- Email: [email protected]
Congenital Hydrocephalus Associated with Anomalies of Midline Telencephalic Structures
Path. Res. Pract. 187, 939-942 (1991)
Congenital Hydrocephalus Associated with Anomalies of Midline Telencephalic Structures A Case Report H. Koo and K. Y. Lee 1 Departments of Pathology and Obstetrics and IGynecology, College of Medicine, Hallym University, Seoul, Korea
Je G. Chi Department of Pathology, Seoul National University, College of Medicine, Seoul Korea
SUMMARY
Hydrocephalus is classified into communicating, if cerebrospinal fluid (CSF) can flow freely from the ventricle to the subarachnoid space, and into non-communicating, if it cannot. The cause of hydrocephalus is diverse, either congenital or acquired conditions causing obstruction to the flow of CSF. The location of the lesion is more important than the size or nature of the lesion. We describe an unusual case of congenital communicating hydrocephalus associated with multiple malformations of midline telencephalic structures. This was a premature male baby of 30 weeks gestational age. The pregnancy was terminated after revealing a hydrocephalus by ultrasonography. The cerebral hemispheres showed marked thinning of parenchyme with dilated lateral ventricles, communication of lateral and third ventricles with dilated foramina of Monro, absence of the septum pellucidum, and hypoplasia or focal agenesis of posterior portion of corpus callosum with dorsal dilation of the third ventricle. The right fornix appeared as a single thick midline cord in its approximately normal position and the hippocampi were poorly developed, especially in the left side. The left fornix was rudimentary.
Introduction Midline telencephalic structures include cerebral commissures, septal area, primordium hippocampi, septum pellucidum, and cavum septi. Developmental defects in these structures include holoprosencephaly, complete and partial agenesis of corpus callosum, agenesis of septum pellucidum, and absence of anterior commissure. These anomalies occur in varying degree and present as a single manifestation or as a combination with other malformations. Hydrocephalus associated with these malformations are congenital and either communicating or noncommu© 1991 by Gustav fischer Verlag, Stuttgart
nicating type. We experienced an unusual case of congenital hydrocephalus associated with several developmental defects of midline telencephalic structures. In this paper, the case is presented with brief review of embryonal development of these structures and review of the literatures. Case Report This premature male baby was born by vaginal delivery to a 28-year-old G 1 PO Korean female ar 30 \\ccks 0344-03381::1 110 187-0939$3.50/0
940 . H. Koo, K. Y. Lee and Je G. Chi gestation with intravenous oxytocin infusion. The pregnancy was terminated after revealing a hydrocephalus by ultrasonography. Autopsy revealed a male baby weighing 1800 g. The head circumference was 34 cm and chest and abdominal circumferences 25 cm apiece. There was a skin defect on the left scrotum with herniated testis. No other congenital malformations were noted on external examination. Examination of internal organs revealed a bilobed right lung and petechial hemorrhages on the right pleura, heart and thymus. The brain weighed 310 g and 15 g of supratentorial and infratentorial respectively. The cerebral hemispheres measured 11.3 cm, 9 cm, and 7.8 cm in anteroposterior, biparietal and height respectively. The cerebellum measured 2.3 cm, 4 cm, and 2.3 cm in anteroposterior, biparietal and height respectively. The cerebral hemispheres showed diffuse flattening of gyri, especially in occipital and temporal lobes (Fig. 1). The cerebellar hemispheres were smaller than normal and showed upward herniation (Fig. 2). The cranial nerves were unremarkable. After coronal sectioning, the cerebral hemispheres showed a marked thinning of parenchyma with distended ventricles. The mantle measured up to 2 cm in thickness. The lateral and third ventricles were communicating with markedly dilated foramina of Monro (Fig. 3). The septum pellucidum was absent. The corpus callosum was relatively well preserved in anterior portion (Fig. 3), although it is markedly attenuated or focally absent in posterior portion, which measures 0.8 cm and 0.1 cm in
Fig. 1. The base of the brain shows flattening of the gyri and an
ovoid mass formation (arrow) between olfactory tracts and in front of the optic chiasm.
