lntemational Journal of Research in Medical SciencesAgale SV et al. Int J Res Med Sci. 201 5 Sep;3(9):xxx-xxxwww.msjonline.org pISSN 2320-607 1 I eISSN 2320-6012

Case ReportDOI: http://dx.doi.orgl 10.18203 12320-60 1 2.ijrms20 I 5000 I

Pneumatosis intestinalis in an adult: a report of an unusual case

Shubhangi vinayah Agale*, Bhushan Malhari warpe, Arvind Valand, Geeta Kumari

Department of Pathology Grant Government Medical College, Mumbai, Maharashtra, India

Received: 15 july 2015Accepted: 11 August 2015

*Correspondence:

Dr. Shubhangi Vinayak Agale,E-mail : shubhagale@hotrnail.com

Copyright: @ the audtor(s), publisher and licensee Medip Academy. This is an open-access article distributed underthe terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercialuse, distribution, and reproduction in any medium, provided the original work is properly cited.

AI'STRACT

Pneumatosis intestinalis (PI) is a rare condition affecting 0.03-0.37Vc of general population. This entity has variedpresen[ation which ranges from asymptomatic to surgical emergency in the form of acute abdonren. The acuteabdomen presentatioll can be bowel necrosis. perforation or persistent bowel obstruction. We present a case of pI ofsmall intestine in an adult patient admitted in emergency department who presented with acute ibdomen and pneumo-peritoneum.

I(eylvords: PneumrLtosis (cystoides) intestilalis, Pneumatosis coli, Emphysematous gastroenteritis

INTROI)UCTION

Pneunntosis intestinalis (PI) of the srnall bowel is a raredisease charac,terized by prcsence of gaseous cysts in thebowel wall.r-s PI was first describecl by Du Vermnoy in1730 in autopsy findings.3 The incidence of 0.037o hasgoue up to 0.37V0 due to accurate diagnosis oncomputerized tomography scan.3'a PI can be classified asprimary (idiopathic) and secondary based on the absenceor presence of etiological factors.3-5

Primary PI accounts for.|S%o of cases and commonlyinvolves the sub-mucosal layer of colon, whereassecondary PI accounts for 85Vo of cases which involvesth,) sub-serosal layer of small intestines, and it is seen inass<.rciation with necrotic, non-necrotic gastro-intestinalor pulrnonary diseases.3,a The secondary PI rvas describedby Koss in 1952 after analyzing 213 pathologicalspecirnens.s

We prcsent a case of pneulnatosis intestinalis of smallintestine with acute abdomen and pneumo-peritoneum inan adult patient admitted in emergency department.

CASBREPORT

A 45-year-old male who was a non-smoker and non-alcoholic presented in the emergency wirh acuteabdomen. He also had complaints of recurent abdominalpain and vomiting since last seven months. Theabdominal pain was crampy and diffuses wittr no clearlocalization in the abdomen and had no clear relationshipwith meals or evacuation. Bowel habit was notcharacterized by diarrhoea but by constipation since fivemonths. There was no history of bleeding per rectum.There was no history of diabetes mellitus, tuberculosisand bronchial asthma.

General physical examination did not reveal pallor,edema, lymphadenopathy or icterus. Blood pressure was-100/70 mm Hg, pulse rate-7S rpm, respiratory rate-l3cycles per minute. The baseline investigations revealed:Hb-15.7 g/dl, Total white-blood cell counr-13,800 /cumm, Differential leukocyte counr: N-787o, L-20Vo,E-lVo,M-17o, Random blood sugar level-109 mg/di, Amylase-66 Un, Serum electrolytes: sodium-137 rnEq/I,potassium-l.4 mEq/I, Serum trea-29 mg7o, Serumcreatinine- 0.8 mgVo, HIV-I & II and HBsAg-nonreactive. On per abdominal examination, the abdornenwas distended with increased bowel sounds with

IntemationalJournalofResearchinMedicalscienceslseptember20l5lvol3llssueg pagel

tendemess over the umbilical area and no palpable mass.Plain X-ray abdomen showed features of intestinalobstruction. CT scan of abdomen showed multiple extra-luminal intra-peritoneal free air foci suggestive ofpneumo-peritoneum. However no lead point or abnormaldilatation of proximal bowel loops was seen suggestive oftransient intussusception (FigudC)lr-andJ-&), As theclinical diagnosis was acute irLKnal obstruction, thepatient underwent exploratory laparotomy.

