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Intussusception presenting with lower gastrointestinal hemorrhage in a hemophiliac
of 58% in 193 consecutive autopsies. 2 Kameya et al., however, reported an incidence of only 0.8% in 2,000 patients examined endoscopically.3 Similar frequencies have been reported in other endoscopic series: 0.33% by Zimmerman et al.,4 0.61 % by Aste et al.,5 and 0.38% by Terruzzi et al. 6 The lesion tends to be multiple in most series. Location of the xanthoma can vary, but in postgastrectomy patients the lesion is usually found near the stoma. 3,4 They are small lesions, ranging in size from 0.5 to 3 mm, but they have been reported to be as large as 10 mm. 2-5 No correlation exists between serum lipid profiles and the presence of gastric xanthoma.2-5,7 Both patients presented in our report had normal serum lipid profiles. The pathogenesis and etiology of gastric xanthoma is unknown, but a strong correlation exists between diseased gastric mucosa and the presence of xanthoma. In addition, an increased incidence has been observed in patients after gastric surgery.3,4 In previously reported series, xanthoma has been associated with gastritis, carcinoma, intestinal metaplasia of the gastric epithelium, and ulcer disease. 2-5,7,8 Terruzzi et al. 4 found a frequency of gastric xanthoma of 18% in patients who have undergone gastric resection. Domellof et al. 8 found that the prevalence of xanthoma after Billroth I resection varied with the interval after operation with 1.8% occurring 1 to 3 years after gastric surgery, 37.5% after 10 years, and 43% after 11 to 23 years. Following Billroth II
resection, xanthomas were found in 6.3% of the patients 1 to 3 years postoperatively, 35.7% after 15 years, 43.9% after 20 years, and in 59.5% after 23 years. 8 These data support the hypothesis of biliary reflux with impaired capacity of injured gastric mucosa to transport lipids, leading to lipid accumulation within the cells. 8 The presence of gastric xanthoma in itself is probably of little clinical significance. It is highly unlikely that xanthomas are responsible for any clinical symptoms, but are rather a reflection of either past or concurrent gastric pathology, especially in association with bile reflux.
Intussusception presenting with lower gastrointestinal hemorrhage in a hemophiliac
hemophiliac secondary to a cecocolic intussusception. At colonoscopy, the intussusception masqueraded as a polyp in the distal transverse colon. Our purpose is to report an extremely rare cause of lower gastrointestinal hemorrhage and to alert endoscopists to a potentially dangerous clinical situation: inadvertent snare cautery of an intussusception masquerading as a bleeding polyp.
M. P. Pauly, MD E. Watson-Williams, MD W. L. Trudeau, MD
Gastrointestinal hemorrhage is a significant complication of hemophilia in adults. The incidence ranges from 10% to 25%,1-3 with most of the bleeding lesions located in the upper gastrointestinal tract. Colonic sites are implicated in less than 20% of cases, and bleeding etiologies within this group vary widely.2 Intramural hematoma has been reported as a source of gastrointestinal hemorrhage, but rarely does it serve as a lead point of intussusception. This is a case report of hematochezia in an adult From the Department of Internal Medicine, Division of Gastroenterology, University of California, Davis, Davis, California. Reprint requests: W. L. Trudeau, MD, Division of Gastroenterology, 4301 X Street, Sacramento, California 95817. VOLUME 33, NO.2, 1987
REFERENCES 1. Feyrter F. Herdformige Lipoidablagerung in der Schleimhaut des Magens (Lipoidinseln der Magenschleimhaut-Lubarsch) Lipoidzellenkzotchen in der Schleimhaut des Darmes. Virchows Arch Pathol Anat Physiol 1929;273:736-41. 2. Kimura K, Hiramoto T, Buncher CR. Gastric xanthelasma, Arch PathoI1969;87:110-7. 3. Kameya S, Nakamura S, Mizutani K, et al. Xanthoma in the stomach. Gastrointest Endosc 1963;5:37-41. 4, Zimmermann W, Andratschke C, Leidl E, Pavel A, Breitkopf P. Hyperlipamie und Xanthomatose des Magen. Aktuel Gastrologie 1977;6:551-3. 5. Aste H, Cheli R, Nicolo G, Santi L. Gastric xanthelasma. Acta Endosc 1978;8:1-4. 6. Terruzzi V, Minoli G, Butti GC, Rossini A. Gastric lipid islands in the gastric stump and in non-operated stomach. Endoscopy 1980;12:58-62. 7. Mast A, Elewaut A, Mortier G, et al. Gastric xanthoma. Am J GastroenteroI1976;65:311-7. 8. Domellof L, Eriksson S, Helander HF, Janunger KG. Lipid islands in the gastric mucosa after resection for benign ulcer disease. Gastroenterology 1977;72:14-8.
