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Dandy-walker cyst associated with occipital meningocele
Dandy-Walker Cyst Associated with Occipital Meningocele Tadashi Kojima, M.D., Shiro Waga, M.D., Takeo Shimizu, M.D., and Tadashi Sakakura, M.D.
Two cases of Dandy-Walker cyst associated with occipital meningocele are presented. Only 9 cases with such association have been described in the literature. In our patients computed tomography clearly demonstrated direct communication of a posterior fossa cyst with an occipital meningocele at one end and with the fourth ventricle at the other. Occipital meningocele might simply be an expression of increased intracystic pressure in DandyWalker cyst in embryonic life, or such association might suggest its morphogenetic situation during embryogenesis. Kojima T, Waga S, ShimizuT, SakakuraT: Dandy-Walkercyst associatedwith occipital meningocele. SurgNeurol 17:52-56, 1982 Hydrocephalus associated with a posterior fossa cyst and agenesis of the cerebellar vermis was first described by Sutton [24] in 1887. Dandy and Blackfan [7] in 1914 were the first to clearly demonstrate the clinical picture of this type of hydrocephalus, which they attributed to atresia of the foramina of Luschka and Magendie. Taggert and Walker [25] in 1942 summarized and reviewed the reported cases, including their own 3 cases, and coined the term congenital atresia of the foramens of Luschka and Magendie. Benda [1] in 1954 reported 6 such cases, and he renamed the disorder Dandy-Walker syndrome. Dandy-Walker syndrome has been controversial not only as to its definition and pathogenesis but in terms of its management. In this paper we use the definition proposed by Hart and colleagues [12], that of there being hydrocephalus, a partial or complete absence of the cerebellar vermis, and a posterior fossa cyst continuous with the fourth ventricle. Although a large variety of anomalies in the central nervous system have been described in cases of Dandy-Walker cyst, association with an occipital encephalocele has been rare. Recently we treated 2 patients with a Dandy-Walker cyst associated with occipital meningocele.
From the Departmentof Neurosurgery,Mie UniversityMedical School, Tsu, Mie, Japan. Address reprint requests to Dr. Tadashi Kojima, Department of Neurosurgery,Mie UniversityMedicalSchool, 2-174Edobashi, Tsu, Mie, Japan 514. Key words: Dandy-Walker cyst; encephalocele; meningocele; hydrocephalus; posteriorcranial fossa.
Case Reports
Patient 1 This 26-day-old female infant with a midline occipital mass was admitted to the hospital on November 25, 1980. She was born at full-term and the pregnancy had been uneventful. There was no family history of neurological disease. Physical and neurological examinations revealed no abnormality except a cystic mass in the occipital region. The head shape was dolichocephalic, and head circumference was 38 cm. The fontanelles were flat. Axial computed tomographic (CT) scans showed a large posterior fossa cyst that directly communicated with the fourth ventricle (Fig. 1). The cerebellar vermis was not seen. There was mild dilatation of the lateral and third ventricles. A coronal CT scan revealed the relationship of the cyst with the fourth ventricle and brain stem (Fig. 2). A direct sagittal CT scan confirmed the presence of a large posterior fossa cyst communicating with the fourth ventricle at one end and with the occipital meningocele at the other (Fig. 3). Cerebral angiography demonstrated a large posterior fossa cyst and a high torcular Herophili; the sagittal and lateral sinuses formed an inverted Y configuration. Posterior fossa exploration and repair of the occipital meningocele were performed. Operation revealed a large posterior fossa cyst that communicated directly with the occipital meningocele and the fourth ventricle. The floor of the fourth ventricle was easily seen because of the absence of the cerebellar vermis. The foramina of Luschka and Magendie could not be identified. A cystoperitoneal shunt was placed. The postoperative course was smooth and uneventful, and the patient was discharged without neurological deficit. She was readmitted to the hospital on January 19, 1981, because of enlargement of the head. CT scans confirmed a moderate hydrocephalus. A ventriculoperitoneal shunt was installed. Although she developed meningitis, it was controlled with parenteral antibiotics, and she was discharged from the hospital without any neurological deficit.