On gross examination numerous grape-like variably-sizedcysts filled with air (gas) were seen involving the serosaof the resected part of ileum. Cysts ranged in size from0.5 to 1.5 cms in diameter (Figuffi$q Crepitus couldalso be elicited while handling the$,Limen. Cut sectionshowed no such cysts in the mucosa and submucosa butattenuated mucosa 3 cm and 6 cm away from one end ofthe resected ileal segment. The intestinal obstruction wasseen due to reduced intestinal lumen--secondary tothickened wall and sub-serosal cysts (FigulQF,

On microscopic examination, the sections studiedrevealed mucosa, sub-mucosa, muscle coat and serosa(Figure 3a).The lamina propria showed mixedinflammatory infiltrate comprised of lymphocytes,plasma cells, neutrophils and eosinophils extending up tothe serosa. The muscularis propria showed vacuolardegeneration with gas dissecting through the muscularispropria separating the smooth muscle bundles. Serosashowed variable sized gas-filled cystic spaces lined byforeign-body type giant cells and mixed inflammatoryinfiltrate comprised of lymphocytes, plasma cells,neutrophils and eosinophils (Figure 3b). The diagnosis ofpneumatosis intestinalis was rendered based on the grossand microscopic findings.

Agate SV et al. Int J Res'Med Sci.20l5 Sep;3(9):xxx-xxx

li'ig lb

l'igure 2: (2a) Gross examination showed cystsranging in size from 0.5 to 1,5 cm in diameter filled

with air (gas) involving the serosa of the resected partofileurn. (2b) Cut section showerl reduced intestinallunren secondary to tlrickened lvall and sub-serosal

cysts (arrorvs).

Fig.lr }ig lh

Figure 3: (3a) Microscopic examination shorvetl' Iarnina propr[@[ sub-seiosal gas-lilled cyst is seen(II&E, X40). lisJt shows gas-filled cyst with arrowshowing f<rreign-body type giant cells (H&Itr, X100)

(3b) Sub serosa shorved gas-fillcd cystic space lined byforeign-body type giant cells (H&I,), X 400).

nigure {@leu,s microscopic examination showed laminapropria Srrlltrated by mixed inflamrnatory infiltratecomprised of lymphocytes, plasma cells, neutrophils andeosinophils extending upto the serosa. The muscularispropria showed vacuolar degeneration with gas dissectingthrough the muscularis propria separating the smoothmuscle bundles. A sub-serosal gas-filled cyst is seen(H&E, X40). Inset shows gas-filled cyst with anowshowing foreign-body type giant cells (H&E, Xl00).Figure 3b shows sub'serosa showed gas-filled cysticspace lined by foreign-body type giant cells (arrow) andmixed inflammatory infiltrate comprised of lymphocytes,plasma cells, neutrophils and eosinophils (H&E, X 400).

DISSCUSION

Pneumatosis intestinalis (PI) is an unusual conditionwhich may involve any portion of the gastro-intestinaltract from stomach to rectum. Most reports of pI describecontinuous portions of diseased bowel but there are fewcase reports of segmental involvement. The variousstudies have reported PI involving small intestine in 20Zo

Irig la

figuffi*-ls: CT scan of abdonren shorvingrnultiplKltra-luminal iutra-peritoneal l'ree air iirci

suggestive of pneunro-pelitoneurn.

Intemational Joumal of Research in Medical sciences I september 2015 I vol 3 I Issue 9 page 2

5

. i I .: .:

llg 2r

Agale SV et al. Int J Res Med Sci. 2015 Sep;3(9)::*x-m

to 51.6%, colon in 36% to 787o and both in 2 to 22%o.s-7

The synonyms for PI include pneumatosis intestinaliscoli, pneumatosis cystoides intestinalis, pneumatosisintestinorium cystoided hominus and a simple term'benign intre-intramual gas.' In P[ presence of intra-peritoneal free air and/ or retro-peritoneal air is veryuncommon with few reported cases.s

The etiopathogenesis of PI is uncertain and three maintheories have been suggested a:

l. Meclrunical tlrcory: The gaS in the intestine is pushedto the mucosal defect into lymphatic channels and isthen distributed distally by peristalsis. This mayhappen due to trauma, surgery, bowel obstruction orcolonoscopy.

2. Bacterial theory: The sub-mucosal localization ofClostridia and E. coli leads to production of gas

which is retained by sub-rnucosa and lymphaticchannels.

3. Puhnonary theory: The gas freed by the rupture ofalveoli travels through the mediastinurn into theretro-peritoneal space and then comes to the peri-vascular spaces into the intestinal wall.

Recently added theories are:2

4. Chemical theory / nutritional deficiency theory:5. PI associatgd with chemotherapy, hormonal therapy

and connective tissue disorders.

