Screening for arteriovenous malformations in hereditary haemorrhagic telangiectasia

AbstractObjective:To determine whether patients with hereditary haemorrhagic telangiectasia were being screened according to international guidelines, and to review recent evidence in order to provide up-to-date guidelines for the initial systemic management of hereditary haemorrhagic telangiectasia.Methods:A retrospective case note analysis was conducted, assessing patients in terms of screening for: genetics, cerebral arteriovenous malformations, pulmonary and hepatic arteriovenous malformations, and gastrointestinal telangiectasia. Databases searched included Medline, the Cumulative Index to Nursing and Allied Health Literature, and Embase.Results:Screening investigations were most frequently performed for hepatic arteriovenous malformations and least frequently for genetics. Recent data suggest avoiding routine genetic and cerebral arteriovenous malformation screening because of treatment morbidities; performing high-resolution chest computed tomography for pulmonary arteriovenous malformation screening; using capsule endoscopy (if possible) to reduce complications from upper gastrointestinal endoscopy; and omitting routine liver enzyme testing in favour of Doppler ultrasound.Conclusion:Opportunities for systemic arteriovenous malformation screening are frequently overlooked. This review highlights the need for screening and considers the form in which it should be undertaken.

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Retrograde flow in the distal aortic arch and proximal descending aorta—a clue to presence of a major cerebral arteriovenous malformation

Cardiology in the Young ◽

10.1017/s104795110000144x ◽

1993 ◽

Vol 3 (2) ◽

pp. 161-163 ◽

Cited By ~ 4

Author(s):

Andrew M. Davis ◽

Samuel Menahem

Keyword(s):

Early Diagnosis ◽

Arteriovenous Malformation ◽

Cardiac Failure ◽

Arteriovenous Malformations ◽

Cerebral Arteriovenous Malformation ◽

Cranial Ultrasound ◽

Retrograde Flow ◽

Cerebral Arteriovenous Malformations ◽

Descending Aorta ◽

Satisfactory Outcome

SummaryTwo neonates are described presenting in severe cardiac failure within a few hours of birth. Echocardiography and the demonstration by color-coded Doppler of retrograde diastolic flow in the proximal descending aorta suggested the presence of large cerebral arteriovenous malformations. Such lesions were demonstrated by cranial ultrasound. The early diagnosis permitted early intervention, albeit with less than satisfactory outcome.

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Anaesthesia for Closed Embolisation of Cerebral Arteriovenous Malformations

Anaesthesia and Intensive Care ◽

10.1177/0310057x8801600313 ◽

1988 ◽

Vol 16 (3) ◽

pp. 318-323 ◽

Cited By ~ 12

Author(s):

B. J. O'Mahony ◽

S. N. C. Bolsin

Keyword(s):

Arteriovenous Malformation ◽

Glyceryl Trinitrate ◽

Sodium Nitroprusside ◽

Arteriovenous Malformations ◽

Anaesthetic Management ◽

Cerebral Arteriovenous Malformation ◽

Vascular Network ◽

Arterial Hypotension ◽

Cerebral Arteriovenous Malformations ◽

Neurological Assessment

Cerebral arteriovenous malformation embolisation is a therapeutic, neuroradiological procedure involving injection of bucrylate glue into the nidus of the A V malformation to obliterate the abnormal vascular network. These procedures may involve significant risks, are often long and thereby necessitate the need for some form of sedation and for adequate monitoring of the cerebral, cardiovascular and respiratory systems. The anaesthetic management of a series of twenty patients undergoing embolisation of a cerebral arteriovenous malformation is outlined, seven general and nineteen neurolept anaesthetics being administered. Neurolept anaesthesia is the preferred technique as neurological assessment during the procedure is possible and complications may be diagnosed immediately. Systemic arterial hypotension may facilitate the embolisation process and various agents, including glyceryl trinitrate and sodium nitroprusside, have been employed for this purpose.

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Seizures after Onyx Embolization for the Treatment of Cerebral Arteriovenous Malformation

Interventional Neuroradiology ◽

10.1177/159101991101700308 ◽

2011 ◽

Vol 17 (3) ◽

pp. 331-338 ◽

Cited By ~ 10

Author(s):

K. De Los Reyes ◽

A. Patel ◽

A. Doshi ◽

N. Egorova ◽

F. Panov ◽

...

Keyword(s):

Arteriovenous Malformation ◽

Retrospective Review ◽

Arteriovenous Malformations ◽

Clinical History ◽

Median Number ◽

Cerebral Arteriovenous Malformation ◽

Brain Arteriovenous Malformation ◽

Cerebral Arteriovenous Malformations ◽

History Of ◽

New Onset

Onyx embolization of cerebral arteriovenous malformations (AVM) has become increasingly common. We explored the risk of seizures after Onyx use. A retrospective review was conducted of 20 patients with supratentorial brain arteriovenous malformation (AVM) who received Onyx embolization between 2006 and 2009. Baseline demographics, clinical history, seizure history, AVM characteristics and treatment were compared between those who developed post-onyx seizure and those who did not. MRIs were reviewed for edema following Onyx treatment. Of 20 patients who underwent Onyx embolization, the initial AVM presentation was hemorrhage in 40% (N=8). The median number of embolizations was two (range 1–4) and the median final obliteration amount was 90% (range 50–100%). A history of seizure was present in 50% (N=10) of patients pre-embolization and 12 (60%) patients received seizure medications (treatment or prophylaxis) prior to embolization. Seizur post-Onyx embolization occurred in 45% (N=9). The median time to seizur post-Onyx was seven days (range 0.3–210). Four patients (20%) with seizures post-Onyx had no seizure history. Two of these patients (10%) had no other identifiable cause for seizure other than recent Onyx embolization. Seizures in these two patients occurred within 24 hours of Onyx administration. Among patients with post-Onyx seizures, there was a trend toward larger AVM size (P=0.091) and lower percent obliteration (P=0.062). Peri-AVM edema was present in 75% of MRIs performed within one month of Onyx treatment and may represent a possible etiology for seizures. New onset seizures post-Onyx embolization are not uncommon. Further study of seizure prevention is warranted.

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Spontaneous subarachnoid hemorrhage first from an intracranial and then from a spinal arteriovenous malformation

Journal of Neurosurgery ◽

10.3171/jns.1977.47.6.0965 ◽

1977 ◽

Vol 47 (6) ◽

pp. 965-968 ◽

Cited By ~ 16

Author(s):

Dwight Parkinson ◽

Michael West

Keyword(s):

Subarachnoid Hemorrhage ◽

Arteriovenous Malformation ◽

Arteriovenous Malformations ◽

Cerebral Arteriovenous Malformation ◽

Cerebral Arteriovenous Malformations ◽

Content Type ◽

Spinal Arteriovenous Malformation ◽

Spontaneous Subarachnoid Hemorrhage ◽

Spinal Avm ◽

Fine Print

✓ A patient presented with spontaneous subarachnoid hemorrhage (SAH) from a cerebral arteriovenous malformation (AVM) which was later totally removed at surgery. The patient presented again with a new SAH from a spinal AVM that was also totally removed at surgery. Coexistence of spinal and cerebral arteriovenous malformations are exceedingly rare and hemorrhage from each is not previously reported. This case emphasizes the importance of investigating the spinal canal in otherwise unexplained spontaneous SAH.

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