Long-Term Health Status in Benign External Hydrocephalus

Abstract

Purpose: Benign external hydrocephalus (BEH) is characterized by excess cerebrospinal fluid in the frontal subarachnoid spaces in infants evaluated for macrocephaly. We sought to describe the natural history of this disorder, specifically its clinical presentation, disease course and long-term health status impact using the validated, disease-specific Hydrocephalus Outcome Questionnaire (HOQ). Methods: An inception cohort of children >5 and <18 years old, with a history of BEH was assembled by ICD-9 and a key word search of medical and radiology records. Review confirmed the diagnosis of BEH, excluded major medical comorbidities and assessed presentation, radiographic features and head size/growth velocity. The HOQ was administered by mail. Results: We identified 99 patients, 5–12 years old (55% males). Twenty were born prematurely, 12 with <33 weeks gestation. Children presented at an average age of 9 ± 4.8 months (mean ± SD). The presenting complaint was macrocephaly in 65 cases. Other presenting findings were positional head shape deformity and torticollis; 10% had a family history of macrocephaly. Developmental delay was present in 21% of patients (4% verbal, 20% gross motor, 4% fine motor delay). Four patients had small subdural hematomas, none with suspicion of a non-accidental trauma. During clinical reassessment over a mean follow-up of 13 months, the average head percentile was stable and none of the patients developed new subdural hematomas. Gross motor delay resolved in 15/20 and fine motor delay in 4/4 patients. Verbal delay resolved in 2/4 patients, but interestingly, was newly detected in 6 other children. None of the patients required cerebrospinal fluid shunting. The response rate to the HOQ was 25% (median age 7 years, 74% females). The average overall HOQ score was 0.75 ± 0.24 versus 0.68 ± 0.19 for a previously published cohort of shunted hydrocephalic children. Conclusions: BEH patients in this series generally saw resolution of presenting motor developmental delays; however, new verbal delays were detected in a non-trivial number of patients. Quality of life measurements suggest some reduction in health status, but less so than is seen with shunted hydrocephalus.