Cerebral arterial angioplasty in a patient with Loeys-Dietz syndrome
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Published on: May 8, 2011
Christopher P Kellner1, Eric S Sussman, Christopher Donaldson
1Department of Neurosurgery, Columbia University, New York, New York, USA.
Insights
Loeys-Dietz syndrome (LDS) patients experiencing subarachnoid hemorrhage face risks of delayed aneurysm formation and severe vasospasm. Refractory vasospasm in this pediatric case presented significant neurointerventional challenges.
Area of Science:
- Neuroscience
- Cardiovascular Science
- Genetics
Background:
- Loeys-Dietz syndrome (LDS) is a rare genetic disorder affecting connective tissue, predisposing individuals to aortic and arterial aneurysms.
- Subarachnoid hemorrhage (SAH) is a serious complication, often associated with aneurysms, leading to significant neurological morbidity.
Observation:
- A 14-year-old boy with LDS presented with SAH, initially without an identifiable aneurysm.
- Delayed presentation of a basilar tip aneurysm and subsequent severe, refractory vasospasm complicated the clinical course.
Findings:
- Endovascular coil embolization of the newly identified basilar tip aneurysm provided temporary improvement.
- Medical and intra-arterial vasodilator treatments failed to resolve severe proximal vasospasm, posing a critical management dilemma.
Implications:
- This case highlights the potential for delayed aneurysm formation and severe vasospasm in pediatric LDS patients post-SAH.
- Management decisions for refractory vasospasm in this high-risk population require careful consideration of interventional risks versus medical treatment limitations.
Abstract:
A 14-year-old boy with Loeys-Dietz syndrome (LDS) had an acute neurologic decline 6 days after a subarachnoid hemorrhage. Cerebral angiography at presentation did not show an aneurysmal source of the hemorrhage. However, on post-bleed day 6 the patient experienced an acutely worsening headache and subsequently lost consciousness. Head CT showed new subarachnoid blood and repeat angiography demonstrated a basilar tip aneurysm. Endovascular coil embolization was performed and his neurologic status improved postoperatively until post-bleed day 9 when he became unresponsive. A CT angiogram demonstrated severe proximal vasospasm. After an unsuccessful attempt to treat the vasospasm medically, the patient was transported to the neurointerventional suite for intra-arterial vasodilator treatment, which also failed to ameliorate the vasospasm. The endovascular surgeons were then faced with the conundrum of attempting a high-risk cerebral angioplasty in a pediatric patient with LDS or returning to maximal medical treatment for severe refractory vasospasm.