The pathological findings in resected specimen ofintestine also vary depending upon the location of the

cysts. The sub-nrucosal cysts which are seen in primaryPI have bubble-like appearance and in secondary PI, the

sub-serosal cysts are readily recognizable ou externalexamination as band-like continuous lines.l'eIvlicroscopically, the lesions of PI are characterized bypresence of multiple air filled cysts which arc devoid ofan endothelial lining. The surrounding tissue usuallyshows mixed inflammatory inftltrate of lymphocytes,neutrophils, plasma cells, macrophages and foreign-bodygiant cells. In cases where the giant cells and

inflammatory component is predominant, it has to be be

differentiated from Crohn's disease, tuberculosis and

other granulomatous inflammatory conditions of theintestind. The histological differential diagnoses of PIwhich should be considered are lymphangiectasia andpseudoJipomatosis.a

The most common presenting symptoms of Pl are

dianhea or constipation, bloody stools, abdominal pain,

flatus or weight loss. The complications of PI includepneumo-peritoneum, volvulus, intestinal obstruction,intussusception, haemorrhage and perforation.a

Pneumo-peritoneum usually represents a ruptured serosal

cyst which was seen in this case. Pneumo-peritoneum isevident in up to ll%o of cases with small bowelpneumatosis intestinalis and ZVo of those with large

bowel pneumatosis.ro Development of pneumo-peritoneum in absence of signs and symptoms ofperitoneal irritation is pathognomonic feature of PI.

CONCLUSION

This case highlights a rare and potentially ominouscomplication of pneumo-peritoneum in pneumatosisintestinalis.

Funding: NoneConflict of interest: None declaredEthical approval: Not required

ITEFERENCT]S

1. Benitto D, Crincoli R, Iacobellis F, Iasiello F,PizzaNL, Lassandro F, et al. Primary pneumatosisintestinalis of small bowel: a case of a rare disease.Case Rep Surg. 2014;l:1-4.

2. Wu LL, Yang YS, Dou Y, Liu QS. A systematicanalysis of pneumatosis cystoids intestinalis. WorldJ Gastroenterol . 20 13 :19 :497 3 -8.

3. Slesser AA, Patel PH, Das SC, l,eahy A,Livingstone J, Riaz AA. A rare case of segmentalsmall bowel pneumatosis intestinalis: A case report.Int J Surg Case Rep. 20ll:2r185-7.

4. Onder A, Kapan M, Onder H, Taqkesen F, Giil M,Aliosmanoplu I, et al. Pneumatosis CystoidesIntestinalis: Clinical Experience in a Single Center.Eur I Gen Med-20L2;9:27-32.

5. Arikanoglu Z, Aygen E, Camci C, Akbulut S,

Basbug M, Dogru O, Cetinkaya Z, Kirkil C.

Pneumatosis cystoides intestinalis: a single centerexperience. World I Gastroenterol. 2012;18:453-7.

6. Azzaroli F, Turco L, Ceroni L, Galloni SS,

Buonfiglioli F, Calvanese C, Mazzella G.Pneumatosis cystoides intestinalis. World J

Gastroenterol. 20ll ;17 :4932-6.7. Greenstein AJ, Nguyen SQ, Berlin A, Corona J, Lee

J, Wong E, et al. Pneumatosis intestinalis in adults:management, surgical indications, and risk factorsfor mortality. J Gastrointest Surg. 2007;11:1268:14.

8. Sakurai Y, Hikichi M, Isogaki J, Furuta S,

Sunagawa R, Inaba K, et al. Pneumatosis cystoidesintestinalis associated with massive free airmimicking perforated diffuse peritonitis. World J

Gastroenterol. 2008;14 :67 53 -6.9. Ochiai T, Igri K, Kumagai Y, Iida M, Yamazaki S.

Education and imaging. Gastrointestinal: massiveportal venous gas and pneumatosis intestinalis. J

Gastroenterol Hepatol. 2010;25:1178.10. Dawe N, Akhtar S. Pneumatosis intestinalis

presenting with a pneumoperitoneum in a patientwith chronic bronchiectasis: a delayed diagnosis ofsuperior mesenteric artery ischaemia. BMJ Case

Rep. 2010;5:20-1.

Cite this article as: Agale SV, Warpe BM, ValandA, Kumari G. Pneumatosis intestinalis in an adult: areport of an unusual case. Int J Res Med Sci2015;3(9):xxx-xx.

International Journal of Research in Medical Sciences I September 20 15 I Vol 3 I Issue 9 Page 3