Lymphangioma accounts for 5% of benign pediatric tumors and less than 1% is located in the abdomen. Indeed mesenteric cystic lymphangioma is a rare benign abdominal mass and its pathogenesis is still unknown.Mesenteric cystic lymphangioma is often diagnosed incidentally. Nevertheless, in some cases its clinical presentation can mimic several common surgical pathologies. Here we describe a case of mesenteric cystic lymphangioma mimicking an ileo-colic intussusception

Elisa Mina

Francesco Molinaro

Giulia Fusi

Mario Messina

Mirko Bertozzi

Mohamed Vincenti

Niccolò Nardi

Rossella Angotti

English

Open Access Repository

Journal of Pediatric Surgery Case Reports 57 (2020) 101461Available online 15 April 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 Mesenteric cystic lymphangioma mimicking an ileo - colic intussusception Elisa Mina a , * , Giulia Fusi a , Rossella Angotti a , Niccolò Nardi b , Mohamed Vincenti a , Mario Messina a , Francesco Molinaro a , Mirko Bertozzi b a Division of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Le Scotte Polyclinic Hospital, Viale Bracci 14, 53100, Siena, Italy b Pediatric Surgery Unit, Department of Surgical and Biomedical Sciences, University of Perugia, S. Maria della Misericordia Hospital, Piazzale Giorgio Menghini 1, 06129, Perugia, Italy A R T I C L E  I N F O  Keywords : Mesenteric cystic lymphangioma Lymphangioma Intussusception Children Emergency Surgery Pediatric surgery A B S T R A C T  Lym phan gioma ac counts for 5% of be nign pe di atric tu mors and less than 1% is lo cated in the ab domen. In - deed mesen teric cys tic lym phan gioma is a rare be nign ab dom i nal mass and its patho gen e sis is still un known. Mesen teric cys tic lym phan gioma is of ten di ag nosed in ci den tally. Nev er the less, in some cases its clin i cal pre sen ta tion can mimic sev eral com mon sur gi cal patholo gies. Here we de scribe a case of mesen teric cys tic lym phan gioma mim ic k ing an ileo - colic in tus sus cep tion. 1 . Introduction Mesen teric cys tic lym phan gioma (MCL) is a rare be nign in tra - abdominal anom aly with un cer tain eti ol ogy, pre dom i nantly oc cur ring in chil dren [ 1 ]. In the pe di atric age group, males seem to be more af - fected than fe males [ 2 ]. The di ag no sis of MCL is of ten in ci den tal and al most 60% are de - tected be fore the fifth year of life [ 3 ]. Most MCL pa tients are ini tially asymp to matic, with vague ab dom i nal symp toms. Nev er the less, acute pre sen ta tion due to com pli ca tions may oc cur: tor sion, rup ture, he m or - rhage, volvu lus, com pres sion of ad ja cent or gans, in testi nal is chemia and mal ro ta tion are re ported in lit er a ture and they can be life - threatening [ 1 , 4 ]. The op ti mal treat ment for mesen teric cys tic lym phan gioma is a sur gi cal ap proach with re sec tion of the mass, some times in clud ing re - sec tion of the ad ja cent bowel to achieve com plete ex ci sion [ 4 ]. How ever, a com plete re sec tion of a MCL may be lim ited by the size and site of the le sion since this may re quire a too ex ten sive bowel re sec tion, re sult ing in a short bowel syn drome [ 2 , 5 ]. In this case re port we de scribe a unique pre sen ta tion of a MCL in chil dren and its suc cess ful sur gi cal treat ment. 1. 1 . Case report A 9 - month - old boy pre sented at our emer gency de part ment with crampy col ics. The phys i cal ex am i na tion re vealed ab dom i nal dis ten - sion, ab dom i nal ten der ness with guard ing and scarce bowel sounds. Ab dom i nal pal pa tion re sulted dif fi cult due to the child's un easi - ness. A lit tle amount of red jelly stool was seen in the di a per. Lab o ra - tory re sults were nor mal. Ul tra sonog ra phy was car ried out for fur ther di ag no sis show ing mul ti ple fluid - filled bowel loops with out a clear vi su al iza tion of the ap pen dix or signs of in tus sus cep tion. A “wait - and - see” pol icy was fol - lowed in deed. Af ter 4 hours of ob ser va tion the pa tient pre sented with vom it ing and the ab dom i nal col icky pain got worse. For these rea sons we per formed a bar ium en ema