Fig. 2. The cerebellum is smaller than normal and shows upward herniation of cerebellar hemisphere (Arrows represent the level of incisura tentori).
Fig. 3. Coronal section through the diencephalon at the level of infundibulum (posterior surface). The third and lateral ventricles are communicating with markedly dilated foramina of Monro. The corpus callosum is relatively well preserved and septum pellucidum is absent. The anterior portion of third ventricle is not dilated below the fornix and anterior commissure. The right
fornix appears as a single thick midline cord and is devided into two columns of postcommissural fornix (arrow).
Congenital Hydrocephalus . 941
thickness respectively (Figs. 4 and 5). Average width of body of corpus callosum at 30 weeks gestational age is 1.1 em. The third ventricle showed marked dorsal dilatation with thin membranous roof. The Probst's bundles were not well-defined, but there were thick areas laterally along the roof of the third ventricle, which could be poorly formed ones (Fig. 5). The cingulate gyri were poorly developed. The right fornix appeared as a single thick midline cord to the level of dividing point of anterior columns of the fornix caudal to the rather well-developed anterior commissure (Fig. 3). The connection between these postcommissural fornix and mammillary bodies was not well-defined and the mammillary bodies were poorly formed. The hippocampi were small and hypoplastic, more on the left side (Fig. 5). The left fornix was attached to the ventricular surface of the corpus callosum as a rudimentary structure (Figs. 2 and 5). There was an ovoid mass in front of the optic chiasm and between the olfactory tract, measuring 7 mm in diameter (Fig. 1). The nature of this mass was not clear but thought to be a part of septal nuclei as a destination of precommissural fornix.
Fig. 4. Coronal section through posterior thalamus (posterior surface). The corpus callosum is thin, 0.1 em in thickness. The lateral and third ventricles are communicating with dorsal dilatation of third ventricle. The hippocampi are poorly formed.
Fig. 5. Coronal section through posterior parietal lobe (posterior surface). The corpus callosum is markedly attenuated as a thin membrane with two thick longitudinal bundles mimiking Probst's bundles (arrows). The right fornix (arrowhead) is forming a single thick midline cord. The left fornix is rudimentary.
Discussion HisS first named the dorsal part of anterior telencephalic wall as lamina reuniens and made a distinction from lamina terminalis. Ventral part of lamina reuniens is the region in which the area precommissuralis, prospective septal area with its nuclei (septal nuclei and prospective paleocortex), and anterior commissure develop. Dorsal part of lamina reuniens produces primordium hippocampi, and eventually fornix, hippocampal commissure, corpus callosum, septum pellucidum, and cavum septi are developed 6,8,14. At 9-10 weeks of fetal stage, lamina reuniens is distinct from thin lamina term ina lis, and anlage of anterior commissure appears in the ventral part of lamina reuniens (area precommissuralis). Dorsal part shows fornix bundles growing and prim odium hippocampi becomes distinct. At the same time, the banks of median groove (sulcus medianus telencephali medii, SMTM) fuse into massa commissuralis (commissural plate) in the floor of interhemispheric fissure in the region of prospective primordium hippocampi. During 10-11 weeks, hippocampal commissure appears between primordium of fornix in primordium hippocampi. Anlage of corpus callosum appears at 11-12 weeks (50-60 mm stage) in massa commissuralis and penetrates hippocampal primordia dorsal to the hippocampal commissure. The corpus callosum expands posteriorly and the hippocampal commissure shifts with it. During 14-16 weeks, corpus callosum begins to flex anteroventrally over the pocket of the SMTM, which is open until the fibers of rostrum grow through massa commissuralis and seal the pocket into closed cavum septi (16-20 weeks). The remnant of allocortex above the corpus callosum persists as hippocampal rudiment and fragment below it as a subcallosal gray rudiment and septum pellucidum, in which the fornices are embedded. Accordingly, development of septum pellucidum and
