PORENCEPHALY
 
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PORENCEPHALY

 

DEFINTION:

A congenital anomaly of the central nervous system (CNS) resulting in the formation of fluid-filled cysts or cavities within the cerebrum.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • infancy to childhood
  • risk factors:
    • a well-defined destructive event (see below)
  • associated anomalies:
    • induction disorders
      • encephalocele
    • proliferation disorders
      • microcephaly
    • migration disorders
      • heterotopic grey matter
      • pachygyria
      • absence of the corpus callosum & septum pellucidum
      • linear sebaceous nevi
    • developmental failure of cerebral venous sinuses
    • alopecia or other cranial defects

TYPES:

1. Type I - True Porencephalic Cysts

  • cysts arise by a faulty induction process and/or aberrant neuronal migration
  • associated with disorders of induction, proliferation, and other migration disorders
  • cysts most frequently located in region of the sylvian fissure
  • cysts may communicate with the ventricles, cerebral cortical surface, and/or subarachnoid space
  • a cyst communicating with the ventricles can act as a rapidly growing space-occupying lesion if a one-way valve mechanism occurs
  • associated with more severe neurologic manifestations

2. Type II - Pseudoporencephalic Cysts

  • cysts arise by disruption of normal brain tissue
  • cysts arise after a well-defined destructive event (vascular or infectious) occurring in late fetal or early infantile life (IVH, HIE, PVL, congential infections) -> cavitation of the necrotic region -> cyst formation within the parenchyma of the cerebral hemispheres
  • usually not associated with other disorders
  • tend to be unilateral
  • usually do not communicate with other structures
  • associated with hemiparesis and focal seizures in 1st year of life

CLINICAL FEATURES:

1. Neurologic Manifestations

  • infantile hypotonia
  • seizure disorder (myoclonic)
  • developmental delay
  • mental retardation
  • motor dysfunction
    • vary from asymptomatic to severe
    • may be focal or asymmetric
    • spastic monoparesis -> hemiplegia -> quadriparesis
    • hypotonia
    • athetosis
    • supranuclear bulbar palsy

2. Complications

  • failure to thrive
  • optic atrophy
  • delayed limb growth
  • hydrocephalus with increased intracranial pressure

INVESTIGATIONS:

1. Imaging Studies

1. Ultrasound/CT/MRI

  • abnormal CSF-filled cavities within the cerebral hemispheres
  • cysts can be asymmetric & not aligned with primary fissures

MANAGEMENT:

1. Team Approach

  • periodic multidisciplinary follow-up
    • Paediatrics, Neurology, Ortho, PT, OT
    • anticonvulsants

2. Surgery

1. VP Shunt

  • hydrocephalus
  • drainage of porencephalic cyst

2. Removal of cyst

 

 

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