Anorectal Agenesis with Rectovaginal Fistula Associated with Uterus Didelphys and Vaginal Septum
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Journal of Pediatric Surgery Case Reports 55 (2020) 101328 Contents lists available at ScienceDirect Journal of Pediatric Surgery Case Reports Anorectal agenesis with rectovaginal fistula associated with uterus didelphys and vaginal septum Maher AlZaiem a , *, Abdulrahman Almaghrebi b, Asim A. Asghar c, Feras Zaiem d a Maternity and Children Hospital, Mecca, Saudi Arabia b Pediatric Surgery Department, Maternity and Children Hospital, Saudi Arabia c Maternity and Children Hospital, Mecca, Saudi Arabia d Department of Pathology, Karmanos Cancer Institute, Wayne State University School of Medicine, Detroit, MI, USA ARTICLE INFO ABSTRACT Keywords : Anorec tal age n e sis with rec to vagi nal fistula are ex tremely rare type of anorec tal malfor ma tions (ARM) char ac - Anorectal malformations ter ized by the ab sence of the anus and ab nor mal con nec tion of the rec tum to the vagina. Uterus didel phys and Rectovaginal fistula lon gi tu di nal vagi nal septum are va ri eties of mul ler ian anom alies. In this ar ticle, we re port a case of ARM with Uterus didelphys rec to vagi nal fistula as so ci ated with uterus diphe dus and lon gi tu di nal vagi nal septum with the sur gi cal man - Vaginal septum age ment. Results : The clin ical and ra di olog ical findings and the surgi cal man agement were dis cussed. 1 . Introduction lad muller ian fu sion was first de scribed by Crosby and Hill in 1962 [ 4 ]. Dur ing the embry onic period, the caudal end of the hindgut gives rise to the cloaca. By the 6 weeks of ges ta tion, the urorectal septum 2 . Case presentation moves cau dally to di vide the cloaca into an ante rior chamber named the urogen i tal si nus, and a poste rior chamber that will form the A full - term two -day old baby girl with a birth weight of 2800 g, anorec tal canal. Fail ure of the urorectal septum forma tion results in a was referred to our hos pital for the absence of an anal opening. Upon fistula between the bowel and uri nary tract in the males, or with the ad mission, she passed a small amount of meconium through vagina vagina in the females [ 1 ]. At the same time, the Mul lerian ducts Phys ical ex amin ing showed a soft abdomen and an absent anal open- (para mesonephric) de velop from coelomic epithe lium and grow cau - ing, however, nor mal urethral and vagi nal orifices were iden tified and dally along the Wolf fianducts (mesonephric) to ward the uro gen ital si- a small spot of meconium seen through the hy men ( Fig. 1 ). Rou tine nus, forming the two uterovaginal canals. At the 11 weeks of ges ta - blood workup was within the nor mal range. The baby was further tion, the Mul lerian ducts are fused lat erally to form a sin gle canal eval u ated for any as so ci ated anom alies. Echocardio gram showed an which be comes the uterus and the upper part of the vagina. atrial septal de fect and ven tral septal defect. The whole- body skeletal Mean while, the sino - vaginal bulbs in vagi nate from the uro gen ital sur vey and the abdomen ul trasonog raphy were un remark able and she sinus and meet the caudal end of the fused Mul lerian ducts to form was then sched uled for surgery. the vagi nal plate. Finally, the vagi nal plate is reab sorbed and canal - In the oper a tion room, perineal ex ami nation under anes thesia re- ized to form the lower part of the vagina. This process of resorp tion is vealed lon gitu di nal vagi nal septum ( Fig. 2 ). Therefore, a sig moid loop com pleted by the 24 weeks of ges ta tion. Failure of fu sion of the Mul - colostomy was performed as a first man age ment stage. The post oper a - lerian ducts dur ing early embry olog ical life (8– 12 weeks of gesta tion) tive course was unevent ful and the patient was dis charged home in results in uterus didel phys (dou ble uterus) or septated uterus. The sta ble con di tion. On a fol low- up visit, a dis tal loop colostogram was vagina may be septated as well [ 2 , 3 ]. This theory of cau dal to cepha- performed showing the rectum is con nected to the top of the vagi nal vault. Then, this con trast