Porencephaly diagnosed byisotope cisternography · clear Chicago) and a rectilinear scanner (Pho...

Journal of Neurology, Neurosurgery, and Psychiatry, 1972, 35, 669-675

Porencephaly diagnosed by isotope cisternographyD. FRONT, J. W. F. BEKS, AND L. PENNING'

From the Departments of Neuroradiology and Neurosurgery, University Hospital, Gronintgen,The Netherlands

SUMMARY The diagnosis of porencephaly by isotope cisternography is described. In the threecases presented, porencephaly was associated with non-resorptive hydrocephalus. The communi-cating hydrocephalus caused the isotope to enter the ventricular system and visualize the cyst, andthe diagnosis of both disorders was established by RIHSA cisternography. The method is simpleand non-traumatic and provides information about abnormalities which air may fail to demonstrate.

Isotope cisternography has proved to be very

useful in the diagnosis of disturbances of flowand absorption of cerebrospinal fluid (CSF) andtheir resultant hydrocephalus (Di Chiro, Reames,and Matthews, 1964; Bannister, Gliford, andKocen, 1967; James, DeLand, Hodges, and Wag-ner, 1970; Front, 1971), and in the recognition ofCSF rhinorrhoea (Di Chiro and Grove, 1966;Di Chiro, Ommaya, Ashburn, and Briner, 1968;Front and Penning, 1971).

In addition, we have found this investigationto be very valuable in the diagnosis of local ab-normalities of the CSF spaces. In this paperthree cases will be described in which por-encephaly was diagnosed by isotope cisterno-graphy.

METHOD

Radioiodinated human serum albumin (RIHSA) isused. Routinely 100 Ci are injected into the lumbartheca. Freshly prepared RIHSA of high specificactivity (1 mg albumin/100 [. Ci) is used. The thyroidgland is blocked by three drops of Lugol's solutiondaily for nine days, starting one day before the in-vestigation. Each study is performed with both a

scintillation gamma camera (Pho Gamma III Nu-clear Chicago) and a rectilinear scanner (Pho dot,Nuclear Chicago). There are several reasons for this.A preliminary study with the gamma camera givesa quick general orientation and suggests whether itis necessary to use views and positions besides theroutine anterior and lateral ones. The rectilinearscanner gives somewhat better resolution in the1 Reprint requests: Professor Dr. L. Penning, University Hospital,Department of Neuroradiology, Groningen. The Netherlands.

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plane of the collimator than does the gamma camera.Every patient is studied at four, 24, and 48 hoursafter injection.

CASE 1

A 39 year old man suffered a head injury in a roadaccident. Bleeding was noticed from his nose andmouth but no abnormality was found on neurologi-cal examination. Plain radiographs of the skullshowed fractures of the nasal, right maxillary, andzygomatic bones. Some days later his conscious leveldecreased, and he was found to have bitemporalhemianopsia and a dilated right pupil. There wassome leakage of clear fluid from his nose. A rightcarotid angiogram was performed and this showedthat both anterior cerebral arteries were pushed up-wards, especially anteriorly, suggesting a spaceoccupying lesion below them. Operation for correc-tion of rhinorrhoea and exploration of the chias-matic region showed a blood clot which was com-pressing the chiasm. The immediate post-operativecourse was uneventful, but his vision deterioratedand he showed signs of mental impairment. RIHSAcisternography was performed (Fig. 1). The fourhour scintigrams showed filling of the ventricles incontinuity with a large abnormal accumulation ofradioactivity in the right frontal region. At the 24hour examination no radioactivity was found overthe brain convexities. At 48 hours the radioactivityin the ventricles was indistinct, but the abnormalfrontal radioactivity was very marked. The resultswere thought to indicate a large right anterior por-encephalic cyst, connected with the right lateral ven-tricle. The fact that no radioactivity was found overthe brain convexities and that the ventricles werefilled with the radiopharmaceutical indicated the

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... ;.; ,, Ogr,6 .<y .7, -*,-...* v | . ... tw.FIG. 1 RIHSA cisternography. Case 1. Porencephalic cyst and hydrocephalus. (a) Anterior and (b) lateralviews at four hours. The radiopharmaceutical fills the cisterna magna, basal cisterns and mildly enlarged ven-tricles, and a large anterior cyst to the right of the midline. This cyst is in continuity with the anterior horn ofthe right ventricle. (c) Anterior and (d) lateral views at 24 hours. Hardly any radioactivity is recorded over thebrain convexities. The ventricles and the cyst are filled with the radiopharmaceutical. (e) Anterior and (f) lateralviews at 48 hours. Most ofthe radioactivity is seen in the cyst andpart of it in the ventricles. Little radioactivityis recorded over the brain convexities.

