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Pediatric cryptic vascular malformations: presentation, diagnosis and treatment
S F Ciricillo1, P H Cogen, M S Edwards
1Division of Pediatric Neurosurgery, University of California, San Francisco.
Insights
Cryptic vascular malformations (CVMs) are increasingly found in children. Surgical excision is recommended for symptomatic CVMs, with radiation therapy playing no role in treatment.
Area of Science:
- Neurology
- Pediatric Neurosurgery
- Vascular Imaging
Background:
- Cryptic vascular malformations (CVMs) are increasingly diagnosed in pediatric patients.
- The natural history and optimal treatment for CVMs remain uncertain.
- Magnetic resonance (MR) imaging has improved CVM detection.
Purpose of the Study:
- To review the presentation, diagnostic methods, treatment, and outcomes of pediatric CVMs.
- To evaluate the effectiveness of surgical intervention for CVMs.
- To clarify the role of radiation therapy in CVM management.
Main Methods:
- Retrospective review of 37 pediatric patients with CVMs treated between 1982 and 1992.
- Analysis of clinical presentation, MR imaging, angiography, surgical procedures, and patient outcomes.
- Assessment of complications and long-term follow-up.
Main Results:
- MR imaging was diagnostic in all cases; angiography was often negative.
- Surgical excision was the primary treatment, with no surgical mortality.
- One-third of patients experienced perioperative complications, mostly resolving quickly.
- Radiation-induced CVMs generally remained asymptomatic with follow-up, except for one hemorrhage case.
- Two patients with complex CVMs showed neurological decline post-surgery.
Conclusions:
- CVMs are more common in children than previously thought.
- Complete surgical excision is the recommended treatment for symptomatic CVMs presenting with seizures or hemorrhage.
- Radiation therapy is not indicated for CVMs and may contribute to their formation.
- Close clinical and radiological follow-up is essential for assessing treatment efficacy and disease progression.
Abstract:
Since the advent of magnetic resonance (MR) imaging, angiographically occult or 'cryptic' vascular malformations (CVMs) have been detected with increasing frequency in the pediatric population. The natural history of these lesions is uncertain and treatment remains controversial. We respectively reviewed the presentation, radiology, treatment and outcome of 37 pediatric patients with CVMs seen at our institution between 1982 and 1992. MR imaging was diagnostic in all patients studied. Angiography was negative in 20 of 21 patients studied, while 1 teenage girl was found to have a venous malformation. Total surgical excision was the treatment goal in all patients presenting with symptomatic CVMs. There has been no surgical mortality. Perioperative complications occurred in one third of the patients but quickly resolved in most cases. Only 2 patients with multiple CVMs, including brainstem lesions, who presented with progressive neurologic deficits were clearly worse following surgery. Six of seven patients with radiation-induced CVMs have remained asymptomatic with close radiographic follow-up, while 1 patient with a hemorrhage has required surgical evacuation. We conclude that CVMs occur more commonly in the pediatric population than previously assumed. Patients presenting with seizures or hemorrhage should undergo craniotomy with the goal of complete excision. Radiation therapy plays no role in the treatment of these lesions and may actually result in their formation. Careful clinical and radiological follow-up is critical to assess the completeness of surgical removal and to evaluate progression of lesions in patients with multiple or radiation-induced CVMs treated conservatively.