Colloid Cyst of the Third Ventricle

MAJ ,Darwin J. ferry, MC, USA* COL LudwigG. Kempe, MC, USA*

THE FIRST documented case of colloid cyst of TABLE I the third ventricle was presented by Wallman PRESENTING SYMPTOMS IN SEVEN PATIENTS WITH in 1858. This tumor was popularized by COLLOID CYST OF THE THIRD VENTRICLE Dandy in his monograph on benigh tumors of

No. of Downloaded from https://academic.oup.com/milmed/article/133/9/734/4918333 by guest on 28 September 2021 the third ventricle published in 1933. Colloid Symptoms cyst of the third ventricle is a rare lesion rep­ Patients resenting 0.55 per cent of all brain tumors or 1. Headache 7 two per cent of all intracranial gliomas." 2. Vomiting 4 Seven cases of colloid cyst of the third ventri­ 3. Visual changes 3 cle from the files of the Neurosurgery Service, 4. Symptoms influenced by change in posture 3 Walter Reed General Hospital are presented 5. Change in personality 2 6. Vertigo 1 with a brief review of the literature. 7. Disturbance of gait 1 8. Sudden loss of consciousness 1 Clinical Picture

Our patients, all males, ranged in age from ported cases, with postural changes influenc­ 19 to 37 years at the time of their admission. ing this distress in 30 per cent." Yennerman The clinical picture (Table I) was character­ et al.:" reported that 44 per cent of their cases ized by intermittent attacks of headache, of colloid cyst of the third ventricle presented often associated with vomiting and transient with no symptoms other than headache, with diplopia or blurred vision. The headache was or without papilledema and vomiting. These throbbing, usually generalized, of sudden same authors cited 11 cases from the litera­ onset, increasing in severity during the day. ture which did not have headache during the There were periods of complete remission course of their illness. Sudden transient weak­ ranging from days to several weeks. A strong ness of the lower extremities ("drop sei­ positional factor was noted in three patients. zures"), often with falling, has been reported Two patients obtained relief by lying down, in 16 cases,"- 18 Riddoch in 1936 emphasized while the other noted increased distress with that progressive dementia may dominate the recumbency. Marked personality changes were clinical picture, even in the absence of head­ observed in two patients over a several month ache and papilledema. Yennerman et al.18 re­ period prior to their admission. Symptoms in­ ported periodic mental and emotional changes creased in frequency and severity with time, in eight cases with persistent derangement in and were present for an average of four five cases. These authors also reported seven months (four days to one year) before admis­ cases of colloid cyst of the third ventricle with sion to the hospital. Physical findings (Table attacks of unconsciousness without seizure. II) were nonspecific and reflected increased intracranial pressure. Diagnostic Studies The clinical picture presented conforms to Lumbar puncture was performed in evalua­ the "classical" history of colloid cyst of the tion of two patients. Cerebrospinal fluid pres­ third ventricle. 8 The reported age of onset of sure and protein were normal in each case. symptoms varies from two months" to 72 Plain skull x-rays showed erosion of the poste­ years, with an average range of 23 to 50 years rior clinoids and dorsum sella, and increased of age. In general there is no sexual predilec­ convolutional markings in two of seven cases. tion. Headache is present in 96 per cent of re- The skull x-rays were considered normal in * Neurosurgery Service, Walter Reed General Hospi­ five cases. tal, Washington, D.C. Right percutaneous carotid arteriography 734 Colloid Cyst of the Third Ventricle 735

was performed in all cases. Lateral projection T ABL E II

of the arterial phase showed bowing of the an­ P OSITIVE PHYSICAL F IND IN GS UPON Ansnssrox IN terior cerebral artery, suggestive of internal SEVE N PATIENTS WITH C OLLOID CYST OF hydrocephalus. These changes were reflected THET HIRD V ENTRICLE in lateral displacement of the striothalamic No. of veins on frontal projection. The internal cere­ Sign P atient s bral veins were displaced downward and flat­ tened in the midportion (Fig. 1a & b). 1. Papilledema 6 Downloaded from https://academic.oup.com/milmed/article/133/9/734/4918333 by guest on 28 September 2021 The normal configura tion of the central ve­ 2. Altered state of consciousness 4 nous system as defined by Fisher- in his study 3. Pupillar y signs 3 of 200 normal cerebral venograms was pro­ 4. Ataxia 2 5. Visual field defect 1 jected onto the cranio-cerebral-topornetric sys­ tem described by Delmas and Pertuiset." A line was projected from the tub erculum sella was projected onto this normal scheme. The to the bregma and another from the bregma to venous angle was displaced downward thr ee to the lambda. These lines were each divided six mm., displaced posteriorly four to nine into three equal segments. The correct venous mm., and the middle third of the internal ce­ angle is located at the intersection of a line rebral vein was displaced downward eight to perpendicular to the upper 0 of bregma­ eleven mm. lambda line and a line perpendicular to the Ventriculography was performed through 4 lower 0 of the sella-bregma line , 9 (Fig. 2) . bifrontal burr holes. Symmetrically dilated The central venous system in its lateral view lateral ventricles with nonfilling of the third ventricle was found. On frontal projection a bulge at the foramen of Monro would be seen, while the cyst itself was outlined in lateral projection (Fig. 3a & b). These changes con­ form to standard descriptions.v -"

