<strong>June</strong> 20<strong>09</strong>KUWAIT MEDICAL JOURNAL 143Case ReportPneumatosis Intestinalis of Small Bowelin an Adult: A Case ReportRajan Arora 1 , Amany Abd El-Hameed 1 , Obaid Al Harbi 21Department of Pathology and 2 Department of Surgery, Al Farwaniya Hospital, KuwaitABSTRACTKuwait Medical Journal 20<strong>09</strong>; <strong>41</strong> (2): 143-145Pneumatosis Intestinalis (PI) is rare in adults although itcan be seen in the pediatric population as a complicationof necrotizing enterocolitis. We report a case of PI affectingthe small bowel in a 27-year-old patient who presentedwith signs and symptoms of acute abdomen due toperforated duodenal ulcer. Histopathologic findings aredemonstrated and the pathogenesis is discussed with theobjective of highlighting that PI is not a diagnosis but afinding which needs further evaluation and managementin view of the underlying etiology.KEY WORDS: gastrointestinal, necrotizing enterocolitis, small bowelINTRODUCTIONPneumatosis intestinalis (PI) defined as gaswithin the wall of gastrointestinal (GI) tract, isnot a diagnosis but a physical or radiologicalfinding. It is secondary to an underlying diseaseprocess in 85% of cases which include obstructivepulmonary disease [1] , cystic fibrosis [2] , obstructiveGI disease (e.g., volvulus [3] and intussusception [4] ),gastroduodenal ulcer, ulcerative colitis, necrotizingenterocolitis [5,6] , acquired immunodeficiencysyndrome, and trauma [7] . Secondary form typicallyinvolves the small bowel but may occur throughoutthe GI tract. Since the etiology is so varied, the courseand outcome varies from benign self resolving tofatal depending on the underlying disease process.The primary form which accounts for 15%of cases is a benign idiopathic condition usuallyaffecting the colon [8] . Histopathologic findingsin both are usually the same but pathogenesisis complex depending on interaction of manyfactors like mucosal integrity [5] , intraluminalpressure [7] , bacterial flora [9] , and intraluminalgas [10] . Management depends on treating theunderlying cause and surgery is indicated whenacute complications appear such as perforationand peritonitis.CASE REPORTClinical findingsA 27-year-old male was admitted to surgicalcasualty with signs and symptoms of acuteabdomen. Radiological investigations revealed gasunder the diaphragm (indicative of perforation)as well as air in relation to bowel wall on plainradiograph. Computerized tomographic scan couldnot be done due to the nature of the emergency, andlaparotomy was performed.Operative findingsA perforated duodenal ulcer was found whichwas closed by patch omentoplasty. The terminal180 cm of the small bowel all the way to theileocecal junction showed multiple thin walled,tense, air filled cysts on the serosal surface (fewwere perforated and collapsed). The cecum andcolon were not involved. The small bowel proximalto the involved portion was distended. A limitedright colectomy and resection of the involvedsmall bowel was done. After resection, the patientwas managed in the intensive care unit and wasdischarged from the hospital after 10 days. He is onfollow up and is in good health till date.Pathological findingsGross features: The specimen (comprising ofterminal ileum, cecum with appendix, and partof ascending colon) was received in 10% neutralbuffered formalin. It was floating in the containerdue to buoyancy of the air. Outer surface of wholelength of small bowel (180 cm) showed congestionand multiple cysts in grape like manner (Fig. 1).On cut section, the mucosa showed cobblestoneAddress correspondence to:Dr. Rajan Arora, Senior Registrar, Department of Pathology, Farwaniya Hospital, PO Box : 18373, Farwaniya 81004, Kuwait. (F) 0<strong>09</strong>65-4893078, E-mail: arorarajan73@rediffmail.com, drrajarora@yahoo.com
144Pneumatosis Intestinalis of Small Bowel in an Adult: A Case Report<strong>June</strong> 20<strong>09</strong>Fig. 1: Outer surface of small bowel showing multiple air filledcysts (pneumocysts) in grape like mannerFig. 2: Mucosal surface showing “cobblestone” appearanceFig. 3: Pneumocysts in the bowel wallappearance (Fig. 2) with numerous air filled cysts(pneumocysts) in the wall (Fig. 3). The cysts variedin size from 0.2 to 2 cm in diameter. No mass,polyp, diverticula, volvulus, or intussuception wasidentified.Microscopic features: The cysts identifiedgrosslyin small bowel were located in the submucosal(Fig. 4), and subserosal region. They were linedby mostly multinucleated giant cells. No truelining epithelium was seen. There were no featuresof any granulomatous inflammation, necrosis,inflammatory bowel disease, or malignancy.Moderate to severe serositis was observed. Specialstains (Periodic acid-Schiff, Grocott’s methaminesilver, and Gram’s stain) for micro-<strong>org</strong>anisms werenegative.DISCUSSIONPI is rare and preliminary diagnosis depends onclinical and radiological findings. Computerizedtomographic scan is the best imaging modalityFig. 4: Photomicrograph showing submucosal air-filled cysts(H&E, x 40)although plain radiograph also shows characteristicfindings. The pathogenesis has been debated foryears and various explanations have been suggestedby various authors [5,7,9,10] . However the spectrum ofdiseases which underlie the development of thesecysts point toward a multifaceted phenomenon.Two most crucial considerations are: (a) from wherethe gas came, and (b) how it got into the bowel wall.Three possible sources of bowel gas are intraluminalgas, bacterial production of gas, and pulmonary gas.Intraluminal gas can leak to the bowel wall due toincreased intraluminal pressure and mucosal injury,either of them occurring singly [5,6] , or together invarious conditions, e.g., GI obstruction, and ulcerativecolitis [3-5,10] . Bacterial production of gas has beensuggested as an inciting factor for PI and is supportedby the fact that gas disappears after antimicrobialdrug treatment [9] . The original theory of pulmonarygas leaking as a result of alveolar rupture leadingto dissection along vascular planes to mediastinumand then tracing caudally to retroperitoneum