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Spontaneous Cerebral Ventriculostium: Two Cases J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.35.5.676 on 1 October 1972. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry, 1972, 35, 676-681 Spontaneous cerebral ventriculostium: two cases G. C. KANJILAL From Cranage Hall Hospital, Holnes Chapel, Cheshire SUMMARY Two cases of 'spontaneous cerebral ventriculostium' are reported. In one case the diagnosis was made during life, and the hydrocephalus was not relieved by the ostium. The unique feature of the other has been the presence of a large defect in the skull bones, posteriorly, which has been a 'nature's help' towards maintaining the hydrocephalus stationary, after an initial period of progression. The spontaneous formation of an opening be- CASE 1 tween a dilated cerebral ventricle and the brain P.S., a female aged 29 years, had been vaguely un- surface has rarely been recorded. Sweet (1940) well for three months, and had complained of a stiff described two such cases and found only two pre- neck, occipital headache, and difficulty in walking for viously recorded examples (MacGillavry, 1910; six weeks. She had been deaf for several years and de Lange, 1929). Leslie and Alker (1964) reported was stated always to have been mentally subnormal. a further case, and while reviewing the literature Amenorrhoea had been noted for the past year. SheProtected by copyright. on the subject maintained that the rupture of an was a well-nourished woman ofmasculine build, with obstructed cerebral ventricle directly into the heavy jaw, moderate hair development on the upper with lip, and a male distribution of pubic hair. The head subarachnoid space consequent bypassing was bent forward while walking and there was some of the block appears to be exceedingly rare. They ataxia. Rombergism was present. Fundi showed bi- stated that before their case report a search ofthe lateral papilloedema, more marked on the right. The literature had yielded only four such cases right pupil was smaller than the left, and did not (MacGillavry, 1910; de Lange, 1929; Sweet, react to light but both reacted normally on accom- 1940; Tandon and Harkmark, 1959) in contrast modation. Ocular movements were full. Nystagmus with over 20 ventricular diverticula. Another case was induced by looking upwards or to either side. of spontaneous ventriculostomy has been re- The left soft palate showed slight paresis. Labyrin- ported in the Hungarian literature by Scheda and thine tests and tests for auditory function were not Csanadi (1963). Liss and Mervis (1965) reported possible due to lack of cooperation. There were bi- a case of spontaneous ventriculostomy through lateral extensor plantar reflexes; abdominal reflexes the lamina terminalis and Lavender and Du were absent. The right knee jerk was normal, the left absent. Some dysdiadokokinesia of the arms was http://jnnp.bmj.com/ Bouley (1965) described three cases of aqueduct noted but none of legs. The WR was negative in stenosis exhibiting a cystic expansion of the blood and cerebrospinal fluid (CSF). The CSF con- posterior end of the third ventricle into the pos- tained protein 30 mg/100 ml., cells 1 per c.mm, terior fossa. pressure 350 mm water. Blood urea was 31 mg/100 The two cases reported below are interesting in ml. Ventriculography (Fig. 1 a, b) revealed the that they are in the rare group of only six cases presence of a big cavity in the arachnoid space below reported so far, and, in addition, show ventricu- the posterior horn of the lateral ventricle. The pre- lar diverticula as well. The second case is also a sence of air in the cavity confirmed that there was an on September 23, 2021 by guest. good clinical example of multiple complications abnormal communication between this and the ven- in tricular system. of hydrocephalus one individual. These are the An exploratory operation was proposed, but death only two cases seen in the Pathology laboratory, occurred during induction of anaesthesia. It should North Staffordshire Royal Infirmary, over the be stressed that this operation was carried out in last 30 years, and this also emphasizes the rarity 1941, when the risks of giving general anaesthetics to of this condition. this type of case were not appreciated. From what is j76 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.35.5.676 on 1 October 1972. Downloaded from Spontaneous cerebral ventriculostium: two cases 677 a;I FIG. 1 a and b. Ventriculograms showing thepresence ofa big cavity in the arachnoid space below the posterior horn of the lateral ventricle. The presence of air in this cavity signifies an abnormal communication with the ventricular system. Protected by copyright. ..... g _~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~_Fr-<>~~~ http://jnnp.bmj.com/ on September 23, 2021 by guest. FIG. 2. Sagittal section of the cerebral hemisphere showing the presence of an abnormal cavity above the cerebellum, the latter being considerably deformed as a result. The opening in the anterior wall through which the third ventricle is communicating with this cavity is arrowed. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.35.5.676 on 1 October 1972. Downloaded from 678 G. C. Kanjilal now known about the rises in CSF pressure which was scanty. The cervix and body of the uterus were of occur under all forms of general anaesthesia, it approximately equal length and the greater part of seems likely that death was inevitable. the cervix was supravaginal. Both ovaries showed thick white capsules with numerous cysts 0-625-1 25 NECROPSY FINDINGS Skull and brain The skull was cm in diameter in the cortical zone; the medulla con- thin with prominent vascular markings. There was sisted of homogeneous white connective tissue. compression of the pituitary body and erosion of the posterior clinoid processes by the distended third Microscopy The mass in the brain-stem was an ventricle. astrocytoma consisting of interwoven glial fibres and The gyri were flattened; the distended third ven- numerous piloid astrocytes, and in its anterior por- tricle bulged into the interpeduncular fossa, and tion there were fairly numerous multinucleated there was a moderately well-developed cerebellar large-bodied astrocytes. The destruction of the pressure cone. Between the tentorium cerebelli and aqueduct appeared to be complete and no structure the cerebellum was a fluid-containing cavity formed suggesting aqueductal tissue was found. by arachnoid membrane, and situated in the mid- The adrenals showed slight convolution of the cor- line. tex (within normal limits). No fuchsinophil material A sagittal section showed extreme dilatation of the was demonstrated by Vine's method. The pituitary first three ventricles and enlargement of both fora- gland appeared normal. mina of Monro (left 7 x 5 mm, right 9 x 4 mm). The corpus callosum was raised and stretched so that its COMMENTS The clinical and pathological find- posterior portion passed forward to terminate in the ings in this case suggest a very slowly progressive splenium. The cause of the hydrocephalus was lesion. The deafness which had been present for obliteration of the cerebral aqueduct by a mass years was possibly central in origin, and caused situated ventral to the quadrigeminal plate, measur- by the tumour. The symptoms which the ing 16 x 21 x 18 mm. It was firm, sharply demarcated brought Protected by copyright. from the surrounding brain, and its cut surface patient under observation, and among which showed grey and white interlacing fibres. The normal stiffneck and occipital headache were prominent, site of commencement of the cerebral aqueduct was were produced by a rapid increase in the sub- marked by a U-shaped depression, but no remnant tentorial pressure resulting from obstruction of of the canal itself could be found. the tentorium, mainly by the expanding arach- Situated in the posterior commissure was a roughly noid cavity. The masculine characteristics and circular opening 8 mm in diameter, and through this the regressive changes in the ovaries and uterus the third ventricle communicated with the fluid- were considered a consequence of disturbance of containing cavity already noted. The superior colli- the pituitary gland or related neural centres re- culi lay below this opening, and the pineal gland and from distension of the third tela chorioideae of the third ventricle above it. The sulting ventricle. cavity with which the ventricle communicated, was situated in the mid-line between the cerebellum and CASE 2 the tentorium cerebelli, and was triangular in the P.A., a male aged 15 years had been an inpatient of sagittal section, measuring 45 mm anteroposteriorly Stallington Hospital since 4 months old. He had been and 30 mm transversely. Compression by this cavity admitted from a local general hospital (City General http://jnnp.bmj.com/ had resulted in considerable deformity of the cere- Hospital) where he was admitted soon after birth, bellum (Fig. 2). Its floor was formed posteriorly by and diagnosed as a case of severe rapidly increasing the superior surface of the cerebellum, and anteriorly hydrocephalus, his head circumference having grown by the quadrigeminal plate and anterior medullary by 12-5 cm during two and a half months stay there. vellum. The anterior wall contained the opening The patient was crying day and night, moving his already noted and the attenuated posterior commis- head frequently as if in pain, rubbing it on the pillow sure. The roof was formed by arachnoid mater run- and needing frequent sedation. This, together with ning from the anterior wall to the posterior limit of the psychological effect of the patient's appearance on September 23, 2021 by guest. the floor. on the parents and the disturbing effect on other sib- lings, precipitated his admission to Stallington Other organs Both lungs were conspicuously small, Hospital. the right weighing 230 g and the left 213 g.
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