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Unusual cases of intussusception
Printed in the USA * Copyright 0 1991 Pergamon Press plc
The Journal of Emergency Medicine, Vol. 9, pp. 347-355, 1991
UNUSUAL CASES OF INTUSSUSCEPTION Charles V. Pollack, Jr, *Division of Emergency Reprint address:
Medicine
MA, MD,*
and tDivision
of Pediatric Emergency Medicine, University of Mississippi Medical Center, Jackson, Mississippi Charles V. Pollack, Jr, MD, Emergency Medicine Division, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216-4505
0 Abstract - Intussusception occurs most commonly in the first five years of life and is classically associated with intense intermittent abdominal pain, vomiting, bloody mucoid diarrhea, and a palpable abdominal mass. These cardinal findings are frequently not present, however, particularly outside the usual age range. The emergency physician must therefore be vigilant in considering intussnsception as a potential cause for intestinal obstruction in all patients, if lschemic complications are to be avoided. We present three cases of “unusual” intussusception, and provide a review of this entity and a guide to its consideration and work-up in the emergency department.
broad range of clinical and pathologic presentations of intussusception. The emergency physician must consider this diagnosis even in patients who do not present with typical findings. We present three “atypical” cases and a review of other unusual presentations of this entity.
CASE REPORTS Case 1
0 Keywords - intussusceptlon, pediatric; intussusception, adult; intestinal obstruction; hematochezia; abdominal pain
A 12-year-old girl presented to the emergency department (ED) with a 3-day history of abdominal pain, vomiting, diarrhea, and low-grade fever. The pain was described as intermittent and crampy but not localized. There was no history of hematemesis, hematochezia, or melena. Past medical and surgical history was negative. Vital signs in the ED were as follows: temperature, 37.8 “C; pulse, 120/min; respirations, 24/min; and blood pressure, 112/62 mmHg. The child was alert, was in mild distress, and appeared to be mildly dehydrated. Head and neck examinations were normal, as were the pulmonary and cardiovascular examinations. The abdomen was slightly distended and diffusely tender, with voluntary guarding and decreased bowel sounds. There was no palpable mass or organomegaly. Rectal examination revealed a small amount of soft hematest-negative stool in the vault; there was no fullness, mass, or tenderness. Neurologic examination was normal.
INTRODUCTION
Intussusception occurs from the telescoping of a segment of bowel into the lumen of an adjacent distal segment, with resulting obstruction and potential ischemic injury to the proximal bowel. Most cases occur in the first five years of life, with a peak incidence between 5 and 9 months (l-3). The most common type of intussusception is ileocolic (4,5), sometimes resulting from a pathologic “lead point” near the ileocecal valve. A triad of symptoms has traditionally been associated with intussusception: intense intermittent abdominal pain, vomiting, and bloody mucoid stools (3,4). However, these age ranges, anatomic landmarks, and “classic symptoms” describe only a portion of the
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Laboratory studies revealed a white blood cell (WBC) count of 13,270 with 67% polys and 10% bands. The hemoglobin was 15.7 g/dL and the hematocrit was 47%. Serum chemistries and urinalysis were normal; urine pregnancy screen was negative. Abdominal radiographs revealed moderate distention of a number of small bowel loops, suggestive of ileus or early small bowel obstruction. Intravenous hydration was begun and the patient was admitted to the pediatric surgical service. Over the next several hours, she developed increasing abdominal pain and continued to vomit. Nasogastric suction was initiated, the child was started on broad-spectrum antibiotics, and she was taken to the operating room with a preoperative diagnosis of appendicitis. At surgery she was found to have an ileo-ileocolic intussusception with a Meckel’s diverticulum as a lead point. After unsuccessful attempts to reduce the intussusception, the patient underwent resection of the terminal ileum and right hemicolon with primary reanastamosis. After surgery she did well and was discharged home on the 15th postoperative day.
Case 2 An 8-month-old male presented to the ED with a lo-day history of fever and upper respiratory infection (URI) and a l-day history of vomiting and lethargy. He had received a partial course of AugmentinR for his URI symptoms, but had taken no antibiotics for 4 days. There was no history of diarrhea, melena, or hematochezia. Past medical history was negative. Growth and development prior to this illness had been normal. Vital signs were as follows: temperature, 39.1 “C rectally; pulse, 16O/min; respirations, 64/min; and blood pressure, 90 mmHg systolic. Physical examination revealed a lethargic infant with a tense anterior fontanelle. His skin was doughy and the mucous membranes were mildly dry. Head and neck examinations were otherwise normal except for scant nasal discharge. The lungs were clear to auscultation, and the cardiovascular examination was normal. The abdomen was scaphoid with the suggestion of a mass in the right midabdomen. Bowel sounds were normal. Stool was hematest-negative. Neurologic evaluation was nonfocal peripherally; the child was markedly lethargic. Complete blood count revealed 10,000 WBCs with 8% polys, 37% band forms, 41% lymphocytes, and 12% monocytes. Hemoglobin/hematocrit were 7.41 33.7%. Serum chemistries were normal with the exception of a serum bicarbonate of 17 mEq/L. A lumbar
Charles V. Pollack, Jr, and Emily S. Pender
Figure 1. Spot radlograph from barium enema performed on patient in Case 2. The distal small bowel, appendlx, and lleocollc Intussusceptlon (arrow) are clearly demonstrated.
puncture was performed and revealed a cerebrospinal fluid (CSF) cell count of 225 WBC/mm3 with 78% polys and 22% lymphocytes. CSF glucose was 6 mg/dL and protein was 180 mg/dL. Gram’s stain of the CSF revealed abundant pleiomorphic gram-negative rods. The child was admitted to the hospital with a diagnosis of Hemophilus injluenzae meningitis and was started on IV ampicillin and chloramphenicol. In view of the abdominal findings on physical examination, abdominal radiographs were obtained. These revealed a mass effect in the right side of the abdomen, with dilated small bowel loops and a paucity of gas on the right side deemed suggestive of intussusception. After surgical consultation, a barium enema was obtained. Hydrostatic reduction of a visualized ileocolic intussusception (Figure 1) was successful. The child subsequently experienced a complicated course with development of difficult-to-control seizures secondary to his meningitis, but he had no further problems referable to his intussusception. He was discharged home on the 11th hospital day.
Case 3
1
A 43-year-old male presented to the ED with a l-month history of crampy periumbilical pain. He denied nausea, vomiting, melena, or hematochezia. On systems review, he gave a several-week history of cough pro-
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Figure 3. Abdominal CT scan of patlent In Case 3, demonstratlng “target sign” of lntussriscepted bowel In the RLQ (arrow). Oral contrast had been admlnlstered.
