Br.J. Anaesth. (1979), 51, 983
KETAMINE AND AGENESIS OF THE CORPUS CALLOSUM I. F. RUSSELL SUMMARY
Ketamine did not provide adequate anaesthesia for pneumoencephalography in a 10-week-old child with agenesis of the corpus callosum. Associated neurological defects are the most likely reason for this failure.
CASE REPORT
The baby was born with the aid of forceps at 36 weeks gestation and weighed 2600 g. He was the sixth child in an apparently normal family. Initial progress was uneventful until, at 5 weeks, he was admitted to hospital for investigation of vomiting; an abnormal increase in head size was noted. A ventriculogram suggested agenesis of the corpus callosum with hydrocephalus and a prosencephalic cyst of the right cerebral hemisphere. Pneumoencephalography under general anaesthesia was performed at the age of 10 weeks (weight 3990 g). Premedication was with atropine O.lmgi.m. During the procedure, heart rate and ventilation were monitored with a precordial stethoscope and arterial pressure was measured with an infant pressure cuff and anaeroid manometer. Within 2 min of the injection of ketamine 50 mg i.m. (10.3 mg kg"1) the muscles became rigid and the child exhibited extended limbs and opisthotonos. Arterial pressure, pulse IAN
F.
RUSSELL, B.MED.BIOL.,
M.B.,
CH.B.,
F.F.A.R.C.S.,
Department of Anaesthesia, Dr Charles A. Janeway Child Health Centre, St Johns, Newfoundland, Canada. Present address: Department of Anaesthesia, Aberdeen Royal Infirmary, Aberdeen AB9 2ZD. 0007-0912/79/100983-03 S01.00
and skin colour remained normal, but breathing became irregular. The rigidity decreased over the next few minutes and regular breathing was resumed. The baby was strapped into a modified infant seat and positioned for lumbar puncture. Over the next 30 min, while air was being injected for pneumoencephalography, a further 35 mg of ketamine (5-mg increments) was administered i.v. because of continued withdrawal movements in response to the insertion and positioning of the lumbar puncture needle. Because of persisting muscle stiffness, diazepam 0.5 mg was given i.v. over 3 min in an attempt to smooth the progress of the anaesthetic. Some muscle relaxation resulted, but the major effect was depression of breathing—a slowing of rate with deep jerky inspiratory efforts. Normal respiratory rhythm resumed within 10 min. Over the last hour of the procedure while x-rays were being obtained, a further 15 mg of ketamine was required (5-mg increments) and, during this time, the baby opened his eyes twice. The investigation lasted 1 h 45 min and, except for respiratory rhythm, other signs were normal. Despite the large dose of ketamine (50 mg i.m. and 50 mg i.v.) the baby's eyes were open before leaving for the recovery area and there were no complications after anaesthesia. Ten days later, a repeat ventriculogram showed a significant increase in the size of the ventricles. A ventriculo-peritoneal shunt was performed under general anaesthesia with nitrous oxide, oxygen, halothane, tubocurarine and controlled ventilation; there were no problems during or after operation. DISCUSSION
Ketamine is usually described as a "dissociative anaesthetic" because it produces e.e.g. changes suggesting dissociation of the cortex from the limbic system (Corssen, Miyasaka and Domino, 1968). However, more recent evidence does not entirely © Macmillan Journals Ltd 1979
Downloaded from http://bja.oxfordjournals.org/ at The Univesity of Calgary on May 27, 2015
Agenesis of the corpus callosum is a rare condition which may not be associated with any detectable neurological deficit. When a deficit does occur it is frequently related to the severity of associated brain anomalies (Harcourt-Webster and Rack, 1965). When agenesis of the corpus callosum is suspected in infancy pneumoencephalography is recommended for definitive diagnosis and general anaesthesia is usually required. The anaesthetic technique used for this procedure is rarely mentioned in published case reports. This communication describes the use of ketamine for pneumoencephalography in a 10-weekold child with suspected agenesis of the corpus callosum.
