We have so far in this series gone through various headaches including migraines, cluster headaches and trigeminal neuralgia. I have also talked about subarachnoid haemorrhages and the headaches patients experience subsequent to such lesions. This article will discuss a very different form of headache resulting from a very rare condition – a cerebral haemangioma or cavernoma. It is always interesting to listen to the patient’s symptoms and this should give one an indication as to whether the headaches are serious and needs immediate referral.
CASE 1
A 55-year-old female patient booked an urgent appointment having experienced pressure pain behind the left eye for the past two weeks. She was also finding that her eyes hurt on looking up and reported headaches at the back of the head. Further symptoms included a tingling sensation on top of the head.
Ocular Findings
R -3.00DS (6/12 & N5 unaided)
L -3.00DS (6/9 & N5 unaided)
Anterior examination – bilateral blue-dot cataracts (left eye more significant)
Muscle balance – orthophoria.
Motility revealed no incomitancy though the left eye appeared painful on looking up.
Fundus examination – no optic nerve swelling and no vascular abnormalities.
Intraocular Pressures – R. 15mmHg & L. 15mmHg (12.30pm).
Visual fields – no obvious hemianopia except for a few missed points (figure 1 - above).
Action taken
Patient was referred via her general practitioner to a neurologist to rule out the following:
- Retrobulbar neuritis – in view of the eyes hurting on movement.
- Aneurysm or sub-arachnoid haemorrhage – in view of the head pain and tingling sensation.
Neurologist findings
Blood tests and blood pressure tests revealed no abnormalities, and blood pressure was normal. MRI scan revealed no signs of sub-arachnoid haemorrhage, no aneurysm of the cerebral vessels and no mass or lesions within the cortex.
Interestingly, the MRI scan did reveal a tiny spot of blood or a minor bleed in the frontal lobe on the left side (figure 2 – shown as blue shading in the right frontal lobe). There was no evidence of middle cerebral artery leak. The conclusion drawn from this bleed that it was a cavernous cavernoma that resulted in the symptoms experienced by the patient.
Figure 2: MRI scan revealed a minor bleed in the frontal lobe on the left side – shown as blue shading in the right frontal lobe
CASE 2
This is personal summary of a patient’s experience during a fateful week:
‘My symptoms began while away on holiday. I had a very high temperature followed by a bad headache. After taking painkillers, I felt a little better for two days. Then the headache returned on the left side. I felt very nauseous, couldn’t eat and needed to lay down all day. The pain then started in my neck and I had a tingling sensation in my left arm. This was the day before we were due to fly home. I knew then it was more serious than I had originally thought but didn’t want to go to a Spanish hospital. The next day I woke up feeling very unwell and began vomiting. I managed to travel home, although I had pain in my head and neck and the numbness in my arm had worsened. I didn’t want to sit in A and E for hours so, once home, I called NHS Direct.
‘After much messing around on their part, I finally had an appointment to see an out-of-hours doctor at Watford General. She examined me and, while bending my head, made the pain significantly worse. She thought it might be severe migraines but due to the high temperature they were concerned it might be meningitis. I was sent to the acute assessment unit where I had all sorts of tests, including a CAT scan. I was treated with antibiotics for meningitis. They then noticed a shadow on the scan and an MRI was arranged. It took five days and lots of consultation with the specialists at Queens Square to find there was an abnormality in my brain but they were not clear what it was.
‘By this time my left arm was completely numb and could not use it. I was transferred to Queens Square and the next day given an angiogram to check how serious the problem was. The result was positive and I was then told about the cavernoma.
‘I was still in severe pain. They kept me there for five days treating me with only pain killers. The bleed had stopped and the blood needed to disperse and the swelling in the brain to go down. The pain was most severe in my left ear by this stage. I did have a strange episode with my vision while watching TV. The screen went fuzzy, from the outside in, and my vision went completely for a few seconds. I was told that if this happened at the same time as pain in my head I would be sent straight for a scan but it didn’t happen again. The feeling had improved in my arm. This had been their main concern all along.
‘They had said all along that they would avoid operating as the location was very delicate with no room for error. After begging to go home as I hadn’t seen my children for so long, they agreed I could go home but I would be monitored with regular scans. The pain and symptoms did persist for quite some time and I was back and forth to the doctors. The scans have all been positive but there is a risk it could happen again. I’m to be scanned annually unless something happens.’
