Clinical Review & Education
JAMA Surgery Clinical Challenge
Lower Abdominal Pain With Nausea and Vomiting Valentino Fiscon, MD; Giuseppe Portale, MD; Paola Cusatelli, MD
A
B
Figure. A, Abdominal computed tomography suggesting the presence of an ileal intussusception. B, Colonoscopy revealing the presence of a bulge in the cecal lumen.
A woman in her 40s presented to our department with lower abdominal pain, lasting for a few weeks, with nausea and vomiting but no diarrhea or fever. Her medical history was unremarkable; in particular, she had no history of endometriosis. She had visited the emergency department of another hospital 2 weeks before with the same symptoms and had received Quiz at jamasurgery.com conservative treatment. On physical examination in the present visit, she had lower abdominal tenderness but no sign of peritoneal irritation. A mass was palpable in the lower right abdominal quadrant. The results of routine blood investigations, including white blood cell count, hemoglobin concentration, and C-reactive protein level, were within normal limits, as they had been in the previous hospital. Abdominal ultrasonographic examination was performed, followed by abdominal computed tomography; the results were suggestive of an ileal intussusception (Figure, A). A colonoscopy revealed the presence of a bulge in the cecal lumen (Figure, B).
jamasurgery.com
WHAT IS THE DIAGNOSIS?
A. Appendiceal abscess B. Ileal intussusception C. Cecal volvulus D. Appendiceal intussusception
JAMA Surgery July 2014 Volume 149, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Purdue University User on 06/15/2015
739
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis D. Appendiceal intussusception
Discussion Laparoscopic exploration was offered to the patient, and she consented. A cecal thickening with complete appendiceal intussusception (AI) was seen during the procedure. No sign of even small foci of endometriosis was noted on exploration. The appendix could not be identified at the confluence of the teniae, and any careful attempt to reduce the intussuscepted appendix was unsuccessful. Given the risk of disseminating neoplastic cells if this was a carcinoid tumor or an adenocarcinoma or if foci of endometriosis were present, we decided to convert the procedure to open surgery (using a small, infraumbilical incision). The intussuscepted appendix on manual examination was extremely enlarged and remained difficult to reduce. It was very close to the ileocecal valve; therefore, we decided to perform an ileocecectomy. When the specimen was opened, the entire appendix appeared inverted (complete intussusception of the appendix, type V, according to the McSwain classification).1 Histologic examination of the specimen revealed several foci of previously unsuspected endometriosis. The patient’s recovery was uneventful, and she was discharged home on postoperative day 5. She was evaluated by a gynecologist and began receiving hormonal suppression of endometriosis. Intussusception of the appendix is a very rare condition, estimated to be 0.01% in a large historical study on surgical specimens of human appendixes.2 Since the first report by McKidd3 in a 7-yearold boy in 1858, the literature on AI has been limited to case reports or very small case series because of the rarity of this condition.4 In addition, endometriosis as a cause of AI is even more rare, with only 38 cases published.5,6 In fact, when endometriosis involves the gastrointestinal tract (usually one-third of patients with pelvic endometriosis), it commonly involves the rectosigmoid (72%) or the rectovaginal septum (13%) but rarely the appendix (3%).7 In the largest series published to date,8,9 the prevalence of endometriosis of the appendix ranged from 0.02% to 0.8%. Lesions within the lumen of the appendix may act as lead points for AI: peristalsis of the bowel loops drags the lesion forward and
causes the invagination of a bowel loop with its mesenteric fold into the lumen of an adjacent portion of bowel. The most common cause of intussusception in children is appendiceal inflammation; among adults, inflammation as the cause is far less common. Endometriosis (33%) with hyperplasia or hypertrophy of the muscularis propria, adenoma/adenocarcinoma (17%), mucocele (14%), and carcinoid tumors (7%) are causes in most of the adult cases.2 A mobile appendix and mesoappendix as well as a wide appendicular lumen make AI more likely.10 An anatomical classification proposed in 1941 by McSwain1 and still in use is based on the portion of the appendix invaginated, ranging from type I (only the tip of the appendix) to type V (the entire appendix, as in our case). In adults, the intussuscepted appendix should be removed for 2 reasons: eventually AI will recur, causing symptoms, and the appendiceal lesion, which acts as lead point and causes the intussusception, may be malignant. Unfortunately, the nature of AI cannot be determined preoperatively in most, if not all, cases. At best, it can be suspected, for example, in women with a history of endometriosis. Colonoscopy reduction of an intussuscepted appendix is not recommended because of the risk of perforation and dissemination of a potentially malignant lesion with peritoneal seeding. Appendectomy is considered adequate when AI involves only the tip or the base of the appendix and can be easily reduced in the abdominal cavity. However, in case of complete intussusception of the appendix with a large mass close to the ileocecal valve, a lesion involving the base of the appendix, or a suspected malignant mucinous lesion, ileocecal resection or right hemicolectomy should be performed, either laparoscopically or with open incision, to achieve an adequate tumorfree margin. If endometriosis is suspected because of the patient’s medical history, laparoscopy is strongly recommended: if endometriosis is present it can reveal (although not in our case) yellowbrown discoloration of small patches in the ovaries, broad ligament, and other abnormalities, allowing a full evaluation of the extent of the disease. Gentle traction should be applied to the appendix in cases of AI with endometriosis, as with other causes of AI in adults. However, this maneuver should be performed with caution to avoid disseminating the foci of endometriosis. If reduction of the appendix fails, ileocecectomy or right hemicolectomy should be performed.
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of General Surgery, Azienda Unità Locale Socio Sanitaria 15, Alta Padovana, Cittadella, Padova, Italy (Fiscon, Portale); Department of Pathology, Azienda Unità Locale Socio Sanitaria 15, Alta Padovana, Cittadella, Padova, Italy (Cusatelli).
1. McSwain B. Intussusception of the appendix. South Med J. 1941;34:263-271.
Corresponding Author: Giuseppe Portale, MD, Department of General Surgery, Azienda Unità Locale Socio Sanitaria 15, Alta Padovana, Via Casa di Ricovero, 40, 35013 Cittadella, Padova, Italy ([email protected] portale.giuseppe @libero.it).
2. Collins DC. 71,000 Human appendix specimens: a final report, summarizing forty years’ study. Am J Proctol. 1963;14:265-281. 3. McKidd J. Case of invagination of caecum and appendix. Edinburgh Med J. 1858;4:793-797. 4. Chaar CI, Wexelman B, Zuckerman K, Longo W. Intussusception of the appendix: comprehensive review of the literature. Am J Surg. 2009;198:122128. 5. Saleem A, Navarro P, Munson JL, Hall J. Endometriosis of the appendix: report of three cases. Int J Surg Case Rep. 2011;2(2):16-19.
Section Editor: Carl E. Bredenberg, MD. Published Online: May 14, 2014. doi:10.1001/jamasurg.2013.378. Conflict of Interest Disclosures: None reported.
740
Endometriosis Causing Appendiceal Intussusception
7. Prystowsky JB, Stryker SJ, Ujiki GT, Poticha SM. Gastrointestinal endometriosis: incidence and indications for resection. Arch Surg. 1988;123(7): 855-858. 8. Collins DC. A study of 50,000 specimens of the human vermiform appendix. Surg Gynecol Obstet. 1955;101(4):437-445. 9. Marudanayagam R, Williams GT, Rees BI. Review of the pathological results of 2660 appendicectomy specimens. J Gastroenterol. 2006; 41(8):745-749. 10. Fink VH, Santos AL, Goldberg SL. Intussusception of the appendix: case reports and review of the literature. Am J Gastroenterol. 1964;42:431-441.
6. Mehmood S, Phair A, Sahely S, et al. Appendiceal intussusception caused by endometriosis. Lancet. 2012;380(9848):1202. doi:10.1016/S0140-6736(12) 60819-8.
