Cervical spine arteriovenous fistula associated with hereditary haemorrhagic telangiectasia

McGurgan, Iain; Lonergan, Roisin; Killeen, Ronan P.; McGuigan, Christopher

doi:10.1136/bcr-2016-218172

Cited by 4 publications

(3 citation statements)

References 19 publications

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“…The underlying mechanism is venous hypertension with broad clinical presentations including lower-extremity weakness and sensory disturbance that may manifest in gait abnormalities, sensory loss, ill-defined lower-back pain, loss of bowel and/or bladder control, as well as sexual dysfunction [4] . The rarer incidence of cervical dAVF vs the thoracolumbar variants has been ascribed to anatomical differences in venous drainage patterns, with small caliber radiculospinal veins [8] . When they do occur in cervical region the dAVF, they have the same demographics in general with presenting symptoms including myelopathy, radiculopathy, and cranial nerve dysfunction [9] .…”

Section: Discussionmentioning

confidence: 99%

A rare case of a cervical dural arteriovenous fistula presenting in a younger patient with vertex subarachnoid hemorrhage: Case report and literature review

Hamid

Choi

Raghuram

et al. 2020

Radiology Case Reports

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Spinal dural arteriovenous fistulas (SDAVF) are rare entities, which mainly occur in the thoracic and lumbar regions of older adults with a significant male predominance. The clinical manifestations are nonspecific such as myelopathy and this can make it challenging to diagnose. Rarely these have been described in the cervical region with even rare manifestations including subarachnoid hemorrhage. In the few reports of hemorrhage, it is usually infratentorial. We present a case of 40-year-old female (uncommon gender and younger age) who presented with headaches and was found to have supra tentorial subarachnoid hemorrhage and a suspicious lesion in the spinal canal. This proved to be a type 1 AVM of the spinal canal (per the American British and French classification). This was successfully treated endovascularly. With the discussion of the relevant literature we hope that this case can add to our medical knowledge as another presentation of this uncommon condition and ultimately help in diagnosing this illusive and rare entity.

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Section: Discussionmentioning

confidence: 99%

A rare case of a cervical dural arteriovenous fistula presenting in a younger patient with vertex subarachnoid hemorrhage: Case report and literature review

Hamid

Choi

Raghuram

et al. 2020

Radiology Case Reports

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“…One of the cases we found describes HHT patient presenting typical multiple cutaneous telangiectases, progressive spastic paraparesis, and subsequent development of upper limb weakness; in addition, in the same patient, cervical arteriovenous fistulas were diagnosed, causing progressive myelopathy, subarachnoid hemorrhage, and brainstem dysfunction. [ 7 ] According to the review of literature performed by Román et al, [ 8 ] 61% of HHT patients presenting neurological symptoms had lesions secondary to a pulmonary arteriovenous fistula.…”

Section: Discussionmentioning

confidence: 99%

Thyroplasty in unilateral vocal fold paresis with coexisting hereditary hemorrhagic telenagiectasia

et al. 2018

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Rationale:The coincidence of an idiopathic unilateral vocal fold paresis and hereditary hemorrhagic telenagiectasia (HHT) is extremely rare and has not been described in the available literature yet.Patients concerns:A 55-year-old female was admitted to hospital due to acute onset of hoarseness, voice fatigue, and effort dyspnea. In the past, the patient was diagnosed with HHT and on admission presented characteristic vascular lesions in the oral cavity. She reported also experiencing a moderate epistaxis at least once per month.Diagnoses:The otolaryngological examination (fiberolaryngoscopy, phoniatric examination) revealed unchanged mobility and morphology of the right vocal fold and paresis of the left vocal fold in intermediate position. Computed tomography and magnetic resonance imaging of head, neck, and chest were inconclusive and showed no pathologic findings.Interventions:The unilateral paresis was treated for 12 months as idiopathic, with extensive rehabilitation. However, no improvement was observed. As a patient suffering from HHT is a challenge for anesthesiologists in terms of general anesthesia, the decision to perform type I thyroplasty (medialization) in local anesthesia was made.Outcomes:There were no complications intraoperatively or in postoperative period. The implemented treatment was successful, as the voice quality improved both in perceptual evaluation (GRBAS scale) and acoustic analysis (F0, jitter, shimmer, NHR).Lessons:A routine surgical treatment in patients with HHT is a challenge. However, in this case, it was uneventful and successful, thus it can be recommended in other patients with similar background.

