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Atlas of Neonatal Pathology
Marta Ježová, Josef Feit
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+ Introduction
+ Using this atlas
+ Conditions of using this atlas
+ Technical notes
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+ Support
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+ Neonatal pathology
+ Basic terms and classification of newborn infants
+ The Apgar score
+ Classification of newborn infants by weight and gestation
+ Pathology of gestation length
+ Prematurity
+ Postmaturity (Clifford syndrome)
+ Growth and developement
+ Intrauterine growth retardation (IUGR), small for gestational age infant (SGA)
+ Large for gestational age infant (LGA)
+ Physical maturity
+ Pathology of prematurity
+ Gross appearance of premature infant
+ Difficulties in maintaining homeostasis
+ Thermoregulation
+ Hypoglycemia
+ Hyperbilirubinemia
+ Fluids and electrolytes
+ Apnea of prematurity
+ Anemia of prematurity
+ Hyaline membrane disease (HMD)
+ Necrotizing enterocolitis (NEC)
+ Intraventricular hemorrhage (IVH)
+ Periventricular leukomalacia (PVL)
+ Patent ductus arteriosus (PDA)
+ Iatrogenic diseases and damage
+ Respiratory system
+ Injuries caused by endotracheal intubation
+ Pulmonary air leak
+ Bronchopulmonary dysplasia (BPD)
+ Retinopathy of prematurity (ROP)
+ Cardiovascular system
+ Total parenteral nutrition associated hepatic damage
+ Infections
+ Viability, survivability and long term sequelae of prematurity
+ Viability
+ Survivability in prematurity
+ Severe long term sequelae in babies born prematurely
+ Birth asphyxia
+ Hypoxic-ischemic encephalopathy (HIE)
+ Meconium aspiration syndrome (MAS)
+ Persistent pulmonary hypertension of the newborn (persistent fetal circulation)
+ Birth trauma
+ Extracranial lesions
+ Soft tissus injuries
+ Intraabdominal injuries
+ Peripheral and cranial nerve injuries
+ Bone injuries
+ Cranial trauma
+ Perinatal infection
+ Intrauterine infections (TORCH group)
+ Sepsis neonatorum
+ Blenorrhea neonatorum
+ HIV infection
+ Stillbirth and perinatal mortality
+ Stillbirth
+ Perinatal mortality
+ Neonatal pathology nonrelated to prematurity, birth asphyxia or infection
+ Congenital anomalies in liveborn infants
+ Kernicterus
+ Hemorrhagic disease of the newborn
+ Spontenous gastric perforation in a neonate
Intraventricular hemorrhage (IVH)
Introduction

Germinal matrix consists of primitive neuroectodermal cells lying between the ependyma and nc. caudatus. Primitive neurons and later glial cells are generated in the zone and migrate out to their final position. The germinal zone is programmed to melt away after 32 to 34 weeks. Hemorrhage into the germinal matrix with spread of blood to the lateral ventricles is characteristic of premature infants less than 32 weeks gestation. There is strong correlation of IVH and mechanical ventilation, barotrauma, severe HMD, hypoxia, hypercapnia, patent ductus arteriosus, rapid volume expansion in the first days and intrauterine growth retardation. The incidence and severity are inversely related to gestational age.

Etiology

The pathogenesis is multifactorial. The final hypothesis is that damage from factors listed below especially hypoxia and transient rises of arterial pressure lead to rupture of fragile capillaries in the periventricular germinal matrix which is the origin of germinal matrix hemorrhage. This deletorious effect is even accentuated by the cerebral vasodilation caused by the hypercapnia and hypoxia with RDS.

