- Email: [email protected]
Budd-Chiari syndrome
115
Although much of the pioneering work in service development and the research that underpins this approach was conducted in India, it looks as though Pakistan will be the first to produce a substantial change in its mental illness services, since there seems a greater willingness to grasp the principle that mental health is a major public health issue. Mental health programmes must attract funds in the same way as other major programmes, and must be pursued with
equal vigour. Mental Illness Research Unit, University of Manchester, UK
David
part of the problem, Queensland is proposing a new dam that will rob northern NSW of further water. Lastly there are sectional interests at play. Many of the cotton farms of northern NSW, which do most of the irrigating, are owned by some of the country’s largest agricultural companies, and they have so far been ominously silent on the topic of algae, despite the protests of some small farmers in the region. And the long-awaited rain may prove to be a mixed blessing because it has washed the problem off the front pages and out of the politicians’ minds.
Goldberg
N, Murthy R, Harding T. A model for rural psychiatric services—Raipur Rani Experience. Indian J Psychiatrty 1981; 23:
Nick Lush
1. Wig
275-90. 2. Nizamie S, Sharma
S, Palit C. National Mental Health Programme—a progress report (1982-1990). New Delhi: Director General of Health Services, 1991. 3. Isaac M. Collaborative study on severe mental morbidity. New Delhi: Indian Council of Mental Health, 1987. 4. Goldberg D. Pakistan: a quantum leap for community psychiatry. Lancet 1987; ii: 1445-46.
Australia: Summer
brings toxic vengeance
The arrival of summer here coincides neatly with Christmas and the holiday season, but this year it has brought a nastier companion-river pollution by toxic blue-green algae. Australia’s largest and most valuable river system, the Murray/Darling, is threatened. The system rises in southern Queensland, covers most of western New South Wales and northern Victoria, and stretches into southeastern South Australia. Water polluted by the algae kills stock and native animals and causes vomiting, diarrhoea, and rashes in man. It has to be carbon-filtered to be made safe for drinking. The threat it poses to public health has led to the army being called in to filter water for affected towns. Toxic algal pollution is not new. Predictions of an attack as serious as the present one were made ten years ago by Prof Ian Falconer of the University of New England (NSW). A critical combination of weather and human interventions in natural processes was all that was needed. The human interventions were there in the form of phosphorus in sewage discharged from the 170 treatment plants throughout the river system, run-off from farms and feed-lots, carrying fertiliser and animal wastes, and of excessive irrigation, especially by cotton farmers, cutting river flow. The missing link-drought-arrived this winter. When summer arrived the water was rich in nutrients, especially phosphorus, and hardly flowing. A few weeks of sun and still no rain meant perfect conditions for the poisonous algae to spread in larger and larger patches from southern Queensland to the rivers’ emptying point in Lake Alexandrina, South Australia. Since then some rain has arrived. Enough, insists the Deputy Premier of NSW, Wal Murray, to "wash it all out to sea". But environmental critics do not agree and predict a recurrence of the problem before the summer is out. As Australians in the eastern states--especially those in the country, most of whom are already in their worst economic plight since the 1939-45 war-wait to see whether the algae rise again, everybody is asking why nothing has been done. In part, the answer seems to lie in the massive bureaucracy governing water resources. In NSW alone the Murray/ Darling system is overseen by seven bodies, which seem unable to coordinate their activities. Interstate rivalry is another factor. Despite knowledge that poor river flow is
Conference Budd-Chiari
syndrome
Budd-Chiari
syndrome, a syndrome of congestive hepatomegaly, portal hypertension, ascites, and varying degrees of liver failure, results from occlusion of hepatic venous drainage channels. When the occlusion is primarily in the cava or is associated with secondary caval obstruction due to caudate-lobe (segment I) hypertrophy, then renal impairment and, in the more chroniccases, dilated anastomatic veins in the anterior abdominal wall develop. Since the occlusion can come about in several different ways, there are different presentations, many different therapeutic options, and considerable confusion about management. An international conference held in Kyoto, Japan, in October produced more questions than answers, but it identified clearly the current state of knowledge and the need for further carefully focused studies. In the Orient and India the major cause of the syndrome is a "caval web" (a membrane, stricture, or coarctation) associated with varying degrees of thrombosis and obstruction affecting some or all of the hepatic veins and the inferior vena cava. This can vary in severity from a localised constriction to total caval occlusion stretching from the right atrium to the level of the renal veins. It results in a comparatively chronic form of the syndrome that is not immediately life-threatening. The aetiology is disputed: some favour the theory of thrombosis and organisation, others a congenital origin based on non-union or malunion of veins during the development of the hepatic veins and cava.
