- Email: [email protected]
Perinatally discovered complete tubular colonic duplication associated with anal atresia
Journal of Pediatric Surgery (2012) 47, E19–E23
www.elsevier.com/locate/jpedsurg
Perinatally discovered complete tubular colonic duplication associated with anal atresia☆ Mohamed A. Jellali a,⁎, Mongi Mekki b , Jamel Saad a , Ahmed Zrig a , Imene Elanes b , Walid Mnari a , Mezri Maatouk a , Wissem Harzallah a , Samir Toumi c , Imed Krichène b , Randa Salem a , Abdellatif Nouri b , Mondher Golli a a
Department of Radiology, CHU Fattouma-Bourguiba, 1st JUNE Street, Monastir 5000, Tunisia Department of Pediatric Surgery, CHU Fattouma-Bourguiba, 1st JUNE Street, Monastir 5000, Tunisia c Department of Anaesthesia and Chirurgical Intensive, CHU Fattouma-Bourguiba, 1st JUNE Street, Monastir 5000, Tunisia b
Received 18 May 2011; revised 17 January 2012; accepted 24 January 2012
Key words: Duplication; Colon; Anal atresia; Children; Malformation
Abstract Complete tubular colonic duplication (CTCD) is exceedingly rare. The association of CTCD with an anorectal malformation is unusual. This malformation may be found unexpectedly at laparotomy. We present 3 cases of surgically proven neonate CTCD discovered at laparotomy for anal atresia. We reviewed the mode of clinical presentation, the imaging, and laparotomy findings. Our series illustrates that this rare disease presents perinatally in association with anal atresia, with or without other associated anomalies. © 2012 Elsevier Inc. All rights reserved.
Complete tubular colonic duplication (CTCD) is exceedingly rare [1]. The association of CTCD with an anorectal malformation is unusual. This malformation may be found unexpectedly at laparotomy [2]. We present 3 cases of surgically proven neonatal CTCD discovered at laparotomy for anal atresia. The aims of this study were to illustrate the clinical manifestations and to describe and discuss the imaging features and treatment options.
1. Patients and method This is a retrospective study done in the pediatric surgery department of Monastir between 1984 and 2000. We ☆ Conflict of Interest: None. ⁎ Corresponding author. Tel.: +216 73461144. E-mail address: [email protected] (M.A. Jellali).
0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2012.01.082
reviewed the mode of clinical presentation, imaging, and laparotomy findings in 3 neonatal cases with surgically proven CTCD. The mode of clinical presentation and means of definitive diagnosis were recorded.
1.1. Case 1 A female infant was referred to our institution at 2 days of age for anorectal malformation discovered at birth. Clinical examination revealed an anal atresia and split vaginal orifice. There was no evidence of rectovaginal or rectourethral fistula. An invertogram showed high type of anorectal malformation. A colostomy was performed as an emergency. During surgery, a CTCD was noted. A contrast enema confirmed the diagnosis of CTCD with a proximal communication and with both limbs ending blindly in the pelvis without either an enterourinary or enterogenital fistula (Fig. 1). An anorectal reconstruction was successfully performed at 5 months of age
E20
M.A. Jellali et al. colonic tubular duplication with presence of 2 ceca and 2 appendices. Contrast enema studies showed a CTCD that communicated proximally at the level of the ileocecal junction. Distally, both segments were not fused, resulting in a cul-de-sac twin limbs with both segments ending as fistulae to the urethra (Fig. 2A and B). An anorectal reconstruction was successfully performed at 5 months of age. A distal communication was made surgically by creation of a communicating window of approximately 5 cm between the 2 sigmoid colons and rectums as low down as possible. We excised the bilateral rectourethral fistulae through a posterior sagittal approach (Penã and Devries procedure) [3]. The colostomy was closed 2 months later. Twelve years after surgery, the patient has only intermittent gastrointestinal symptoms comprising alternating diarrhea and constipation.
1.3. Case 3
Fig. 1 Contrast enema through both colostomy orifices (case 1), showing CTCD with both limbs ending separately blindly in the pelvis without neither enterourinary nor enterogenital fistula.
with the “Stephens procedure.” A distal communication was made surgically between the duplicated colons to enable emptying of the twin colon into the anal canal. During surgical exploration, a single uterus with 2 fallopian tubes were found. The colostomy was closed 3 months later. Microscopic examination showed a muscular coat in 2 layers and colonic mucosal lining that was indistinguishable from the normal colon, except the cul-de-sac of the duplication, which was covered with urinary-type epithelium. The patient is doing well 17 years after the surgery.
