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Updated: May 4, 2026
Isolation and Cannulation of Cerebral Parenchymal Arterioles
Published on: May 23, 2016
Cerebral arterial angioplasty in a patient with Loeys-Dietz syndrome
Christopher P Kellner1, Eric S Sussman1, Christopher Donaldson1
1Department of Neurosurgery, Columbia University, New York, New York, USA.
Insights
This case study highlights a pediatric Loeys-Dietz syndrome patient experiencing delayed aneurysm rupture and severe vasospasm after subarachnoid hemorrhage. Refractory vasospasm presented a critical treatment challenge, underscoring the complexity of managing vascular complications in LDS.
Area of Science:
- Neurology
- Cardiovascular Research
- Genetics
Background:
- Loeys-Dietz syndrome (LDS) is a rare genetic connective tissue disorder associated with a high risk of aortic and arterial aneurysms and dissections.
- Subarachnoid hemorrhage (SAH) is a life-threatening condition often caused by ruptured intracranial aneurysms, requiring prompt diagnosis and management.
Observation:
- A 14-year-old boy with LDS presented with SAH, initially without an identifiable aneurysm source.
- Delayed neurological decline occurred on post-bleed day 6, with CT showing new SAH and angiography revealing a basilar tip aneurysm.
- Severe proximal vasospasm developed on post-bleed day 9, refractory to medical and intra-arterial vasodilator treatments.
Findings:
- Endovascular coil embolization was performed for the basilar tip aneurysm.
- Despite initial improvement, the patient developed severe, refractory vasospasm unresponsive to multiple treatment modalities.
- The case presented a critical decision point regarding high-risk cerebral angioplasty versus continued maximal medical management for vasospasm in a pediatric LDS patient.
Implications:
- This case underscores the potential for delayed aneurysm formation or rupture in LDS patients following initial SAH.
- Refractory vasospasm poses a significant management challenge in the context of LDS, necessitating consideration of aggressive interventional strategies.
- Further research into the vascular manifestations and optimal treatment paradigms for pediatric LDS patients experiencing cerebrovascular events is warranted.
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.