delin eated the vagina and drained out of the * Corresponding author. Maternity and Children Hospital, Mecca, PO Box: 12955, Saudi Arabia. E- mail addresses: maher_ zaiem@hotmail. com (M. AlZaiem), magabdo5@ hotmail. com (A. Almaghrebi), asimniaz72@ yahoo. com (A. A. Asghar), feras. za3im@ gmail. com (F. Zaiem). https://doi.org/10.1016/j.epsc.2019.101328 Received 27 August 2019; Received in revised form 15 October 2019; Accepted 16 October 2019 Available online 19 October 2019 2213-5766/© 2019 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/). M. AlZaiem et al. Journal of Pediatric Surgery Case Reports 55 (2020) 101328 Fig. 1 . Perineal ex ami na tion two open ings in the in troitus: U the urethral open ing. H the hymen through. M the meconium spot in ter nal to the hymenal Fig. 3 . Dis tal colostogram show ing: R: the rec tum, RV: the rec to vagi nal fistula ring. and V: the vagina. Fig. 4 . La paroscopic demon stra tion of the uterus didel phus. R: rec tum, RU: right uterus, LU: left uterus. V the vagi nal dome, B: the blad der. The anal area was mapped on the ex ter nal sur face of the per- ineum. Us ing a muscle stimu la tor, we de termined the an terior and poste rior limits of the fu ture anus and a 1.5 cm midline verti cal in ci - sion was made over the perineum at the site of the proposed anal ori- fice. The in tra- sphincteric plane was dis sected bluntly from its cau dal end and con tin ued cephalic in the midline under la paro scopic guid - Fig. 2 . Perineal ex ami na tion demon strates the vagi nal sep tum.V.S, the ure thra U. ance. The tract was then di lated radi ally and the rectal pouch was grasped and pulled down to the perineum within the sphincter muscle perineum ( Fig. 3 ). This ra di o log i cal finding was sug ges tive of the high com plex. Finally, the anas to mosis was com pleted between the pulled lo cation of the recto vagi nal fistula. rectum and new anus, us ing an in ter rupted 5 -0 vicryl su ture. At the At the age of one year, the baby under went la paro scopic - assisted end of the proce dure, the vagi nal septum was resected us ing pull - through proce dure. She was placed in a litho tomy posi tion with a diathermy. 15° head- down tilt. Mean while, a naso gas tric tube and a uri nary The baby well tol erated the proce dure. Oral feeding was resumed catheter are inserted. The oper a tion started by opting open method on the first post oper a tive day, then she was dis charged home on the for umbil i cal port in ser tion and pneumoperi toneum was cre ated by fifth post op era tive day. An anal di lata tion program was started post - CO2 pressure maintained at 10 mmHg. The la paro scopic in ter vention op era tively. The reverse colostomy was performed af ter six weeks of was car ried out by us ing three 5 mm ports; one umbil i cal port for 30° the de fini tive surgery. On a fol low- up visit, the baby passed well - tele scope and two working ports. formed stools with a frequency of about 2 –3 times a day. Physi cal ex - The la paro scopic ex ploration revealed that the rectum is con - ami na tion showed sym metric anal con trac tion with strong squeeze on nected to the dome of the vagina and along with it, the didel phys the dig ital rectal ex ami na tion. The cos metic appear ance was also sat - uterus is com muni cat ing with the vagi nal dome on both sides of the isfac tory. However, a long term fol low- up was recom mended. recto vagi nal fistula ( Fig. 4 ). Then, la paro scopic rectal dis sec tion was car ried out at the level of peritoneal reflec tion by us ing ul tra- scissors. 3 . Discussion The dis sec tion con tin ued until it reached the rectal pouch, where it becomes nar row at the level of the recto vagi nal fistula. The fistula Anorec tal malfor ma tions (ARM) is a spectrum of abnor mal de vel - was trans- ligated us ing vicryl 3/0 stitch and di vided in close proxim - op ment of the anus and rectum with an inci dence rate of 1 in 2700 to ity to the vagina. 1 in 5000 live births [ 5 , 6 ]. The in ci dence of ARM with Recto vagi nal 2 M. AlZaiem et al. Journal of Pediatric Surgery Case Reports 55 (2020) 101328 fistula (RVF) was reported to be less than 1% of all ARM sub types [ 7 ].