CASE REPORT
A 25-year-old, factor VIII-deficient hemophiliac with inhibitors was admitted to the University of California, Davis Medical Center at Sacramento with hematochezia. Eighteen hours prior to admission he noted the abrupt onset of severe, intermittent, right-sided, crampy abdominal pain, initially associated with an episode of nausea and vomiting. Subsequently, he developed hematochezia with four bloody stools over the next 24 hours. The patient denied a recent history of trauma, constipation, rectal instrumentation, diarrhea, antibiotics, aspirin, or excessive alcohol. On examination, he was alert and oriented. His blood 115
pressure was 140/70 mm Hg, and he had postural tachycardia. The only other abnormalities noted were decreased bowel sounds and diffuse abdominal tenderness (more on the right). There were no palpable masses or organomegaly. There was a large amount of dark red blood in the rectal vault. Laboratory data included a white blood cell count of 10,000/mm3 , a hematocrit of 20%, and a platelet count of 333,000/mm3 • A urinalysis was normal. The bilirubin was 1.7 mg/dl, while other liver function tests were normal. Throughout his hospital course the abdominal findings varied. At one time his abdomen was distended and tympanic bowel sounds and rushes were heard; another time there was a fullness on the right side. Most of the time he was resting comfortably in bed while receiving intravenous meperidine every few hours. During the first 36 hours he was transfused 6 units of packed red blood cells and treated with Autoplex S (antiinhibitor coagulant complex), but the bloody stools continued. A plain film ofthe abdomen was normal. A technetium99 labeled red blood cell scan showed a discrete bleeding site in the left upper quadrant which was felt to be in the distal transverse colon (Fig. 1). On the second hospital day he was switched to porcine factor VIII (Hyate C) and on the third hospital day the bleeding stopped. At that time his hematocrit was 32%. On the fourth hospital day, colonoscopy was performed. At the mid-transverse colon a 2- to 3-cm discrete polypoid mass was encountered. The tip of the lesion was ulcerated and necrotic with an adherent clot, and it appeared to have a thick stalk (Fig. 2). The mucosa up to and surrounding the lesion appeared normal. Removal of the lesion by snare cautery was considered. With prolonged observation, the appearance of the lesion changed; the surrounding mucosa seemed to swell and decompress, and there was a marked amount of to and fro movement in an accordion-like fashion. At this point it was decided to proceed to surgical exploration. At laparotomy it was apparent that the lesion that mimicked a polyp was actually a submucosal hemorrhage at the tip of the cecum that served as the lead point for intussusception into the transverse colon. The tip of the cecum was polypoid with areas of hemorrhage and ulceration. The patient underwent right hemicolectomy and his subsequent course was uneventful. The surgical specimen consisted of a dusky ascending colon and a cecal mass completely invaginated into the most distal ascending colon and proximal transverse colon. A localized hematoma in the tip of the cecum was isolated and identified as the lead point of the intussusception. Microscopic examination of the mass confirmed that it was an intramural hematoma with organized clot, mucosal necrosis, and ulceration. DISCUSSION
Hemophilia A (factor VIII deficiency) is a disorder commonly associated with hemorrhage and trauma is the major precipitating factor. 4 However, spontaneous bleeding may occur involving the oral cavity, joints, muscles, urinary tract, and less frequently the gastrointestinal tract. 5 ,6 Gastrointestinal hemorrhage is 116
A
15 min.
