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Hydranencephaly is a type of cephalic disorder. This is a rare condition in which the cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid.
In “hemihydranencephaly”, only half of the brain is filled with fluid.[1]
Usually the cerebellum and brainstem are formed normally, although in some cases the cerebellum may also be absent. An infant with hydranencephaly may appear normal at birth or may have some distortion of the skull and upper facial features due to fluid pressure inside the skull. The infant’s head size and spontaneous reflexes such as sucking, swallowing, crying, and moving the arms and legs may all seem normal, depending on the severity of the condition. However, after a few weeks the infant usually becomes irritable and has increased muscle tone (hypertonia). After several months of life, seizures and hydrocephalus may develop. Other symptoms may include visual impairment, lack of growth, deafness, blindness, spastic quadriparesis (paralysis), and intellectual deficits.
Some infants may have additional abnormalities at birth including seizures, myoclonus (involuntary sudden, rapid jerks), and respiratory problems.
Hydranencephaly is an extreme form of porencephaly, which is characterized by a cyst or cavity in the cerebral hemispheres, and may be caused by vascular insult or injuries, infections, or traumatic disorders after the 12th week of pregnancy.
Diagnosis may be delayed for several months because the infant’s early behavior appears to be relatively normal. Transillumination, an examination in which light is passed through body tissues, usually confirms the diagnosis.
Preliminary diagnosis may be made in utero via standard ultrasound, and can be confirmed with a level II or higher ultrasound.
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