Clin. Radio. (1978) 29, 529-534 T R A N S I E N T S M A L L B O W E L I N T U S S U S C E P T I O N IN A D U L T C O E L I A C D I S E A S E M.D. COHEN and D. J. LINTOTT From the Department of Diagnostic Radiology, The General Infirmary, Leeds Considerable disagreement exists in the literature concerning the occurrence and incidence of transient non-obstructive intussusception in adult coeliac disease. Only a few case reports have been published and several standard texts do not mention the association at all. We report six adult patients, all with coeliac disease, who demonstrated transient non-obstructive intussusception on small bowel meal examination. The technique of the small bowel meal and the radiological signs of malabsorption are briefly described. The literature is reviewed and reasons for the disagreement herein are discussed. We conclude that the incidence of finding intussusception in adult coeliac disease is related to the intensity of searching and in our series this association was seen in at least 20% of cases. It is considered to be an additional radiological sign of coeliac disease although in no case did it occur in the absence of other prominent radiological evidence of malabsorption.
Intussusception confined to the small bowel is rare. In adults, as distinct from children, an organic cause is usually identifiable. A review of the literature revealed disagreement concerning the occurrence and incidence of transient non-obstructive intussusception in adult coeliac disease. The case histories and radiological findings of six adult coeliac patients with transient intussusception are described and the possible reasons for disagreement in the literature discussed.
DISCUSSION
(a) Diagnosis of Coeliac Disease
Coeliac disease (idiopathic steatorrhoea) is characterised by diarrhoea, abnormal stools, weight loss and the effects of malabsorption. It presents in a wide spectrum of severity ranging from severe illness, through mild intermittent diarrhoea to asymptomatic patients (Cooke and Asquith, 1974). The diagnosis is made by obtaining an abnormal jejunal mucosal biopsy, demonstrating improvement on a gluten-free diet and relapse on further gluten challenge. Cooke and METHODS Asquith (1974) discuss the problems with this rigid At the General Infirmary at Leeds patients with definition (e.g. the response to diet may only be suspected small bowel diseases are examined by a minimal) and suggest that one should accept all specific small bowel meal. The patient is prepared patients with a flat jejunal as having coeliac disease. The radiological signs of malabsorption are nonas for a barium meal by low residue diet, purgation and fasting. After a control film, 300 ml of a dilute, specific although they are typically most marked in isotonic, cold barium suspension, with 10ml Gastro- coeliac disease, compared with other causes of realgrafin added as an accelerator, is given orally. Prone absorption. There is uniform, flaccid jejunal dilatation overcouch films are taken at 10, 30 and 60min to 30mm or more (Laws, 1964) which is related to supplemented by screening and spot films with and the patient's clinical condition rather than to the without compression. If the terminal ileum has not degree of mucosal atrophy or steatorrhoea (Laws, been reached at 60 min Metaclopramide 10 mg i.v. 1964; Burrows and Toye, 1974) (Fig. 3). Dilatation is given and later films taken as required (96% of all causes the mucosal folds to be transversely orientated, examinations are completed by 90min using this but unless there is associated hypoproteinaemia or method). The whole examination is conducted by inflammation, they are not thickened (Marshak and one radiologist and radiographic team in one room Lindner, 1966). The folds may be obviously atrophic being shallow and widely spaced and in severe cases devoted exclusively to the small bowel meal session. may be completely effaced. In addition there is an excess of fluid in the bowel which dilutes the barium (Fig. 2). The transit time may be markedly prolonged RESULTS compared with the normal range for the technique used. The use of a stable barium suspension largely See Table 1.
530
CLINICAL
RADIOLOGY
Fig. 1 - Case 1. B.D. (a) The 20 m i n radiograph shows dilated
loops o f small bowel with straightening o f m u c o s a l folds and dilution of contrast. No intussusception is seen. (b) Intussusception shown at 1 h. prevents flocculation, but this may still be a prominent feature in coeliac disease (Figs. 2, 4). All six of our patients had typical clinical, biochemical and radiologicat features of coeliac disease. In four, diagnostic jejunal biopsies were obtained. In the fifth patient biopsy was technically unsuccessful and the sixth patient left the country before biopsy could be obtained (Table 1).