  • intravascular factors
    • inadequate autoregulatory mechanisms in sick preterm infants (all alterations in systemic blood pressure are transmitted directly to the germinal matrix vasculature)
    • fluctuating cerebral blood flow (both increases and decreases in cerebral blood are likely to play a role)
    • increase in cerebral blood flow is related to hypercapnia and rapid elevations of systemic blood flow with handling, tracheal suctioning, seizures, pneumothorax
    • decreases in cerebral blood flow are most likely to occur with perinatal asphyxia, postnatally with episodes of prolonged apnea with hypotension
    • increase in cerebral venous pressure occurs during labor and delivery with extensive skull deformation in preterm infants, with asphyxia and respiratory difficulties after birth
  • vascular
    • tenuous capillary integrity
    • endothelium is extremely vulnerable to hypoxic-ischemic injury
  • extravacular
    • deficient vascular support
    • high fibrinolytic activity in the periventricular region
Classification
  • Grade I.  —  germinal matrix hemorrhage
  • Grade II.  —  intraventricular hemorrhage without ventricular dilatation
  • Grade III.  —  intraventricular hemorrhage with ventricular dilatation
  • Grade IV.  —  hemorrhage extending into the brain parenchyma irrespective of the presence or absence of intraventricular hemorrhage (periventricular hemorrhagic infarction). The infarction is possibly venous, typically unilateral and extensive.
Clinical signs
  • sudden detorioration with bulging fontanelle
  • worsening of the respiratory diseases often with apnea
  • cardiovascular instability with marked variability in blood pressure
  • decreased hematocrit
  • seizures
  • lethargy, coma
  • asymptomatic course is not uncommon.
  • 90% of hemorrhage occur within 72 hours after delivery
  • diagnosed using cranial ultrasound
  • prognosis is correlated with the grade of IVH
  • complications
    • posthemorrhagic hydrocephalus
    • major psychomotoric delay in more than 90% of survivors with grade IV. Associated lesions (periventricular leukomalacia, pontosubicular necrosis etc.) also contribute to suboptimal outcome in survivors

Death is the result of increased intracranial pressure, hernation and depression of function of the vital medullary centers.

Macroscopic appearance

Subependymal hematoma over the nc. caudatus, may be bilateral or mutiple.

Intraventricular hemorrage follows the rupture of subependymal hematoma with spread through the ventricular system  —  to the opposite lateral ventricle, third ventricle, aqueduct, fourth ventricle and subarachnoid space of the cisterna magna.

Pictures

Intraventricular hemorrhage, 1st grade: Intraventricular hemorrhage, 1st grade, Macro, autopsy (74397)

Intraventricular hemorrhage, 3rd grade: Intraventricular hemorrhage, 3rd grade, Macro, autopsy (74398)

Intraventricular hemorrhage in premature 27-week at 2 days of age in a newborn child who also suffered from severe respiratory distress syndrome. The birth weight was 990 g. Intraventricular hematoma, Macro, autopsy (73828) Intraventricular hematoma, Macro, autopsy (73829)

Intraventricular hemorrhage in extremely premature 24-week neonate, twin B. Birth weight was 780 g. Hemorrhage in lateral ventricles is assymetric, almost unilateral. Hematoma is readily seen in subarachoid cisterna magna. Death occured at the age of 6 days. Intraventricular hematoma, Macro, autopsy (73826) Intraventricular hematoma, Macro, autopsy (73827)

Periventricular hemorrhagic infarction in premature twin infant born at 29 weeks with birth weight 1150 g. The baby suffered from birth asphyxia (Apgar score 1-3-6) and respiratory distress syndrome. Hemorhagic infarction, periventricular leucomalacia, Macro, autopsy (73792) Hemorhagic infarction, periventricular leucomalacia, Macro, autopsy (73793) Hemorhagic infarction, periventricular leucomalacia, Macro, autopsy (73794) Hemorhagic infarction, periventricular leucomalacia, Macro, autopsy (73795) Hemorhagic infarction, periventricular leucomalacia, Macro, autopsy (73796) Hemorhagic infarction, periventricular leucomalacia, Macro, autopsy (73797)

Periventricular hemorrhage in the right occipital lobe with spread of hemorrhage in the ventricle system. Premature birth at 36 weeks with birth asphyxia. Intraventricular hematoma, Macro, autopsy (73830)

Intraventricular hemorrhage: Intraventricular hemorrhage, Macro, autopsy (74399)