In the west the disease usually results from hepatic venous thrombosis, but in many cases the exact cause is not known. Recent studies suggest an underlying occult, iatrogenic, or myeloproliferative thrombotic tendency in most cases. This type of blockage of the hepatic venous drainage results in an acute illness, which may occasionally improve spontaneously or become chronic but is more likely to result in early death if untreated.
Treatment varies from conservative medical measures through interventional radiological techniques and a variety of surgical vascular bypass and decompression procedures to liver transplantation. Treatment must be tailored to the site of the obstruction and the degree of liver failure for each patient. In acute cases this must be designed to promote hepatic sinusoidal decompression, so the exact site of the causative vascular obstruction must be clearly identified. Ultrasound visualisation of the hepatic veins and the inferior vena cava is essential for diagnosis. Other diagnostic tests such as computed tomographic and magnetic resonance imaging scans, functional hepatograms, hepatic
116
and caval angiography, liver function tests, and liver biopsy contribute to decisions on management. For the patient presenting with western type acute occlusion thrombolytic therapy may be successful if given at a very early stage. If the patient’s condition deteriorates rapidly from progressive secondary hepatocellular damage, then early liver transplantation may be necessary, although there is a view that progressive liver cell failure can be reversed if the sinusoids can be acutely decompressed by means of a side-to-side portosystemic shunt or a mesocaval shunt. For either shunt to work it must drain into a low-pressure system. Theoretically, the inferior vena cava below the liver is such a system, but the pressure at this point can be high owing to compression of the hypertrophied caudate lobe (segment I). Measurement of infrahepatic caval pressure is thus mandatory, either before or at the time of surgery. If the pressure is only of the same magnitude as the portal pressure then either the shunt must be connected to an alternative low-pressure venous system above the liver or the caval obstruction must be overcome by creating a shunt between the superior mesenteric vein or portal vein and the right atrium or innominate vein. Unfortunately this procedure is associated with a high thrombosis rate (up to 30%), and a cavo-porto-atrial shunt may carry less risk. Relief of caval strictures can be achieved either by means of expanding metal stents inserted into the narrowed section of the cava with a percutaneous transfemoral vein puncture technique or by means of a surgical bypass procedure on the cava carried out at the same time as the portal decompression
operation. There is general agreement that in the more chronic eastern forms of the disease treatment should be directed at attempting to relieve the major venous obstructions. In Japan, surgeons undertake web splitting (with a finger or instrument introduced into the upper cava through the right atrium), venacaval-atrial bypass procedures using prosthetic shunts, or complex operations designed to open the cava and hepatic veins, remove thrombus, and then reanastomose them to the right atrium. Interventional radiologists offer balloon angioplasty, followed by insertion of expanding metal stents into or through the occluded cava and hepatic veins. Unfortunately comparative studies of these different approaches have not been done. The value of liver transplantation in the management of Budd-Chiari syndrome is hotly debated, but the cause of the disease is a major deciding factor. If untreated, the syndrome carries a bad prognosis, especially in the western type when progressive liver failure develops. Transplantation carries a 30% mortality, but long-term survivors (up to 17 years) are also known. However, there are reports of similar survival periods either with no
following appropriate decompression surgery. transplant surgeons have difficulty in producing convincing evidence to support their chosen form of treatment, at least in the early stages of the disease before significant hepatocellular failure develops. There is some agreement that if initial decompression surgery fails and the patient’s liver function continues to deteriorate then transplantation is indicated. treatment or
Thus
After all the debate where do
we
stand? The numbers of
patients with the syndrome, especially in the west, are probably too small to justify controlled prospective studies, except multicentre ones, which seem to be ruled out at present because of the enthusiasm of each clinician for his own approach. It is probable that personal prejudices of the clinicians involved will continue to dictate treatment.
