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Cerebral Aqueduct Stenosis

Snapshot

  • An infant boy is brought to the emergency department due to lethargy and enlarging head circumference. The parents deny any recent infections or trauma to the patient. They report he had 2 episodes of emesis and is inconsolable. On physican exam, head circumference is larger than expected. An MRI of the head is performed, which shows aqueductal funnelling and enlargment of the lateral and third ventricles. (Congenital aqueductal stenosis)

Introduction

  • Clinical definition
    • narrowing of the cerebral aqueduct
  • Epidemiology
    • incidence
      • most common cause of 
        • congenital obstructive hydrocephalus
  • Pathogenesis 
    • narrowing of the cerebral aqueduct results in
      • increased cerebral spinal fluid volumes in the ventricular system resulting in
        • obstructive hydrocephalus
    • etiologies include
      • congenital narrowing of the cerebral aqueduct
      • compression (e.g., malignancy and vascular malformation)
      • hemorrhage
      • infection
  • see CSF topic for synthesis/resorption pathway 

Presentation

  • Symptoms
    • headache
    • vomiting
    • decrease consciousness
    • hyperreflexia 
  • Physial exam
    • macrocephaly
      • newborn skulls are not fused allowing the head circumference to expand
  • upward gaze palsy

Imaging

  • MRI
    • indication
      • performed to further evaluate the cause of probable hydrocephalus based on history and physical exam
    • findings
  • enlargement of the lateral and third ventricles

Treatment

  • Operative
    • ventriculostomy
      • indication
        • a treatment option for communicating hydrocephalus
    • ventriculoperitoneal shunt
      • indication
        • a treatment option for communicating hydrocephalus