409
patients with fulminant hepatitis were selected’ for liver transplantation and referred to our unit where they were put in a special emergency programme aimed at finding a liver donor as quickly as possible.2 No patient was withdrawn from the programme. For 14 no donor was found, and all of them died. In the other 51 liver transplantation was done and 1-year-survival was 80%. We conclude that selection based on the criteria of the Beaujon group1 is satisfactory; and, on ethical grounds, we do not see ourselves taking part in a controlled trial for this category of
patient. Hepatobiliary Surgery and Liver Transplant Research Unit, Hôpital Paul Brousse, 94800 Villejuif, France
H. BISMUTH D. SAMUEL
1. Bemuau J, Goudeau A, Poynard T, et al. Multivariate analysis of prognostic factors of fulminant hepatitis B. Hepatology 1986; 6: 648-51. 2. Bismuth H, Samuel D. Liver transplantation in fulminant and subfulminant hepatitis. In: Morris PJ, Tilney NL, eds. Transplantation reviews 3. Philadelphia: Saunders, 1989: 47-58.
Lack of atherosclerosis in omental arteries SiR,—Ihave studied the omentum over the past 25 years in man and in animals, and I have consistently observed the absence of atherosclerosis in omental arteries. This anti-atherosclerotic capacity has been noted in small omental arteries as well as in the large gastroepiploic vessels sited within the omental apron. This sparing of omental arteries has even been observed in the presence of generalised and severe atherosclerosis. I have asked many pathologists over the years if they had ever noticed atherosclerosis in omental vessels and I have yet to hear an affirmative answer. The long-term administration of a high-lipid diet in monkeys also fails to affect omental arteries despite widespread atherosclerosis throughout the vascular system. Omental tissue has proved to contain angiogenic factors,1 neurotransmitters/,3 and neurotropic substances.4 Clinicians have long appreciated the anti-infectious and antineoplastic characteristics of the omentum. Does the omentum also have a detectable anti-atherosclerotic factor? If such a substance could be identified, it might have clinical potential. University School of Medicine, Boston, Massachusetts 02118, USA Boston
Anaesthesia and
myotonic dystrophy
SIR,-Your medicolegal correspondent has discussed (Oct 28,
1053) the
that may have led
p
the death of a patient with myotonic dystrophy given a general anaesthetic for tendon transfer surgery. We would like to suggest an alternative sequence of events. Patients with myotonic dystrophy are prone to periods of apnoea during sleep.1-4 Apnoea may also occur during relaxed wakefulness4 and is exacerbated by anaesthetic agents such as thiopentone.5 As Mrs Brahams points out, many anaesthetic agents can worsen respiratory function in patients with myotonic dystrophy. It is possible that a combination of a barbiturate induction with subsequent inhalational maintenance, coupled with what was a reasonable dose of a narcotic analgesic, exacerbated the tendency to apnoea, leading to prolonged hypoxia and cardiac arrest. Aspiration of gastric contents could have happened after rather than before the events
to
cardiorespiratory arrest. In these circumstances it would be illogical to recommend electrocardiographic monitoring. The most effective method for preventing further problems postoperatively in patients with myotonic dystrophy would be to concentrate on respiratory monitoring. We have been involved in the care of two such patients who underwent surgical procedures under general anaesthetic before myotonic dystrophy had been diagnosed, presented as "failure to reverse" and required prolonged ventilatory support. Another similar case has recently been described,6 and in that case prolonged apnoea occurred only after a second general anaesthetic, recovery after the first having been normal. We would recommend that all patients with myotonic dystrophy undergoing anaesthesia should be carefully monitored. Monitoring should include pulse oximetry and, where possible, some measure of respiratory rate and depth (with, for example, a ’Respitrace’ monitor). Electrocardiographic monitoring may reveal no abnormalities in these patients until hypoxic cardiac arrest has occurred. Whipps Cross Hospital, London E11
J. H. COAKLEY
Department of Medicine, University of Liverpool, Liverpool L69 3BX, UK
P. M. A. CALVERLEY
1. Coccagna
G, Mantovani M, Parchi L, et al. Alveolar hypoventilation and hypersomnia in myotonic dystrophy. J Neurol Neurosurg Psychiatry 1975; 38: 977-84. 2 Cadieux RJ, Kalesa A, McGlynn TJ, Jackson D, Manders EK, Simmonds MA. Sleep apnoea precipitated by pharyngeal surgery in a patient with myotonic dystrophy. Arch Otolaryngol 1984; 110: 611-13. 3. Veale D, Cooper BG, Gilmartin JJ, et al. Breathing during sleep in patients with myotonic dystrophy and non myotonic respiratory muscle weakness. Thorax 1986; 41: 710. 4. Coakley JH, Edwards RHT, Calverley PMA. Mazindol reduces sleep apnoea in myotonic dystrophy. Eur J Clin 1989; 19: A50. 5. Kaufmann L. Anaesthesia in dystrophia myotonica. Proc Roy Soc Med 1963; 153: 183-88. 6. Moore JK, Moore AP. Postoperative complications of dystrophia myotonica. Anaesth 1987; 42: 529-33.
