[Multicystic encephalomalacia. Review of 19 cases]

G Orejón de Luna1, F Mateos Beato, R Simón de las Heras

  • 1Unidad de Neurología Pediátrica, Hospital Doce de Octubre, Madrid.

Insights

Multicystic encephalomalacia (EMQ) is a rare pediatric brain disorder characterized by brain tissue replaced by cavities. Early diagnosis via cerebral ultrasonography is crucial due to the consistently poor outcomes observed in affected children.

Area of Science:

  • Pediatric Neurology
  • Neuroimaging
  • Developmental Pediatrics

Background:

  • Multicystic encephalomalacia (EMQ) is a severe pediatric condition where brain tissue is replaced by fluid-filled cavities.
  • Understanding the etiology, diagnostic methods, and clinical progression of EMQ is essential for managing affected children.

Purpose of the Study:

  • To retrospectively analyze the causes, diagnostic approaches, and clinical and radiological outcomes of multicystic encephalomalacia (EMQ) in a cohort of pediatric patients.
  • To evaluate the effectiveness of different neuroimaging modalities in the early diagnosis of EMQ.

Main Methods:

  • Retrospective analysis of 19 children diagnosed with multicystic encephalomalacia (EMQ).
  • Review of etiological factors, diagnostic imaging (cerebral ultrasonography, CT, MRI), and clinical and radiological evolution.
  • Assessment of patient outcomes, including mortality, neurological deficits, and long-term consequences.

Main Results:

  • Common etiologies included twin pregnancy (12 cases), perinatal hypoxia (4 cases), and perinatal infection (1 case).
  • Cerebral ultrasonography proved highly effective for early and reliable EMQ diagnosis.
  • The clinical outcome was predominantly unfavorable, with 73% of patients experiencing severe consequences such as intellectual disability, microcephaly, spastic tetraplegia, and epilepsy. Two children died, and the remaining showed spastic hemiplegia.

Conclusions:

  • Multicystic encephalomalacia (EMQ) is a rare pediatric disease associated with a very poor prognosis.
  • Early and accurate diagnosis is critical for managing EMQ.
  • Cerebral ultrasonography is the preferred imaging modality for the early detection of EMQ in children.
Abstract

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