Patient 2 On August 27, 1978, a full-term female newborn was admitted to the hospital 24 hours after birth because of an occipital mass and a large head. The pregnancy had been uneventful. There was no family history of neurological disease. Examination showed a cystic mass in the occipital re-
52 0090.3019/82/010052.05501.25 © 1981 by Little, Brown and Company (Inc.)
Kojima et al: Dandy-Walker Cyst
53
Fig. 3. Patient 1. Sa~ttal C T scan shows a large posterior fossa cyst communicating with the fourth ventricle and with the occipital meningocele. The cerebellar vermis is not seen. Fig. 1. Patient 1. Axial C T scans show a large posterior fossa cyst directly communicating with the fourth ventricle. The cerebellar vermis is not seen.
Fig. 2. Patient 1. Coronal C T scans show the relationship of the cyst with the fourth ventricle, brain stem, and tentorium.
54 SurgicalNeurology Vol 17 No 1 January 1982
gion, and the head circumference was 39 cm. The fontanelles were tense and prominent. CT scans showed a large posterior fossa cyst, communicating with the fourth ventricle and the occipital meningocele (Fig. 4). The brain stem was compressed toward the clivus; the cerebellar vermis could not be seen; and the small cerebellar hemispheres were compressed and displaced laterally. A marked dilatation of the lateral and third ventricles was also seen. Cerebral angiography was consistent with a Dandy-Walker cyst associated with hydrocephalus. A ventriculoperitoneal shunt was first installed. A week later a posterior fossa ekploration and repair of the occipital meningocele were performed. At operation a Dandy-Walker cyst was found to be continuous with the fourth ventricle and an occipital meningocele. The cerebellar vermis and the foramina of Luschka and Magendie could not be identified. A cystoperitoneal shunt was also installed. The patient's postoperative course was uneventful, and she was discharged from the hospital without neurological deficit. Two and one-half years after the operation, she was in good health and CT scanning showed that the Dandy-Walker cyst was still present without hydrocephalus. Discussion
Dandy-Walker cyst is a relatively common congenital anomaly characterized by cystic enlargement of the fourth ventricle, usually in association with hypoplasia or agenesis of the cerebellar vermis and hydrocephalus. Several theories concerning the pathogenesis of Dandy-Walker cyst have been proposed, and they have been the subject of a
Fig. 4. Patient 2. Axial C T scans show a large posterior fossa cyst communicating with the fourth ventricle. The brain stem is compressed toward the clivus. The cerebellar vermis is not seen.
controversy that remains unsettled [1, 3, 5, 7, 9, 12, 15, 18, 19, 21, 23, 25]. Taggert and Walker [25] proposed that failure of the foramina of Luschka and Magendie to develop would result in cystic enlargement of the fourth ventricle with consequent failure of proper development of the vermis. Benda [1] stated that it was a more complex developmental anomaly and that it was a dysraphic disorder of the cerebellum in which the vermis failed to fuse during embryogenesis. Gardner [9, 10] stated that a Dandy-Walker cyst communicates with the fourth ventricle via the foramen of Magendie and, therefore, probably originates during embryonal life from an overdistension of Weed's area membranacea inferior. The Dandy-Walker cyst is often associated with a large variety of cerebral and systemic anomalies. Raimondi and associates [20] reported that there was a dysgenesis of the corpus callosum in more than 75% of their patients. Hart and co.workers [13] reported that a variety of anomalies in varying combinations were present in 19 of 28 cases (68%), including nonspecific cerebral gyral anomalies; specific malformations of the cerebrum in the form of heterotopia, polymicrogyria, agyria, and macrogyria; agenesis of the corpus callosum; malformations of the inferior olivary nuclei; cerebellar folial anomalies and heterotopias; aqueductal
Kojima et al: Dandy-Walker Cyst
55
Reported Cases of Dandy-Walker Cyst Associated with Encephalocele Author
Type of Encephalocele
Communication with Dandy-Walker Cyst
Confirmation
Sutton [24], 1887 Dandy [6], 1953 Schreiber [22], 1954 Nakai [16], 1962 McLaurin [14], 1964
Meningoencephalocystocele Meningocele Cystic swelling Meningoencephalocele Encephalocele Heterotopic glial rest Meningocele (?) Meningoencephalocele Meningoencephalocele Meningocele Meningocele
+ + + + + + + +
Autopsy Surgery (?) Surgery & autopsy Surgery Autopsy Autopsy Surgery & autopsy Surgery & autopsy Surgery Radiology & surgery Radiology & surgery
Raimondi [20], 1969 Joubert [131, 1969 Tsuchida [26], 1981 Kojima (present report) + = yes; - = no.
