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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
Insights
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.
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.
Purpose Of Review:
Arteriovenous malformations (AVMs) are congenital vascular anomalies composed of a 'nidus' of inappropriately connected arteries and veins with an unlimited growth potential. This review aims to explore new information on the natural history, pathophysiology, and treatment for pediatric extracranial AVMs.
Recent Findings:
AVMs are frequently dormant during childhood with only subtle signs of disease. Expansion occurs, on average, during early adolescence, during which hormonal changes are thought to play a role. AVMs have a site predilection for the midface, oral cavity, and limbs. Relentless disease progression with local invasion is the rule. Focal or diffuse disease may be present, with the latter being more difficult to treat. Location and depth of disease affect patient outcomes. Genetic and molecular factors involved in vascular stabilization are aberrant in AVMs. Management techniques include embolization and surgical resection, separately or in combination. Expectant observation is no longer recommended. Pharmacotherapy is currently not an option. Early intervention with supraselective embolization followed by surgical extirpation has the best chance of disease control. Unfortunately, disease margins are difficult to identify, and recurrence rates are high (93%).
Summary:
Extracranial AVMs are a dangerous group of vascular birthmarks. Lack of research into AVMs has curtailed development of novel treatment options. Surgical excision and intravascular embolization are effective tools offering control but not necessarily a cure.
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