Figure 2. Chest radlogmph of patient in Case 3, demonstrating blaplcal infiltrates.
ductive of thick white sputum. He also noted a 15pound weight loss over that period, which he attributed to a decreased appetite. He related a 2-week history of night sweats. His past medical history was significant for heavy ethanol abuse, peptic ulcer disease treated medically, and “a spot on the lung” diagnosed 5 years earlier and treated with medication for one year. There was no history of previous abdominal surgery. Vital signs in the ED were as follows: temperature, 37.2 “C; pulse, 88/min; respirations, 20/min; and blood pressure, 140/80 mmHg. Head and neck examinations were unremarkable; the lungs were clear to auscultation, but breath sounds were decreased in the upper fields bilaterally. Cardiovascular examination was normal. The abdomen was soft with mild midepigastric tenderness and a palpable, minimally tender, 4-cm mass in the right midabdomen. Rectal examination revealed no tenderness and trace hematest-positive stool. Extremity examination was unremarkable. The patient’s white blood cell count was 9,700 without left shift. His hematocrit was 38.6% and serum chemistries were normal. Chest x-ray study (Figure 2)
revealed bilateral apical infiltrates suggestive of mycobacterial disease; flat and upright abdominal films showed three air fluid levels in the right lower quadrant (RLQ) without significant bowel loop distention. Examination of the sputum revealed heavy acid-fast bacilli. The patient was admitted to the hospital and started on aggressive antituberculous therapy. Over the subsequent 12 hours he complained of the onset of nausea and accompanying increased abdominal pain. Reexamination revealed the right-sided mass to be larger and significantly more tender. The patient’s stool was now strongly hematest-positive. An urgent computed tomography (CT scan) of the abdomen was obtained, which revealed dilated loops of small bowel and an abnormal RLQ density consistent with ileocolic intussusception (Figure 3). A gastrograffin enema was then performed, which confirmed the presence of intussusception. Attempts at nonoperative reduction were unsuccessful, and the patient was prepared for surgery. At laparotomy, the terminal 8 to 10 inches of ileum was found to be intussuscepted into the ascending colon. There was no obvious lead point recognized, but multiple mesenteric nodules were noted in the area. A right hemicolectomy with distal ileal resection and primary reanastamosis was performed. The patient tolerated the procedure well and his postoperative course was uneventful. Pathologic examination of the operative specimen revealed ileocolic intussusception secondary to massive tuberculous mesenteric lymphadenitis and chronic ulcerative tuberculosis involving the ileum, cecum, appendix, and ascending colon. Tissue culture eventually tuberculosis. There was no evigrew Mycobacterium dence of malignancy.
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Table 1. Characterlatlc Features of Intusausceptlon (baaed on references 1,3-9,11-21,22-28, 5mos-5yrs “typical” Male:Female Abdominal pain
2:l Predominant symptom; 80% - 95% Occurs early; > 60%
Vomiting
Hematochezia Abdominal mass Lethargy Pathologic lead point Predisposition
Neonate <2:1 Unreliable
Predominant symptom; 80% 50% Common 60% - 85% Variable Common Common 15% - 20% > 30% Viremia, Congenital previous intus anomalies > 75%
Success of reduction at BE
c 40%
among Varlour Age Groups W-48, and 57-59 5 yrs. adolescence 4:l Predominant symptom; 80% - 95% Variable
Adults >4:l Frequent, often protracted If small bowel involved
< 50% Variable Variable Uncommon Uncommon Uncommon > 90% 35% - 45% HSP, PJS, CF, Prev abd surg, malignancy, prev intus, previous intus malignancy 50% To surgery even when successful
Abbreviations: intus = intussusception; abd = abdominal; HSP = Henoch-Schonlein purpura; PJS = Peutz-Jehgers syndrome; CF = cystic fibrosis.
DISCUSSION Selected characteristics of intussusception among various age groups are summarized in Table 1.
‘‘Typical” intussusception
Intussusception in children between the ages of 5 months and 5 years of age is a frequent cause of bowel obstruction and is one of the most common surgical emergencies in infancy and early childhood. It is twice as common in males as in females. The classic triad of symptoms, intermittent abdominal pain, vomiting, and hematochezia, occurs infrequently even among children in this age group (4-8). As many as 40% of patients have none or only one of these symptoms (7). Crampy abdominal pain is the most reliable cardinal symptom, occurring in 80% to 95% of patients (7). It often appears suddenly in previously healthy, thriving
children, recurring at 5- to 30-min intervals. The pain is usually accompanied by screaming, crying, and flexion of the legs. Between episodes, the child may appear normal until well into the course of the illness. Vomiting occurs in at least 60% of patients and is more frequent early in the course of the obstruction (7,9). Bloody, mucoid stools, classically described as resembling currant jelly, are most common in children under one year of age (lo), but occur overall in more man half of patients under five (11). An abdominal mass is present in 60% to 85% of cases of “typical” intussusception (9,ll). In the ileocolic form, which is most common, the mass is usually sausage-shaped, is found in the right upper quadrant (RUQ), and may enlarge noticeably during episodes of pain (10). Abdominal tenderness is a highly variable finding, but when present suggests that the intussuscepted bowel is ischemic (4,9). Behavioral changes, usually described as “apathy” or “lethargy,” may be a predominant symptom in
Table 2. Commonly Reported Lead Polnts for Intussusceptlon, 5 months - 5 years “Typical” Meckel’s Appendix Mesenteric nodes Neoplasm
by Age Group
Neonates
5 years - adolescence
Adults
Meckel’s Cecal duplication Polyps Hamartoma
Meckel’s Appendix Neoplasm Mesenteric nodes Mural hematoma lnspissated mucous/stool (CF)
Neoplasm Suture line Appendix Mesenteric nodes Mural hematoma
Abbreviation: CF = cystic fibrosis.