1.2. Case 2 A newborn baby boy was referred to our institution for an anorectal malformation discovered at birth. Physical examination revealed anal atresia with history of meconium passed in the urine. There was neither cutaneous fistula nor perineal bulge convexity at the supposed site of the anus. There was no anomaly of external genitalia. An invertogram showed high type of anorectal malformation. A right transverse colostomy was performed. At surgical exploration, the baby was found to have a complete side-to side
A newborn baby girl was transferred 7 hours after birth for an anorectal malformation discovered at birth. Physical examination revealed anal and vaginal atresia, without evidence of a fistula. Radiographs of the spine showed lumbosacral scoliosis with sacral hemivertebra. An invertogram done at 24 hours of age showed high type of anorectal malformation. A colostomy was performed at 2 days of age. During surgery, a CTCD was noted, communicating proximally with presence of 1 cecum and 1 appendix. Contrast study via the distal colostomy orifices confirmed the diagnosis and revealed the presence of 2 rectourinary fistulae allowing opacification of 2 bladders (Fig. 3). Ultrasonographic examination showed a bilateral ureteral dilatation and 2 bladders with no presacral mass. The intravenous urography showed duplicated bladder and urethra and confirmed bilateral ureteral dilatation. These findings were confirmed by an endoscopic examination, which revealed 2 rectourethral fistulae. There was only 1 vagina, which was atretic at its distal portion. Surgical correction was planned, but unfortunately, the patient died before this could be implemented.
2. Discussion Gastrointestinal duplication is an infrequent congenital abnormality. The reported incidence is 1 in 5000 live births [2,4]. It represents only 0.1 % to 0.3 % of all malformations [5,6]. Colonic localization accounts only for 4% to 18 % of all gastrointestinal duplication [7]. Duplications of the colon are most commonly isolated cystic structures, but rarely are they complete, side-by-side duplications of the entire colon, including the rectum. They can occur at any age, but most are discovered during the neonatal period or infancy particularly when they are associated with anorectal or urogenital malformation [6,8,9]. In our series, no case of CTCD was
Perinatally discovered complete tubular colonic duplication
E21
Fig. 2 A and B, Contrast enema studies (case 2) showed a CTCD that communicated proximally at the level of the ileocecal junction. Distally both segments were not fused resulting in a cul-de-sac twin limbs with both segments ending as fistulas to the urethras.
diagnosed in antenatal period. The 3 cases (2 girls and 1 boy) were diagnosed at the neonatal period because of their associated anal atresia. The precise etiology of these anomalies is still unclear. Proposed theories to explain duplications of the gastrointestinal tract are failure of regression of embryonic diverticula, sequestration of bowel during development, atavism, median septum development, partial twinning theory, and split notochord syndrome [8]. As noted, duplications that affect the large intestine are commonly associated with genitourinary and anorectal malformations. A more severe condition termed caudal duplication syndrome has also been described for cases in which there are associated gastrointestinal, genitourinary, and distal neural tube defects as found in our third case [10-13]. Total tubular colonic duplications may be asymptomatic and may remain undiagnosed for years or diagnosed later incidentally (60%-80 %). In cases of isolated colonic duplication, the diagnosis is invariably delayed until symptoms alert clinicians and prompt diagnostic evaluation is undertaken [14]. If symptomatic, they manifest by obstruction, bleeding, constipation, and perforation. Few cases revealed by malignancy are also reported [15]. In cases where associated congenital anomalies are present, the duplication is usually detected early. Female patients have a high prevalence of rectogenital fistulas and associated duplications of internal or external genital structures, whereas male patients have a high prevalence of rectourinary fistulas and duplication of external genitalia. Colorectal tubular duplications are commonly associated with vertebral anomalies as well as other gastrointestinal and skeletal malformations regardless of the patient's sex [8,16]. Yousefzadeh et al [17] described 57 patients with colon duplications of which 80% had associated anomalies. Our
series demonstrates imperforate anus in all 3 cases. In addition, CTCD was associated with a duplicated vagina in the first case, an appendicular duplication in the second case, and a caudal duplication syndrome in the third case. The neighboring colon in complete colonic duplication either terminates with a blind pouch or opens via rectourinary or rectovaginal fistulas [18,19]. Rectourinary fistulas were noted in cases 2 and 3. Barium contrast enema studies are considered essential for the diagnosis of tubular colonic duplication with opacification of 2 colons being the diagnostic sign [17]. In male infants who have a rectourethral fistula, improved
Fig. 3 Contrast study via the distal colostomy orifices (case 3): CTCD with both limbs ending distally separately in 2 enterourinary fistulae allowing opacification of 2 bladders.
