Acquired Developmental Defects


Germinal Matrix Hemorrhage

Definition

  • Bleeding into the subependymal germinal matrix zone with or without subsequent dissection into the lateral ventricle (intraventricular hemorrhage)

  • Germinal matrix zone (GMZ):

    • Fetal periventricular structure that forms between the developing deep cerebral nuclei and ependymal lining

    • Present between 13 and 36 weeks of gestation

    • Composed of immature neuroepithelial cells and thin-walled blood vessels with little supportive stroma

Clinical Findings

Epidemiology

  • Prematurity is most important risk factor for germinal matrix hemorrhage (GMH)

    • Affects about 15% of newborns weighing less than 1500 g

    • Neonates with birth weight of 500 g to 750 g at highest risk

  • Incidence declining because of advances in neonatal care

  • Rare after 35 weeks gestational age

  • Acute chorioamnionitis is also a risk factor

  • The causes of GMH are multiple

    • Increased arterial blood pressure, venous congestion, hypoxia, trauma with rupture, and hemorrhage of thin-walled germinal matrix vessels

    • Local anatomic factors include high vascularity, immature fragile capillary bed with poor stromal support

Presentation

  • Respiratory distress, abnormal eye movements, hypotonia, in utero fetal demise

  • CSF obstruction by intraventricular blood with posthemorrhagic hydrocephalus and “myelination delay”

  • Various neurologic sequelae: cerebral palsy, mental retardation, seizures, coma

Prognosis and Treatment

  • Neurodevelopmental outcome correlates with the grade of GMH and the presence of associated abnormalities

  • High mortality with extensive intraventricular hemorrhage

    • About 75% mortality with grades II and IV intraventricular hemorrhage

  • Shunting required for post hemorrhagic hydrocephalus

Imaging Characteristics

  • Four grades of GMH by ultrasound, CT, or MRI:

    • Grade I: confined to the germinal matrix

    • Grade II: extension into the lateral ventricles without ventricular dilatation

    • Grade III: ventricular hemorrhage with ventricular dilatation/hydrocephalus

    • Grade IV: extension in parenchyma/white matter hemorrhage

Pathologic Findings

Gross

  • Pinpoint to massive periventricular hemorrhages adjacent to the deep cerebral nuclei (especially the caudate nucleus)

  • Occur commonly at the level of the foramen of Monro

  • Larger hemorrhages extend into the lateral ventricles

  • Hydrocephalus when CSF low is obstructed by blood or related gliosis/scarring

Histology

  • Acute GMH:

    • Extravasated red cells within GMZ

    • Dissection of blood through stroma-poor GMZ followed by intraventricular hemorrhage

    • Periventricular hemorrhagic infarction

  • Chronic sequelae of GMH:

    • Cavitation of hemorrhagic necrosis with formation of gliotic cyst(s)

    • Hemosiderin-laden macrophages; scarring of ependyma and leptomeninges

    • May have associated hyaline membrane disease and respiratory distress syndrome

Main Differential Diagnoses

  • Hemorrhagic periventricular leukomalacia

  • Coagulopathy

  • Trauma

Fig 1, Germinal matrix hemorrhage. Such hemorrhages are most common in premature, low-birth-weight infants. The image shows bilateral acute GMHs that occurred at about 16 to 18 weeks of gestation.

Fig 2, Germinal matrix hemorrhage. At low magnification, the highly cellular GMZ contains several acute hemorrhages. The developing caudate nucleus ( bottom center ) is below the GMZ.

Fig 3, Germinal matrix hemorrhage. The highly cellular GMZ is composed of immature neuronal and glial cell precursors and contains dilated, thin-walled capillaries, one of which shows recent hemorrhage ( lower left ).

Fig 4, Germinal matrix hemorrhage. Hemorrhages can readily dissect through the stroma-poor GMZ and enter the adjacent ventricle.

Fig 5, Intraventricular hemorrhage/hematoma. An acute blood clot fills the right lateral ventricle. Such hemorrhages may extend throughout the pathways of CSF flow.

Fig 6, Intraventricular hemorrhage/hematoma. Extensive blood in the basal subarachnoid space may occur when intraventricular blood passes through the basal outflow foramina of Luschka and Magendie.

Periventricular Leukomalacia

Definition

  • Selective ischemic injury of periventricular white matter during the fetal/perinatal period and the most common ischemic brain injury in premature infants; periventricular leukomalacia (PVL) encompasses focal necrotic lesions as well as diffuse white matter gliosis (also known as perinatal telencephalic leukoencephalopathy ).

Clinical Features

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