8
40 min.
c
78 min. Lateral Figure 1. Technetium-99 labeled red bloodcell scan A, B, and
C. Note the pooling in the splenic flexure. uncommon and Mittal et a1. 2 recently reported a 10% incidence in 243 hemophiliacs followed over 10 years. Earlier reports of gastrointestinal bleeding in hemophiliacs range from 14.5% 7 to 25%.! Peptic ulcer disease is the most commonly diagnosed cause of gastrointestinal hemorrhage in hemophiliacs and accounted for over 22% of the bleeding esisodes in one series2 and 53% in another.! Other upper tract sources of hemorrhage in hemophiliacs parallel that of the general population and include gastritis, esophagitis, varices, and Mallory-Weiss tears. 1,2 Hemophiliacs presenting with gastrointestinal GASTROINTESTINAL ENDOSCOPY
Figure 2. Colonoscopic appearance of intussusception in transverse colon. bleeding are far less likely to have a lesion in the lower gastrointestinal tract. Less than 15% of the episodes of bleeding in Mittal's group of patients were due to colonic lesions. 2 Isolated cases of diverticulosis, proctitis, hemorrhhoids, and fissures were reported as well as two cases of intramural hematoma involving the small bowel and the sigmoid colon. Submucosal hemorrhages usually resolve upon correction of the clotting deficiency with factor VIII therapy.8 Rarely, lesions can present with bleeding into the intestinal lumen, and an occasional case has been reported with bleeding into the peritoneal cavity.5 Mechanical obstruction of the bowel has been reported with submucosal hemorrhage.5.8 Intussusception appears to be a rare complication of intramural hematoma. 8.9- 11 Review of the literature over the past 25 years revealed only six other cases of
intussusception in hemophiliacs with intramural hematoma, and these were all in the pediatric population (Table 1). The younger patients were more acute and classic in their presentation. Our patient, the oldest reported, typified the presentation so often encountered in adults-that of variable symptomatology with a subacute or indolent course. 12-I5 The classic triad of abdominal pain, vomiting, and rectal bleeding occurs in less than 28% of cases reported in detaiU 6 Intussusception in adults is rare, accounting for less than 5% of all cases of intussusception. Most large centers reporting their experiences with intussusception have less than two cases per year. 12. 13. 15-17 Colonic intussusceptions are far less common than enteric 15 with 55% to 90% due to a pathologic source such as lipomas, leiomyomas, and pOlyps,12.13,15-17 Since colonic intussusception in an adult more commonly has an organic cause, it is likely that the cecal submucosal hematoma in our patient acted as the lead point for the intussusception. I3 ,17 Because of the variable course of the disease and the paucity of cases seen by any one individual, the diagnosis of intussusception is often made later in the work-up or at laparotomy. A careful history and a high index of suspicion cannot be underestimated. Physical examination is very important and often suggestive of intussusception. Dance's sign (right-sided emptiness on abdominal examination), when found, correlates well with an ileocolic or cecocolic intussusception.IS Plain x-rays frequently are nonspecific but may show an ileus. 19 Intussusception has a characteristic appearance on barium enema,20 and descriptions of intussusception have been reported on CT scan 21 and ultrasonography.22.2:l Fiberoptic colonoscopy is rapidly establishing itself as a first line diagnostic procedure for the evaluation
