Radiation-Induced Moyamoya Syndrome in Children with Brain Tumors: Case Series and Literature Review

Scala, Marcello; Fiaschi, Pietro; Cama, Armando; Consales, Alessandro; Piatelli, Gianluca; Giannelli, Flavio; Barra, Salvina; Satragno, Camilla; Pacetti, Mattia; Secci, Francesca; Tortora, Domenico; Garrè, Maria Luisa; Pavanello, Marco

doi:10.1016/j.wneu.2019.11.155

Cited by 28 publications

(27 citation statements)

References 56 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Moyamoya is a chronic cerebrovascular disorder consisting in the bilateral stenosis or occlusion of the terminal portion of the internal carotid arteries and the proximal tracts of the anterior and middle cerebral arteries [1][2][3]. This condition can be classified into pure moyamoya disease (MMD), when no associated risk factor is identifiable, and moyamoya syndrome (MMS) or quasi-MMD, when the vasculopathy develops in association with clear risk factors [2,4,5]. Surgical treatment remains the cornerstone in patient management in order to reduce the risk ischemic or hemorrhagic sequelae [4,6].…”

Section: Introductionmentioning

confidence: 99%

Limits and pitfalls of indirect revascularization in moyamoya disease and syndrome

et al. 2020

Self Cite

View full text Add to dashboard Cite

Moyamoya vasculopathy is a rare chronic cerebrovascular disorder characterized by the stenosis of the terminal branches of the internal carotid arteries and the proximal tracts of anterior and middle cerebral arteries. Although surgical revascularization does not significantly change the underlying pathogenic mechanisms, it plays a pivotal role in the management of affected individuals, allowing to decrease the risk of ischemic and hemorrhagic complications. Surgical approaches may be direct (extracranial-intracranial bypass), indirect, or a combination of the two. Several indirect techniques classifiable according to the tissue (muscle, periosteum, galea, dura mater, and extracranial tissues) or vessel (artery) used as a source of blood supply are currently available. In this study, we reviewed the pertinent literature and analyzed the advantages, disadvantages, and pitfalls of the most relevant indirect revascularization techniques. We discussed the technical aspects and the therapeutical implications of each procedure, providing a current state-of-the-art overview on the limits and pitfalls of indirect revascularization in the treatment of moyamoya vasculopathy.

show abstract

Section: Introductionmentioning

confidence: 99%

Limits and pitfalls of indirect revascularization in moyamoya disease and syndrome

et al. 2020

Self Cite

View full text Add to dashboard Cite

show abstract

“…Pathogenesis of RIMS is debated in the literature: several authors support the theory of a chronic arteritis obliterans caused by the formation of reactive oxygen species that provoke reactive proliferation of the endothelial lining and subendothelial connective tissue with progressive stenosis of vascular lumen. [3,11,13] Radiation-induced biochemical alterations and stressogenic stimuli can drive morphological and functional alterations in endothelial cells, remodeling of tight junctions and neurovascular units interactions, increases in permeability of endothelial lining of microvasculature, cell death, and detachment from the basement membrane. [5] e major aspect of RIMS is the total/partial absence of collateral circulation as compared with traditional Moyamoya disease.…”

Section: Discussionmentioning

confidence: 99%

“…RT is an effective therapeutic modality for the treatment of many pediatric brain tumors, playing a role in local disease control and improving patients’ overall survival, especially those patients who do not respond to CT.[ 11 ] Nevertheless, cranial irradiation is the major risk factor for late cerebrovascular complications (LCC) in pediatric population due to iatrogenic damages of cerebrovascular tissue surrounding the radiation target. [ 1 ] These are defined as lesions secondary to small, medium, or large vessel abnormality occurring after RT within the irradiated volume.…”

Section: Discussionmentioning

confidence: 99%

Acute ischemic stroke secondary to ventriculoperitoneal shunt dysfunction in a child with Moyamoya syndrome

Vitulli

Spennato

Cicala

et al. 2022

Surgical Neurology International

View full text Add to dashboard Cite

Background: Patients with brain vascular disease and hydrocephalus may be predisposed to acute ischemic stroke in case of shunt dysfunction and subsequent increased intracranial pression. Patients with brain tumor may develop hydrocephalus as a consequence of obstruction of cerebrospinal fluid pathways and radiation-induced moyamoya syndrome secondary (RIMS) to radiotherapy (RT). Case Description: A 15-year-old male patient, affected by hydrocephalus and RIMS, presented acute cerebral ischemia after an episode of shunt malfunction. The shunt was promptly revised and the areas of ischemia visible at magnetic resonance imaging significantly decreased. Conclusion: Children who receive RT for brain tumor, particularly if the circle of Willis region is involved, require close surveillance for the development of vasculopathy and consequent stroke. This surveillance must be even tighter if the patient has been treated with ventricular shunt for the possible synergistic interaction between the two causes on reducing cerebral perfusion and increasing the risk of acute ischemic events.

show abstract

“…Of note, moyamoya syndrome is different from primary or idiopathic moyamoya disease as it develops secondary to an underlying disorder such as Down syndrome, sickle cell disease, PHACE syndrome, neurofibromatosis or other RASopathies, or may occur after brain radiation therapy [ 5 , 11 ]. Accordingly, the surgical management of Moyamoya syndrome might be complex, since the long-term clinical course may be complicated by the frequently unpredictable natural history of the associated disorder [ 7 , 11 , 12 ].…”

Section: Introductionmentioning

confidence: 99%

A rare triad of morning glory disc anomaly, moyamoya vasculopathy, and transsphenoidal cephalocele: pathophysiological considerations and surgical management

et al. 2021

Self Cite

View full text Add to dashboard Cite

Morning glory disc anomaly is a congenital abnormality of the optic disc and peripapillary retina reported as an isolated condition or associated with various anomalies, including basal encephaloceles and moyamoya vasculopathy. However, the co-occurrence of these three entities is extremely rare and the pathogenesis is still poorly understood. Moreover, data on the surgical management and long-term follow-up of the intracranial anomalies are scarce. Here, we describe the case of a 11-year-old boy with morning glory disc anomaly, transsphenoidal cephalocele, and moyamoya vasculopathy, who underwent bilateral indirect revascularization with encephalo-duro-myo-arterio-pericranio-synangiosis at the age of 2 years, and endoscopic repair of the transsphenoidal cephalocele at the age of 6 years. A rare missense variant (c.1081T>C,p.Tyr361His) was found in OFD1, a gene responsible for a X-linked ciliopathy, the oral-facial-digital syndrome type 1 (OFD1; OMIM 311200). This case expands the complex phenotype of OFD1 syndrome and suggests a possible involvement of OFD1 gene and Shh pathway in the pathogenesis of these anomalies.

show abstract

Radiation-Induced Moyamoya Syndrome in Children with Brain Tumors: Case Series and Literature Review

Cited by 28 publications

References 56 publications

Limits and pitfalls of indirect revascularization in moyamoya disease and syndrome

Limits and pitfalls of indirect revascularization in moyamoya disease and syndrome

Acute ischemic stroke secondary to ventriculoperitoneal shunt dysfunction in a child with Moyamoya syndrome

A rare triad of morning glory disc anomaly, moyamoya vasculopathy, and transsphenoidal cephalocele: pathophysiological considerations and surgical management

Contact Info

Product

Resources

About