Publication: Hydranencephaly
Date
2010
Journal Title
Journal ISSN
Volume Title
Publisher
Kathmandu University
Abstract
Abstract
Hydranencephaly is a rare congenital condition where the greater portions of the cerebral hemispheres and the corpus
striatum are replaced by cerebrospinal fluid and glial tissue. The meninges and the skull are well formed, which is
consistent with earlier normal embryogenesis of the telencephalon. Bilateral occlusion of the internal carotid arteries in
utero is a potential mechanism. Clinical features include intact brainstem reflexes without evidence of higher cortical
activity. The infant’s head size and the spontaneous reflexes such as sucking, swallowing, crying, and moving the arms
and legs may all seem normal at birth. However, after a few weeks the infant usually becomes irritable and has increased
muscle tone and after a few months of life, seizures and hydrocephalus (excessive accumulation of cerebrospinal fluid
in the brain) may develop. Other symptoms may include visual impairment, lack of growth, deafness, blindness, spastic
quadriparesis (paralysis), and intellectual deficits. Since the early behaviour appears to be relatively normal, the diagnosis
may be delayed for months sometimes. There is no definitive treatment for hydranencephaly. The outlook for children
with hydranencephaly is generally poor, and many children with this disorder die before their first birthday.
Key words: hydranencephaly, congenital anomaly, vascular disruption, thromboplastin,
Description
Pant S 1, Kaur G2, JK De3
1Medical Officer, 2Associate Professor, 3Professor and Head, Department of Obstetrics and Gynaecology, Manipal College of Medical Sciences