(b) Mechanism of Intussusception Reymond (1972) discusses the physical principles of the formation of an intussusception. He divides the causes of intussusception into two groups. In his first group there is a mechanical linkage across nonadjacent segments, e.g. adhesions, inflammation. The second group have a local area of bowel wall which does not contract normally. Unbalanced peristaltic forces might result when a normal peristaltic wave reaches an abnormal area of bowel wall. These forces may be sufficient to rotate a segment of bowel wall inwards, thus initiating the intussusception. In coeliac disease it is postulated that dilated flaccid loops of bowel might disturb normal peristalic waves, resulting in intussusception.
(c) Intussusception in Coeliac Disease The overall incidence of intussusception in children is approximately 2 per 1000 live births. It is much less common in adults (Morson and Dawson, 1972). Intussusception of small bowel into small bowel is rare and represents only 6% of all intussusceptions (Ward, 1960). In children it is usual to find no organic cause for the intussusception. In adults an organic aetiology is found in over 80% of casesfrequently a tumour or polyp, (Morson and Dawson, 1972; Bloch and Peck, 1964). Case reports of intussusception in coeliac disease are uncommon. Golden (1959) reported a 53-year-old male with steatorrhoea upon whom two small bowel barium studies eight days apart both showed evidence Fig. 2 Case 2. S.B. There is m a r k e d flocculation o f the barium with some uniform dilatation. A typically intus- of malabsorption and an intussusception, the site of which differed on each examination. Bloch and Peck susception is seen. r
7
Fig. 3 - Case 3. M.G. (a) The 3 0 m i n radiograph shows flaccid dilatation of loops. The mucosal folds are straightened, but not thickened. (b) Intussusception shows at 2% h.
Fig. 4 - Case 4. C.J. (a) Despite marked flocculation an intussusception is demonstrated. (b) A close-up of the intussusception shown in (a).
\
i 4a \.J
~ , 2 -~ 7 ~ ~ ;
532
CLINICAL RADIOLOGY
Table 1 - Details o f cases of transient small bowel intussusception in adult coeliac disease
Presentmg symptoms
Fecal fat
Case 1. B.D. Male born 1920
1964: Ten weeks diarrhoea 1975: Weight loss and diarrhoea (in association with dietary lapse)
76g per three days
Case 2. S.B. Female born 1940
1974: Six months diarrhoea. 7 kg weight loss pallor
1974: Subtotalvillous 1974: Uniform, flac- Excellentatrophy with plasma cid dilatation of weight incell infiltration small bowel loops creased 10 1975 : (One year after with transverse muco- kg in three starting gluten free sal folds, marked months diet) - marked imflocculation and provement but not dilution of contrast. completely normal A transient nonobstructive intussusception was seen at 60 rain (Fig. 2)
Case 3. M.G. Male born 1937
1977: Two years intermittent diarrhoea. 12 kg weight loss. Pale smelly stools
1977: Villous atrophy 1977: Dilated flaccid Diet started and cellular infiltrate loops of small bowel April 1977 with atrophic folds, and excess of fluid and flocculation of contrast. A transient non-obstructive intussusception was seen on the 23Ah radiograph (Fig. 3)
Case 4. C.J. Female born 1948
1972: Three weeks diarrhoea settled spontaneously 1973: Five weeks diarrhoea, pale stool 1974: Well
1974: Attempted biopsy-duodenal mucosa was obtained
159 g per five days
_Jejunal biopsy
Small bowel meal
Response to gluten-free diet
Present state
1964: Subtotal villous 1964: Barium follow Clinical imWell atrophy with no through was reprovement. cellular infiltrate ported as showing Well until 1975 : Subtotal villous 'dilated jejunal relapse in atrophy with a loops with an 1975 heavy infiltration intussusception in 1975: Dramatic of plasma cells, the left iliac fossa' improvement lymphocytes and 1975: Flaccid dilataon reinstieosinophils tion of loops and tuting dietary persistent floccucontrol lation with a nonobstructive intussusception at 60 min (Fig. 1)
1977: Marked flocculation and segmentation of the barium. Dilatation not marked. At least two transient non-obstructive intussusceptions were seen (Fig. 4)
Case 5. S.N.T. 1974: Persistent epiFemale born gastric pain on and off 1946 for years. Alternating diarrhoea and constipation
1974: Uniform flaccid Not done Not done-departed dilatation (Fig. 5) overseas. No followup
Case 6. E.V.T. 1976: Lower abdominal Male born pain and bleeding per 1939 rectum. Pale stool. No diarrhoea
Improvement March 1977: Loss of Dilatation, flocculation and promivilli infiltration with plasma cells, lympho- nent transverse mucosal folds. An cytes, macrophages intussusception is and some polyseen on the 1½h film rnorphis (Fig. 6) May 1977: Improvement but not normal
Well
No diarrhoea since 1974not on gluten-free diet
TRANSIENT
SMALL
BOWEL
INTUSSUSCEPTION
:
®
IN ADULT
COELIAC
DISEASE
533
o ,2 ~
..