942 . H. Koo, K. Y. Lee and Je G. Chi
cavum septi is bound with the events preceding and following the formation of massa commissura lis and commissuration of the hemispheric vesicles by the fibers of prospective corpus callosum through the primordium hippocampi. Among various malformations of these midline telencephalic structures, agenesis of corpus callosum has been most frequently described in the literature since it was first described by Reil in 18122,3,7,9,10,11. It may be totally or partially absent or hypoplastic in an otherwise normal brain or it appears as a part of complex malformations, such as holoprosencephaly with absence of the anterior commissure, heterotopias, Dandy-Walker syndrome, anomalies in cerebral cortical architecture, polymicrogyria, porencephaly, or hydrocephalus. In partial agenesis, the rostrum and genu are usually intact while splenium and variable portions of the body are absent, although some cases of anterior agenesis have been reported 15 • Most of the cases occur sporadic, but familial cases have been reported 12,13. Experimentally, radiation of pregnant rat or mouse during 11-21 day of gestation can cause agenesis of corpus callosum and other cerebral malformations 4 . Agenesis is usually associated with abnormal gyral pattern of perpendicular arrangement and poorly developed cingulum. These radial sulci are considered as an altered growth pattern related to absence of the corpus callosum. Invariable dorsal dilatation of the third ventricle present in agenesis of corpus callosum also results from failure of the diencephalic roof to be limited dorsally by the corpus callosum, and the third ventricle maintains its primitive form. Probst's bundle usually runs in the apex of the lateral ventricles or in the roof, near the interhemispheric fissure. Hippocampi are always present in temporal lobes in agenesis of corpus callosum and the fornices are also present although frequently malpositioned9 . Loeser and Alvord 10 presented 12 autopsy cases with thorough review of pathogenesis. They considered the agenesis of corpus callosum as one manifestation of dorsal midline dysgenesis, closely related to but less severe than holoprosencephaly. They showed the schematic presentation of the forms of ventricles in agenesis of corpus callosum. At the level of foramen of Monro, the lateral and third ventricles are extensively communicated with great elongation of foramen of Monro. The hippocampal system remains adjacent to the third ventricle roof and there is no medial wall of the ventricle. The present case showed similar overall pattern of ventricles at the level of foramen of Monro, except for the presence of well-developed corpus callosum, absence of septum pellucidum, and malformed fornices (Fig. 3). At the level posterior to foramen of Monro, the medial walls consist of fornix-hippocampal system and Probst's bundle-cingulate gyrus complex. In
comparison, the present case showed diffuse wide communication between lateral and third ventricles at this level. It could be well explained by poor development of hippocampal system and Probst's bundle-cingulate gyrus complex (Figs. 4 and 5). Hydrocephalus, not infrequently associated with agenesis of corpus callosum, is of varying type, communicating and non-communicating. The pathogenesis could not be ascertained in most of the cases. Since the anterior commissure is largely well-developed in this case, main malformations seemed to be occurring in the structures arising from dorsal portion of lamina reuniens, probably