stenosis; macrocephaly; bilateral posterior fossa lipomas compressing the pons and medulla oblongata; infundibular hamartoma; diverticular cyst of the third ventricle; and syringomyelia. Sutton [24] in 1887 first described a case of so-called Dandy-Walker cyst associated with occipital meningocele. Since then such an association has been reported only rarely. We could find only 11 such cases in the literature including the present cases. The Table outlines the reported cases with such association. In 3 the cystic masses in the scalp did not communicate with a posterior fossa cyst. The masses were a cystic swelling [13], heterotopic glial rest [15], and meningoencephalocele [22]. In 8 the overlying cystic lesions communicated with a posterior fossa c y s t - meningocele in 4, meningoencephalocystocele in 2, and encephalocele and meningoencephalocete in 1 each [6, 14, 16, 20, 24, 26]. In all the published cases except those being reported here, the communication between the posterior fossa cyst and the cystic tumor in the scalp was demonstrated at operation or autopsy, or both. In our patients, C T findings were diagnostic of and consistent with Dandy-Walker cyst communicating with an occipital meningocele, and the diagnosis was clearly confirmed at operation. Since the advent of computed tomography, the relationship of a posterior fossa cyst to the fourth ventricle, the brain stem, the tentorium, and even an encephalocele has become easily demonstrable. Many conditions associated with partial or complete agenesis of the cerebellar vermis with or without hydrocephalus, and with or without occipital encephalocele, have been reported [2, 4, 8, 9, 11, 13, 17-19, 21]. They include familial agenesis of the cerebellar vermis, tectocerebellar dysrhaphia, unsegmented midbrain tectum, mega cisterna magna, and inverse cerebellum. Joubert and colleagues [13] reported 4 cases of familial agenesis of the cerebellar vermis, and in I of these cases there was com-
plete agenesis of the vermis and a large midline cavity in the posterior fossa associated with occipital meningoencephalocele. Association of inverse cerebellum with occipital encephalocele was also reported [19, 23]. Smith and Huntington [23] reported 2 cases of inverse cerebellum and occipital encephalocele and stated that inverse cerebellum with occipital encephalocele is morphogenetically situated between Arnold-Chiari malformation and Dandy-Walker cyst. Although occipital encephalocele in Dandy-Walker cyst might simply be an expression of increased intracystic pressure during embryonal life, Dandy-Walker cyst with occipital encephalocele as well as inverse cerebellum with occipital encephalocele might suggest its morphogenetic origin in embryogenesis.