984
BRITISH JOURNAL OF ANAESTHESIA
Downloaded from http://bja.oxfordjournals.org/ at The Univesity of Calgary on May 27, 2015
support this concept (Kayama and Iwama, 1972; that if, as a result of fundamental neural reorganizaMorse and Cave-Smith, 1974). Since Morse and tion, abnormal pathways link the cortex and limbic Cave-Smith had successfully used ketamine to system, ketamine may not provide adequate analgesia. anaesthetize an infant with no cerebral cortex, it was decided to use ketamine on this occasion. ACKNOWLEDGEMENTS Although the procedure was performed successfully The author is indebted to the Medical Administration of by an experienced radiologist, the anaesthetic was not the Dr Charles A. Janeway Child Health Centre, Dr C. U. ideal. Bizarre and uncontrolled neuromuscular acti- Henderson and Dr W. D. Sprague for their co-operation in vity in infants less than 6 months of age have been presenting this case; to Dr D. G. Ross for his helpful described with ketamine (Corssen, Miyasaka and criticism, and to Mrs June Manwell for her invaluable Domino, 1968; Dillon, 1971; Szappanyos, Gemperle secretarial assistance. and Rifat, 1971). On the other hand, similar neuroREFERENCES muscular reactions have been observed in patients with pre-existing neurological deficiencies (Drury Buskop, J. J., Price, M., and Molner, I. (1967). Untoward and Clark, 1970; Dillon, 1971). In this baby, effect of diazepam. N. Engl.J. Med., 277, 316. opisthotonos and muscular stiffness could be Carpenter, M. B., and Druckenmiller, W. H. (1953). Agenesis of the corpus callosum diagnosed during life. described as a bizarre and uncontrolled reaction AM.A. Arch. Neurol. Psychiatr., 69, 305. which did not respond to diazepam or to further increments of ketamine, a finding in agreement with Corrsen, G., Miyasaka, M., and Domino, E. F. (1968). Changing concepts in pain control during surgery: the results of other work on normal infants dissociative anesthesia with Cl-581; a progress report. (Szappanyos, Gemperle and Rifat, 1971). ConAnesth. Analg. (Cleve.), 47, 746. versely, the withdrawal movements initiated by Dillon, J. B. (1971). Rational use of ketamine as an anaesstimulation from the lumbar puncture needle were thetic. Proc. R. Soc. Med., 64, 1153. well co-ordinated and suggested failure to achieve Drury, W. L., and Clark, L. C. (1970). Ketamine failure in acute brain injury—a case report. Anesth. Analg. (Cleve.), adequate analgesia. The reasons for the failure of 49, 859. ketamine are not clear. While ketamine is usually described as a mild Ettlinger, G., Blakemore, C. B., Milner, A. D., and Wilson, J. (1972). Agenesis of the corpus callosum: a behavioural respiratory stimulant, large doses may cause depresinvestigation. Brain, 95, 327. sion of breathing. In this case respiratory depression occurred only following the administration of di- Harcourt-Webster, J. N., and Rack, J. H. (1965). Agenesis of the corpus callosum. Postgrad. Med. J., 41, 73. azepam. This emphasizes the unpredictability of diazepam respiratory depression (Buskop, Price and Hunter, A. R. (1967). Diazepam (Valium) as a muscle relaxant during general anaesthesia. Br. J. Anaesth., 22, Molner, 1967; Hunter, 1967) in the presence of other 633. anaesthetic agents. A high mortality rate is associated with agenesis of Kayama, Y., and Iwama, K. (1972). The e.e.g. evoked potentials, and single unit activity during ketamine the corpus callosum when the symptoms present in anesthesia in cats. Anesthesiology, 36, 316. infancy (Slager, Kelly and Wagner, 1957; Shapira and Cohen, 1973). This is the result of other severe Morse, N., and Cave-Smith, P. (1974). Ketamine anesthesia in a hydranencephalic infant. Anesthesiology, 40, 407. congenital defects affecting many systems (Slager, Shapira, Y., and Cohen, T. (1973). Agenesis of the corpus Kelly and Wagner, 1957). There is frequently callosum in two sisters. J. Med. Genet., 10, 266. clinical evidence of a diffuse neurological deficit Slager, V. T., Kelly, A. B., and Wagner, J. A. (1957). (Carpenter and Druckenmiller, 1953) and the Congenital absence of the corpus callosum. N. Engl. J. abnormal brain development leads to a reorganization Med., 256,1171. of neural pathways Unking the cortex and limbic Szappanyos, G., Gemperle, M., and Rifat, K. (1971). system (Ettlinger et al., 1972). The pneumoenceSelective indication for ketamine anaesthesia. Proc. R. phalogram confirmed the absence of the corpus Soc. Med., 64, 1156. callosum and this may have contributed to the KETAMINE ET AGENESIE DU CORPS CALLEUX relative failure of ketamine anaesthesia. However, it is RESUME likely that other associated anomalies, including a La ketamine n'a pas provoqui l'anesthesie appropriee lors cystic lesion of the right cerebral hemisphere and d'une pneumoencephalographie chez un enfant age de 10 semaines, atteint d'une agenesie du corps calleux. II est hydrocephalus, played a significant part by further vraisemblable que la raison de cet echec provienne d'anomadisrupting normal neural pathways. It would appear lies neurologiques associees.
