Postgrad Med J: first published as 10.1136/pgmj.27.306.160 on 1 April 1951. Downloaded from i6o

BLOOD- VESSEL TUMOURS OF THE BRAIN* By C. WORSTER-DROUGHT, M.A., M.D., F.R.C.P. Physician, West End Hospitalfor Nervous Diseases; Physician and Neurologist, Metropolitan Hospital, London, etc.

Blood-vessel tumours or angiomata which occur in the brain may be classified under two headings: (i) Malformations of blood-vessels and (2) Haemangioblastomata, or true neoplasms of the elements forming blood-vessels. The former result from a fault in development, and differ from the haemangioblastomata in that they consist of an accumulation of definite, although enlarged, blood-vessels with nervous tissue, intact or com- pressed, between the vascular loops constituting the angioma. It is generally agreed that all types of intra- cranial angiomata are of congenital origin, although often of only slow growth and development. Angiomatous malformations include the following conditions: by copyright. (a) Telangiectatic angiomata. These are small, mainly capillary, lesions similar to those frequently seen in the skin, and are of no clinical importance as they do not cause symptoms. Such telangiec- tases are discovered only incidentally during post- mortem examinations, and occur most frequently in the floor of the fourth ventricle and in the upper part of the pons. (b) Capillary angiomata involve the meninges http://pmj.bmj.com/ and surface of the brain, and frequently co-exist FIG. I.-Sturge-Kalischer-Weber Syndrome. A girl, with similar capillary naevi of the face; and some- aged 4* years, showing extensive cutaneous naevi times of the trunk on the same side of the on left side of face, upper part of chest and arm. body. The upper part of the back on the left side, the This association is known as the Sturge-Kalischer- left leg, both buttocks, and to a lesser extent the Weber syndrome. right side of the face, also showed scattered naevus Sturge, in 1879, reported the case of a girl, formations. The child was mentally deficient and suffered from and six with an extensive naevus, right-sided hemiplegia Jack- on September 28, 2021 by guest. Protected aged years, capillary sonian epilepsy. X-ray examinationr of the skull especially of the right side of the face and head, showed bilateral intracranial calcification, but more and focal epileptiform attacks starting in the left pronounced on the left side. hand. He concluded that the right side of the brain was involved in the naevoid condition. X-ray examination of the skull revealed a more or Kalischer, in I897, reported a case of diffuse con- less calcified lesion on the surface of the left genital naevus involving the scalp and face which, cerebral hemisphere. on post-mortem examination, showed a lesion The symptoms and signs of Sturge-Kalischer- described as a blood-vessel tumour composed of Weber syndrome consist in the presence of a facial capillaries and veins. Parkes Weber, in 1922, naevus, with or without similar lesions in other reported the radiographic appearances of the skull parts of the body (Fig. i), and epilepsy-usually of a typical case which showed a large capillary Jacksonian in type and involving the side of the naevus involving especially the left side of the body contralateral to the facial naevus. Not in- face, and a right-sided upper neurone hemiplegia. frequently, a definite hemiparesis is present (Fig. i) with consequent increase in tendon reflexes and * Based on a postgraduate lecture given at the West an extensor plantar reflex. These conditions may End Hospital for Nervous Diseases, London. also be associated with buphthalmos (ox-eye or Postgrad Med J: first published as 10.1136/pgmj.27.306.160 on 1 April 1951. Downloaded from

April 1951 W()RSTER-DR(UGIIT: Blood-Vessel Tlumouirs of the Brain I6I by copyright.