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Porencephaly diagnosed by isotope cisternography

FIG. 2. Pneumoencephalography. Case 1. Poren-cephalic cyst and hydrocephalus. Lateral tomogramsdemonstrating the frontal cyst in continuity with an

enlarged right ventricle.

presence of communicating non-resorptive hydro-cephalus. Pneumoencephalography (Fig. 2) revealeda large anterior cyst communicating with the anteriorhorn of the right lateral ventricle, and dilated ven-

tricles, thus confirming the findings of RIHSAcisternography.At operation the cerebral cortex in the basal

region of the right frontal lobe was very thin. Itenclosed a brain cyst communicating with the an-

terior horn of the ventricle. The cyst was opened andcommunication was established between the rightventricle and the subarachnoid space. In view of thehydrocephalus a ventriculoatrial shunt was installed.

After operation his general condition and especi-ally his mental state improved rapidly and markedly.

CASE 2

A 41 year old man was admitted because of recurrentattacks of pneumococcal meningitis probably re-

lated to rhinorrhoea. Nine years previously he hadbeen admitted to another hospital because of a dis-charge of CSF from the nose. Radiographs of the

skull had shown erosion of the sella and digitateimpressions of the vault, obviously due to increasedintracranial pressure. Tomograms had revealed adefect in the lamina cribrosa on the left side. Duringhis stay in hospital he had developed pneumococcalmeningitis which had responded to antibiotics. Atoperation several defects in the floor of the anteriorfossa had been plugged with muscle and coveredwith fascia.RIHSA cisternography (Fig. 3) during the present

admission demonstrated filling of enlarged lateralventricles, which persisted until 72 hours. No radio-activity was seen over the convexities. A large accu-mulation of abnormal activity was shown in thefrontobasal region of the brain, continuous with theleft lateral ventricle and overlying the anterior partof the roof of the nose. The results were interpretedas being due to a large anterior porencephalic cystcommunicating with the anterior horn of the leftlateral ventricle. The filling of the ventricles and thelack of radioactivity over the convexities indicatedthe presence of non-resorptive communicatinghydrocephalus.Pneumoencephalography (Fig. 4) demonstrated

enlarged ventricles communicating with a por-encephalic cyst of the left frontal lobe.

Operation revealed many defects in the floor ofthe left anterior fossa which were covered. The por-encephalic cyst was opened so that it communicatedwith the subarachnoid space. The post-operativecourse was uneventful and slow recovery followed.

CASE 3

A 57 year old man suffered a head injury in a roadaccident. He was comatose, and was bleeding fromthe nose, ears, and mouth. A right oculomotor pare-sis was noticed. Plain radiographs of the skull showedfractures of the floor of the anterior fossa and rightorbit. The patient slowly regained consciousness butremained very disoriented and confused. He com-plained of severe headaches, and his gait was mark-edly abnormal. An electroencephalograph (EEG)showed a right frontotemporal disturbance, and abrain scan showed an abnormal area of uptake in theright frontotemporal region. A right carotid angio-gram did not demonstrate any space occupyinglesion, but the thalamostriate vein was markedly dis-placed laterally, suggesting hydrocephalus. Pneumo-encephalography confirmed the presence of hydro-cephalus but did not show any other abnormality.RIHSA cisternography (Fig. 5) was undertaken: atfour hours it showed filling of markedly enlargedventricles, and at 24 hours a large abnormal accumu-lation of radioactivity was seen lateral to the right

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FIG. 3. RIHSA cisternography. Case 2. Porencephalic cyst and hydrocephalus. (a) Anterior and (b) lateralviews atfour hours. The radiopharmaceutical fills the basal cisterns and enlarged ventricles. An accumulation ofradioactivity is seen lateral to the left ventricle in the anterior view, and anterior to it in the lateral view, repre-senting early filling of the cyst. (c) Anterior and (d) lateral views at 24 hours. The radiopharmaceutical outlinesthe lateral ventricles and fills the porencephalic cyst more completely. No radioactivity is recorded over thebrain convexities. (e) Anterior and (f) lateral views at 48 hours. The radiopharmaceuticalfills the whole extentof the cyst and the enlarged lateral ventricles. A very large amount of radioactivity still remains in the head.

