Idiopathic Chronic Pneumatosis Cystoids Intestinalis with Benign Pneumoperitoneum: A Rare Endoscopic Finding

Endoscopic Snapsho GE Po J Gas oen e ol 2022;29:209–211 Idiopa hic Ch onic Pneuma osis Cys oids In es inalis wi h Benign Pneumope i oneum: A Ra e Endoscopic Finding Cláudia Macedo a Elisa G a i o-Soa es a, b Ma a G a i o-Soa es a, b Ped o Ama o a Rui Cae ano-Oli ei a c Ped o Figuei edo a, b a Gas oen e ology Depa men , Cen o Hospi ala e Uni e si á io de Coimb a, Coimb a, Po ugal; b Facul y o Medicine, Uni e si y o Coimb a, Coimb a, Po ugal; c Pa hology Depa men , Cen o Hospi ala e Uni e si á io de Coimb a, Coimb a, Po ugal Recei ed: No embe 22, 2020 Accep ed: Janua y 21, 2021 Published online: Ma ch 23, 2021 Cláudia Macedo Gas oen e ology Depa men Cen o Hospi ala e Uni e si á io de Coimb a P ace a P o . Mo a Pin o, PT–3000-075 Coimb a (Po ugal) claudia.macedo.07 @ ho mail.com © 2021 Sociedade Po uguesa de Gas en e ologia Published by S. Ka ge AG, Basel ka ge @ka ge .com www.ka ge .com/pjg DOI: 10.1159/000514723 Keywo ds Pneuma osis cys oids in es inalis · Colon · Endoscopy · Idiopa hic · Pneumope i oneum Pneuma ose cís ica in es inal c ónica idiopá ica com pneumope i oneu benigno: um achado endoscópico a o Pala as Cha e Pneuma ose cís ica in es inal · Cólon · Endoscopia · Idiopá ica · Pneumope i oneu A 51-yea -old man, o me smoke (20 pack-yea s), wi h hemo hoidal disease, i i able bowel synd ome, pulmona y emphysema and a mechanical p os he ic ao - ic al e unde wa a in, p esen ed o pos -polypec omy su eillance colonoscopy. He complained o equen e- nesmus, dia hea (2–3 liquid dejec ions in he mo ning), bloa ing and abdominal discom o . Se e al polypoid g ape-like submucosal masses we e iden i ied in a 10-cm segmen o he descenden colon (Fig.1a, b). Since he le- sions we e easily inden ed wi h gen le p essu e and had a bluish hue, pneuma osis cys oids in es inalis (PCI) was suspec ed. Biopsy o one o hese masses caused immedi- a e de la ion, con i ming he diagnosis. His opa hologi- cal e alua ion showed no mal c yp a chi ec u e and a conges i e edema ous lamina p op ia wi h minimal in- lamma o y in il a e sugges ing eac i e changes (Fig.2). The pa ien had no his o y o abdominal auma and no pha macological (alpha-glucosidase inhibi o s, s e oids) o in ec ious (Clos idioides di icile, ube culosis, human immunode iciency in ec ion) causes. O e a long- e m ollow-up o 12 yea s, he mild symp oms we e managed conse a i ely wi h lope amide, spasmoly ics and p obi- o ics, which showed an imp o emen . In his pe iod, wo su eillance colonoscopies we e pe o med showing he same unchanged endoscopic indings desc ibed abo e. Recen ly, due o su eillance o an ascending ao ic aneu- ysm, a con as ed ches compu ed omog aphy (CT) iden i ied a small pneumope i oneum in he le sub- ph enic egion. Abdominal CT ex ension showed mo e ee pe i oneal ai in he le hypochond ium and ai bub- bles adjacen o he lumen o he bowel, some o hem be- ing subse osal (Fig.3a, b). The p e alence o PCI is 0.03% in he gene al popula- ion and i s pa hogenesis is poo ly unde s ood [1]. PCI is cha ac e ized by he p esence o gas, o ming mul ilocu- This a icle is licensed unde he C ea i e Commons A ibu ion- NonComme cial-NoDe i a i es 4.0 In e na ional License (CC BY- NC-ND) (h p://www.ka ge .com/Se ices/OpenAccessLicense). Usage and dis ibu ion o comme cial pu poses as well as any dis- ibu ion o modi ied ma e ial equi es w i en pe mission. Macedo e al. GE Po J Gas oen e ol 2022;29:209–211 210 DOI: 10.1159/000514723 la cys s, in he in es inal wall anging om an inciden al inding o a li e- h ea ening in a-abdominal condi ion [1, 2]. In 85% o cases, PCI is seconda y o se e al condi- ions including immunological dis u bances, in ec ions, pulmona y diso de s and diseases a ec ing gas oin es i- nal mo