FIG. 2.-Sturge-Kalischer-Weber Syndrome. Skiagram of skull in the case shown in Fig. i. The calcification occurring in the capillary angioma extends from the frontal region to the superior occipital region and http://pmj.bmj.com/ is seen in the form of radial markings, particularly in the parieto-occipital zone. The markings in the posterior frontal region suggest enlargement of the middle meningeal artery. (Reproducedfrom the author's conmmurnication by courtesy of the Editor, British Medical Journal ') congenital glaucoma) on the same side as the main An extensive naevus involving one side of the cutaneous naevus, and with mental deficiency. face co-existing with contralateral Jacksonian In view of the latter symptom the condition is epilepsy or a hemiparesis, or with both conditions, sometimes termed ' naevoid amentia.' X-ray is sufficient to justify the diagnosis of cortico- on September 28, 2021 by guest. Protected examination not infrequently reveals the intra- meningeal capillary (mainly) angioma, even if not cranial calcification (Fig. 2) to which reference revealed by calcification on X-ray examination. has already been made. (c) Venous angiomata. Cerebral angiomata con- As judged by the few post-mortem examinations sisting entirely of veins are less frequently encoun- it has been possible to carry out, the intracranial tered than other forms of angiomata. Cushing lesion would appear to be mainly a capillary and Bailey (1928) in a series of 1,822 intracranial angioma involving the leptomeninges, although tumours verified by operation or autopsy, met with some enlarged veins may also be present. The 29 examples of blood-vessel tumour, only six of affected cerebral hemisphere is sometimes smaller which were venous angiomata. than that of the opposite side. Very rarely the WNith cerebral venous angiomata, symptoms condition is bilateral; one case was reported by develop at a relatively later age than in either the writer (I948) and N. 1I. M. Burke, in a sub- capillary or arterio-venous angiomata.- In most of sequent communication, mentioned a case that the recorded cases, the first symptoms appeared showed bilateral meningeal angiomata, confirmed in the early twenties. As a rule; the initial symp- by post-mortem examination, but in which the tom is epilepsy-usually focal or Jacksonian- cutaneous naevi were comparatively slight. indicating that the venous angioma in most cases Postgrad Med J: first published as 10.1136/pgmj.27.306.160 on 1 April 1951. Downloaded from

162 POSTGRADUATE MEDICAL J(URNAL April 195 t

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FIG. 3.-Venous Angioma of Cerebrum. Drawing of the operation area exposing a venous angioma involving the right cerebral hemisphere and consisting of enlarged and dilated thin-walled veins. At one point a firm adhesion is seen between an enlarged vein and the dura. The patient, a man aged 31, had suffered from left- sided Jacksonian epilepsy of four years' duration, with gradually increasing hemiparesis. Follow- ing operation (local decompression), the hemi- paresis did not progress and the Jacksonian epileptic attacks were reduced in frequency. Death occurred six years later from intracranial haemorrhage, from one of the dilated and thin- walled veins. (Reproduced from the author's communication by courtesy of the Editor, ' The Lancet ') by copyright. http://pmj.bmj.com/ on September 28, 2021 by guest. Protected