with a sus pi cion of an in ter mit tent ileo - colic in tus sus cep tion. The bar ium en ema showed a stop in the con trast pro gres sion at the he patic flex ure level ap par ently due to an in tus sus cep tion ( Fig. 1 A and B). Be cause of the failed res o lu tion of the sus pected in tus sus cep tion at the bar ium en ema and the wors ened symp to ma tol ogy, we de cided to per form a sur gi cal ex plo ration. * Corresponding author. E - mail addresses: elisamina. mc@ gmail. com (E. Mina), gf. giuliafusi@ gmail. com (G. Fusi), rossella. angotti@ ao - siena. toscana. it (R. Angotti), chirurgia. pediatrica@ ospedale. perugia. it (N. Nardi), dr. mohamed. vincenti@ gmail. com (M. Vincenti), mario. messina@ unisi. it (M. Messina), francesco. molinaro@ ao - siena. toscana. it (F. Molinaro), mirkobertozzi@ hotmail. com (M. Bertozzi). https://doi.org/10.1016/j.epsc.2020.101461 Received 28 February 2020; Received in revised form 27 March 2020; Accepted 4 April 2020 Journal of Pediatric Surgery Case Reports 57 (2020) 1014612E. Mina et al. Fig. 1 . A - B. Bar ium en ema: Pos tero - anterior view show ing the stop of the con - trast in the right colic flex ure. B. Bar ium en ema: An tero - posterior view show - ing the same stop of the con trast. Sur pris ingly, la paro tomy showed a big mul ti cys tic mass start ing from the mesen tery, tightly ad her ent to the right colon and wrap ping - around it ( Fig. 2 ). The size of the mul ti cys tic mass was ap prox i mately 16x14x10 cm. In or der to pre serve the en tire right colon and the ileo - cecal valve to gether with the last ileal loop, a sur gi cal enu cle ation of the mass was tried avoid ing dam age of the mesen teric blood sup ply. In this way we re moved about the 95% of the mass. Com plete re sec tion was not pos si ble with out com pro mis ing the colon, be cause of dif fusely spread le sions in its mesen tery. Some very small cysts at the root of the mesen tery were only in cised and evac u ate be cause of the risk of ves sels dam age. The pa tient had an un event ful post - operative re cov ery and he was dis charged af ter 7 days. No ex pla na tion for rec tal bleed ing was found. Histopatho log i cal ex am i na tion of the spec i men re vealed a mesen teric cys tic lym phan gioma. Fol low - up by ul tra sonog ra phy was sched uled. Ul tra sound imag ing at 3, 6 and 12 months showed some very lit tle cysts at the root of the ileo - cecal mesen tery. At the 24 months ul ta sonog ra phy we ob served a dis ap pear ance of the cysts. The ab sence of the cysts was also con - firmed in the next three an nual sched uled ul tra sounds. 2 . Discussion MCL are rare with fewer than 200 de tailed re ports pub lished in the Eng lish - language lit er a ture [ 6 ]. Fig. 2 . B. MCL start ing from the colic mesen tery, tightly ad her ent to the right colon and wrap ping - around it. The eti ol ogy of MCL is un known, though some be lieve that it might be as so ci ated with de vel op men tal anom alies of the lym phat ics [ 7 ]. Other pos si ble eti olo gies in clude bleed ing or in flam ma tion in the lym phatic chan nels, both lead ing to ob struc tion and sub se quent lym - phan gioma for ma tion [ 8 – 10 ] as well as chronic in flam ma tion, neo - plasm, trauma and de gen er a tion of lymph nodes [ 11 ]. Pe di atric lym phan gioma ac counts for 5% of all be nign tu mors in chil dren and MCLs are re spon si ble for 1/ 20.000 hos pi tal ad mis sions in the pe di atric age group [ 12 ]. Among all lym phan giomas less than 1% af fect the mesen tery, greater omen tum, and retroperi toneum [ 1 , 7 , 13 ]. Among the in traab - dom i nal lo ca tions, the meso colon and the retroperi toneum are in - volved in 10% and 5% of cases re spec tively; the small bowel mesen - tery is more fre quently af fected [ 1 , 6 , 7 ]. In the pe di atric age group, males seem to be pre dom i nantly af - fected with a re ported male to fe male ra tio of 1.5:1 up to 3:1 [ 2 ]. MCL is of ten an in ci den tal find ing dur ing imag ing stud ies per - formed for un re lated clin i cal con di tions [ 1 ]. Most MCL pa tients are ini tially asymp to matic, with