Idiopathic macrocephaly in the infant: long-term neurological and neuropsychological outcome

Muenchberger, Heidi; Assaad, Nazih; Joy, Pamela; Brunsdon, Ruth; Shores, E. Arthur

doi:10.1007/s00381-006-0080-0

Cited by 44 publications

(49 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…2 Enlarged SS typically normalize by 2-3 years of age in BESS. 2,22,37 The patients with macrocrania and normal SS in our study may be at the later stages of this condition. There are some limitations to this study.…”

Section: Discussionmentioning

confidence: 67%

Prevalence of Subdural Collections in Children with Macrocrania

Greiner

Richards

Caré

et al. 2013

AJNR Am J Neuroradiol

View full text Add to dashboard Cite

BACKGROUND AND PURPOSE:The relationship between enlarged subarachnoid spaces and subdural collections is poorly understood and creates challenges for clinicians investigating the etiology of subdural collections. The purpose of this study was to determine the prevalence of subdural collections on cross sectional imaging in children with macrocephaly correlating with subarachnoid space enlargement.

show abstract

Section: Discussionmentioning

confidence: 67%

Prevalence of Subdural Collections in Children with Macrocrania

Greiner

Richards

Caré

et al. 2013

AJNR Am J Neuroradiol

View full text Add to dashboard Cite

show abstract

“…One such study by Muenchberger et al [23] found that 4/9 BEH patients evaluated with imaging and full neuropsychiatric testing an average of 15 years after initial diagnosis demonstrated problems with tests of attention, sequencing and visual motor scanning, functions that are generally attributed to the frontal lobe. However, the authors concluded that the overall intellectual abilities of the study group as a whole were within normal range.…”

Section: Discussionmentioning

confidence: 99%

Long-Term Health Status in Benign External Hydrocephalus

et al. 2011

View full text Add to dashboard Cite

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.

show abstract

“…The subtype “external hydrocephalus” is usually defined as a rapid increase in head circumference, combined with enlarged subarachnoid spaces as seen on neuroimaging—especially overlying the frontal lobes—and normal or only moderately enlarged ventricles [4, 91, 105, 118, 140, 143]. It occurs mainly during infancy, and the subarachnoid space enlargement gradually decreases and disappears over the next years [91, 110, 118].…”

Section: Introductionmentioning

confidence: 99%

Benign external hydrocephalus: a review, with emphasis on management

et al. 2011

View full text Add to dashboard Cite

Benign external hydrocephalus in infants, characterized by macrocephaly and typical neuroimaging findings, is considered as a self-limiting condition and is therefore rarely treated. This review concerns all aspects of this condition: etiology, neuroimaging, symptoms and clinical findings, treatment, and outcome, with emphasis on management. The review is based on a systematic search in the Pubmed and Web of Science databases. The search covered various forms of hydrocephalus, extracerebral fluid, and macrocephaly. Studies reporting small children with idiopathic external hydrocephalus were included, mostly focusing on the studies reporting a long-term outcome. A total of 147 studies are included, the majority however with a limited methodological quality. Several theories regarding pathophysiology and various symptoms, signs, and clinical findings underscore the heterogeneity of the condition. Neuroimaging is important in the differentiation between external hydrocephalus and similar conditions. A transient delay of psychomotor development is commonly seen during childhood. A long-term outcome is scarcely reported, and the results are varying. Although most children with external hydrocephalus seem to do well both initially and in the long term, a substantial number of patients show temporary or permanent psychomotor delay. To verify that this truly is a benign condition, we suggest that future research on external hydrocephalus should focus on the long-term effects of surgical treatment as opposed to conservative management.

show abstract

Idiopathic macrocephaly in the infant: long-term neurological and neuropsychological outcome

Cited by 44 publications

References 20 publications

Prevalence of Subdural Collections in Children with Macrocrania

Prevalence of Subdural Collections in Children with Macrocrania

Long-Term Health Status in Benign External Hydrocephalus

Benign external hydrocephalus: a review, with emphasis on management

Contact Info

Product

Resources

About