®-N
Fig. 5 - Case 5. S.N.T. A less o b v i o u s b u t d e f i n i t e intuss u s c e p t i o n p r e s e n t in i l e u m a t 1% h.
Fig. 6 - Case 6. E.V.T. I n t u s s u s c e p t i o n p r e s e n t in j e j u n u m at 1% h a l m o s t o b s c u r e d b y a s s o c i a t e d f l o c c u l a t i o n .
(1964) reported a 29-year-old female with diarrhoea, weight loss and jejunal biopsy typical of coeliac disease with radiological evidence of malabsorption and a symptomless non-obstructive intussusception in the distal jejunum. Ruoff et al. (1968) stated that 'intussusception is not uncommon in sprue' and reported one case of a 45-year-old male who presented with severe upper abdominal pain. Barium studies of the small bowel two weeks later, when pain was no longer present, showed signs of malabsorption and a non-obstructive intussusception. Jejunal biopsy was typical of coeliac disease. Cihak et al. (1969) review the notes of an adult patient with proven coeliac disease. At the age of 7 years this patient had pale bulky stools and at 12 years of age had presented with intestinal obstruction. Laparotomy revealed a terminal ileal intussusception in association with enlarged mesenteric nodes (a recognised cause of intussusception). This patient is not typical of the cases we have presented and it is probable that the intussusception was unrelated to the coeliac disease. Isbell et al. (1969) noted transient intussusceptions in three out of 33 patients with coeliac disease and also one of two with giardiasis, in review of 68 small bowel barium studies with signs of malabsorption.
They considered it to be a valuable sign in establishing the diagnosis of sprue. Burrows and Toye (1974) reported having seen intussusception in 18 children with coefiac disease and they imply that this occurs commonly in adult coeliac patients. They quoted reports by Ruoff (1968) as above and Ward (1960) who had described nine cases of jejuno-jejuno and jejuno-ileal intussusception. In eight of these patients a tumour was present. In the ninth no cause was found, laparotomy and barium studies were negative and there was no direct evidence of coeliac disease. Masterson and Sweeney (1976) reported transient intussusception in five of 31 patients with coehac disease (15%) and in one patient with tropical sprue. It was n o t seen in 18 other patients with malabsorption due to other causes. This was the only radiological sign in all their cases of malabsorption which was specific for coeliac/sprue patients. Marshak and Lindner (1976) stated that intussusception is not uncommon in coeliac disease, but that it is easily missed on account of its transient nature. Sellink (1976) expressed strong doubts about the occurrence of transient intussusception in coeliac disease and considered that reports of such cases were due to incorrect interpretation because of inadequate
534
CLINICAL RADIOLOGY
filling and misleading patterns. His statement that 'we have never been able to demonstrate this phenomenon in our patients with the enteroclysis m e t h o d ' is likely to reflect upon the technique rather than file occurrence o f this phenomenon. No mention of intussusception in adult coeliac disease is made in several standard gastrointestinal and radiological texts (Margulis and Burhenne, 1967; Cecil and Loeb, 1971; Morson and Dawson, 1972; Bockhus, 1974; Sutton, 1975). There is, therefore, disagreement in the literature concerning the occurrence of transient non-obstructive intussusception in adult coeliac disease and, despite statements that the association is 'not u n c o m m o n ' there are few case reports.