E22 visualization of the duplication is achieved during cystourethrography or a retrograde urethrogram. In our series, colonic opacification was performed after surgical colostomy, showing CTCD in all cases with signs of enterourinary fistulas demonstrated in 2 cases. Other imaging modalities such as ultrasound, computed tomography, and magnetic resonance imaging may be helpful with cystic duplications, but in instances of tubular duplication ultrasonography computed tomography, or magnetic resonance imaging may be used in the investigation of the commonly occurring associated anomalies [8,20,21]. Endoscopy can have an interesting role for both the diagnosis and the treatment. The endoscopic examination under general anesthesia carried out in the third patient showed a complex cloacal malformation and revealed 2 rectourethral fistulae as well as a partial vaginal atresia. Indications for surgical intervention often arise in an acute setting, in the form of complications. [21-24]. The possibility of malignant degeneration within the duplication was reported [25-28]. The methods of surgical management are dependent primarily on size, location, communication with the gastrointestinal tract, and involvement of mesenteric vasculature [29,30]. Surgery is not indicated for an uncomplicated colonic duplication alone. It must be remembered that the treatment of this benign lesion must aim to eliminate symptoms and prevent complications; therefore, the surgical procedure should not be more radical than necessary. In most cases, resection of the supernumerary colon or microcolon is impossible because the 2 colons often share a common blood supply and, in most cases, there is only a single mesentery. The simplest approach is to perform a single anastomosis between the duplications, and it was first described by Yucesan et al [18]. Complete resection of the duplication is possible if both colic vascular supplies are separated, but this is exceptional (7% of cases). In our series, for the first 2 cases, treatment consisted of excision of the distal common septum with creation of a common channel followed by the lowering (pull through) of 2 duplicated colons by the technique of Stephens in the first case and Pena and Devries (posterior sagittal anorectoplasty) in the second case. The third patient died before the definitive surgical treatment could be implemented. Surgical treatment by excision of the distal common septum with creation of a common channel should prevent accumulation of feces in the false lumen and hopefully avoid constipation [31]. Mortality of CTCD is low: 4% to 8%. It is mainly seen in forms associated with severe malformations or with malignant transformation. Total tubular colonic duplication treated before the onset of complications generally have a favorable prognosis, hence the importance of an early diagnosis. Complete colonic duplications are very rare and usually associated with genitourinary duplications and complex congenital anomalies. Our series of 3 cases illustrates that
M.A. Jellali et al. CTCD presents perinatally in association with anal atresia, with or without other associated anomalies.
References [1] Kaur N, Nagpal K, Sodhi P, et al. Hindgut duplication—case report and literature review. Pediatr Surg Int 2004;20:640-2. [2] Craigie RJ, Abbaraju JS, Ba'ath ME, et al. Anorectal malformation with tubular hindgut duplication. J Pediatr Surg 2006;41:e31-4. [3] Peña A, Devries PA. Posterior sagittal anorectoplasty: important technical considerations and new applications. J Pediatr Surg 1982;17 (6):796-811. [4] Kokoska ER, Steinhardt GF, Tomita SS, et al. Prostatorectal fistula asscoaited with tubular colorectal duplication. J Pediatr Surg 1999;34: 1546-8. [5] Becmeur F, Viville B, Langer B, et al. Prenatal and neonatal management of digestive tract duplications. Diagnostic difficulties and therapeutic implications. J Gynecol Obstet Biol Reprod (Paris) 1999;28:388-92. [6] Noel L, Becmeur F, Jacques C, et al. Multiple gastrointestinal tract duplication: a neonatal case report. J Radiol 2001;82:676-8. [7] Gopal SC, Gangopadhyay AN, Gupta DK, et al. A unique presentation of atypical complete duplication of terminal ileum, colon, rectum, and urinary bladder. J Pediatr Surg 1997;32:1250-1. [8] Macpherson RI. Gastrointestinal tract duplications: clinical, pathologic, etiologic, and radiologic considerations. Radiographics 1993;13: 1063-80. [9] Alahyane A, Echarrab M, Lachkar A, et al. Colonic duplication associated with a common mesenterium revealed by cecal volvulus in a young adult. Gastroenterol