Contribuição ao estudo das malformações occipito-cervical, particularmente da impressão basilar

Canelas, Horacio M.; Zaclis, José; Tenuto, Rolando A.

doi:10.1590/S0004-282X1952000400001

Resumo

The authors outline the development of the spine and skull, particularly of axis, atlas and occipital bone. As neuro-skeletal dysmorphisms, the occipito-cervical malformations belong to the neurodysplastic group. They are classified as skeletal anomalies, associated nervous malformations and meningeal reactions. Vertebralization of the occipital bone and occipitalization of atlas, subluxation of odontoid process, dysplasia of the occipital bone, dystrophia brevicollis and other anomalies are discussed. Special care is given to the study of basilar impression; its concept, history, incidence, clinical and neurological symptoms, radiological characterization (craniographic, perimyelographic and iodoventriculographic aspects) and surgical treatment are reviewed. The authors report five cases of occipito-cervical malformations, which are the first references in Brazilian literature. In case 1 the anomalies (manifestation of occipital vertebra and Arnold-Chiari deformity) were disclosed at an operation for cisticercosis of the posterior fossa. In the following four cases invagination of the basilar portion of the occipital bone (basilar impression) could be radiologically demonstrated; in case 2 a suboccipital craniectomy and a laminectomy of atlas and axis were performed but the patient died a week later and the necroscopic examination confirmed the neuro-skeletal anomalies. In all cases there were several associated malformations. In case 2 there were occipitalization of the atlas, fusion of the first and second cervical vertebrae, supernumerary rib of the seventh cervical vertebra, supernumerary lumbar vertebra, and Arnold-Chiari deformity; at necropsy it was found a syringomyelic cyst on the cervical cord and a fibrous dural ring over the foramen magnum. Case 3 showed the syndrome of Klippel-Feil, besides supernumerary ribs of the seventh cervical and first dorsal vertebrae, Arnold-Chiari malfotmation and probable aplasia of cell groups in the nuclei of oculomotor and facial nerves. In case 4 there were occipitalization of atlas and Chiari deformity. In case 5 vertebral reduction in the cervical region was evidenced but bony coalescence could not be proved. In the last four cases the constitutional morphologic type was determined. Electroencephalograms of cases 3, 4 and 5 showed no abnormalities; in case 1 slow bilateral occipital waves were recorded. The authors call attention to the importance of the diagnosis of these anomalies susceptible of successful surgical correction, which can simulate many nervous diseases of untoward course, like lateral amyotrophic sclerosis, syringomyelia (which can be actually associated to the bony anomaly, as in case 2), sclerosis of dorsal funiculi of the spinal cord, tabes, multiple sclerosis, heredocerebellar ataxia, etc. The authors also stress the clinico-radiological differences between basilar impression and platybasia, frequently taken as synonyms. Although both anomalies may coexist (as in cases 4 and 5) their association is not an absolute rule; on the contrary, in case 2 the basal angle was in the inferior limit of normality and in case 3 it was much lower. Emphasis is put on the value of the Chamberlain's line and chiefly in the McGregor basal line for the correct diagnosis of basilar invagination of the occipital bone.

Horacio M. Canelas^I; José Zaclis^II; Rolando A. Tenuto^III

^IAssistente de Clínica Neurológica da Fac. Med. da Univ. de São Paulo (Prof. Adherbal Tolosa)

^IIAssistente de Neurorradiologia (Prof. Raphael de Barros) e de Clínica Neurológica (Prof. Adherbal Tolosa) da Fac. Med. da Univ. de São Paulo

^IIINeurocirurgião da Clínica Neurológica da Fac. Med. da Univ. de São Paulo (Prof. Adherbal Tolosa)

SUMMARY

The authors outline the development of the spine and skull, particularly of axis, atlas and occipital bone. As neuro-skeletal dysmorphisms, the occipito-cervical malformations belong to the neurodysplastic group. They are classified as skeletal anomalies, associated nervous malformations and meningeal reactions. Vertebralization of the occipital bone and occipitalization of atlas, subluxation of odontoid process, dysplasia of the occipital bone, dystrophia brevicollis and other anomalies are discussed. Special care is given to the study of basilar impression; its concept, history, incidence, clinical and neurological symptoms, radiological characterization (craniographic, perimyelographic and iodoventriculographic aspects) and surgical treatment are reviewed.

