Narindra Ramnarine, Patrick Knight, Devindra Ramnarine
Department of Neurological Surgery, Eric Williams Medical Sciences Complex, Trinidad and Tobago
Corresponding Author
Dr. Devindra Ramnarine
Lecturer in Neurosurgery
Department of Clinical Surgical Sciences
Faculty of Medical Sciences
UWI, St. Augustine
Email: [email protected]
Copyright: This is an open-access article under the terms of the Creative Commons Attribution License which permits use, distribution, and reproduction in any medium, provided the original work is properly cited.
©2021 The Authors. Caribbean Medical Journal published by Trinidad & Tobago Medical Association
Background and Objectives:
SAH in the paediatric population is an uncommon entity accounting for 1-2% of SAH in all age groups & only 18% of ICH in the paediatric population. Trauma is the most common cause of isolated SAH in the paeditric population, with ruptured intracerebral aneurysms although still rare, being the most common cause of spontaneous SAH in children. AVMs are uncommon source of spontaneous SAH. Although they are 10 times more common than cerebral aneurysms in the paediatric population, they rarely present with isolated SAH. This report aims to present such a rare case presented to a tertiary care hospital in Trinidad.
Case Presentation:
We present a 3-year-old girl admitted with sudden onset of headaches, weakness of the right leg, photophobia and neck stiffness. Examination revealed MRC grade 2/5 power in the right leg. Non contrast CT scan revealed blood in the subarachnoid space and occipital horns of the lateral ventricles. Cerebral CT angiogram demonstrated neither intracerebral aneurysms nor AVM, but CT angiogram of the spine revealed a left sided DAVF with the feeding vessel entering the spinal canal at the left C6/7 foramen. A C6 and C7 laminoplasty was performed with ligation of the feeding artery. Postoperative obliteration of the DAVF was confirmed radiologically and clinical resolution of leg weakness was noted. The patient developed non communicating hydrocephalus, necessitating the need for a VP shunt.
Conclusion:
Spontaneous subarachnoid haemorrhage in the paediatric population is an uncommon entity with several possible causes. Clinicians must have a high index of suspicion of an underlying lesion.
Introduction
Subarachnoid haemorrhage refers to the extravasation of blood into the subarachnoid space between the pial and arachnoid membranes. It can occur in various clinical grades ranging from low grade with no neurological symptoms to high grade in which the clinical course can be devastating.
In approximately 15% of patients with spontaneous intracranial subarachnoid haemorrhage (SAH), the cause of the haemorrhage is non aneurysmal in origin. Two-thirds of these patients, thus comprising 10% of all patients with intracranial SAH, have a non-aneurysmal perimesencephalic haemorrhage. The remaining 5% of intracranial SAH are caused by a variety of rare conditions which include cerebral arteriovenous malformation and dural arteriovenous fistula [1, 2]. Spinal arteriovenous fistulae, in particular if localized in the cervical region, have been reported to present with symptoms and signs suggesting an intracranial cause of the spontaneous SAH [1, 2].
Subarachnoid haemorrhage in the paediatric population is uncommon, accounting for 1% to 2% of SAH in patients of all age groups and only 18% of intracranial haemorrhage in the paediatric population [3, 4, 5]. In the older paediatric population, the symptoms at presentation after a spontaneous haemorrhage are similar to those in adults, including the sudden onset of severe headache (61%), nausea and vomiting (45%), decreased level of consciousness (42%), seizures (26%), and focal neurological deficits (13%) [4]. Younger patients tend to present with increasing irritability and lethargy [6]. Meyer-Heim et al. in a review of paediatric SAH in 2003, noted that the onset of symptoms was acute in 53% of patients and subacute in 47% [4].
