Acta Neurochir (Wien) (1990) 104:54--58

:Acta . N urochlrurgica
9 by Springer-Verlag1990

CSF Dynamics in Dandy-Walker Syndrome

T. Ozgen and B. A$ikgfz
Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey

Summary
As neuro-imaging techniques have improved, variations of the Dandy-Walker malformation are being reported. In order to succeed in the therapy and avoid or minimize the complications, the fluid dynamics have to be evaluated by contrast CT studies. We are reporting three different types of this malformation and discuss the therapeutic possibilities.
Keywords: Dandy-Walker syndrome; hydrocephalus; computerized tomography; Iohexol.

Introduction
The clinical syndrome known as the Dandy-Walker malformation is characterized by cystic dilatation of the fourth ventricle with agenesis or hypoplasia of the cerebellar vermis and hydrocephalus 14. Although in 1887 a case of hydrocephalus with a posterior fossa cyst and hypoplasia of the cerebellar vermis was reported by Sutton 23, D a n d y and Blackfan are the first to describe the condition as a clinical triad 8. D a n d y 7, and Taggart and Walker 2s, in 1921, pointed to atresia of the foramina of Magendie and Luschka as the cause. In 1954 Benda 2 named the syndrome Dandy-Walker Syndrome, unfortunately excluding the names of Blackfan and Taggart. He stated that the main pathology is a developmental disturbance of the cerebellum 2. In 1955 Gibson 12 reported some cases with patent foramina of Luschka's and he used the term partial communicating hydrocephalus. Brodal and Hauglie-Hanssen 4 reporting their observations on hydrocephalic mice in 1959 stated that before opening of the fourth ventricle, two cerebellar hemispheres fuse on the midline. Gardner 11 on the other hand described a variant of this syndrome which he named DandyWalker Cyst. He believed that the ependymal layer of the rhombic r o o f is more permeable to CSF than the

outer glia and pia, and the cyst contains high-protein fluid. To date a lot of variations have been described as neuroimaging techniques have improved 15. These variations are very important in planning treatment. We believe that lack of standardization o f treatment is the result of these variations. The presence of hydrocephalus is very important and surgery is indicated when there is such evidence 14' 16 In the past, pneumoencephalography, ventriculography, angiography and radio-isotopes have been commonly used for diagnosis1' 5, 6, 20, 22, 24, 29. Nowadays CT and M R I are the investigations 12 most frequently used. Plain CT has been found insufficient by several authors in evaluating the condition 1' 18. Some advise contrast dynamic CT studies 13,18,27. We share the same opinion, and believe that we have to know the fluid dynamics in order to succeed with treatment and avoid or minimize complications. The aim of this report is to point out this necessity and discuss the errors of interventions performed without adequate preoperative investigations.

Material and Methods

We have twenty patients who had surgical intervention for Dandy-Walker Syndrome at our institution and chosen four cases from this group to present and discuss the histories, the neuroradiological investigations and the modes of treatment.

Case Reports
Case 1:

This 40-day-old boy was admitted to Hacettepe Medical Centre with macrocephaly.Head circumferencewas 41 cm, the anterior fontanelle was tense. There were also typical ophthalmic "sunset" signs. CT examination revealed an enlarged cystic fourth ventricle, besides vermian hypoplasia and hydrocephalus. A ventriculo-atrial shunt

T. Ozgen and B. Aqikg6z: CSF Dynamics in Dandy-Walker Syndrome was inserted. On the fifth postoperative day he became unconscious. Control CT examination revealed bilateral subdural haematomas. The haematomas were evacuated immediately and the shunt was removed. A few days later dynamic CT examination was performed. Contrast material (Iohexol) was given to the intravenricular CSF and there was no filling of the cystic cavity due to obliteration of the aqueduct (Fig. 1). Contrast material as a second step was introduced by lumbar puncture and there was no filling of the cyst cavity again. One day later there appeared a sudden respiratory and cardiac arrest, resuscitation failed and the patient died. Unfortunately we could not receive permission for postmortem examination. After this experience we decided to perform dynamic studies on every case of so-called Dandy-Walker syndrome.

