548
Proc. roy. Soc. Med. Volume 70 August 1977
Na loss and inappropriate ADH secretion. In addition, he had weakness, drowsiness, disorientation and epilepsy but not the pyramidal or extapyramidal signs described in that series. The generalized diffuse EEG abnormalities in this patient were similar to those in the Belgian study but no further fits occurred after correction of his biochemical status. While the EEG reverted to normal within eight months in this patient, Demanet et al. (1971) reported an abnormal EEG in 4 out of 5 cases even after correction of electrolyte imbalance. In a Danish series of 5 cases of electrolyte disturbances in beer drinkers (Hilden & Svendsen 1975) hyponatrxmia, hypokalemia and hypo-osmolality were considered to be due to low sodium content of beer and a poor dietary intake of sodium and potassium. The urine osmolalities of their patients were low which indicated that inappropriate ADH secretion was not present in their series. The etiology of myoneuropathy in our case was probably complex and due to a combination of alcohol and dietary deficiency of vitamin B1 and folate. The patient recovered from a variety of serious complications of beer drinking in a combination which has not previously been described and with some of the clinical features of a cobalt cardiomyopathy but without significant levels of cobalt being found in the beers. REFERENCES Champion H R, Caplan Y H, Baker S P, Long W B, Benner C, Cowley R A, Fisher R & Gill W (1975) Lancet i, 1402 Demanet J C, Bonnyns M, Bleiberg H & Stevens-Roemans C (1971) Lancet ii, 1115 Dorwart W V & Chalmers L (1975) Clinical Chemistry 21, 190 Duckham J M & Lee H A (1976) Quarterly Journal of Medicine 45, 277 Evans W (1964) Progress in Cardiovascular Diseases 7, 151 Hilden T & Svendsen T L (1975) Lancet ii, 245 Jacobs M B (1967) The Analytical Toxicology of Industrial Inorganic Poisons, Interscience Chemical Analysis Vol 22. Interscience/John Wiley, New York &c.; p 453 Kesteloot H, Roelandt J, WiHems J, Claes J H & Joossens J V (1968) Circulation 37, 854 Lapointe A (1967) Canadian Medical Association Journal 97, 905 McAllen P M (1954) British Heart Journal 17, 5 McCance R A & Widdowson E M (1973) The Composition of Foods. Medical Research Council Series 297 McDermott P H, Delaney R L, Egan J D & Sullivan J F (1966) Journal of the American Medical Association 198, 163 Morin Y (1967) Canadian Medical Association Journal 97, 901 Morin Y, Tetu A & Mercier G (1971) British Heart Journal 33, 175 Moses A M & Miller M (1974) New England Journal of Medicine 291, 1234 Nadeau G & Milon H (1967) Canadian Medical Association Journal 97, 889
Regan T J (1971) Circulation 44, 957 Regan T J, Koroxenidis G, Moschos C B, Oldewurtel H A, Lehan P H & Hellems H K (1966) Journal of Clinical Investigation 45, 270 Regan T J, Levinson G E, Oldewurtel H A, Frank M J, Weisse A B & Moschos C B (1969) Journal of Clinical Investigation 48, 397 Schrire V & Gant J (1959) South AJrican Journal of Laboratory and Clinical Medicine 5, 195 Woods H F (1974) Symposium on Advanced Medicine (London) 10, 53
Juvenile Dermatomyositis with Complications J Winfield MB MRCP (for J M Gumpel MRCP) (Northwick Park Hospital, Harrow, Middlesex) A 14-year-old schoolboy (R B), referred by Dr T J Goodwin, was admitted to hospital in May 1976 with a one week history of malaise, muscle pain and weakness. Mild facial erythema had been noted after exposure to sun three weeks prior to admission. On admission he had a fever of 37.8°C; a marked erythematous facial rash with periorbital (edema was present, which extended onto his trunk and limbs. His throat and buccal mucosa were ulcerated, and infectious mononucleosis was considered a possible diagnosis. Over two days facial and buccal cedema became pronounced with the development of excoriated skin lesions around his mouth and eyelids. His face, neck and chest wall became cedematous and then brawny and indurated. The diagnosis of acute dermatomyositis was based on the clinical presentation and characteristic skin rash and was confirmed by skin and muscle biopsy. The creatine phosphokinase (CPK) level was 1240 iu/l rising to 15 000 iu/l one week after admission. Prednisolone 40 mg a day was started and over seven days the skin rash and fever responded and muscle pain decreased. However, he gained 3 kg in weight over two days and became hypertensive with a blood pressure of 150/100 mmHg and the periorbital cedema became much more marked. Diuretics were given to reduce his fluid overload. Later his general condition deteriorated with diffuse abdominal pain, and he had a hTmetamesis of some 300 ml ofaltered blood and a melaena and he became shocked. He improved with transfusion, but soon after there was an insidious change in respiration which became progressively more shallow and he became anoxic. He was admitted to the intensive care unit at Northwick Park Hospital in a moribund condition, when his respiratory rate was 60 per minute with a tidal volume estimated to be 20 ml. He was
Clinical Section
549
Fig 1 X-ray showing chest wall and pleural a?dema
sedated, intubated and ventilated and full sup- barium examination, and some barium was seen to portive therapy given with further blood trans- spill into the trachea. As he improved he complained of impaired fusions. Within two hours on this treatment his general condition was improved and his blood vision in the right eye. A large central scotoma in pressure rose to 120/60. The prednisolone dosage the right eye and two small scotomata in the left was increased to 150 mg a day and within two days eye were found; extensive exudates were seen there was marked resolution of his cedema and skin located particularly around the macular region in rash, although chest wall inflammation and cedema both eyes but changes were more marked in the continued to splint the chest and produced depres- right eye (Fig 2). These had not been seen on sion of the lung apices (Fig 1). Decubitus ulcers admission to Mount Vernon Hospital and to the developed despite careful nursing on a water bed intensive care unit: it was likely the retinopathy with sheepskin protection. Contractures were pre- had developed quickly over two to three days. His vented with light plaster of Paris splints for his legs blood pressure during this time was normal at and he was given passive physiotherapy. 110/75 mmHg. His prednisolone dosage at this After five days in the intensive care unit he was time was still 100 mg a day. During the next six weeks his general condition extubated but was still unable to tolerate oral fluids because of nasal regurgitation. He was able to swallow semi-solid food and to take oral prednisolone. The prednisolone dosage was decreased to 100 mg a day with his clinical improvement but progress was delayed by a right basal pneumonia and septicemia. Staphylococcus aureus was isolated from sputum, blood cultures and the subclavian infusion site and catheter. He responded well to intravenous flucloxacillin and oral probenecid, but again became intolerant of fluids and had abdominal distension. He passed two large melena stools. Upper gastrointestinal tract endoscopy (Dr A J Levi) revealed a normal stomach: two large superficial serpiginous ulcers were seen in the duodenum. Recent bleeding was evident and therefore no biopsy was taken. The lower duodenum was seen to be normal; it was possible that there were also ileal ulcers. Acute gastric dilation occurred and he was so intolerant of fluids for five days that full intravenous feeding was started. The Fig 2 Retinopathy in acute dermatomyositis. Two of the upper cesophagus was markedly abnormal on more discrete lesions are indicated
550
Proc. roy. Soc. Med. Volume 70 August 1977
steadily improved. Most of his skin cedema re- Discussion solved quickly and the skin rash healed pro- A number of serious complications of acute gressively, with some residual scarring over his juvenile dermatomyositis are exhibited by this eyelids, the dorsum of both hands, knees and case. The insidious onset of respiratory and palatal elbows. The biopsy site and infusion sites began to muscle weakness is frequently difficult to recogheal. His muscular power improved progressively nize, while weakness severe enough to require and his vision was back to normal within two assisted ventilation is relatively uncommon and in weeks of the initial retinal changes. His steroid this boy was only just detected in time. Regular dosage was gradually reduced successively and he tidal volume measurements might have detected developed no further complications apart from a this complication earlier. Involvement of the hypomild flare in erythema of his face which quickly pharyngeal musculature with the development of subsided. It was noted that he had a weak voice dysphagia, regurgitation or aspiration is common and still has, six months after his illness. This is and probably of prognostic significance in that 5 of probably related to intrinsic laryngeal muscle the 6 patients with this complication reported by weakness. His palatal weakness improved but he Everett & Curtis (1957) died. Measures to prevent still complains of slight difficulty in swallowing, aspiration and subsequent infection are essential. particularly with fluids. Intermittent episodes of Weakness of these muscles as a critical factor in vague abdominal pain continued with no severe survival has also been stressed by Wedgwood et al. flare for four to six weeks, but this gradually (1953) who found weakness in muscles of resubsided without further melena with return to a spiratory and deglutition in 13 of 26 children studied; this was considered to be a significant normal diet at two months. Six months after his illness he remains well, is factor in the fatal outcome of at least 5 children in back at school and he is increasing his general their series. Ulceration of any part of the gastrointestinal activities to include sports. His muscle enzymes have been monitored, particularly creatine kinase, tract may occur and Banker & Victor (1966) have and have shown a steady fall and this goes along stressed the role of vasculitis in the development of with his clinical improvement (Fig 3). His pred- these lesions. Extensive ulceration of the gastronisolone dosage has been reduced progressively. intestinal tract, with vasculitis as the pathological His skin rash has continued to improve steadily basis for these lesions, was well described even with residual scarring over his upper eyelids with a before the use of steroid therapy and therefore purplish discoloration around his eyes. There is a must be regarded as a primary feature of the marked degree of dermal atrophy on his hands disease and not simply as a complication of steroid with early scarring over the dorsum of both hands, therapy (Walton & Adams 1958). High dose predparticularly located over the metacarpophalangeal nisone may mask the clinical signs of perforation in and proximal interphalangeal joints. There is also these patients and it is often difficult to exclude purplish discoloration of the skin over the length perforation without repeated abdominal plain of the fingers. Residual rash on his knees, elbows X-rays. Retinopathy in dermatomyositis is rare and the and venepuncture sites has healed with keloid formation. He was previously known to develop majority of cases have been reported in children. keloid and scars and this is an unfortunate ad- The mechanism is probably vasculitis with capilditional complication. To date he has no contrac- lary damage and retinal ischimia. In this case the short episode of hypertension as an additional tures or evidence of calcinosis. factor in the development of retinopathy cannot be overlooked. The retinopathy in this boy may have developed three weeks after his initial symptoms but resolved quickly in two to three days with no Haemoglobin , subsequent visual impairment. Retinal photoMelaena 9/lOOml graphs (Fig 2) showed extensive exudates located 15009 in the paramacular region in both eyes with the Creatine typical appearance of cotton wool exudates, charphosphokinase 100] acteristic of those previously described (Bruce iu/I 50 0 1938, Harrison et al. 1973, Finman et al. 1976). This case is of particular interest because visual acuity returned to normal within two to three days Prednisolone A despite the persistence of extensive exudates; a mgmslIday possible mechanism may be resolution of macular cedema. The retinopathy may lead to significant july dec 1976 visual impairment, and optic atrophy has been reported. Repeat retinal photographs (Fig 4), Fig 3 Progress of R B and response to steroids ,s
-
5
1-M
1501
00
may
june
aug
sept
oct
Clinical Section
~ ~. : 551
On examination the abdomen was slightly distended and there was a large 'cystic' mass in the central epigastrium extending below the navel. The upper margin was indistinguishable from the costal margin, the mass was dull to percussion. A provisional diagnosis of hydatid disease was made. Hematological and biochemical indices were normal. A Casoni test was negative as was a hydatid complement fixation test. Barium swallow and meal showed a mass in the abdomen displacing the lower half of the stomach downwards and causing some compression of the duodenal cap. An hepatic scan with indium- 117 showed decreased uptake in the left lobe and lower half of the right lobe.
First operation (24.11.72, Professor H Ellis): The
Fig 4 Resolving retinopathy with pigmientation of retin'a taken two months after the onset of retinopathy showed complete resolution of the exudates but areas of pigmentation were also seen. Pigmentary changes where exudates were located has also been noted by Harrison et al. (1973) and Nutt (1951) and may reflect ischxemic damage to the choroid. REFERENCES Banker B Q & Victor M (1966) Medicine 45, 261-289 Bruce G M. (1938) Transactionts f the American Oph ofhaltological Society, 36, 282 Everett M A & Curtis A C (1957) Archives of Internal Medicine 100, 70-76 Finman L 5, Ragsdale C G, Sullivan D B & Petty R E 1976) Journal o Pcediateics88, 267 barrisonS M, Frenkel N, Grossman B J & Matalon R (1973) American Journal of Ophthalmology 76, 786 Nutt A B
(1951) Proceedings oj'the Royal Society oj'Medicine 44, 979 Walton J N & Adams R D
(1958) Polymyositis. E & S Livingstone, Edinburgh Wedgwood R P, Cook C D & Cohen J (1953) Pediatrics 12, 447-466
abdomen was explored through a midline epigastric excision. A thin-walled cyst was found measuring some 20 x 15 cm which occupied the inferior aspect of the liver and extended into the falciform ligament. The cyst was aspirated of two litres of clear bile and decapped. A small biliary fistula in the floor of the cyst was oversewn with thread sutures. The patient made an uneventful recovery. Histology of the cyst showed it to be lined by columnar epithelium, probably of bile duct origin. She remained well for two and half years. In July 1975 she returned with a three-month history of epigastric discomfort. On examination a cystic mass was again palpable in the right and left
*.,,,..::...: . .
Solitary Liver Cyst Treated by Cystogastrostomy M R Lock FRCS (Surgical Unit, Westminster Hospital, London S WI) Mrs E P, a 51-year-old housewife of mixed racial extraction from South Africa, presented in November 1972 with a four-day history of burning retrosternal pain, worse at night and on stooping. The pain was relieved by alkalis and she had had similar but less severe symptoms for two years previously. Her general health was good and there were no other complaints.
Fig 1 Plain abdominal X-ray showing soft tissue mass of recurrent cyst
Proc. roy. Soc. Med. Volume 70 August 1977
Na loss and inappropriate ADH secretion. In addition, he had weakness, drowsiness, disorientation and epilepsy but not the pyramidal or extapyramidal signs described in that series. The generalized diffuse EEG abnormalities in this patient were similar to those in the Belgian study but no further fits occurred after correction of his biochemical status. While the EEG reverted to normal within eight months in this patient, Demanet et al. (1971) reported an abnormal EEG in 4 out of 5 cases even after correction of electrolyte imbalance. In a Danish series of 5 cases of electrolyte disturbances in beer drinkers (Hilden & Svendsen 1975) hyponatrxmia, hypokalemia and hypo-osmolality were considered to be due to low sodium content of beer and a poor dietary intake of sodium and potassium. The urine osmolalities of their patients were low which indicated that inappropriate ADH secretion was not present in their series. The etiology of myoneuropathy in our case was probably complex and due to a combination of alcohol and dietary deficiency of vitamin B1 and folate. The patient recovered from a variety of serious complications of beer drinking in a combination which has not previously been described and with some of the clinical features of a cobalt cardiomyopathy but without significant levels of cobalt being found in the beers. REFERENCES Champion H R, Caplan Y H, Baker S P, Long W B, Benner C, Cowley R A, Fisher R & Gill W (1975) Lancet i, 1402 Demanet J C, Bonnyns M, Bleiberg H & Stevens-Roemans C (1971) Lancet ii, 1115 Dorwart W V & Chalmers L (1975) Clinical Chemistry 21, 190 Duckham J M & Lee H A (1976) Quarterly Journal of Medicine 45, 277 Evans W (1964) Progress in Cardiovascular Diseases 7, 151 Hilden T & Svendsen T L (1975) Lancet ii, 245 Jacobs M B (1967) The Analytical Toxicology of Industrial Inorganic Poisons, Interscience Chemical Analysis Vol 22. Interscience/John Wiley, New York &c.; p 453 Kesteloot H, Roelandt J, WiHems J, Claes J H & Joossens J V (1968) Circulation 37, 854 Lapointe A (1967) Canadian Medical Association Journal 97, 905 McAllen P M (1954) British Heart Journal 17, 5 McCance R A & Widdowson E M (1973) The Composition of Foods. Medical Research Council Series 297 McDermott P H, Delaney R L, Egan J D & Sullivan J F (1966) Journal of the American Medical Association 198, 163 Morin Y (1967) Canadian Medical Association Journal 97, 901 Morin Y, Tetu A & Mercier G (1971) British Heart Journal 33, 175 Moses A M & Miller M (1974) New England Journal of Medicine 291, 1234 Nadeau G & Milon H (1967) Canadian Medical Association Journal 97, 889
Regan T J (1971) Circulation 44, 957 Regan T J, Koroxenidis G, Moschos C B, Oldewurtel H A, Lehan P H & Hellems H K (1966) Journal of Clinical Investigation 45, 270 Regan T J, Levinson G E, Oldewurtel H A, Frank M J, Weisse A B & Moschos C B (1969) Journal of Clinical Investigation 48, 397 Schrire V & Gant J (1959) South AJrican Journal of Laboratory and Clinical Medicine 5, 195 Woods H F (1974) Symposium on Advanced Medicine (London) 10, 53
Juvenile Dermatomyositis with Complications J Winfield MB MRCP (for J M Gumpel MRCP) (Northwick Park Hospital, Harrow, Middlesex) A 14-year-old schoolboy (R B), referred by Dr T J Goodwin, was admitted to hospital in May 1976 with a one week history of malaise, muscle pain and weakness. Mild facial erythema had been noted after exposure to sun three weeks prior to admission. On admission he had a fever of 37.8°C; a marked erythematous facial rash with periorbital (edema was present, which extended onto his trunk and limbs. His throat and buccal mucosa were ulcerated, and infectious mononucleosis was considered a possible diagnosis. Over two days facial and buccal cedema became pronounced with the development of excoriated skin lesions around his mouth and eyelids. His face, neck and chest wall became cedematous and then brawny and indurated. The diagnosis of acute dermatomyositis was based on the clinical presentation and characteristic skin rash and was confirmed by skin and muscle biopsy. The creatine phosphokinase (CPK) level was 1240 iu/l rising to 15 000 iu/l one week after admission. Prednisolone 40 mg a day was started and over seven days the skin rash and fever responded and muscle pain decreased. However, he gained 3 kg in weight over two days and became hypertensive with a blood pressure of 150/100 mmHg and the periorbital cedema became much more marked. Diuretics were given to reduce his fluid overload. Later his general condition deteriorated with diffuse abdominal pain, and he had a hTmetamesis of some 300 ml ofaltered blood and a melaena and he became shocked. He improved with transfusion, but soon after there was an insidious change in respiration which became progressively more shallow and he became anoxic. He was admitted to the intensive care unit at Northwick Park Hospital in a moribund condition, when his respiratory rate was 60 per minute with a tidal volume estimated to be 20 ml. He was
Clinical Section
549
Fig 1 X-ray showing chest wall and pleural a?dema
sedated, intubated and ventilated and full sup- barium examination, and some barium was seen to portive therapy given with further blood trans- spill into the trachea. As he improved he complained of impaired fusions. Within two hours on this treatment his general condition was improved and his blood vision in the right eye. A large central scotoma in pressure rose to 120/60. The prednisolone dosage the right eye and two small scotomata in the left was increased to 150 mg a day and within two days eye were found; extensive exudates were seen there was marked resolution of his cedema and skin located particularly around the macular region in rash, although chest wall inflammation and cedema both eyes but changes were more marked in the continued to splint the chest and produced depres- right eye (Fig 2). These had not been seen on sion of the lung apices (Fig 1). Decubitus ulcers admission to Mount Vernon Hospital and to the developed despite careful nursing on a water bed intensive care unit: it was likely the retinopathy with sheepskin protection. Contractures were pre- had developed quickly over two to three days. His vented with light plaster of Paris splints for his legs blood pressure during this time was normal at and he was given passive physiotherapy. 110/75 mmHg. His prednisolone dosage at this After five days in the intensive care unit he was time was still 100 mg a day. During the next six weeks his general condition extubated but was still unable to tolerate oral fluids because of nasal regurgitation. He was able to swallow semi-solid food and to take oral prednisolone. The prednisolone dosage was decreased to 100 mg a day with his clinical improvement but progress was delayed by a right basal pneumonia and septicemia. Staphylococcus aureus was isolated from sputum, blood cultures and the subclavian infusion site and catheter. He responded well to intravenous flucloxacillin and oral probenecid, but again became intolerant of fluids and had abdominal distension. He passed two large melena stools. Upper gastrointestinal tract endoscopy (Dr A J Levi) revealed a normal stomach: two large superficial serpiginous ulcers were seen in the duodenum. Recent bleeding was evident and therefore no biopsy was taken. The lower duodenum was seen to be normal; it was possible that there were also ileal ulcers. Acute gastric dilation occurred and he was so intolerant of fluids for five days that full intravenous feeding was started. The Fig 2 Retinopathy in acute dermatomyositis. Two of the upper cesophagus was markedly abnormal on more discrete lesions are indicated
550
Proc. roy. Soc. Med. Volume 70 August 1977
steadily improved. Most of his skin cedema re- Discussion solved quickly and the skin rash healed pro- A number of serious complications of acute gressively, with some residual scarring over his juvenile dermatomyositis are exhibited by this eyelids, the dorsum of both hands, knees and case. The insidious onset of respiratory and palatal elbows. The biopsy site and infusion sites began to muscle weakness is frequently difficult to recogheal. His muscular power improved progressively nize, while weakness severe enough to require and his vision was back to normal within two assisted ventilation is relatively uncommon and in weeks of the initial retinal changes. His steroid this boy was only just detected in time. Regular dosage was gradually reduced successively and he tidal volume measurements might have detected developed no further complications apart from a this complication earlier. Involvement of the hypomild flare in erythema of his face which quickly pharyngeal musculature with the development of subsided. It was noted that he had a weak voice dysphagia, regurgitation or aspiration is common and still has, six months after his illness. This is and probably of prognostic significance in that 5 of probably related to intrinsic laryngeal muscle the 6 patients with this complication reported by weakness. His palatal weakness improved but he Everett & Curtis (1957) died. Measures to prevent still complains of slight difficulty in swallowing, aspiration and subsequent infection are essential. particularly with fluids. Intermittent episodes of Weakness of these muscles as a critical factor in vague abdominal pain continued with no severe survival has also been stressed by Wedgwood et al. flare for four to six weeks, but this gradually (1953) who found weakness in muscles of resubsided without further melena with return to a spiratory and deglutition in 13 of 26 children studied; this was considered to be a significant normal diet at two months. Six months after his illness he remains well, is factor in the fatal outcome of at least 5 children in back at school and he is increasing his general their series. Ulceration of any part of the gastrointestinal activities to include sports. His muscle enzymes have been monitored, particularly creatine kinase, tract may occur and Banker & Victor (1966) have and have shown a steady fall and this goes along stressed the role of vasculitis in the development of with his clinical improvement (Fig 3). His pred- these lesions. Extensive ulceration of the gastronisolone dosage has been reduced progressively. intestinal tract, with vasculitis as the pathological His skin rash has continued to improve steadily basis for these lesions, was well described even with residual scarring over his upper eyelids with a before the use of steroid therapy and therefore purplish discoloration around his eyes. There is a must be regarded as a primary feature of the marked degree of dermal atrophy on his hands disease and not simply as a complication of steroid with early scarring over the dorsum of both hands, therapy (Walton & Adams 1958). High dose predparticularly located over the metacarpophalangeal nisone may mask the clinical signs of perforation in and proximal interphalangeal joints. There is also these patients and it is often difficult to exclude purplish discoloration of the skin over the length perforation without repeated abdominal plain of the fingers. Residual rash on his knees, elbows X-rays. Retinopathy in dermatomyositis is rare and the and venepuncture sites has healed with keloid formation. He was previously known to develop majority of cases have been reported in children. keloid and scars and this is an unfortunate ad- The mechanism is probably vasculitis with capilditional complication. To date he has no contrac- lary damage and retinal ischimia. In this case the short episode of hypertension as an additional tures or evidence of calcinosis. factor in the development of retinopathy cannot be overlooked. The retinopathy in this boy may have developed three weeks after his initial symptoms but resolved quickly in two to three days with no Haemoglobin , subsequent visual impairment. Retinal photoMelaena 9/lOOml graphs (Fig 2) showed extensive exudates located 15009 in the paramacular region in both eyes with the Creatine typical appearance of cotton wool exudates, charphosphokinase 100] acteristic of those previously described (Bruce iu/I 50 0 1938, Harrison et al. 1973, Finman et al. 1976). This case is of particular interest because visual acuity returned to normal within two to three days Prednisolone A despite the persistence of extensive exudates; a mgmslIday possible mechanism may be resolution of macular cedema. The retinopathy may lead to significant july dec 1976 visual impairment, and optic atrophy has been reported. Repeat retinal photographs (Fig 4), Fig 3 Progress of R B and response to steroids ,s
-
5
1-M
1501
00
may
june
aug
sept
oct
Clinical Section
~ ~. : 551
On examination the abdomen was slightly distended and there was a large 'cystic' mass in the central epigastrium extending below the navel. The upper margin was indistinguishable from the costal margin, the mass was dull to percussion. A provisional diagnosis of hydatid disease was made. Hematological and biochemical indices were normal. A Casoni test was negative as was a hydatid complement fixation test. Barium swallow and meal showed a mass in the abdomen displacing the lower half of the stomach downwards and causing some compression of the duodenal cap. An hepatic scan with indium- 117 showed decreased uptake in the left lobe and lower half of the right lobe.
First operation (24.11.72, Professor H Ellis): The
Fig 4 Resolving retinopathy with pigmientation of retin'a taken two months after the onset of retinopathy showed complete resolution of the exudates but areas of pigmentation were also seen. Pigmentary changes where exudates were located has also been noted by Harrison et al. (1973) and Nutt (1951) and may reflect ischxemic damage to the choroid. REFERENCES Banker B Q & Victor M (1966) Medicine 45, 261-289 Bruce G M. (1938) Transactionts f the American Oph ofhaltological Society, 36, 282 Everett M A & Curtis A C (1957) Archives of Internal Medicine 100, 70-76 Finman L 5, Ragsdale C G, Sullivan D B & Petty R E 1976) Journal o Pcediateics88, 267 barrisonS M, Frenkel N, Grossman B J & Matalon R (1973) American Journal of Ophthalmology 76, 786 Nutt A B
(1951) Proceedings oj'the Royal Society oj'Medicine 44, 979 Walton J N & Adams R D
(1958) Polymyositis. E & S Livingstone, Edinburgh Wedgwood R P, Cook C D & Cohen J (1953) Pediatrics 12, 447-466
abdomen was explored through a midline epigastric excision. A thin-walled cyst was found measuring some 20 x 15 cm which occupied the inferior aspect of the liver and extended into the falciform ligament. The cyst was aspirated of two litres of clear bile and decapped. A small biliary fistula in the floor of the cyst was oversewn with thread sutures. The patient made an uneventful recovery. Histology of the cyst showed it to be lined by columnar epithelium, probably of bile duct origin. She remained well for two and half years. In July 1975 she returned with a three-month history of epigastric discomfort. On examination a cystic mass was again palpable in the right and left
*.,,,..::...: . .
Solitary Liver Cyst Treated by Cystogastrostomy M R Lock FRCS (Surgical Unit, Westminster Hospital, London S WI) Mrs E P, a 51-year-old housewife of mixed racial extraction from South Africa, presented in November 1972 with a four-day history of burning retrosternal pain, worse at night and on stooping. The pain was relieved by alkalis and she had had similar but less severe symptoms for two years previously. Her general health was good and there were no other complaints.
Fig 1 Plain abdominal X-ray showing soft tissue mass of recurrent cyst