Another congress will be held in three years’ time, in London. Then an attempt will be made to classify the disease and identify for each type a dedicated programme of management. That may be a start to establishing a rational approach to this complex clinical problem. University Department of Surgery, Royal Free Hospital and School of Medicine, London NW3 2QG, UK
K. E. F. HOBBS
Medicine and the Law Anaesthesia
awareness: an
orthopaedic case
A case in which the health authority had admitted liability but the sum to be paid to the plaintiff as compensation had not been agreed came before Mr Justice Schiemann in the High Court on Oct 18, 1991. The plaintiff had required surgery on his leg that involved deep cutting and drilling down to the bone. He was told that he would have an injection and that when he woke up the operation would be over. Unfortunately, consciousness returned when he was in the operating theatre; he could hear nurses talking and was aware of a tube down his throat. He had been given a muscle relaxant drug but he wiggled his toes in an attempt to draw attention to the fact that he was awake. The nurses remarked on the fact that the patient’s toes were moving but a man’s voice said it was "just reflexes" and that the nurses should ignore it. Somebody then grasped his leg and applied a tourniquet to his groin, and he heard a man say that this should be removed after 541 minutes. It was then that the patient realised that the operation, far from being over, was only just about to start, and that he was conscious and sensitive to pain but unable to move.
What then followed was nightmarish. The plaintiff experienced a burning pain to his leg from the scalpel, and four holes were drilled into the bone. He was artificially ventilated. He felt very sick and stopped breathing in the hope that this would indicate that something was wrongbut the machine started "breathing for him". The judge accepted the plaintiffs account of his experience and its aftermath. The failure to agree damages had meant that the plaintiff had concentrated on his operation in a way that he might not have done had agreement been reached. The plaintiff had started recalling his operation when in bed, perhaps because he was lying down as he had been in the operating-theatre, so the consequences "include the possibility that he may have to relive the experience". There was no conventional figure for compensation in cases such as this. In Ackers v Wigan Area Health Authority (19912 Med LR 232) damages were awarded for insufficient anaesthesia during a caesarean section. However, there were significant differences between the two cases, the judge said. For the experience on the operating table the judge awarded the man /J5000, and in general damages for pain, suffering, and loss of amenity he awarded 10 000. In Ackers the pain had not been as acute as that here but it lasted longer. That patient had been awarded k 12 000 (plus C 1775 in respect of treatment for consequent psychiatric illness). Phelan
0’
Cumbria Health Authority
(QBD Schiemann V). Med L R [1991]
419.
Diana Brahams
Although much of the pioneering work in service development and the research that underpins this approach was conducted in India, it looks as though Pakistan will be the first to produce a substantial change in its mental illness services, since there seems a greater willingness to grasp the principle that mental health is a major public health issue. Mental health programmes must attract funds in the same way as other major programmes, and must be pursued with
equal vigour. Mental Illness Research Unit, University of Manchester, UK
David
part of the problem, Queensland is proposing a new dam that will rob northern NSW of further water. Lastly there are sectional interests at play. Many of the cotton farms of northern NSW, which do most of the irrigating, are owned by some of the country’s largest agricultural companies, and they have so far been ominously silent on the topic of algae, despite the protests of some small farmers in the region. And the long-awaited rain may prove to be a mixed blessing because it has washed the problem off the front pages and out of the politicians’ minds.
Goldberg
N, Murthy R, Harding T. A model for rural psychiatric services—Raipur Rani Experience. Indian J Psychiatrty 1981; 23:
Nick Lush
1. Wig
275-90. 2. Nizamie S, Sharma
S, Palit C. National Mental Health Programme—a progress report (1982-1990). New Delhi: Director General of Health Services, 1991. 3. Isaac M. Collaborative study on severe mental morbidity. New Delhi: Indian Council of Mental Health, 1987. 4. Goldberg D. Pakistan: a quantum leap for community psychiatry. Lancet 1987; ii: 1445-46.
