Clinical Review & Education
JAMA Surgery Clinical Challenge
The Cocoon Abdomen Gary B. Deutsch, MD; Jaime Shamonki, MD; Anton J. Bilchik, MD, PhD
A
C B
Figure 1. Cross-sectional computed tomographic scan of the abdomen. Dilated small intestine with air-fluid levels extending to the terminal ileum (A and B). C, Intraoperative image of involved small intestine. Tightly coiled and twisted small bowel with adhesive bands.
An 85-year-old man with a history of occupational exposure to asbestos presented with 1 week of nausea and vomiting without any bowel function. He reported only mild abdominal pain. The patient denied any history of abdominal surgery. On examination, he was mildly distended and tender focally on the right side. In the emergency department, a nasogastric tube was placed, yielding a moderate amount of dark, foul-smelling output. Laboratory results were significant for a white blood cell count of 13 300/μL (to convert to ×109 per liter, multiply by 0.001) (bands 20%). A contrast-enhanced computed tomographic scan of the abdomen and pelvis demonstrated dilated small intestine (measuring up to 4.7 cm), with air-fluid levels extending to the terminal ileum (Figure 1A and B). The entire colon was collapsed. There were several loops of normal-caliber bowel proximal to the area of concern. The decision was made to take him to the operating room for exploration given the lack of any previous abdominal surgical procedures and no other explanation for his symptoms. Intraoperatively, a large portion of the small intestine was involved in dense adhesions. After performing careful lysis on the distal involved bowel, a tightly coiled and twisted segment was encountered (Figure 1C). At this point, further attempts to separate loops of small intestine would have been difficult, and the decision was made to resect this segment (approximately 110 cm) and perform a primary anastomosis.
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Peritoneal mesothelioma B. Sclerosing encapsulated peritonitis C. Malrotation with Ladd bands D. Peritoneal encapsulation
JAMA Surgery November 2014 Volume 149, Number 11
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Nanyang Technological University User on 06/18/2015
1201
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis
A
B. Sclerosing encapsulated peritonitis
Discussion Pathologic analysis revealed a thickened, edematous peritonealized surface that was grossly and microscopically contiguous with the outer layer of a distorted segment of small intestine. A highpower view demonstrates markedly thickened serosa, usually limited to a single layer of mesothelium, expanded by dense paucicellular fibrous tissue (bracketed area) (Figure 2). There was no evidence of amyloid deposition or infection with acid-fast bacteria. The patient rapidly regained bowel function after surgery and was discharged on a regular diet by the fourth postoperative day. In a patient without a history of abdominal surgery, the differential diagnosis of small-bowel obstruction should include incarcerated hernia, malignancy, or an initial presentation of Crohn disease. Internal hernia, intestinal volvulus, gallstone ileus, and bezoars are less-common possibilities, as is sclerosing encapsulated peritonitis, also called the abdominal cocoon syndrome. Although this rare disease has been associated with long-term peritoneal dialysis, sarcoidosis, systemic lupus erythematosus, subclinical viral peritonitis, and protein S deficiency, most cases of sclerosing encapsulated peritonitis are idiopathic and likely secondary to a distant unrecognized inflammatory event. Sclerosing encapsulated peritonitis most often occurs in young females but may be discovered later in life and in males; only a few cases have been reported in the geriatric population. One of the largest series contained 24 patients treated over a 10-year period: 37% were men and no patient was older than 57 years. Most had symptoms of intestinal obstruction; only 3 patients had an asymptomatic abdominal mass revealed by examination or imaging.1 Advances in cross-sectional imaging have improved preoperative diagnosis of sclerosing encapsulated peritonitis. Computed tomography may show peritoneal thickening, loculated fluid collections, peritoneal calcification, bowel tethering or matting, peritoneal enhancement, and/or bowel-wall thickening and calcification.2 Immediate operative exploration may not be indicated in subacute or chronic presentations if symptoms are absent.3 Peritoneal mesothelioma, a rare abdominal manifestation of primary mesothelioma, can mimic the bowel obstruction caused by sclerosing encapsulated peritonitis, although it is more likely to be diffuse than focal. When the mesothelioma occurs in the mesentery, thickening of the peritoneal lining is seen.4 Intestinal malrotation with Ladd bands is most commonly associated with infancy or childhood but can
B
Serosal layer
Figure 2. Histologic image of small-bowel serosal layer (high power). Markedly thickened serosa with dense paucicellular fibrous tissue (hematoxylin-eosin; A, original magnification ×40; B, original magnification ×100).
occur in nearly 50% of adults diagnosed as having intestinal malrotation.5 The presence of fibrous bands across the cecum and failure of the duodenum to cross the midline are pathognomonic. These patients usually present with chronic abdominal pain, sometimes with nausea and vomiting. Peritoneal encapsulation, a congenital anomaly that covers the small intestine with a thin peritoneal sac, can also mimic sclerosing encapsulated peritonitis. However, this scenario is usually encountered incidentally on exploration for other reasons and is not associated with extensive fibrosis and scarring.6
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of Surgical Oncology, John Wayne Cancer Institute, Santa Monica, California (Deutsch, Bilchik); Department of Pathology, Providence Saint John’s Health Center, Santa Monica, California (Shamonki).