HARRY S. GOLDSMITH
HS, Chen WF, Duckett SW. Brain vascularization by intact omentum. Arch Surg 1973; 106: 695-98. 2. Goldsmith HS, McIntosh T, Vezina RM, Colton T. Vasoactive neurochemicals identified in omentum. Br J Neurosurg 1987; 1: 359-64. 3. Goldsmith HS, Marquis JK, Siek G. Choline acetyltransferase activity in omental tissue. Br J Neurosurg 1987; 1: 463-66. 4. Siek GC, Marquis JK, Goldsmith HS. Experimental studies of omentum-derived neurotrophic factors. In: Goldsmith HS, ed. The omentum: research and clinical applications. London: Royal Society of Medicine, 1990: 83-95. 1. Goldsmith
Development of multiple sclerosis in patient on long-term sulfasalazine SIR,-Preliminary data on the suppression of experimental allergic encephalomyelitis (EAE) by sulfasalazinel have prompted interest in the value of this drug in the treatment of multiple sclerosis (MS).2 A German-Swiss multicentre study is planned. We report on a patient in whom, during 7 years of treatment with sulfasalazine, MS
developed. A 41-year-old man presented with progressive sensory symptoms of the extremities and trunk and impotence over 2 weeks. Ulcerative colitis had been diagnosed 10 years earlier. He had been treated with 3 g sulfasalazine per day for 7 years but over the past 6 months he had reduced daily intake to 1 -5 g because of disease inactivity for more than 2 years. In 1988 he had an intervertebral disc operation. Neurological examination, visual evoked potentials, and oligoclonal bands in the cerebrospinal fluid pointed to MS. Magnetic resonance imaging (MRI) of the brain disclosed multiple hyperintense lesions in T2-weighted images but no gadolinium enhancement, and in the spinal cord two elongated cervical lesions were found, one showing gadolinium enhancement. Corticosteroid therapy was accompanied by marked amelioration. The patient returned to sulfasalazine 3 g daily. 1 month later sensory symptoms of the arms waxed and waned after tapering of the steroid medication and spinal MRI disclosed a new, gadolinium-enhancing thoracic lesion. Clinical examination and tests pointed to dissemination in space. Dissemination in time, which is required to fulfil the classical diagnostic criteria for MS, was suggested by the coexisting gadolinium enhancing and non-enhancing lesions on MRI;3 disclosure of a new thoracic lesion in the second spinal MRI further confirmed the diagnosis.’ The coincidence of MS and inflammatory bowel diseases has been described,5-7 and shared susceptibility genes or a generalised immune disorder, rather than cause and effect, have been discussed as possible aetiological factors. Since in this case colitis had been inactive for the previous 2 years, a causal relation between this man’s neurological findings and his bowel disease is unlikely. Nor is the reduction in sulfasalazine dose likely to be significant since even the reduced dosage (1’5g daily) is not far below the recommended maintenance dosage of 2 g. Furthermore the new MRI spinal lesion developed under a sulfasalazine dosage of 3 g.