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Unusual Intussusception
children with intussusception (12-14). The duration of unusual behavior may be as long as 3 days prior to diagnosis, and may prompt investigation of other organ systems before the GI tract is considered as an etiology (13,14). Due to this neglect, lethargy and irritability are considered ominous findings that correlate with the degree of bowel viability, fluid deficit and electrolyte imbalance, and blood loss (13,14). Acute viral respiratory illness has been proposed as an etiology for many cases of intussusception in this age group. Viremia is thought to cause hypertrophy of Peyer’s patches along the distal ileum, which then may act as lead points (10). ‘‘Atypical ’’pediatric intussusception. Unusual cases of intussusception in children may be considered in three groups: 1) neonatal cases; 2) intussusception in children older than 5 years; and 3) cases associated with unusual lead points (Table 2) or systemic disease processes. Recurrent, chronic, and postoperative intussusception also occur in children, but are considered separately below. Intussusception during the first month of life accounts for fewer than 1% of all cases (10,15). It is estimated, however, that 3% of all cases of neonatal intestinal obstruction are caused by intussusception (15). The diagnosis is often obscure, and delay in operation results in high mortality rates. Vomiting is usually the predominant symptom of neonatal intussusception; hematochezia is commonly found on rectal examination, and may occur later in the course than in older children (1,15). Lethargy and a history of poor feeding are relatively common findings. A history of pain is often difficult to elicit, and the presence of a palpable abdominal mass is highly variable (1). Abdominal x-ray studies usually show evidence of small bowel obstruction (1,15). As many as one-third of neonatal patients have an identifiable lead point at laparotomy (Table 2), such as cecal duplication, congenital intestinal polyp, Meckel’s diverticulum, or hamartoma (15). Ileocolic intussusception is the most common form in this age group, but nonoperative reduction is less likely to be successful than in older patients, (1,15). Intussusception among children over 5 years of age (as many as 90% of cases have been reported to occur under the age of two years (16)) is often characterized by a longer history of recurrent abdominal pain (17,18), as illustrated by Case 1. This corresponds with a longer period of viability of the intussuscepted bowel than in younger children (16,17). Pain is the predominant symptom, while rectal bleeding is markedly less common
than in infants, and a mass is often not palpable. Vomiting may be cyclic (19) and may lead to significant fluid and electrolyte disturbances in some patients. As many as 40% of these cases are associated with an identifiable lead point (Table 2); for this reason, although hydrostatic reduction may be successful, operative repair is often advocated as first-line therapy. This is based primarily on the relatively high prevalence of malignant lesions, primarily lymphosarcomas (6,16, 20,21), as lead points. Other commonly reported lead points include a Meckel’s diverticulum polyp, HenochSchonlein purpura, mesenteric nodes, and an inverted appendiceal stump (6,18). Most cases of “juvenile” intussusception are again ileocolic, but with increasing age, the proportions of ileoileal, jejunojejunal, and colocolic intussusception increase ( 18). Some systemic illnesses and conditions place pediatric patients at higher risk for intussusception and, therefore, encourage an aggressive search for this entity when abdominal complaints prompt evaluation. The most common of these are Henoch-Schonlein purpura (HSP), Peutz-Jeghers syndrome (PJS), and cystic fibrosis (CF) (22). It has been reported that 5% of patients with HSP eventually develop intussusception, usually associated with a small bowel mural hematoma (23). Small bowel intussusceptions are also relatively common in PJS and may be seen in familial polyposis (21,24). In CF, inspissated mucofecal masses may act as lead points (2). Nephrotic syndrome and ascariasis have also been associated with increased risk of intussusception. Ectopic rests of pancreatic or gastric mucosa may act as unusual lead points in older children and adults (2). We were unable to find another report of concurrent intussusception and serious systemic infection (as in Case 2), and can only speculate as to the mechanism. intussusception. Recurrent intussusception is uncommon, and usually occurs after nonoperative reduction. Prevalence is reported as 1% to 3% after operative repair, and 10% to 15% after hydrostatic reduction (2,25). Most recurrences occur early, often during the same hospitalization as the initial episode (2). Recurrence is not related to patient age or anatomic location, but underlying predisposing medical conditions often result in recurrences of ileocolic intussusception until ileocolectomy is performed (3). Adults with recurrent intussusception should be presumed to have a malignant lesion as a lead point until proven otherwise (3,26). Recurrent intussusception should be differentiated from chronic intussusception, a rare entity that also of-
Recurrent
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ten presents without typical findings. Signs of acute intussusception are often absent, and pathologic lead points are common (27,28). In young children, chronic intussusception may present as failure to thrive (29). Postoperative intussusception. Postoperative intussusception accounts for as many as 10% of postoperative bowel obstructions in the pediatric age group (30,31). It occurs most frequently after retroperitoneal procedures , abdominoperineal procedures, and appendectomies; 90% of cases occur within 2 weeks of surgery. Although bowel wall hematomas and suture lines are sometimes identified as lead points, in most cases no lead point is obvious (26,30). In adults, postoperative intussusception may occur after a much longer delay from the initial procedure. Small bowel intussusceptions are most common. The most common presenting symptom in postoperative intussusception is bilious vomiting or a sudden increase in nasogastric output; hematochezia is infrequent (31). Abdominal trauma as the cause for initial surgery may be associated with a higher prevalence of postoperative intussusception (32). Adult intussusception. Intussusception is the cause of only about 5% of all mechanical intestinal obstructions in adult patients (33). More than 90% of adults with intussusception have an identifiable lead point (26,33, 34). In the majority of cases, tumors are the etiology (Table 2). In large bowel intussusceptions, the tumor is most often malignant, while in small bowel intussusceptions it is usually benign (33). Postoperative intussusception is the second most common etiology in adults (35). The preponderance of ileocolic cases within the pediatric population is not reproduced among adults, probably because of the variety and anatomic dispersion of lead points responsible for the lesion in the latter group. Symptoms are highly variable, depending upon the location of the obstruction; generally, these patients present as relatively typical small or large bowel obstructions, and the intussusception is not diagnosed before laparotomy (33,34). The history of abdominal pain is often a protracted one. Vomiting is more common with small bowel lesions than with colon involvement. Hematochezia is a highly variable finding and cannot be considered reliable for making the diagnosis of intussusception in adults. The presence of an abdominal mass is also variable, and there are no typical CNS changes associated with adult intussusception (33,34). The most common tumors responsible for intussus-
Charles V. Pollack, Jr, and Emily S. Pender