Table 1. Case reports of intussusception in hemophiliacs Author, year reported"
Patient's age (yr)
Type of intussusception
Passaro et al., 1960"
12.0
Ileocolic
Quick, 1966"
15.0
Colocolic
Collins and Miller, 1968'0
2.5
Ileocolic
Donaldson and Clark, 1968"
9.0
Ileocolic
13.5
Ileocolic
Fripp and Karabus, 1977"
LeBlanc, 1982' Present case, 1985
8.0 25.0
Jejunojejunal Cecocolic
Signs and symptoms Crampy abdominal pain, rectal mass Abdominal pain, hematochezia Nausea, vomiting, abdominal pain, rightsided mass Vomiting, abdominal pain, no melena, 6+ stool Vomiting, abdominal pain, RUQ mass, negative stool Abdominal pain, epi. mass, no bleeding Abdominal pain
Plain x-rays Distended SB with fluid levels
Diagnosis Laparotomy X-ray studies
SBO
Laparotomy
Ileus
Laparotomy
Decreased air in colon
Reduced by BE
Distended SB and coIon Nonspecific gas pattern
Laparotomy Laparotomy
" Superior numbers are reference numbers. VOLUME 33, NO.2, 1987
117
of lower gastrointestinal bleeding. 24 •25 Colonoscopy has been more sensitive than a barium enema in the diagnosis of colonic lesions responsible for bleedini 4- 27 and has therapeutic advantages. In our patient the evaluation had to be accomplished as quickly as possible because of the anticipated anamnestic response of factor VIII antibodies occurring between 5 and 10 days after initiating treatment. At colonoscopy, the intussusception in our patient mimicked a polyp. However, the nausea, vomiting, abdominal pain, and tenderness were not consistent with the usual presentation of an uncomplicated bleeding polyp. The history and the physical examination were keys to the correct diagnosis of intussusception. Our case was actually a typical presentation of an extremely rare disease and one not usually encountered or diagnosed by colonoscopy. Intussusception in hemophiliacs can occur and is classically secondary to an intramural hematoma. Such a case is uncommon and has not previously been reported in an adult. The disease occurs so infrequently in adults that the diagnosis is often elusive. In this case the intussusception was first encountered at colonoscopy for lower gastrointestinal bleeding and masqueraded as a polyp. Subtle differences on colonoscopy and the clinical presentation suggested a more complex process. As colonoscopy increases in frequency as the primary diagnostic procedure for lower gastrointestinal bleeding, the examining physician must be aware of the polyp that is more than a polyp.
REFERENCES 1. Forbes CD, Barr RD, Prentice CRM, Douglas AS. Gastrointestinal bleeding in haemophilia. Q J Med 1973;167:503-11. 2. Mittal R, Spero JA, Lewis JH, et al. Patterns of gastrointestinal hemorrhage in hemophilia. Gastroenterology 1985;88:515-22. 3. Wilkinson JF, Nour-Eldin F, Esraels MCG, et al. Haemophilia syndromes: a survey of 267 patients. Lancet 1961;2:947-50. 4. LeBlanc KE. Jejuno-jejunal intussusception in a hemophiliac: a case report. Ann Emerg Med 1982;11:149-51. 5. Fripp RR, CD Karabus. Intussusception in haemophilia. A case report. SA Med J 1977;52:617-8. 6. Quick AJ. Emergencies in hemophilia. Am J Med Sci 1966;40916.
