Gastrointestinal
Gastrointest RadioI 2, 109-111 (1977)
Radiology i;2 by Springer-Verlag 1977
Pneumatosis Cystoides Intestinalis and Obstructing Intussusception in Celiac Disease Paul H. Frank and Denis J. O'Connell Department of Radiology, The University of Chicago, Chicago, Illinois, USA
Abstract. Spontaneous pneumoperitoneum and pneumatosis cystoides intestinalis have been undescribed complications of celiac disease. In the case reported, small bowel gas cysts caused an obstructing intussusception. The etiology of the pneumatosis cystoides intestinalis is discussed. Key words: Celiac disease - Spontaneous pneumoperitoneum - Pneumatosis cystoides intestinalis Obstructing intussusception.
The radiologic appearances of celiac disease have been well described [1, 2]. The clinical and radiologic literature contains many references to the more unusual features and complications. These include transient intussusception [2, 3], megacolon [4], benign ulceration and strictures of the small intestine [5], and increased incidence of neoplasms in the gastrointestinal tract [6]. We wish to report the details of a case of celiac disease associated with pneumatosis cystoides intestinalis (PCI) of the small intestine and obstructing intussusception, complications not previously described in the literature.
Case Report The patient, a 48-year-old Caucasian female, was admitted to The University of Chicago Hospitals and Clinics in December 1976 with weight loss. abdominal pain, diarrhea, and ankle edema. A diagnosis of celiac disease had been made in 1969. There had been a moderate response to gluten and lactose restriction. The patient's symptoms were controlled when 15 mg of prednisone daily were added. The corticosteroids were later reduced, and she remained well on a 5 mg maintenance dose of prednisone daily. In 1975 a mastectomy was performed for an early infiltrative carcinoma. The patient developed weight loss in January I976, associated with A&h'ess reprint requests to." D.J. O'Connell M.D., Department of Radiology (Box 429), The University of Chicago, 950 East 59th Street, Chicago, IL 60637, USA
pain in the legs. ankle edema, and muscle weakness. She was admitted elsewhere and considered to have iatrogenic Cushing's disease with steroid-induced myopathy. Before her admission to The University of Chicago Hospitals and Clinics, the patient complained of abdominal pain and distention. The initial investigations were as follows:hemoglobin 13.3 g/100 ml, hematocrit 39.9%, mean corpuscular hemaglobin concentration 12 (normal 26 37), white cell count 12,000. normal platelet count. Reticulocytes constituted 4% of the peripheral red cell count. The blood film showed polychromasia, anisocytosis, and poikilocytosis. The serum iron was low (45 I-tg/%) with a binding capacity of 420 pg/%. There were Ilo other biochemical abnormalities. The admission chest radiograph showed free air under the right diaphragm. The lungs were clear. Twelve hours later a barimn follow-through examination rexealed a diffuse abnormality consisting of nonpliable jejunal loops with complete loss of the normal fold pattern and thickening of the bowel wall. There was PCI in several loops of ilemn in the pelvis and right upper quadrant (Fig. 1). The p n e u m o p e r i t o n e u m was thought to be secondary to the PC1 [7]. A small bowel biopsy of the jejunum, performed four days later, showed partial villous atrophy with a heavy infiltrate of histiocytes and lymphocytes in the lamina propria of the bowel wall. The PC1 was again noted five days later when a plain abdominal radiograph was taken (Fig. 2). There was then a dramatic change in the patient's clinical condition. She complained of acute abdominal pain. On examination there was diffuse abdominal tenderness and guarding, but no masses were palpable. There were increased bowel sounds. The plain abdominal radiograph (Fig. 3) revealed a large filling defect in the transverse colon, expanding the lumen of the colon, and associated dilatation. A diagnosis of an ileocolic intussusception was made and confirmed at laparotomy. The surgeon found extensive pneumatosis intestinalis in the distal jejunum and in the distal five feet of the ileum. The portion of the ileum containing the intramural gas was involved in the intussusception. There were no signs of bowel necrosis at the site of the intussusception, which was easily reduced. No evidence of malignancy in the remainder of the small bowel was found. The patient made an uneventful recovery and was discharged on a gluten-free diet and 5 mg of prednisone daily. A plain abdominal radiograph taken two weeks alter the surgical procedure showed no evidence of PCI or pneumoperitoneum.
