Craniovertebral lipoma associated with hydrocephalus: report of two cases

Kirill Sysoev1, Arina Kulaeva2, Dimitrios Tsiptsios3

  • 1Division of Pediatric Neurosurgery, Almazov National Medical Research Centre, St. Petersburg, Russian Federation. sysoev.rnsi@mail.ru.

Insights

Craniovertebral lipomas in children, though rare, can cause hydrocephalus. Surgical removal of these tumors may still necessitate shunts due to persistent ventriculomegaly, with unclear underlying causes.

Area of Science:

  • Pediatric Neurosurgery
  • Neurology
  • Developmental Biology

Background:

  • Craniovertebral lipomas are rare congenital tumors.
  • Hydrocephalus is an uncommon but serious complication of craniovertebral lipomas.
  • The exact pathogenesis of hydrocephalus in these cases remains poorly understood.

Purpose of the Study:

  • To report two pediatric cases of craniovertebral lipomas associated with hydrocephalus.
  • To discuss the management and outcomes of these rare conditions.
  • To highlight the challenges in understanding hydrocephalus development in this context.

Main Methods:

  • Case presentation of two pediatric patients with craniovertebral lipomas and hydrocephalus.
  • Surgical resection of the craniovertebral lipomas.
  • Management of hydrocephalus, including ventriculoperitoneal shunt placement.
  • Long-term clinical follow-up.

Main Results:

  • Both patients presented with craniovertebral lipomas and hydrocephalus.
  • Surgical lipoma resection did not resolve the hydrocephalus in either case.
  • Both patients required ventriculoperitoneal shunts for exaggerated ventriculomegaly.
  • Symptoms of increased intracranial pressure regressed, and patients remained stable long-term.

Conclusions:

  • Craniovertebral lipomas can lead to complex hydrocephalus requiring surgical intervention beyond tumor removal.
  • The etiology of progressive hydrocephalus post-lipoma resection in children is not fully elucidated.
  • Further research is needed to understand the pathogenesis of hydrocephalus in craniovertebral lipomas.

Related Concept Videos

Brain Abscess l: Introduction01:26

Brain Abscess l: Introduction

A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial infections,...
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...
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...