Appendiceal intussusception caused by villous adenoma

May 25, 2007
Matthew S. Pugliese,MD

Surgical Rounds, September 2006, Volume 0, Issue 0

Matthew S. Pugliese, Resident, Department of General Surgery, Swedish Medical Center, Seattle, WA; Steven F. Counter, Attending Surgeon, The Polyclinic, Department of Surgery, Swedish Medical Center, Seattle, WA

Matthew S. Pugliese,MD

Resident

Department of General

Surgery

Swedish Medical Center

Seattle, WA

Steven F. Counter, MD

Attending Surgeon

The Polyclinic

Department of Surgery

Swedish Medical Center

Seattle, WA

Intussusception rarely presents in adults and, when found, is most commonly the result of a definable pathologic lead point. Unlike its pediatric counterpart, which is generally managed with nonsurgical reduction, operative intervention is the primary treatment for adult intussusception. We report the case of appendiceal intussusception in an 81-year-old woman. Pathologic evaluation identified the lead point to be a villous adenoma. This case is interesting because it demonstrates a relatively uncommon type of adult intussusception, one that is secondary to a rare neoplastic lesion.

Case report

An 81-year-old woman presented to the emergency department with the chief symptom of nonresolving, cramping abdominal pain of 3 days’ duration. The pain was initially periumbilical but had migrated to the patient’s right lower quadrant. She reported having felt similar pains in the past, although not as severe. The patient had no obstructive gastrointestinal symptoms and had never had a screening colonoscopy. Her medical history included arthritis, hypertension, and osteoporosis, for which she took prednisone, hydrochlorothiazide, and alendronate, respectively. Her surgical history was significant for a hysterectomy, which had been performed many years earlier.

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The patient was afebrile with normal vital signs. Physical examination was significant for diffuse abdominal tenderness with localized peritoneal signs in the right lower quadrant. A distinct, mobile mass was palpable in the area where maximum tenderness was elicited. Laboratory studies showed leukocytosis (21,000/L) associated with bandemia. Computed tomography (CT) scans of the abdomen showed a “target sign” in the right lower quadrant (Figure 1). A diagnosis of intussusception was made, and after discussing treatment options with the patient, an exploratory laparotomy was decided upon.

During laparotomy, a diffusely enlarged, erythematous, nonperforated appendix measuring approximately 15 cm in length and 2 cm in width was found (Figure 2). The appendix was partially intussuscepted at its base and associated with a small cecocolic intussusception. The ileocolic valve was intact. The liver was palpated and the remainder of the exploration did not identify any additional disease. A partial cecectomy was performed. Pathology examination revealed diffuse villous adenoma of the appendix (Figure 3). The specimen showed no invasive disease and negative margins were obtained. The patient recovered uneventfully and was discharged to home without complications. She was referred for outpatient colonoscopy.

Discussion

Intussusception has long been described in the medical literature. The first report was by Barbette of Amsterdam in 1674.1 In the mid-1700s, Cornelius Henrik Velse was the first to describe a successful operation on adult intussusception.2 In 1837, John Hunter reported three cases of adult intussusception, one of which involved ileoileal intussusception in a ship’s cabin boy.3 The etiology in this case was parasitic, and Hunter’s proposed initial therapy was nonoperative. He recommended inducing emesis to “invert the peristaltic motion of the containing gut, which will have a tendency to bring the intestines into their natural situation.”

Most patients presenting with adult intussusception will report vague abdominal pain that has been intermittent or chronic. Acute symptomatology would suggest ischemia or impending perforation. Azar and Berger reported abdominal pain as the most common symptom.4 Nausea, bleeding, diarrhea, constipation, a mass, and weight loss were reported less often. Physical examination may reveal an abdominal mass, with previous series indicating that masses can be palpated between 24% and 42% of the time.5,6 Masses that shift and are only palpable during symptomatic periods should raise suspicion of intussusception.

In previous decades, diagnosing adult intussusception preoperatively was difficult. Most patients would proceed to the operating room with a diagnosis of obstruction. The increased use of new technology is making the preoperative diagnosis of adult intussusception more common. Azar and Berger found 40% of their institution’s patients were diagnosed preoperatively.4 In their review, CT scanning was the most accurate imaging modality for identifying adult intussusception.