The authors report five cases of occipito-cervical malformations, which are the first references in Brazilian literature. In case 1 the anomalies (manifestation of occipital vertebra and Arnold-Chiari deformity) were disclosed at an operation for cisticercosis of the posterior fossa. In the following four cases invagination of the basilar portion of the occipital bone (basilar impression) could be radiologically demonstrated; in case 2 a suboccipital craniectomy and a laminectomy of atlas and axis were performed but the patient died a week later and the necroscopic examination confirmed the neuro-skeletal anomalies. In all cases there were several associated malformations. In case 2 there were occipitalization of the atlas, fusion of the first and second cervical vertebrae, supernumerary rib of the seventh cervical vertebra, supernumerary lumbar vertebra, and Arnold-Chiari deformity; at necropsy it was found a syringomyelic cyst on the cervical cord and a fibrous dural ring over the foramen magnum. Case 3 showed the syndrome of Klippel-Feil, besides supernumerary ribs of the seventh cervical and first dorsal vertebrae, Arnold-Chiari malfotmation and probable aplasia of cell groups in the nuclei of oculomotor and facial nerves. In case 4 there were occipitalization of atlas and Chiari deformity. In case 5 vertebral reduction in the cervical region was evidenced but bony coalescence could not be proved. In the last four cases the constitutional morphologic type was determined. Electroencephalograms of cases 3, 4 and 5 showed no abnormalities; in case 1 slow bilateral occipital waves were recorded.

The authors call attention to the importance of the diagnosis of these anomalies susceptible of successful surgical correction, which can simulate many nervous diseases of untoward course, like lateral amyotrophic sclerosis, syringomyelia (which can be actually associated to the bony anomaly, as in case 2), sclerosis of dorsal funiculi of the spinal cord, tabes, multiple sclerosis, heredocerebellar ataxia, etc. The authors also stress the clinico-radiological differences between basilar impression and platybasia, frequently taken as synonyms. Although both anomalies may coexist (as in cases 4 and 5) their association is not an absolute rule; on the contrary, in case 2 the basal angle was in the inferior limit of normality and in case 3 it was much lower. Emphasis is put on the value of the Chamberlain's line and chiefly in the McGregor basal line for the correct diagnosis of basilar invagination of the occipital bone.

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112. Ingraham, F. D., Swan, H. e outros - Spina bifida and cranium bifidium. Separatum do New England J. Med., 1944. Cit. por Furtado e col.¹¹¹

113. Gardner, W. J. e Goodall, R. J. - The surgical treatment of Arnold-Chiari malformation in adults. An explanation of its mechanism and importance of encephalography in diagnosis. J. Neurosurg., 7:199-206 (maio) 1950.

114. Swanson, H. S. e Fincher, E. F. -- Arnold-Chiari deformity without bony anomalies. J. Neurosurg., 6:314-319, 1949.

115. Hurteau, E. F. - Arnold-Chiari malformation. J. Neurosurg., 7:282-284 (maio) 1950.

116. Ogryzlo, M. A. - The Arnold-Chiari malformation. Arch. Neurol, a. Psychiat., 48:30-46 (julho) 1942.

117. Barraquer Ferré, L. e Barraquer Bordas, L. - Siringomielia y espina bifida cervical. Deducciones genéticas y diagnósticas. Med. Españ., 14:101-113 (agôsto) 1945.

118. Lichtenstein, B. W. - Spinal dysraphism; spina bifida and myelodysplasia. Arch. Neurol, a. Psychiat, 44:792-810 (outubro) 1940.

119. Meyer, B. C. - Platybasia. Report of a case. Arch. Neurol, a. Psychiat., 52:552-553 (dezembro) 1944.

120. Grunthal, E. - Über den Hirnbefund bei Pagetscher Krankheit des Schadels. Zugleich ein Beitrag zur Kenntnis der Entstehung systematischer Klein-hirnatrophien. Ztschr. f. d. ges. Neurol, u. Psychiat., 136:656-675, 1931.

121. Cit. por Barraquer Bordas²⁵.

122. Custis, D. L. e Verbrugghen, A. - Basilar impression resembling cerebelar tumor. Report of a case. Arch. Neurol, a. Psychiat., 52:412-415 (novembro) 1944.

123. Jimenez Dias, C, Obrador, S., Urquiza, P. e Albert, C. - Un caso operado de impresión basilar (platibasia). Rev. Clín. Españ., 28:180-185 (15 fevereiro) 1948.

124. Garcin, R., Guillaume, J., Kipfer, M. e Lesobre, R. - Anomalie congénitale des deux premières vertèbres cervicales avec quadriparésie et troubles bulbaires. Intervention. Méningite séreuse kystique de la fosse postérieure. Amélioration. Rev. Neurol., 80:223-228 (março) 1948.

125. Ray, B. - Platybasia with involvement of the central nervous system. Ann. Surg., 116:231-250 (agôsto) 1942.

126. Stevens, R. H. - Platybasia. A report of a case treated surgically with improvement. Surgery, 12:943-951 (dezembro) 1942.