Comment." This patient with known celiac disease presented with abdominal pain, PCI, and pneumoperitoneum. Five days later an acute, obstructing intussusception developed in the region of the pneumatosis intestinalis. It is thought that the ileal pneumatosis was the underlying cause of the intussusception.
110
P.H. Frank and D.J. O'Connell: Pneumatosis and Intussusception in Celiac Disease
Fig. 1. The j e j u n u m is smooth, narrow, and featureless. There is pneumatosis cystoides intestinalis of several ileal loops in the pelvis and right upper quadrant (arrows)
Fig. 3, A mass representing the head of the ileocolic intussusception is arrowed. The transverse colon is expanded at this site, and there is proximal small bowel dilatation. There is no evidence of PCI
Fig. 2. The small bowel pneumatosis persists. The intramural gas is similar in position to that noted previously
Discussion
A large number of gastrointestinal diseases have been described in association with PCI [8]. When the PCI is confined to the small intestine, there is usually an underlying disorder of an obstructive, ischemic,
or ulcerative nature [8]. This is in contrast to pneumatosis coli, which is frequently idiopathic or related to chronic airway obstruction [9, 10]. In the context of this patient's proven background of celiac disease, it is of interest to speculate on the possible etiology of the PC1 and subsequent obstruct-
P.H. Frank and D.J. O'Connell: Pneumatosis and Intussusception in Celiac Disease
ing ileocolic intussusception. Small bowel intussusceptions are accepted as a frequent occurrence in celiac disease [2, 3]. However, these intussusceptions are classically transient and nonobstructing, with the exception of a single case of prolonged intussusception occurring in tropical sprue [11]. In the case described here the barium follow-through examination revealed PCI of the ileum, but no intussusceptions were present until the development of the obstructing ileocolic intussusception eight days after the radiologic diagnosis of PCI. Several authors have described jejunoileal ulceration and strictures in celiac disease [4,5]. Theoretically, the mucosal ulceration caused by these rare complications might explain the introduction of intraluminal gas into the bowel wall. The barium followthrough examination of our patient, however, showed no evidence of strictures or ulceration, and this was confirmed at laparotomy. There is an increased incidence of gastrointestinal carcinoma and lymphoma in patients with celiac disease [4, 6]. Obstructing malignant lesions can occasionally cause PCI [12]. In the case described here there was no evidence of malignancy either in the radiological study of the small bowel or at laparotomy. The role of corticosteroids is of some interest. Borns and Johnston [13] described 15 cases of childhood leukemia complicated by pneumatosis intestinalis of the ileum and cecum. Their patients were taking immunosuppressives and steroids. These authors postulated that steroids caused lymphoid depletion and atrophy of the Peyer's patches in the ileocecal region, thereby allowing air to dissect into the bowel wall. While this complication of steroid therapy is an attractive theory, it has not been described in adults. As our patient was taking maintenance doses of steroids, it is conceivable that steroids were a contributing factor in the pathogenesis of the PCI. Yunich and Fradkin [14] described a case of diffuse PCI of the small intestine associated with profuse diarrhea and malabsorption. At autopsy their patient had no microscopic evidence of celiac disease. These authors postulated that the acute diarrheal syndrome was caused by the extensive small bowel PCI. It is known that pneumatosis coli can cause diarrhea [9], a fact which supports Yunich and Fradkin's hypothesis.
111
The clinical presentation and underlying pathology in our case appears to be unique. It is felt that our patient's documented celiac disease was the cause of the small intestinal PCI. As polypoid gastrointestinal lesions are well known for their association with intussusception [15], we believe that the small intestinal gas cysts were the cause of the obstructing ileocolic intussusception. Acl,-nowledgments. We are grateful to Dr. Charles S. Winans for providing the clinical details of the case.