ception in adults are colonic adenocarcinoma, lymphosarcoma, villous adenoma, and small bowel adenoma and hamartoma (33,34,36). There have also been reports of melanoma (that is most commonly associated with a small bowel intussusception) (37,38) lipoma (39), Kaposi’s sarcoma and AIDS regional enteritis (35,40), duodenal carcinoma (41), and carcinoid tumor (42) as lead points for an intussusception. Congenital anomalies such as fibrous bands (43), Meckel’s diverticulum (44), and duplication of the terminal ileum (45) may not be detected until they cause intussusception in an adult patient. Submucosal gastrointestinal (GI) tract hemorrhages as found in patients with hemophilia (46) and idiopathic thrombocytopenic purpura (47) have been reported as lead points. A multitude of causes of mesenteric adenitis (including mycobacterial disease, as in Case 3 above) may also be responsible. Differential and ED evaluation. Since even the cardinal symptoms of intussusception are variable, other diagnoses must also be considered. The complaints and physical findings associated with the early course of intussusception are largely nonspecific and can be ascribed to a number of disease processes, including gastroenteritis, dysentery, infantile colic, incarcerated hernia, appendicitis, Meckel’s diverticulitis, inflammatory bowel disease, and obstruction or peritonitis. Because of the potential ischemic threat to the involved bowel segment, the emergency physician should entertain the diagnosis of intussusception in all cases of intestinal obstruction. In children, the absence of cardinal symptoms must not preclude early barium study when suspicion of intussusception exists. In infants and children with lethargy unexplained by central nervous system (CNS) work-up in the ED, intussusception must be considered, even in the absence of significant abdominal symptomatology. In all age groups, the presence of a systemic illness associated with an increased propensity for intussusception, for example, HSP, PJS, CF, Kaposi’s sarcoma, hemophilia, or ITP, should prompt a vigorous search for the entity when these patients present with abdominal complaints. Neonates with abdominal distention must undergo a rectal examination to check for hematochezia. Vomiting in very young infants may also be a sign of intussusception, and the diagnosis must be considered even if other suggestive findings are absent (1). Older children with recurrent abdominal pain should have their stools tested for blood. In all patients, a history of previous abdominal surgery, particularly as a result of
Unusual lntussusception
trauma, is a key factor in the consideration of intussus ception as an etiology for bowel obstruction. Likewise, patients with abdominal complaints and a previous history of intussusception reduced nonoperatively, or of intraperitoneal neoplasm, warrant specific work-up in the ED. and treatment. Once the diagnosis is suspected, urgent surgical consultation should be obtained. A nasogastric tube should be placed for proximal decompression, and IV hydration should be promptly instituted. Broad-spectrum antibiotics are indicated if necrotic bowel is suspected (3). Radiographic studies are generally employed to confirm the diagnosis. Plain radiography of the abdomen is useful to demonstrate the presence of bowel obstruction. While it is rarely diagnosic, occasionally the apex of the intussusception is outlined by gas. There may also be evidence of perforation, which constitutes an absolute contraindication to barium enema (BE) (48). Computed tomography (CT scan) of the abdomen is not particularly helpful in pediatric patients, but may be diagnostic of intussusception in adults, revealing “target lesions” of alternating high and low density (Figure 3) (49-51). Ultrasonographic findings can be highly suggestive of intussusception, but are not diagnostic. This modality is most useful in patients with peritonitis or an atypical or confusing history in whom contrast studies might be contraindicated (52-54). When ultrasonography is used as a primary screening procedure, equivocal results should be confirmed by BE. Endoscopy is occasionally useful in establishing the diagnosis (19,55,56). A barium enema is the diagnostic and first-line therapeutic procedure of choice in intussusception. Contraindications to barium study are perforation, frank peritonitis, prolonged or severe bowel obstruction requiring immediate surgery, and hypovolemic shock (4,5,57,58). The intussusception appears on a contrast study as a ‘‘coiled spring” of bowel with surrounding air-fluid levels. Up to 75% of intussusceptions in the typical age group can be reduced by BE, but neonatal and adult intussusceptions are generally more resistant to nonoperative therapy (4,57). Many authors feel there is an inverse relationship between the duration of the intussusception and the rate of success of barium reduction (57). It is also less useful when necrotic bowel is present, when there is a significant mechanical lead point, and when the intussusception is proximal to the Diagnosis
353
cecum (3-5,59). The technique is well described in the literature (4,5). Ideally, attempted reduction should be a joint venture between radiologist and surgeon. A maximum of three attempts should be made (3). It is considered worthwhile by most authorities to perform this study to confirm the diagnosis, even if operative repair is already planned due to suspicion of the presence of a significant anatomic lead point (3-5). If laparotomy is required, manual reduction by milking out the intussuscepted bowel is performed in much the same fashion as in a hydrostatic reduction. If reduction is not possible, if bowel infarction has occurred, or if there is suspicion of a pathologic lead point, resection of the involved proximal and distal segments is performed (3,33,34). In colocolic intussusception, especially in adults, the risk of malignancy is high enough to warrant serious consideration of a cancer resection in all cases. If the left colon is involved, exteriorization and colostomy are usually performed, with reanastamosis in a subsequent procedure. Right colon involvement may not preclude primary reanastamosis (3). When children in the typical age range have intussusception that reduces spontaneously under general anesthesia, an appendectomy should be performed (35). Older children, even without an obvious lead point, should undergo resection because of the likelihood of a grossly inapparent pathologic process (such as an intraluminal neoplasm) and of recurrence (3,22). Adults should uniformly undergo resection and careful pathologic examination of the operative specimen (33,34).
SUMMARY Intussusception should be considered as a possible diagnosis in virtually all patients who present with signs and symptoms of bowel obstruction. The cardinal findings of intermittent crampy abdominal pain, vomiting, and currant jelly stools are helpful when present, but they are not constant even in the typical age group. Specific warning signs such as lethargy and presence of an abdominal mass in young children; associated diseases such as HSP, PJS, CF, hemophilia, ITP, or even AIDS; or a history of malignancy or previous (especially recent) abdominal surgery, must prompt early investigation to avoid ischemic complications. A CT scan and ultrasound may be helpful in adults, but in all patients , contrast enemas are considered diagnostic.
