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.

Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele

“…3,5,17,22,27,34 The prognosis for a newborn with an encephalocele depends on a number of factors and includes the location, size, and contents of the encephalocele, along with microcephaly and associated anomalies. 4,16,27,34 A significant proportion of these children lack normal developmental milestones and may have neurological problems such as ataxia, visual impairment, mental and growth retardation, and seizures. 22 Previous studies report that prognosis for patients with occipital and parietal encephaloceles is directly related to the presence of neural tissue, hydrocephalus, microcephaly, and accompanying cerebral malformations.…”

Anaesthetic Management of a Giant Encephalocele: Size Does Matter

“…Factors well known to determine the prognosis of patients diagnosed with occipital encephaloceles include the size of the sac, neural tissue content, hydrocephaly, infection, and pathologies that accompany this condition [13]. In this case, although the size of the sac was bigger than ever reported before and neural tissue was also involved, we experienced a better immediate postoperative outcome, perhaps because of the larger amount of remaining functional brain mass after the surgery.…”

Occipital Meningoencephalocele case report and review of current literature

“…There are some factors that may influence the prognosis of patients with meningoencephalocele, The size of the herniated sac and the amount of brain tissue it contains determine the prognosis of these patients. Frontoethmoidal meningoencephalocele appears to have better prognosis [3]. Zuckerman et al reported that the reoccurrence of such cases after the initial surgery was approximately 16% with an average of 7 month interval between the first surgery and the reoccurrence [11].…”

“…In addition, hydrocephaly, infections, and other anomalies accompanying the condition determine the prognosis as well. The mortality rate approaches 30% despite the applied appropriate treatments [3].…”