References 1. Laws JW, Booth CC, Shawdon H, Stewart JS: Correlation of radiological and histological findings in idiopathic steatorrhoea. Br M e d J 1:1311 1314, 1963 2. Masterson JB, Sweeney EC : The role of the small bowel followthrough examination in the diagnosis of coeliac disease. Br J Radiol 49:660 664, 1976 3. Ruoff M, Lindner AE. Marshak RH: Intussusception in sprue. Am J Roentgenol Radium Ther Nucl Med 104:525 528, 1968 4. Burrows FGO, Toye D K M : Barium studies. Clin Gastroentero13:91 106, 1974 5. Bayless TM, Kaplowitz RF, Shelley WM, Ballinger II WF, Hen&ix TR: Intestinal u l c e r a t i o n - A complication of coeliac disease. N Engl J M e d 276:996 1002. 1967 6. Gough Kr, Read AE, Naish JM: Intestinal reticulosis as a complication of idiopathic steatorrhoea. Gut 3:232 239, 1962 7. Wright FW, Lumsden K: Recurrent pneumoperitoneum due to jejunal diverticulosis. Clin Radiol 26:327 33I, 1975 8. Olmsted WW, Madewell JE: Pneumatosis cystoides intestinalis. Gastrointest Radiol l:177 181, 1976 9. O'Connell D J. Dewbury KC. Green B, Wyatt AP: The plain abdominal radiograph in pneumatosis coli. Clm Radiol 27:563 568, 1976 10. Keyting WS. McCarver RR, Kovarik JL. Daywitt AL: Pneumatosis intestinalis : A new concept. Radiology 76:733 741, 1961 11. Cortell S, Rieber EE, Sheehy TW, Conrad AE: Tropical sprue and intussusception: An unusual association. Am J Dig Dis 12:216 221, 1967 12. Shallal J. van Heerden J. Bartholomew L. Cain JW: Pneumatosis cystoides intestinalis. Mayo Clin Proc 49:180 184. 1974 13. Borns PF, Johnston TA: Indolent pneumatosis of bowel wall associated with minimal suppressive therapy. Ann Radiol (Paris2 16:I63 166, 1973 14. Yunich AM, Fradkin NF: Fatal sprue (malabsorption) syndrome secondary to extensive pneumatosis cystoides intestinalis. Gastroenterology 35:212 216, 1958 15. Ward PR: Jejunal intussusception. Br J Radiol 33:691 694, 1960
Received." June 27, 1977: accepted: Ju(l" 21. 1977
Gastrointest RadioI 2, 109-111 (1977)
Radiology i;2 by Springer-Verlag 1977
Pneumatosis Cystoides Intestinalis and Obstructing Intussusception in Celiac Disease Paul H. Frank and Denis J. O'Connell Department of Radiology, The University of Chicago, Chicago, Illinois, USA
Abstract. Spontaneous pneumoperitoneum and pneumatosis cystoides intestinalis have been undescribed complications of celiac disease. In the case reported, small bowel gas cysts caused an obstructing intussusception. The etiology of the pneumatosis cystoides intestinalis is discussed. Key words: Celiac disease - Spontaneous pneumoperitoneum - Pneumatosis cystoides intestinalis Obstructing intussusception.
The radiologic appearances of celiac disease have been well described [1, 2]. The clinical and radiologic literature contains many references to the more unusual features and complications. These include transient intussusception [2, 3], megacolon [4], benign ulceration and strictures of the small intestine [5], and increased incidence of neoplasms in the gastrointestinal tract [6]. We wish to report the details of a case of celiac disease associated with pneumatosis cystoides intestinalis (PCI) of the small intestine and obstructing intussusception, complications not previously described in the literature.