985
AGENESIS OF CORPUS GALLOSUM KETAMIN UND OVARIALAPLASIE DES GEHIRNBALKENS
QUETAMINA Y AGENESIA DEL CUERPO CALLOSO
ZUSAMMENFASSUNG
SUMARIO
Ketamin war als Narkosemittelfttr Pneumoenzephalographie bei einem lOwochigen Kind mit Ovarialaplasie des Gehirnbalkens nicht ausreichend. Ein wahrscheinlicher Grund fiir dieses Versagen besteht in vorhandenen neurologischen Defekten.
La quetamina no proveyd una anestesia adecuada para la neumoencefalografia de un nino de 10 semanas con agenesia del cuerpo calloso. Los defectos neurologicos asociados constituyeron muy probablemente la causa de esta deficiencia.
Downloaded from http://bja.oxfordjournals.org/ at The Univesity of Calgary on May 27, 2015
KETAMINE AND AGENESIS OF THE CORPUS CALLOSUM I. F. RUSSELL SUMMARY
Ketamine did not provide adequate anaesthesia for pneumoencephalography in a 10-week-old child with agenesis of the corpus callosum. Associated neurological defects are the most likely reason for this failure.
CASE REPORT
The baby was born with the aid of forceps at 36 weeks gestation and weighed 2600 g. He was the sixth child in an apparently normal family. Initial progress was uneventful until, at 5 weeks, he was admitted to hospital for investigation of vomiting; an abnormal increase in head size was noted. A ventriculogram suggested agenesis of the corpus callosum with hydrocephalus and a prosencephalic cyst of the right cerebral hemisphere. Pneumoencephalography under general anaesthesia was performed at the age of 10 weeks (weight 3990 g). Premedication was with atropine O.lmgi.m. During the procedure, heart rate and ventilation were monitored with a precordial stethoscope and arterial pressure was measured with an infant pressure cuff and anaeroid manometer. Within 2 min of the injection of ketamine 50 mg i.m. (10.3 mg kg"1) the muscles became rigid and the child exhibited extended limbs and opisthotonos. Arterial pressure, pulse IAN
F.