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“…These lesions are commonly observed in patients with HHT and RASA1 mutations. 4 These lesions are characterized by a markedly hypertrophied spinal artery connecting directly to an even more hypertrophied spinal vein. Symptomatically, these lesions can present with congestive myelopathy from venous congestion, compressive myelopathy related to engorgement and/or thrombosis of a venous varix, or sudden-onset myelopathy from rupture of either a feeding arterial aneurysm or venous varix.…”

Section: Cervical Spine Perimedullary Avfsmentioning

confidence: 99%

Clinical and angioarchitectural characteristics of spinal vascular malformations of the cervical spine

Brinjikji

Colombo²,

Lanzino

2020

Journal of Neurosurgery: Spine

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OBJECTIVEVascular malformations of the cervical spine are exceedingly rare. To date there have been no large case series describing the clinical presentation and angioarchitectural characteristics of cervical spine vascular malformations. The authors report their institutional case series on cervical spine vascular malformations diagnosed and treated at their institution.METHODSThe authors retrospectively reviewed all patients with spinal vascular malformations from their institution from January 2001 to December 2018. Patients with vascular malformations of the cervical spine were included. Lesions were characterized by their angioarchitectural characteristics by an interventional neuroradiologist and endovascular neurosurgeon. Data were collected on clinical presentation, imaging findings, treatment outcomes, and long-term follow-up. Descriptive statistics are reported.RESULTSOf a total of 213 patients with spinal vascular malformations, 27 (12.7%) had vascular malformations in the cervical spine. The mean patient age was 46.1 ± 21.9 years and 16 (59.3%) were male. The most common presentations were lower-extremity weakness (13 patients, 48.1%), tetraparesis (8 patients, 29.6%), and lower-extremity sensory dysfunction (7 patients, 25.9%). Nine patients (33.3%) presented with hemorrhage. Fifteen patients (55.6%) had modified Rankin Scale scores of 0–2 at the time of diagnosis. Regarding angioarchitectural characteristics, 8 patients (29.6%) had intramedullary arteriovenous malformations (AVMs), 5 (18.5%) had epidural arteriovenous fistulas (AVFs), 4 (14.8%) had paraspinal fistulas, 4 (14.8%) had mixed epidural/intradural fistulas, 3 (11.1%) had perimedullary AVMs, 2 (7.4%) had dural fistulas, and 1 patient (3.7%) had a perimedullary AVF.CONCLUSIONSThis retrospective study of 27 patients with cervical spine vascular malformations is the largest series to date on these lesions. The authors found substantial angioarchitectural heterogeneity with the most common types being intramedullary AVMs followed by epidural AVFs, paraspinal fistulas, and mixed intradural/extradural fistulas. Angioarchitecture dictated the clinical presentation as intradural shunts were more likely to present with hemorrhage and acute onset myelopathy, while dural and extradural shunts presented as either incidental lesions or gradually progressive congestive myelopathy.

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Cervical spine arteriovenous fistula associated with hereditary haemorrhagic telangiectasia

Cited by 4 publications

References 19 publications

A rare case of a cervical dural arteriovenous fistula presenting in a younger patient with vertex subarachnoid hemorrhage: Case report and literature review

A rare case of a cervical dural arteriovenous fistula presenting in a younger patient with vertex subarachnoid hemorrhage: Case report and literature review

Thyroplasty in unilateral vocal fold paresis with coexisting hereditary hemorrhagic telenagiectasia

Clinical and angioarchitectural characteristics of spinal vascular malformations of the cervical spine

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