55 by dynamic CT scan. Iohexol was given intrathecally by the lumbar route. Half of the cyst cavity was filled with contrast. The contrast was then given by the ventricular route. In the initial scan the contrast did not pass through the aqueduct (Fig. 2 b). Five hours later another scan was performed which revealed filling of the cyst (Fig. 2 c). A more magnified section revealed a septum partially dividing the cavity into two parts (Fig. 2 d). Using the same postero-parietal skin flap, a ventricular catheter was introduced and also a second catheter was placed into the posterior cyst and the two were connected to the distal portial using a "Y" connector. Control examination six month later revealed head circumference to be 43 cm's and the baby was neurologically normal. This patient is an example of Gibson's partial communicating hydrocephalus 12
Case 3:

Case 2J

This 6-month.-old female was admitted to hospital because of darkbrown lesions scattered throughout her skin. Head circumference was 47 cm. There was no history of trauma or infection. An enlarged fourth ventricle and vermian hypoplasia was found on CT (Fig. 2 a). There was also hydrocephalus. The patient was examined

This 5-month-old female patient was admitted with macrocephaly. Examination revealed increased head circumference. There was also slight bilateral abducens paresis and a low grade atrial septal defect. CT examination revealed a hydrocephalic patient with a posterior fossa cyst as well as vermian agenesis. Contrast material was inserted into the lateral ventricle from the right frontal regien. The contrast filled the cavity but there was no communication between the cyst and the subarachnoid space (Fig. 3). Also the cavity was

Fig. 1. Contrast ventriculography indicating the obstruction of the aqueductus sylvii

Fig. 3. Contrast ventriculography, the cyst was filled with contrast indicating a patent aqueductus

Fig. 2 a. Posterior fossa cyst with vermian hypoplasia Fig. 2 b. Contrast ventriculography, initial phase, indicating obstruction of aqueductus, also, partial filling of the cystic cavity with lumbar contrast injection can be seen Fig. 2c. Contrast ventriculography, late phase, complete filling of the cyst cavity

56

T. }zgenand B. Agikg6z: CSF Dynamics in Dandy-Walker Syndrome

Fig. 4. An example of an isolate posterior fossa Dandy-Walker Cyst. (a) Cystic dilatation of the fourth ventricle with vermian hypoplasia. (b) Contrast cisternography, the cystic cavity has no communication with the subarachnoid cisterns. (c) Contrast ventriculography indicating a nonpatent aqueductus

not filled with contrast given by the lumbar subarachnoid space. A ventriculo-peritoneal shunt was performed. She was normal and the shunt was working one year after the procedure. She also has control examinations for atrial septal defect in another hospital. This patient was a typical example of atresia of the foramina Magendie and Luschka.
Case 4:

This boy was brought to hospital at one year of age because of increasing head circumference. Physical examination revealed a large-sized head (52 cm). On CT examination hydrocephalus with a cystic dilatation of the fourth ventricle was found besides vermian hypoplasia (Fig. 4 a). CT with iohexol given by the lumbar route revealed subarachnoid cisterns but there was no contrast in the fourth ventricle (Fig. 4 b). CT ventriculography revealed a non-patent aqueduct (Fig. 4 c). Because the patient had non-communicating hydrocephalus with a non-patent aqueduct, two shunt catheters, one to the ventricle, the other to the cyst cavity, were introduced and connected to the peritoneum by a Y connector. On the third postoperative day there was a CSF leak to the subgaleal area. So the shunt system was revised. Two months later the patient was found to be in good condition with a functioning shunt. This case is an example of isolated fourth ventricular cyst, which might have developed due to compression of the aqueduct by a posterior fossa Dandy-Walker Cyst as stated by Gardner n.

Discussion
There are several questions which must be clarified in the preoperative evaluation o f the D a n d y - W a l k e r malformation. Presence or absence o f hydrocephalus, position and patency o f the a q u e d u c t o f Sylvius, presence or absence o f c o m m u n i c a t i o n between the cavity and the subarachnoid space and degree o f c o m m u n i cation, if it exists, are a m o n g them. Differential diagnosis o f a posterior fossa arachnoid cyst or an enlarged cisterna m a g n a is also essentialt' 9, 19. Other associated abnormalities are also i m p o r t a n t 6' 14, 17, 21. Connections o f the fourth ventricle 3' a8 m u s t

