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A unique presentation of atypical complete duplication of terminal ileum, colon, rectum, and urinary bladder
A Unique
Presentation of Atypical Complete Duplication Ileum, Colon, Rectum, and Urinary Bladder
By S. Chaoramani
Gopal,
A.N. Gangopadhyay,
D.K. Gupta, L.B. Sharma Varanasi, tn$ia
0 A unique case of complete duplication of urinary bladder, 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: mesocolon.
Duplication,
colon,
ileum,
rectum,
bladder,
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 mesocolons and vascular arcades has not yet been reported. c
Chandrasen
Kr. Sinha,
of Terminal
Sakfi P Sahoo,
and
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
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; Division 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
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 dupiication of colon reported so far are lower urinary tract duplication, distal ileum duplication, omphalocele, vertebra1 column, anorectal, and‘other anomalies.‘-l2 Our case showed an association of complete duplication of distal ileum, colon, rectum, and lower genitourinary tract, but no vertebral anomaly. In these duplications, the cranial end of the primitive streak becomes split to form two blastopores. From these arise two notochords slightly separated at their caudal ends, which subsequently 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 separation. 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 rectovaginal fistula.11-16In our case, the patent normal rectum was communicating with the blind pouch, which was communicating 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 intercommunication between these two rectums.
1250
Journa/
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
ofPediatric
Surgery,
Vol32,
No 8 (August),
1997: pp 1250-‘I251
UNUSUAL
ORGAN
DUPLICATIONS
_ FULL THICKNESS COLON EXCISED
A Fig 1. operation performed.
Schematic diagram (A) par operative
B showing findings
the detail of the and (B) operative
first-stage procedure
For duplicated lower genitourinary tract, joining the two bladders and preserving the correct phallus and urethra is widely a accepted method.4 But for complete duplication of colon, various operative techniques have been devised, such as dividing the septum to convert it into a common colonic channel;3 long side-to-side anastomosis of adjacent duplicated bowel;14 transection of the rectum over the peritoneal reflection and anastomosis of the proximal end of the neighboring colon to the main colon;15 after closing the fistula, only the distal few centimeters of septum may be removed to create a common distal channel;16-I7 and mucosal stripping of the duplicated colon. r7-l* But when two colons have separate
Fig 2. Schematic diagram of the second-stage operation, comprising (A) duplicated bladder anastomosed to make it one. (B,C) The blind-ending ileo-cola-rectal segment was excised in full thickness up to the proximal third of rectum abdominally. The distal two thirds of the rectum was removed by mucosal stripping through posterior sagital approach.
mesocolon and separate vascular arcades (as in our case), the blind ending duplicated colon should be excised abdominally, whereas the blind-ending rectum and the fistula are better dealt with by posterior sagital approach. The complete reconstruction (ie, the second- and the third-stage operation) may be tried in a single stage. The present case brings to light a unique presentation of an atypical variant of complete tubular duplication of colon and emphasizes the fact that despite the best effort, the complete anatomical details and final operation plan may become clear only after surgical exploration.