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infants and children. Arch Dis Child. 1955;30:180-3. 28. Momoh JT, Lawrie JH. Tropical intussusception-is it a different disease entity? Ann Trop Pediatr. 1981;1:2374. 29. Page AC, Price JF, Salishury JR, et al. Chronic intussusception. Arch Dis Child. 1990;65:134-5. 30. Ein SH. Recurrent intussusception in children. J Pediatr Surg. 1975;10:751-5. 31. Mollitt DL, Ballantine TVN, Grosfeld JL. Postoperative intussusception in infancy and childhood: analysis of 119 cases. Surgery. 1979;86:402-8. 32. Duncan AO, Phillips TF, Sclafani SJ, et al. Intussusception following abdominal trauma. J Trauma. 1987;27:1193-9. 33. Coleman MJ, Hugh TB, May RE, Jensen MJ. Intussusception in the adult. Aust NZ J Surg. 1981;51:179-80. 34. Murdoch RWG, Wallace JR. Adult intussusception in Glasgow 1968-74. Br J Surg. 1977$X679-80. 35. Balthazar EJ, Reich CB, Pachter HL. The significance of small bowel intussusception in acquired immune deficiency syndrome. Am J Gastroenterol. 1986;81:1073-5. 36. Kalmar JA, Merritt CRB. Villous adenoma of the duodenum with intussusception. South Med J. 1980;73:651-3. 37. Gupta S, Rastogi BL. Metastatic anal melanoma presenting as double intussusception of the small bowel. Am J Proctol. 1977;28: 49-57. 38. Kruse J, Heath R. Melanoma metastic to the gastrointestinal tract. Am Fam Physician. 1990;41:165-8. 39. Zamboni WA, Fleisher H, Zander JD, Folse JR. Spontaneous expulsion of lipoma per rectum occurring with colonic intussusception. Surgery. 1987;101:104-7. 40. Hofstetter SR, Stollman N. Adult intussusception in association with the acquired immune deficiency syndrome and intestinal Kaposi’s sarcoma. Am J Gastroenterol. 1988;83:1304-5. 41. Sellke FW, Williams GB. Distal duodenal carcinoma and intussusception. South Med J. 1987;80:644-6. 42. Skaane P, Eide TJ. Malignant appendiceal carcinoid with intussusception of the base manifesting as a cecal tumor. Dis Colon Rectum. 1977;20:511-14. 43. Robertson JFR, Howatson AG. Simultaneous intussusception and volvulus in an adult due to a congenital fibrous band. Scott Med J. 1986;31:245-6. 44. Wong T-Y, Enriquez RE, Modlin IM, et al. Recurrent hemorrhage from an invaginated Meckel’s diverticulum in a 78-yearold man. Am J Gastroenterol. 1990;85:195-8. 45. Frieden JH, Kaplan L. Duplication of the terminal ileum with intussusception in an adult. Am J Gastroenterol. 1980;73:523-7. 46. Pauly MP, Watson-Williams E, Trudeau WL. Intussusception presenting with lower gastrointestinal hemorrhage in a hemophiliac. Gastrointest Endoscopy. 1987;33:115-18. 47. Gupta HL, Malik TK. Intussusception in idiopathic thrombocytopenic purpura. J Indian Med Assoc. 1976;67:145-7. 48. Nyhan WL. Intussusception. West J Med. 1986;144:722-7. 49. Lorigan JG, DuBrow RA. The computed tomographic appearances and clinical significance of intussusception in adults with malignant neoplasms. Br J Radiol. 1990;63:257-62. 50. Merine D, Fishman EK, Jones B, Siegehnann SS. Enteroenteric intussusception: CT findings in nine patients. AJR. 1987;148: 1129-32. 51. Iko BO, Teal JS, Siram SM, Chinwuba CE, Roux VJ, Scott VF. Computed tomography of adult colon intussusception: clinical and experimental studies. AJR. 1984;143:769-72. 52. Alessi V, Salerno G. The “hay-fork” sign in the ultraaonographic diagnosis of intussusception. Gastroenteml Radiol. 1985;lO: 177-9. 53. Pracros JP, Tran-Minh VA, Wright C. Ultrasound in diagnosis of intussusception. Lancet. 1985;2:7334. 54. Swischuk LE, Hayden CK, Boulden T. Intussusception: indica-
Unusual lntussusception
tions for sonography and an explanation of the doughnut and pseudokidney signs. Pediatr Radiol. 1985;15:388-91. 55. Goldin E, Lisbon E. Intussusception in intestinal lymphoma: the role of colonoscopy. Postgrad Med J. 1986;62:1139-40. 56. Hurwitz LM, Gertier SL. Colonoscopic diagnosis of ileocolic intussusception. Gastrointest Endoscopy. 1986;32:217-18. 57. Spamon AL, Little KET, Morris LL. Intussusception in child-
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hood: a review of 139 cases. Aust NZ J Surg. 1984;54:353-6. 58. Wilson-Storey D, Ma&inlay GA, Prescott S, Hendry GMA. Intussusception: a surgical condition. J R Co11 Surg Edinb. 1988;33:270-3. 59. Mackay AJ, MacKellar A, Sprague P. Intussusception in children: a review of 91 cases. Aust NZ J Surg. 1987;57:15-17.
The Journal of Emergency Medicine, Vol. 9, pp. 347-355, 1991
UNUSUAL CASES OF INTUSSUSCEPTION Charles V. Pollack, Jr, *Division of Emergency Reprint address:
Medicine
MA, MD,*
and tDivision
of Pediatric Emergency Medicine, University of Mississippi Medical Center, Jackson, Mississippi Charles V. Pollack, Jr, MD, Emergency Medicine Division, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216-4505
0 Abstract - Intussusception occurs most commonly in the first five years of life and is classically associated with intense intermittent abdominal pain, vomiting, bloody mucoid diarrhea, and a palpable abdominal mass. These cardinal findings are frequently not present, however, particularly outside the usual age range. The emergency physician must therefore be vigilant in considering intussnsception as a potential cause for intestinal obstruction in all patients, if lschemic complications are to be avoided. We present three cases of “unusual” intussusception, and provide a review of this entity and a guide to its consideration and work-up in the emergency department.
broad range of clinical and pathologic presentations of intussusception. The emergency physician must consider this diagnosis even in patients who do not present with typical findings. We present three “atypical” cases and a review of other unusual presentations of this entity.
CASE REPORTS Case 1
0 Keywords - intussusceptlon, pediatric; intussusception, adult; intestinal obstruction; hematochezia; abdominal pain
A 12-year-old girl presented to the emergency department (ED) with a 3-day history of abdominal pain, vomiting, diarrhea, and low-grade fever. The pain was described as intermittent and crampy but not localized. There was no history of hematemesis, hematochezia, or melena. Past medical and surgical history was negative. Vital signs in the ED were as follows: temperature, 37.8 “C; pulse, 120/min; respirations, 24/min; and blood pressure, 112/62 mmHg. The child was alert, was in mild distress, and appeared to be mildly dehydrated. Head and neck examinations were normal, as were the pulmonary and cardiovascular examinations. The abdomen was slightly distended and diffusely tender, with voluntary guarding and decreased bowel sounds. There was no palpable mass or organomegaly. Rectal examination revealed a small amount of soft hematest-negative stool in the vault; there was no fullness, mass, or tenderness. Neurologic examination was normal.
INTRODUCTION
Intussusception occurs from the telescoping of a segment of bowel into the lumen of an adjacent distal segment, with resulting obstruction and potential ischemic injury to the proximal bowel. Most cases occur in the first five years of life, with a peak incidence between 5 and 9 months (l-3). The most common type of intussusception is ileocolic (4,5), sometimes resulting from a pathologic “lead point” near the ileocecal valve. A triad of symptoms has traditionally been associated with intussusception: intense intermittent abdominal pain, vomiting, and bloody mucoid stools (3,4). However, these age ranges, anatomic landmarks, and “classic symptoms” describe only a portion of the
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Laboratory studies revealed a white blood cell (WBC) count of 13,270 with 67% polys and 10% bands. The hemoglobin was 15.7 g/dL and the hematocrit was 47%. Serum chemistries and urinalysis were normal; urine pregnancy screen was negative. Abdominal radiographs revealed moderate distention of a number of small bowel loops, suggestive of ileus or early small bowel obstruction. Intravenous hydration was begun and the patient was admitted to the pediatric surgical service. Over the next several hours, she developed increasing abdominal pain and continued to vomit. Nasogastric suction was initiated, the child was started on broad-spectrum antibiotics, and she was taken to the operating room with a preoperative diagnosis of appendicitis. At surgery she was found to have an ileo-ileocolic intussusception with a Meckel’s diverticulum as a lead point. After unsuccessful attempts to reduce the intussusception, the patient underwent resection of the terminal ileum and right hemicolon with primary reanastamosis. After surgery she did well and was discharged home on the 15th postoperative day.