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FIG. 4. Pneumoencephalography. Case 2. Poren-cephalic cyst and hydrocephalus. Lateral tomogramdemonstrating the frontal cyst in continuity with an

enlarged left lateral ventricle.

ventricle; this accumulation was even more evidentat 48 hours.The focal abnormality was taken to indicate a large

porencephalic cyst communicating with the rightlateral ventricle. The filling of the ventricles and thelack of any radioactivity over the convexities were

thought to be the signs of communicating non-

resorptive hydrocephalus.At operation a cyst of the right frontal lobe was

found, with a narrow communication between thecyst and the frontal horn of the right lateral ventricle.The basal cortex of the right frontal horn was ex-

cised, and a pathway was established between theright ventricle and the subarachnoid space.

There was a significant improvement in headaches,mental state, and the gait after the operation.

DISCUSSION

Porencephaly, to use the definition of Le Countand Semerak (1925), is 'a defect communicatingwith the ventricles or separated from them by a

thin layer of brain tissue and covered on the

outside by the arachnoid'. Generally, however,the term is used to describe any defect in thebrain tissue communicating with the ventricles(Pendergrass and Perryman, 1946; Naef, 1958;Barrett and Mendelsohn, 1965; Hamer andPiscol, 1971).According to their aetiology, porencephalic

cysts are divided into developmental and ac-quired (Naef, 1958). Acquired cysts are theresult of destruction of cerebral tissue from vari-ous causes, such as trauma, vascular insufficiencyor infection. In cases 1 and 3 the cysts wereprobably the result of trauma, and case 2 wasknown to have recurrent bouts of meningitis.

These cysts, although sometimes 'silent', oftencause a variety of clinical symptoms, and mayprogress in size (Drew and Grant, 1948; Handaand Bucy, 1956; Naef, 1958; Barrett and Men-delsohn, 1965; Cantu and Le May, 1967; Hamerand Piscol, 1971). These facts, to use the wordsof Barrett and Mendelsohn (1965) 'justify morevigorous attempts at early diagnosis and treat-ment'.Pneumoencephalography is the usual method

for the diagnosis of porencephaly, but it may notalways reveal the full extent of the lesion (Pen-dergrass and Perryman, 1946). Even when ameticulous technique is employed it may com-pletely fail to demonstrate the cyst, as happenedin case 3. The small opening by which the cystcommunicated with the lateral ventricle probablyexplains why air failed to enter it. RIHSA, asshown in our cases, enters the cysts easily anddemonstrates their communication with the ven-tricle and their size. The manoeuvring and differ-ent positioning used in pneumoencephalographyor ventriculography are not necessary. Large ex-perience has shown that isotope cisternographyis a simple and harmless method (Front, 1971).RIHSA investigations in our patients showed

that, in addition to a porencephalic cyst, theyhad a hydrocephalus of the communicating non-resorptive type, due to obstruction to CSF flowover the cerebral convexities. This associationwas also reported by Hamer and Piscol (1971)who were unable to determine the nature of thehydrocephalus. In our cases the hydrocephaluswas caused by post-traumatic subarachnoid hae-morrhage in two cases and by meningitis in thethird. Such disorders lead to meningeal adhesionsand obliteration of the subarachnoid space, so

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FIG. 5. RIHSA cisternography. Case 3. Porencephalic cyst and hydrocephalus. (a) Anterior and (b) lateralviews at four hours. The radiopharmaceutical fills the cisterna magna, basal cisterns, and enlarged lateral ven-tricles. An accumulation of radioactivity is seen lateral to the right ventricle in the anterior view, and the lateralview shows that it is in continuity with the anterior horn. This represents early filling of the cyst. (c) Anteriorand (d) lateral views at 24 hours. The radiopharmaceutical fills the lateral ventricles and a large cyst lateral tothe right ventricle in the frontal region. No radioactivity is recordedfrom the brain convexities. (e) Anterior and(f) lateral views at 48 hours. The radiopharmaceutical is mainly in the cyst. Radioactivity is also recordedfromthe ventricles. There is no radioactivity over the brain convexities.

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that CSF and RIHSA can no longer reach thenormal site of absorption along the superiorsagittal sinus. They are then absorbed by analternative mechanism, through the walls of theventricles (Wislocki and Putnam, 1921; Beringand Sato, 1963; Sahar, Hochwald, and Ranso-hoff, 1969; Sahar, Hochwald, and Ransohoff,1970; Front, 1971). This transventricular absorp-tion is thought to account for the flow of CSFand RIHSA into the ventricles. As RIHSA in-jected in the lumbar region does not enter theventricles in normal individuals, the visualizationof the porencephalic cysts in our cases was madepossible by the simultaneous presence of non-resorptive hydrocephalus. As shown in Figs leand f, 3e and f, 5e and f, a large amount of radio-activity remained in the cysts at 48 hours. Itseems probable, therefore, that CSF and RIHSAare only slowly absorbed through the cyst wallor that they have to reenter the ventricles inorder to be absorbed.