be k n o w n in detail. Some investigators have performed air studies in order to see the fourth ventricle 1, 3. The evidence o f a n o r m a l and patent fourth ventricle refutes the diagnosis o f D a n d y - W a l k e r m a l f o r m a t i o n 1' 10,18 M a s d e u 18 on the contrary, reported the importance o f slits between the cerebellar hemispheres which m a y cause misdiagnosis in air studies. O u r second case is a g o o d example o f this view. The septa dividing the cyst cavity can easily cause misdiagnosis. In the past some authors tried to get the answer by air or radioisotope studies 16, 20. Conventional CT, the m o s t usual technique nowadays, m a y be insufficient to explain this complex entity. As far as we k n o w the best w a y is to p e r f o r m metrizamide or iohexol ventriculogr a p h y and cisternography. We believe that b o t h have to be performed to evaluate the fluid dynamics in detail. O u r first case is a g o o d example supporting this concept. We m u s t k n o w the type o f m a l f o r m a t i o n in the preoperative period. A n o n c o m m u n i c a t i n g type with n o n - p a t e n t aqueduct must be treated by c o m b i n e d ventricular and cystoperitoneal shunting in order to prevent u p w a r d herniation as r e p o r t e d in the literature 21' 2s. Only inserting a ventricular catheter caused the error in our first case. The first step in diagnosis is to identify whether it is the c o m m u n i c a t i n g type or the n o n - c o m m u n i c a t i n g type. I f the cyst was f o u n d to be a c o m m u n i c a t i n g one, then the patency o f the aqueduct must be investigated. I f an aqueduct obstruction does not exist than there is no need for drainage 13 unless the pressure is elevated. The mechanism o f hydrocephalus f o u n d in cases o f this type c a n n o t be cleared. As seen f r o m the study o f our second case, the diminished rate o f flow t h r o u g h the aqueduct m a y be a cause. A semi-permeability or ballvalve like m e c h a n i s m m a y be another cause as sug-

T. 0zgen and B, A9ikg6z: CSF Dynamics in Dandy-Walker Syndrome

57 cases with particular reference to so-called atresia of the foramina of Magendie and Luschka. J Neurol Neurosurg Psychiatry 22:99-108 Carmel PW, Antunes JL, Hilal SK etal (1977) Dandy-Walker Syndrome. Clinico-pathological features and re-evahiation of modes of treatment. Surg Neurol 8:132-138 D'Agostino AN, Kernohan JW, Brown JR (1963) The DandyWalker Syndrome. J Neuropath Exp Neurol 22:450470 Dandy WE (1921) The diagnosis and treatment of hydrocephalus due to occlusions of the foramina of Magendie and Luschka. Surg Gynec Obstet 32:112-124 Dandy WE, Blackfan KD (1917) Internal hydrocephalus. Am J Dis Child 14:424-443 Fileni A, Colosimo C Jr, Mirk P e t a l (1983) Dandy-Walker Syndrome diagnosis is utero by means of ultrasound and CT correlations. Neuroradiology 24:233-235 French BN (1982) Midline fusion defects and defects of formation. In: Youmans JL (ed) Neurological surgery. WB Saunders, Philadelphia, pp 1288-1289 Gardner WJ (1977) Hydrodynamic factros in Dandy-Walker and Arnold-Chiari malformations. Child's Brain 3:200-212 Gibson JB (1955) Congenital hydrocephalus due to atresia of the foramen of Magendie. J Neuropath Exp Neurol 14: 244262 Groenhout CM, Gooskens RH, Veiga-Pires JA et al (1984) Value of sagittal sonography and direct sagittal CT of the DandyWalker Syndrome. AJNR 5:476M77 Hart MN, Malamud N, Ellis WG (1972) The Dandy-Walker Syndrome. A clinico-pathological study based on 28 cases. Neurology 22:771-778 Harwood-Nash DC, Fitz CR (1976) Congenital malformations of the brain. In: Neuroradiology in infants and children. CV Mosby, St Louis, pp998-I053 Hirsch JF, Pierre-Kahn A, Reiner D e t a l (1984) The DandyWalker malformation. A review of 40 cases. J Neurosurg 61 : 515-522 Kojima T, Waga S, Shimisu T, Sakakura T (1982) DandyWalker cyst associated with occipital meningocele. Surg Neurol 17:52-57 Masden JC, Dobben GD, Azar-Kia B (1983) Dandy-Walker Syndrome studied by computed tomography and pneumoencephalography. Radiology 147:109-114 Mercader JM, Llenas JS (1982) Arachnoid cyst of the posterior fossa associated with hydrocephalus. Neurochirurgia 25: 135137 Palma A, Nazar N, Cas-ro M e t al. (1982) Dandy-Walker malformation and the contribution of radioisotopic studies of cerebral spinal fluid dynamics to its diagnosis. Case Report. Acta Neurochir (Wien) 61:319-324 Raimondi AJ, Samuelson G, Yarzagaray L etal (1969) Atresia of the foramina of Luschka and Magendie. The Dandy-Walker cyst. J Neurosurg 31:202-216 Sawaya R, McLaurin RL (1981) Dandy-Walker Syndrome. Clinical analysis of 23 cases. J Neurosurg 55:89-98 Sutton JB (1887) The lateral recess of the fourth ventricle. Their relation to certain cysts and tumors of the cerebellum and to occipital meningocele. Brain 9: 352-361 Strandgaard L (I970) The Dandy-Walker Syrldrome. A case with a patent foramen of the fourth ventricle demonstrated by encephalography. Br J Radiol 43:738-740