Case 2 An 8-month-old male presented to the ED with a lo-day history of fever and upper respiratory infection (URI) and a l-day history of vomiting and lethargy. He had received a partial course of AugmentinR for his URI symptoms, but had taken no antibiotics for 4 days. There was no history of diarrhea, melena, or hematochezia. Past medical history was negative. Growth and development prior to this illness had been normal. Vital signs were as follows: temperature, 39.1 “C rectally; pulse, 16O/min; respirations, 64/min; and blood pressure, 90 mmHg systolic. Physical examination revealed a lethargic infant with a tense anterior fontanelle. His skin was doughy and the mucous membranes were mildly dry. Head and neck examinations were otherwise normal except for scant nasal discharge. The lungs were clear to auscultation, and the cardiovascular examination was normal. The abdomen was scaphoid with the suggestion of a mass in the right midabdomen. Bowel sounds were normal. Stool was hematest-negative. Neurologic evaluation was nonfocal peripherally; the child was markedly lethargic. Complete blood count revealed 10,000 WBCs with 8% polys, 37% band forms, 41% lymphocytes, and 12% monocytes. Hemoglobin/hematocrit were 7.41 33.7%. Serum chemistries were normal with the exception of a serum bicarbonate of 17 mEq/L. A lumbar
Charles V. Pollack, Jr, and Emily S. Pender
Figure 1. Spot radlograph from barium enema performed on patient in Case 2. The distal small bowel, appendlx, and lleocollc Intussusceptlon (arrow) are clearly demonstrated.
puncture was performed and revealed a cerebrospinal fluid (CSF) cell count of 225 WBC/mm3 with 78% polys and 22% lymphocytes. CSF glucose was 6 mg/dL and protein was 180 mg/dL. Gram’s stain of the CSF revealed abundant pleiomorphic gram-negative rods. The child was admitted to the hospital with a diagnosis of Hemophilus injluenzae meningitis and was started on IV ampicillin and chloramphenicol. In view of the abdominal findings on physical examination, abdominal radiographs were obtained. These revealed a mass effect in the right side of the abdomen, with dilated small bowel loops and a paucity of gas on the right side deemed suggestive of intussusception. After surgical consultation, a barium enema was obtained. Hydrostatic reduction of a visualized ileocolic intussusception (Figure 1) was successful. The child subsequently experienced a complicated course with development of difficult-to-control seizures secondary to his meningitis, but he had no further problems referable to his intussusception. He was discharged home on the 11th hospital day.
Case 3
1
A 43-year-old male presented to the ED with a l-month history of crampy periumbilical pain. He denied nausea, vomiting, melena, or hematochezia. On systems review, he gave a several-week history of cough pro-
Unusual lntussusception
349
Figure 3. Abdominal CT scan of patlent In Case 3, demonstratlng “target sign” of lntussriscepted bowel In the RLQ (arrow). Oral contrast had been admlnlstered.
Figure 2. Chest radlogmph of patient in Case 3, demonstrating blaplcal infiltrates.
ductive of thick white sputum. He also noted a 15pound weight loss over that period, which he attributed to a decreased appetite. He related a 2-week history of night sweats. His past medical history was significant for heavy ethanol abuse, peptic ulcer disease treated medically, and “a spot on the lung” diagnosed 5 years earlier and treated with medication for one year. There was no history of previous abdominal surgery. Vital signs in the ED were as follows: temperature, 37.2 “C; pulse, 88/min; respirations, 20/min; and blood pressure, 140/80 mmHg. Head and neck examinations were unremarkable; the lungs were clear to auscultation, but breath sounds were decreased in the upper fields bilaterally. Cardiovascular examination was normal. The abdomen was soft with mild midepigastric tenderness and a palpable, minimally tender, 4-cm mass in the right midabdomen. Rectal examination revealed no tenderness and trace hematest-positive stool. Extremity examination was unremarkable. The patient’s white blood cell count was 9,700 without left shift. His hematocrit was 38.6% and serum chemistries were normal. Chest x-ray study (Figure 2)
revealed bilateral apical infiltrates suggestive of mycobacterial disease; flat and upright abdominal films showed three air fluid levels in the right lower quadrant (RLQ) without significant bowel loop distention. Examination of the sputum revealed heavy acid-fast bacilli. The patient was admitted to the hospital and started on aggressive antituberculous therapy. Over the subsequent 12 hours he complained of the onset of nausea and accompanying increased abdominal pain. Reexamination revealed the right-sided mass to be larger and significantly more tender. The patient’s stool was now strongly hematest-positive. An urgent computed tomography (CT scan) of the abdomen was obtained, which revealed dilated loops of small bowel and an abnormal RLQ density consistent with ileocolic intussusception (Figure 3). A gastrograffin enema was then performed, which confirmed the presence of intussusception. Attempts at nonoperative reduction were unsuccessful, and the patient was prepared for surgery. At laparotomy, the terminal 8 to 10 inches of ileum was found to be intussuscepted into the ascending colon. There was no obvious lead point recognized, but multiple mesenteric nodules were noted in the area. A right hemicolectomy with distal ileal resection and primary reanastamosis was performed. The patient tolerated the procedure well and his postoperative course was uneventful. Pathologic examination of the operative specimen revealed ileocolic intussusception secondary to massive tuberculous mesenteric lymphadenitis and chronic ulcerative tuberculosis involving the ileum, cecum, appendix, and ascending colon. Tissue culture eventually tuberculosis. There was no evigrew Mycobacterium dence of malignancy.
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Charles V. Pollack, Jr, and Emily S. Pender
Table 1. Characterlatlc Features of Intusausceptlon (baaed on references 1,3-9,11-21,22-28, 5mos-5yrs “typical” Male:Female Abdominal pain
2:l Predominant symptom; 80% - 95% Occurs early; > 60%
Vomiting
Hematochezia Abdominal mass Lethargy Pathologic lead point Predisposition
Neonate <2:1 Unreliable
Predominant symptom; 80% 50% Common 60% - 85% Variable Common Common 15% - 20% > 30% Viremia, Congenital previous intus anomalies > 75%
Success of reduction at BE
c 40%
among Varlour Age Groups W-48, and 57-59 5 yrs. adolescence 4:l Predominant symptom; 80% - 95% Variable
Adults >4:l Frequent, often protracted If small bowel involved
< 50% Variable Variable Uncommon Uncommon Uncommon > 90% 35% - 45% HSP, PJS, CF, Prev abd surg, malignancy, prev intus, previous intus malignancy 50% To surgery even when successful
Abbreviations: intus = intussusception; abd = abdominal; HSP = Henoch-Schonlein purpura; PJS = Peutz-Jehgers syndrome; CF = cystic fibrosis.
DISCUSSION Selected characteristics of intussusception among various age groups are summarized in Table 1.
