Treatment was by endoscopic third ventriculostomy. In this procedure a rigid endoscope, introduced via a frontal burrhole, is passed into the lateral ventricle and ...
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Chronic headaches due to occult hydrocephalus Richard J Edwards FRCS Henry Marsh FRCS
Gavin W Britz MD 1
J R Soc Med 2003;96:77–78
Hydrocephalus due to aqueduct stenosis usually presents in childhood. In some cases the hydrocephalus arrests spontaneously but returns in adult life with the steady deterioration associated with raised pressure hydrocephalus, or the chronic ataxia, dementia and incontinence of ‘normal pressure’ hydrocephalus. A less well recognized presentation is chronic headache.
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Case 3
A woman of 31 had experienced headache for eighteen months—intermittent but occurring on most days and often persisting for several days at a time. The headaches were generalized. Severity would fluctuate but was not worse in early morning. Severe exacerbations were occasionally associated with nausea, and less often with vomiting. There was no suggestion of a progressive increase in severity over time. There was no preceding aura although transient visual obscurations, with blurring of the peripheral visual field, occurred up to twenty times a day. These were provoked by the head-down position, which also exacerbated the headaches. On examination she had mild bilateral papilloedema; visual fields and acuity were normal. In view of the postural nature of the chronic headaches and early papilloedema, an MRI scan was requested.
CASE HISTORIES
Case 1
A woman of 22 had suffered headaches for 5 years. They were intermittent but occurred on most days and lasted for several hours or occasionally days. They were generalized and their severity (which was moderate) had not increased over time. They were not worse in the early morning. When she was in head-down position, the headaches were associated with mild nausea and blurring of vision. Neurological examination and fundoscopy were unremarkable. Because of the postural nature of the headaches an MRI scan was requested. Camino-bolt intracranial pressure (ICP) monitoring demonstrated frequent pressure spikes and plateau waves. Case 2
A man aged 52 had a 2-year history of headache, constant and generalized. Severity fluctuated, but it did not increase in the early morning and had not progressively worsened over time. It was exacerbated by coughing and by shouting at his children. There was no associated aura. Results of neurological examination and fundoscopy were normal. Previously he had experienced occasional typical migrainous headaches, unilateral, associated with an aura and triggered by red wine. Because of the change in the headache—in particular the constant, generalized nature and exacerbation by coughing—an MRI scan was requested. ICP monitoring was not performed.
Department of Neurosurgery, Atkinson Morley’s Hospital, Copse Hill, Wimbledon, London SW20 0NE; 1Department of Neurological Surgery, University of Washington School of Medicine, and Harborview Medical Center, Seattle, Washington, USA Correspondence to: Mr H Marsh FRCS, Department of Neurosurgery, Atkinson Morley’s Hospital, Copse Hill, Wimbledon, London SW20 0NE, UK
CASE REPORTS
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INVESTIGATIONS AND TREATMENT
In all three patients MRI scans demonstrated chronic moderate to severe triventriculomegaly consistent with a diagnosis of idiopathic aqueduct stenosis (Figure 1). Treatment was by endoscopic third ventriculostomy. In this procedure a rigid endoscope, introduced via a frontal burrhole, is passed into the lateral ventricle and through the foramen of Monro into the third ventricle. The floor of the third ventricle is then fenestrated by means of a 5-French Fogarty balloon catheter1. This bypasses the aqueductal obstruction enabling the cerebrospinal fluid to be absorbed by the arachnoid granulations, obviating the need for a traditional ventriculoperitoneal shunt procedure. The procedure was uncomplicated and all three patients had complete resolution of their headaches. At latest follow-up (mean 18 months) all were free of recurrent symptoms. COMMENT
Idiopathic aqueduct stenosis may arise as a result of either congenital abnormalities of aqueduct development or aqueductal gliosis, the aetiology of which is unclear. None of the three patients reported here had a history of childhood hydrocephalus or of previous neurological disorders. The acronym LOVA (longstanding overt ventriculomegaly in adults) has been used to describe patients with (presumed) infantile aqueductal stenosis that presents in adult life2. Five main modes of initial presentation, either alone or in combination, can be identified in LOVA—(i) impairment of memory and intellectual decline; (ii) gait unsteadiness and falls; (iii) epilepsy; (iv) ‘pressure-type’ headaches; (v) papilloedema or optic atrophy with visual failure2,3. Rarer presentations include cerebrospinal fluid rhinorrhoea or pituitary endocrine dysfunction2.
