Operative survival of Children with Congenital Encephalocele at Zewditu Memorial Hospital, Addis Ababa University, College of health sciences and School of Medicine, Department of Surgery, Neurosurgery Unit, Addis Ababa, Ethiopia
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Date
2024-10-03
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Addis Ababa University
Abstract
Background; Encephalocele, a subtype of neural tube defects, is characterized by the protrusion of cranial contents through a defect in the skull. The goal of surgery for encephalocele involves excision of the sac, followed by watertight dural repair, and often reconstruction of the bony defect. Survival of children born with EC varies widely across the regions.
Objective; the main purpose of the study was to determine the operative survival rates of children with congenital encephalocele and the factors predicting operative survival outcomes. The study also aimed to assess patterns of congenital encephalocele occurrence. Materials and Methods; we retrospectively reviewed the medical charts of 126 patients who underwent repair of congenital encephalocele at ZMH Pediatric Neurosurgery Center between January 2018 and June 2024. Relevant demographic and clinical data, including age, sex, birth status, location, size and content of encephalocele, neurologic status, associated congenital anomalies, perioperative, and follow-up data were recorded. Survival analyses, including Life Table, Kaplan–Meier, log-rank test, and Cox proportional hazard model, were used to determine the probability of survival at specific times, examine variation in survival of variables by time of surgery, and assess prognostic factors.
Results; Out of a total of 126 cases, 40.5% were males and 59.5% females. The mean age in days at surgery was 130+-50. At delivery, most of the patients (90.5%) were term. Occipital encephalocele was the most common, accounting for 67.5%, followed by occipito-cervical encephalocele (13.5%) and fronto-ethmoidal (9.5%). The sac of EC contained no neuronal tissue in 66.7% of patients and had contained dysplastic/neuronal tissue in 33.3%. Motor strength of the extremities was intact in majority of patients (78.8%) and incomplete in 28 patients (22%). A diagnosis of congenital anomaly was made in 40 patients (31.7%.. The survival probability was 98% at 1 month, 92% at 2 years, 85% at 4 years, and 75% at 6.5 years, with a median follow-up time of 26.86 months (95% CI: 63.102, 73.996). The Kaplan-Meier log-rank test showed that the presence of neuronal tissue within the sac, occipito-cervical location, preterm delivery, presence of preoperative neurologic deficit, and associated congenital anomalies possibly correlated with worse survival outcomes. However, the Cox proportional hazard model demonstrated, presence of neuronal tissue within the sac(p=0.01) and associated congenital anomalies(p=0.006) were statistically significant in predicting operative survival outcomes.
Conclusion; Our study demonstrated that surgery for congenital encephalocele is a common practice in our institution. The presence of neuronal tissue within the sac, and associated congenital malformations, were factors statistically significant correlated with worse survival outcomes. Despite the challenges, our study's overview of the survival outcomes of children operated on for congenital encephalocele has shown almost comparable result to other centers. We believe that this may serve as a foundation for future prospective and long-term studies. It is also important for decision-makers and caregivers who plan for subsequent follow-up and long-term care in patients with congenital Encephalocele.
Keywords: Encephalocele, Operative survival, cranial anomalies
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Keywords
Encephalocele Operative survival cranial anomalies