‘‘Typical” intussusception
Intussusception in children between the ages of 5 months and 5 years of age is a frequent cause of bowel obstruction and is one of the most common surgical emergencies in infancy and early childhood. It is twice as common in males as in females. The classic triad of symptoms, intermittent abdominal pain, vomiting, and hematochezia, occurs infrequently even among children in this age group (4-8). As many as 40% of patients have none or only one of these symptoms (7). Crampy abdominal pain is the most reliable cardinal symptom, occurring in 80% to 95% of patients (7). It often appears suddenly in previously healthy, thriving
children, recurring at 5- to 30-min intervals. The pain is usually accompanied by screaming, crying, and flexion of the legs. Between episodes, the child may appear normal until well into the course of the illness. Vomiting occurs in at least 60% of patients and is more frequent early in the course of the obstruction (7,9). Bloody, mucoid stools, classically described as resembling currant jelly, are most common in children under one year of age (lo), but occur overall in more man half of patients under five (11). An abdominal mass is present in 60% to 85% of cases of “typical” intussusception (9,ll). In the ileocolic form, which is most common, the mass is usually sausage-shaped, is found in the right upper quadrant (RUQ), and may enlarge noticeably during episodes of pain (10). Abdominal tenderness is a highly variable finding, but when present suggests that the intussuscepted bowel is ischemic (4,9). Behavioral changes, usually described as “apathy” or “lethargy,” may be a predominant symptom in
Table 2. Commonly Reported Lead Polnts for Intussusceptlon, 5 months - 5 years “Typical” Meckel’s Appendix Mesenteric nodes Neoplasm
by Age Group
Neonates
5 years - adolescence
Adults
Meckel’s Cecal duplication Polyps Hamartoma
Meckel’s Appendix Neoplasm Mesenteric nodes Mural hematoma lnspissated mucous/stool (CF)
Neoplasm Suture line Appendix Mesenteric nodes Mural hematoma
Abbreviation: CF = cystic fibrosis.
351
Unusual Intussusception
children with intussusception (12-14). The duration of unusual behavior may be as long as 3 days prior to diagnosis, and may prompt investigation of other organ systems before the GI tract is considered as an etiology (13,14). Due to this neglect, lethargy and irritability are considered ominous findings that correlate with the degree of bowel viability, fluid deficit and electrolyte imbalance, and blood loss (13,14). Acute viral respiratory illness has been proposed as an etiology for many cases of intussusception in this age group. Viremia is thought to cause hypertrophy of Peyer’s patches along the distal ileum, which then may act as lead points (10). ‘‘Atypical ’’pediatric intussusception. Unusual cases of intussusception in children may be considered in three groups: 1) neonatal cases; 2) intussusception in children older than 5 years; and 3) cases associated with unusual lead points (Table 2) or systemic disease processes. Recurrent, chronic, and postoperative intussusception also occur in children, but are considered separately below. Intussusception during the first month of life accounts for fewer than 1% of all cases (10,15). It is estimated, however, that 3% of all cases of neonatal intestinal obstruction are caused by intussusception (15). The diagnosis is often obscure, and delay in operation results in high mortality rates. Vomiting is usually the predominant symptom of neonatal intussusception; hematochezia is commonly found on rectal examination, and may occur later in the course than in older children (1,15). Lethargy and a history of poor feeding are relatively common findings. A history of pain is often difficult to elicit, and the presence of a palpable abdominal mass is highly variable (1). Abdominal x-ray studies usually show evidence of small bowel obstruction (1,15). As many as one-third of neonatal patients have an identifiable lead point at laparotomy (Table 2), such as cecal duplication, congenital intestinal polyp, Meckel’s diverticulum, or hamartoma (15). Ileocolic intussusception is the most common form in this age group, but nonoperative reduction is less likely to be successful than in older patients, (1,15). Intussusception among children over 5 years of age (as many as 90% of cases have been reported to occur under the age of two years (16)) is often characterized by a longer history of recurrent abdominal pain (17,18), as illustrated by Case 1. This corresponds with a longer period of viability of the intussuscepted bowel than in younger children (16,17). Pain is the predominant symptom, while rectal bleeding is markedly less common
than in infants, and a mass is often not palpable. Vomiting may be cyclic (19) and may lead to significant fluid and electrolyte disturbances in some patients. As many as 40% of these cases are associated with an identifiable lead point (Table 2); for this reason, although hydrostatic reduction may be successful, operative repair is often advocated as first-line therapy. This is based primarily on the relatively high prevalence of malignant lesions, primarily lymphosarcomas (6,16, 20,21), as lead points. Other commonly reported lead points include a Meckel’s diverticulum polyp, HenochSchonlein purpura, mesenteric nodes, and an inverted appendiceal stump (6,18). Most cases of “juvenile” intussusception are again ileocolic, but with increasing age, the proportions of ileoileal, jejunojejunal, and colocolic intussusception increase ( 18). Some systemic illnesses and conditions place pediatric patients at higher risk for intussusception and, therefore, encourage an aggressive search for this entity when abdominal complaints prompt evaluation. The most common of these are Henoch-Schonlein purpura (HSP), Peutz-Jeghers syndrome (PJS), and cystic fibrosis (CF) (22). It has been reported that 5% of patients with HSP eventually develop intussusception, usually associated with a small bowel mural hematoma (23). Small bowel intussusceptions are also relatively common in PJS and may be seen in familial polyposis (21,24). In CF, inspissated mucofecal masses may act as lead points (2). Nephrotic syndrome and ascariasis have also been associated with increased risk of intussusception. Ectopic rests of pancreatic or gastric mucosa may act as unusual lead points in older children and adults (2). We were unable to find another report of concurrent intussusception and serious systemic infection (as in Case 2), and can only speculate as to the mechanism. intussusception. Recurrent intussusception is uncommon, and usually occurs after nonoperative reduction. Prevalence is reported as 1% to 3% after operative repair, and 10% to 15% after hydrostatic reduction (2,25). Most recurrences occur early, often during the same hospitalization as the initial episode (2). Recurrence is not related to patient age or anatomic location, but underlying predisposing medical conditions often result in recurrences of ileocolic intussusception until ileocolectomy is performed (3). Adults with recurrent intussusception should be presumed to have a malignant lesion as a lead point until proven otherwise (3,26). Recurrent intussusception should be differentiated from chronic intussusception, a rare entity that also of-
Recurrent
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ten presents without typical findings. Signs of acute intussusception are often absent, and pathologic lead points are common (27,28). In young children, chronic intussusception may present as failure to thrive (29). Postoperative intussusception. Postoperative intussusception accounts for as many as 10% of postoperative bowel obstructions in the pediatric age group (30,31). It occurs most frequently after retroperitoneal procedures , abdominoperineal procedures, and appendectomies; 90% of cases occur within 2 weeks of surgery. Although bowel wall hematomas and suture lines are sometimes identified as lead points, in most cases no lead point is obvious (26,30). In adults, postoperative intussusception may occur after a much longer delay from the initial procedure. Small bowel intussusceptions are most common. The most common presenting symptom in postoperative intussusception is bilious vomiting or a sudden increase in nasogastric output; hematochezia is infrequent (31). Abdominal trauma as the cause for initial surgery may be associated with a higher prevalence of postoperative intussusception (32). Adult intussusception. Intussusception is the cause of only about 5% of all mechanical intestinal obstructions in adult patients (33). More than 90% of adults with intussusception have an identifiable lead point (26,33, 34). In the majority of cases, tumors are the etiology (Table 2). In large bowel intussusceptions, the tumor is most often malignant, while in small bowel intussusceptions it is usually benign (33). Postoperative intussusception is the second most common etiology in adults (35). The preponderance of ileocolic cases within the pediatric population is not reproduced among adults, probably because of the variety and anatomic dispersion of lead points responsible for the lesion in the latter group. Symptoms are highly variable, depending upon the location of the obstruction; generally, these patients present as relatively typical small or large bowel obstructions, and the intussusception is not diagnosed before laparotomy (33,34). The history of abdominal pain is often a protracted one. Vomiting is more common with small bowel lesions than with colon involvement. Hematochezia is a highly variable finding and cannot be considered reliable for making the diagnosis of intussusception in adults. The presence of an abdominal mass is also variable, and there are no typical CNS changes associated with adult intussusception (33,34). The most common tumors responsible for intussus-
