Acute and subacute brain injury due to perinatal asphyxia.
The fundamental abnormality in HIE is a deficit of oxygen supply. This is due to hypoxemia (diminished amount of oxygen in th blood supply) and ischemia (a diminished amount of blood perfusing the brain). Loss of cerebrovascular autoregulation (the ability of brain vessels to maintain a constant cerebral blood flow in spite of fluctuations in the cerebral perfusion pressure) which occurs in asphyxiated newborns also contributes to the injury.
During the perinatal period hypoxemia and/or ischemia result most commonly from asphyxia. Various causes are listed above. Only 10% of cases occur in the early postnatal period with diseases in organs responsible for oxygen delivery. These are pulmonary system diseases (airway obstruction, tracheoesophageal fistula, pneumothorax, pneumonia, hypoplastic lungs, diaphragmatic hernia etc.) and nonpulmonary causes (heart defects, heart failure etc.).
There is no single presentation of this disease. Extension and distribution of brain lesions depend upon the maturational stage of the brain and the severity and duration of asphyxial injury. In premature infant the target region for pathologic damage is the periventricular white matter. Anoxic and ischemic damage to the cerebral cortex is characteristic to the term infant. Ischemic damage to the basal ganglia, thalamus and nuclei od midbrain and brain stem may be seen in infants of any gestational age.
Mild HIE
Moderate and severe HIE
Prognosis
Mortality rate is 50 – 75% in severe HIE. Most deaths occur in the first weeks of life due to multiorgan failure, cardiorespiratory arrest or pneumonia. 80% of infants who survive develop serious complications — mental retardation, epilepsy, cerebral palsy or combination of these. Some infants with history of mild to moderate HIE have significant learning disabilities and ADHD in spite of absence of obvious signs of brain injury
Cerebral palsy is non-progressive motor deficit sustained in the perinatal period. The neuropathology of cerebral palsy is complex including not only hypoxic-ischemic encephalopathy itn the perinatal period but also malformations, traumatic lesions, infections and inborn metabolic disordes. The exact time of injury often remains uncertain, probably only 10 — 25% cases are related to intrapartum asphyxia.
Major neuropathological patterns of injury in HIE
Selective neuronal necrosis
White matter lesions
Combined grey and white matter lesions
Late stages of HIE in survivors
Unifocal pseudocyst
Normal brain, term infant: Normal brain, newborn, Macro, autopsy (73839) Normal brain, newborn, Macro, autopsy (73840) Normal brain, newborn, Macro, autopsy (73838)
Cut slides of normal brain, term neonate: Normal brain, newborn, Macro, autopsy (73841)
Term infant with severe birth asphyxia due to amniotic fluid embolism in the mother which suddenly developed during the delivery. Apgar score 0-3-3-4. Early onset of seizures after birth, coma, multiorgan failure. Severe hypoxic-ischemic encephalopathy and death at 1 month of age. Hypoxic encephalopathy, Macro, autopsy (73808) Hypoxic encephalopathy, Macro, autopsy (73812) Hypoxic encephalopathy, Macro, autopsy (73811) Hypoxic encephalopathy, Macro, autopsy (73815) Hypoxic encephalopathy, Macro, autopsy (73810) Hypoxic encephalopathy, Macro, autopsy (73813) Hypoxic encephalopathy, Macro, autopsy (73814) Hypoxic encephalopathy, Macro, autopsy (73809)
Severe hypoxic-ischemic encephalopathy in term neonate: Hypoxic encephalopathy, Macro, autopsy (73804) Hypoxic encephalopathy, Macro, autopsy (73802) Hypoxic encephalopathy, Macro, autopsy (73807) Hypoxic encephalopathy, Macro, autopsy (73805) Hypoxic encephalopathy, Macro, autopsy (73803) Hypoxic encephalopathy, Macro, autopsy (73806)
Hypoxic-ischemic encephalopathy in a child surving for 1 year: Hypoxic encephalopathy, Macro, autopsy (73798) Hypoxic encephalopathy, Macro, autopsy (73799) Hypoxic encephalopathy, Macro, autopsy (73800) Hypoxic encephalopathy, Macro, autopsy (73801)
Porencephaly in 7-week old infant who died of protracted septic shock: Porencephaly, Macro, autopsy (73875) Porencephaly, Macro, autopsy (73874)
Hypoxic-ischemic encephalopathy: Hypoxic encephalopathy, Macro, autopsy (74392) Hypoxic encephalopathy, Macro, autopsy (74393)
Hypoxic-ischemic encephalopathy, multicystic: Hypoxic encephalopathy, multicystic, Macro, autopsy (74385) Hypoxic encephalopathy, multicystic, Macro, autopsy (74386) Hypoxic encephalopathy, multicystic, Macro, autopsy (74387) Hypoxic encephalopathy, multicystic, Macro, autopsy (74388) Hypoxic encephalopathy, multicystic, Macro, autopsy (74389) Hypoxic encephalopathy, multicystic, Macro, autopsy (74390) Hypoxic encephalopathy, multicystic, Macro, autopsy (74391)