Cecal duplication cysts are very rare including 0.4 of all GI tract duplications. The ultrasound (US) is the imaging of choice for duplication cysts detection in pediatrics. Cyst's wall is made up of an inner mucosal layer, which is echogenic and an outer muscular layer, which is hypoechoic at US (called â��pseudo kidneyâ�� appearance on longitudinal view or â��doughnutâ�� appearance on transverse view). Intussusception is one of the duplication cyst's complications. Intussusception presented with trans-anal protrusion (prolapsed intussusception) is a rare and confusing condition which can cause delayed diagnosis and further complications. We present an 18-month old boy with Cecal duplication cyst causing intussusception, which protruded from anus. Â© 2020 The Author

Alamdaran, S.A.

Darban Hosseini Amirkhiz, G.

Dehghanian, P.

Ghamari khameneh, A.

eprints Iran University of Medical Sciences

Journal of Pediatric Surgery Case Reports 54 (2020) 101388Available online 22 January 20202213-5766/© 2020 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license(http://creativecommons.org/licenses/by-nc-nd/4.0/).Contents lists available at ScienceDirect Journal of Pediatric Surgery Case Reports Cecal duplication cyst complicated by prolapsed ileocolic intussusception Afshar Ghamari khameneh MD a , Gisoo Darban Hosseini Amirkhiz MD b , * , Seyed Ali Alamdaran MD a , Paria Dehghanian MD c a Department of Radiology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran b Research Center for Prevention of Cardiovascular Disease, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran c Department of Pathology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran A R T I C L E  I N F O  Keywords : Cecal duplication cyst Ileocolic intussusception Prolapsed intussusception A B S T R A C T  Ce cal du pli ca tion cysts are very rare in clud ing 0.4% of all GI tract du pli ca tions. The ul tra sound (US) is the imag ing of choice for du pli ca tion cysts de tec tion in pe di atrics. Cys t's wall is made up of an in ner mu cosal layer, which is echogenic and an outer mus cu lar layer, which is hy poe choic at US (called “pseudo kid ney” ap - pear ance on lon gi tu di nal view or “dough nut” ap pear ance on trans verse view). In tus sus cep tion is one of the du pli ca tion cys t's com pli ca tions. In tus sus cep tion pre sented with trans - anal pro tru sion (pro lapsed in tus sus cep - tion) is a rare and con fus ing con di tion which can cause de layed di ag no sis and fur ther com pli ca tions. We pre - sent an 18 - month old boy with Ce cal du pli ca tion cyst caus ing in tus sus cep tion, which pro truded from anus. 1 . Introduction One of un com mon in her ited dis eases is en teric du pli ca tion cyst, its in ci dence is 1 per 4500 live births [ 1 ]. It is more com mon in boys un - der 2 years old [ 2 ]. It can be pre sent in all parts of al i men tary tract from the mouth to anus. The most preva lent site is the small bowel. In or der of fre quency, it oc curs in the ileum, je junum and duo de num [ 3 ]. Ce cum is one of the rarest lo ca tions. 2 In tus sus cep tion is a com pli - ca tion of en teric du pli ca tion cysts, which leads to bowel ob struc tion [ 1 ]. We pre sent an 18 - month old boy with Ce cal du pli ca tion cyst caus ing in tus sus cep tion, which was pro truded from anus. Each of Ce - cal du pli ca tion cyst and in tus sus cep tion pro lapse is ex tremely a rare en tity. This is the first re ported case of pro lapsed in tus sus cep tion of Ce cal du pli ca tion cyst. 2 . Case presentation The 18 - month old boy was brought to our emer gency de part ment with com plaints of colic ab dom i nal pain from 2 weeks ago, fever and mul ti ple episodes of non - bloody and non - bilious vom it ing from one day ago. This pa tient was ad mit ted to an other small gen eral hos pi tal the day be fore he had been re ferred to us. His re fer ral form men tioned that dur ing the ad mis sion pro tru sion of a mass from the anus oc - curred af ter defe ca tion and that they had per formed ab dominopelvic ul tra sound (US) for find ing the cause. There was an ileo colic in tus sus - cep tion ex tended to the rec tum and pro truded from the anus with no patho logic lead point; as ex pected ow ing to the fact that the pa tient was in 6_36 months age group in which most of the in tus sus cep tions oc cur id io path i cally. The pro lapse was man aged and re duced suc cess - fully be fore the pa tien t's re fer ral, but in tus sus cep tion had not re - sponded to re duc tion, and so the pa tient was re