JAMA Surgery July 2014 Volume 149, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Purdue University User on 06/15/2015
jamasurgery.com
JAMA Surgery Clinical Challenge
Lower Abdominal Pain With Nausea and Vomiting Valentino Fiscon, MD; Giuseppe Portale, MD; Paola Cusatelli, MD
A
B
Figure. A, Abdominal computed tomography suggesting the presence of an ileal intussusception. B, Colonoscopy revealing the presence of a bulge in the cecal lumen.
A woman in her 40s presented to our department with lower abdominal pain, lasting for a few weeks, with nausea and vomiting but no diarrhea or fever. Her medical history was unremarkable; in particular, she had no history of endometriosis. She had visited the emergency department of another hospital 2 weeks before with the same symptoms and had received Quiz at jamasurgery.com conservative treatment. On physical examination in the present visit, she had lower abdominal tenderness but no sign of peritoneal irritation. A mass was palpable in the lower right abdominal quadrant. The results of routine blood investigations, including white blood cell count, hemoglobin concentration, and C-reactive protein level, were within normal limits, as they had been in the previous hospital. Abdominal ultrasonographic examination was performed, followed by abdominal computed tomography; the results were suggestive of an ileal intussusception (Figure, A). A colonoscopy revealed the presence of a bulge in the cecal lumen (Figure, B).
jamasurgery.com
WHAT IS THE DIAGNOSIS?
A. Appendiceal abscess B. Ileal intussusception C. Cecal volvulus D. Appendiceal intussusception
JAMA Surgery July 2014 Volume 149, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Purdue University User on 06/15/2015
739
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis D. Appendiceal intussusception
Discussion Laparoscopic exploration was offered to the patient, and she consented. A cecal thickening with complete appendiceal intussusception (AI) was seen during the procedure. No sign of even small foci of endometriosis was noted on exploration. The appendix could not be identified at the confluence of the teniae, and any careful attempt to reduce the intussuscepted appendix was unsuccessful. Given the risk of disseminating neoplastic cells if this was a carcinoid tumor or an adenocarcinoma or if foci of endometriosis were present, we decided to convert the procedure to open surgery (using a small, infraumbilical incision). The intussuscepted appendix on manual examination was extremely enlarged and remained difficult to reduce. It was very close to the ileocecal valve; therefore, we decided to perform an ileocecectomy. When the specimen was opened, the entire appendix appeared inverted (complete intussusception of the appendix, type V, according to the McSwain classification).1 Histologic examination of the specimen revealed several foci of previously unsuspected endometriosis. The patient’s recovery was uneventful, and she was discharged home on postoperative day 5. She was evaluated by a gynecologist and began receiving hormonal suppression of endometriosis. Intussusception of the appendix is a very rare condition, estimated to be 0.01% in a large historical study on surgical specimens of human appendixes.2 Since the first report by McKidd3 in a 7-yearold boy in 1858, the literature on AI has been limited to case reports or very small case series because of the rarity of this condition.4 In addition, endometriosis as a cause of AI is even more rare, with only 38 cases published.5,6 In fact, when endometriosis involves the gastrointestinal tract (usually one-third of patients with pelvic endometriosis), it commonly involves the rectosigmoid (72%) or the rectovaginal septum (13%) but rarely the appendix (3%).7 In the largest series published to date,8,9 the prevalence of endometriosis of the appendix ranged from 0.02% to 0.8%. Lesions within the lumen of the appendix may act as lead points for AI: peristalsis of the bowel loops drags the lesion forward and