Embryology Sjovell in 1910 suggested the paraphysis as the site of origin of colloid cysts of the third ventricle. This concept became widely ac­ cepted and the term "paraphysial cyst" was coined. In 1955 Ariens-Kappers" published a careful study of 30 human embryos, describ-

Fig. 2. The dotted lines represent the projected dis­ Fig. 1a and 1b. Carotid arteriography showing dis­ placement of the internal cerebral veins in the reported placement of internal cerebral veins. seven cases . 736 Military Medicine-September, 1968 ing the development of the paraphysis. He one em. perforation was made in the septum concludes that most colloid cysts of the third pellucidum to assure communication of the ventricle arise from diencephalic ependymal lateral ventricle. recesses and accepts two cases of colloid cyst In this series of seven cases there were two described by Zeitlin and Lichtenstein in 1933 postoperative deaths. Four patients are as of paraphysial origin. Shuangshoti and asymptomatic and have returned to military Netsky'" regard colloid cysts or neuroepithe­ duty. One patient is mentally incompetent. lial cysts as enfoldings of the epithelium and The first patient in this series died, never Downloaded from https://academic.oup.com/milmed/article/133/9/734/4918333 by guest on 28 September 2021 stroma of the choroid plexuses. These authors awakening from anesthesia. Autopsy revealed found histological evidence of such cysts in edema, hemorrhage and infarction about the the majority of 124 (75 mm. embryo to foramen of Monro, with generalized cerebral adult) diencephalic choroid plexuses and 40 edema. The second patient who died did well myelencephalic choroid plexuses studied. They for five days postoperatively and then devel­ present cases and cite reported cases of colloid oped signs of increased intracranial pressure. (neuro-epithelial) cysts occurring elsewhere The craniotomy site was re-explored. The lat­ within the cerebrum near ependymal surfaces, eral ventricle contained a large amount of within the posterior fossa1 3 and the spinal xanthochromic fluid under pressure and the canal. foramen of Monro was occluded by hemor­ rhagic, necrotic tissue. This tissue was de­ Pathology brided, re-establishing communication throu gh The seven cysts within our series varied in the foramen of Monro. External ventricular size from 1.2 cm. to four cm. in diameter. It is of interest to note that the largest reported colloid cyst was nine cm. in diameter." The color of the cysts varied from yellow to blue­ green. They all appeared to arise from the an­ terior extremity of the tela choroidae and in one instance was attached to the pillar of the fornix. The cyst walls were thin, fibrous mem­ branes enclosing a thick tenacious material, in one case approaching the consistency of hya­ line material. The cyst walls were lined with cuboidal epithelium. Nosberg and Black­ wood-s demonstrated ciliated epithelium in 31 of 105 cases reviewed. Coxe and Luse" studied the ultrastructure of the epithelial lin­ ing of a colloid cyst and found abnormal cilia.

Therapy Surgical extirpation of the cyst is the only effective method of treatment. Dandy per­ formed the first successful removal of a colloid cyst of the third ventricle in 1921. Varying his surgical approach in his third case, he resected a core of tissue from the right frontal lobe and entered the lateral ventricle opposite the fora­ men of Monro." This surgical approach was utilized in this series. The cyst contents were Fig. 3a and 3b. Vent,iculography showing dilated aspirated, the base clipped and electrocoagu­ lateral ventricles with nonfilling of the third ventri­ lated, and the capsule excised. In most cases a cle. Colloid Cyst of the Third Ventricle 737 drainage was maintained. Again the patient third ventricle. An electron microscopic study. J. Neu­ improved dramatically. Ventricular drainage ropath. Exp. Neurol., 23 :431-445, 1965. 3 Delmas, A. and Pertuiset, P.: Cranio-cerebral to­ was approximately 200 cc daily and subse­ pometry in man. pp. 148-151, Springfield, Ill., Charles quently a left Holter ventriculo-atrial shunt C Thomas, 1959. was placed. The patient never awakened from 4 Fisher, E.: Die Lagebeziehung der tieferen Hirn­ venen zur Form des Schadels. Dissertation for MD anesthesia and expired several hours later of (Tlibingen) . an unsuspected epidural hematoma at the site 5 Gemperlein, J.: Paraphysial cysts of the third ven­ tricle. Report of two cases in infants. J. Neuropath.