Case Report The patient, a 48-year-old Caucasian female, was admitted to The University of Chicago Hospitals and Clinics in December 1976 with weight loss. abdominal pain, diarrhea, and ankle edema. A diagnosis of celiac disease had been made in 1969. There had been a moderate response to gluten and lactose restriction. The patient's symptoms were controlled when 15 mg of prednisone daily were added. The corticosteroids were later reduced, and she remained well on a 5 mg maintenance dose of prednisone daily. In 1975 a mastectomy was performed for an early infiltrative carcinoma. The patient developed weight loss in January I976, associated with A&h'ess reprint requests to." D.J. O'Connell M.D., Department of Radiology (Box 429), The University of Chicago, 950 East 59th Street, Chicago, IL 60637, USA
pain in the legs. ankle edema, and muscle weakness. She was admitted elsewhere and considered to have iatrogenic Cushing's disease with steroid-induced myopathy. Before her admission to The University of Chicago Hospitals and Clinics, the patient complained of abdominal pain and distention. The initial investigations were as follows:hemoglobin 13.3 g/100 ml, hematocrit 39.9%, mean corpuscular hemaglobin concentration 12 (normal 26 37), white cell count 12,000. normal platelet count. Reticulocytes constituted 4% of the peripheral red cell count. The blood film showed polychromasia, anisocytosis, and poikilocytosis. The serum iron was low (45 I-tg/%) with a binding capacity of 420 pg/%. There were Ilo other biochemical abnormalities. The admission chest radiograph showed free air under the right diaphragm. The lungs were clear. Twelve hours later a barimn follow-through examination rexealed a diffuse abnormality consisting of nonpliable jejunal loops with complete loss of the normal fold pattern and thickening of the bowel wall. There was PCI in several loops of ilemn in the pelvis and right upper quadrant (Fig. 1). The p n e u m o p e r i t o n e u m was thought to be secondary to the PC1 [7]. A small bowel biopsy of the jejunum, performed four days later, showed partial villous atrophy with a heavy infiltrate of histiocytes and lymphocytes in the lamina propria of the bowel wall. The PC1 was again noted five days later when a plain abdominal radiograph was taken (Fig. 2). There was then a dramatic change in the patient's clinical condition. She complained of acute abdominal pain. On examination there was diffuse abdominal tenderness and guarding, but no masses were palpable. There were increased bowel sounds. The plain abdominal radiograph (Fig. 3) revealed a large filling defect in the transverse colon, expanding the lumen of the colon, and associated dilatation. A diagnosis of an ileocolic intussusception was made and confirmed at laparotomy. The surgeon found extensive pneumatosis intestinalis in the distal jejunum and in the distal five feet of the ileum. The portion of the ileum containing the intramural gas was involved in the intussusception. There were no signs of bowel necrosis at the site of the intussusception, which was easily reduced. No evidence of malignancy in the remainder of the small bowel was found. The patient made an uneventful recovery and was discharged on a gluten-free diet and 5 mg of prednisone daily. A plain abdominal radiograph taken two weeks alter the surgical procedure showed no evidence of PCI or pneumoperitoneum.
Comment." This patient with known celiac disease presented with abdominal pain, PCI, and pneumoperitoneum. Five days later an acute, obstructing intussusception developed in the region of the pneumatosis intestinalis. It is thought that the ileal pneumatosis was the underlying cause of the intussusception.
110
P.H. Frank and D.J. O'Connell: Pneumatosis and Intussusception in Celiac Disease
Fig. 1. The j e j u n u m is smooth, narrow, and featureless. There is pneumatosis cystoides intestinalis of several ileal loops in the pelvis and right upper quadrant (arrows)
Fig. 3, A mass representing the head of the ileocolic intussusception is arrowed. The transverse colon is expanded at this site, and there is proximal small bowel dilatation. There is no evidence of PCI
Fig. 2. The small bowel pneumatosis persists. The intramural gas is similar in position to that noted previously
Discussion
A large number of gastrointestinal diseases have been described in association with PCI [8]. When the PCI is confined to the small intestine, there is usually an underlying disorder of an obstructive, ischemic,
or ulcerative nature [8]. This is in contrast to pneumatosis coli, which is frequently idiopathic or related to chronic airway obstruction [9, 10]. In the context of this patient's proven background of celiac disease, it is of interest to speculate on the possible etiology of the PC1 and subsequent obstruct-
