Aims: To review and present the current knowledge of incidence, signs and symptoms,
diagnosis and treatment of the occipital encephalocele.
Background: Encephalocele (E) is a defect of the neural tube that refers to congenital malformations
featured by skull defect and dura with extracranial spread of intracranial structures. Occipital
encephalocele (OE) are the most common form of this congenital disorder and are manifested as a
swelling of different sizes over the occipital bone in the midline. Proper diagnosis and treatment is
highly important in the management of this congenital malformation of brain.
Objective: To review and present the current knowledge of incidence, signs and symptoms, diagnosis
and treatment of the occipital encephalocele.
Methods: We conducted a search of case reports or case-series of patients by the use of electronic
databases: Pub Med, Medline, Index Medicus, Scorpus. The key words were: encephalocele, occipital
encephalocele, neural tube defect, congenital malformation. The search was updated to December
31, 2018. Papers published in English were the only source of information.
Results: Occipital encephalocelle are more frequent in females than in males. The incidence is between
1 in 3000 to 1 in 10,000 live births; approximately 90% of them involve the midline. Magnetic
resonance imaging is the method of choice in diagnosis and surgery is the best option for the
treatment of OE. Overall morbidity and mortality is still high in spite of advenced surgical management,
but have been significantly improved in recent years thanks to sophisticated highresolution
imaging, adequate and proper surgical treatment and decent post-operative care.
Conclusion: Occipital encephalocele is the most common form of encephalocele. The diagnosis is
mostly based by the use of neuroimaging techniques. Operation is the best option for treatment.
Overall morbidity and mortality is still high, but have been significantly improved in recent years
thanks to sophisticated high-resolution imaging, adequate and proper surgical treatment and decent