Figure 3: Upper cervical cavernoma seen in sagittal view and axial view. The top images appear to show the bleed (yellow arrow) in much greater detail. The bottom images appear to show just a faint bleed. Grey (grey arrow) is the spinal cord and black is the bleed while the white (white arrow) is the cerebrospinal fluid surrounding the cord
Optometrist involvement
The patient attended for an eye examination on the recommendation of her mother. Her chief complaints were of blurring of vision while watching television and also when reading. She found she was unable to concentrate when reading, and this was even worse when using the computer. She was taking fleconide for heart palpitations.
Ocular Findings
(6/9) R. -0.25DS (6/6 & N5)
(6/9) L. -0.25DS (6/6 & N5)
Colorimetry Test
Hue/colour 260 and saturation/depth of colour 40. Spectacles were made with precision tint of blue 1 and purple 6 plus a UV coating was made for constant wear.
Diagnosis
Pattern glare or visual stress due to her illness and high temperature which had caused difficulties with her vision and hence precision tinted spectacles were prescribed for the patient’s benefit.
Neurological Scans (shared by the patient)
Angiography results were negative for any bleed associated with vertebral, basilar, internal carotid, anterior cerebral, posterior cerebral or middle cerebral vessels. The MRI scan showed a very subtle bleed on the superior part of the spinal cord and inferior section of the medulla oblongata. The swelling would have extended up the brainstem and to the pons, explaining the earache as the vestibular nerve (eighth nerve) is close by. Similarly, the sensory nerve is in close proximity, and this would explain the numbness of the left side of the face and left arm.
Discussion
Cavernoma (or haemangiomia or angioma) is a collection of abnormal vessels filled with blood that resemble a blackberry because of the bubble like caverns (figure 4). The lesions are benign non-malignant tumours derived from the endothelial cells of the blood or lymphatic vessels. Cavernomas are located in the brain, the brainstem and the spinal cord and they vary in size from a few millimetres to a few centimetres in diameter.
Figure 4: Clockwise from top left: Blackberry with bubble like caverns, eyelid haemangioma showing abnormal capillaries in a similar fashion, T2 echo gradient MRI image showing the cavernoma as a black lesion due to blooming artefact
Cavernomas located in the brainstem have the highest likelihood of causing harm. Due to the malformation of blood vessels, blood flow through the caverns or cavities is slow and the structural support for the smooth muscle by the endothelial cells is weak leading to periodic bleeds. It is this bleeding that gives rise to the variety of symptoms such as headaches, seizures and strokes. Periodic bleeds, and the resultant haemosiderosis, gives rise to locules of various size containing blood products in different stages of evolution giving a ‘popcorn like’ appearance. Both our patients had recent bleeds, either in the brain or the spinal cord, and being fresh there was a rounded black defect rather than the classic popcorn ball with a haemosiderin rim.
Most cavernomas are congenital and develop over the course of a life time. They occur in about one in 200 people sporadically, although inherited forms do exist giving rise to characteristic multiple lesions. Annually, one in 120 cavernomas will bleed and usually the symptoms of headaches or seizures would lead to either a CT or MRI scan to be undertaken. Due to there being a slow bleed, contrast enhancement CT will not show the cavernoma while the enhanced CT scan may show a hyper dense nodule or calcification. Gradient echo T2 and susceptibility weighted MRI will markedly increase the sensitivity of the MRI to detect small cavernomas. They will appear as black lesions due to blooming artefacts. There is no definite cause but research shows genetic mutation results in the onset of the malformations.
Most cavernomas resulting in headaches are monitored annually but, for those that result in haemorrhage, focal neurological symptoms or seizures, microsurgery is indicated to remove the malformation. In our second patient the location of the cavernoma on the spinal cord was risky and hence no surgical intervention was undertaken and she is also monitored annually.
Conclusion
I hope optometrist’s eyes are open to very unusual conditions giving rise to headaches and once again I am grateful to the two patients who have provided their scans for our learning.
Kirit Patel works in private practice in Radlett, Hertfordshire.