References 1. Benda CE: The Dandy-Walker syndromeor the so-called atresia of the foramens of Magendie. J Neuropathol Exp Neurol 13:14-29, 1954 2. Bergstr6mK, Sauner G: Pneumoencephalography in non-progressive ataxic syndrome. A study of 26 children and adolescents. Acta Paediatr Scand 63:732-734, 1974 3. Brown JR: The Dandy-Walker syndrome, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Amsterdam, New York, Oxford: North-Holland, 1977, Vol 30 (Part I), pp 623-646 4. Calogem JA: Vermian agenesis and unsegmented midbrain tectum: case report. J Neurosurg 47:605-608, 1977 5. D'Agostino AN, Kemohan JW, Brown JR: The Dandy-Walker syndrome. J Neuropathol Exp Neurol 22:450-470, 1963 6. Dandy WE: Brain, in Lewis D (ed): Practice of Surgery. Hagerstown, MD: Prior, 1953, Vol 12, pp 202-252 7. Dandy WE, Blackfan KD: Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child 8:406-482, 1914 8. Friede RL: Uncommon syndromes of cerebellar vermis aplasia. I1: tecto-cerebellar dysraphia with occipital encephalocele. Dev Med Child Neurol 20:764-772, 1978 9. Gardner WJ: The Dystaphic States. From Syringomyelia to Anencephaly. Amsterdam: Excerpta Medica, 1973, pp 127-143, 145-166 10. Gardner WJ, Abdullah AF, McCormack LJ: Varying expressions of embryonal atresia of the fourth ventricle in adults: Amold-Chiari malformation, Dandy-Walker syndrome, "arachnoid" cyst of the cerebellum, and syringomyelia. J Neurosurg 14:591-605, 1957
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11. Gonsette R, Potvliege G, Andre-Balisaux G, Stenuit J: La m6ga grande cistene: &ude clinique, mdiologiqueet anatomopathologique. Acta Neurol Belg 68:559-570, 1968 12. Hart MN, Malamud N, Ellis WG: The Dandy-Walkersyndrome: a clinicopathological study based on 28 cases. Neurology22:771-780, 1972 13. Joubert M, Eisenring J, Robb JP, Andermann F: Familial agenesis of the cerebellar vermis: a syndrome of episodic hyperpnea, abnormal eye movement, ataxia, and retardation. Neurology19:818-825, 1969 14. McLaurin RL: Parietal cephaloceles. Neurology 14:764-772, 1964 15. Mercuri S, Curatolo P, Giuffr6 R, Di Lorenzo N: Agenesis of the vermis cerebelli and malformationsof the posterior fossa in childhood and adolescence. Neurochirurgia (Stuttg) 22:180-188, 1979 16. Nakai K, Sato M, Ishi I, Kaneko K, Takayama T, Soma Y: The Dandy-Walker cyst with occipital encephalocele: case report. Nigata Igakkai Zasshi 76:440, 1962 17. Nova HR: Familial communicatinghydrocephalus, posterior cerebellar agenesis, mega cisterna magna, and port-wine nevi: report of five members of one family. J Neurosurg 51:862-865, 1979 18. Padget DH: Development of so-called dysraphism; with embryologic evidence of clinical Arnold-Chiari and Dandy-Walker malformations. Johns Hopkins Med J 130:127-165, 1972
19. Padget DH, Lindenberg R: Inverse cerebellum morphogeneticallyrelated to Dandy-Walker and Arnold-Chiari syndromes: bizarre malformed brain with occipital encephalocele. Johns Hopkins Med J 131:228-246, 1972 20. Raimondi AJ, Samuelson G, YarzagarayL, Norton T: Atresia of the foramina of Luschka and Magendie: the Dandy-Walker cyst. J Neurosurg 31:202-216, 1969 21. Sahs AL: Congenital anomaly of the cerebellar vermis. Arch Pathol 32:52-63, 1941 22. Schreiber MS, Reye RDK: Posterior fossa cysts due to congenital atresia of the foramina of Luschka and Magendie. Med J Aust 2: 743-748, 1954 23. Smith MT, Huntington HW: Inverse cerebellum and occipital encephalocele: a dorsal fusion defect uniting the Amold-Chiari and Dandy-Walker spectrum. Neurology 27:246-251, 1977 24. Sutton JB: The lateral recessesof the fourth ventricle; their relation to certain cysts and tumours of the cerebellum, and to occipital meningocele. Brain 9:352-361, 1887 25. Taggart JK, Walker AE: Congenital atresia of the foramens of Luschka and Magendie. Arch Neurol Psychiatry43:583-612, 1942 26. Tsuchida T, Okada K, Ueki K: The prognosisof encephaloceles. No Shinkei Geka 9:143-150, 1981