Australia: Summer
brings toxic vengeance
The arrival of summer here coincides neatly with Christmas and the holiday season, but this year it has brought a nastier companion-river pollution by toxic blue-green algae. Australia’s largest and most valuable river system, the Murray/Darling, is threatened. The system rises in southern Queensland, covers most of western New South Wales and northern Victoria, and stretches into southeastern South Australia. Water polluted by the algae kills stock and native animals and causes vomiting, diarrhoea, and rashes in man. It has to be carbon-filtered to be made safe for drinking. The threat it poses to public health has led to the army being called in to filter water for affected towns. Toxic algal pollution is not new. Predictions of an attack as serious as the present one were made ten years ago by Prof Ian Falconer of the University of New England (NSW). A critical combination of weather and human interventions in natural processes was all that was needed. The human interventions were there in the form of phosphorus in sewage discharged from the 170 treatment plants throughout the river system, run-off from farms and feed-lots, carrying fertiliser and animal wastes, and of excessive irrigation, especially by cotton farmers, cutting river flow. The missing link-drought-arrived this winter. When summer arrived the water was rich in nutrients, especially phosphorus, and hardly flowing. A few weeks of sun and still no rain meant perfect conditions for the poisonous algae to spread in larger and larger patches from southern Queensland to the rivers’ emptying point in Lake Alexandrina, South Australia. Since then some rain has arrived. Enough, insists the Deputy Premier of NSW, Wal Murray, to "wash it all out to sea". But environmental critics do not agree and predict a recurrence of the problem before the summer is out. As Australians in the eastern states--especially those in the country, most of whom are already in their worst economic plight since the 1939-45 war-wait to see whether the algae rise again, everybody is asking why nothing has been done. In part, the answer seems to lie in the massive bureaucracy governing water resources. In NSW alone the Murray/ Darling system is overseen by seven bodies, which seem unable to coordinate their activities. Interstate rivalry is another factor. Despite knowledge that poor river flow is
Conference Budd-Chiari
syndrome
Budd-Chiari
syndrome, a syndrome of congestive hepatomegaly, portal hypertension, ascites, and varying degrees of liver failure, results from occlusion of hepatic venous drainage channels. When the occlusion is primarily in the cava or is associated with secondary caval obstruction due to caudate-lobe (segment I) hypertrophy, then renal impairment and, in the more chroniccases, dilated anastomatic veins in the anterior abdominal wall develop. Since the occlusion can come about in several different ways, there are different presentations, many different therapeutic options, and considerable confusion about management. An international conference held in Kyoto, Japan, in October produced more questions than answers, but it identified clearly the current state of knowledge and the need for further carefully focused studies. In the Orient and India the major cause of the syndrome is a "caval web" (a membrane, stricture, or coarctation) associated with varying degrees of thrombosis and obstruction affecting some or all of the hepatic veins and the inferior vena cava. This can vary in severity from a localised constriction to total caval occlusion stretching from the right atrium to the level of the renal veins. It results in a comparatively chronic form of the syndrome that is not immediately life-threatening. The aetiology is disputed: some favour the theory of thrombosis and organisation, others a congenital origin based on non-union or malunion of veins during the development of the hepatic veins and cava.
In the west the disease usually results from hepatic venous thrombosis, but in many cases the exact cause is not known. Recent studies suggest an underlying occult, iatrogenic, or myeloproliferative thrombotic tendency in most cases. This type of blockage of the hepatic venous drainage results in an acute illness, which may occasionally improve spontaneously or become chronic but is more likely to result in early death if untreated.
Treatment varies from conservative medical measures through interventional radiological techniques and a variety of surgical vascular bypass and decompression procedures to liver transplantation. Treatment must be tailored to the site of the obstruction and the degree of liver failure for each patient. In acute cases this must be designed to promote hepatic sinusoidal decompression, so the exact site of the causative vascular obstruction must be clearly identified. Ultrasound visualisation of the hepatic veins and the inferior vena cava is essential for diagnosis. Other diagnostic tests such as computed tomographic and magnetic resonance imaging scans, functional hepatograms, hepatic
116
and caval angiography, liver function tests, and liver biopsy contribute to decisions on management. For the patient presenting with western type acute occlusion thrombolytic therapy may be successful if given at a very early stage. If the patient’s condition deteriorates rapidly from progressive secondary hepatocellular damage, then early liver transplantation may be necessary, although there is a view that progressive liver cell failure can be reversed if the sinusoids can be acutely decompressed by means of a side-to-side portosystemic shunt or a mesocaval shunt. For either shunt to work it must drain into a low-pressure system. Theoretically, the inferior vena cava below the liver is such a system, but the pressure at this point can be high owing to compression of the hypertrophied caudate lobe (segment I). Measurement of infrahepatic caval pressure is thus mandatory, either before or at the time of surgery. If the pressure is only of the same magnitude as the portal pressure then either the shunt must be connected to an alternative low-pressure venous system above the liver or the caval obstruction must be overcome by creating a shunt between the superior mesenteric vein or portal vein and the right atrium or innominate vein. Unfortunately this procedure is associated with a high thrombosis rate (up to 30%), and a cavo-porto-atrial shunt may carry less risk. Relief of caval strictures can be achieved either by means of expanding metal stents inserted into the narrowed section of the cava with a percutaneous transfemoral vein puncture technique or by means of a surgical bypass procedure on the cava carried out at the same time as the portal decompression
operation. There is general agreement that in the more chronic eastern forms of the disease treatment should be directed at attempting to relieve the major venous obstructions. In Japan, surgeons undertake web splitting (with a finger or instrument introduced into the upper cava through the right atrium), venacaval-atrial bypass procedures using prosthetic shunts, or complex operations designed to open the cava and hepatic veins, remove thrombus, and then reanastomose them to the right atrium. Interventional radiologists offer balloon angioplasty, followed by insertion of expanding metal stents into or through the occluded cava and hepatic veins. Unfortunately comparative studies of these different approaches have not been done. The value of liver transplantation in the management of Budd-Chiari syndrome is hotly debated, but the cause of the disease is a major deciding factor. If untreated, the syndrome carries a bad prognosis, especially in the western type when progressive liver failure develops. Transplantation carries a 30% mortality, but long-term survivors (up to 17 years) are also known. However, there are reports of similar survival periods either with no
following appropriate decompression surgery. transplant surgeons have difficulty in producing convincing evidence to support their chosen form of treatment, at least in the early stages of the disease before significant hepatocellular failure develops. There is some agreement that if initial decompression surgery fails and the patient’s liver function continues to deteriorate then transplantation is indicated. treatment or
Thus
After all the debate where do
we
stand? The numbers of
patients with the syndrome, especially in the west, are probably too small to justify controlled prospective studies, except multicentre ones, which seem to be ruled out at present because of the enthusiasm of each clinician for his own approach. It is probable that personal prejudices of the clinicians involved will continue to dictate treatment.
Another congress will be held in three years’ time, in London. Then an attempt will be made to classify the disease and identify for each type a dedicated programme of management. That may be a start to establishing a rational approach to this complex clinical problem. University Department of Surgery, Royal Free Hospital and School of Medicine, London NW3 2QG, UK
K. E. F. HOBBS
Medicine and the Law Anaesthesia
awareness: an
orthopaedic case
A case in which the health authority had admitted liability but the sum to be paid to the plaintiff as compensation had not been agreed came before Mr Justice Schiemann in the High Court on Oct 18, 1991. The plaintiff had required surgery on his leg that involved deep cutting and drilling down to the bone. He was told that he would have an injection and that when he woke up the operation would be over. Unfortunately, consciousness returned when he was in the operating theatre; he could hear nurses talking and was aware of a tube down his throat. He had been given a muscle relaxant drug but he wiggled his toes in an attempt to draw attention to the fact that he was awake. The nurses remarked on the fact that the patient’s toes were moving but a man’s voice said it was "just reflexes" and that the nurses should ignore it. Somebody then grasped his leg and applied a tourniquet to his groin, and he heard a man say that this should be removed after 541 minutes. It was then that the patient realised that the operation, far from being over, was only just about to start, and that he was conscious and sensitive to pain but unable to move.
What then followed was nightmarish. The plaintiff experienced a burning pain to his leg from the scalpel, and four holes were drilled into the bone. He was artificially ventilated. He felt very sick and stopped breathing in the hope that this would indicate that something was wrongbut the machine started "breathing for him". The judge accepted the plaintiffs account of his experience and its aftermath. The failure to agree damages had meant that the plaintiff had concentrated on his operation in a way that he might not have done had agreement been reached. The plaintiff had started recalling his operation when in bed, perhaps because he was lying down as he had been in the operating-theatre, so the consequences "include the possibility that he may have to relive the experience". There was no conventional figure for compensation in cases such as this. In Ackers v Wigan Area Health Authority (19912 Med LR 232) damages were awarded for insufficient anaesthesia during a caesarean section. However, there were significant differences between the two cases, the judge said. For the experience on the operating table the judge awarded the man /J5000, and in general damages for pain, suffering, and loss of amenity he awarded 10 000. In Ackers the pain had not been as acute as that here but it lasted longer. That patient had been awarded k 12 000 (plus C 1775 in respect of treatment for consequent psychiatric illness). Phelan
0’
Cumbria Health Authority
(QBD Schiemann V). Med L R [1991]
419.
Diana Brahams