Clin Biol 2009;33:499-501. [10] Shah KR, Joshi A. Complete genitourinary and colonic duplication: a rare presentation in an adult patient. J Ultrasound Med 2006;25: 407-11. [11] Banu T, Chowdhury TK, Hoque M, et al. Congenital double anus with total colon duplication: a case report. J Pediatr Surg 2007;42:E1-2. [12] McNeill SA, Rance CH, Stewart RJ. Fecolith impaction in a duplex vermiform appendix: an unusual presentation of colonic duplication. J Pediatr Surg 1996;31:1435-7. [13] McPherson AG, Trapnell JE, Airth GR. Duplication of the colon. Br J Surg 1969;56:138-42. [14] Blickman JG, Rieu PH, Buonomo C, et al. Colonic duplications: clinical presentation and radiologic features of five cases. Eur J Radiol 2006;59:14-9. [15] Bakir B, Gokce S, Dursun M, et al. An unusual cause of diffuse ascites in an infant: colonic duplication associated with bladder duplication. Diagn Interv Radiol 2009;15:210-1. [16] Kumar A, Kumar J, Gadodia A, et al. Multiple short-segment colonic duplications. Pediatr Radiol 2008;38:567-70. [17] Yousefzadeh DK, Bickers GH, Jackson JH, et al. Tubular colonic duplication—review of 1876-1981 literature. Pediatr Radiol 1983;13: 65-71. [18] Yucesan S, Zorludemir U, Olcay I. Complete duplication of the colon. J Pediatr Surg 1986;21:962-3. [19] Hunter TB, Tonkin IL. Complete duplication of the colon in assoication with urethral duplication. Gastrointest Radiol 1979;4:93-5. [20] Berrocal T, Lamas M, Gutieerrez J, et al. Congenital anomalies of the small intestine, colon, and rectum. Radiographics 1999;19:1219-36. [21] Misra S, Chaudhary A, Wakhlu A. Unusual CT sign of colonic duplication in a child. Pediatr Radiol 2006;36:462. [22] Stylianos S, Berdon WE, Hicks BA, et al. Complex colon duplication mimicking an obstructed, non-functioning kidney in a newborn with imperforate anus and spinal dysraphism. Pediatr Radiol 1995;25: 269-71. [23] Soper RT. Tubular duplication of the colon and distal ileum: case report and discussion. Surgery 1968;63:998-1004.
Perinatally discovered complete tubular colonic duplication [24] Schwartz DL, Becker JM, Schneider KM, et al. Tubular duplication with autonomous blood supply: resection with preservation of adjacent bowel. J Pediatr Surg 1980;15:341-2. [25] Birnbaum W. Squamous cell carcinoma and adenoacanthoma of the colon. JAMA 1970;212:1511-3. [26] Heiberg ML, Marshall KG, Himal HS. Carcinoma arising in a duplicated colon. Case report and review of literature. Br J Surg 1973; 60:981-2. [27] Hickey WF, Corson JM. Squamous cell carcinoma arising in a duplication of the colon: case report and literature review of squamous
E23
[28] [29] [30] [31]
cell carcinoma of the colon and of malignancy complicating colonic duplication. Cancer 1981;47:602-9. Lee J, Jeon YH, Lee S. Papillary adenocarcinoma arising in a duplication of the cecum. Abdom Imaging 2008;33:601-3. Grosfeld JL, O'Neill Jr JA, Clatworthy Jr HW. Enteric duplications in infancy and childhood: an 18-year review. Ann Surg 1970;172:83-90. Gross RE, Holcomb Jr GW, Farber S. Duplications of the alimentary tract. Pediatrics 1952;9:448-68. Fuchs JR, Clark K, Breckler FD, et al. Complete colonic duplication— a case report. J Pediatr Surg 2008;43:E11-3.
www.elsevier.com/locate/jpedsurg
Perinatally discovered complete tubular colonic duplication associated with anal atresia☆ Mohamed A. Jellali a,⁎, Mongi Mekki b , Jamel Saad a , Ahmed Zrig a , Imene Elanes b , Walid Mnari a , Mezri Maatouk a , Wissem Harzallah a , Samir Toumi c , Imed Krichène b , Randa Salem a , Abdellatif Nouri b , Mondher Golli a a
Department of Radiology, CHU Fattouma-Bourguiba, 1st JUNE Street, Monastir 5000, Tunisia Department of Pediatric Surgery, CHU Fattouma-Bourguiba, 1st JUNE Street, Monastir 5000, Tunisia c Department of Anaesthesia and Chirurgical Intensive, CHU Fattouma-Bourguiba, 1st JUNE Street, Monastir 5000, Tunisia b
Received 18 May 2011; revised 17 January 2012; accepted 24 January 2012
Key words: Duplication; Colon; Anal atresia; Children; Malformation
Abstract Complete tubular colonic duplication (CTCD) is exceedingly rare. The association of CTCD with an anorectal malformation is unusual. This malformation may be found unexpectedly at laparotomy. We present 3 cases of surgically proven neonate CTCD discovered at laparotomy for anal atresia. We reviewed the mode of clinical presentation, the imaging, and laparotomy findings. Our series illustrates that this rare disease presents perinatally in association with anal atresia, with or without other associated anomalies. © 2012 Elsevier Inc. All rights reserved.