RUSSELL, B.MED.BIOL.,
M.B.,
CH.B.,
F.F.A.R.C.S.,
Department of Anaesthesia, Dr Charles A. Janeway Child Health Centre, St Johns, Newfoundland, Canada. Present address: Department of Anaesthesia, Aberdeen Royal Infirmary, Aberdeen AB9 2ZD. 0007-0912/79/100983-03 S01.00
and skin colour remained normal, but breathing became irregular. The rigidity decreased over the next few minutes and regular breathing was resumed. The baby was strapped into a modified infant seat and positioned for lumbar puncture. Over the next 30 min, while air was being injected for pneumoencephalography, a further 35 mg of ketamine (5-mg increments) was administered i.v. because of continued withdrawal movements in response to the insertion and positioning of the lumbar puncture needle. Because of persisting muscle stiffness, diazepam 0.5 mg was given i.v. over 3 min in an attempt to smooth the progress of the anaesthetic. Some muscle relaxation resulted, but the major effect was depression of breathing—a slowing of rate with deep jerky inspiratory efforts. Normal respiratory rhythm resumed within 10 min. Over the last hour of the procedure while x-rays were being obtained, a further 15 mg of ketamine was required (5-mg increments) and, during this time, the baby opened his eyes twice. The investigation lasted 1 h 45 min and, except for respiratory rhythm, other signs were normal. Despite the large dose of ketamine (50 mg i.m. and 50 mg i.v.) the baby's eyes were open before leaving for the recovery area and there were no complications after anaesthesia. Ten days later, a repeat ventriculogram showed a significant increase in the size of the ventricles. A ventriculo-peritoneal shunt was performed under general anaesthesia with nitrous oxide, oxygen, halothane, tubocurarine and controlled ventilation; there were no problems during or after operation. DISCUSSION
Ketamine is usually described as a "dissociative anaesthetic" because it produces e.e.g. changes suggesting dissociation of the cortex from the limbic system (Corssen, Miyasaka and Domino, 1968). However, more recent evidence does not entirely © Macmillan Journals Ltd 1979
Downloaded from http://bja.oxfordjournals.org/ at The Univesity of Calgary on May 27, 2015
Agenesis of the corpus callosum is a rare condition which may not be associated with any detectable neurological deficit. When a deficit does occur it is frequently related to the severity of associated brain anomalies (Harcourt-Webster and Rack, 1965). When agenesis of the corpus callosum is suspected in infancy pneumoencephalography is recommended for definitive diagnosis and general anaesthesia is usually required. The anaesthetic technique used for this procedure is rarely mentioned in published case reports. This communication describes the use of ketamine for pneumoencephalography in a 10-weekold child with suspected agenesis of the corpus callosum.
984
BRITISH JOURNAL OF ANAESTHESIA
Downloaded from http://bja.oxfordjournals.org/ at The Univesity of Calgary on May 27, 2015
support this concept (Kayama and Iwama, 1972; that if, as a result of fundamental neural reorganizaMorse and Cave-Smith, 1974). Since Morse and tion, abnormal pathways link the cortex and limbic Cave-Smith had successfully used ketamine to system, ketamine may not provide adequate analgesia. anaesthetize an infant with no cerebral cortex, it was decided to use ketamine on this occasion. ACKNOWLEDGEMENTS Although the procedure was performed successfully The author is indebted to the Medical Administration of by an experienced radiologist, the anaesthetic was not the Dr Charles A. Janeway Child Health Centre, Dr C. U. ideal. Bizarre and uncontrolled neuromuscular acti- Henderson and Dr W. D. Sprague for their co-operation in vity in infants less than 6 months of age have been presenting this case; to Dr D. G. Ross for his helpful described with ketamine (Corssen, Miyasaka and criticism, and to Mrs June Manwell for her invaluable Domino, 1968; Dillon, 1971; Szappanyos, Gemperle secretarial assistance. and Rifat, 1971). On the other hand, similar neuroREFERENCES muscular reactions have been observed in patients with pre-existing neurological deficiencies (Drury Buskop, J. J., Price, M., and Molner, I. (1967). Untoward and Clark, 1970; Dillon, 1971). In this baby, effect of diazepam. N. Engl.J. Med., 277, 