during gestational age 9-12 weeks. References 1 AicardiJ, Goutieres F (1981) The syndrome of absence of the septum pellucidum with porencephalies and other developmental defects. Neuropadiatrie 12: 319-329 2 De Morsier G, Mozer 11 (1935) Agenesie complete dela commissure calleuse et troubles du developpement de l'hemisphere gauche avec hemiparesie droite et intetrite mentale (Le syndrome embryonnaire precoce de l'artere cerebrale anterieure.) Schweizer Archiv fiir Neurologie und Psychiatrie 35: 64-95 3 Field M, Ashton R, White K (1978) Agenesis of the corpus callosum; report of two pre-school children and review of the literature. Dev Med Child Neur 20: 47-61 4 Hicks SP (1954) Developmental malformations produced by radiation. Am J Roentgenol 69: 272-293 5 His W (1904) Die Entwicklung des mensch lichen Gehirns wah rend der ersten Monate. pp. 176, Hirzel S (Ed), Leipzig 6 Hochstetter F (1929) Beitrage zur Entwicklungsgeschichte des menschlichen Gehirns. pp. 170, Deuticke F (Ed), Wien-Leipzig 7 Jellinger K, Gross H (1973) Congenital telencephalic midline defect. Neuropadiatrie 4: 446-452 8 Johnston JB (1913) The morphology of the septum, hippocampus, and pallial commissures in reptiles and mammals. J Comp Neurol23: 371-478 9 Loeser JD, Alvord EC (1968(a)) Clinicopathological correlation in agenesis of the corpus callosum. Neurology 18: 745-756 10 Loeser JD, Alvord EC (1968(b)) Agenesis of the corpus callosum. Brain 91: 553-570 11 Marburg 0 (1949) So-called agenesis of the corpus callosum (callosal defect). Arch Neurol Psychiat 61: 297-312 12 Menkes JH, Philippart M, Clark DE (1964) Hereditary partial agenesis of the corpus callosum. Arch Neurol 11: 198-208 13 Naiman J, Frazer FC (1955) Agenesis of the corpus callosum. Arch Neurol Psychiat 74: 182-185 14 Rakic P, Yakovlev PI (1973) Development of the corpus callosum and cavum septi in man. J Comp Neurol 152: 103-132 15 Zingesser L, Schecter M, Gonatas N, Levy A, Wisoff H (1964) Agenesis of the corpus callosum associated with an interhemispheric arachnoid cysts. Brit J Radiol 37: 905-909
Received September 26, 1990 . Accepted December 4, 1990
Key words: Congenital hydrocephalus - Malformation of the midline telencephalic structures - Fornix anomaly Dr. Je G. Chi, Department of Pathology, Seoul National University Children's Hospital, 28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea
Congenital Hydrocephalus Associated with Anomalies of Midline Telencephalic Structures A Case Report H. Koo and K. Y. Lee 1 Departments of Pathology and Obstetrics and IGynecology, College of Medicine, Hallym University, Seoul, Korea
Je G. Chi Department of Pathology, Seoul National University, College of Medicine, Seoul Korea
SUMMARY
Hydrocephalus is classified into communicating, if cerebrospinal fluid (CSF) can flow freely from the ventricle to the subarachnoid space, and into non-communicating, if it cannot. The cause of hydrocephalus is diverse, either congenital or acquired conditions causing obstruction to the flow of CSF. The location of the lesion is more important than the size or nature of the lesion. We describe an unusual case of congenital communicating hydrocephalus associated with multiple malformations of midline telencephalic structures. This was a premature male baby of 30 weeks gestational age. The pregnancy was terminated after revealing a hydrocephalus by ultrasonography. The cerebral hemispheres showed marked thinning of parenchyme with dilated lateral ventricles, communication of lateral and third ventricles with dilated foramina of Monro, absence of the septum pellucidum, and hypoplasia or focal agenesis of posterior portion of corpus callosum with dorsal dilation of the third ventricle. The right fornix appeared as a single thick midline cord in its approximately normal position and the hippocampi were poorly developed, especially in the left side. The left fornix was rudimentary.