causes the invagination of a bowel loop with its mesenteric fold into the lumen of an adjacent portion of bowel. The most common cause of intussusception in children is appendiceal inflammation; among adults, inflammation as the cause is far less common. Endometriosis (33%) with hyperplasia or hypertrophy of the muscularis propria, adenoma/adenocarcinoma (17%), mucocele (14%), and carcinoid tumors (7%) are causes in most of the adult cases.2 A mobile appendix and mesoappendix as well as a wide appendicular lumen make AI more likely.10 An anatomical classification proposed in 1941 by McSwain1 and still in use is based on the portion of the appendix invaginated, ranging from type I (only the tip of the appendix) to type V (the entire appendix, as in our case). In adults, the intussuscepted appendix should be removed for 2 reasons: eventually AI will recur, causing symptoms, and the appendiceal lesion, which acts as lead point and causes the intussusception, may be malignant. Unfortunately, the nature of AI cannot be determined preoperatively in most, if not all, cases. At best, it can be suspected, for example, in women with a history of endometriosis. Colonoscopy reduction of an intussuscepted appendix is not recommended because of the risk of perforation and dissemination of a potentially malignant lesion with peritoneal seeding. Appendectomy is considered adequate when AI involves only the tip or the base of the appendix and can be easily reduced in the abdominal cavity. However, in case of complete intussusception of the appendix with a large mass close to the ileocecal valve, a lesion involving the base of the appendix, or a suspected malignant mucinous lesion, ileocecal resection or right hemicolectomy should be performed, either laparoscopically or with open incision, to achieve an adequate tumorfree margin. If endometriosis is suspected because of the patient’s medical history, laparoscopy is strongly recommended: if endometriosis is present it can reveal (although not in our case) yellowbrown discoloration of small patches in the ovaries, broad ligament, and other abnormalities, allowing a full evaluation of the extent of the disease. Gentle traction should be applied to the appendix in cases of AI with endometriosis, as with other causes of AI in adults. However, this maneuver should be performed with caution to avoid disseminating the foci of endometriosis. If reduction of the appendix fails, ileocecectomy or right hemicolectomy should be performed.
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of General Surgery, Azienda Unità Locale Socio Sanitaria 15, Alta Padovana, Cittadella, Padova, Italy (Fiscon, Portale); Department of Pathology, Azienda Unità Locale Socio Sanitaria 15, Alta Padovana, Cittadella, Padova, Italy (Cusatelli).
1. McSwain B. Intussusception of the appendix. South Med J. 1941;34:263-271.
Corresponding Author: Giuseppe Portale, MD, Department of General Surgery, Azienda Unità Locale Socio Sanitaria 15, Alta Padovana, Via Casa di Ricovero, 40, 35013 Cittadella, Padova, Italy ([email protected] portale.giuseppe @libero.it).
2. Collins DC. 71,000 Human appendix specimens: a final report, summarizing forty years’ study. Am J Proctol. 1963;14:265-281. 3. McKidd J. Case of invagination of caecum and appendix. Edinburgh Med J. 1858;4:793-797. 4. Chaar CI, Wexelman B, Zuckerman K, Longo W. Intussusception of the appendix: comprehensive review of the literature. Am J Surg. 2009;198:122128. 5. Saleem A, Navarro P, Munson JL, Hall J. Endometriosis of the appendix: report of three cases. Int J Surg Case Rep. 2011;2(2):16-19.
Section Editor: Carl E. Bredenberg, MD. Published Online: May 14, 2014. doi:10.1001/jamasurg.2013.378. Conflict of Interest Disclosures: None reported.
740
Endometriosis Causing Appendiceal Intussusception
7. Prystowsky JB, Stryker SJ, Ujiki GT, Poticha SM. Gastrointestinal endometriosis: incidence and indications for resection. Arch Surg. 1988;123(7): 855-858. 8. Collins DC. A study of 50,000 specimens of the human vermiform appendix. Surg Gynecol Obstet. 1955;101(4):437-445. 9. Marudanayagam R, Williams GT, Rees BI. Review of the pathological results of 2660 appendicectomy specimens. J Gastroenterol. 2006; 41(8):745-749. 10. Fink VH, Santos AL, Goldberg SL. Intussusception of the appendix: case reports and review of the literature. Am J Gastroenterol. 1964;42:431-441.
6. Mehmood S, Phair A, Sahely S, et al. Appendiceal intussusception caused by endometriosis. Lancet. 2012;380(9848):1202. doi:10.1016/S0140-6736(12) 60819-8.
JAMA Surgery July 2014 Volume 149, Number 7
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Purdue University User on 06/15/2015
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