118
7. Carron DB, Boon TH, Walher FC. Peptic ulcer in the hemophiliac and its relation to gastrointestinal bleeding. Lancet 196;2:1036-9. 8. Adelman MI, Gishen P, Dubbins P, Mibashan RS. Localized' intramesenteric haemorrhage-a recognisable syndrome in haemophilia? Br Med J 1979;2:642-3. 9. Passaro EP, Dettman PM, Smith B. Surgery in the hemophiliac patient. Arch Surg 1960;81:864-9. 10. Collins DL, Miller KE. Intussusception in hemophilia. J Pediatr Surg 1968;3:599-603. 11. Donaldson MH, Clark RE. Successful operation for intussusception in the classic hemophiliac. Ann Surg 1968;6:1043-7. 12. Dean DL, Ellis FH, Sauer WG. Intussusception in adults. AMA Arch Surg 1955;6-11. 13. Roper A. Intussusception in adults. Surg Gynecol Obstet 1956;103:267-278. 14. Fawaz KA, Bloom SM, Pappas CA, Kellum JM. Adult intussusception presenting with transient intestinal ischemia. Am J Gastroenterol 1980;73:265-70. 15. Weilbaecher D, Bolin JA, Hearn D, Ogden W. Intussusception in adults. Review of 160 cases. Am J Surg 1971;121:531-5. 16. Smith MAP, Dent DM, Botha JBC. Intussusception in adults. S Afr J Surg 1978;16:139-43. 17. Donhauser JL, Kelley RC. Intussusception in the adult. Am J Surg 1950;79:673-6. 18. Sty JR, Babbitt DP, Boedecker RA. Radionuclide "dance sign." Clin Nucl Med 1980:11;502-3. 19. White SJ, Blane CEo Intussusception: additional observations on the plain radiograph. AJR 1982;139:511-3. 20. Young R, Bruk D. The radiology corner. Colonic intussusception in uremia. Am J GastroenteroI1979;71:229-32. 21. Donovan AT, Goldman SM. Computed tomography of ileocecal intussusception: mechanism and appearance. J Comput Assist Tomogr 1982;6:630-2. 22. Morin ME, Blumental DH, Ta A, Yuk LP. The ultrasonic appearance of ileocolic intussusception. J Clin Ultrasound 1981;9:516-8. 23. Holt S, Samuel E. Multiple concentric ring sign in the ultrasonographic diagnosis of intussusception. Gastrointest Radiol 1978;3:307-9. 24. Tedesco FJ, Gottfried EB, Corless JK, Brownstein RD. Prospective evaluation of hospitalized patients with nonactive lower intestinal bleeding-timing and role of barium enema and colonoscopy. Gastrointest Endosc 1984;30:281-3. 25. Jensen DM, Machicado GA. Emergent colonoscopy in patients with severe lower gastrointestinal bleeding (abstract). Gastroenterology 1981;80:1184. 26. Tedesco FJ, Waye JD, Raskin JB, Morris SJ, Greenwald RA. Colonoscopic evaluation of rectal bleeding. A study of 304 patients. Ann Intern Med 1978;89:907-9. 27. Brand ED, Sullivan BH, Sivak MV, Rankin GB. Colonoscopy in the diagnosis of unexplained rectal bleeding. Ann Surg 1980;192:111-3.
GASTROINTESTINAL ENDOSCOPY
resection, xanthomas were found in 6.3% of the patients 1 to 3 years postoperatively, 35.7% after 15 years, 43.9% after 20 years, and in 59.5% after 23 years. 8 These data support the hypothesis of biliary reflux with impaired capacity of injured gastric mucosa to transport lipids, leading to lipid accumulation within the cells. 8 The presence of gastric xanthoma in itself is probably of little clinical significance. It is highly unlikely that xanthomas are responsible for any clinical symptoms, but are rather a reflection of either past or concurrent gastric pathology, especially in association with bile reflux.
Intussusception presenting with lower gastrointestinal hemorrhage in a hemophiliac
hemophiliac secondary to a cecocolic intussusception. At colonoscopy, the intussusception masqueraded as a polyp in the distal transverse colon. Our purpose is to report an extremely rare cause of lower gastrointestinal hemorrhage and to alert endoscopists to a potentially dangerous clinical situation: inadvertent snare cautery of an intussusception masquerading as a bleeding polyp.