REFERENCES 1. Goh DW, Davey RB, Dewan PA: Bladder, urethral and vaginal duplication. J Pediatr Surg 30:125-126, 1995 2. Dutta T, George V, Meenakshi PK, et al: Rare combination of duplication of genito-urinary tract, hindgut, vertebral column and other associated anomalies. Br J Urol46:577-582, 1974 3. Pierett RV, Lago .I: Complete tubular side by side duplication of the colon and distal ileum. Pediatr Surg Int 9:127-128, 1994 4. Azmi AF: Complete duplication of the hindgut and lower urinary tract with diphallus. J Pediatr Surg 25:647-649, 1990 5. Yousefzadeh DK, Bickers GH, Jackson JH, et al: Tubular colonic duplication-review of 1876-1981 literature. Pediatr Radio1 13:65-71, 1983 6. Kapoor R, Saha MM: Complete duplication of the bladder, urethra and external genitalla. .I Urol 137:1243-1244, 1987 7. Norris RW, Brereton RJ, Wright VM, et al: A new surgical approach to duplications of the intestine. J Pediatr Surg 21: 167.170. 1986 8. Ravitch MM: Hindgut duplication-Doubling of colon and genito urinary tract. Ann Surg 137:588-601, 1953 9. Veeraraghavan KA, Gonzales ET Jr., Gibbons MD, et al: Cloaca1 duplication: Genitourinary and lower intestinal duplications, J Urol 129:389-391, 1983 10. Sharma SP, Gangopadhyay AN, Gupta DK, et al: A rare
association of diaphallus, colonic duplications, ileal atresia and anorectal malformation. Pediatr Surg Int 11:414-415, 1996 11. Hunter TB, Tonkin LD: Complete duplication of the colon in association with urethral duplication. Gastrointest Radio1 4:93-95, 1979 12. Shearer LT, Holt HA, Young LW: Radiological case of the month (duplication of the bladder and colon). Am J Dis Child 135:661-662, 1981 13. Yucesan S, Zorludemir U, Oleay I: Complete duplication of the colon. J Pediatr Surg 21:962-963, 1986 14. Lister J: Duplication of the alimentary tract, in Lister J, Irving I (eds): Neonatal Surgery (ed 3). London, Butterworth, 1990, pp 474-484 15. Ravitch MM: Duplication of the gastrointestinal tract, in Ravitch MM, Welch KJ, Bandolph JG (eds): Pediatric Surgery. Chicago, IL, Year Book Medical, 1986, pp 91 l-920 16. Wrenn EL: Alimentary tract duplications, in Ashcraft KW, Holder TM (eds): Pediatric Surgery (ed 2). Philadelphia, PA, Saunders, 1993, pp 421-434 17. Holcomb III GW, Gheissari A, O’Neill JA, et al: Surgical management of alimentary tract duplication. Arch Surg 209: 167-174, 1989 18. Cooksey G, Wagget J: Tubular duplication of rectum treated by mucosal resection. J Pediatr Surg 19:318-320, 1984
Presentation of Atypical Complete Duplication Ileum, Colon, Rectum, and Urinary Bladder
By S. Chaoramani
Gopal,
A.N. Gangopadhyay,
D.K. Gupta, L.B. Sharma Varanasi, tn$ia
0 A unique case of complete duplication of urinary bladder, 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: mesocolon.
Duplication,
colon,
ileum,
rectum,
bladder,
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 mesocolons and vascular arcades has not yet been reported. c
Chandrasen
Kr. Sinha,
of Terminal
Sakfi P Sahoo,
and
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
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; Division 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
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 dupiication of colon reported so far are lower urinary tract duplication, distal ileum duplication, omphalocele, vertebra1 column, anorectal, and‘other anomalies.‘-l2 Our case showed an association of complete duplication of distal ileum, colon, rectum, and lower genitourinary tract, but no vertebral anomaly. In these duplications, the cranial end of the primitive streak becomes split to form two blastopores. From these arise two notochords slightly separated at their caudal ends, which subsequently 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 separation. 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 rectovaginal fistula.11-16In our case, the patent normal rectum was communicating with the blind pouch, which was communicating 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 intercommunication between these two rectums.
1250
Journa/
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
ofPediatric
Surgery,
Vol32,
No 8 (August),
1997: pp 1250-‘I251
UNUSUAL
ORGAN
DUPLICATIONS
_ FULL THICKNESS COLON EXCISED
A Fig 1. operation performed.