(d) Reasons for Disagreement 1. Intussusception in coeliac disease is nonobstructive and symptomless and the patient does not, therefore, seek medical advice when it occurs. 2. The lesion is transient and may, therefore, be missed on radiological examination. 3. The techniques of small bowel examination vary greatly and it is not surprising that the traditional unsupervised follow-through routine fails to demonstrate transient lesions. A specific small bowel meal results in much greater supervision with a consequently higher pick-up rate. 4. It is unclear why enteroclysis techniques do not demonstrate these lesions, but this may be related to the bowel dilatation by contrast infusion.
CONCLUSION In the period between August 1974 and May 1977 we have seen six cases of intussusception in adult coeliac disease. During this period about 400 small bowel meals were performed annually, the majority for known or suspected inflammatory bowel disease. Signs o f malabsorption were demonstrated in 5% o f all examinations, but only half o f these actually had proven or suspected coeliac disease. Thus approximately 30 cases of coeliac disease were examined in the period and the m i n i m u m incidence o f intussusception was, therefore 20%. Thus intussusception in adult coeliac disease is truly 'not u n c o m m o n ' , the incidence o f finding it being related to the intensity o f searching. It is,
therefore, an additional radiological sign in the diagnosis of coeliac disease although in none of our cases was it seen in the absence o f other prominent radiological evidence o f malabsorption. Also it may well be specific for coeliac disease. Acknowledgements. - We are indebted to the Department of Medical Photography at Leeds (St James's) University Hospital for the illustrations and to Miss Janet Lunn for the many hours spent preparing the typescript.
REFERENCES Bloch, C. & Peck, H. (1964). Transeint intussusception in sprue. Journal of the Mount Sinai Hospital, 31,236-241. Bockhus, H. (1974). Gastro-enterology, Saunders, London. Burrows, F. G. O. & Toye, D. M. (1974). Barium studies in coeliac disease. Clinics in Gastro-enterology, 3, 91-109. Cecil, R. L. & Loeb, R. S. (1971). Textbook of Medicine. Saunders, London. Cihak, R. J., Keynes, W. M. & Schiller, K. F. R. (1969). Gluten induced enterography and stagnant loop syndrome in the same patient. Annals of Surgery, 169, 429-435. Cooke, W. T. & Asquith, P. A. (1974). Introduction and definitions of coeliac disease. Clinics in Gastroenterology. 3, 3-9. Golden, R. (1959). Radiologic Examination o f the Small Intestine. Thomas, Springfield. Isbell, R. G., Carlson, M. C. & Hoffman, M. N. (1969). Roentgenolic-patholic correlation in malabsorption syndromes. American Journal of R oentgenology, 107, 158-169. Laws, J. W. (1964). Recent Advances m Radiology, 4th ed, ed. Lodge, T., pp. 103-123. Margulis, A. R. & Burhenne, H. J. (1967). Alimentary Tract Roentgenology. Mosby, St Louis. Marshak, R. H. & Lindner, A. E. (1966). Malabsorption syndrome. Seminars in Roentgenology, 1,138-177. Marshak, R. H. & Lindner, A. E. (1976). Radiology o¢ the Small Intestine. Saunders, London. Masterson, J. B. & Sweeney, R. C. (1976). The role of small bowel follow-through examination in the diagnosis of coeliac disease. British Journal of Radiology, 49, 660-664. Morson, B. C. & Dawson, I. M. P. (1972). Gastrointestinal Pathology. Blackwell, London. Reymond, R. D. (1972). The mechanism of Intussusception. British Journal of Radiology, 45, 1-7. Ruoff, M., Lindner, A. E. & Marshak, R. H. (1968). Intussusception in sprue. American Journal o¢ Roentgenology 104, 525-528. Sellink, J. L. (1976). Radiologic Atlas of Common Diseases of the Small Bowel Kroese, Leiden. Sutton, D. (1975). Textbook of Radtology. Livingstone, Edinburgh. Ward, P. R. (1960). Jejunal intussusception. British Journal of Radiology, 33,691-694.
Intussusception confined to the small bowel is rare. In adults, as distinct from children, an organic cause is usually identifiable. A review of the literature revealed disagreement concerning the occurrence and incidence of transient non-obstructive intussusception in adult coeliac disease. The case histories and radiological findings of six adult coeliac patients with transient intussusception are described and the possible reasons for disagreement in the literature discussed.
