An unusual cause of Hematomyelia




Ø 36 Years old male
Ø 2 Nov 2008 : sudden onset of weakness, decreased sensation below neck, urinary and fecal incontinence
ØExam: E4V5M6
ØSensory Loss below D6 level
ØPower: UL 3-4/5, LL 1/5

MRI:13 Nov 2008- showed extensive cord changes and a tortuous flow void in the anterior thecl sac with a globular void at top of the serpentine vessel.


MRI:20 Dec 2008: clearly shows presence of severe hematomyelia. A diagnosis of spinal arterio-venous malformation was kept.

DSA: 2nd March 2009:

The aarch aortogram revealed a bunch of tortuous vessels aat root of neck bilaterally as well as amidline tortuous vessel (the anterior spinal artery) with an aneurysm. But what was surprising was that some of these vessels opened in the aorta lower down.

Suprised a thoracic aortogram was done which rrevealed a coarctation!


And, a review of the MR image donw earlier showed the finding very well.


The chest x ray reviewed now revealed ell-tale signs-large heart and rib notching!

and no one had read it well.

The problems in this case were:

1. Femoral pulses were palpable and equal on both sides

2. Radial pulses not compared with femoral pulses initially

3. Patient was non-hypertensive

4. No evidence of limb ischaemia

5. During angiogram the catheter and guidewire used to pass up straight due to the low coarctation hence could not be made out.


Trans-coarctation gradient of 30 mm Hg was recorded at angiography

Two Embolisation Attempts: 12 March and 16th March 2009

Failed as the feeding artery located within the mesh of tortuous vessels could not be catheterized

Patient was sent to the surgeons who attempted to operate -ligated some of the vessels entering the Cervical canal from the right side at C6 level in the hopw that the feeding artery could be excluded from circulation.

Post surgery MRI: 2nd April 2009 (POD2)

Discussion

¨Untreated patients having coarctation of aorta have an average life expectancy of 32 years
¨Related complications --heart failure, endocarditis, cerebrovascular haemorrhage and aortic rupture
¨About 25.5% of patients die of cardiac failure
¨The incidence of all forms of aneurysm formation in aortic coarctation markedly increases with age, up to 42% in patients over the age of forty
¨Some of the patients can survive normally to adulthood without any specific symptoms

Etiology of Spinal artery aneurysm (Rengachary et al)

¨SCAVMs (25/57)
¨coarctation of aorta (4/57)
¨syphillis and arteritis (3/57)
¨fibromuscular hyperplasia (1/57)
¨Pseudoxamthoma Elasticum (1/57)
¨idiopathic (23/57) - ? Segmental vulnerability

Ø Pathogenesis of spinal arterial aneurysm associated with coarctation of the aorta appears to result from haemodynamic stress related to increased blood flow into the collateral circulation rather than segmental arterial disease or angiodysplastic disease
ØSpinal artery aneurysms usually present as subarachnoid hemorrhage or uncommonly as compressive myelopathy due to mass effect
ØMost of the reported aneurysms are no larger than 3 mm in diameter

P. JIARAKONGMUN, P. CHEWIT, S.PONGPECH. Ruptured Anterior Spinal Artery Aneurysm Associated with Coarctation of Aorta: Case Report and Literature Review. Interventional Neuroradiology 8: 285-292, 2002

Berenstein A, Lasjaunias P: Spine and Spinal Cord Vascular Lesions: Surgical Neuroangiography 5: 32-39, Springer-Verlag.

Rengachary SS, Derek A: Spinal Arterial Aneurysm: Case Report. J Neurosurg 33: 125-130, 1993.

Feng L, Yu-Hai B: Myelopathy and Multiple Aneurysms Associated with Aortic Arch Interruption: Case Report. J Neurosurg 35: 310-313, 1994

Smith BS, Penka CF, Erickson LS: Subarachnoid haemorrhage due to anterior spinal artery aneurysm. Neurosurgery 18: 217-219, 1986

Vincent FM: Anterior spinal artery aneurysm presenting as a subarachnoid haemorrhage. Stroke 12: 230-232, 1981

Djindjian R: Clinical symptomatology and natural history of arteriovenous malformation of the spinal cord – a study of the clinical aspects and prognosis, based on 150 cases. In: Pia HW, Djindjian R (eds) Spinal angiomas: advances in diagnosis and therapy. Springer, Berlin Heidelberg, New York, 1978

Djindjian R, Merland JJ et Al: Angiography of spinal column and spinal cord tumors. Neurological atlas. G Thieme, New York, 1981.




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