Abstract

Background: Different surgical management of infants suffering neural tube defects (NTD) associated hydrocephalus were reported in the literature. Great debate whether combining endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) is more effective than ventriculoperitoneal (VP) shuntfor management of congenital hydrocephalus secondary to neural tube defects ininfants younger than 1 year of age. Purpose: To evaluate and compare the therapeutic efficacy of ETV combined with CPC versus VP shunts in infants with infantile hydrocephalus associated with NTD. Methods: Thirty infants with infantile NTD associated hydrocephalus (de novo), were equally divided and randomly allocated to each intervention group either ETV/CPC or VP shunts). They were monitored for at least 6 months for any sign of raised intracranial pressure (rICP) and/or hydrocephalic metrics (fontanelle quality, HC, and ventricular size) were also documented and compared between two groups. Statistical Analysis: The association between intervention group and outcome was tested with Chi-square test and P=0.05 or less was considered statistically significant. Results: Of the total thirty patients included in the study, thirteen patients (43.3%) were male and seventeen were female (56.7%) with mean age of 80.3±11.5 days. Twenty patients (66%) were under 6 months of age. The overall success rate in 1-year follow-up was 54% and 60% for VP shunt and ETV/CPC, respectively; with the difference being not statistically significant. Conclusion: Combined ETV-CPC is considered a successful surgical option for treatment of infantile hydrocephalus associated with NTD; achieving success rate better than VPS implantation.

Highlights

  • Hydrocephalusis described as abnormal accumulation of cerebrospinal fluid (CSF) within the brainventricles, as a result of disturbance of CSF formation, flow, or absorption and results in raised intracranial pressure leading to abnormal increase in head circumference when occurring in infancy [1, 3, 15]

  • Many theories tried to explain the pathophysiology of Neural tube defects (NTDs)-associated hydrocephalus but it is not fully explained; one theory claimed that inappropriate in-utero CSF outflow through the meningiocele defect leads to under development of normal CSF drainage pathways

  • Pre-operative clinical examination demonstrated that all our cases in both groups had bulged AF, signs of raised intracranial pressure (rICP) and the HC was above 2SD in all cases

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Summary

Introduction

Hydrocephalusis described as abnormal accumulation of cerebrospinal fluid (CSF) within the brainventricles, as a result of disturbance of CSF formation, flow, or absorption and results in raised intracranial pressure (rICP) leading to abnormal increase in head circumference when occurring in infancy [1, 3, 15].Neural tube defects (NTDs) are a group of conditions in which an opening in the spinal cord or brain remains from embryonic life resulting in group of disorders including spina bifida, an encephaly and encephalocele. Many theories tried to explain the pathophysiology of NTD-associated hydrocephalus but it is not fully explained; one theory claimed that inappropriate in-utero CSF outflow through the meningiocele defect leads to under development of normal CSF drainage pathways These theories constitute the scientific background for the initial several researches both in animals and human aiming at assessment of role of prenatal surgical closure MMC, trying to initiate more hope. Methods: Thirty infants with infantile NTD associated hydrocephalus (de novo), were divided and randomly allocated to each intervention group either ETV/CPC or VP shunts). They were monitored for at least 6 months for any sign of raised intracranial pressure (rICP) and/or hydrocephalic metrics (fontanelle quality, HC, and ventricular size) were documented and compared between two groups. Conclusion: Combined ETV-CPC is considered a successful surgical option for treatment of infantile hydrocephalus associated with NTD; achieving success rate better than VPS implantation

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