1. Wei B, Wei H-B, Guo W-P, et al. Diagnosis and treatment of abdominal cocoon: a report of 24 cases. Am J Surg. 2009;198(3):348-353.
Corresponding Author: Anton J. Bilchik, MD, PhD, Department of Surgical Oncology, John Wayne Cancer Institute, 2200 Santa Monica Blvd, Santa Monica, CA 90404 ([email protected]). Section Editor: Jonathan R. Hiatt, MD. Published Online: September 10, 2014. doi:10.1001/jamasurg.2014.81.
2. George C, Al-Zwae K, Nair S, Cast JEI. Computed tomography appearances of sclerosing encapsulating peritonitis. Clin Radiol. 2007;62(8): 732-737. 3. Célicout B, Levard H, Hay J, Msika S, Fingerhut A, Pelissier E; French Associations for Surgical Research. Sclerosing encapsulating peritonitis: early and late results of surgical management in 32 cases. Dig Surg. 1998;15(6):697-702.
4. Kuroda K, Ishizawa S, Kudo T, et al. Localized malignant mesenteric mesothelioma causing small bowel obstruction. Pathol Int. 2008;58(4):239-243. 5. Nehra D, Goldstein AM. Intestinal malrotation: varied clinical presentation from infancy through adulthood. Surgery. 2011;149(3):386-393. 6. Deeb LS, Mourad FH, El-Zein YR, Uthman SM. Abdominal cocoon in a man: preoperative diagnosis and literature review. J Clin Gastroenterol. 1998;26 (2):148-150.
Conflict of Interest Disclosures: None reported.
1202
JAMA Surgery November 2014 Volume 149, Number 11
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Nanyang Technological University User on 06/18/2015
jamasurgery.com
JAMA Surgery Clinical Challenge
The Cocoon Abdomen Gary B. Deutsch, MD; Jaime Shamonki, MD; Anton J. Bilchik, MD, PhD
A
C B
Figure 1. Cross-sectional computed tomographic scan of the abdomen. Dilated small intestine with air-fluid levels extending to the terminal ileum (A and B). C, Intraoperative image of involved small intestine. Tightly coiled and twisted small bowel with adhesive bands.
An 85-year-old man with a history of occupational exposure to asbestos presented with 1 week of nausea and vomiting without any bowel function. He reported only mild abdominal pain. The patient denied any history of abdominal surgery. On examination, he was mildly distended and tender focally on the right side. In the emergency department, a nasogastric tube was placed, yielding a moderate amount of dark, foul-smelling output. Laboratory results were significant for a white blood cell count of 13 300/μL (to convert to ×109 per liter, multiply by 0.001) (bands 20%). A contrast-enhanced computed tomographic scan of the abdomen and pelvis demonstrated dilated small intestine (measuring up to 4.7 cm), with air-fluid levels extending to the terminal ileum (Figure 1A and B). The entire colon was collapsed. There were several loops of normal-caliber bowel proximal to the area of concern. The decision was made to take him to the operating room for exploration given the lack of any previous abdominal surgical procedures and no other explanation for his symptoms. Intraoperatively, a large portion of the small intestine was involved in dense adhesions. After performing careful lysis on the distal involved bowel, a tightly coiled and twisted segment was encountered (Figure 1C). At this point, further attempts to separate loops of small intestine would have been difficult, and the decision was made to resect this segment (approximately 110 cm) and perform a primary anastomosis.
