Natural history of hydrocephalus in children with spinal open neural tube defect

Essam A Elgamal1

  • 1Neurosurgery Division, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.

Insights

Hydrocephalus is common in Spinal Open Neural Tube Defect (SONTD) patients, often linked to Chiari malformation type II. Early MRI and prompt treatment are crucial for managing SONTD-associated hydrocephalus.

Area of Science:

  • Pediatric Neurosurgery
  • Developmental Neuroscience
  • Clinical Neurology

Background:

  • Long-term prognosis for Spinal Open Neural Tube Defect (SONTD)-associated hydrocephalus remains poorly understood.
  • This study aimed to determine the incidence and natural history of hydrocephalus in SONTD patients.

Purpose of the Study:

  • To investigate the incidence and natural history of hydrocephalus in patients with SONTD.
  • To analyze the management and outcomes of hydrocephalus in SONTD patients.

Main Methods:

  • Retrospective study of 82 SONTD patients over 1-16 years.
  • Patients classified into groups with active, compensated, or no hydrocephalus.
  • Analysis of treatment timing, complications, and hydrocephalus status.

Main Results:

  • Hydrocephalus affected 72% of SONTD patients, frequently associated with Chiari malformation type II.
  • Ventriculoperitoneal shunt (VPS) and endoscopic third ventriculostomy (ETV) were primary treatments; VPS revision/replacement occurred in 19.6% due to complications.
  • No treatment was required for patients with compensated or no hydrocephalus during follow-up.

Conclusions:

  • Hydrocephalus is prevalent in SONTD patients with Myelomeningocele and Chiari malformation type II, necessitating close monitoring and timely intervention.
  • Routine brain and craniocervical junction MRI is recommended for SONTD patients to assess ventricular size and Chiari malformation type II.
Abstract

Related Concept Videos

Neurulation01:30

Neurulation

Neurulation is the embryological process which forms the precursors of the central nervous system and occurs after gastrulation has established the three primary cell layers of the embryo: ectoderm, mesoderm, and endoderm. In humans, the majority of this system is formed via primary neurulation, in which the central portion of the ectoderm—originally appearing as a flat sheet of cells—folds upwards and inwards, sealing off to form a hollow neural tube. As development proceeds, the anterior...
Increased Intracranial Pressure l: Introduction01:14

Increased Intracranial Pressure l: Introduction

Intracranial hypertension is a sustained elevation of intracranial pressure (ICP) above 22 mm Hg. In supine adults, normal ICP is ~7–15 mm Hg.The rigid, nonexpandable cranium contains three components—brain tissue, blood, and cerebrospinal fluid (CSF)—that total ~1,700 mL in a typical adult: 1,400 mL brain (~80%), 150 mL blood (~10%), and 150 mL CSF (~10%). According to the Monro–Kellie doctrine, total intracranial volume is effectively fixed. When one component expands, CSF and venous blood...
Increased Intracranial Pressure ll: Pathophysiology01:29

Increased Intracranial Pressure ll: Pathophysiology

Increased intracranial pressure (ICP) refers to a potentially life-threatening rise in pressure inside the skull. This usually happens when there is a major change in the volume of brain tissue, blood, or cerebrospinal fluid (CSF) — the three components inside the skull. According to the Monro-Kellie doctrine, if the volume of one component increases, the volumes of the other components must decrease to maintain normal pressure. If this does not happen, ICP rises.The process often begins with...
Anatomy of the Brain: Ventricles01:18

Anatomy of the Brain: Ventricles

There are hollow fluid-filled cavities known as ventricles deep inside the human brain. There are two lateral ventricles, one in each cerebral hemisphere, and each has three different projections — the anterior, inferior, and posterior horns visible from the lateral side. A thin membrane called the septum pellucidum separates the two lateral ventricles. The slender third ventricle in the diencephalon is connected to each lateral ventricle via a channel called the interventricular foramen. The...
Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...
Cerebral Edema l: Introduction01:19

Cerebral Edema l: Introduction

Cerebral edema is a pathological increase in brain water content that disrupts intracranial pressure regulation and impairs neurological function. Because the cranial vault is rigid, even modest increases in tissue volume can compromise cerebral perfusion, distort neural structures, and initiate secondary injury. Cerebral edema develops through four principal mechanisms: vasogenic, cytotoxic, interstitial, and ionic.Vasogenic EdemaVasogenic edema arises from disruption of the blood–brain...