CONCLUSION

When associated with non-resorptive communi-cating hydrocephalus isotope cisternography canprovide information about the size, site, andcommunication of porencephalic cysts and, whenair fails to enter the cyst, it may be the onlymeans of making the diagnosis. It also demon-strates that the associated hydrocephalus iscaused by disturbances in CSF flow and absorp-tion; this information is essential for adequateneurosurgical treatment.

REFERENCES

Bannister, R., Gliford, E., and Kocen, R. (1967). Isotopeencephalography in the diagnosis of dementia due to com-municating hydrocephalus. Lancet, 2, 1014-1017.

Barrett, J. W., and Mendelsohn, R. A. (1965). Post-traumatic

porencephaly in infancy. A report of three unusual cases.Journal of Neurosurgery, 23, 522-527.

Bering, E. A. Jr., and Sato, 0. (1963). Hydrocephalus: chan-ges in formation and absorption of cerebrospinal fluidwithin the cerebral ventricles. Jolurnal of Neurosuirgery, 20,1050-1063.

Cantu, R. C., and LeMay, M. (1967). Porencephaly caused byintracerebral hemorrhage. Radiology, 88, 526-530.

Di Chiro, G., Reames, P. M., and Matthews, W. B. Jr. (1964).RISA-ventriculography and RISA-cisternography. Neu-rology, 14, 185-191.

Di Chiro, G., and Grove, A. S. Jr. (1966). Evaluation of sur-gical and spontaneous cerebrospinal fluid shunts by isotopescanning. Journal of Nelurosurgery, 24, 743-748.

Di Chiro, G., Ommaya, A. K., Ashburn, W. L., and Briner,W. H. (1968). Isotope cisternography in the diagnosis andfollow-up of cerebrospinal fluid rhinorrhea. Journal ofNeurosurgery, 28, 522-529.

Drew, J. H., and Grant, F. C. (1948). Benign cysts of thebrain. An analysis with comparison of results of operativeand non-operative treatment in thirty cases. Journal ofNeurosurgery, 5, 107-123.

Front, D. (1971). Scinticisternography and scintiventriculo-graphy. Thesis, University of Groningen, The Netherlands.

Front, D., and Penning, L. (1971). Occult spontaneous cere-brospinal fluid rhinorrhoea diagnosed by isotope cisterno-graphy. Neuroradiology, 2, 167-169.

Hamer, J., and Piscol, K. (1971). Die Bedeutung des ven-trikulo-atrialen Shunts in der Behandlung der wachsendenPorenzephalie. Acta Neurochirurgica, 24, 187-200.

Handa, H., and Bucy, P. C. (1956). Benign cysts of the brainsimulating brain tumor. Journal of Neurology, 13, 489-499.

James, A. E. Jr., DeLand, F. H., Hodges, F. J. Ill, andWagner, H. N. Jr. (1970). Normal-pressure hydrocephalus.Role of cisternography in diagnosis. Journal of the Ameri-can Medical Association, 213, 1615-1622.

LeCount, E. R., and Semerak, C. B. (1925). Porencephaly.Archives of Neuirology and Psychiatry, 14, 365-383.

Naef, R. W. (1958). Clinical features of porencephaly. Areview of thirty-two cases. Archives of Neurology and Psy-chiatry, 80, 133-147.

Pendergrass, E. P., and Perryman, C. R. (1946). Por-encephaly. American Journal of Roentgenology, 56, 441-463.

Sahar, A., Hochwald, G. M., and Ransohoff, J. (1969).Alternate pathway for cerebrospinal fluid absorption inanimals with experimental obstructive hydrocephalus.Experimental Neurology, 25, 200-206.

Sahar, A., Hochwald, G. M., and Ransohoff, J. (1970).Passage of cerebrospinal fluid into cranial venous sinusesin normal and experimental hydrocephalic cats. Experi-mental Neuirology, 28, 113-122.

Wislocki, G. B., and Putnam, T. J. (1921). Absorption fromthe ventricles in experimentally produced internal hydro-cephalus. American Journal of Anatomy, 29, 313-320.

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