Hydranencephaly

Hydranencephaly is a rare neurological disorder that affects the brain's development. It is characterized by the absence of the cerebral hemispheres, which are replaced by sacs filled with cerebrospinal fluid.

Overview

Hydranencephaly is a type of cerebral cortical dysplasia, which refers to disorders related to the abnormal development of the cerebral cortex. It is considered a severe form of porencephaly, a condition where a cyst or cavity disrupts the brain's normal structure.

Causes

The exact cause of hydranencephaly is unknown. However, it is believed to result from vascular disruptions in the brain during the prenatal period. These disruptions could be due to infection, trauma, or ischemia.

Symptoms

Symptoms of hydranencephaly may include microcephaly (abnormally small head), spastic quadriparesis (paralysis of all four limbs), seizures, and intellectual disability. Infants with this condition may also have an abnormal growth pattern, poor thermoregulation, and difficulties with swallowing.

Diagnosis

Diagnosis of hydranencephaly is typically made through ultrasound during pregnancy or after birth. Other diagnostic tools may include magnetic resonance imaging (MRI) and computed tomography (CT) scans.

Treatment

There is no cure for hydranencephaly. Treatment is supportive and aims to manage symptoms. This may include physical therapy, occupational therapy, and medication to control seizures.

Prognosis

The prognosis for individuals with hydranencephaly is generally poor, with most individuals not surviving past infancy. However, with appropriate supportive care, some individuals may live into childhood or adolescence.

See also

• Holoprosencephaly
• Alobar holoprosencephaly
• Semilobar holoprosencephaly
• Lobar holoprosencephaly