Book Review Neurosecretion and Brain Peptides: Implications for Brain Functions and Neurological Disease Edited by Joseph B. Martin, Seymour Reichlin, and Katherine L. Bick, New York, Raven Press, 198 I 727 pp., $65.00 Reviewed by K. Patrick Ober, M.D., Winston-Salem, North Carolina In recent years, the separation between classical endocrinology and classical neurology has become less and less distinct, and it is now apparent that the endocrine system and neurological system are both components of the organism's overall attempt at maintaining homeostasis. This attempt at homeostasis involves communication by chemical messengers. These chemical signals may be very localized (as with a neurotransmitter crossing a synapse}, or the message conveyed may be systemic (as with hormone secretion into the general circulation). In many cases, it is no longer possible to clearly identify some compounds as strictly "neurotransmitters" or "hormones," since functions may overlap. A number of peptides originally described as hormones have been found to be present in the central nervous system, and it is apparent that these substances are acting as neurotransmitters. Neurosecretion and Brain Pept/des extensively covers recently acquired information re-
garding the synthesis, anatomic distribution, and function of these various neuropeptides. It also addresses the possibility that the pathogenesis of many neurological disorders may be better understood as we learn more about these neuropeptides; deficiencies of more classic neurotransmitters have been shown to play a significant role in the development of Parkinson's disease and Huntington's chorea, and it is possible that there are many more analogous situations involving the newly described neuropeptides. This book covers a very complex and rapidly evolving field, and much of its content is probably more than most casual readers will be able to assimilate. This book should be very useful to the basic scientist who is working in the field of neuroendocrinology, but will be of much less interest to the practicing clinician. Of some clinical interest are chapters on the possible role of opioid peptides in pain inhibition and seizures, the role of nerve growth factor for the survival of peripheral neurons, the potential role of neuropeptides in genetic disorders of the central nervous system, the implications of these peptides in neurological disease, and the use of peptide analogs as pharmacological modulators of brain function. Although probably few physicians would find this text a worthwhile addition to their personal library, selected sections of the book would be of great interest to most individuals who are working in the areas of clinical neurology, neurosurgery, endocrinology, or psychiatry.
Two cases of Dandy-Walker cyst associated with occipital meningocele are presented. Only 9 cases with such association have been described in the literature. In our patients computed tomography clearly demonstrated direct communication of a posterior fossa cyst with an occipital meningocele at one end and with the fourth ventricle at the other. Occipital meningocele might simply be an expression of increased intracystic pressure in DandyWalker cyst in embryonic life, or such association might suggest its morphogenetic situation during embryogenesis. Kojima T, Waga S, ShimizuT, SakakuraT: Dandy-Walkercyst associatedwith occipital meningocele. SurgNeurol 17:52-56, 1982 Hydrocephalus associated with a posterior fossa cyst and agenesis of the cerebellar vermis was first described by Sutton [24] in 1887. Dandy and Blackfan [7] in 1914 were the first to clearly demonstrate the clinical picture of this type of hydrocephalus, which they attributed to atresia of the foramina of Luschka and Magendie. Taggert and Walker [25] in 1942 summarized and reviewed the reported cases, including their own 3 cases, and coined the term congenital atresia of the foramens of Luschka and Magendie. Benda [1] in 1954 reported 6 such cases, and he renamed the disorder Dandy-Walker syndrome. Dandy-Walker syndrome has been controversial not only as to its definition and pathogenesis but in terms of its management. In this paper we use the definition proposed by Hart and colleagues [12], that of there being hydrocephalus, a partial or complete absence of the cerebellar vermis, and a posterior fossa cyst continuous with the fourth ventricle. Although a large variety of anomalies in the central nervous system have been described in cases of Dandy-Walker cyst, association with an occipital encephalocele has been rare. Recently we treated 2 patients with a Dandy-Walker cyst associated with occipital meningocele.
From the Departmentof Neurosurgery,Mie UniversityMedical School, Tsu, Mie, Japan. Address reprint requests to Dr. Tadashi Kojima, Department of Neurosurgery,Mie UniversityMedicalSchool, 2-174Edobashi, Tsu, Mie, Japan 514. Key words: Dandy-Walker cyst; encephalocele; meningocele; hydrocephalus; posteriorcranial fossa.