P.H. Frank and D.J. O'Connell: Pneumatosis and Intussusception in Celiac Disease
ing ileocolic intussusception. Small bowel intussusceptions are accepted as a frequent occurrence in celiac disease [2, 3]. However, these intussusceptions are classically transient and nonobstructing, with the exception of a single case of prolonged intussusception occurring in tropical sprue [11]. In the case described here the barium follow-through examination revealed PCI of the ileum, but no intussusceptions were present until the development of the obstructing ileocolic intussusception eight days after the radiologic diagnosis of PCI. Several authors have described jejunoileal ulceration and strictures in celiac disease [4,5]. Theoretically, the mucosal ulceration caused by these rare complications might explain the introduction of intraluminal gas into the bowel wall. The barium followthrough examination of our patient, however, showed no evidence of strictures or ulceration, and this was confirmed at laparotomy. There is an increased incidence of gastrointestinal carcinoma and lymphoma in patients with celiac disease [4, 6]. Obstructing malignant lesions can occasionally cause PCI [12]. In the case described here there was no evidence of malignancy either in the radiological study of the small bowel or at laparotomy. The role of corticosteroids is of some interest. Borns and Johnston [13] described 15 cases of childhood leukemia complicated by pneumatosis intestinalis of the ileum and cecum. Their patients were taking immunosuppressives and steroids. These authors postulated that steroids caused lymphoid depletion and atrophy of the Peyer's patches in the ileocecal region, thereby allowing air to dissect into the bowel wall. While this complication of steroid therapy is an attractive theory, it has not been described in adults. As our patient was taking maintenance doses of steroids, it is conceivable that steroids were a contributing factor in the pathogenesis of the PCI. Yunich and Fradkin [14] described a case of diffuse PCI of the small intestine associated with profuse diarrhea and malabsorption. At autopsy their patient had no microscopic evidence of celiac disease. These authors postulated that the acute diarrheal syndrome was caused by the extensive small bowel PCI. It is known that pneumatosis coli can cause diarrhea [9], a fact which supports Yunich and Fradkin's hypothesis.
111
The clinical presentation and underlying pathology in our case appears to be unique. It is felt that our patient's documented celiac disease was the cause of the small intestinal PCI. As polypoid gastrointestinal lesions are well known for their association with intussusception [15], we believe that the small intestinal gas cysts were the cause of the obstructing ileocolic intussusception. Acl,-nowledgments. We are grateful to Dr. Charles S. Winans for providing the clinical details of the case.
References 1. Laws JW, Booth CC, Shawdon H, Stewart JS: Correlation of radiological and histological findings in idiopathic steatorrhoea. Br M e d J 1:1311 1314, 1963 2. Masterson JB, Sweeney EC : The role of the small bowel followthrough examination in the diagnosis of coeliac disease. Br J Radiol 49:660 664, 1976 3. Ruoff M, Lindner AE. Marshak RH: Intussusception in sprue. Am J Roentgenol Radium Ther Nucl Med 104:525 528, 1968 4. Burrows FGO, Toye D K M : Barium studies. Clin Gastroentero13:91 106, 1974 5. Bayless TM, Kaplowitz RF, Shelley WM, Ballinger II WF, Hen&ix TR: Intestinal u l c e r a t i o n - A complication of coeliac disease. N Engl J M e d 276:996 1002. 1967 6. Gough Kr, Read AE, Naish JM: Intestinal reticulosis as a complication of idiopathic steatorrhoea. Gut 3:232 239, 1962 7. Wright FW, Lumsden K: Recurrent pneumoperitoneum due to jejunal diverticulosis. Clin Radiol 26:327 33I, 1975 8. Olmsted WW, Madewell JE: Pneumatosis cystoides intestinalis. Gastrointest Radiol l:177 181, 1976 9. O'Connell D J. Dewbury KC. Green B, Wyatt AP: The plain abdominal radiograph in pneumatosis coli. Clm Radiol 27:563 568, 1976 10. Keyting WS. McCarver RR, Kovarik JL. Daywitt AL: Pneumatosis intestinalis : A new concept. Radiology 76:733 741, 1961 11. Cortell S, Rieber EE, Sheehy TW, Conrad AE: Tropical sprue and intussusception: An unusual association. Am J Dig Dis 12:216 221, 1967 12. Shallal J. van Heerden J. Bartholomew L. Cain JW: Pneumatosis cystoides intestinalis. Mayo Clin Proc 49:180 184. 1974 13. Borns PF, Johnston TA: Indolent pneumatosis of bowel wall associated with minimal suppressive therapy. Ann Radiol (Paris2 16:I63 166, 1973 14. Yunich AM, Fradkin NF: Fatal sprue (malabsorption) syndrome secondary to extensive pneumatosis cystoides intestinalis. Gastroenterology 35:212 216, 1958 15. Ward PR: Jejunal intussusception. Br J Radiol 33:691 694, 1960
Received." June 27, 1977: accepted: Ju(l" 21. 1977