patients with fulminant hepatitis were selected’ for liver transplantation and referred to our unit where they were put in a special emergency programme aimed at finding a liver donor as quickly as possible.2 No patient was withdrawn from the programme. For 14 no donor was found, and all of them died. In the other 51 liver transplantation was done and 1-year-survival was 80%. We conclude that selection based on the criteria of the Beaujon group1 is satisfactory; and, on ethical grounds, we do not see ourselves taking part in a controlled trial for this category of
patient. Hepatobiliary Surgery and Liver Transplant Research Unit, Hôpital Paul Brousse, 94800 Villejuif, France
H. BISMUTH D. SAMUEL
1. Bemuau J, Goudeau A, Poynard T, et al. Multivariate analysis of prognostic factors of fulminant hepatitis B. Hepatology 1986; 6: 648-51. 2. Bismuth H, Samuel D. Liver transplantation in fulminant and subfulminant hepatitis. In: Morris PJ, Tilney NL, eds. Transplantation reviews 3. Philadelphia: Saunders, 1989: 47-58.
Lack of atherosclerosis in omental arteries SiR,—Ihave studied the omentum over the past 25 years in man and in animals, and I have consistently observed the absence of atherosclerosis in omental arteries. This anti-atherosclerotic capacity has been noted in small omental arteries as well as in the large gastroepiploic vessels sited within the omental apron. This sparing of omental arteries has even been observed in the presence of generalised and severe atherosclerosis. I have asked many pathologists over the years if they had ever noticed atherosclerosis in omental vessels and I have yet to hear an affirmative answer. The long-term administration of a high-lipid diet in monkeys also fails to affect omental arteries despite widespread atherosclerosis throughout the vascular system. Omental tissue has proved to contain angiogenic factors,1 neurotransmitters/,3 and neurotropic substances.4 Clinicians have long appreciated the anti-infectious and antineoplastic characteristics of the omentum. Does the omentum also have a detectable anti-atherosclerotic factor? If such a substance could be identified, it might have clinical potential. University School of Medicine, Boston, Massachusetts 02118, USA Boston
Anaesthesia and
myotonic dystrophy
SIR,-Your medicolegal correspondent has discussed (Oct 28,
1053) the
that may have led
p
the death of a patient with myotonic dystrophy given a general anaesthetic for tendon transfer surgery. We would like to suggest an alternative sequence of events. Patients with myotonic dystrophy are prone to periods of apnoea during sleep.1-4 Apnoea may also occur during relaxed wakefulness4 and is exacerbated by anaesthetic agents such as thiopentone.5 As Mrs Brahams points out, many anaesthetic agents can worsen respiratory function in patients with myotonic dystrophy. It is possible that a combination of a barbiturate induction with subsequent inhalational maintenance, coupled with what was a reasonable dose of a narcotic analgesic, exacerbated the tendency to apnoea, leading to prolonged hypoxia and cardiac arrest. Aspiration of gastric contents could have happened after rather than before the events
to
cardiorespiratory arrest. In these circumstances it would be illogical to recommend electrocardiographic monitoring. The most effective method for preventing further problems postoperatively in patients with myotonic dystrophy would be to concentrate on respiratory monitoring. We have been involved in the care of two such patients who underwent surgical procedures under general anaesthetic before myotonic dystrophy had been diagnosed, presented as "failure to reverse" and required prolonged ventilatory support. Another similar case has recently been described,6 and in that case prolonged apnoea occurred only after a second general anaesthetic, recovery after the first having been normal. We would recommend that all patients with myotonic dystrophy undergoing anaesthesia should be carefully monitored. Monitoring should include pulse oximetry and, where possible, some measure of respiratory rate and depth (with, for example, a ’Respitrace’ monitor). Electrocardiographic monitoring may reveal no abnormalities in these patients until hypoxic cardiac arrest has occurred. Whipps Cross Hospital, London E11
J. H. COAKLEY
Department of Medicine, University of Liverpool, Liverpool L69 3BX, UK
P. M. A. CALVERLEY
1. Coccagna
G, Mantovani M, Parchi L, et al. Alveolar hypoventilation and hypersomnia in myotonic dystrophy. J Neurol Neurosurg Psychiatry 1975; 38: 977-84. 2 Cadieux RJ, Kalesa A, McGlynn TJ, Jackson D, Manders EK, Simmonds MA. Sleep apnoea precipitated by pharyngeal surgery in a patient with myotonic dystrophy. Arch Otolaryngol 1984; 110: 611-13. 3. Veale D, Cooper BG, Gilmartin JJ, et al. Breathing during sleep in patients with myotonic dystrophy and non myotonic respiratory muscle weakness. Thorax 1986; 41: 710. 4. Coakley JH, Edwards RHT, Calverley PMA. Mazindol reduces sleep apnoea in myotonic dystrophy. Eur J Clin 1989; 19: A50. 5. Kaufmann L. Anaesthesia in dystrophia myotonica. Proc Roy Soc Med 1963; 153: 183-88. 6. Moore JK, Moore AP. Postoperative complications of dystrophia myotonica. Anaesth 1987; 42: 529-33.