Case Reports
Patient 1 This 26-day-old female infant with a midline occipital mass was admitted to the hospital on November 25, 1980. She was born at full-term and the pregnancy had been uneventful. There was no family history of neurological disease. Physical and neurological examinations revealed no abnormality except a cystic mass in the occipital region. The head shape was dolichocephalic, and head circumference was 38 cm. The fontanelles were flat. Axial computed tomographic (CT) scans showed a large posterior fossa cyst that directly communicated with the fourth ventricle (Fig. 1). The cerebellar vermis was not seen. There was mild dilatation of the lateral and third ventricles. A coronal CT scan revealed the relationship of the cyst with the fourth ventricle and brain stem (Fig. 2). A direct sagittal CT scan confirmed the presence of a large posterior fossa cyst communicating with the fourth ventricle at one end and with the occipital meningocele at the other (Fig. 3). Cerebral angiography demonstrated a large posterior fossa cyst and a high torcular Herophili; the sagittal and lateral sinuses formed an inverted Y configuration. Posterior fossa exploration and repair of the occipital meningocele were performed. Operation revealed a large posterior fossa cyst that communicated directly with the occipital meningocele and the fourth ventricle. The floor of the fourth ventricle was easily seen because of the absence of the cerebellar vermis. The foramina of Luschka and Magendie could not be identified. A cystoperitoneal shunt was placed. The postoperative course was smooth and uneventful, and the patient was discharged without neurological deficit. She was readmitted to the hospital on January 19, 1981, because of enlargement of the head. CT scans confirmed a moderate hydrocephalus. A ventriculoperitoneal shunt was installed. Although she developed meningitis, it was controlled with parenteral antibiotics, and she was discharged from the hospital without any neurological deficit.
Patient 2 On August 27, 1978, a full-term female newborn was admitted to the hospital 24 hours after birth because of an occipital mass and a large head. The pregnancy had been uneventful. There was no family history of neurological disease. Examination showed a cystic mass in the occipital re-
52 0090.3019/82/010052.05501.25 © 1981 by Little, Brown and Company (Inc.)
Kojima et al: Dandy-Walker Cyst
53
Fig. 3. Patient 1. Sa~ttal C T scan shows a large posterior fossa cyst communicating with the fourth ventricle and with the occipital meningocele. The cerebellar vermis is not seen. Fig. 1. Patient 1. Axial C T scans show a large posterior fossa cyst directly communicating with the fourth ventricle. The cerebellar vermis is not seen.
Fig. 2. Patient 1. Coronal C T scans show the relationship of the cyst with the fourth ventricle, brain stem, and tentorium.
54 SurgicalNeurology Vol 17 No 1 January 1982
gion, and the head circumference was 39 cm. The fontanelles were tense and prominent. CT scans showed a large posterior fossa cyst, communicating with the fourth ventricle and the occipital meningocele (Fig. 4). The brain stem was compressed toward the clivus; the cerebellar vermis could not be seen; and the small cerebellar hemispheres were compressed and displaced laterally. A marked dilatation of the lateral and third ventricles was also seen. Cerebral angiography was consistent with a Dandy-Walker cyst associated with hydrocephalus. A ventriculoperitoneal shunt was first installed. A week later a posterior fossa ekploration and repair of the occipital meningocele were performed. At operation a Dandy-Walker cyst was found to be continuous with the fourth ventricle and an occipital meningocele. The cerebellar vermis and the foramina of Luschka and Magendie could not be identified. A cystoperitoneal shunt was also installed. The patient's postoperative course was uneventful, and she was discharged from the hospital without neurological deficit. Two and one-half years after the operation, she was in good health and CT scanning showed that the Dandy-Walker cyst was still present without hydrocephalus. Discussion
Dandy-Walker cyst is a relatively common congenital anomaly characterized by cystic enlargement of the fourth ventricle, usually in association with hypoplasia or agenesis of the cerebellar vermis and hydrocephalus. Several theories concerning the pathogenesis of Dandy-Walker cyst have been proposed, and they have been the subject of a
Fig. 4. Patient 2. Axial C T scans show a large posterior fossa cyst communicating with the fourth ventricle. The brain stem is compressed toward the clivus. The cerebellar vermis is not seen.