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suspicion of intracranial abnormality with associated raised ICP, and should prompt radiological investigation, even in the absence of other indicators of raised ICP such as earlymorning exacerbation, papilloedema or impairment of consciousness. Headaches due to raised ICP are not necessarily progressive. A substantial change in the character of a headache (as in patient 2) should also prompt investigation. ICP monitoring may be helpful in determining the clinical significance of radiological ventriculomegaly4. The reasons for late decompensation of hydrocephalus in LOVA remain obscure2. Once symptoms have developed, patients may remain static or progressively deteriorate. There is a risk of occult visual field loss and visual failure2. If patients with mild static symptoms are managed conservatively, regular monitoring of vision is mandatory. Previous reports have highlighted the risks of ventricular shunt procedures in treating massive ventriculomegaly. Subdural haematoma formation, which may be catastrophic, has been reported as complicating up to 78% of shunt insertions for LOVA2. Long-term shunt-related morbidity is also high with revision eventually necessary in more than 50% of patients undergoing ventricular shunting5. Many neurosurgeons would not contemplate shunt surgery in patients with headache alone, because of the risk of complications6. Endoscopic third ventriculostomy has now emerged as the treatment of choice for both children and adults with aqueduct stenosis1. Because ventricular size decreases only modestly, the risk of haemorrhagic complications is low. A recent meta-analysis reported a 0.1% mortality, a 1.3% risk of permanent neurological deficit and a 0.3% risk of subdural haemorrhage1. The procedure makes physiological sense, restoring cerebrospinal fluid flow between the obstructed ventricular system and the subarachnoid space. Endoscopic third ventriculostomy offers a safe and effective method of controlling chronic headaches in patients with LOVA in whom the risks of surgery may formerly have been judged too high. Figure 1 MRI and CT scans in patient 3. (A) Preoperative, TI-weighted, axial images showing triventriculomegaly due to idiopathic aqueduct stenosis. (B) Postoperative axial CT showing modest reduction in ventricular size following endoscopic third ventriculostomy
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Our cases are unusual in that all patients presented solely with chronic headaches of mild to moderate severity with a paucity of other symptoms and signs. However, the headaches all had some features suggestive of raised intracranial pressure. First, they were generalized; second, they were exacerbated on leaning forwards, coughing or shouting; third, in two cases transient visual obscurations were associated with changes in posture. In patients with chronic headache these features should always raise
REFERENCES
1 Pople IK, Edwards RJ, Aquilina K. Endoscopic methods of hydrocephalus treatment. Neurosurg Clin N Am 2001;12:719–35 2 Oi S, Shimoda M, Shibata M, et al. Pathophysiology of long-standing overt ventriculomegaly in adults. J Neurosurg 2000;92:933–40 3 Harrison MJ, Robert CM, Uttley D. Benign aqueduct stenosis in adults. J Neurol Neurosurg Psychiatry 1974;37:1322–8 4 Iborra J, Pages E, Cuxart A, Poca A, Sahuquillo J. Increased intracranial pressure in myelomeningocele (MMC) patients never shunted: results of a prospective preliminary study. Spinal Cord 2000;38:495–7 5 Casey AT, Kimmings EJ, Kleinlugtebeld AD, Taylor WA, Harkness WF, Hayward RD. The long-term outlook for hydrocephalus in childhood. A ten-year cohort study of 155 patients. Pediatr Neurosurg 1997;27:63–70 6 Honeycutt JH, Einhaus SL, Hill RF, Shappley WV, Sanford RA. Is my shunt revision rate lower than yours? (abstract). J Neurosurg 2002;96:419