FIG. 4.-Venous Angionta of Cerebrlum. Vertical coronal section through the brain of case illustrated by Fig. 3 showing venous angioma of the right cerebral hemisphere with thin-walled and dilated veins extending from outer to inner aspect of hemisphere. Death occurred from intracranial haemorrhage owing to rupture of one of the enlarged veins. (Reproduced from the author's communication by courtesy of the Editor 'Jou4rnal of Neurology, Neurosurgery% and Psychiatry ') Postgrad Med J: first published as 10.1136/pgmj.27.306.160 on 1 April 1951. Downloaded from April 1951 W()RSTER-DROUGHT: Blood-Vessel Tumlours of the Brain I63 involves the motor cortex of the cerebrum. matic in late childhood or in early adult life. Occasional headaches also occur and in other Epilepsy, Jacksonian in type, is then an almost cases there is slow development of hemiparesis. constant symptom, and is followed later by hemi- Papilloedema has been recorded as a late mani- plegia and possibly hemianopia. Some cases festation in a few cases, but is more frequent in show exophthalmos, usually on the side of the arterio-venous angioma. Fatal haemorrhage from lesion but occasionally bilateral, with or without rupture of one of the thin-walled veins is not swelling of the optic discs, a condition rarely seen infrequent. with venous angioma. Increased vascularity of Unlike the mainly capillary angioma of the the scalp and temporal region, with enlarged and Sturge-Kalischer-Weber syndrome, the purely pulsating arteries, hypertrophy and increased pul- venous angioma does not tend to calcify and is, sation of one or both carotid arteries, with therefore, not demonstrable by radiography. secondary cardiac enlargement, may also occur. The venous angioma usually consists of large, Diagnosis ofintracranial angioma. The diagnosis dilated, thin-walled and non-pulsating veins con- of the Sturge-Kalischer-Weber syndrome has taining purely venous blood and involving a cir- already been discussed. Cerebral venous angio- cumscribed area of the cerebrum, especially the mata may be more common than generally sup- cortex (Fig. 3). According to their anatomical posed, as if such angiomata involve a so-called characters, venous angiomata can be classified into: silent area of the brain, they may give rise to no (i) Simple varices-a much enlarged single vein; symptoms beyond occasional headache and so (ii) Serpentine varices, consisting of several en- remain unsuspected. Most recorded cases of larged and tortuous veins; and (iii) Racemose or cerebral venous angioma have been diagnosed Cirsoid varices, which are formed of a collection of either on exposure of the lesion by surgical opera- tangled veins, usually forming a cone-shaped mass, tion, carried out on account of focal epilepsy,. or the base of which approximates to the cerebral discovered on examination. The cortex the into post-mortem (Fig. 4), apex penetrating deeply arterio-venous angioma (angioma arteriale), being by copyright. the brain and often reaching the lateral ventricle. usually aneurysmal in character, may give rise to The terms ' cirsoid,' usually employed by French an audible bruit on ascultation of the skull and so authors, and ' racemose' by German writers, have reveal its presence; occasionally enlarged blood- often and inappropriately been applied to arterio- vessels may be seen in the scalp, temporal regions, venous angiomata, that is, a mainly arterial blood- or neck. The co-existence of such enlarged vessel tumour, whereas a cirsoid or racemose vessels with an audible bruit is pathognomonic of angioma may consist entirely of veins. Cushing arterio-venous angioma. and Bailey suggested the term' angioma venosum,' In other cases in contrast to ' developing Jacksonian epilepsy, qualified by racemosum, angioma with or without mild hemiplegic signs and no http://pmj.bmj.com/ arteriale,' similarly qualified. papilloedema, and a normal cerebrospinal fluid, (d) Arterio-venous angioma, which Cushing and an angioma may be suspected. An arterio-venous Bailey termed ' angioma arteriale,' consists of a angioma (angioma arteriale) will often be visible mass ofsinuous and tangled pulsating blood-vessels as a partially calcified and diffuse mass on X-ray of varying size, both thin-walled and thickened, examination of the skull, but this is not so with through which arterial blood flows from enlarged a purely venous angioma. In any case, the next arteries of entry. The angioma is almost invariably step is to carry out angiography by means of the aneurysmal in character, showing arterio-venous injection of io cc. of diodone solution, preferably on September 28, 2021 by guest. Protected communications, either from its inception or 30 to 40 per cent., into the carotid artery, and by developing later. The blood escapes from the the percutaneous route rather than by open angioma by one or more greatly dilated veins. operation. The first exposure is made as the The arterio-venous angioma almost certainly injection proceeds, with two further exposures at arises from the pial vessels and, although visible intervals of two seconds, in order to obtain both in the cortex of the brain beneath the arachnoid, arteriograms and venograms (Fig. 5). Lateral and invariably extends deeply into the brain and may antero-posterior views shofld be taken, if possible, even reach the lateral ventricle. Occasionally, the to demonstrate the full extent of the angioma. angioma has extensive vascular connections with Treatment of cerebral venous and arterio-venous the dura, while the blood-vessels of the scalp, angioma. Decompression over the lesion may lead especially in the temporal region, and the carotid to symptomatic improvement and especially to artery, may also be enlarged (Fig. 5a). As with the relief of headache, if present, as well as to some angioma venosum, the angioma arteriale may be reduction in the frequency and intensity of qualified as simple, serpentine or racemose, the epileptic attacks. In the writer's experience, how- latter being the most common. ever, ligature of the carotid artery, on the same The angioma arteriale usually becomes sympto- side as the cerebral lesion, yields even better Postgrad Med J: first published as 10.1136/pgmj.27.306.160 on 1 April 1951. Downloaded from

