Clinical Review & Education

JAMA Surgery Clinical Challenge

Rare Cause of Recurrent Small-Bowel Obstruction June-Ho Kim, AB; Mimmie Kwong, AB; James Griffin, MD; Diane Schwartz, MD

A

B

Figure 1. Axial and coronal views of computed tomography imaging on initial presentation (A) and 1 year later (B).

A 34-year-old female Jehovah’s Witness presented to the emergency department with a 3-day history of colicky lower abdominal pain, nausea without vomiting, and constipation after passing a single small bloody bowel movement. Physical examination demonstrated a soft, nontender, and nondistended abdomen without peritoneal signs or palpable masses. Digital rectal exQuiz at jamasurgery.com amination revealed no stool in the vault and gross blood and pelvic examination were negative. Surgical history was notable only for a previous cesarean delivery. Medical history was significant for 2 years of waxing and waning generalized abdominal pain and a hospital admission for small-bowel obstruction 11 months prior that was managed conservatively. Endoscopic evaluation revealed multiple polyps in the colon, stomach, and duodenum consistent with a diagnosis of juvenile polyposis syndrome. Admission laboratories demonstrated a lactate level of 18 mg/dL (to convert to millimoles per liter, multiply by 0.111), white blood cell count of 6000/μL (to convert to ×109/L, multiply by 0.001), and hemoglobin level of 13.3 g/dL (to convert to grams per liter, multiply by 10). Amylase and lipase levels and chemistry findings were normal. Computed tomography with oral and intravenous contrast was obtained and is shown with imaging from her initial presentation 11 months prior (Figure 1).

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WHAT IS THE DIAGNOSIS?

A. Colorectal carcinoid tumor B. Ileocolic intussusception C. Cecal volvulus D. Metastatic carcinomatosis

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Clinical Review & Education JAMA Surgery Clinical Challenge

Diagnosis B. Ileocolic intussusception

Discussion This patient presented with recurrent bowel obstruction due to an extensive ileocolic intussusception. The diagnosis was made radiographically based on the presence of classic computed tomography findings, including targetlike and sausage-shaped masses, a rim of oral contrast encircling the intussusceptum, and the pathognomonic bowel-withinbowelappearanceofintraluminalmesentericfatandvessels(Figure1).1 Although the patient had hoped to avoid surgery based on her religious beliefs, nonoperative management failed at 11 months and she agreed to an operation. Intraoperatively, the intussusception extended from the ileocolic junction to mid-descending colon and was reduced primarily to reveal complete inversion of the appendix into the cecum. An ileocecectomy with primary ileocolonic anastomosis was performed and the patient was discharged home on postoperative day 5. The appendix was reduced in the postoperative specimen and noted to be grossly fibrotic. This patient had a history of juvenile polyposis syndrome, a rare autosomal dominant disorder characterized by multiple gastrointestinal hamartomas in the colon or rectum (98%), stomach (14%), small bowel (7%), and duodenum (2%).2 Final pathology revealed a clustering of at least 8 juvenile polyps in the cecum extending into the appendiceal orifice, which was noted on colonoscopy performed during the patient’s first admission (Figure 2). Prior to surgery, an ileal mass was suspected as the most likely lead point for intussusception. However, intraoperative findings and final pathology suggested a different mechanism whereby the appendiceal masses initiated an appendiceal intussusception that then served as the mechanical lead point for ileocolic intussusception. Alternatively, the mass of cecal polyps may have acted as the initial lead point for intestinal intussusception and caused a secondary or simultaneous appendiceal intussusception by tethering the base of the appendix. Intussusception is a rare cause of bowel obstruction in adults, accounting for less than 1% of obstructions and 5% of total intussusception cases.1,3-6 Symptoms are nonspecific, often chronic or subacute, and include abdominal pain, nausea, vomiting, constipation, diarrhea, andbloodperrectum.Surgicalmanagementisgenerallyindicatedsince up to 90% of cases are associated with an underlying pathological process, such as benign and malignant tumors, vascular anomalies, and inflammatory lesions that act as anatomical lead points.3-6 Appendiceal