Introduction Midline telencephalic structures include cerebral commissures, septal area, primordium hippocampi, septum pellucidum, and cavum septi. Developmental defects in these structures include holoprosencephaly, complete and partial agenesis of corpus callosum, agenesis of septum pellucidum, and absence of anterior commissure. These anomalies occur in varying degree and present as a single manifestation or as a combination with other malformations. Hydrocephalus associated with these malformations are congenital and either communicating or noncommu© 1991 by Gustav fischer Verlag, Stuttgart
nicating type. We experienced an unusual case of congenital hydrocephalus associated with several developmental defects of midline telencephalic structures. In this paper, the case is presented with brief review of embryonal development of these structures and review of the literatures. Case Report This premature male baby was born by vaginal delivery to a 28-year-old G 1 PO Korean female ar 30 \\ccks 0344-03381::1 110 187-0939$3.50/0
940 . H. Koo, K. Y. Lee and Je G. Chi gestation with intravenous oxytocin infusion. The pregnancy was terminated after revealing a hydrocephalus by ultrasonography. Autopsy revealed a male baby weighing 1800 g. The head circumference was 34 cm and chest and abdominal circumferences 25 cm apiece. There was a skin defect on the left scrotum with herniated testis. No other congenital malformations were noted on external examination. Examination of internal organs revealed a bilobed right lung and petechial hemorrhages on the right pleura, heart and thymus. The brain weighed 310 g and 15 g of supratentorial and infratentorial respectively. The cerebral hemispheres measured 11.3 cm, 9 cm, and 7.8 cm in anteroposterior, biparietal and height respectively. The cerebellum measured 2.3 cm, 4 cm, and 2.3 cm in anteroposterior, biparietal and height respectively. The cerebral hemispheres showed diffuse flattening of gyri, especially in occipital and temporal lobes (Fig. 1). The cerebellar hemispheres were smaller than normal and showed upward herniation (Fig. 2). The cranial nerves were unremarkable. After coronal sectioning, the cerebral hemispheres showed a marked thinning of parenchyma with distended ventricles. The mantle measured up to 2 cm in thickness. The lateral and third ventricles were communicating with markedly dilated foramina of Monro (Fig. 3). The septum pellucidum was absent. The corpus callosum was relatively well preserved in anterior portion (Fig. 3), although it is markedly attenuated or focally absent in posterior portion, which measures 0.8 cm and 0.1 cm in
Fig. 1. The base of the brain shows flattening of the gyri and an
ovoid mass formation (arrow) between olfactory tracts and in front of the optic chiasm.
Fig. 2. The cerebellum is smaller than normal and shows upward herniation of cerebellar hemisphere (Arrows represent the level of incisura tentori).
Fig. 3. Coronal section through the diencephalon at the level of infundibulum (posterior surface). The third and lateral ventricles are communicating with markedly dilated foramina of Monro. The corpus callosum is relatively well preserved and septum pellucidum is absent. The anterior portion of third ventricle is not dilated below the fornix and anterior commissure. The right
fornix appears as a single thick midline cord and is devided into two columns of postcommissural fornix (arrow).
Congenital Hydrocephalus . 941
thickness respectively (Figs. 4 and 5). Average width of body of corpus callosum at 30 weeks gestational age is 1.1 em. The third ventricle showed marked dorsal dilatation with thin membranous roof. The Probst's bundles were not well-defined, but there were thick areas laterally along the roof of the third ventricle, which could be poorly formed ones (Fig. 5). The cingulate gyri were poorly developed. The right fornix appeared as a single thick midline cord to the level of dividing point of anterior columns of the fornix caudal to the rather well-developed anterior commissure (Fig. 3). The connection between these postcommissural fornix and mammillary bodies was not well-defined and the mammillary bodies were poorly formed. The hippocampi were small and hypoplastic, more on the left side (Fig. 5). The left fornix was attached to the ventricular surface of the corpus callosum as a rudimentary structure (Figs. 2 and 5). There was an ovoid mass in front of the optic chiasm and between the olfactory tract, measuring 7 mm in diameter (Fig. 1). The nature of this mass was not clear but thought to be a part of septal nuclei as a destination of precommissural fornix.
Fig. 4. Coronal section through posterior thalamus (posterior surface). The corpus callosum is thin, 0.1 em in thickness. The lateral and third ventricles are communicating with dorsal dilatation of third ventricle. The hippocampi are poorly formed.