DISCUSSION
(a) Diagnosis of Coeliac Disease
Coeliac disease (idiopathic steatorrhoea) is characterised by diarrhoea, abnormal stools, weight loss and the effects of malabsorption. It presents in a wide spectrum of severity ranging from severe illness, through mild intermittent diarrhoea to asymptomatic patients (Cooke and Asquith, 1974). The diagnosis is made by obtaining an abnormal jejunal mucosal biopsy, demonstrating improvement on a gluten-free diet and relapse on further gluten challenge. Cooke and METHODS Asquith (1974) discuss the problems with this rigid At the General Infirmary at Leeds patients with definition (e.g. the response to diet may only be suspected small bowel diseases are examined by a minimal) and suggest that one should accept all specific small bowel meal. The patient is prepared patients with a flat jejunal as having coeliac disease. The radiological signs of malabsorption are nonas for a barium meal by low residue diet, purgation and fasting. After a control film, 300 ml of a dilute, specific although they are typically most marked in isotonic, cold barium suspension, with 10ml Gastro- coeliac disease, compared with other causes of realgrafin added as an accelerator, is given orally. Prone absorption. There is uniform, flaccid jejunal dilatation overcouch films are taken at 10, 30 and 60min to 30mm or more (Laws, 1964) which is related to supplemented by screening and spot films with and the patient's clinical condition rather than to the without compression. If the terminal ileum has not degree of mucosal atrophy or steatorrhoea (Laws, been reached at 60 min Metaclopramide 10 mg i.v. 1964; Burrows and Toye, 1974) (Fig. 3). Dilatation is given and later films taken as required (96% of all causes the mucosal folds to be transversely orientated, examinations are completed by 90min using this but unless there is associated hypoproteinaemia or method). The whole examination is conducted by inflammation, they are not thickened (Marshak and one radiologist and radiographic team in one room Lindner, 1966). The folds may be obviously atrophic being shallow and widely spaced and in severe cases devoted exclusively to the small bowel meal session. may be completely effaced. In addition there is an excess of fluid in the bowel which dilutes the barium (Fig. 2). The transit time may be markedly prolonged RESULTS compared with the normal range for the technique used. The use of a stable barium suspension largely See Table 1.
530
CLINICAL
RADIOLOGY
Fig. 1 - Case 1. B.D. (a) The 20 m i n radiograph shows dilated
loops o f small bowel with straightening o f m u c o s a l folds and dilution of contrast. No intussusception is seen. (b) Intussusception shown at 1 h. prevents flocculation, but this may still be a prominent feature in coeliac disease (Figs. 2, 4). All six of our patients had typical clinical, biochemical and radiologicat features of coeliac disease. In four, diagnostic jejunal biopsies were obtained. In the fifth patient biopsy was technically unsuccessful and the sixth patient left the country before biopsy could be obtained (Table 1).
(b) Mechanism of Intussusception Reymond (1972) discusses the physical principles of the formation of an intussusception. He divides the causes of intussusception into two groups. In his first group there is a mechanical linkage across nonadjacent segments, e.g. adhesions, inflammation. The second group have a local area of bowel wall which does not contract normally. Unbalanced peristaltic forces might result when a normal peristaltic wave reaches an abnormal area of bowel wall. These forces may be sufficient to rotate a segment of bowel wall inwards, thus initiating the intussusception. In coeliac disease it is postulated that dilated flaccid loops of bowel might disturb normal peristalic waves, resulting in intussusception.
(c) Intussusception in Coeliac Disease The overall incidence of intussusception in children is approximately 2 per 1000 live births. It is much less common in adults (Morson and Dawson, 1972). Intussusception of small bowel into small bowel is rare and represents only 6% of all intussusceptions (Ward, 1960). In children it is usual to find no organic cause for the intussusception. In adults an organic aetiology is found in over 80% of casesfrequently a tumour or polyp, (Morson and Dawson, 1972; Bloch and Peck, 1964). Case reports of intussusception in coeliac disease are uncommon. Golden (1959) reported a 53-year-old male with steatorrhoea upon whom two small bowel barium studies eight days apart both showed evidence Fig. 2 Case 2. S.B. There is m a r k e d flocculation o f the barium with some uniform dilatation. A typically intus- of malabsorption and an intussusception, the site of which differed on each examination. Bloch and Peck susception is seen. r
7
Fig. 3 - Case 3. M.G. (a) The 3 0 m i n radiograph shows flaccid dilatation of loops. The mucosal folds are straightened, but not thickened. (b) Intussusception shows at 2% h.
