Hydrocephalus associated with occipital encephalocele: surgical management and clinical outcome

Abstract

Background Encephaloceles are cystic congenital malformations in which central nervous system (CNS) structures, in communication with cerebrospinal fluid (CSF) pathways, herniate through a defect in the cranium. Hydrocephalus occurs in 60–90% of patients with occipital encephaloceles. Objective Assessment of the surgical management of hydrocephalus associated with occipital encephalocele and its effect on the clinical outcome. Methods Between October 2015 and October 2019, a retrospective study was conducted on seventeen children with occipital encephaloceles who were operated upon. The presence of progressive hydrocephalus was determined by an abnormal increase in head circumference and an increase in the ventricular size on imaging studies. A ventriculoperitoneal (VP) shunt was applied in patients who had hydrocephalus. The clinical outcome was graded according to the developmental milestones of the children on outpatient follow-up visits. Results The mean age at surgery was 1.6 (range, 0–15) months. There were ten girls (58.8%) and seven boys (41.2%). Ten encephaloceles (58.8%) contained neural tissue. Ten patients (58.8%) had associated cranial anomalies. Eleven children (64.7%) had associated hydrocephalus: four of them (36.4%) diagnosed preoperatively, while seven children (63.6%) developed hydrocephalus postoperatively. Ten of them (90.9%) were managed by VP shunt. All children with hydrocephalus had some degree of developmental delay, including six (54.5%) with mild/moderate delay and five (45.5%) with severe delay. Half of the patients (50%) of the children with occipital encephalocele without hydrocephalus had normal neurological outcome during the follow-up period (p value= 0.034). Conclusions Occipital encephalocele is often complicated by hydrocephalus. The presence of hydrocephalus resulted in a worse clinical outcome in children with occipital encephalocele, so it can help to guide prenatal and neonatal counseling.

The neurodevelopmental outcomes of children with encephalocele: a series of 102 patients

“…15,23,24 In general, ECs have a favorable prognosis with a mortality rate below 10%. 4,6,13,20 However, the presence of some predictors has been shown to worsen the prognosis 9,14 and affect the neurodevelopmental outcome. 2,6,11,12 Many studies have descriptively reported neurodevelopmental outcomes of patients with ECs.…”

“…The general estimated rate of NDD in patients with EC has varied among several studies, with some authors 9,17,19,25 reporting overall rates between 16% and 31%. 6 However, other authors-for example, Lo et al, 2 Da Silva et al, 6 and Nagy and Saleh 20 -estimated the overall rate of NDD to be 52%, 60%, and 82.4%, respectively. Although 94 patients in the present cohort were able to have their NDD outcomes evaluated, the overall NDD rate was estimated to be 33%, which is slightly higher than the average estimated rate in the literature.…”

“…However, nonambulatory and nonverbal status were the main criteria for severe NDD in most studies. 6,16,20 In contrast, Lo et al used two criteria including IQ levels and concurrent deficit in at least two adaptive functions to evaluate NDD status. 2 This approach had limitations when intracranial lesions were present because they caused significant differences in verbal and nonverbal IQs.…”

“…Our data revealed that the posterior EC sac was positively but not statistically associated with NDD, in contrast to anterior ECs, which are frequently associated with normal intelligence and motor development. 5,20 Smaller EC sac size has been associated with normal physical and mental status. 14 Children with posterior EC without hydrocephalus were observed to have normal neurodevelopment.…”

“…1,3,6 Although NDD has been descriptively reported among many studies of ECs, 9,[14][15][16][17][18][19] very few studies have objectively evaluated outcomes and the correlation between predictors and NDD outcomes. 2,6,12,20 In this study, a series of patients with EC has been reviewed for identification of NDD via clinical evaluation and according to the Centers for Disease Control and Prevention (CDC) criteria for child development. In addition, the effect of predictors on NDD and overall prognosis have been investigated.…”