Association of Hydrocephalus in Children with an Encephalocele




Dandy-Walker malformation, Microcephaly, Chiari III malformation, Encephalocele


Objectives: To describe the association between the occipital encephaloceles with hydrocephalus and how to improve the outcome of patient.

Methodology: Our Prospective study collected records of fifty patients diagnosed with posterior encephaloceles. Data were collected on the gender, location of the encephalocele, presence of neural tissue , dandy walker, microcephaly and hydrocephalus.

Results: Twenty-nine females and twenty-one males were present. Over half of the lesions were supratorcular, while the remaining eight (16%) were torcular and the remaining seventeen (34%) were infratorcular. Primary encephalocele repair were done at an average of 9 days (range 2.5–120 days). In twenty-five of the instances, a diagnosis of hydrocephalus was made. Twenty-four patients had a ventriculo-peritoneal shunt implanted. The average age of implantation of VP shunt was only 1.2 months (range 0.3–9 months). One patient underwent an endoscopic third ventriculostomy with good results. Hydrocephalus was related with Dandy-Walker and ventriculomegaly before encephalocele surgery was performed (p values 0.01 and 0.05, respectively). For hydrocephalus therapy, 

Conclusions: Patients born with encephaloceles frequently suffer from hydrocephalus (50%). This is especially true in cases where the patient also has Dandy-Walker syndrome, CMIII malformation, or pre-existing ventriculomegaly. The severity of large encephaloceles is a limiting factor for the development of hydrocephalus when connected with torcular types and microcephaly.

Author Biographies

Iram Bokhari

Head of department of neurosurgery at jinnah postgradute medical centre, karachi

Tanveer Ahmed

Consultant at neurosurgery department jinnah postgraduate medical centre, karachi

Rabail Akbar

Consultant at neurosurgery department of jinnah postgraduate medical centre, Karachi

Raheel Gohar

Postgraduate traineed of neurosurgery at jinnah postgraduate medical centre, Karachi

Anas Ahmed

postgradute traineed at neurosurgery department of jinnah postgradute medical centre, Karachi


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