Fig. 5. Coronal section through posterior parietal lobe (posterior surface). The corpus callosum is markedly attenuated as a thin membrane with two thick longitudinal bundles mimiking Probst's bundles (arrows). The right fornix (arrowhead) is forming a single thick midline cord. The left fornix is rudimentary.
Discussion HisS first named the dorsal part of anterior telencephalic wall as lamina reuniens and made a distinction from lamina terminalis. Ventral part of lamina reuniens is the region in which the area precommissuralis, prospective septal area with its nuclei (septal nuclei and prospective paleocortex), and anterior commissure develop. Dorsal part of lamina reuniens produces primordium hippocampi, and eventually fornix, hippocampal commissure, corpus callosum, septum pellucidum, and cavum septi are developed 6,8,14. At 9-10 weeks of fetal stage, lamina reuniens is distinct from thin lamina term ina lis, and anlage of anterior commissure appears in the ventral part of lamina reuniens (area precommissuralis). Dorsal part shows fornix bundles growing and prim odium hippocampi becomes distinct. At the same time, the banks of median groove (sulcus medianus telencephali medii, SMTM) fuse into massa commissuralis (commissural plate) in the floor of interhemispheric fissure in the region of prospective primordium hippocampi. During 10-11 weeks, hippocampal commissure appears between primordium of fornix in primordium hippocampi. Anlage of corpus callosum appears at 11-12 weeks (50-60 mm stage) in massa commissuralis and penetrates hippocampal primordia dorsal to the hippocampal commissure. The corpus callosum expands posteriorly and the hippocampal commissure shifts with it. During 14-16 weeks, corpus callosum begins to flex anteroventrally over the pocket of the SMTM, which is open until the fibers of rostrum grow through massa commissuralis and seal the pocket into closed cavum septi (16-20 weeks). The remnant of allocortex above the corpus callosum persists as hippocampal rudiment and fragment below it as a subcallosal gray rudiment and septum pellucidum, in which the fornices are embedded. Accordingly, development of septum pellucidum and
942 . H. Koo, K. Y. Lee and Je G. Chi
cavum septi is bound with the events preceding and following the formation of massa commissura lis and commissuration of the hemispheric vesicles by the fibers of prospective corpus callosum through the primordium hippocampi. Among various malformations of these midline telencephalic structures, agenesis of corpus callosum has been most frequently described in the literature since it was first described by Reil in 18122,3,7,9,10,11. It may be totally or partially absent or hypoplastic in an otherwise normal brain or it appears as a part of complex malformations, such as holoprosencephaly with absence of the anterior commissure, heterotopias, Dandy-Walker syndrome, anomalies in cerebral cortical architecture, polymicrogyria, porencephaly, or hydrocephalus. In partial agenesis, the rostrum and genu are usually intact while splenium and variable portions of the body are absent, although some cases of anterior agenesis have been reported 15 • Most of the cases occur sporadic, but familial cases have been reported 12,13. Experimentally, radiation of pregnant rat or mouse during 11-21 day of gestation can cause agenesis of corpus callosum and other cerebral malformations 4 . Agenesis is usually associated with abnormal gyral pattern of perpendicular arrangement and poorly developed cingulum. These radial sulci are considered as an altered growth pattern related to absence of the corpus callosum. Invariable dorsal dilatation of the third ventricle present in agenesis of corpus callosum also results from failure of the diencephalic roof to be limited dorsally by the corpus callosum, and the third ventricle maintains its primitive form. Probst's bundle usually runs in the apex of the lateral ventricles or in the roof, near the interhemispheric fissure. Hippocampi are always present in temporal lobes in agenesis