M. P. Pauly, MD E. Watson-Williams, MD W. L. Trudeau, MD
Gastrointestinal hemorrhage is a significant complication of hemophilia in adults. The incidence ranges from 10% to 25%,1-3 with most of the bleeding lesions located in the upper gastrointestinal tract. Colonic sites are implicated in less than 20% of cases, and bleeding etiologies within this group vary widely.2 Intramural hematoma has been reported as a source of gastrointestinal hemorrhage, but rarely does it serve as a lead point of intussusception. This is a case report of hematochezia in an adult From the Department of Internal Medicine, Division of Gastroenterology, University of California, Davis, Davis, California. Reprint requests: W. L. Trudeau, MD, Division of Gastroenterology, 4301 X Street, Sacramento, California 95817. VOLUME 33, NO.2, 1987
REFERENCES 1. Feyrter F. Herdformige Lipoidablagerung in der Schleimhaut des Magens (Lipoidinseln der Magenschleimhaut-Lubarsch) Lipoidzellenkzotchen in der Schleimhaut des Darmes. Virchows Arch Pathol Anat Physiol 1929;273:736-41. 2. Kimura K, Hiramoto T, Buncher CR. Gastric xanthelasma, Arch PathoI1969;87:110-7. 3. Kameya S, Nakamura S, Mizutani K, et al. Xanthoma in the stomach. Gastrointest Endosc 1963;5:37-41. 4, Zimmermann W, Andratschke C, Leidl E, Pavel A, Breitkopf P. Hyperlipamie und Xanthomatose des Magen. Aktuel Gastrologie 1977;6:551-3. 5. Aste H, Cheli R, Nicolo G, Santi L. Gastric xanthelasma. Acta Endosc 1978;8:1-4. 6. Terruzzi V, Minoli G, Butti GC, Rossini A. Gastric lipid islands in the gastric stump and in non-operated stomach. Endoscopy 1980;12:58-62. 7. Mast A, Elewaut A, Mortier G, et al. Gastric xanthoma. Am J GastroenteroI1976;65:311-7. 8. Domellof L, Eriksson S, Helander HF, Janunger KG. Lipid islands in the gastric mucosa after resection for benign ulcer disease. Gastroenterology 1977;72:14-8.
CASE REPORT
A 25-year-old, factor VIII-deficient hemophiliac with inhibitors was admitted to the University of California, Davis Medical Center at Sacramento with hematochezia. Eighteen hours prior to admission he noted the abrupt onset of severe, intermittent, right-sided, crampy abdominal pain, initially associated with an episode of nausea and vomiting. Subsequently, he developed hematochezia with four bloody stools over the next 24 hours. The patient denied a recent history of trauma, constipation, rectal instrumentation, diarrhea, antibiotics, aspirin, or excessive alcohol. On examination, he was alert and oriented. His blood 115
pressure was 140/70 mm Hg, and he had postural tachycardia. The only other abnormalities noted were decreased bowel sounds and diffuse abdominal tenderness (more on the right). There were no palpable masses or organomegaly. There was a large amount of dark red blood in the rectal vault. Laboratory data included a white blood cell count of 10,000/mm3 , a hematocrit of 20%, and a platelet count of 333,000/mm3 • A urinalysis was normal. The bilirubin was 1.7 mg/dl, while other liver function tests were normal. Throughout his hospital course the abdominal findings varied. At one time his abdomen was distended and tympanic bowel sounds and rushes were heard; another time there was a fullness on the right side. Most of the time he was resting comfortably in bed while receiving intravenous meperidine every few hours. During the first 36 hours he was transfused 6 units of packed red blood cells and treated with Autoplex S (antiinhibitor coagulant complex), but the bloody stools continued. A plain film ofthe abdomen was normal. A technetium99 labeled red blood cell scan showed a discrete bleeding site in the left upper quadrant which was felt to be in the distal transverse colon (Fig. 1). On the second hospital day he was switched to porcine factor VIII (Hyate C) and on the third hospital day the bleeding stopped. At that time his hematocrit was 32%. On the fourth hospital day, colonoscopy was performed. At the mid-transverse colon a 2- to 3-cm discrete polypoid mass was encountered. The tip of the lesion was ulcerated and necrotic with an adherent clot, and it appeared to have a thick stalk (Fig. 2). The mucosa up to and surrounding the lesion appeared normal. Removal of the lesion by snare cautery was considered. With prolonged observation, the appearance of the lesion changed; the surrounding mucosa seemed to swell and decompress, and there was a marked amount of to and fro movement in an accordion-like fashion. At this point it was decided to proceed to surgical exploration. At laparotomy it was apparent that the lesion that mimicked a polyp was actually a submucosal hemorrhage at the tip of the cecum that served as the lead point for intussusception into the transverse colon. The tip of the cecum was polypoid with areas of hemorrhage and ulceration. The patient underwent right hemicolectomy and his subsequent course was uneventful. The surgical specimen consisted of a dusky ascending colon and a cecal mass completely invaginated into the most distal ascending colon and proximal transverse colon. A localized hematoma in the tip of the cecum was isolated and identified as the lead point of the intussusception. Microscopic examination of the mass confirmed that it was an intramural hematoma with organized clot, mucosal necrosis, and ulceration. DISCUSSION
Hemophilia A (factor VIII deficiency) is a disorder commonly associated with hemorrhage and trauma is the major precipitating factor. 4 However, spontaneous bleeding may occur involving the oral cavity, joints, muscles, urinary tract, and less frequently the gastrointestinal tract. 5 ,6 Gastrointestinal hemorrhage is 116
A
15 min.
