Prognostic factors in patients with occipital encephalocele

Abstract

Background: An encephalocele is a herniation of the brain and the meninges through a skull defect protruding towards the exterior. The condition is not rare when compared to spinal dysraphisms, but the worldwide incidence is not precisely known. The cases involving occipital encephaloceles which we have diagnosed in our clinic and the surgical approaches for this rare condition are presented herein.

Methods: Thirty patients who were diagnosed with occipital encephaloceles and referred to our Neurosurgery Clinic at the Yuzuncu Yil University, Faculty of Medicine Research Hospital between 2000 and 2009 were enrolled in this study. The age of the patient, size of the sac, pathologies that accompanied the condition, and treatments applied were assessed.

Results: In the present study, 30 patients (22 girls and 8 boys), whose ages varied between newborn and 14 months, were evaluated. The encephalocele sac was located in the occipital region in 27 patients (90%) and in the occipitocervical region in 3 patients (3%). Nine (30%) of the 30 patients died; 2 in the preoperative period, 2 in the postoperative early period (0-7 days) and 5 in the late postoperative period (first week to 3 months). With the exception of the 2 patients who died preoperatively, surgery was performed on all of the patients. The mortality rate in our study was 29%.

Conclusions: Our study demonstrated that factors which determine the prognosis of patients diagnosed with occipital encephaloceles include the size of the sac, the contents of the neural tissue, hydrocephaly, infections, and pathologies that accompany the condition. An occipital encephalocele is a congenital neurologic condition with an extremely high morbidity and mortality in spite of the treatments rendered pre- and postoperatively.