Schematic diagram (A) par operative
B showing findings
the detail of the and (B) operative
first-stage procedure
For duplicated lower genitourinary tract, joining the two bladders and preserving the correct phallus and urethra is widely a accepted method.4 But for complete duplication of colon, various operative techniques have been devised, such as dividing the septum to convert it into a common colonic channel;3 long side-to-side anastomosis of adjacent duplicated bowel;14 transection of the rectum over the peritoneal reflection and anastomosis of the proximal end of the neighboring colon to the main colon;15 after closing the fistula, only the distal few centimeters of septum may be removed to create a common distal channel;16-I7 and mucosal stripping of the duplicated colon. r7-l* But when two colons have separate
Fig 2. Schematic diagram of the second-stage operation, comprising (A) duplicated bladder anastomosed to make it one. (B,C) The blind-ending ileo-cola-rectal segment was excised in full thickness up to the proximal third of rectum abdominally. The distal two thirds of the rectum was removed by mucosal stripping through posterior sagital approach.
mesocolon and separate vascular arcades (as in our case), the blind ending duplicated colon should be excised abdominally, whereas the blind-ending rectum and the fistula are better dealt with by posterior sagital approach. The complete reconstruction (ie, the second- and the third-stage operation) may be tried in a single stage. The present case brings to light a unique presentation of an atypical variant of complete tubular duplication of colon and emphasizes the fact that despite the best effort, the complete anatomical details and final operation plan may become clear only after surgical exploration.
REFERENCES 1. Goh DW, Davey RB, Dewan PA: Bladder, urethral and vaginal duplication. J Pediatr Surg 30:125-126, 1995 2. Dutta T, George V, Meenakshi PK, et al: Rare combination of duplication of genito-urinary tract, hindgut, vertebral column and other associated anomalies. Br J Urol46:577-582, 1974 3. Pierett RV, Lago .I: Complete tubular side by side duplication of the colon and distal ileum. Pediatr Surg Int 9:127-128, 1994 4. Azmi AF: Complete duplication of the hindgut and lower urinary tract with diphallus. J Pediatr Surg 25:647-649, 1990 5. Yousefzadeh DK, Bickers GH, Jackson JH, et al: Tubular colonic duplication-review of 1876-1981 literature. Pediatr Radio1 13:65-71, 1983 6. Kapoor R, Saha MM: Complete duplication of the bladder, urethra and external genitalla. .I Urol 137:1243-1244, 1987 7. Norris RW, Brereton RJ, Wright VM, et al: A new surgical approach to duplications of the intestine. J Pediatr Surg 21: 167.170. 1986 8. Ravitch MM: Hindgut duplication-Doubling of colon and genito urinary tract. Ann Surg 137:588-601, 1953 9. Veeraraghavan KA, Gonzales ET Jr., Gibbons MD, et al: Cloaca1 duplication: Genitourinary and lower intestinal duplications, J Urol 129:389-391, 1983 10. Sharma SP, Gangopadhyay AN, Gupta DK, et al: A rare
association of diaphallus, colonic duplications, ileal atresia and anorectal malformation. Pediatr Surg Int 11:414-415, 1996 11. Hunter TB, Tonkin LD: Complete duplication of the colon in association with urethral duplication. Gastrointest Radio1 4:93-95, 1979 12. Shearer LT, Holt HA, Young LW: Radiological case of the month (duplication of the bladder and colon). Am J Dis Child 135:661-662, 1981 13. Yucesan S, Zorludemir U, Oleay I: Complete duplication of the colon. J Pediatr Surg 21:962-963, 1986 14. Lister J: Duplication of the alimentary tract, in Lister J, Irving I (eds): Neonatal Surgery (ed 3). London, Butterworth, 1990, pp 474-484 15. Ravitch MM: Duplication of the gastrointestinal tract, in Ravitch MM, Welch KJ, Bandolph JG (eds): Pediatric Surgery. Chicago, IL, Year Book Medical, 1986, pp 91 l-920 16. Wrenn EL: Alimentary tract duplications, in Ashcraft KW, Holder TM (eds): Pediatric Surgery (ed 2). Philadelphia, PA, Saunders, 1993, pp 421-434 17. Holcomb III GW, Gheissari A, O’Neill JA, et al: Surgical management of alimentary tract duplication. Arch Surg 209: 167-174, 1989 18. Cooksey G, Wagget J: Tubular duplication of rectum treated by mucosal resection. J Pediatr Surg 19:318-320, 1984