controversy that remains unsettled [1, 3, 5, 7, 9, 12, 15, 18, 19, 21, 23, 25]. Taggert and Walker [25] proposed that failure of the foramina of Luschka and Magendie to develop would result in cystic enlargement of the fourth ventricle with consequent failure of proper development of the vermis. Benda [1] stated that it was a more complex developmental anomaly and that it was a dysraphic disorder of the cerebellum in which the vermis failed to fuse during embryogenesis. Gardner [9, 10] stated that a Dandy-Walker cyst communicates with the fourth ventricle via the foramen of Magendie and, therefore, probably originates during embryonal life from an overdistension of Weed's area membranacea inferior. The Dandy-Walker cyst is often associated with a large variety of cerebral and systemic anomalies. Raimondi and associates [20] reported that there was a dysgenesis of the corpus callosum in more than 75% of their patients. Hart and co.workers [13] reported that a variety of anomalies in varying combinations were present in 19 of 28 cases (68%), including nonspecific cerebral gyral anomalies; specific malformations of the cerebrum in the form of heterotopia, polymicrogyria, agyria, and macrogyria; agenesis of the corpus callosum; malformations of the inferior olivary nuclei; cerebellar folial anomalies and heterotopias; aqueductal
Kojima et al: Dandy-Walker Cyst
55
Reported Cases of Dandy-Walker Cyst Associated with Encephalocele Author
Type of Encephalocele
Communication with Dandy-Walker Cyst
Confirmation
Sutton [24], 1887 Dandy [6], 1953 Schreiber [22], 1954 Nakai [16], 1962 McLaurin [14], 1964
Meningoencephalocystocele Meningocele Cystic swelling Meningoencephalocele Encephalocele Heterotopic glial rest Meningocele (?) Meningoencephalocele Meningoencephalocele Meningocele Meningocele
+ + + + + + + +
Autopsy Surgery (?) Surgery & autopsy Surgery Autopsy Autopsy Surgery & autopsy Surgery & autopsy Surgery Radiology & surgery Radiology & surgery
Raimondi [20], 1969 Joubert [131, 1969 Tsuchida [26], 1981 Kojima (present report) + = yes; - = no.
stenosis; macrocephaly; bilateral posterior fossa lipomas compressing the pons and medulla oblongata; infundibular hamartoma; diverticular cyst of the third ventricle; and syringomyelia. Sutton [24] in 1887 first described a case of so-called Dandy-Walker cyst associated with occipital meningocele. Since then such an association has been reported only rarely. We could find only 11 such cases in the literature including the present cases. The Table outlines the reported cases with such association. In 3 the cystic masses in the scalp did not communicate with a posterior fossa cyst. The masses were a cystic swelling [13], heterotopic glial rest [15], and meningoencephalocele [22]. In 8 the overlying cystic lesions communicated with a posterior fossa c y s t - meningocele in 4, meningoencephalocystocele in 2, and encephalocele and meningoencephalocete in 1 each [6, 14, 16, 20, 24, 26]. In all the published cases except those being reported here, the communication between the posterior fossa cyst and the cystic tumor in the scalp was demonstrated at operation or autopsy, or both. In our patients, C T findings were diagnostic of and consistent with Dandy-Walker cyst communicating with an occipital meningocele, and the diagnosis was clearly confirmed at operation. Since the advent of computed tomography, the relationship of a posterior fossa cyst to the fourth ventricle, the brain stem, the tentorium, and even an encephalocele has become easily demonstrable. Many conditions associated with partial or complete agenesis of the cerebellar vermis with or without hydrocephalus, and with or without occipital encephalocele, have been reported [2, 4, 8, 9, 11, 13, 17-19, 21]. They include familial agenesis of the cerebellar vermis, tectocerebellar dysrhaphia, unsegmented midbrain tectum, mega cisterna magna, and inverse cerebellum. Joubert and colleagues [13] reported 4 cases of familial agenesis of the cerebellar vermis, and in I of these cases there was com-
plete agenesis of the vermis and a large midline cavity in the posterior fossa associated with occipital meningoencephalocele. Association of inverse cerebellum with occipital encephalocele was also reported [19, 23]. Smith and Huntington [23] reported 2 cases of inverse cerebellum and occipital encephalocele and stated that inverse cerebellum with occipital encephalocele is morphogenetically situated between Arnold-Chiari malformation and Dandy-Walker cyst. Although occipital encephalocele in Dandy-Walker cyst might simply be an expression of increased intracystic pressure during embryonal life, Dandy-Walker cyst with occipital encephalocele as well as inverse cerebellum with occipital encephalocele might suggest its morphogenetic origin in embryogenesis.