316. opisthotonos and muscular stiffness could be Carpenter, M. B., and Druckenmiller, W. H. (1953). Agenesis of the corpus callosum diagnosed during life. described as a bizarre and uncontrolled reaction AM.A. Arch. Neurol. Psychiatr., 69, 305. which did not respond to diazepam or to further increments of ketamine, a finding in agreement with Corrsen, G., Miyasaka, M., and Domino, E. F. (1968). Changing concepts in pain control during surgery: the results of other work on normal infants dissociative anesthesia with Cl-581; a progress report. (Szappanyos, Gemperle and Rifat, 1971). ConAnesth. Analg. (Cleve.), 47, 746. versely, the withdrawal movements initiated by Dillon, J. B. (1971). Rational use of ketamine as an anaesstimulation from the lumbar puncture needle were thetic. Proc. R. Soc. Med., 64, 1153. well co-ordinated and suggested failure to achieve Drury, W. L., and Clark, L. C. (1970). Ketamine failure in acute brain injury—a case report. Anesth. Analg. (Cleve.), adequate analgesia. The reasons for the failure of 49, 859. ketamine are not clear. While ketamine is usually described as a mild Ettlinger, G., Blakemore, C. B., Milner, A. D., and Wilson, J. (1972). Agenesis of the corpus callosum: a behavioural respiratory stimulant, large doses may cause depresinvestigation. Brain, 95, 327. sion of breathing. In this case respiratory depression occurred only following the administration of di- Harcourt-Webster, J. N., and Rack, J. H. (1965). Agenesis of the corpus callosum. Postgrad. Med. J., 41, 73. azepam. This emphasizes the unpredictability of diazepam respiratory depression (Buskop, Price and Hunter, A. R. (1967). Diazepam (Valium) as a muscle relaxant during general anaesthesia. Br. J. Anaesth., 22, Molner, 1967; Hunter, 1967) in the presence of other 633. anaesthetic agents. A high mortality rate is associated with agenesis of Kayama, Y., and Iwama, K. (1972). The e.e.g. evoked potentials, and single unit activity during ketamine the corpus callosum when the symptoms present in anesthesia in cats. Anesthesiology, 36, 316. infancy (Slager, Kelly and Wagner, 1957; Shapira and Cohen, 1973). This is the result of other severe Morse, N., and Cave-Smith, P. (1974). Ketamine anesthesia in a hydranencephalic infant. Anesthesiology, 40, 407. congenital defects affecting many systems (Slager, Shapira, Y., and Cohen, T. (1973). Agenesis of the corpus Kelly and Wagner, 1957). There is frequently callosum in two sisters. J. Med. Genet., 10, 266. clinical evidence of a diffuse neurological deficit Slager, V. T., Kelly, A. B., and Wagner, J. A. (1957). (Carpenter and Druckenmiller, 1953) and the Congenital absence of the corpus callosum. N. Engl. J. abnormal brain development leads to a reorganization Med., 256,1171. of neural pathways Unking the cortex and limbic Szappanyos, G., Gemperle, M., and Rifat, K. (1971). system (Ettlinger et al., 1972). The pneumoenceSelective indication for ketamine anaesthesia. Proc. R. phalogram confirmed the absence of the corpus Soc. Med., 64, 1156. callosum and this may have contributed to the KETAMINE ET AGENESIE DU CORPS CALLEUX relative failure of ketamine anaesthesia. However, it is RESUME likely that other associated anomalies, including a La ketamine n'a pas provoqui l'anesthesie appropriee lors cystic lesion of the right cerebral hemisphere and d'une pneumoencephalographie chez un enfant age de 10 semaines, atteint d'une agenesie du corps calleux. II est hydrocephalus, played a significant part by further vraisemblable que la raison de cet echec provienne d'anomadisrupting normal neural pathways. It would appear lies neurologiques associees.
985
AGENESIS OF CORPUS GALLOSUM KETAMIN UND OVARIALAPLASIE DES GEHIRNBALKENS
QUETAMINA Y AGENESIA DEL CUERPO CALLOSO
ZUSAMMENFASSUNG
SUMARIO
Ketamin war als Narkosemittelfttr Pneumoenzephalographie bei einem lOwochigen Kind mit Ovarialaplasie des Gehirnbalkens nicht ausreichend. Ein wahrscheinlicher Grund fiir dieses Versagen besteht in vorhandenen neurologischen Defekten.
La quetamina no proveyd una anestesia adecuada para la neumoencefalografia de un nino de 10 semanas con agenesia del cuerpo calloso. Los defectos neurologicos asociados constituyeron muy probablemente la causa de esta deficiencia.
Downloaded from http://bja.oxfordjournals.org/ at The Univesity of Calgary on May 27, 2015
