediatric Neurosurgery

Background:

• Benign external hydrocephalus (BEH) is defined by excess cerebrospinal fluid in frontal subarachnoid spaces in infants presenting with macrocephaly.
• Understanding the natural history and long-term outcomes of BEH is crucial for clinical management.

Purpose of the Study:

• To describe the clinical presentation, disease course, and long-term health status impact of Benign external hydrocephalus (BEH).
• To utilize the Hydrocephalus Outcome Questionnaire (HOQ) for validated, disease-specific quality of life assessment in BEH patients.

Main Methods:

• An inception cohort of 99 children aged 5-12 years with a history of BEH was assembled.
• Diagnosis was confirmed, comorbidities excluded, and clinical/radiographic features assessed.
• The Hydrocephalus Outcome Questionnaire (HOQ) was administered via mail to assess health status.

Main Results:

• Most patients presented with macrocephaly; 21% had developmental delays (motor and verbal).
• Over a mean follow-up of 13 months, head size remained stable, and motor delays largely resolved.
• New verbal delays were detected in some patients, and HOQ scores indicated a reduced health status compared to controls.

Conclusions:

• Benign external hydrocephalus (BEH) patients often experience resolution of motor delays, but vigilance for new verbal delays is necessary.
• Quality of life in BEH is affected, but generally less severely than in hydrocephalus requiring cerebrospinal fluid shunting.