Unlike pediatric cases, intussusception in adults is rare, accounting for fewer than 5% of total intussusceptions and causing fewer than 1% of intestinal obstructions. Another key difference between adult and pediatric intussusception is the etiology. Adult intussusception is most often caused by a definable pathologic lead point. Peristalsis pushes this lead point downstream, causing the intussusceptum to telescope into the receiving intussuscipiens. As the bowel intussuscepts, it pulls its mesentery with it. This can trap the vascular supply between the intussusceptum and the intussuscipiens, providing a mechanism for ischemia of the bowel wall and perforation. Although intussusception tends to occur more frequently at junctions between freely moving and fixed portions of the bowel, it may occur almost anywhere throughout the gastrointestinal tract.

Begos and colleagues performed a meta-analysis on the location and etiology of adult intussusception.7 They evaluated 1,048 cases and found that 64% occurred in the small intestines. Most of these (63%) were secondary to benign lead points. Idiopathic (23%) and malignant (14%) disease accounted for the remaining small bowel cases. Colonic intussusception only accounted for 36% of cases. In contrast to intussusception of the small bowel, most colonic intussusceptions (58%) were from malignant lead points. Benign (29%) and idiopathic (13%) processes accounted for the other colonic cases.

Because adult intussusception is most often secondary to a pathologic lead point, the involved bowel generally should be resected without reduction, helping to avoid the risk of spillage. This method also optimizes the potential oncologic value of the operation; however, reduction before resection may be considered when benign disease is suspected or in cases where preserving bowel length is important.

Intussusception of the appendix

—Appendiceal intussusception is rare. Collins and associates identified seven such cases among the 71,000 pathologic specimens reviewed.8 In 1941, McSwain reported a case of appendiceal intussusception and provided a review of the literature.9 In his discussion, he identified five types of appendiceal intussusception, which are still referenced today (Figure 4). A sixth type, however, has been added recently to include compound intussusceptions or those associated with significant cecocolic intussusception. Our patient had a type 3 intussusception, which Fink and associates note as the most common type.10 To date, approximately 200 cases of appendiceal intussusception have been described. In 1964, Fink and associates identified and discussed 118 cases.10 They reported the average age of diagnosis at 20 years, with ages ranging from 1 year to 85 years. They also found a greater incidence among men, with a 4:1 male-to-female ratio. Recent case reports, however, show a nearly equal distribution between the sexes.

Fink and associates concluded that appendiceal intussusception is typically the result of either anatomic susceptibility or a pathologic lead point. Anatomic susceptibility is related to the width of the appendiceal base and the mobility of the appendix. They also discussed the various etiologies of the pathologic lead points but not their relative incidence. The authors recommended operative management, designating simple appendectomy as the procedure of choice with larger resection necessary for bulky disease or in cases of associated cecocolic intussusceptions.

Appendiceal intussusceptions are thought to be either idiopathic or secondary to an identifiable pathologic lead point; however, due to their rarity, no large series or meta-analyses are available and the incidence of idiopathic versus secondary intussusception is unknown. Reported pathologic lead points include fecaliths, foreign bodies, parasites, endometrial implants, lymphoid tissue, polyps, mucoceles, carcinoids, and adenocarcinoma. Adenocarcinoma of the appendix has obvious treatment implications, but recent case reports suggest most appendiceal intussusceptions are not secondary to adenocarcinoma. A July 2005 PubMed search for case reports of appendiceal intussusception yielded 53 abstracts that described the etiology of the intussusception (Table). The earliest reported case was from 1976 and the most recent was from 2005. Only seven of these intussusceptions resulted from malignant disease; thus, it can be inferred that a routine recommendation of right hemicolectomy for appendiceal intussusception may lead to significant overtreatment.11

As recommended by Fink and associates, appendectomy appears to be the operation of choice for appendiceal intussusception. The operative decision making, however, must be selective and based upon the specific pathology encountered. Ileocolic resection may be required for a bulky appendiceal mass or for associated cecocolic intussusception; right hemicolectomy is required if adenocarcinoma is suspected or identified.12

Conclusion

Appendiceal intussusception is rare in adults. While the potential causes for appendiceal intussusception are understood, the true incidence of the various etiologies is unknown. Appendectomy is the treatment of choice, but a larger resection may be required for a bulky mass, associated cecocolic intussusception, or for malignant disease.

References

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8. Collins DC. 71,000 human appendix specimens. A final report, summarizing 40 years’ study. Am J Proctol. 1963; 14(12):265-281.

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12. Nitecki SS, Wolff BG, Schlinkert MD, et al. The natural history of surgically treated primary adenocarcinoma of the appendix. . 1994; 219(1):51-57.

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