Spinal DAVF presents with gradually worsening sensory disturbances, diffuse back and muscle pain, weakness and sphincter disturbances. Acute onset of symptoms is mostly attributed to spinal haemorrhage either into the subarachnoid space or intramedullary and rarely to venous thrombosis [7, 8]. Spinal DAVF commonly presents with signs and symptoms of progressive myelopathy. Spinal AVM may also present with pain, acute myelopathy, or radiculopathy [9]. Spinal SAH is reported in approximately half of symptomatic spinal cord AVM [10,11] and is frequently accompanied by intracranial signs and symptoms [12]
Case Report
A 3-year-old presented with sudden onset of headaches, weakness of the right leg, photophobia and neck stiffness. on examination MRC grade 2/5 power was noted in the right leg.
Non contrast CT scan revealed blood in the subarachnoid spaces and occipital horns of the lateral ventricles. Cerebral CT angiogram demonstrated no intracerebral aneurysms or AVM. However, blood was noted at the foramen magnum which raised suspicion of a spinal origin of the SAH. (Figure 1).
CT angiogram of the spine revealed a left sided Type IV dural AVF with a feeding vessel entering the spinal cord at the left C6C7 foramen (Figure 2).
A C6-C7 laminectomy was performed followed by a longitudinal durotomy to expose the dural fistula. A temporary clip was applied to the suspected feeding artery and subsequent collapse of the malformation was noted. The artery was then ligated using ligaclips and the temporary clip was removed. Watertight closure of the dura was obtained, and the C6C7 lamina replaced using a microplating system.
Postoperative resolution of leg weakness was noted. However, the patient developed non communicating hydrocephalus as a result of the intracerebral blood, necessitating the need for a VP shunt.
Discussion
Spinal vascular lesions are partially understood and is often described by overlapping terms in the different classification schemes. incidence is about 4% of primary intraspinal masses, with 80% occurring between ages 20-60 years. Many classification schemes have appeared in the literature since Bergstrand’s first attempt in 1964.
Spinal DAVF have been reported as a rare cause of intracranial SAH. However, mainly patients with cervical DAVF have been described as being indistinguishable from patients with an intracranial source of bleeding [1, 2, 13]
The pathological mechanism of intracranial SAH from a Spinal DAVF remains unclarified. The most straightforward mechanism is migration or extension of subarachnoid blood from the spinal to the intracranial level [14, 15]. Haemorrhage may be caused by venous hypertension when arterialized blood flows via the medullary vein to the valveless coronal venous plexus and radial vein [16, 17]. Another hypothesis suggests that the vein around the midbrain is compressed or stretched by the tentorial incisura when, eg., physical exercise elevates the ICP, which then leads to aggravation of venous hypertension with subsequent rupture of the vein [18]. In patients with intracranial drainage of their Spinal DAVF, the relatively fast venous flow may cause formation of a varix on the draining vessel, which may result in intracranial SAH after rupture [19]. Ascending venous drainage was associated with an increased risk of SAH in patients with CCJ perimedullary and dural AVF [20]. In this light, it is not surprising that in patients with Spinal DAVF, cranial symptoms and signs other than those suggestive of intracranial SAH caused by rupture of a saccular aneurysm have been reported, such as intermittent double vision, slurred speech, and nystagmus [21]
Conclusion
Spontaneous subarachnoid haemorrhage in the paediatric population is an uncommon entity with several possible causes, with a Spinal DAVF being even more uncommon. Clinicians must have a high index of suspicion of an underlying lesion and in today’s practice, the use of available investigations is of utmost importance.
References
1. Rinkel GJ, van Gijn J, Wijdicks EF. Subarachnoid hemorrhage without detectable aneurysm. A review of the causes. Stroke 1993; 24:1403–9
2. van Gijn J, Rinkel GJ. Subarachnoid haemorrhage: diagnosis, causes and management. Brain 2001; 24: 249–78
3. Kneyber MCJ, Rinkel GJE, Ramos MP, Tulleken CA, Braun KP. Early posttraumatic subarachnoid hemorrhage due to dissecting aneurysms in three children. Neurology 2005; 65: 1663–1665
4. Meyer-Heim A, Boltshauser E. Spontaneous intracranial hemorrhage in children: aetiology, presentation and outcome. Brain Dev 2003; 25: 416–421
5. Williams FC, Zabramski JM, Spetzler RF, Rekate HL. Anterolateral transthoracic transvertebral resection of an intramedullary spinal arteriovenous malformation. Case report. J of Neurosurgery 1991; 74:1004–1008
6. Duhaime AC, Christian, CW, Rorke LB, Zimmerman RA. Nonaccidental head injury in infants – the ‘‘shaken-baby syndrome.’’ N Engl J Med 1998; 338(25): 1822–1829
7. Ferch RD, Morgan MK, Sears WR. Spinal arteriovenous malformations: a review with case illustrations. J Clin Neurosci 2001; 8: 299–304
8. Krings T, Mull M, Gilsbach JM, Thron A. Spinal vascular malformations. Eur Radiol 2005; 15:267–278
9. Spetzler RF, Detwiler PW, Riina HA, Porter RW. Modified classification of spinal cord vascular lesions. J Neurosurg Spine 2002; 96:145–156
10. Niimi Y, Berenstein A, Setton A, Pryor J. Symptoms, vascular anatomy and endovascular treatment of spinal cord arteriovenous malformations. Intervent Neuroradiol 2000; 6:199–202
11. Rosenblum B, Oldfield EH, Doppman JL, Di Chiro G. Spinal arteriovenous malformations: a comparison of dural arteriovenous fistulas and intradural AVM’s in 81 patients. J Neurosurg 1987; 67:795–802 1049
12. Caroscio JT, Brannan T, Budabin M, Huang YP, Yahr MD. Subarachnoid hemorrhage secondary to spinal arteriovenous malformation and aneurysm. Report of a case and review of the literature. Arch Neurol 1980; 37:101–103
13. Warlow CP, Dennis MS, van Gijn J, Hankey GJ, Sandercock PAG, Bamford JM, Wardlaw JM. What caused this subarachnoid haemorrhage? Stroke: a practical guide to management. Blackwell Science Ltd, Oxford, England 2001.
14. Clark RS, Orr RA, Atkinson CS, Towbin RB, Pang D. Retinal hemorrhages associated with spinal cord arteriovenous malformation. Clin Pediatr (Phila) 1995; 34:281–283
15. Maggioni F, Rossi P, Casson S, Fiore D, Zanchin G. Initially migraine like manifestation of a ruptured spinal arteriovenous malformation. Cephalalgia 1995; 15:237–40
16. Do HM, Jensen ME, Cloft HJ, Kallmes DF, Dion JE. Dural arteriovenous fistula of the cervical spine presenting with subarachnoid hemorrhage. Am J Neuroradiol 1999; 20:348–350
17. Morimoto T, Yoshida S, Basugi N. Dural arteriovenous malformation in the cervical spine presenting with subarachnoid hemorrhage: case report. Neurosurgery 1992; 31:118–20
18. Hashimoto H, Iida J, Shin Y, Hironaka Y, Sakaki T. Spinal dural arteriovenous fistula with perimesencephalic subarachnoid haemorrhage. J Clin Neurosci 2000; 7:64–66
19. Kinouchi H, Mizoi K, Takahashi A, Nagamine Y, Koshu K, Yoshimoto T. Dural arteriovenous shunts at the craniocervical junction. J Neurosurg 1998; 89:755–761
20. Kai Y, Hamada J, Morioka M, Yano S, Mizuno T, Kuratsu J. Arteriovenous fistulas at the cervicomedullary junction presenting with subarachnoid hemorrhage: six case reports with special reference to the angiographic pattern of venous drainage. Am J Neuroradiol 2005; 26:1949–1954
21. Caroscio JT, Brannan T, Budabin M, Huang YP, Yahr MD. Subarachnoid hemorrhage secondary to spinal arteriovenous malformation and aneurysm. Report of a case and review of the literature. Arch Neurol 1980; 37:101–103