Case Report
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. Feb 27, 2016; 8(2): 173-178
Published online Feb 27, 2016. doi: 10.4240/wjgs.v8.i2.173
Pneumatosis intestinalis with obstructing intussusception: A case report and literature review
Yujiro Itazaki, Hironori Tsujimoto, Nozomi Ito, Hiroyuki Horiguchi, Shinsuke Nomura, Kyohei Kanematsu, Shuichi Hiraki, Suefumi Aosasa, Junji Yamamoto, Kazuo Hase
Yujiro Itazaki, Hironori Tsujimoto, Nozomi Ito, Hiroyuki Horiguchi, Shinsuke Nomura, Kyohei Kanematsu, Shuichi Hiraki, Suefumi Aosasa, Junji Yamamoto, Kazuo Hase, Department of Surgery, National Defense Medical College, Saitama 359-8513, Japan
Author contributions: Itazaki Y, Tsujimoto H and Hiraki S designed the research; Ito N, Horiguchi H, Nomura S, Kanematsu K and Aosasa S performed the research; Itazaki Y, Tsujimoto H, Yamamoto J and Hase K wrote the paper; all authors had substantial contributions to conception, design, and reviewing the manuscript; all authors have approved the manuscript and agree with submission to the journal.
Supported by JSPS KAKENHI, No. 15K10042.
Institutional review board statement: This study had reviewed by National Defense Medical College.
Informed consent statement: Written informed consent was obtained from the patient.
Conflict-of-interest statement: All authors certify that they have no commercial associations that might pose a conflict of interest in connection with submitted article.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Hironori Tsujimoto, MD, PhD, Department of Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama 359-8513, Japan. tsujihi@ndmc.ac.jp
Telephone: +81-4-29951637 Fax: +81-4-29965205
Received: May 20, 2015
Peer-review started: May 21, 2015
First decision: August 4, 2015
Revised: September 16, 2015
Accepted: October 16, 2015
Article in press: October 17, 2015
Published online: February 27, 2016

Abstract

Pneumatosis intestinalis (PI) often represents a benign condition that should not be considered as an argument for surgery. We report a patient with PI and obstructing intussusception who underwent urgent colectomy and review the literatures regarding PI with intussusception. A 20-year-old man presented at our hospital with a 3-d intermittent lower abdominal pain history. He underwent steroid therapy for membranoproliferative glomerulonephritis for 4 years. Computed tomography revealed ascending colon intussusception with air within the wall. Intraoperative colonoscopy revealed numerous soft polypoid masses with normal overlying mucosa and right hemicolectomy was performed. Histological examination of colonic wall sections revealed large cysts in the submucosal layer. The pathological diagnosis was PI. Nine cases of intussusception associated with primary PI have been reported. Although primary PI often represents a benign condition that should not be considered as an argument for surgery, if the case involves intussusception and obstruction, emergent laparotomy should be considered.

Key Words: Pneumatosis intestinalis, Intussusception, Urgent surgery, Immunosuppressive drug, Ischemia of the intestine

Core tip: We report a patient with pneumatosis intestinalis (PI) and obstructing intussusception who underwent urgent colectomy and review the literatures regarding PI with intussusception. A 20-year-old man presented at our hospital with abdominal pain, and has undergone steroid therapy for 4 years. Computed tomography revealed ascending colon intussusception with air within the wall, and colectomy was performed. Histological examination of colonic wall sections revealed large cysts in the submucosal layer. Nine cases of intussusception associated with primary PI have been reported. Although primary PI often represents a benign condition, if the case involves intussusception and obstruction, emergent laparotomy should be considered.



INTRODUCTION

Pneumatosis intestinalis (PI) is a rare condition characterized by the presence of gas within the wall of the gastrointestinal tract. This condition can result from a wide variety of pathologies, including chronic obstructive lung disease, collagen diseases, necrotizing enterocolitis in premature infants, intestinal infections, ischemic bowel disorders, and immunosuppressive drug therapy[1]. PI often represents a benign condition that should not be considered as an argument for surgery[2]; however, immediate surgery may be required in some life-threatening circumstances such as the presence of bowel obstruction, perforation, or ischemia[3].

Here we describe a case of PI in the ascending colon with obstructing intussusception for which urgent surgery was performed, and review the available published literature on PI with intussusception. Written informed consent was obtained from the patient.

Search strategy

The literature search strategy for this study was based on published systematic review guidelines[4]. Literature databases such as PubMed MEDLINE (National Library of Medicine) were searched from 1980 to 2015 using the following medical subject headings: “PI (or Pneumatosis cystoides intestinalis)” and “intussusception” or “PI (or Pneumatosis cystoides intestinalis)” and “invagination”. In addition, references within the retrieved articles were reviewed. We identified 24 manuscripts using this search strategy and selected 8 case reports for this review. Nineteen articles were excluded because their content was not applicable to this review and 7 articles were excluded because they were not written in English.