gested before ~0. In these cases, especially if the patient is in the elderly group, cyst excision with posterior fossa approach may be advisable if the subarachnoid space is open in dynamic studies 26. Also a lumbo-peritoneal shunt may help in further drainage if the cyst excision does not solve the problem. Instead of such major surgery we prefer cystoperitoneal and ventriculorperitoneal shunts to be placed at the same time. If the aqueduct is obstructed and the fourth ventricle is communicating with the subarachnoid space, then a ventricular shunt is sufficient. The idea of inserting cystoperitoneal catheters in all cases will not work in this type. The second major group consists of the non-communicating type. If the aqueduct is patent, drainage can be achieved with a single shunt connecting the cyst or ventricle to elesewhere. Some authors prefer ventriculoperitoneal catheters io and some cystoperitoneal catheters 16,22. If the aqueduct is occluded and there is obstruction of the fourth ventricle, then we have two different cystic cavities. One is the supratentorial ventricle and the other the cystic posterior fossa. So these two closed systems must be drained as advised by several investigators 5' 16, 21. Case number four is an example of this situation. Upward herniation is the commonly associated complication in this type when simultaneous cyst drainage is not performed or due to malfunction of the shunts in the postoperative period. On the other hand ventricular pressure will be progressively high, if only a cystoperitoneal shunt is inserted. Up to this date many varieties of Dandy-Walker Syndrome have been reported. In conclusion we want to state that in order to diagnose the type and because it is necessary to know the fluid dynamics for treatment planning, CT ventriculography plus cisternography has to be performed in all cases of so-called "Dandy-Walker Malformation".

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1. Archer CR, Darwish H, Smith K Jr (1978) Enlarged cisternae magna and fossa cysts simulating Dandy-Walker Syndrome on computed tomograph~,. Radiology 127:681-686 2. Benda CE (1954) The Dandy-Walker Syndrome or the so-called atresia of the foramen of Magendie. J Neuropath Exp Neurol 13:14-29 3. Bentson JR, Alberti J (1978) The fourth ventricle. In: Newton TH, Potts DG (eds) Radiology of skull and brain, vol 4: Ventricles and cisterns. CV Mosby, St Louis, pp 3303-3363 4. Brodal A, Hauglie-Hanssen E (1959) Congenital hydrocephalus with defective development of the cerebellar vermis (DandyWalker Syndrome). Clinical and anatomical findings in two 21.

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58 25. Taggart JK, Walker AE (1942) Congenital atresia of the foramens of Luschka and Magendie. Arch Neurol Psychiatry 48: 583-612 26. Udverhelyi GB, Epstein MH (1975) The so-called DandyWalker Syndrome. Analysis of twelve operated cases. Child's Brain 1:158-182 27. Unsgaard G, Sand T, Stovring J et al (1987) Adult manifestation of the Dandy-Walker Syndrome. Neurochirurgia 30:21-24

T. Ozgen and B. Agikg6z: CSF Dynamics in Dandy-Walker Syndrome 28. Wang AN, Carson BS (1987) Upward herniation of the posterior fossa cyst in the shunted child. Surg Neurol 28:215-220 29. Wolpert SM, Haller JS, Rabe EF (1970) The value of angiography in the Dandy-Walker Syndrome and posterior fossa extraaxial cysts. Am J Roentgen 109:261-272 Correspondence and Reprints: T. ()zgen, M.D., Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey.