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[Idiopathic external hydrocephaly and familial macrocephaly in infancy]

R Palencia Luaces1, J Aldana Gómez, F Tresierra Unzaga

  • 1Neuropediatría, Hospital Universitario, Facultad de Medicina, Valladolid.

Anales Espanoles De Pediatria
|March 1, 1992
PubMed

Insights

Idiopathic external hydrocephalus, a cause of infant macrocephaly, often resolves spontaneously. Cranial imaging normalizes by age 48 months without intervention.

Area of Science:

  • Pediatric Neurology
  • Neuroradiology

Background:

  • Idiopathic external hydrocephalus is a common cause of macrocephaly in infants.
  • Characterized by enlarged subarachnoid spaces, particularly frontal, and sometimes enlarged ventricles on CT scans.

Purpose of the Study:

  • To report on a cohort of infants diagnosed with idiopathic external hydrocephalus.
  • To describe the clinical course and radiological findings in these patients.

Main Methods:

  • Retrospective review of 16 infants diagnosed with idiopathic external hydrocephalus.
  • Analysis of clinical data including family history, motor development, and head circumference.
  • Review of serial cranial CT scans.

Main Results:

  • Seven patients had a family history of megalo-encephaly.
  • Four patients experienced delayed motor development, transient in three.
  • Head circumference normalized by 18 months in most cases, but 4 developed megalo-encephaly after age 3.
  • Radiological images normalized between 24 and 48 months without treatment.

Conclusions:

  • Idiopathic external hydrocephalus in infants often shows spontaneous resolution.
  • Radiological and clinical normalization occurs typically by 48 months without specific treatment.
  • Monitoring is essential as some cases may present with persistent macrocephaly beyond 3 years.

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