CASE REPORT

A 20-year-old man presented our hospital with a 3-d history of intermittent lower abdominal pain. He had been on steroid therapy (methylprednisolone 25 mg/d) for membranoproliferative glomerulonephritis for 4 years. A physical examination revealed tenderness in the lower right quadrant of the abdomen. His body temperature was 37.7 °C and pulse was 81 beats per minute. All serum levels tested were within the normal range, with the exception of serum total bilirubin (1.5 mg/dL; normal range, 0.3-1.2 mg/dL). White blood cells (WBCs) (21000/μL), hemoglobin concentration (17.1 g/dL), and the C-reactive protein concentration (0.5 mg/dL) were also elevated, indicating acute inflammation and dehydration, with a level of base excess of 2.4 mmol/L. Abdominal X-ray showed multiple air-filled lucencies in the right upper quadrant and multiple distended loops of small bowel with fluid (Figure 1A). Computed tomography (CT) revealed intussusception of the ascending colon with air within the wall (Figure 1B). We performed an urgent laparotomy under the diagnosis of acute abdomen with obstructing intussusception. The colo-colic intussusception that was caudal to the polypoid lesion easily resolved using Hutchinson’s maneuver and soft polypoid masses were palpable from the cecum to the ascending colon (Figure 2). Intraoperative colonoscopy revealed the presence of numerous soft polypoid masses with normal overlying mucosa (Figure 3). A right hemicolectomy was performed because other polyposis diseases or intussusception relapse could not be ruled out, and to help make a final pathological diagnosis. A gross examination showed that the mucosa of the resected colon appeared normal with no evidence of ulceration or ulcer-related lesions, but instead a number of soft, yellowish cystic masses (Figures 4). A histological examination of sections of the colonic wall revealed large cysts in the submucosal layer (Figure 5A). The cysts were empty, but were surrounded by a distinct fibrous wall and were lined by macrophages that frequently coalesced to form multinucleated giant cells (Figure 5B). Based on these findings, the patient was diagnosed with pneumatosis cystoides intestinalis. The postoperative course was uneventful and the patient was discharged on postoperative day 9. No recurrence was noted on radiographic imaging performed on postoperative 14 mo.

Figure 1
Figure 1 Abdominal radiograph. It showing multiple distended loops of small bowel with fluids and multiple air pockets (A) and computed tomography showing multiple gas-filled cysts, a streaky collection of air in the bowel wall, and an intussusception of the colon (B).
Figure 2
Figure 2 Intraoperative findings showed intussusception of the ascending colon with palpable soft polypoid masses.
Figure 3
Figure 3 Intraoperative colonoscopy showed numerous soft polypoid masses with normal overlying mucosa located between the ascending colon and middle part of transverse colon.
Figure 4
Figure 4 The resected specimen revealed polypoid lesions with normal mucosa and cystic structures (A), submucosal cysts had a spongy consistency (B).
Figure 5
Figure 5 Histopathological examination revealed cystic air-filled spaces within the submucosa, which were partially lined by clusters of foreign-body macrophages (arrow heads) (hematoxylin-eosin stain; A: × 40, B: × 400).
DISCUSSION

Classically, PI can be subdivided into 2 distinct groups: primary PI, representing 15% of cases, and secondary PI, representing 85% of cases[5]. Secondary PI, where the gas accumulates as linear collections and reflects a pathological condition, has been attributed to endoscopic procedures, immunological disturbances, bowel mucosal disruptions, and intra-abdominal pathologies. In contrast to secondary PI, primary PI is characterized by intramural gas that is cystic and benign in nature and does not always require urgent laparotomy[6]. Although PI may occur in association with acquired immunodeficiency[7], transplant status[8], cancer treatment[9,10], scleroderma[11], cystic fibrosis[12], systemic lupus[13], inflammatory bowel disease[2], intestinal ischemia[14], colitis[15], or trauma[16], the exact etiology of both primary and secondary PI remains unknown.

There is currently no consensus on the appropriate management of PI, although many mechanical, bacterial, and pulmonary hypotheses have been proposed regarding PI etiopathogenesis, and its management can be challenging for surgeons[17]. Many studies have investigated the use of risk factors as predictors of a compromised bowel and the probable need for surgery, such as patient age and the presence of hypotension, peritonitis, renal failure, or serum lactate levels[18]. Other studies have attempted to create algorithms for PI management that, while helpful, are also tedious and may be difficult to apply clinically in circumstances where the patient requires rapid evaluation[19]. In this case, we performed urgent laparotomy because he had intestinal obstruction due to intussusception and several inflammatory symptoms.

Although the course of primary PI may be benign or may not frequently result in a need for urgent surgery, laparotomy should be considered in cases with intestinal obstruction due to intussusception. To our knowledge, 9 reported cases, including the present case, had intussusception associated with primary PI (Table 1)[20-27]. The mean patient age was 19.0 ± 16.0 years (range 0-48 years), which is younger than has been reported previously[19]. It is notable that more than 44% of these patients received an immunosuppressive drug, and few patients appeared to have intestinal ischemia. All patients had the intussusception in the colon, particularly on its right side. Five out of 9 patients had co-morbidities, most of which required an immunosuppressive drug. With the exception of the article placed as reference number 22, no other articles referred to the presence of portal venous gas, which often indicates ischemic bowel disease. Only 2 cases experienced successful reduction of the intussusception with barium enema[22,23], although long-term outcomes were not reported. Among the 4 cases with initial successful reduction of the intussusception by colonoscopy or barium enema, all of these patients eventually needed surgery: One case had persistent abdominal pain after solution of intussusception; in one case, the intussusception could not be completely resolved; one case had bowel obstruction; and for one case the indication of surgery was not clearly stated. For the present case, we performed an urgent laparotomy because of bowel obstruction with intussusception of the ascending colon. Right hemicolectomy for this case was performed because of the possibility of there being another polyposis disease present and because relapse of the intussusception could not be ruled out.

Table 1 Reported cases that had intussusception associated with primary pneumatosis intestinalis.
Ref.YearSexImmunosuppressive drugIschemiaPVGSiteTreatmentIndication of surgeryCo-morbidity
Nagata et al[20]23 yrMaleNoNoNoA/CCS→SurgeryAbdominal painNone
Emanuel et al[21]48 yrMaleNRNRNRD/CSurgeryObstructionHybrid perineurioma-schwannoma
Sugita et al[22]5 yrFemaleYesNoYesA/CBE-CML
Stern et al[23]32 yrMaleNoNoNoA/CBE-None
Morrison et al[24]3 moNRYesYesNRT/CBE→Surgerycould not resolved intussusceptionPeter's anomaly
Dubinsky et al[25]1 yrMaleYesNRNRA/CSurgeryObstructionCrohn's disease
Navarro et al[26]13 yrMaleNoNoNRT/CBE→SurgeryObstructionNone
Ahrar et al[27]29 yrMaleNoNoNRA/CBE→SurgeryNRNone
Our case20 yrMaleYesNoNoA/CSurgeryObstructionMPGN

In conclusion, although primary PI often represents a benign condition that should not be considered as an argument for surgery, emergent laparotomy should be considered for cases with intussusception, obstruction, and unsuccessful resolution of intussusception by colonoscopy or barium enema.

COMMENTS
Case characteristics

A 20-year-old man presented our hospital with a 3-d history of intermittent lower abdominal pain.

Clinical diagnosis

The authors performed an urgent laparotomy under the diagnosis of acute abdomen with obstructing intussusception.

Differential diagnosis

A right hemicolectomy was performed because other polyposis diseases or intussusception relapse could not be ruled out.

Laboratory diagnosis

All serum levels tested were within the normal range, with the exception of serum total bilirubin (1.5 mg/dL; normal range, 0.3-1.2 mg/dL). White blood cells (WBCs) (21000/μL), hemoglobin concentration (17.1 g/dL), and the C-reactive protein concentration (0.5 mg/dL) were also elevated.

Imaging diagnosis

Computed tomography revealed intussusception of the ascending colon with air within the wall.

Pathological diagnosis

A gross examination showed that the mucosa of the resected colon appeared normal with no evidence of ulceration or ulcer-related lesions, but instead a number of soft, yellowish cystic masses, suggesting that to be pneumatosis cystoides intestinalis.

Treatment

A right hemicolectomy was performed because other polyposis diseases or intussusception relapse could not be ruled out, and to help make a final pathological diagnosis.

Related reports

To our knowledge, 9 reported cases, including the present case, had intussusception associated with primary pneumatosis cystoides intestinalis.

Experiences and lessons

Although primary pneumatosis intestinalis (PI) often represents a benign condition that should not be considered as an argument for surgery, emergent laparotomy should be considered for cases with intussusception, obstruction, and unsuccessful resolution of intussusception by colonoscopy or barium enema.

Peer-review

This is an interesting article summarising a case of PI and intususception with a review of the cases in the literature.

Footnotes

P- Reviewer: Lee HC, Sodergren MH S- Editor: Ji FF L- Editor: A E- Editor: Wu HL

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