HARRY S. GOLDSMITH
HS, Chen WF, Duckett SW. Brain vascularization by intact omentum. Arch Surg 1973; 106: 695-98. 2. Goldsmith HS, McIntosh T, Vezina RM, Colton T. Vasoactive neurochemicals identified in omentum. Br J Neurosurg 1987; 1: 359-64. 3. Goldsmith HS, Marquis JK, Siek G. Choline acetyltransferase activity in omental tissue. Br J Neurosurg 1987; 1: 463-66. 4. Siek GC, Marquis JK, Goldsmith HS. Experimental studies of omentum-derived neurotrophic factors. In: Goldsmith HS, ed. The omentum: research and clinical applications. London: Royal Society of Medicine, 1990: 83-95. 1. Goldsmith
Development of multiple sclerosis in patient on long-term sulfasalazine SIR,-Preliminary data on the suppression of experimental allergic encephalomyelitis (EAE) by sulfasalazinel have prompted interest in the value of this drug in the treatment of multiple sclerosis (MS).2 A German-Swiss multicentre study is planned. We report on a patient in whom, during 7 years of treatment with sulfasalazine, MS
developed. A 41-year-old man presented with progressive sensory symptoms of the extremities and trunk and impotence over 2 weeks. Ulcerative colitis had been diagnosed 10 years earlier. He had been treated with 3 g sulfasalazine per day for 7 years but over the past 6 months he had reduced daily intake to 1 -5 g because of disease inactivity for more than 2 years. In 1988 he had an intervertebral disc operation. Neurological examination, visual evoked potentials, and oligoclonal bands in the cerebrospinal fluid pointed to MS. Magnetic resonance imaging (MRI) of the brain disclosed multiple hyperintense lesions in T2-weighted images but no gadolinium enhancement, and in the spinal cord two elongated cervical lesions were found, one showing gadolinium enhancement. Corticosteroid therapy was accompanied by marked amelioration. The patient returned to sulfasalazine 3 g daily. 1 month later sensory symptoms of the arms waxed and waned after tapering of the steroid medication and spinal MRI disclosed a new, gadolinium-enhancing thoracic lesion. Clinical examination and tests pointed to dissemination in space. Dissemination in time, which is required to fulfil the classical diagnostic criteria for MS, was suggested by the coexisting gadolinium enhancing and non-enhancing lesions on MRI;3 disclosure of a new thoracic lesion in the second spinal MRI further confirmed the diagnosis.’ The coincidence of MS and inflammatory bowel diseases has been described,5-7 and shared susceptibility genes or a generalised immune disorder, rather than cause and effect, have been discussed as possible aetiological factors. Since in this case colitis had been inactive for the previous 2 years, a causal relation between this man’s neurological findings and his bowel disease is unlikely. Nor is the reduction in sulfasalazine dose likely to be significant since even the reduced dosage (1’5g daily) is not far below the recommended maintenance dosage of 2 g. Furthermore the new MRI spinal lesion developed under a sulfasalazine dosage of 3 g.