127. Riser, M., Lazorthes, G., Géraud, J. e Puig, R. - Platybasie et hydrocéphalie secondaires à une maladie de Paget. Semaine d. Hôp., 25:728-730 (2 março) 1949.

128. Lindgren - Roentgenological views on basilar impression. Acta Radiol., 22:297-302 (julho) 1941.

129. Boogaard, J. A. - De indrukking der grondolakte van den Schedel door de wervelkolom, hare corzaken en gevolgen. Nederl. Tijdschr. v. Geneesk, 2:81-108, 1865.

130. Ricard, A., Girard, P.-F., J. L. e Replumaz, P. - Malformations congénitales de la charnière occipito-vertébrale. Lyon Chir., 44:679-692 (novembro-dezembro) 1949.

131. Hajdu, N. e Kauntze, R. - Cranio-skeletal dysplasia. Brit. J. Radiol., 21: 42-48 (janeiro) 1948.

132. Rokitansky, C. - Lehrbuch der pathologisch Anatomie. Viena, 1846, vol. 2, pág. 149. Cit. por Mayersky ¹⁴².

133. Berg, F. T. e Retzius, A. - Museum Anatomiae Holmense, Stockholm, 1865. Cit. por Mayersky ¹⁴².

134. Wycis, H. T. - Basilar impression (platybasia). A case secondary to advanced Paget's disease with severe neurological manifestations. Successful surgical result. J. Neurosurg., 1:299-305 (setembro) 1944.

135. Virchow, R. - Beitrage zur physischen Anthropologic der Deutschen mit besonderer Berucksichtigung der Friesen. G. Vogt, Berlim, 1876.

136. Grawitz, P. - Beitrag zur Lehre von der basilaren Impression des Schadels. Virchows Arch. f. path. Anat., 80:449-474, 1880.

137. Saunders, W. W. - La impresión basilar y la posición de la apófisis odon-toides. Rev. Radiol, y Fisiot., 12:87-88 (julho-agôsto) 1945.

138. Lysholm, E. - Die spezielle Chirurgie der Gehirnkrankheiten. Stuttgart, 1941. Cit. por Jimenez Diaz e col.^123.

139. Davidoff, L. - Discussão do trabalho de Gustafson e Oldberg ⁵⁹.

140. Cit. por Chamberlain⁵.

141. Recklinghausen, D. J. - Über osteomalacische Knochenstruturen. Zentralbl. f. allg. path. Anat., 8:858, 1897.

142. Mayersky, C. - Platybasia. J. Neurol., Neurosurg. a. Psychiat., 12:61-65 (fevereiro) 1949.

143. Brailsford, J. F. - The Radiology of Bones and Joints. Churchill, Londres, 1944.

144. Marie, P., Léri, A. e Chatelin - Déformation de la base du crâne dans la maladie de Paget. Bull, et Mém. Soc. Méd. Hôp. d. Paris, 34:89-91, 1912.

145. Garcin, R. e Kipfer, M. - Sur un cas d'impression basilaire avec troubles neurologiques. Rev. Neurol., 79:767-770 (dezembro) 1947.

146. Epstein, I. - Platybasia associated with an unusual case of pulmonary tuberculosis. Dis. of Chest, 15:563-567 (maio) 1949.

147. Girard, P. F., Gerest, Devic, M. e Bonnet, J. L. - Malformation de la charnière occipito-vertébrale revelée par un syndrome vestibulo-médullaire complexe; impression basilaire. Rev. d'Otoneuro-ophtalmol., 21:382 (agôsto-setembro) 1949.

148. Chamosa, V. S. S. - Un caso de impresión basilar. Rev. Españ. Otoneuro-oftalmol. y Neurocir., 7:186-191 (maio-junho) 1950.

149. Chorobski, J. e Stepien, L. - On the syndrome of Arnold-Chiari. Report of a case. J. Neurosurg., 5:495-499 (setembro) 1948.

150. Lhermitte, J. e Sigwald, J. - Sur l'astéreognosie spasmodique consecutive à l'hyperplasie du basioccipital. Rev. Neurol., 74:141-142 (mareo-abril) 1942.

151. Lhermitte, J. - L'hyperplasie du basioccipital. Rev. Neurol., 82:399-401 (maio) 1950.

152. Perez Velasco, O. - Contribuição anátomo-clínica às atuais concepções sôbre a epilepsia. Trabalho apresentado ao VI Congresso Brasileiro de Psiquiatria, Neurologia e Medicina Legal, Rio-São Paulo, 1949. Arq. Neuro-Psiquiat, 8:301-334 (dezembro) 1950.