I64 POSTGRADUATE MEDICAL JOURNAL April 195 r

L (a) (1,) FI,. 5.-Arterio- Venous Atngioma of righit Cerebral Hemisphere. (a) Right lateral carotid angiogram taken by copyright. during the injection of diodone. In addition to the arterio-venous angioma, enlargement of the right internal carotid artery is seen. (b) Angiogram taken by exposure two seconds after (a). The arterio- venous angioma is shown occupying mainly the right Sylvian fissure. The case was that of a man, aged 40, who had been healthy until two years previously. At that time he had an epileptic attack, followed by a second attack ten months ago, since which he had complained of lethargy and lack of concentration. There were no abnormal physical signs, but a bruit was heard over the right eyeball after the angiogram had revealed the lesion. (Included by kind permission of Dr. J. W. D. Bull.) results and reduces the risk of haemorrhage from vascular spaces with a stroma of endothelial cells http://pmj.bmj.com/ the angioma. If exposed by operation, attempts containing fatty granules, the whole being sur- at ligature of the thin-walled veins of a venous rounded by a hyalinized zone; (b) Cellular, con- angioma may lead to disastrous results and they taining large epithelial cells with prominent nuclei are best left alone. and granules, between which run the vascular Superficial cutaneous angiomata appear sus- channels; (c) Cavernous, composed of large vas- ceptible to radiotherapy, and improvement has cular cavities lined with endothelial cells and been reported in some cases of proven cerebral showing, in the surrounding tissue, epitheloid cells on September 28, 2021 by guest. Protected angiomata. Radiotherapy, with or without de- with a fine reticulum. These neoplasms often compression, may offer some hope of benefit and form cysts of varying size containing xantho- consequently this form of treatment is worthy of chromic fluid, in which the angioblastoma is trial. represented by a mural nodule. Occasionally, the Haemangioblastoma. The haemangioblastomata cysts in the cerebellum are multiple. are true neoplasms composed of primitive vaso- In some cases, a cerebellar haemangioblastoma formative cells (angioblasts) which enter into the is associated with a similar condition in the retina, development of the primordial blood-channels. the combination often being termed van Hippel's They are congenital in origin, often single but disease. In other cases, the angioblastomata are sometimes multiple, and they arise almost ex- found not only in the cerebellum but also in the clusively in the cerebellum. Ilere they are often spinal cord, kidneys, suprarenals, pancreas and median tumours, solid or cystic, and appear to liver, the syndrome usually being known as arise from the posterior end of the roof of the Lindau's disease. Several cases showing a heredo- fourth ventricle. familial incidence have been reported. The haemangioblastoma may be: (a) Capillary, Cerebellar haemangioblastomata should be consisting of a small mass of capillaries or small treated whenever possible by surgical extirpation. Postgrad Med J: first published as 10.1136/pgmj.27.306.160 on 1 April 1951. Downloaded from April 1951 ;KAPLAN: Oesophageal Hiatis Iernia I65 \When the tumour is cystic, following evacuation the symptoms will recur, either from refilling of of the contents of the cyst, care must be taken to the cystic cavity or from progressive growth of the remove the nodule in the cyst wall, as otherwise angioblastomi.