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Peritoneal mesothelioma B. Sclerosing encapsulated peritonitis C. Malrotation with Ladd bands D. Peritoneal encapsulation
JAMA Surgery November 2014 Volume 149, Number 11
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Nanyang Technological University User on 06/18/2015
1201
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis
A
B. Sclerosing encapsulated peritonitis
Discussion Pathologic analysis revealed a thickened, edematous peritonealized surface that was grossly and microscopically contiguous with the outer layer of a distorted segment of small intestine. A highpower view demonstrates markedly thickened serosa, usually limited to a single layer of mesothelium, expanded by dense paucicellular fibrous tissue (bracketed area) (Figure 2). There was no evidence of amyloid deposition or infection with acid-fast bacteria. The patient rapidly regained bowel function after surgery and was discharged on a regular diet by the fourth postoperative day. In a patient without a history of abdominal surgery, the differential diagnosis of small-bowel obstruction should include incarcerated hernia, malignancy, or an initial presentation of Crohn disease. Internal hernia, intestinal volvulus, gallstone ileus, and bezoars are less-common possibilities, as is sclerosing encapsulated peritonitis, also called the abdominal cocoon syndrome. Although this rare disease has been associated with long-term peritoneal dialysis, sarcoidosis, systemic lupus erythematosus, subclinical viral peritonitis, and protein S deficiency, most cases of sclerosing encapsulated peritonitis are idiopathic and likely secondary to a distant unrecognized inflammatory event. Sclerosing encapsulated peritonitis most often occurs in young females but may be discovered later in life and in males; only a few cases have been reported in the geriatric population. One of the largest series contained 24 patients treated over a 10-year period: 37% were men and no patient was older than 57 years. Most had symptoms of intestinal obstruction; only 3 patients had an asymptomatic abdominal mass revealed by examination or imaging.1 Advances in cross-sectional imaging have improved preoperative diagnosis of sclerosing encapsulated peritonitis. Computed tomography may show peritoneal thickening, loculated fluid collections, peritoneal calcification, bowel tethering or matting, peritoneal enhancement, and/or bowel-wall thickening and calcification.2 Immediate operative exploration may not be indicated in subacute or chronic presentations if symptoms are absent.3 Peritoneal mesothelioma, a rare abdominal manifestation of primary mesothelioma, can mimic the bowel obstruction caused by sclerosing encapsulated peritonitis, although it is more likely to be diffuse than focal. When the mesothelioma occurs in the mesentery, thickening of the peritoneal lining is seen.4 Intestinal malrotation with Ladd bands is most commonly associated with infancy or childhood but can
B
Serosal layer
Figure 2. Histologic image of small-bowel serosal layer (high power). Markedly thickened serosa with dense paucicellular fibrous tissue (hematoxylin-eosin; A, original magnification ×40; B, original magnification ×100).
occur in nearly 50% of adults diagnosed as having intestinal malrotation.5 The presence of fibrous bands across the cecum and failure of the duodenum to cross the midline are pathognomonic. These patients usually present with chronic abdominal pain, sometimes with nausea and vomiting. Peritoneal encapsulation, a congenital anomaly that covers the small intestine with a thin peritoneal sac, can also mimic sclerosing encapsulated peritonitis. However, this scenario is usually encountered incidentally on exploration for other reasons and is not associated with extensive fibrosis and scarring.6
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of Surgical Oncology, John Wayne Cancer Institute, Santa Monica, California (Deutsch, Bilchik); Department of Pathology, Providence Saint John’s Health Center, Santa Monica, California (Shamonki).
1. Wei B, Wei H-B, Guo W-P, et al. Diagnosis and treatment of abdominal cocoon: a report of 24 cases. Am J Surg. 2009;198(3):348-353.
Corresponding Author: Anton J. Bilchik, MD, PhD, Department of Surgical Oncology, John Wayne Cancer Institute, 2200 Santa Monica Blvd, Santa Monica, CA 90404 ([email protected]). Section Editor: Jonathan R. Hiatt, MD. Published Online: September 10, 2014. doi:10.1001/jamasurg.2014.81.
2. George C, Al-Zwae K, Nair S, Cast JEI. Computed tomography appearances of sclerosing encapsulating peritonitis. Clin Radiol. 2007;62(8): 732-737. 3. Célicout B, Levard H, Hay J, Msika S, Fingerhut A, Pelissier E; French Associations for Surgical Research. Sclerosing encapsulating peritonitis: early and late results of surgical management in 32 cases. Dig Surg. 1998;15(6):697-702.
4. Kuroda K, Ishizawa S, Kudo T, et al. Localized malignant mesenteric mesothelioma causing small bowel obstruction. Pathol Int. 2008;58(4):239-243. 5. Nehra D, Goldstein AM. Intestinal malrotation: varied clinical presentation from infancy through adulthood. Surgery. 2011;149(3):386-393. 6. Deeb LS, Mourad FH, El-Zein YR, Uthman SM. Abdominal cocoon in a man: preoperative diagnosis and literature review. J Clin Gastroenterol. 1998;26 (2):148-150.
Conflict of Interest Disclosures: None reported.
1202
JAMA Surgery November 2014 Volume 149, Number 11
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Nanyang Technological University User on 06/18/2015
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