8
40 min.
c
78 min. Lateral Figure 1. Technetium-99 labeled red bloodcell scan A, B, and
C. Note the pooling in the splenic flexure. uncommon and Mittal et a1. 2 recently reported a 10% incidence in 243 hemophiliacs followed over 10 years. Earlier reports of gastrointestinal bleeding in hemophiliacs range from 14.5% 7 to 25%.! Peptic ulcer disease is the most commonly diagnosed cause of gastrointestinal hemorrhage in hemophiliacs and accounted for over 22% of the bleeding esisodes in one series2 and 53% in another.! Other upper tract sources of hemorrhage in hemophiliacs parallel that of the general population and include gastritis, esophagitis, varices, and Mallory-Weiss tears. 1,2 Hemophiliacs presenting with gastrointestinal GASTROINTESTINAL ENDOSCOPY
Figure 2. Colonoscopic appearance of intussusception in transverse colon. bleeding are far less likely to have a lesion in the lower gastrointestinal tract. Less than 15% of the episodes of bleeding in Mittal's group of patients were due to colonic lesions. 2 Isolated cases of diverticulosis, proctitis, hemorrhhoids, and fissures were reported as well as two cases of intramural hematoma involving the small bowel and the sigmoid colon. Submucosal hemorrhages usually resolve upon correction of the clotting deficiency with factor VIII therapy.8 Rarely, lesions can present with bleeding into the intestinal lumen, and an occasional case has been reported with bleeding into the peritoneal cavity.5 Mechanical obstruction of the bowel has been reported with submucosal hemorrhage.5.8 Intussusception appears to be a rare complication of intramural hematoma. 8.9- 11 Review of the literature over the past 25 years revealed only six other cases of
intussusception in hemophiliacs with intramural hematoma, and these were all in the pediatric population (Table 1). The younger patients were more acute and classic in their presentation. Our patient, the oldest reported, typified the presentation so often encountered in adults-that of variable symptomatology with a subacute or indolent course. 12-I5 The classic triad of abdominal pain, vomiting, and rectal bleeding occurs in less than 28% of cases reported in detaiU 6 Intussusception in adults is rare, accounting for less than 5% of all cases of intussusception. Most large centers reporting their experiences with intussusception have less than two cases per year. 12. 13. 15-17 Colonic intussusceptions are far less common than enteric 15 with 55% to 90% due to a pathologic source such as lipomas, leiomyomas, and pOlyps,12.13,15-17 Since colonic intussusception in an adult more commonly has an organic cause, it is likely that the cecal submucosal hematoma in our patient acted as the lead point for the intussusception. I3 ,17 Because of the variable course of the disease and the paucity of cases seen by any one individual, the diagnosis of intussusception is often made later in the work-up or at laparotomy. A careful history and a high index of suspicion cannot be underestimated. Physical examination is very important and often suggestive of intussusception. Dance's sign (right-sided emptiness on abdominal examination), when found, correlates well with an ileocolic or cecocolic intussusception.IS Plain x-rays frequently are nonspecific but may show an ileus. 19 Intussusception has a characteristic appearance on barium enema,20 and descriptions of intussusception have been reported on CT scan 21 and ultrasonography.22.2:l Fiberoptic colonoscopy is rapidly establishing itself as a first line diagnostic procedure for the evaluation