vague and ob scure ab dom i nal symp toms de pend ing on the cys t's size and lo ca tion [ 6 ]. Symp toms can be ab - dom i nal dis com fort, pain and ab dom i nal dis ten tion with or with out vom it ing [ 2 , 14 ]. Nev er the less, chil dren pre sent with acute com plaints more fre quently than adults [ 15 ]. Acute clin i cal pic tures re quir ing emer gency la paro tomy in clude trau matic rup ture, in tra cav i tary or in - traab dom i nal bleed ing, in testi nal ob struc tion with necro sis, in testi nal gan grene due to volvu lus, and cyst in farc tion [ 6 ]. If a MCL is sus pected, ul tra sonog ra phy, MRI and CT can pro vide a high pre op er a tive di ag nos tic ac cu racy [ 5 , 16 ]. When pos si ble, we pre - fer to per form ul tra sonog ra phy and MRI avoid ing the dis ad van tages of ir ra di a tion in the de vel op ing chil dren [ 17 , 18 ]. Nev er the less, in our case, the ab dom i nal ul tra sound in ves ti ga tion per formed at the emer gency de part ment was not di ag nos tic. This may be due to the fact that ul tra sonog ra phy is highly op er a tor - dependant and it is par tic u larly sus cep ti ble to er rors when per formed in emer - gency set tings by non - dedicated ra di ol o gists [ 19 ]. There fore, as in the case re ported by Clement et al. [ 1 ], the cysts were not iden ti fied and were even falsely in ter preted as fluid - filled bowel loops. For this rea son and for a sus pect of in ter mit tent ileo - colic in tus sus - cep tion a bar ium en ema was per formed. The clin i cal pic ture of acute ab domen, sup ported by the im ages of con trast en ema in ter preted as ileo - colic in tus sus cep tion, lead us to de cide for a sur gi cal ex plo ration. Whether or not the di ag no sis of MCL is ob tained be fore the in ter - ven tion the com plete sur gi cal re sec tion is in di cated, since this be nign tu mor tend to re cur and in vade neigh bor ing struc tures [ 13 ] and the risk of com pli ca tions in creases with size [ 16 ]. To tal re moval of a le sion that in vades po ten tially re sectable in - traab dom i nal struc tures such as bowel, spleen, or pan creas is pos si ble [ 20 ]. Nev er the less, high cau tion must be done dur ing surgery be cause ad he sions to vi tal struc tures such as ma jor ves sels and nerves can make re sec tion haz ardous or even im pos si ble and a too ex ten sive bowel re sec tion could re sult in a pos si ble short bowel syn drome [ 2 , 5 ]. In our case, we pre ferred to per form an ac cu rate enu cle ation of the mass in stead of a right colec tomy, be cause this pro ce dure should have in cluded the re moval of the as cend ing colon and the ileo - cecal valve with out any guar an tee of com plete ex ci sion of the lym phan gioma, since it was ex tended to the root of the mesen tery and to the retroperi toneum. There fore, we re moved about 95% of the mass and the re main ing mil li met ric cysts at the root of the mesen tery were only in cised an drained, even if in lit er a ture there is a lack of long - term re - sults with this pro ce dure [ 21 ]. In this way, al though the pro ce dure was not rad i cal, we spared the as cend ing colon and the ileo - cecal valve. In our pa tient, the 5 - year fol low - up tes ti fies to the suc cess of the sur gi cal pro ce dure per formed. Journal of Pediatric Surgery Case Reports 57 (2020) 1014613E. Mina et al. 3 . Conclusion MCL is a rare con di tion with a high per cent age of pos si ble mis di - ag no sis es pe cially in the case of pre sen ta tion with acute symp toms. Our case shows a new pos si ble clin i cal pic ture mim ic ked by the MCL. In the case of near to tal re sec tion of the MCL due to the in clu sion of ma jor ves sels, the re main ing lit tle cysts may be opened and evac u - ated with pos si bil ity of re cov ery. Patient consent Con sent to pub lish the case re port was not ob tained. This re port does not con tain any per sonal in for ma tion that could lead to the iden - ti fi ca tion of the pa tient. Funding No fund ing or grant sup port. Authorship All au thors at test that they meet the cur rent ICMJE cri te ria for Au - thor ship. Declaration of competing interest The fol low ing au thors have no fi nan cial dis clo sures: M E, F G, A R, N N, V M, M M, M F, B M. References [1] Clement C , Snoekx R , Ceulemans P , Wyn I , Matheï J . An acute presentation of pediatric mesenteric lymphangioma: a case report and literature overview . Acta Chir Belg 2018 Oct ; 118 ( 5 ) : 331 – 5 . [2] Kim SH , Kim HY , Lee C , Min HS , Jung SE . Clinical features of mesenteric lymphatic malformation in children . J Pediatr Surg 2016 Apr ; 51 ( 4 ) : 582 – 7 . [3] Caro PA , Mahboubi S , Faerber EN . Computed tomography in the diagnosis of lymphangiomas in children . Clin Imag 1991 Jan - Mar ; 15 ( 1 ) : 41 – 6 . [4] Xu X , Zheng C , He X , Zhao Y , Hong T . Gastrointestinal: giant mesenteric cystic lymphangioma . J Gastroenterol Hepatol 2017 Feb ; 32 ( 2 ) : 290 . [5] Goh BK , Tan YM , Ong HS , Chui CH , Ooi LL , Chow PK , et al . Intra - abdominal and retroperitoneal lymphangiomas in pediatric and adult patients . World J Surg 2005 Jul ; 29 ( 7 ) : 837 – 40 . [6] Losanoff JE , Richman BW , El - Sherif A , Rider KD , Jones JW . Mesenteric cystic lymphangioma . J Am Coll Surg 2003 Apr ; 196 ( 4 ) : 598 – 603 . [7] Mayer M , Fartab M , Villiger A , Yurtsever H . Cystic lymphangioma of the transverse mesocolon Chirurg 1994 Jun ; 65 ( 6 ) : 561 – 3 . [8] Niwa H , Sumita N , Ishihara K , Hoshino T , Iwase H , Kuwabara Y . A Case of Retroperitoneal Chylous cyst Developed after Cholecystectomy and Choledochotomy Nihon Geka Gakkai Zasshi . 89 ; 1988 Feb . p. 282 – 5 . 2 . [9] Gleason TJ , Yuh WT , Tali ET , Harris KG , Mueller DP . Traumatic cervical cystic lymphangioma in an adult . Ann Otol Rhinol Laryngol 1993 Jul ; 102 ( 7 ) : 564 – 6 . [10] Schmidt M . Intra - abdominal lymphangioma . Kans Med 1992 May ; 93 ( 5 ) : 149 – 50 . [11] Weeda VB , Booij KA , Aronson DC . Mesenteric cystic lymphangioma: a congenital and an acquired anomaly? Two cases and a review of the literature . J Pediatr Surg 2008 Jun ; 43 ( 6 ) : 1206 – 8 . [12] Méndez - Gallart R , Bautista A , Estévez E , Rodríguez - Barca P . Abdominal cystic lymphangiomas in pediatrics: surgical approach and outcomes . Acta Chir Belg 2011 Nov - Dec ; 111 ( 6 ) : 374 – 7 . [13] Hancock BJ , St - Vil D , Luks FI , Di Lorenzo M , Blanchard H . Complications of lymphangiomas in children . J Pediatr Surg 1992 Feb ; 27 ( 2 ) : 220 – 4 . ; discussion 224 - 6 . [14] Luo CC , Huang CS , Chao HC , Chu SM , Hsueh C . Intra - abdominal cystic lymphangiomas in infancy and childhood . Chang Gung Med J 2004 Jul ; 27 ( 7 ) : 509 – 14 . [15] Merrot T , Chaumoitre K , Simeoni - Alias J , Alessandrini P , Guys JM , Panuel M . Abdominal cystic lymphangiomas in children. Clinical, diagnostic and therapeutic aspects: apropos of 21 cases . Ann Chir 1999 ; 53 ( 6 ) : 494 – 9 . [16] Li Q , Ji D , Tu KS , Dou CW , Yao YM . Clinical analysis of intraperitoneal lymphangioma . Chin Med J 2015 Nov 20 ; 128 ( 22 ) : 3043 – 9 . [17] Bertozzi M , Riccioni S , Valoncelli C , Appignani A . The diagnosis and management of ovarian cysts in children . J Pediatr Adolesc Gynecol 2017 Apr ; 30 ( 2 ) : 265 . [18] Bertozzi M , Magrini E , Bellucci C , Riccioni S , Appignani A . Recurrent ipsilateral ovarian torsion: case report and literature review . J Pediatr Adolesc Gynecol 2015 Dec ; 28 ( 6 ) : e197 - 201 . [19] Pinto A , Pinto F , Faggian A , Rubini G , Caranci F , Macarini L , et al . Sources of error in emergency ultrasonography . Crit Ultrasound J 2013 ; 5 ( Suppl 1 ) : S1 . [20] Stopinski J , Stephan S , Staib I . Intra - abdominal cystic lymphangioma and mesenteric cysts as a cause of abdominal discomfort . Langenbecks Arch Chir 1994 ; 379 ( 3 ) : 182 – 7 . [21] Mabrut JY , Grandjean JP , Henry L , Chappuis JP , Partensky C , Barth X , et al . Mesenteric and mesocolic cystic lymphangiomas. Diagnostic and therapeutic management . Ann Chir 2002 May ; 127 ( 5 ) : 343 – 9 . 

Mesenteric cystic lymphangioma mimicking an ileo-colic intussusception

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Mesenteric cystic lymphangioma mimicking an ileo-colic intussusception

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