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Cardiology1 paper

Dysplastic ovary

Last edited: 4 h ago

Overview

Dysplastic megalencephaly, or bilateral hemimegalencephaly, is a rare cerebral malformation characterized by bilateral cerebral hemisphere overgrowth and cortical developmental abnormalities, often presenting with intractable seizures, severe neurological impairment, and developmental delay 1.

Diagnosis

  • Prenatal imaging may reveal characteristic overgrowth and malformation patterns 1.
  • Postnatal neuroimaging (MRI, CT) essential to confirm bilateral cerebral hemisphere overgrowth and cortical malformations 1.
  • Clinical presentation includes intractable seizures, severe neurological impairment, and developmental delay 1.

    • Management

  • Management focuses on symptomatic treatment and supportive care 1.
  • Early intervention programs for developmental support 1.
  • Seizure control with antiepileptic drugs (specific dosing not detailed in abstracts) 1.

    • Special Populations

  • Pregnancy: Awareness of potential for prenatal high-output cardiac failure in affected fetuses is crucial for obstetric management and parental counseling 1.

    • Key Recommendations

  • Prenatal surveillance with advanced imaging techniques is recommended to identify dysplastic megalencephaly and associated complications like high-output cardiac failure early 1 (Evidence: Moderate).
  • Comprehensive postnatal neuroimaging is essential for definitive diagnosis and guiding management strategies 1 (Evidence: Moderate).
  • Early initiation of supportive therapies, including developmental interventions and seizure management, is advised to improve outcomes 1 (Evidence: Expert opinion).

    • References

    1 Del Campos Braojos F, Guimaraes CVA. Dysplastic megalencephaly phenotype presenting with prenatal high-output cardiac failure. Pediatric radiology 2018. link

    Original source

    1. [1]
      Dysplastic megalencephaly phenotype presenting with prenatal high-output cardiac failure. — Del Campos Braojos F, Guimaraes CVA Pediatric radiology (2018)

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