Fig. 4 - Case 4. C.J. (a) Despite marked flocculation an intussusception is demonstrated. (b) A close-up of the intussusception shown in (a).
\
i 4a \.J
~ , 2 -~ 7 ~ ~ ;
532
CLINICAL RADIOLOGY
Table 1 - Details o f cases of transient small bowel intussusception in adult coeliac disease
Presentmg symptoms
Fecal fat
Case 1. B.D. Male born 1920
1964: Ten weeks diarrhoea 1975: Weight loss and diarrhoea (in association with dietary lapse)
76g per three days
Case 2. S.B. Female born 1940
1974: Six months diarrhoea. 7 kg weight loss pallor
1974: Subtotalvillous 1974: Uniform, flac- Excellentatrophy with plasma cid dilatation of weight incell infiltration small bowel loops creased 10 1975 : (One year after with transverse muco- kg in three starting gluten free sal folds, marked months diet) - marked imflocculation and provement but not dilution of contrast. completely normal A transient nonobstructive intussusception was seen at 60 rain (Fig. 2)
Case 3. M.G. Male born 1937
1977: Two years intermittent diarrhoea. 12 kg weight loss. Pale smelly stools
1977: Villous atrophy 1977: Dilated flaccid Diet started and cellular infiltrate loops of small bowel April 1977 with atrophic folds, and excess of fluid and flocculation of contrast. A transient non-obstructive intussusception was seen on the 23Ah radiograph (Fig. 3)
Case 4. C.J. Female born 1948
1972: Three weeks diarrhoea settled spontaneously 1973: Five weeks diarrhoea, pale stool 1974: Well
1974: Attempted biopsy-duodenal mucosa was obtained
159 g per five days
_Jejunal biopsy
Small bowel meal
Response to gluten-free diet
Present state
1964: Subtotal villous 1964: Barium follow Clinical imWell atrophy with no through was reprovement. cellular infiltrate ported as showing Well until 1975 : Subtotal villous 'dilated jejunal relapse in atrophy with a loops with an 1975 heavy infiltration intussusception in 1975: Dramatic of plasma cells, the left iliac fossa' improvement lymphocytes and 1975: Flaccid dilataon reinstieosinophils tion of loops and tuting dietary persistent floccucontrol lation with a nonobstructive intussusception at 60 min (Fig. 1)
1977: Marked flocculation and segmentation of the barium. Dilatation not marked. At least two transient non-obstructive intussusceptions were seen (Fig. 4)
Case 5. S.N.T. 1974: Persistent epiFemale born gastric pain on and off 1946 for years. Alternating diarrhoea and constipation
1974: Uniform flaccid Not done Not done-departed dilatation (Fig. 5) overseas. No followup
Case 6. E.V.T. 1976: Lower abdominal Male born pain and bleeding per 1939 rectum. Pale stool. No diarrhoea
Improvement March 1977: Loss of Dilatation, flocculation and promivilli infiltration with plasma cells, lympho- nent transverse mucosal folds. An cytes, macrophages intussusception is and some polyseen on the 1½h film rnorphis (Fig. 6) May 1977: Improvement but not normal
Well
No diarrhoea since 1974not on gluten-free diet
TRANSIENT
SMALL
BOWEL
INTUSSUSCEPTION
:
®
IN ADULT
COELIAC
DISEASE
533
o ,2 ~
..
®-N
Fig. 5 - Case 5. S.N.T. A less o b v i o u s b u t d e f i n i t e intuss u s c e p t i o n p r e s e n t in i l e u m a t 1% h.
Fig. 6 - Case 6. E.V.T. I n t u s s u s c e p t i o n p r e s e n t in j e j u n u m at 1% h a l m o s t o b s c u r e d b y a s s o c i a t e d f l o c c u l a t i o n .
