A Unique Presentation of Atypical Complete Duplication of Terminal Ileum, Colon, Rectum, and Urinary Bladder By S. Chaoramani Gopal, A.N. Gangopadhyay, D.K. Gupta, Chandrasen Kr. Sinha, Sakfi P Sahoo, and L.B. Sharma Varanasi, tn$ia 0 A unique case of complete duplication of urinary blad- der, distal ileum, cecum, appendix, colon, and rectum with twd mesocolons and separate vascular arcades is’being reported. Copyright @ 7997 by W. B. Saunders Company INDEX WORDS: Duplication, colon, ileum, rectum, bladder, mesocolon. c OMPLETE DUPLICATION of colon, bladder, and urethra are rare anomalies even when occurring individually.1-4 All of them occurring in the same patient is extremely rare. Complete duplication of terminal ileum, colon, and rectum having two separate mesoco- lons and vascular arcades has not yet been reported. CASE REPORT A 5-year-old boy presented with constipation, severe straining, and prolapse of a globular mass during defecation, along with fever, pyuria, and toxic symptoms. Two months before he ingested a 1.5.cm diameter glass bail. This child had undergone an excision of a small rudimentary phallus adjacent to a normal phallus at the age of 1 month at another hospital. There was no history of passage of urine through that rudimentary phallus. There was a history of intermittent passage of feces and mucus per urethra during micturation since birth, and a micturating cystourethrogram performed 6 months previously showed duplication of bladder. At the time of admission the child was having severe bouts of intermittent abdominal pain and excessive straining during defecation. The mother had observed a glass ball partially extruding from the rectum during defecation, but it slipped back into the rectum and could not be removed. On rectal examination a 1.5~cm diameter ball was felt extrarectally through the anterior rectal wall. Results of an emergency laparotomy showed complete duplication of bladder (RU & LU), tubular duplication of distal 25 cm of ileum and whole colon with separate mesocolon, and two sets of superior and inferior mesenteric vascular arcades (Fig 1). Rut the.distal two thirds of the two rectums shared a common wall, and the anterior one was ending blindly with a fistulous communication to the urethra. Into this blind rectum (D) the glass ball was lodged. The duplicated gut with blind ending rectum (D) was excised from ileum to mid sigmoid colon, and the proximal end of the residual sigmoid colon was fixed to the abdominal wall as mucous fistula. A defunctioning sigmoid loop colostomy was performed in the patent colon (M) to facilitate the second stage of operation (because of poor general condition). After 3 months, through abdominal route, the two bladders were anastomosed side by side in their full length. The colon with mucous fistula was From the Division of Pediatric Surgery, Institute of Medical Sciences, Banaras Hindu University Varanasi 221005, India. Address reprint requests to DrA.N. Gangopadhyay, MD, Readel; Di- vision of Pediatric Surgery, Department of Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi 221 005 (U.P.), India. Copyright 0 1997 by WB. Saunders Company 0022-3468/97/3208-0026$03.00/O 1250 excised up to the proximal third of rectum. The remaining distal part of blind rectal pouch was denuded of its mucosa and was left behind (Fig 2). During the third operation (performed after 2 weeks), through Pena’s approach, the posterior wall of the patent rectum (v) was opened in midline. A rectorectal fistula was seen at 6 cm from anal verge. On opening the common wall, the fistulqus communication between the blind pouch and posterior urethra was revealed. This fistula was closed after coring out the mucosa:The pouch (D) was then obliterated by interrupted stitches. Anterior and posterior wall of the patent rectum was repaired, and the wound was closed in layers. After ruling out the possibility of any rectourinary communication by contrast study, the colostomy was closed after 6 weeks. Recent follow-up (after 1 year) showed that the child is continent of both urine and feces, and empties the bladder completely. DISCUSSION The first case of colonic duplication was published in 1876.5 Duplication of the colon and rectum is a rare (4% to 18%) anomaly of all gastrointestinal duplication. The tubular duplication is far less common than the spherical one. But all the tubular duplication reported until now. had single mesocolon and single vascular arcade.2-7 The various associated anomalies with complete tubular dupii- cation of colon reported so far are lower urinary tract duplication, distal ileum duplication, omphalocele,verte- bra1 column, anorectal, and‘other anomalies.‘-l2 Our case showed an association of complete duplica- tion of distal ileum, colon, rectum, and lower genitouri- nary tract, but no vertebralanomaly. In these duplica- tions, the cranial end of the primitive streak becomes split to form two blastopores. From these arise two notochords slightly separatedat their caudal ends, which subse- quently fuse into one during their cranial elongation.i3-l6 In its extreme form, the disturbance seems to start with a divided notochord that fuses again cranial to the separa- tion. But the caudal part ‘may remain separated from the beginning, giving rise to two separate mesocolon and two vascular arcades, as seen in our’case. In most other reported cases, the duplicated colon has proximal connection with patent gut, and distally it terminates into bliud pouch with rectourinary or rectovagi- nal fistula.11-16 In our case, the patent normal rectum was communicating with the blind pouch, which was commu- nicating to the posterior urethra. Further, the ingested glass ball lodged in the blind pouch was causing the presenting symptom of excessive painful straining during defecation, and the mother’s observation the ball in normal rectum was possibly because of the intercommu- nication between these two rectums. Journa/ ofPediatric Surgery, Vol32, No 8 (August), 1997: pp 1250-‘I251