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Related Concept Videos

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...
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Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
14:58

Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations

Published on: October 20, 2017

Pediatric extracranial arteriovenous malformations.

Gresham T Richter1, James Y Suen

  • 1Department of Otolaryngology-Head and Neck Surgery, University of Arkansas for Medical Sciences, Arkansas, USA. gtrichter@uams.edu

Current Opinion in Otolaryngology & Head and Neck Surgery
|May 4, 2012
PubMed
Summary

Pediatric extracranial arteriovenous malformations (AVMs) are dangerous vascular birthmarks that require early intervention. While embolization and surgery offer control, high recurrence rates highlight the need for further research and novel treatments.

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Area of Science:

  • Vascular Surgery
  • Pediatric Surgery
  • Medical Genetics

Background:

  • Arteriovenous malformations (AVMs) are congenital vascular anomalies characterized by abnormal connections between arteries and veins.
  • These anomalies possess unlimited growth potential and often present subtly in childhood, with significant expansion typically occurring during early adolescence.
  • AVMs commonly affect the midface, oral cavity, and limbs, exhibiting relentless local invasion and progression.

Purpose of the Study:

  • To review current knowledge on the natural history, pathophysiology, and treatment of pediatric extracranial AVMs.
  • To explore recent advancements and challenges in managing these complex vascular lesions in children.
  • To emphasize the need for improved therapeutic strategies due to high recurrence rates.

Main Methods:

  • Literature review focusing on pediatric extracranial AVMs.
  • Analysis of disease progression, genetic factors, and current management modalities.
  • Evaluation of treatment outcomes, including recurrence rates.

Main Results:

  • AVMs typically present with subtle signs in childhood, progressing significantly in adolescence, potentially influenced by hormonal changes.
  • Disease location and depth critically impact patient outcomes, with diffuse disease being more challenging to treat.
  • Current management involves embolization and surgical resection; expectant observation is not advised, and pharmacotherapy is unavailable.
  • Early intervention combining supraselective embolization and surgical extirpation offers the best disease control, despite high recurrence rates (93%) due to difficulty in identifying disease margins.

Conclusions:

  • Extracranial AVMs represent a significant clinical challenge due to their dangerous nature and limited research.
  • While surgical excision and embolization provide disease control, they do not guarantee a cure.
  • Further research is crucial for developing novel treatment options to improve outcomes for pediatric patients with AVMs.