(1964) reported a 29-year-old female with diarrhoea, weight loss and jejunal biopsy typical of coeliac disease with radiological evidence of malabsorption and a symptomless non-obstructive intussusception in the distal jejunum. Ruoff et al. (1968) stated that 'intussusception is not uncommon in sprue' and reported one case of a 45-year-old male who presented with severe upper abdominal pain. Barium studies of the small bowel two weeks later, when pain was no longer present, showed signs of malabsorption and a non-obstructive intussusception. Jejunal biopsy was typical of coeliac disease. Cihak et al. (1969) review the notes of an adult patient with proven coeliac disease. At the age of 7 years this patient had pale bulky stools and at 12 years of age had presented with intestinal obstruction. Laparotomy revealed a terminal ileal intussusception in association with enlarged mesenteric nodes (a recognised cause of intussusception). This patient is not typical of the cases we have presented and it is probable that the intussusception was unrelated to the coeliac disease. Isbell et al. (1969) noted transient intussusceptions in three out of 33 patients with coeliac disease and also one of two with giardiasis, in review of 68 small bowel barium studies with signs of malabsorption.
They considered it to be a valuable sign in establishing the diagnosis of sprue. Burrows and Toye (1974) reported having seen intussusception in 18 children with coefiac disease and they imply that this occurs commonly in adult coeliac patients. They quoted reports by Ruoff (1968) as above and Ward (1960) who had described nine cases of jejuno-jejuno and jejuno-ileal intussusception. In eight of these patients a tumour was present. In the ninth no cause was found, laparotomy and barium studies were negative and there was no direct evidence of coeliac disease. Masterson and Sweeney (1976) reported transient intussusception in five of 31 patients with coehac disease (15%) and in one patient with tropical sprue. It was n o t seen in 18 other patients with malabsorption due to other causes. This was the only radiological sign in all their cases of malabsorption which was specific for coeliac/sprue patients. Marshak and Lindner (1976) stated that intussusception is not uncommon in coeliac disease, but that it is easily missed on account of its transient nature. Sellink (1976) expressed strong doubts about the occurrence of transient intussusception in coeliac disease and considered that reports of such cases were due to incorrect interpretation because of inadequate
534
CLINICAL RADIOLOGY
filling and misleading patterns. His statement that 'we have never been able to demonstrate this phenomenon in our patients with the enteroclysis m e t h o d ' is likely to reflect upon the technique rather than file occurrence o f this phenomenon. No mention of intussusception in adult coeliac disease is made in several standard gastrointestinal and radiological texts (Margulis and Burhenne, 1967; Cecil and Loeb, 1971; Morson and Dawson, 1972; Bockhus, 1974; Sutton, 1975). There is, therefore, disagreement in the literature concerning the occurrence of transient non-obstructive intussusception in adult coeliac disease and, despite statements that the association is 'not u n c o m m o n ' there are few case reports.
(d) Reasons for Disagreement 1. Intussusception in coeliac disease is nonobstructive and symptomless and the patient does not, therefore, seek medical advice when it occurs. 2. The lesion is transient and may, therefore, be missed on radiological examination. 3. The techniques of small bowel examination vary greatly and it is not surprising that the traditional unsupervised follow-through routine fails to demonstrate transient lesions. A specific small bowel meal results in much greater supervision with a consequently higher pick-up rate. 4. It is unclear why enteroclysis techniques do not demonstrate these lesions, but this may be related to the bowel dilatation by contrast infusion.
CONCLUSION In the period between August 1974 and May 1977 we have seen six cases of intussusception in adult coeliac disease. During this period about 400 small bowel meals were performed annually, the majority for known or suspected inflammatory bowel disease. Signs o f malabsorption were demonstrated in 5% o f all examinations, but only half o f these actually had proven or suspected coeliac disease. Thus approximately 30 cases of coeliac disease were examined in the period and the m i n i m u m incidence o f intussusception was, therefore 20%. Thus intussusception in adult coeliac disease is truly 'not u n c o m m o n ' , the incidence o f finding it being related to the intensity o f searching. It is,
therefore, an additional radiological sign in the diagnosis of coeliac disease although in none of our cases was it seen in the absence o f other prominent radiological evidence o f malabsorption. Also it may well be specific for coeliac disease. Acknowledgements. - We are indebted to the Department of Medical Photography at Leeds (St James's) University Hospital for the illustrations and to Miss Janet Lunn for the many hours spent preparing the typescript.
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