ili y [3, 4]. In hese cases, he main symp oms and also ea men s a e ela ed o he p ima y disease. In he emaining 15%, PCI is idiopa hic [3]. Mos pa ien s a e asymp oma ic o paucisymp oma ic and do no need ea men [1, 2]. When symp oma ic, PCI can p esen ab ab Fig. 1. a, b Colonoscopy showing polypoid g ape-like submucosal masses in he descenden colon. Fig. 2. Pa hological indings wi h hema ox- ylin and eosin s ain (×40): no mal c yp a - chi ec u e and a conges i e edema ous lamina p op ia wi h minimal in lamma o- y in il a e sugges ing eac i e changes. Fig. 3. a, b Abdominopel ic CT scan, axial sec ion (a) and co onal sec ion (b). Pneu- mope i oneum and ai bubbles adjacen o he lumen o he bowel (whi e a ows), some o hem in subse osal loca ion (yel- low a ow). Idiopa hic Ch onic wi h Benign Pneumope i oneum 211 GE Po J Gas oen e ol 2022;29:209–211 DOI: 10.1159/000514723 wi h dia hea, hema ochezia, abdominal pain and/o dis- en ion, cons ipa ion, la ulence, loss o appe i e and e- nesmus [1, 2]. An ibio ics, elemen al die as well as inha- la ional and hype ba ic oxygen he apy ha e been used, al hough he e idence o suppo hei e icacy is limi ed and he ecu ence a e is high. The diagnosis o PCI is based on endoscopy o imageology [1, 2]. Endoscopically, cys s ha e a pale o bluish appea ance and a y in size om a ew millime e s o se e al cen ime e s, and when biopsied, hey can apidly de la e wi h an audible hiss [1, 5]. Cha ac e is ic indings on abdominal CT a e collec- ions o ai adjacen o bowel lumen, wi h subse osal cys s commonly seen in small in es inal pneuma osis, bu ee pe i oneal ai has a ely been epo ed [1]. Complica ions associa ed wi h PCI occu in app oxima ely 16.3% o cas- es and include in es inal obs uc ion o in es inal pe o a- ion [2]. This case highligh s PCI as a benign en i y and ocus- es on he ole o pa ien s’ clinical assessmen in he man- agemen . I also emphasizes PCI as a cause o benign pneumope i oneum. Al hough he pa ien did no mee he diagnos ic c i e ia o ch onic obs uc i e pulmona y disease ha has been associa ed wi h PCI, we ecognize he po en ial ole o pulmona y emphysema in he e iol- ogy. S a emen o E hics W i en in o med consen was gi en by he pa ien ha au ho- ized publica ion o he case, including images. Con lic o In e es S a emen The au ho s ha e no con lic s o in e es o decla e. Funding Sou ces No unding was ecei ed. Au ho Con ibu ions Cláudia Macedo was esponsible o he da a acquisi ion and edi ing, manusc ip w i ing and e iewed he li e a u e. Ped o Ama o was esponsible o he da a acquisi ion and e iewed he manusc ip . Rui Cae ano-Oli ei a was esponsible o he da a ac- quisi ion. Elisa G a i o-Soa es, Ma a G a i o-Soa es and Ped o Figuei edo e iewed he manusc ip . All au ho s app o ed he published e sion o he manusc ip and ag eed o be accoun able o all aspec s o he wo k, ensu ing ques ions ela ed o he accu- acy o in eg i y o any pa o he wo k we e app op ia ely e alu- a ed and esol ed. Re e ences 1 Heng Y, Schu le MD, Haggi RC, Roh mann CA. Pneuma osis in es inalis: a e iew. Am J Gas oen e ol. 1995 Oc ; 90(10): 1747–58. 2 Wu LL, Yang YS, Dou Y, Liu QS. A sys em- a ic analysis o pneuma osis cys oids in es i- nalis. Wo ld J Gas oen e ol. 2013 Aug; 19(30): 4973–8. 3 Koss LG. Abdominal gas cys s (pneuma osis cys oides in es ino um hominis); an analysis wi h a epo o a case and a c i ical e iew o he li e a u e. AMA A ch Pa hol. 1952 Jun; 53(6): 523–49. 4 Lommen MJ, Zineldine O, Meh a TI, Rad ke LE, Se ano O. Pneuma osis Cys oides In es- inalis Iden i ied on Sc eening Colonoscopy Wi h Associa ed Pneumope i oneum. Cu e- us. 2020 Aug; 12(8):e9512. 5 Miwa W, Hi a suka T, Sa o K, Ka o Y. Pneu- ma osis cys oides in es inalis lesions chang- ing in o yellowish plaque-like elas osis lesions du ing healing. Clin J Gas oen e ol. 2020 Dec; 13(6): 1165–72.