of the shunt pump mechanism. Autopsy re­ Downloaded from https://academic.oup.com/milmed/article/133/9/734/4918333 by guest on 28 September 2021 Exp. Neurol., 19:133-134, 1960. vealed edema and infarction about the fora­ 8 Heavern, R. C. and Young, E. F.: Paraphysial men of Monro with complete occlusion. (colloid) cysts of the third ventricle; Report of eight Two patients within this series were oper­ cases with special attention to diagnostic features. Bull. Los Angeles Neurol. Soc., 24 :138-147, 1959. ated upon as emergencies. One recovered with­ '1 Kapper, J. A.: The development of the paraphysis out neurological deficit. The other patient, cerebri in man with comments in its relationship to who was essentially moribund at the time of the intercolumnar tubercle and its significance for the origin of cystic tumors in the third ventricle. J. Compo surgery, has survived but is mentally incom­ Neurol., 102 :425-498, 1955. petent. 8 Kelly, R. : Colloid cysts of the third ventricle; Anal­ The reported results of surgical treatment ysis of 29 cases. Brain, 74:23-65, 1951. 9 Krayenblihl, H. and Yasargil, M. G.: Die zerebrale of colloid cyst of the third ventricle vary. angiographie. pp. 102-104, Stuttgart, Georg Thieme Weinberger and Boshes'" reviewed 16 cases Verlag, 1965. and added one of their own. All patients sur­ 10 Lu, A. T.: Colloid cysts of the third ventricle of unusual size. Report of two cases. Bull. Los Angeles vived surgery, two were hemiparetic and two Neurol. Soc., 26:89-96, 1961. had mental disturbances. McKisscckt' re­ 11 McKissock, W.: The surgical treatment of colloid ported three deaths in 21 cases. Yennerman et cyst of the third ventricle. A report based upon twenty­ one personal cases. Brain, 74 :1-9, 1951. al.1 8 reported 25 cases which were surgically 12 Nosberg, W. H. and Blackwood, H.: Mucus-se­ treated. There were 12 postoperative deaths. creting cells in colloid cysts of the third ventricle. Seven patients were reported to have a normal J. Neuropath. Exp. Neurol., 13:417-426, 1954. 13 Parkinson, D. and Childe, A. E.: Colloid cyst of existence and six had disabling mental impair­ the fourth ventricle. Report of a case of two colloid ment. cysts of the fourth ventricle. J. Neurosurg. 9 :404-409, 1952. Summary 14 Rusell, D. S. and Rubenstein, L. J.: Pathology of tumors of the nervous system. 2nd Ed., pp. 142-143, Seven cases of colloid cyst of the third ven­ Baltimore, Williams and Wilkins Co., 1963. tricle are reported. The clinical picture, diag­ 15 Shuangshoti, S. and Netsky, M. G.: Neuroepithe­ nostic approach and surgical treatment are lial (colloid) cysts of the nervous system. Further ob­ servations in pathogenesis, location, incidence, and his­ discussed. A method of measuring displace­ tochemistry. Neurol. (Minn.), 16:887-903, 1966. ment of the deep venous structures which has 16 Taveras, J. M. and Wood, E. H.: Diagnostic neu­ been helpful in the diagnosis of colloid cyst of roradiology. pp. 1376-1378, Baltimore, Williams and the third ventricle is discussed. Wilkins Co., 1964. 17 Weinberger, L. M. and Boshes, B.: Paraphysial cyst of the third ventricle. Diagnosis and surgical man­ REFERENCES agement. Surge~, 13:368-384, 1943. 1 Bull, J. D. and Sutton, D.: The diagnosis of para­ 18 Yennerman,M. H., Bowerman, C. I. and Hay­ physial cysts. Brain, 72:487-503, 1949. maker, W.: Colloid cyst of the third ventricle. Acta 2 Coxe, W. S. and Luse, S. A.: Colloid cyst of the Neurosurg., 17:212-277,1958.