BIBLIOGRAPHY BULL, J. WV. D. (1949), Proc. Roy. Mcd(., 42, 88o. WNEBEFR, F'. PARKES (1922), 7. .Xurol. amd P'sychopathol., 3, 134. BURKE, N. H. M. (1948), Brit. Med. J., ii, 531. WORS'lER-DROUGHTC, C., and BALLANCE, C. A. (1922), CUSHING, H., and BAILEY, P. (1928), ' Tumours Arising fromn Lancet, ii, 125. the Blood-vessels of the Brain,' Balliere, London, p. 250. WORSTER-DROUGHT, C., and DICKSON, W. E. CARNEGIE KALISCHER, S. (1897), Berl. klin. Wochenschr., 30, I059. (1927), J. Neurol. and Psychopathol., 8, 19. STURGE, W. A. (1879), Trans. Clin. Soc. Lond., 12, 162. WORSTER-DROUGHT, C. (I948), Brit. Med. J., ii, 214.

OESOPHAGEAL HIATUS HERNIA A Clinical Study of Forty-Five Cases By SAMUII, KAIPLAN, M.). Dc'/,rtmc'nt of illedicine, Univcersity of Witi (latcrsralnd, JIIoanI( 'sburt

Oesophageal hiatus hernia may be defined as a Hiatus herniae of infancy and childhood and trau- transdiaphragmatic evisceration of some part of matic herniae are not included in this study. the abdominal contents into the thoracic In this series the cases are divided into

cavity. categories by copyright. 'The herniation occurs through the oesophageal according to the following classification intro- hiatus and the organ that is most commonly in- duced by Akerlund (1926): vollved is the stomach. It is now well established I. Congenital short oesophagus with partial that herniation through the oesophageal hiatus of tlioracic stomach. the diaphragm is not infrequent. 'The complex 2. Paraoesophageal hiatus hernia. A portion of symptomatology presented by this anatomical the cardiac end of the stomach herniates through variant may, however, render difficulty in its the oesophageal hiatus of the diaphragm. The diagnosis. oesophagus is of normal length and in its normal for which the seek 'I'he symptoms patients may position. http://pmj.bmj.com/ advice may be divided into the following groups: 3. Oesophagogastric hernia. Both the cardiac i. A syndrome of' dyspepsia.' end of the stomach and the lower oesophagus 2. Pain in the chest stimulating myocardial in- herniate through the oesophageal hiatus. farction. The term ' sliding hernia' is used to designate 3. Acute gastrointestinal haemorrhage with those cases in which the stomach returns to its haematemesis, melaena or both. normal sub-diaphragmatic position when the 4. Symptoms of anaemia probably due to patient is erect. Sliding herniae are only demon- chronic gastrointestinal haemorrhage. strable with the patient supine or in the Trendel- on September 28, 2021 by guest. Protected 5. A combination of any or all of the above enburg position. The term 'congenital short groups. oesophagus' may be a misnomer, although in- In addition to the above groups there is a fre- stances of true congenital shortening of the oeso- quent association of other pathological conditions phagus are well known (Gross, I946). In this with hiatus hernia. These include cholecystitis series, however, instances of short oesophagus were with or without cholelithiasis, diverticulosis of the found in elderly patients, the oldest being 89 years large or small intestine, and chest signs possibly of age. Although it is possible that the shortened due to aspiration of oesophageal contents. oesophagus was present from birth, there are other This report concerns the clinical study of 45 causal factors to be considered. A paraoesophageal cases of oesophageal hiatus hernia that presented or oesophagogastric hernia may, for instance, be as- as in and out patients at the Johannesburg General sociated with post-ulcerative fibrosis, which may Hospital over a period of 12 months ending shorten the oesophagus in a manner indistinguish- March 1949. able radiologically from a congenital short oeso- Of the 45 cases, 33 were females and I2 males, phagus. Allison (1948) has produced evidence to giving a ratio of females to males of 3 to I. The show that short oesophagus is usually an acquired age incidence varied from 35 to 89 years (T'able I). condition, due to defects in the diaphragm which