153. Stenvers, H. W. - Röntgenologie des Felsenbeines und des bitemporalen Schadelbildes. J. Springer, Berlim, 1928.

154. Martin, P. e Kleyntjens, F. - Tumeurs sous-durales du trou occipital. Rev. Neurol., 82:313-334 (maio) 1950.

155. Moreton, R. D. - Basilar invagination; so-called platybasia. Proc. Staff Meet., Mayo Clin., 18:353-357 (22 setembro) 1943.

156. Jostes, F. A. - Neck pain. Laminagraph as aid to diagnosis of atlanto-occipital lesions. J.A.M.A., 118:353-359 (31 janeiro) 1942.

157. Kasabach, H. H. - Roentgenographic method for study of second cervical vertebra. Am. J. Roentgenol., 42:782-785, 1939.

158. Juhlin-Dannfelt, C - Platybasie. Svenska Lakartidn., 30:470-473, 1933.

159. Krause, F. - Chirurgie du Cerveau et de la Moelle Epinière. Trad, francêsa de J. Bourquet. Soc. d'Édit. Scientifiques Méd. F. Gittler, Paris, 1912, vol. 2, págs. 564-566.

Trabalho laureado pela Academia Nacional de Medicina (Rio de Janeiro), com o Prêmio "A. Austregésilo" de 1951.

Clínica Neurológica - Hospital das Clínicas - São Paulo.

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56. Green, H. L. H. H. - An unusual case of atlanto-occipital fusion. J. Anat., 65:140-144 (outubro) 1930.
57. Rau, R. K. e Sivasubrahmanian, D. - Anomalous atlanto-occiput. J. Anat., 67:622-623 (julho) 1933.
58. Gilmore, J. H., Stauffer, R. C. e Jacobs, L. G. - Two cases of spinal anomaly, best demonstrated by laminagraphy. Radiology, 46:514-519 (maio) 1946.
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67. Moloney, J. C. e Crawford, A. S. - An unusual case of compression myelitis with posterior luxation of occiput. J.A.M.A., 95:1096-1097 (11 outubro) 1930.
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78. Geipel, P. - a) Zur Kenntnis der Spina bifida des Atlas. Fortschr. a. d. Geb. d. Röntgenstrahlen, 42:583-589 (novembro) 1930;
b) Zur Kenntnis der Spaltbildung des Atlas und Epistropheus. Idem, 46:373-402 (outubro) 1932;
c) Zur Kenntnis der Spaltbildung des Atlas und Epistropheus. Idem, 52:533-570 (dezembro) 1935.
79. Brown, C. E. - Complete absence of the posterior arch of the atlas. Anat. Rec, 81:499-504 (dezembro) 1941.
80. Lawrence, W. S. e Anderson, W. D. - Rare developmental abnormality of atlas. Radiology, 28:55-57, 1937.
81. Plaut, H. F. - Fracture of atlas or developmental abnormality? Radiology, 29:227-231, 1937.
82. Symonds, C. P. e Taylor, J. - Compression of spinal cord in neighbourhood of foramen magnum, with note on surgical aproach. Brain, 60:52-84, 1937.
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85. Scannel, R. C. - Congenital absence of odontoid process. J. Bone a. Joint Surg., 27:714-715 (outubro) 1945.
86. McK.Ivie, J. - Congenital absence of the odontoid process. Radiology, 46: 268-269 (março) 1946.
87. Lombard, P., Munéra, G. e Blanc, Y. - Luxation atloi'do-axoi'dienne pure de nature traumatique liée à l'absence de la dent de l'axis. Rev. d'Orthopédie, 31:179-185 (setembro-dezembro) 1945.
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90. Furst, W. e Ostrum, H. W. - Platybasia, Klippel-Feil syndrome and Sprengel's deformity. Am. J. Roentgenol., 47:588-590, 1942.
91. Guillain, G. e Mollaret, P. - Syndrome de Klippel-Feil avec quadriplégie spasmodique. Rev. Neurol., 55:436-444 (abril) 1931.
92. Pommé, B., Buffé, P. e Marot, R. - Occipitalisation de l'atlas et reduction numérique des vertèbres cervicales avec déhiscence des arcs postérieurs. Lyon Méd, 154:319-325 (30 setembro) 1934.
93. Merio, F. e Risak, E. - Klippel-Feilsches Syndrom, basilare Impression und endokrine Erkrankungen. Ztschr. f. klin. Med., 126:455-468, 1934.
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Contribuição ao estudo das malformações occipito-cervical, particularmente da impressão basilar

On the occipito-cervical malformations, particularly the basilar impression

Datas de Publicação

Publicação nesta coleção
06 Fev 2015
Data do Fascículo
Dez 1952

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

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