What is the Lifespan for a Child with Holoprosencephaly?

Discussion Holoprosencephaly (HPE) is a clefting problem of the brain. “[HPE] the result of incomplete or absent midline division of the embryonic forebrain into distinct cerebral hemispheres (prosencephalon) between the 18th and 28th day after conception.” There are four distinct subtypes: Alobar – both hemispheres are completely fused and are not separated into the left and right hemispheres. There is agenesis of the corpus callosum, arrhinencephaly and a single ventricle with fused thalami. Facial features are almost always affected. Semilobar – the cerebral hemispheres are fused anteriorly but are separated posteriorly. There is agenesis or hypoplasia of the corpus callosum and the olfactory bulbs and tracts are usually absent. Facial features are often affected. Lobar – the cerebral hemispheres are almost completely separated however the corpus callosum and olfactory lobes may be incompletely developed. Facial features may not be affected or are affected to a lesser degree. Middle interhemispheric – occurs when the posterior frontal and parietal lobes are incompletely separated. This is the least severely affected form. The prevalence of HPE is one in 10,000 to 100,000 live births depending on the study. In certain subtypes there is a female preponderance. “Cyclopia a congenital malformation characterized by the presence of a single eye,…located in the middle of the face in the place normally occupied by the roof of t...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news