Cavernous sinus syndrome from an internal carotid artery aneurysm in an infant with tuberous sclerosis

Shelton, Julie; Ramakrishnaiah, Raghu H.; Glasier, Charles M.; Phillips, Paul H.

doi:10.1016/j.jaapos.2011.03.013

Cited by 17 publications

(18 citation statements)

References 5 publications

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“…In many cases, the aneurysms were incidental findings on neuroimaging or autopsy. Intriguingly, in a recent paper, Shelton et al described the first case of cavernous sinus syndrome from an intracavernous ICA aneurysm in a baby with tuberous sclerosis, another relevant phakomatosis 30. Therefore, a hypothesis we can keep in consideration is that phakomatoses give a predisposition, by a possible developmental defect of the arterial wall, to the emergence of intracranial aneurysms, which according to their location can give rise to distinctive symptomatologies (ie, indolent ophthalmoplegia due to intracavernous ICA).…”

Section: Discussionmentioning

confidence: 99%

Giant thrombosed intracavernous carotid artery aneurysm presenting as Tolosa–Hunt syndrome in a patient harboring a new pathogenic neurofibromatosis type 1 mutation: a case report and review of the literature

Conforti¹,

Cirillo²,

Marrone³

et al. 2014

NDT

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Neurofibromatosis type 1 (NF1) is a relatively common single-gene disorder, and is caused by heterozygous mutations in the NF1 gene that result in a loss of activity or in a nonfunctional neurofibromin protein. Despite the common association of NF1 with neurocutaneous features, its pathology can extend to numerous tissues not derived from the neural crest. Among the rare cerebrovascular abnormalities in NF1, more than 85% of cases are of purely occlusive or stenotic nature, with intracranial aneurysm being uncommon. Predominantly, the aneurysms are located in the internal carotid arteries (ICAs), being very rare bilateral aneurysms. This report describes a very unusual case of fusiform aneurysms of both ICAs in a Caucasian NF1 patient, with a new pathogenic intragenic heterozygous deletion of the NF1 gene, presenting at age 22 years with Tolosa–Hunt syndrome, because of partial thrombosis of the left giant intracavernous aneurysm. Medical treatment with anticoagulant therapy allowed a good outcome for the patient. In conclusion, early identification of cerebral arteriopathy in NF1 and close follow-up of its progression by neuroimaging may lead to early medical or surgical intervention and prevention of significant neurologic complications.

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Section: Discussionmentioning

confidence: 99%

Giant thrombosed intracavernous carotid artery aneurysm presenting as Tolosa–Hunt syndrome in a patient harboring a new pathogenic neurofibromatosis type 1 mutation: a case report and review of the literature

Conforti¹,

Cirillo²,

Marrone³

et al. 2014

NDT

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“…Locations include the thoracic and abdominal aorta,[18] pulmonary arteries,[7] iliofemoral arteries,[6] carotid arteries,[14] and cerebral arteries. [822439513114231161617152221] Vascular histopathology has demonstrated abnormal architecture, including fragmented or deficient elastic fibers, mucopolysaccharide deposition, dense fibrous tissue, and calcifications. [1617]…”

Section: Discussionmentioning

confidence: 99%

“…[822439513114231161617152221] Age at presentation varies widely, from 5 months to 53 years, and neither gender is predominant. Exam findings of TS patients with intracranial aneurysms are many.…”

Section: Discussionmentioning

confidence: 99%

“…As in our patient, cranial neuropathies may be the presenting symptom. Other signs and symptoms of cerebral aneurysms include visual loss secondary to optic nerve compression,[1311] cavernous sinus syndrome from third and sixth cranial nerve compression,[22] and more diffuse deficits such as hemiparesis secondary to a mid-basilar artery aneurysm. [17] Intraventricular hemorrhage from aneurysm rupture has also been reported in the literature.…”

Section: Discussionmentioning

confidence: 99%

“…[10] Cerebral aneurysms are less commonly described and usually manifest with cranial nerve deficits. [10131122] Once diagnosed, aneurysm occlusion with surgical clip ligation or endovascular embolization is the treatment goal.…”

Section: Introductionmentioning

confidence: 99%

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Coil embolization of an intracranial aneurysm in an infant with tuberous sclerosis complex: A case report and literature review

Galgano²,

Tovar-Spinoza³

et al. 2012

Surg Neurol Int

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Background:Tuberous sclerosis (TS) is a multiorgan neurocutaneous syndrome. Vascular manifestations are often extracranial. There is a paucity of cases involving TS combined with intracranial aneurysms reported in the literature. As a result, treatment has not been well described.Case Description:We report the case of a 13-month-old female infant with a prior diagnosis of TS that was found to have new onset of left eye ptosis, anisocoria, and papillary mydriasis indicative of left third cranial nerve palsy. A magnetic resonance angiogram (MRA) of the brain revealed a left internal carotid artery (ICA) aneurysm. Endovascular embolization was determined to be the best option for treatment. After a successful balloon test occlusion with neuromonitoring, the left internal carotid artery was sacrificed via coil embolization.Conclusions:This is only the third case report of endovascular coil embolization of an intracranial aneurysm in an infant with TS. We report no complications during the procedure, and the patient was discharged with resolving left third cranial nerve palsy. Neither surgical nor endovascular outcomes have been well described in the literature. Follow-up on this patient will be useful for establishing protocols of treatment.

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Neuro-Ophthalmologic Manifestations of Systemic and Intracranial Disease

Brodsky¹

2016

Pediatric Neuro-Ophthalmology

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Cavernous sinus syndrome from an internal carotid artery aneurysm in an infant with tuberous sclerosis

Cited by 17 publications

References 5 publications

Giant thrombosed intracavernous carotid artery aneurysm presenting as Tolosa–Hunt syndrome in a patient harboring a new pathogenic neurofibromatosis type 1 mutation: a case report and review of the literature

Giant thrombosed intracavernous carotid artery aneurysm presenting as Tolosa–Hunt syndrome in a patient harboring a new pathogenic neurofibromatosis type 1 mutation: a case report and review of the literature

Coil embolization of an intracranial aneurysm in an infant with tuberous sclerosis complex: A case report and literature review

Neuro-Ophthalmologic Manifestations of Systemic and Intracranial Disease

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Cavernous sinus syndrome from an internal carotid artery aneurysm in an infant with tuberous sclerosis

Cited by 17 publications

References 5 publications

Giant thrombosed intracavernous carotid artery aneurysm presenting as Tolosa&ndash;Hunt syndrome in a patient harboring a new pathogenic neurofibromatosis type 1 mutation: a case report and review of the literature

Giant thrombosed intracavernous carotid artery aneurysm presenting as Tolosa&ndash;Hunt syndrome in a patient harboring a new pathogenic neurofibromatosis type 1 mutation: a case report and review of the literature

Coil embolization of an intracranial aneurysm in an infant with tuberous sclerosis complex: A case report and literature review

Neuro-Ophthalmologic Manifestations of Systemic and Intracranial Disease

Contact Info

Product

Resources

About

Giant thrombosed intracavernous carotid artery aneurysm presenting as Tolosa–Hunt syndrome in a patient harboring a new pathogenic neurofibromatosis type 1 mutation: a case report and review of the literature

Giant thrombosed intracavernous carotid artery aneurysm presenting as Tolosa–Hunt syndrome in a patient harboring a new pathogenic neurofibromatosis type 1 mutation: a case report and review of the literature