of corpus callosum and the fornices are also present although frequently malpositioned9 . Loeser and Alvord 10 presented 12 autopsy cases with thorough review of pathogenesis. They considered the agenesis of corpus callosum as one manifestation of dorsal midline dysgenesis, closely related to but less severe than holoprosencephaly. They showed the schematic presentation of the forms of ventricles in agenesis of corpus callosum. At the level of foramen of Monro, the lateral and third ventricles are extensively communicated with great elongation of foramen of Monro. The hippocampal system remains adjacent to the third ventricle roof and there is no medial wall of the ventricle. The present case showed similar overall pattern of ventricles at the level of foramen of Monro, except for the presence of well-developed corpus callosum, absence of septum pellucidum, and malformed fornices (Fig. 3). At the level posterior to foramen of Monro, the medial walls consist of fornix-hippocampal system and Probst's bundle-cingulate gyrus complex. In
comparison, the present case showed diffuse wide communication between lateral and third ventricles at this level. It could be well explained by poor development of hippocampal system and Probst's bundle-cingulate gyrus complex (Figs. 4 and 5). Hydrocephalus, not infrequently associated with agenesis of corpus callosum, is of varying type, communicating and non-communicating. The pathogenesis could not be ascertained in most of the cases. Since the anterior commissure is largely well-developed in this case, main malformations seemed to be occurring in the structures arising from dorsal portion of lamina reuniens, probably during gestational age 9-12 weeks. References 1 AicardiJ, Goutieres F (1981) The syndrome of absence of the septum pellucidum with porencephalies and other developmental defects. Neuropadiatrie 12: 319-329 2 De Morsier G, Mozer 11 (1935) Agenesie complete dela commissure calleuse et troubles du developpement de l'hemisphere gauche avec hemiparesie droite et intetrite mentale (Le syndrome embryonnaire precoce de l'artere cerebrale anterieure.) Schweizer Archiv fiir Neurologie und Psychiatrie 35: 64-95 3 Field M, Ashton R, White K (1978) Agenesis of the corpus callosum; report of two pre-school children and review of the literature. Dev Med Child Neur 20: 47-61 4 Hicks SP (1954) Developmental malformations produced by radiation. Am J Roentgenol 69: 272-293 5 His W (1904) Die Entwicklung des mensch lichen Gehirns wah rend der ersten Monate. pp. 176, Hirzel S (Ed), Leipzig 6 Hochstetter F (1929) Beitrage zur Entwicklungsgeschichte des menschlichen Gehirns. pp. 170, Deuticke F (Ed), Wien-Leipzig 7 Jellinger K, Gross H (1973) Congenital telencephalic midline defect. Neuropadiatrie 4: 446-452 8 Johnston JB (1913) The morphology of the septum, hippocampus, and pallial commissures in reptiles and mammals. J Comp Neurol23: 371-478 9 Loeser JD, Alvord EC (1968(a)) Clinicopathological correlation in agenesis of the corpus callosum. Neurology 18: 745-756 10 Loeser JD, Alvord EC (1968(b)) Agenesis of the corpus callosum. Brain 91: 553-570 11 Marburg 0 (1949) So-called agenesis of the corpus callosum (callosal defect). Arch Neurol Psychiat 61: 297-312 12 Menkes JH, Philippart M, Clark DE (1964) Hereditary partial agenesis of the corpus callosum. Arch Neurol 11: 198-208 13 Naiman J, Frazer FC (1955) Agenesis of the corpus callosum. Arch Neurol Psychiat 74: 182-185 14 Rakic P, Yakovlev PI (1973) Development of the corpus callosum and cavum septi in man. J Comp Neurol 152: 103-132 15 Zingesser L, Schecter M, Gonatas N, Levy A, Wisoff H (1964) Agenesis of the corpus callosum associated with an interhemispheric arachnoid cysts. Brit J Radiol 37: 905-909
Received September 26, 1990 . Accepted December 4, 1990
Key words: Congenital hydrocephalus - Malformation of the midline telencephalic structures - Fornix anomaly Dr. Je G. Chi, Department of Pathology, Seoul National University Children's Hospital, 28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea