1977, British Journal of Radiology, 50, 366-369 Case reports
Although rare, extraosseous myeloma should be considered in the differential diagnosis of multiple polyps in the small intestine.
cytoma of the jejunum. American Journal of Pathology, 53, 33-39. GOLDSTEIN, W. B., and POKER, N., 1966. Multiple myeloma
involving the gastrointestinal tract. Gastroenterology, 51, 87-93. INGEGNO, A. P., 1954. Plasmacytoma of the gastrointestinal
tract: Report of a case involving the jejunum and review of the literature. Gastroenterology, 26, 89-102.
REFERENCES DOLIN, S., and DEWAR, J. P., 1956. Extramedullary plasma-
OBERKIRCHER, P. E., MILLER, W. T., and ARGER, P. H.,
cytoma. American Journal of Pathology, 32, 83-100.
1972. Non-osseous presentation of plasma cell myeloma. Radiology, 104, 515-520.
ESPOSITO, J. J., and STOUT, A. P., 1945. Multiple plasma-
Caroli's disease: an ultrasonic diagnosis By E. M. Bass, M.B.Ch.B., M.Med. (Rad.D.), D.M.R.D., F.R.C.R., M. R. Funston, M.B.B.Ch., D.M.R.D., F.R.C.R., and M. I. Shaft, M.B.B.Ch., D.M.R.D., F.R.C.R. Department of Radiology, Groote Schuur Hospital, Observatory, Cape Town 7925, South Africa (Received October, 1976)
Congenital dilatation of the intrahepatic bile ducts (Caroli's disease) was first reported in 1958 (Caroli et al.) and is usually diagnosed late in the patient's illness and seldom before operation (Watts et al., 1974). Goddard (1974) could only find 16 previous case reports of the disease which attests to its rarity. Caroli (1968) has classified congenital intrahepatic cystic dilatation into two groups: first, a pure form not associated with hepatic cirrhosis or portal hypertension and second, a type associated with congenital hepatic fibrosis. There is probably some overlap but Watts et al. (1974) consider most cases belong to the first group. The radiological diagnosis of Caroli's disease has, in the past, proven difficult. None of the 15 cases collected by Mujahed et al. (1971) were diagnosed before surgery. With improved modern techniques in organ visualization, the pre-operative diagnosis has become easier and we report the following case which was confidently diagnosed by ultrasonography and confirmed by transhepatic cholangiography, thus rendering invaluable assistance to the surgeon.
In February 1976 her jaundice and rigors recurred. She was now pyrexial and her liver was enlarged by 3 cm. Her total bilirubin was 9.3 mg%. An isotopic technetium sulphur colloid scan showed a cold area in the A.I. portion of the right lobe. A Rose-Bengal scan was not performed. Her intravenous pyelogram was normal with no evidence of renal tubular ectasia. Longitudinal and transverse grey-scale ultrasonograms, at high and low gain, were done with Diasonograph NE 4102 scan convertor, using a 2.5 MHz transducer. Four transonic lesions, varying from 2 to 5 cm in diameter, were found in the region of the porta hepatis (Fig. 1). Their cystic nature was confirmed by the absence of internal echoes at high and low gain and clear definition of their posterior walls. Their position was marked on the skin surface and longitudinal, sagittal scans were next done through the plane of these marks. The largest cystic structure was 5 cm in diameter and 12 cm in length (Fig. 2). An
CASE REPORT
A female of 19 years was first admitted to hospital in December 1975 because of increasing jaundice and rigors. Her clinical history was unremarkable except for her occupation as an assistant in a hospital sterilizing department and handling liver biopsy packs. Her total bilirubin was 5 mg% (conjugated level 3.8 mg%) and her alkaline phosphatase was 132 international units. She was thought to have infective hepatitis but her jaundice never settled and a liver biopsy was compatible FIG.1. with cholangitis and biliary stasis. Her jaundice subsequently decreased, her symptoms improved and she was Transverse scan through the region of the porta hepatis showing four transonic structures. discharged. 366
MAY
1977
Case reports undilated gall bladder of 3.6 cm diameter was identified independently and lateral to the largest cystic structure. Superior to the gall bladder and within the liver substance were three cystic lesions of 2-3 cms in diameter (Fig. 3). No biliary calculi were identified. Taking the patient's age and clinical setting into account, a confident ultrasonic diagnosis of congenital cystic dilatation of the intrahepatic bile ducts was made. After intensive treatment with antibiotics, her pyrexia settled and a transhepatic cholangiogram with a small gauge (23 mm) needle was done one week later. Undilated peripheral ducts were entered on the first attempt and
FIG. 4. Percutaneous transhepatic cholangiogram showing several localized dilatations of the intrahepatic ducts of the right lobe of the liver (arrows). The choledochal cyst is well seen (arrowhead).
FIG. 2. Longitudinal, sagittal scan 4 cm to the right of the midline showing a large cystic structure (arrowhead) projecting from the P.I. surface of the liver. A smaller cystic structure (arrow) is seen within the liver substance.
FIG. 3. Longitudinal, sagittal scan, 7 cm to the right of the midline, showing an undilated gall-bladder (arrowhead). Three transonic structures (arrows) are shown within the liver.
FIG. 5. Transhepatic cholangiogram showing additional dilatations in the left intrahepatic ducts (arrows) and the large choledochal cyst (arrowhead).
367
VOL.
50, No. 593 Case reports
contrast flowed into a localized dilatation. On further filling, several localized cystic dilatations were seen in both the right and left intrahepatic ducts (Fig. 4). There was also marked dilatation of the common bile duct in which there were many faceted gall-stones. Contrast flowed freely into the duodenum and the terminal portion of the common bile duct was undilated (Fig. 5). Laparotomy confirmed the pre-operative radiological findings, and showed a 6 cm diameter choledochal cyst containing 12 calculi of varying size. The terminal common bile duct had a normal calibre. A choledochoscope was passed and the bilateral localized dilatations of the intrahepatic ducts well seen. The gall-bladder was full but not dilated. A cholecystectomy and Roux-en-Y choledocystjejunostomy were performed. The histology of the removed cyst showed biliary tract type mucosa with a thickened and fibrosed wall. The calculi consisted of cholesterol, bilirubin and a small trace of calcium.
DISCUSSION
Radiological diagnosis of Caroli's disease has, in the past, been difficult before surgery. Occasionally, mottling of contrast medium over the hepatic surface after cholangiography may indicate the intrahepatic cyst dilatations (Caroli and Corcos, 1964). This may be considerably enhanced by Logetronic technique (Caroli, 1973). Calculi may be visualized but the true nature of the underlying disease is not realized. Frequently, however, cholecystography, intravenous cholangiography and transhepatic cholangiography fail to opacify the ductal system (Foulk, 1970). Watts et al. (1974) consider operative cholangiography to be the most valuable test for the diagnosis, location and extent of the disease process, and mapping out the surgical approach. Isotopic liver scanning may be helpful. A colloidal scan may show several decreased areas of uptake. If Rose-Bengal is then substituted, these areas may show increased uptake and stasis of the isotope. Angiography usually shows non-specific splaying of the vessels around the lesions and defects in the hepatogram. The case presented demonstrates the value of ultrasonography in the early diagnosis of Caroli's disease. The finding of gross, localized cystic dilatations of intrahepatic bile ducts is highly suggestive of the disease. Suruga et al. (1969) reported the successful diagnosis of a choledochal cyst by ultrasonography but we have been unable to find a report of a successful diagnosis of Caroli's disease by this method. Caroli's disease is probably congenital in origin and affects males and females equally. Patients usually present in childhood or young adulthood with a typical symptom complex characterized by recurrent abdominal pain, intermittent obstructive jaundice, and fever. Symptomatology is caused by
stone formation due to bile stasis within the cysts, and by relapsing episodes of cholangitis; however, the disease may be asymptomatic (Foulk, 1970). The dilated areas in Caroli's disease represent true ductal ectasias, which communicate freely with the biliary tree and contain bile. The dilatations may be widespread or localized, saccular, cylindrical or both. When the ductal ectasia is not diffuse it seems to be always localized to the left intrahepatic ductal system (Goddard, 1974). A number of associations with Caroli's disease have been described and include hepatic fibrosis (Kerr et al., 1961), renal tubular ectasia (Kerr et al., 1962), intrahepatic lithiasis (Caroli and Corcos, 1964) and choledochal cyst. However, the association with choledochal cyst may well reflect obstructive dilatation rather than a primary error in intrahepatic ductal development (Kelly, 1971; Gots and Zuidema, 1970). In addition there appears to be an increased incidence of biliary tract carcinoma in patients with Caroli's disease (Watts et al., 1974). The usual surgical treatment for Caroli's disease is internal biliary drainage, especially choledochojejunostomy. Surgical intervention alleviates symptoms temporarily by removing calculi and re-establishing biliary drainage. Sooner or later infection recurs and often becomes uncontrollable with resultant liver abscesses and septicaemia, the usual cause of death. In 1968 Caroli considered that "this disease is not amenable to surgical treatment" which emphasizes the complexity of the surgical problem. REFERENCES CAROLI, J., 1968. Diseases of intrahepatic bile ducts. Israel Journal of Medical Sciences, 4, 21-35. CAROLI, J., 1973. Diseases of the intrahepatic biliary tree. Clinics in Gastroenterology, 2,147-161. CAROLI, J. and CORCOS, V., 1964. La dilatation congenitale
des voies biliares intrahepatiques. Review Medicochirugie Malades Foie, 39, 1 -24. CAROLI, J., SOUPAULT, R., KOSSAKOWSKI, J., PLOCKER, L.
and PARADOWSKA, M., 1958. La dilatation polykystique congenitale de voies biliares intrahepatiques. Essai de classification. Seminars Hospital Paris, 34, 488-495. FOULK, W. T., 1970. Congenital malformations of the intrahepatic biliary tree in the adult. Gastroenterology, 58, 253-256. GODDARD, J. E., 1974. Case of the spring season. Seminars in Roentgenology, 9, 89-90. GOTS, R. E. and ZUIDEMA, G. D., 1970. Dilatation of the
intrahepatic biliary ducts in a patient with a choledochal cyst. American Journal of Surgery, 119, 726. KELLY, M. J., 1971. Case of Caroli's disease. British Medical Journal, 4, 407. KERR, D. N. S., HARRISON, C. V., SHERLOCK, S. and
WALKER, R. M. 1961. Congenital hepatic fibrosis. Quarterly Medicine, 30, 91. KERR, D. N. S., WARWICK, C. K., and HART-MERCER, J.,
368
1962. A lesion resembling medullary sponge kidney in patients with congenital hepatic fibrosis. Clinical Radiology, 13, 85-91.
1977, British Journal of Radiology, 50, 369-371
MAY
1977
Case reports diagnosis of congenital bile duct lesions. Journal of Pediamunicating cavernous ectasia of the intrahepatic ducts tric Surgery, 4, 452-456. (Caroli's disease). American Journal of Roentgenology, 113, WATTS, D. R., LORENZO, G. A., and BEAL, J. M., 1974. 21-26. Congenital dilatation of intrahepatic bile ducts. Archives SURUGA, K., HIRAI, Y., NAGASHIMA, K., WAGAI, T., and of Surgery, 108, 592-597. INUI, M., 1969. Ultrasonic echo examination as an aid in
MUJAHED, Z., GLENN, F., and EVANS, J. A., 1971. Com-
The demonstration by scintigraphy of fractures in osteomalacia By J. D. Macfarlane, B.M., M.R.C.P.,* J. E. Lutkin, B.Sc, Ph.D.,** and R. J. Burwood, M.A., M.D., F.R.C.R.** Departments of Rheumatology* and Nuclear Medicine,** Royal Sussex County Hospital, Brighton (Received April, 1976 and in revised form December, 1976) Scintigraphy is an adjunct to radiology in the investigation of bone disease. It is of value in the diagnosis of neoplastic and inflammatory conditions and in the evaluation of radio- and chemotherapy. Its use in metabolic disease has been less well documented. A patient with osteomalacia secondary to gluten sensitivity, in whom pseudofractures (Looser zones) were identified by bone scintigraphy, is reported. CASE REPORT
A female, aged 56, gave a history of many years of joint pains and a period of diarrhoea 18 months prior to admission. A barium enema was reported to be normal. She was admitted for investigation of right groin pain following a trivial leg injury and volunteered a recent story of pale offensive stools and pains in both arms and shoulders. There was no history of trauma and no relevant past or family ailments. On examination she was thin (height 165 cm, weight 44 kg) and looked pale. There was tenderness in the right groin during examination of the hip but no other abnormalities were observed. Investigations confirmed the suspected malabsorption. Barium follow through studies showed flocculation, segmentation and dilution of the contrast; no structural defect was seen in the bowel. A jejunal biopsy showed almost total villous atrophy. Radiographs demonstrated pseudofractures of the neck of the right femur, both superior and inferior pubic rami, and of the right 6th rib posteriorly (Fig. 1). A bone scan using 10 mCi of technetium-99m-polyphosphate (TcPP) confirmed the radiological lesions and, in addition, identified nine other sites of markedly increased uptake: seven in the ribs and one each in the tip of the right acromion and in the left glenoid fossa. There was an unusually high ratio of activity in bone to soft tissue background (Fig. 2). Her progress on a gluten free diet supported the diagnosis of osteomalacia secondary to gluten sensitivity malabsorption. DISCUSSION
Bone scintigraphy with various radionuclides, and especially with TcPP, is of value in the diagnosis of traumatic, neoplastic, inflammatory and infectious bone and joint disease. Its use in metabolic conditions has been less well documented. In particular,
FIG.1.
Chest radiograph: There is a fracture of right 6th rib. fractures in osteomalacic patients without renal disease have not been described. The confirmation of Looser zones in our patient, and their demonstration in sites where there was no overt radiological change, is analogous to the identification of scaphoid fractures (Lutkin and Burwood, 1977) and stress fractures (Burry and Maisey, 1975) by scintigraphy where early radiographs may appear normal.
369
Although rare, extraosseous myeloma should be considered in the differential diagnosis of multiple polyps in the small intestine.
cytoma of the jejunum. American Journal of Pathology, 53, 33-39. GOLDSTEIN, W. B., and POKER, N., 1966. Multiple myeloma
involving the gastrointestinal tract. Gastroenterology, 51, 87-93. INGEGNO, A. P., 1954. Plasmacytoma of the gastrointestinal
tract: Report of a case involving the jejunum and review of the literature. Gastroenterology, 26, 89-102.
REFERENCES DOLIN, S., and DEWAR, J. P., 1956. Extramedullary plasma-
OBERKIRCHER, P. E., MILLER, W. T., and ARGER, P. H.,
cytoma. American Journal of Pathology, 32, 83-100.
1972. Non-osseous presentation of plasma cell myeloma. Radiology, 104, 515-520.
ESPOSITO, J. J., and STOUT, A. P., 1945. Multiple plasma-
Caroli's disease: an ultrasonic diagnosis By E. M. Bass, M.B.Ch.B., M.Med. (Rad.D.), D.M.R.D., F.R.C.R., M. R. Funston, M.B.B.Ch., D.M.R.D., F.R.C.R., and M. I. Shaft, M.B.B.Ch., D.M.R.D., F.R.C.R. Department of Radiology, Groote Schuur Hospital, Observatory, Cape Town 7925, South Africa (Received October, 1976)
Congenital dilatation of the intrahepatic bile ducts (Caroli's disease) was first reported in 1958 (Caroli et al.) and is usually diagnosed late in the patient's illness and seldom before operation (Watts et al., 1974). Goddard (1974) could only find 16 previous case reports of the disease which attests to its rarity. Caroli (1968) has classified congenital intrahepatic cystic dilatation into two groups: first, a pure form not associated with hepatic cirrhosis or portal hypertension and second, a type associated with congenital hepatic fibrosis. There is probably some overlap but Watts et al. (1974) consider most cases belong to the first group. The radiological diagnosis of Caroli's disease has, in the past, proven difficult. None of the 15 cases collected by Mujahed et al. (1971) were diagnosed before surgery. With improved modern techniques in organ visualization, the pre-operative diagnosis has become easier and we report the following case which was confidently diagnosed by ultrasonography and confirmed by transhepatic cholangiography, thus rendering invaluable assistance to the surgeon.
In February 1976 her jaundice and rigors recurred. She was now pyrexial and her liver was enlarged by 3 cm. Her total bilirubin was 9.3 mg%. An isotopic technetium sulphur colloid scan showed a cold area in the A.I. portion of the right lobe. A Rose-Bengal scan was not performed. Her intravenous pyelogram was normal with no evidence of renal tubular ectasia. Longitudinal and transverse grey-scale ultrasonograms, at high and low gain, were done with Diasonograph NE 4102 scan convertor, using a 2.5 MHz transducer. Four transonic lesions, varying from 2 to 5 cm in diameter, were found in the region of the porta hepatis (Fig. 1). Their cystic nature was confirmed by the absence of internal echoes at high and low gain and clear definition of their posterior walls. Their position was marked on the skin surface and longitudinal, sagittal scans were next done through the plane of these marks. The largest cystic structure was 5 cm in diameter and 12 cm in length (Fig. 2). An
CASE REPORT
A female of 19 years was first admitted to hospital in December 1975 because of increasing jaundice and rigors. Her clinical history was unremarkable except for her occupation as an assistant in a hospital sterilizing department and handling liver biopsy packs. Her total bilirubin was 5 mg% (conjugated level 3.8 mg%) and her alkaline phosphatase was 132 international units. She was thought to have infective hepatitis but her jaundice never settled and a liver biopsy was compatible FIG.1. with cholangitis and biliary stasis. Her jaundice subsequently decreased, her symptoms improved and she was Transverse scan through the region of the porta hepatis showing four transonic structures. discharged. 366
MAY
1977
Case reports undilated gall bladder of 3.6 cm diameter was identified independently and lateral to the largest cystic structure. Superior to the gall bladder and within the liver substance were three cystic lesions of 2-3 cms in diameter (Fig. 3). No biliary calculi were identified. Taking the patient's age and clinical setting into account, a confident ultrasonic diagnosis of congenital cystic dilatation of the intrahepatic bile ducts was made. After intensive treatment with antibiotics, her pyrexia settled and a transhepatic cholangiogram with a small gauge (23 mm) needle was done one week later. Undilated peripheral ducts were entered on the first attempt and
FIG. 4. Percutaneous transhepatic cholangiogram showing several localized dilatations of the intrahepatic ducts of the right lobe of the liver (arrows). The choledochal cyst is well seen (arrowhead).
FIG. 2. Longitudinal, sagittal scan 4 cm to the right of the midline showing a large cystic structure (arrowhead) projecting from the P.I. surface of the liver. A smaller cystic structure (arrow) is seen within the liver substance.
FIG. 3. Longitudinal, sagittal scan, 7 cm to the right of the midline, showing an undilated gall-bladder (arrowhead). Three transonic structures (arrows) are shown within the liver.
FIG. 5. Transhepatic cholangiogram showing additional dilatations in the left intrahepatic ducts (arrows) and the large choledochal cyst (arrowhead).
367
VOL.
50, No. 593 Case reports
contrast flowed into a localized dilatation. On further filling, several localized cystic dilatations were seen in both the right and left intrahepatic ducts (Fig. 4). There was also marked dilatation of the common bile duct in which there were many faceted gall-stones. Contrast flowed freely into the duodenum and the terminal portion of the common bile duct was undilated (Fig. 5). Laparotomy confirmed the pre-operative radiological findings, and showed a 6 cm diameter choledochal cyst containing 12 calculi of varying size. The terminal common bile duct had a normal calibre. A choledochoscope was passed and the bilateral localized dilatations of the intrahepatic ducts well seen. The gall-bladder was full but not dilated. A cholecystectomy and Roux-en-Y choledocystjejunostomy were performed. The histology of the removed cyst showed biliary tract type mucosa with a thickened and fibrosed wall. The calculi consisted of cholesterol, bilirubin and a small trace of calcium.
DISCUSSION
Radiological diagnosis of Caroli's disease has, in the past, been difficult before surgery. Occasionally, mottling of contrast medium over the hepatic surface after cholangiography may indicate the intrahepatic cyst dilatations (Caroli and Corcos, 1964). This may be considerably enhanced by Logetronic technique (Caroli, 1973). Calculi may be visualized but the true nature of the underlying disease is not realized. Frequently, however, cholecystography, intravenous cholangiography and transhepatic cholangiography fail to opacify the ductal system (Foulk, 1970). Watts et al. (1974) consider operative cholangiography to be the most valuable test for the diagnosis, location and extent of the disease process, and mapping out the surgical approach. Isotopic liver scanning may be helpful. A colloidal scan may show several decreased areas of uptake. If Rose-Bengal is then substituted, these areas may show increased uptake and stasis of the isotope. Angiography usually shows non-specific splaying of the vessels around the lesions and defects in the hepatogram. The case presented demonstrates the value of ultrasonography in the early diagnosis of Caroli's disease. The finding of gross, localized cystic dilatations of intrahepatic bile ducts is highly suggestive of the disease. Suruga et al. (1969) reported the successful diagnosis of a choledochal cyst by ultrasonography but we have been unable to find a report of a successful diagnosis of Caroli's disease by this method. Caroli's disease is probably congenital in origin and affects males and females equally. Patients usually present in childhood or young adulthood with a typical symptom complex characterized by recurrent abdominal pain, intermittent obstructive jaundice, and fever. Symptomatology is caused by
stone formation due to bile stasis within the cysts, and by relapsing episodes of cholangitis; however, the disease may be asymptomatic (Foulk, 1970). The dilated areas in Caroli's disease represent true ductal ectasias, which communicate freely with the biliary tree and contain bile. The dilatations may be widespread or localized, saccular, cylindrical or both. When the ductal ectasia is not diffuse it seems to be always localized to the left intrahepatic ductal system (Goddard, 1974). A number of associations with Caroli's disease have been described and include hepatic fibrosis (Kerr et al., 1961), renal tubular ectasia (Kerr et al., 1962), intrahepatic lithiasis (Caroli and Corcos, 1964) and choledochal cyst. However, the association with choledochal cyst may well reflect obstructive dilatation rather than a primary error in intrahepatic ductal development (Kelly, 1971; Gots and Zuidema, 1970). In addition there appears to be an increased incidence of biliary tract carcinoma in patients with Caroli's disease (Watts et al., 1974). The usual surgical treatment for Caroli's disease is internal biliary drainage, especially choledochojejunostomy. Surgical intervention alleviates symptoms temporarily by removing calculi and re-establishing biliary drainage. Sooner or later infection recurs and often becomes uncontrollable with resultant liver abscesses and septicaemia, the usual cause of death. In 1968 Caroli considered that "this disease is not amenable to surgical treatment" which emphasizes the complexity of the surgical problem. REFERENCES CAROLI, J., 1968. Diseases of intrahepatic bile ducts. Israel Journal of Medical Sciences, 4, 21-35. CAROLI, J., 1973. Diseases of the intrahepatic biliary tree. Clinics in Gastroenterology, 2,147-161. CAROLI, J. and CORCOS, V., 1964. La dilatation congenitale
des voies biliares intrahepatiques. Review Medicochirugie Malades Foie, 39, 1 -24. CAROLI, J., SOUPAULT, R., KOSSAKOWSKI, J., PLOCKER, L.
and PARADOWSKA, M., 1958. La dilatation polykystique congenitale de voies biliares intrahepatiques. Essai de classification. Seminars Hospital Paris, 34, 488-495. FOULK, W. T., 1970. Congenital malformations of the intrahepatic biliary tree in the adult. Gastroenterology, 58, 253-256. GODDARD, J. E., 1974. Case of the spring season. Seminars in Roentgenology, 9, 89-90. GOTS, R. E. and ZUIDEMA, G. D., 1970. Dilatation of the
intrahepatic biliary ducts in a patient with a choledochal cyst. American Journal of Surgery, 119, 726. KELLY, M. J., 1971. Case of Caroli's disease. British Medical Journal, 4, 407. KERR, D. N. S., HARRISON, C. V., SHERLOCK, S. and
WALKER, R. M. 1961. Congenital hepatic fibrosis. Quarterly Medicine, 30, 91. KERR, D. N. S., WARWICK, C. K., and HART-MERCER, J.,
368
1962. A lesion resembling medullary sponge kidney in patients with congenital hepatic fibrosis. Clinical Radiology, 13, 85-91.
1977, British Journal of Radiology, 50, 369-371
MAY
1977
Case reports diagnosis of congenital bile duct lesions. Journal of Pediamunicating cavernous ectasia of the intrahepatic ducts tric Surgery, 4, 452-456. (Caroli's disease). American Journal of Roentgenology, 113, WATTS, D. R., LORENZO, G. A., and BEAL, J. M., 1974. 21-26. Congenital dilatation of intrahepatic bile ducts. Archives SURUGA, K., HIRAI, Y., NAGASHIMA, K., WAGAI, T., and of Surgery, 108, 592-597. INUI, M., 1969. Ultrasonic echo examination as an aid in
MUJAHED, Z., GLENN, F., and EVANS, J. A., 1971. Com-
The demonstration by scintigraphy of fractures in osteomalacia By J. D. Macfarlane, B.M., M.R.C.P.,* J. E. Lutkin, B.Sc, Ph.D.,** and R. J. Burwood, M.A., M.D., F.R.C.R.** Departments of Rheumatology* and Nuclear Medicine,** Royal Sussex County Hospital, Brighton (Received April, 1976 and in revised form December, 1976) Scintigraphy is an adjunct to radiology in the investigation of bone disease. It is of value in the diagnosis of neoplastic and inflammatory conditions and in the evaluation of radio- and chemotherapy. Its use in metabolic disease has been less well documented. A patient with osteomalacia secondary to gluten sensitivity, in whom pseudofractures (Looser zones) were identified by bone scintigraphy, is reported. CASE REPORT
A female, aged 56, gave a history of many years of joint pains and a period of diarrhoea 18 months prior to admission. A barium enema was reported to be normal. She was admitted for investigation of right groin pain following a trivial leg injury and volunteered a recent story of pale offensive stools and pains in both arms and shoulders. There was no history of trauma and no relevant past or family ailments. On examination she was thin (height 165 cm, weight 44 kg) and looked pale. There was tenderness in the right groin during examination of the hip but no other abnormalities were observed. Investigations confirmed the suspected malabsorption. Barium follow through studies showed flocculation, segmentation and dilution of the contrast; no structural defect was seen in the bowel. A jejunal biopsy showed almost total villous atrophy. Radiographs demonstrated pseudofractures of the neck of the right femur, both superior and inferior pubic rami, and of the right 6th rib posteriorly (Fig. 1). A bone scan using 10 mCi of technetium-99m-polyphosphate (TcPP) confirmed the radiological lesions and, in addition, identified nine other sites of markedly increased uptake: seven in the ribs and one each in the tip of the right acromion and in the left glenoid fossa. There was an unusually high ratio of activity in bone to soft tissue background (Fig. 2). Her progress on a gluten free diet supported the diagnosis of osteomalacia secondary to gluten sensitivity malabsorption. DISCUSSION
Bone scintigraphy with various radionuclides, and especially with TcPP, is of value in the diagnosis of traumatic, neoplastic, inflammatory and infectious bone and joint disease. Its use in metabolic conditions has been less well documented. In particular,
FIG.1.
Chest radiograph: There is a fracture of right 6th rib. fractures in osteomalacic patients without renal disease have not been described. The confirmation of Looser zones in our patient, and their demonstration in sites where there was no overt radiological change, is analogous to the identification of scaphoid fractures (Lutkin and Burwood, 1977) and stress fractures (Burry and Maisey, 1975) by scintigraphy where early radiographs may appear normal.
369