ferred to our pe di atric spe cial ized hos pi tal for fur ther in ves ti ga tions. On phys i cal ex am i na tion low grade fever was de tected. His ab - domen was dis tended, soft, and non tender. A pal pa ble left lower quad rant mass was found. His com plete blood count showed leuko cy - to sis (WBC = 14.7 * 10 9 /L, Poly mor phonu clear = 65%). The re main - der of the phys i cal ex am i na tion and lab data were un re mark able. US per formed in our hos pi tal showed ileo colic in tus sus cep tion. One large (37*28 mm), thick - walled cyst with a “dou ble lay ered” ap - pear ance was ob served in front of in tus sus cep tion which was act ing as a lead point ( Fig. 1 ). (video 1). Abbreviations : US , ultrasound , GI , gastrointestinal , NEC , necrotizing enterocolitis , DRE , Digital Rectal exam * Corresponding author. Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), No10, Firuzeh Alley, Vali - asr St., Tehran, 1593716615, Iran. E - mail address: Darbanhosseini. g@ iums. ac. ir (G. Darban Hosseini Amirkhiz). https://doi.org/10.1016/j.epsc.2020.101388 Received 11 January 2020; Accepted 18 January 2020 Journal of Pediatric Surgery Case Reports 54 (2020) 1013882A. Ghamari khameneh et al. Fig. 1 . Large, thick - walled cyst with dou ble lay ered ap pear ance and mu ci nous con tent in an 18 - month old boy's ul tra sound. Sup ple men tary video re lated to this ar ti cle can be found at https:// doi. org/ 10. 1016/ j. epsc. 2020. 101388 . The pa tient got ready for op er a tion and then ab domen opened with a trans verse in ci sion above the um bili cus. There was re ac tive fluid within ab domen and ileo colic in tus sus cep tion ex tend ing to the rec tum. Af ter in tus sus cep tion re duc tion, a mass was pal pated in the ce cum (lead point) and then ileo ce cal re sec tion and end to end anas - to mo sis per formed. Post op er a tive pe riod was un event ful di ag no sis af - ter patho logic ex am i na tion was a 40*30 mm mucin filled du pli ca tion cyst orig i nat ing from ce cum and ex tend ing to the ileo ce cal valve ( Fig. 2 ). 3 . Discussion Ce cal du pli ca tion cysts are very rare, in clud ing 0.4% of all GI tract du pli ca tions. 2 They can be found in ci den tally. Half of the Ce cal du pli - ca tion cysts are cor rectly di ag nosed pre op er a tively [ 2 ]. Our pre op er - a tive ul tra sound showed its ori gin cor rectly. Ap pen dic u lar mass, tu - mor and necro tiz ing en te ro col i tis (NEC) are its dif fer en tial di ag noses [ 2 ]. The clin i cal pre sen ta tion is mainly con tin gent upon the size and site. Small cysts usu ally pre sent in the role of lead ing point for in tus - sus cep tion or volvu lus. Big ger cysts can cause a com pres sive ef fect, which leads to GI tract ob struc tion or is chemia [ 3 ]. His to log i cally have cys tic struc tures and share their mus cu lar wall and blood sup ply with the ad ja cent in tes tine [ 3 ]. Amount of mucin in Ce cal du pli ca tion cyst is re lated to du ra tion of ob struc tion. In this case cys t's mu cosa was lined by mu ci nous ep ithe lium and cys t's lu men was par tially loaded by mucin. The US is the imag ing of choice for du pli ca tion cysts de tec tion in pe di atrics. Its speci ficity is re ported as high as 95% and its pos i tive pre dic tive value is 85 – 100% [ 4 ]. The colonic du pli ca tion cysts have spher i cal (> 80% of cases) or tubu lar ap pear ance [ 5 ]. Cys t's wall is made up of an in ner mu cosal layer, which is echogenic and an outer mus cu lar layer, which is hy poe choic at US (called “pseudo kid ney” ap pear ance on lon gi tu di nal view or “dough nut” ap pear ance on trans - verse view) [ 6 ]. The twofold lay ered wall ap pear ance is demon strated in more than half of the cases [ 2 ]. In tus sus cep tion is one of the du pli ca tion cysts' com pli ca tions, which is an in vagi na tion of a part of GI tract into the more dis tal part. They are usu ally id io pathic in pe di atric pa tients but can some times Fig. 2 . A. gross pathol ogy (uniloc u lar in tra mural Ce cal cyst) B. True du pli ca tion cyst of Ce cum with sep a rate mus cu laris pro pria (H&E stain ing, HPF) C. Du pli ca - tion cyst mu cosa lined by mu ci nous ep ithe lium (H&E stain ing, HPF). Journal of Pediatric Surgery Case Reports 54 (2020) 1013883A. Ghamari khameneh et al. have lead points (2.2 – 15%) [ 7 ]. The most com mon lead points in chil - dren are Meck el's di ver tic u lum, Du pli ca tion cyst and in testi nal polyps [ 7 ]. Timely di ag no sis of these sec ondary in tus sus cep tions is very im - por tant