Encephalocele

Encephalocele is a rare and serious deformity that occurs by a protrusion of neural tissue (usually like a sac) through a bony defect in the skull, there are two types congenital (primary) and acquired (secondary) encephaloceles, congenital encephalocele belong to the neural tube defect (NTDs) along with spina bifida and anencephaly. The exact cause of congenital encephalocele is unknown, probably, it is multifactorial and there are roles for genetic and environmental factors, failure of closure of the neural tube during early pregnancy (third to fourth week) is thought the pathogenesis process of congenital encephalocele. According to the contents herniated sac, encephalocele is divided into many subtypes including meningocele, men-ingo-encephalocele, atretic encephalocele, and gliocele. The main classification of encephalocele includes four major types according to the location: occipital, Sincipital, basal, and convexity. The most common type of them depends on geographical region. Usually, encephalocele is present at birth as a visible mass but it may be occult like some cases of basal encephalocele. Ultrasound is the main diagnostic tool for prenatal diagnosis of encephalocele in early pregnancy during the first trimester. MRI plays a major role in diagnosis after birth as MRI demonstrates the contents of the herniated sac and aids in making the surgical decision. in most cases, surgery is the appropriate choice and it is an emergency if there is a risk of meningitis like CSF leakage. Hydrocephalus is the most common complication associated with encephalocele and it should be resolved before the surgery. Morbidity and mortality of encephaloceles have improved in recent years.