Table 1. Case reports of intussusception in hemophiliacs Author, year reported"
Patient's age (yr)
Type of intussusception
Passaro et al., 1960"
12.0
Ileocolic
Quick, 1966"
15.0
Colocolic
Collins and Miller, 1968'0
2.5
Ileocolic
Donaldson and Clark, 1968"
9.0
Ileocolic
13.5
Ileocolic
Fripp and Karabus, 1977"
LeBlanc, 1982' Present case, 1985
8.0 25.0
Jejunojejunal Cecocolic
Signs and symptoms Crampy abdominal pain, rectal mass Abdominal pain, hematochezia Nausea, vomiting, abdominal pain, rightsided mass Vomiting, abdominal pain, no melena, 6+ stool Vomiting, abdominal pain, RUQ mass, negative stool Abdominal pain, epi. mass, no bleeding Abdominal pain
Plain x-rays Distended SB with fluid levels
Diagnosis Laparotomy X-ray studies
SBO
Laparotomy
Ileus
Laparotomy
Decreased air in colon
Reduced by BE
Distended SB and coIon Nonspecific gas pattern
Laparotomy Laparotomy
" Superior numbers are reference numbers. VOLUME 33, NO.2, 1987
117
of lower gastrointestinal bleeding. 24 •25 Colonoscopy has been more sensitive than a barium enema in the diagnosis of colonic lesions responsible for bleedini 4- 27 and has therapeutic advantages. In our patient the evaluation had to be accomplished as quickly as possible because of the anticipated anamnestic response of factor VIII antibodies occurring between 5 and 10 days after initiating treatment. At colonoscopy, the intussusception in our patient mimicked a polyp. However, the nausea, vomiting, abdominal pain, and tenderness were not consistent with the usual presentation of an uncomplicated bleeding polyp. The history and the physical examination were keys to the correct diagnosis of intussusception. Our case was actually a typical presentation of an extremely rare disease and one not usually encountered or diagnosed by colonoscopy. Intussusception in hemophiliacs can occur and is classically secondary to an intramural hematoma. Such a case is uncommon and has not previously been reported in an adult. The disease occurs so infrequently in adults that the diagnosis is often elusive. In this case the intussusception was first encountered at colonoscopy for lower gastrointestinal bleeding and masqueraded as a polyp. Subtle differences on colonoscopy and the clinical presentation suggested a more complex process. As colonoscopy increases in frequency as the primary diagnostic procedure for lower gastrointestinal bleeding, the examining physician must be aware of the polyp that is more than a polyp.
REFERENCES 1. Forbes CD, Barr RD, Prentice CRM, Douglas AS. Gastrointestinal bleeding in haemophilia. Q J Med 1973;167:503-11. 2. Mittal R, Spero JA, Lewis JH, et al. Patterns of gastrointestinal hemorrhage in hemophilia. Gastroenterology 1985;88:515-22. 3. Wilkinson JF, Nour-Eldin F, Esraels MCG, et al. Haemophilia syndromes: a survey of 267 patients. Lancet 1961;2:947-50. 4. LeBlanc KE. Jejuno-jejunal intussusception in a hemophiliac: a case report. Ann Emerg Med 1982;11:149-51. 5. Fripp RR, CD Karabus. Intussusception in haemophilia. A case report. SA Med J 1977;52:617-8. 6. Quick AJ. Emergencies in hemophilia. Am J Med Sci 1966;40916.
118
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GASTROINTESTINAL ENDOSCOPY