due to their po ten tial to cause com pli ca tions and need for surgery [ 5 ]. In tus sus cep tion pre sented with trans - anal pro tru sion (pro lapsed in - tus sus cep tion) is a rare and con fus ing con di tion, which usu ally leads to de layed di ag no sis, high mor bid ity and mor tal ity [ 8 ]. Most of pre vi - ous case re ports are from low and mid dle in come African and Asian coun tries and ne glected cases [ 8 ]. It is usu ally con fused with rec tal pro lapse in the first clin i cal en counter as our case. Af ter the lack of im prove ment with con ser v a tive man age ment of rec tal pro lapse, fur - ther ex am i na tion leads to the di ag no sis of in tus sus cep tion with trans - anal pro tru sion. 8 Pro laps ing rec tal mass with in testi nal ob struc tion is also an other clin i cal clue; Al though in one third of cases it can hap pen with out typ i cal pre sen ta tions of in tus sus cep tion. Dig i tal Rec tal exam (DRE) helps to dif fer en ti ate rec tal pro lapse from in tus sus cep tion pro - lapse. In DRE of in tus sus cep tion pro lapse, the fin ger can in sert par al - lel to in tus sus cepted bowel and the anorec tal is fixed in its po si tion [ 8 ]. Also, us ing the US can help dif fer en ti at ing these two en ti ties. 4 . Conclusion De spite Ce cal du pli ca tion cyst rar ity, it is eas ily di ag nosed by clin i - cal sus pi cion and ul tra sound. Thick - walled cyst or dou ble - layered ap - pear ance is the most im por tant ul tra sound find ing. Quick de tec tion and man age ment is es sen tial in or der to im prove the post - op out - comes; so it is im por tant to keep the di ag no sis in mind for the pa - tients with sim i lar pre sen ta tions. Consent to participate For mal writ ten con sent was ob tained from the pa tien t's le gal guardian (his fa ther in this case as the pa tient was un der 18). Pa tien t's fa ther agreed on pub li ca tion of this case re port and ac com pa ny ing im - ages. Consent for publication The pa tien t's fa ther has given oral and writ ten con sent for use of the ul tra sound and pathol ogy im ages and he is aware that the re port will be pub lished in a med ical jour nal which is dis trib uted world wide for ed u ca tional pur poses. Funding The au thors do not re ceive any fund ing. Declaration of competing interest The au thors de clare no com pet ing in ter est. This study was done with no spe cific fund ing or non - funding sup - port. The au thors have no con flicts of in ter est rel e vant to this ar ti cle to dis close. Acknowledgements not ap plic a ble to this ar ti cle. Appendix A . Supplementary data Sup ple men tary data to this ar ti cle can be found on line at https:// doi. org/ 10. 1016/ j. epsc. 2020. 101388 . References [1] Puligandla PS , Nguyen LT , St - Vil D , et al . Gastrointestinal duplications . J Pediatr Surg 2003 ; 38 ( 5 ) : 740 – 4 . [2] Temiz A , Oguzkurt P , Ezer SS , Ince E , Gezer HO , Hicsonmez A . Different clinical presentations, diagnostic difficulties, and management of cecal duplication . J Pediatr Surg 2013 ; 48 ( 3 ) : 550 – 4 . [3] Lai M , Coakley BA , Webber EM , Goldman RD . Intussusception with cecal cyst as lead point in a child . Pediatr Emerg Care 2018 ; 34 ( 7 ) : e134 – 5 . [4] Radhakrishna V , Rijhwani A , Jadhav B . Cecal duplication: a mimicker of intussusception: a case report and review . Ann Med Surg 2012 ; 31 : 17 – 9 . 2018 . [5] Karaca L , Pirimoglu B , Bayraktutan U , Oral A , Ogul H , Kantarci M . Cecocolic intussusception in a patient with a cecal duplication cyst . Turk J Gastroenterol: Off J Turkish Soc Gastroenterol 2015 ; 26 ( 3 ) : 281 – 2 . [6] Al - Qahtani HH . Enteric duplication cyst as a leading point for ileoileal intussusception in an adult: a rare cause of complete small intestinal obstruction . World J Gastrointest Surg 2016 ; 8 ( 6 ) : 472 – 5 . [7] Zhao L , Feng S , Wu P , Lai XH , Lv C , Chen G . Clinical characteristics and surgical outcome in children with intussusceptions secondary to pathologic lead points: retrospective study in a single institution . Pediatr Surg Int 2019 ; 35 ( 7 ) : 807 – 11 . [8] Ray A , Mandal KC , Shukla RM , Roy D , Mukhopadhyay B , Bhattacharya M . Neglected intussusception presenting as transanal prolapse of small bowel . Indian J Pediatr 2012 ; 79 ( 10 ) : 1370 – 1 . 

Cecal duplication cyst complicated by prolapsed ileocolic intussusception

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Cecal duplication cyst complicated by prolapsed ileocolic intussusception

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