Subarachnoid haemorrhage in paediatric hereditary haemorrhagic telangiectasia: a case report and literature review

PURPOSE: Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterised by telangiectasias and multi-organ arteriovenous malformations (AVMs). Subarachnoid haemorrhage (SAH) is rare in children with HHT, and intracranial aneurysms are scarcely reported. We describe the case of a 12-year-old boy with confirmed HHT who presented with a generalised tonic-clonic seizure, followed by headache and neck pain. MATERIALS AND METHODS: A narrative literature review was conducted to identify reported paediatric cases of SAH in patients with HHT. MEDLINE, Embase, the Cochrane Library, and CINAHL were searched. Five reports describing six paediatric patients were reviewed and summarised. RESULTS: Imaging revealed SAH with intraventricular extension. Initial imaging failed to detect any neurovascular malformation, but a small carotid cave aneurysm was identified as the likely haemorrhage source with subsequent digital subtraction angiography. Following treatment with endovascular flow-diverting stent placement, the patient remained neurologically stable. An unrecognised spinal AVM was discovered by whole-spine magnetic resonance imaging. CONCLUSIONS: This case highlights a rare mechanism of haemorrhage in paediatric HHT, the challenges of detecting small aneurysms with non-invasive imaging, and the importance of a multidisciplinary approach in guiding management. Improved awareness and robust literature on atypical cerebrovascular manifestations may inform future diagnostic and management strategies.

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Publication Details

Journal
British Journal of Neurosurgery
Published
2026-10-05
DOI
https://doi.org/10.1080/02688697.2026.2733174
Primary Topic
Vascular Anomalies and Treatments
Type
article
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article

Subarachnoid haemorrhage in paediatric hereditary haemorrhagic telangiectasia: a case report and literature review

Christian Aloysius Than, Mathew R. Guilfoyle, Ibrahim Jalloh, Mohamed Abdelrahman Elborady et al.
British Journal of Neurosurgery
Vascular Anomalies and Treatments
article

Subarachnoid haemorrhage in paediatric hereditary haemorrhagic telangiectasia: a case report and literature review

Christian Aloysius Than, Mathew R. Guilfoyle, Ibrahim Jalloh, Mohamed Abdelrahman Elborady, Alberto Faccenda, Dilina Rajapakse, Alma Sato, Sarah Elgazar, Ebaney Ghotra
article en

Abstract

PURPOSE: Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterised by telangiectasias and multi-organ arteriovenous malformations (AVMs). Subarachnoid haemorrhage (SAH) is rare in children with HHT, and intracranial aneurysms are scarcely reported. We describe the case of a 12-year-old boy with confirmed HHT who presented with a generalised tonic-clonic seizure, followed by headache and neck pain. MATERIALS AND METHODS: A narrative literature review was conducted to identify reported paediatric cases of SAH in patients with HHT. MEDLINE, Embase, the Cochrane Library, and CINAHL were searched. Five reports describing six paediatric patients were reviewed and summarised. RESULTS: Imaging revealed SAH with intraventricular extension. Initial imaging failed to detect any neurovascular malformation, but a small carotid cave aneurysm was identified as the likely haemorrhage source with subsequent digital subtraction angiography. Following treatment with endovascular flow-diverting stent placement, the patient remained neurologically stable. An unrecognised spinal AVM was discovered by whole-spine magnetic resonance imaging. CONCLUSIONS: This case highlights a rare mechanism of haemorrhage in paediatric HHT, the challenges of detecting small aneurysms with non-invasive imaging, and the importance of a multidisciplinary approach in guiding management. Improved awareness and robust literature on atypical cerebrovascular manifestations may inform future diagnostic and management strategies.

British Journal of Neurosurgery
Cairo University (EG), The University of Queensland (AU), University of Nicosia (CY), Cambridge University Hospitals NHS Foundation Trust (GB)
Openalex Percentile: Top 12%
Vascular Anomalies and Treatments
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