Charles V. Pollack, Jr, and Emily S. Pender
ception in adults are colonic adenocarcinoma, lymphosarcoma, villous adenoma, and small bowel adenoma and hamartoma (33,34,36). There have also been reports of melanoma (that is most commonly associated with a small bowel intussusception) (37,38) lipoma (39), Kaposi’s sarcoma and AIDS regional enteritis (35,40), duodenal carcinoma (41), and carcinoid tumor (42) as lead points for an intussusception. Congenital anomalies such as fibrous bands (43), Meckel’s diverticulum (44), and duplication of the terminal ileum (45) may not be detected until they cause intussusception in an adult patient. Submucosal gastrointestinal (GI) tract hemorrhages as found in patients with hemophilia (46) and idiopathic thrombocytopenic purpura (47) have been reported as lead points. A multitude of causes of mesenteric adenitis (including mycobacterial disease, as in Case 3 above) may also be responsible. Differential and ED evaluation. Since even the cardinal symptoms of intussusception are variable, other diagnoses must also be considered. The complaints and physical findings associated with the early course of intussusception are largely nonspecific and can be ascribed to a number of disease processes, including gastroenteritis, dysentery, infantile colic, incarcerated hernia, appendicitis, Meckel’s diverticulitis, inflammatory bowel disease, and obstruction or peritonitis. Because of the potential ischemic threat to the involved bowel segment, the emergency physician should entertain the diagnosis of intussusception in all cases of intestinal obstruction. In children, the absence of cardinal symptoms must not preclude early barium study when suspicion of intussusception exists. In infants and children with lethargy unexplained by central nervous system (CNS) work-up in the ED, intussusception must be considered, even in the absence of significant abdominal symptomatology. In all age groups, the presence of a systemic illness associated with an increased propensity for intussusception, for example, HSP, PJS, CF, Kaposi’s sarcoma, hemophilia, or ITP, should prompt a vigorous search for the entity when these patients present with abdominal complaints. Neonates with abdominal distention must undergo a rectal examination to check for hematochezia. Vomiting in very young infants may also be a sign of intussusception, and the diagnosis must be considered even if other suggestive findings are absent (1). Older children with recurrent abdominal pain should have their stools tested for blood. In all patients, a history of previous abdominal surgery, particularly as a result of
Unusual lntussusception
trauma, is a key factor in the consideration of intussus ception as an etiology for bowel obstruction. Likewise, patients with abdominal complaints and a previous history of intussusception reduced nonoperatively, or of intraperitoneal neoplasm, warrant specific work-up in the ED. and treatment. Once the diagnosis is suspected, urgent surgical consultation should be obtained. A nasogastric tube should be placed for proximal decompression, and IV hydration should be promptly instituted. Broad-spectrum antibiotics are indicated if necrotic bowel is suspected (3). Radiographic studies are generally employed to confirm the diagnosis. Plain radiography of the abdomen is useful to demonstrate the presence of bowel obstruction. While it is rarely diagnosic, occasionally the apex of the intussusception is outlined by gas. There may also be evidence of perforation, which constitutes an absolute contraindication to barium enema (BE) (48). Computed tomography (CT scan) of the abdomen is not particularly helpful in pediatric patients, but may be diagnostic of intussusception in adults, revealing “target lesions” of alternating high and low density (Figure 3) (49-51). Ultrasonographic findings can be highly suggestive of intussusception, but are not diagnostic. This modality is most useful in patients with peritonitis or an atypical or confusing history in whom contrast studies might be contraindicated (52-54). When ultrasonography is used as a primary screening procedure, equivocal results should be confirmed by BE. Endoscopy is occasionally useful in establishing the diagnosis (19,55,56). A barium enema is the diagnostic and first-line therapeutic procedure of choice in intussusception. Contraindications to barium study are perforation, frank peritonitis, prolonged or severe bowel obstruction requiring immediate surgery, and hypovolemic shock (4,5,57,58). The intussusception appears on a contrast study as a ‘‘coiled spring” of bowel with surrounding air-fluid levels. Up to 75% of intussusceptions in the typical age group can be reduced by BE, but neonatal and adult intussusceptions are generally more resistant to nonoperative therapy (4,57). Many authors feel there is an inverse relationship between the duration of the intussusception and the rate of success of barium reduction (57). It is also less useful when necrotic bowel is present, when there is a significant mechanical lead point, and when the intussusception is proximal to the Diagnosis
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cecum (3-5,59). The technique is well described in the literature (4,5). Ideally, attempted reduction should be a joint venture between radiologist and surgeon. A maximum of three attempts should be made (3). It is considered worthwhile by most authorities to perform this study to confirm the diagnosis, even if operative repair is already planned due to suspicion of the presence of a significant anatomic lead point (3-5). If laparotomy is required, manual reduction by milking out the intussuscepted bowel is performed in much the same fashion as in a hydrostatic reduction. If reduction is not possible, if bowel infarction has occurred, or if there is suspicion of a pathologic lead point, resection of the involved proximal and distal segments is performed (3,33,34). In colocolic intussusception, especially in adults, the risk of malignancy is high enough to warrant serious consideration of a cancer resection in all cases. If the left colon is involved, exteriorization and colostomy are usually performed, with reanastamosis in a subsequent procedure. Right colon involvement may not preclude primary reanastamosis (3). When children in the typical age range have intussusception that reduces spontaneously under general anesthesia, an appendectomy should be performed (35). Older children, even without an obvious lead point, should undergo resection because of the likelihood of a grossly inapparent pathologic process (such as an intraluminal neoplasm) and of recurrence (3,22). Adults should uniformly undergo resection and careful pathologic examination of the operative specimen (33,34).
SUMMARY Intussusception should be considered as a possible diagnosis in virtually all patients who present with signs and symptoms of bowel obstruction. The cardinal findings of intermittent crampy abdominal pain, vomiting, and currant jelly stools are helpful when present, but they are not constant even in the typical age group. Specific warning signs such as lethargy and presence of an abdominal mass in young children; associated diseases such as HSP, PJS, CF, hemophilia, ITP, or even AIDS; or a history of malignancy or previous (especially recent) abdominal surgery, must prompt early investigation to avoid ischemic complications. A CT scan and ultrasound may be helpful in adults, but in all patients , contrast enemas are considered diagnostic.
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Charles V. Pollack, Jr, and Emily S. Pender
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