Definition and Incidence

  • Intraventricular hemorrhage (IVH) refers to bleeding into the ventricles of the brain.
  • It is a condition noted mostly in extreme preterm babies.
  • The bleeding originates from the fragile capillary network of the subependymal germinal matrix.
  • Severe IVH is defined as hemorrhage into the ventricles with ventricular dilatation or periventricular hemorrhagic infarction (PVHI).
  • The incidence of severe IVH is 10% to 25% among survivors born at 24 weeks of gestational age.
  • The incidence drops significantly to less than 5% in neonates born at 28 weeks of gestation and higher.

Pathophysiology

  • The germinal matrix is the most vascular and metabolically active area of the brain in a baby born before 32 weeks of gestation.
  • This area lacks adequate structural support and begins to involute after 34 weeks of gestation.
  • The proposed mechanisms for bleeding include severe ischemia followed by reperfusion injury.
  • Fluctuations in cerebral circulation that fall outside the normal autoregulation range contribute to the injury.
  • Cerebral venous vasodilation, often following hypercarbia, and venous congestion are also major factors.

Risk Factors

Antenatal and Perinatal Factors

  • Chorioamnionitis and severe placental insufficiency increase the risk of IVH.
  • Fetal growth restriction and absent or reversed umbilical artery doppler flow are significant risk factors.
  • A need for extensive resuscitation at birth increases the likelihood of bleeding.

Postnatal Factors

  • Asphyxia causing the disruption of capillaries leads to IVH.
  • Rapid fluid boluses and volume expansion cause sudden hemodynamic shifts.
  • Hypocarbia, hypercarbia, and the occurrence of pneumothorax are known contributors.
  • The presence of a hemodynamically significant patent ductus arteriosus (hsPDA) increases the risk.

Protective Factors

  • Antenatal administration of corticosteroids and magnesium sulphate are highly protective.
  • Delayed cord clamping and the use of volume-targeted ventilation are associated with a decrease in IVH.

Clinical Presentation

Clinically Silent

  • This is the most common presentation in preterm newborns.
  • It is recognized only when a routine cranial ultrasound (CUS) is performed.
  • An unexplained fall in hematocrit may be the only subtle clinical sign.

Saltatory Presentation

  • Symptoms evolve gradually over days to weeks.
  • Neonates may present with lethargy, apnea, tone abnormalities, and a tight popliteal angle.

Catastrophic Presentation

  • This presentation involves rapid clinical evolution over several hours.
  • It manifests with a bulging fontanelle, a sudden drop in hemoglobin, and decreased consciousness.
  • Additional signs include spontaneous abnormal eye movements, hypotonia, decerebrate posturing, seizures, and severe hypotension.

Grading of IVH (Papile Classification)

Grade I

  • Isolated germinal matrix hemorrhage with no bleeding in the ventricles.

Grade II

  • IVH is present without any ventricular dilatation.

Grade III

  • IVH occurs with ventricular dilatation, occupying more than 50% of the ventricle.

Periventricular Hemorrhagic Infarction (PVHI)

  • This was previously referred to as grade IV IVH or parenchymal extension.
  • Neuropathologic studies show it is a separate lesion consisting of a venous hemorrhagic infarction.
  • It results from the obstruction of blood flow in the terminal vein by a large ipsilateral IVH.

Screening and Diagnosis

  • CUS is the primary imaging modality for screening and diagnosis.
  • It provides an ideal acoustic window through the anterior fontanelle without radiation hazards.
  • Routine CUS screening is recommended for all newborns born before 32 weeks of gestation or weighing less than 1500 g.
  • The first screening ultrasound is typically performed between 7 and 14 days of life.
  • A second scan is performed between 36 and 40 weeks to screen for periventricular leukomalacia.

Complications

Progressive Ventricular Dilation (PVD)

  • Also referred to as post-hemorrhagic hydrocephalus (PHH).
  • It may occur days to weeks after the initial IVH.
  • It is primarily caused by impaired cerebrospinal fluid (CSF) resorption or obstruction by particulate blood clots.

Porencephalic Cyst

  • This is the most common sequelae of PVHI.
  • It presents as a large cyst on the same side as the severe IVH.

Management

Acute Supportive Care

  • Lower grades of IVH (I and II) require no specific change in clinical care.
  • Supportive care aims to maintain stable cerebral perfusion by keeping blood pressure and blood gases in a normal range.
  • Preventive measures include avoiding rapid fluid boluses, hypercarbia, hypoxia, and pneumothorax.
  • Thrombocytopenia and coagulation disturbances should be corrected.
  • Transfusion of packed red blood cells may be required for large IVH to restore blood volume.

Management of PVD and PHH

  • Serial ultrasound measurements are necessary to monitor ventricular size using the Levene index.
  • Clinical monitoring includes measuring head circumference on alternate days to detect excessive growth.
  • Serial therapeutic lumbar punctures can be used to remove CSF if communication exists with the subarachnoid space.
  • Slowly progressive PVD may resolve spontaneously.
  • Permanent CSF diversion with a ventriculoperitoneal (VP) shunt may be required for established hydrocephalus.

Prognosis

  • The severity of the IVH is the primary predictor of adverse short-term and long-term outcomes.
  • Even milder grades of IVH have an increased risk of cerebral palsy (CP) and cognitive impairment.
  • Newborns with PVHI have a significantly higher risk for neurodevelopmental deficits.
  • Localized PVHI can cause spastic hemiparesis, whereas extensive PVHI leads to quadriparesis and mental retardation.