Pathophysiology of isolated lateral ventriculomegaly in shunted myelodysplastic children

M S Berger1, J Sundsten, R J Lemire

  • 1University of Washington Medical Center, Seattle.

Pediatric Neurosurgery
|January 1, 1990
PubMed

Insights

Low-pressure shunt valves in myelodysplastic children can cause isolated ventricles, leading to complications. Surgeons should avoid these specific valves to prevent post-shunt ventricle isolation in this patient population.

Area of Science:

  • Pediatric Neurosurgery
  • Neurology
  • Medical Device Safety

Background:

  • Myelomeningocele is a congenital condition often requiring hydrocephalus treatment.
  • Shunt insertion is a common procedure for managing hydrocephalus.
  • Isolated ventriculomegaly can occur post-shunt insertion, particularly in children with myelodysplastic conditions.

Purpose of the Study:

  • To investigate the link between low-pressure distal slit valve systems and isolated lateral ventriculomegaly in myelodysplastic children.
  • To identify potential complications associated with specific shunt valve types in this pediatric population.
  • To provide recommendations for preventing shunt-related complications in myelodysplastic children.

Main Methods:

  • Retrospective analysis of eight myelodysplastic children who developed isolated lateral ventriculomegaly after shunt insertion.
  • Review of shunt system types used, specifically noting the low-pressure distal slit valve (Uni-shunt).
  • Magnetic resonance imaging (MRI) with three-dimensional reconstructions to visualize ventricular anatomy and foramen of Monro distortion.

Main Results:

  • All eight patients developed isolated lateral ventriculomegaly following the insertion of a low-pressure distal slit valve system.
  • Six out of eight children required a second shunt due to symptomatic isolated ventricles.
  • MRI revealed ipsilateral ventricular collapse secondary to foramen of Monro distortion, clearly depicted by 3D reconstructions.

Conclusions:

  • Low-pressure distal slit valves (Uni-shunt) are associated with the development of isolated ventricles in myelodysplastic children.
  • These valves can lead to complications requiring additional surgical intervention.
  • Avoidance of low-pressure distal slit valves is recommended in myelodysplastic children to prevent post-shunt ventricle isolation.

Related Concept Videos

Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in 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...