intussusceptionisevenmorerare,withanestimatedincidenceof0.01% based on a study of 71 000 human appendix surgical specimens.7 Patients may present acutely with symptoms mimicking appendicitis or have a more protracted history of nonspecific, intermittent symptoms similar to intestinal intussusception. Adult women are disproportionately affected (76% of adult cases, 54% of all cases), with appendiceal endometriosis as the most commonly identified lead point in adults (33%).Additionalcausesincludemucocele(19%),inflammation(19%), adenoma (11%), carcinoid (7%), and adenocarcinoma (6%).8,9 Intramural and intraluminal gastrointestinal lesions are believed to cause intussusception by altering normal peristaltic activity, resulting in more vigorous and frequent peristaltic contractions meant to expel the offending lesion. For bowel, this may result in invagination or telescoping of one segment of the bowel (the “intussusceptum”) into the other (the “intussuscipiens”).1-4 For the appendix, in the setting of appropriate anatomic factors such as a fetaltype cecum, mobile mesoappendix, or wide proximal appendicular lumen, partial or total inversion into the cecum may occur.9 Appendiceal intussusception puts the patient at high risk for developing intestinal intussusception, as was likely the case for this patient, because the inverted appendix has been shown to act as a lead point for a subsequent compound ileocolic intussusception.8-10

ARTICLE INFORMATION

REFERENCES

Author Affiliations: Department of Surgery, Johns Hopkins Bayview Medical Center, Baltimore, Maryland.

1. Kim YH, Blake MA, Harisinghani MG, et al. Adult intestinal intussusception. Radiographics. 2006;26 (3):733-744.

Corresponding Author: Diane Schwartz, MD, Department of Surgery, Johns Hopkins Bayview Medical Center, 4940 Eastern Ave, A558, Baltimore, MD 21224 ([email protected]).

2. Chow E, Macrae F. A review of juvenile polyposis syndrome. J Gastroenterol Hepatol. 2005;20(11): 1634-1640.

Section Editor: Carl E. Bredenberg, MD. Published Online: May 21, 2014. doi:10.1001/jamasurg.2013.2531. Conflict of Interest Disclosures: None reported. Additional Contributions: Frederic Askin, MD, and Elizabeth Montgomery, MD, conducted final pathologic evaluation.

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Figure 2. Colonoscopic view of the cecum demonstrating extension of a cluster of juvenile polyps extending into the appendiceal orifice (arrowhead). Image courtesy of Eduardo Gonzalez-Velez, MD.

3. Azar T, Berger DL. Adult intussusception. Ann Surg. 1997;226(2):134-138. 4. Marinis A, Yiallourou A, Samanides L, et al. Intussusception of the bowel in adults: a review. World J Gastroenterol. 2009;15(4):407-411. 5. Barussaud M, Regenet N, Briennon X, et al. Clinical spectrum and surgical approach of adult intussusceptions. Int J Colorectal Dis. 2006;21(8): 834-839.

6. Begos DG, Sandor A, Modlin IM. The diagnosis and management of adult intussusception. Am J Surg. 1997;173(2):88-94. 7. Collins DC. 71,000 Human appendix specimens. Am J Proctol. 1963;14:265-281. 8. Chaar CI, Wexelman B, Zuckerman K, Longo W. Intussusception of the appendix. Am J Surg. 2009; 198(1):122-128. 9. Varsamis N, Pouggouras K, Salveridis N, et al. Appendiceal intussusception. In: Lule G, ed. Current Concepts in Colonic Disorders. Rijeka, Croatia: InTech; 2012:47-64. 10. Liang HH, Huang MT, Wei PL, et al. Endometriosis-induced appendiceal intussusception. Am J Surg. 2009;197(6):e66-e68.

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Rare Cause of Recurrent Small-Bowel Obstruction.

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