References 1. Benda CE: The Dandy-Walker syndromeor the so-called atresia of the foramens of Magendie. J Neuropathol Exp Neurol 13:14-29, 1954 2. Bergstr6mK, Sauner G: Pneumoencephalography in non-progressive ataxic syndrome. A study of 26 children and adolescents. Acta Paediatr Scand 63:732-734, 1974 3. Brown JR: The Dandy-Walker syndrome, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Amsterdam, New York, Oxford: North-Holland, 1977, Vol 30 (Part I), pp 623-646 4. Calogem JA: Vermian agenesis and unsegmented midbrain tectum: case report. J Neurosurg 47:605-608, 1977 5. D'Agostino AN, Kemohan JW, Brown JR: The Dandy-Walker syndrome. J Neuropathol Exp Neurol 22:450-470, 1963 6. Dandy WE: Brain, in Lewis D (ed): Practice of Surgery. Hagerstown, MD: Prior, 1953, Vol 12, pp 202-252 7. Dandy WE, Blackfan KD: Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child 8:406-482, 1914 8. Friede RL: Uncommon syndromes of cerebellar vermis aplasia. I1: tecto-cerebellar dysraphia with occipital encephalocele. Dev Med Child Neurol 20:764-772, 1978 9. Gardner WJ: The Dystaphic States. From Syringomyelia to Anencephaly. Amsterdam: Excerpta Medica, 1973, pp 127-143, 145-166 10. Gardner WJ, Abdullah AF, McCormack LJ: Varying expressions of embryonal atresia of the fourth ventricle in adults: Amold-Chiari malformation, Dandy-Walker syndrome, "arachnoid" cyst of the cerebellum, and syringomyelia. J Neurosurg 14:591-605, 1957
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Book Review Neurosecretion and Brain Peptides: Implications for Brain Functions and Neurological Disease Edited by Joseph B. Martin, Seymour Reichlin, and Katherine L. Bick, New York, Raven Press, 198 I 727 pp., $65.00 Reviewed by K. Patrick Ober, M.D., Winston-Salem, North Carolina In recent years, the separation between classical endocrinology and classical neurology has become less and less distinct, and it is now apparent that the endocrine system and neurological system are both components of the organism's overall attempt at maintaining homeostasis. This attempt at homeostasis involves communication by chemical messengers. These chemical signals may be very localized (as with a neurotransmitter crossing a synapse}, or the message conveyed may be systemic (as with hormone secretion into the general circulation). In many cases, it is no longer possible to clearly identify some compounds as strictly "neurotransmitters" or "hormones," since functions may overlap. A number of peptides originally described as hormones have been found to be present in the central nervous system, and it is apparent that these substances are acting as neurotransmitters. Neurosecretion and Brain Pept/des extensively covers recently acquired information re-
garding the synthesis, anatomic distribution, and function of these various neuropeptides. It also addresses the possibility that the pathogenesis of many neurological disorders may be better understood as we learn more about these neuropeptides; deficiencies of more classic neurotransmitters have been shown to play a significant role in the development of Parkinson's disease and Huntington's chorea, and it is possible that there are many more analogous situations involving the newly described neuropeptides. This book covers a very complex and rapidly evolving field, and much of its content is probably more than most casual readers will be able to assimilate. This book should be very useful to the basic scientist who is working in the field of neuroendocrinology, but will be of much less interest to the practicing clinician. Of some clinical interest are chapters on the possible role of opioid peptides in pain inhibition and seizures, the role of nerve growth factor for the survival of peripheral neurons, the potential role of neuropeptides in genetic disorders of the central nervous system, the implications of these peptides in neurological disease, and the use of peptide analogs as pharmacological modulators of brain function. Although probably few physicians would find this text a worthwhile addition to their personal library, selected sections of the book would be of great interest to most individuals who are working in the areas of clinical neurology, neurosurgery, endocrinology, or psychiatry.