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Year : 2015  |  Volume : 10  |  Issue : 4  |  Page : 416-417

A rare cause of occipital headache

1 Department of Pediatrics, All Institute of Medical Sciences, New Delhi, India
2 Department of Radiodiagnosis, All Institute of Medical Sciences, New Delhi, India

Date of Web Publication20-Jan-2016

Correspondence Address:
Sheffali Gulati
Department of Pediatrics, Division of Child Neurology, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.174448

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How to cite this article:
Saini L, Chakrabarty B, Gulati S, Kumar A. A rare cause of occipital headache. J Pediatr Neurosci 2015;10:416-7

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Saini L, Chakrabarty B, Gulati S, Kumar A. A rare cause of occipital headache. J Pediatr Neurosci [serial online] 2015 [cited 2022 Dec 10];10:416-7. Available from: https://www.pediatricneurosciences.com/text.asp?2015/10/4/416/174448

Dear Sir,

A premorbidly normal, 13-year-old boy, presented with complaints of headache for last 1 month. The headache was intermittent, moderate to severe in intensity, located predominantly in the occipital region with occasional radiation to the neck and partial response to analgesics. There was no associated altered sensorium, speech abnormality, cranial nerve deficit, seizures, focal motor or sensory deficits, photophobia, diplopia, or vomiting. Detailed general physical and systemic examinations revealed normal fundus examination and blood pressure with generalized hyperreflexia.

Magnetic resonance imaging (MRI) brain and magnetic resonance angiography were normal. MRI spine revealed cervical cord arteriovenous malformation (AVM) at C3–C4 level with multiple collaterals [Figure 1]a and [Figure 1]b.
Figure 1: T1-weighted (a) and T2-weighted (b) sagittal magnetic resonance images of the cervical spine show multiple abnormal tortuous vascular flow voids (arrows) anterior to the spinal cord from medulla to C6 vertebral level consistent with spinal arteriovenous malformation. Note the indentation over the cord at C3–C4 level

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Spinal vascular malformations account for only 5–10% of all spinal diseases.[1] The exact pediatric prevalence is unknown, but definitely it is less than in adults. They predominantly affect middle-aged men who present with lower extremity weakness and sensory disturbances. Very rarely, it presents as headache or visual disturbances.[1],[2]

The cause of intermittent headache in this patient can be possibly due to two possible mechanisms; first being referred pain from cervical dermatomes to the occipital region and the second is episodic bleeding into the subarachnoid space causing obstruction of cerebrospinal fluid outflow.[2] These lesions can clinically mimic spinal stenosis which also presents with pain and compressive myelopathy symptoms. A relatively rare but significant complication is subarachnoid hemorrhage. The possibility of spinal AVM should be kept in the differential diagnosis of any patient with a subarachnoid hemorrhage who has a normal cerebral angiogram, although this entity is rarely reported in children.

Delay in diagnosis leads to the development of significant spastic paresis in the majority. In the largest series of pediatric spinal AVM comprising 38 cases, more than 90% patients presented with features of myelopathy.[3]

Spinal AVMs are hypothesized as developmental defects occurring between 4 and 8 weeks of embryonic development.[4] AVMs bypass capillary beds and behave as abnormal communications between arteries and veins. There are three biologically distinct types of spinal AVMs: Dural, intradural, and cavernous. Symptoms usually arise due to hemorrhage, compression, venous hypertension, and ischemia; however, AVMs are rarely symptomatic. Intradural AVMs are more likely to present with pain and acute neurological dysfunctions than spinal dural AVMs.[5]

Early diagnosis is of paramount importance in patients with spinal AVM because of the treatable and preventable potentially catastrophic complications. In terms of medical management, steroids can be used to tide over acute crisis situations, however there is no role of prolonged steroid therapy as it does not modify the underlying disease process and is also associated with significant side effects.

Surgical intervention is decided on symptomatology and topography of the lesion. The options include open microsurgical resection. Minimally invasive neurointervention technique is an upcoming modality, but its application is limited in view of lack of expertise.[6]

Thus, in children with focal occipital headache, a detailed neurological evaluation is warranted to look for features of mild quadriparesis, and imaging should be considered to rule out potentially hazardous entity such as spinal AVM, which is a treatable condition.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Inci MF, Senoglu M, Ozkan F, Yuksel M. Spinal dural arteriovenous malformation presented with intracranial hypertension in a young patient. BMJ Case Rep 2012;2012. pii: Bcr2012007906.  Back to cited text no. 1
Bassuk AG, Burrowes DM, Velimirovic B, Grant J, Keating GF. A child with spinal cord AVM presenting with raised intracranial pressure. Neurology 2003;60:1724-5.  Back to cited text no. 2
Riché MC, Modenesi-Freitas J, Djindjian M, Merland JJ. Arteriovenous malformations (AVM) of the spinal cord in children. A review of 38 cases. Neuroradiology 1982;22:171-80.  Back to cited text no. 3
Fisher WS. Concomitant intracranial aneurysms and arteriovenous malformations. In: Wilkins RH, Rengachary SS, editors. Neurosurgery. 2nd ed. New York: McGraw-Hill; 1996. p. 2429-31.  Back to cited text no. 4
Oldfield DH. Spinal vascular malformations. In: Wilkins RH, Rengachary SS, editors. Neurosurgery. 2nd ed. New York: McGraw-Hill; 1996. p. 2541-8.  Back to cited text no. 5
Patsalides A, Knopman J, Santillan A, Tsiouris AJ, Riina H, Gobin YP. Endovascular treatment of spinal arteriovenous lesions: Beyond the dural fistula. AJNR Am J Neuroradiol 2011;32:798-808.  Back to cited text no. 6


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