Complete tubular colonic duplication (CTCD) is exceedingly rare [1]. The association of CTCD with an anorectal malformation is unusual. This malformation may be found unexpectedly at laparotomy [2]. We present 3 cases of surgically proven neonatal CTCD discovered at laparotomy for anal atresia. The aims of this study were to illustrate the clinical manifestations and to describe and discuss the imaging features and treatment options.
1. Patients and method This is a retrospective study done in the pediatric surgery department of Monastir between 1984 and 2000. We ☆ Conflict of Interest: None. ⁎ Corresponding author. Tel.: +216 73461144. E-mail address: [email protected] (M.A. Jellali).
0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2012.01.082
reviewed the mode of clinical presentation, imaging, and laparotomy findings in 3 neonatal cases with surgically proven CTCD. The mode of clinical presentation and means of definitive diagnosis were recorded.
1.1. Case 1 A female infant was referred to our institution at 2 days of age for anorectal malformation discovered at birth. Clinical examination revealed an anal atresia and split vaginal orifice. There was no evidence of rectovaginal or rectourethral fistula. An invertogram showed high type of anorectal malformation. A colostomy was performed as an emergency. During surgery, a CTCD was noted. A contrast enema confirmed the diagnosis of CTCD with a proximal communication and with both limbs ending blindly in the pelvis without either an enterourinary or enterogenital fistula (Fig. 1). An anorectal reconstruction was successfully performed at 5 months of age
E20
M.A. Jellali et al. colonic tubular duplication with presence of 2 ceca and 2 appendices. Contrast enema studies showed a CTCD that communicated proximally at the level of the ileocecal junction. Distally, both segments were not fused, resulting in a cul-de-sac twin limbs with both segments ending as fistulae to the urethra (Fig. 2A and B). An anorectal reconstruction was successfully performed at 5 months of age. A distal communication was made surgically by creation of a communicating window of approximately 5 cm between the 2 sigmoid colons and rectums as low down as possible. We excised the bilateral rectourethral fistulae through a posterior sagittal approach (Penã and Devries procedure) [3]. The colostomy was closed 2 months later. Twelve years after surgery, the patient has only intermittent gastrointestinal symptoms comprising alternating diarrhea and constipation.
1.3. Case 3
Fig. 1 Contrast enema through both colostomy orifices (case 1), showing CTCD with both limbs ending separately blindly in the pelvis without neither enterourinary nor enterogenital fistula.
with the “Stephens procedure.” A distal communication was made surgically between the duplicated colons to enable emptying of the twin colon into the anal canal. During surgical exploration, a single uterus with 2 fallopian tubes were found. The colostomy was closed 3 months later. Microscopic examination showed a muscular coat in 2 layers and colonic mucosal lining that was indistinguishable from the normal colon, except the cul-de-sac of the duplication, which was covered with urinary-type epithelium. The patient is doing well 17 years after the surgery.
1.2. Case 2 A newborn baby boy was referred to our institution for an anorectal malformation discovered at birth. Physical examination revealed anal atresia with history of meconium passed in the urine. There was neither cutaneous fistula nor perineal bulge convexity at the supposed site of the anus. There was no anomaly of external genitalia. An invertogram showed high type of anorectal malformation. A right transverse colostomy was performed. At surgical exploration, the baby was found to have a complete side-to side
A newborn baby girl was transferred 7 hours after birth for an anorectal malformation discovered at birth. Physical examination revealed anal and vaginal atresia, without evidence of a fistula. Radiographs of the spine showed lumbosacral scoliosis with sacral hemivertebra. An invertogram done at 24 hours of age showed high type of anorectal malformation. A colostomy was performed at 2 days of age. During surgery, a CTCD was noted, communicating proximally with presence of 1 cecum and 1 appendix. Contrast study via the distal colostomy orifices confirmed the diagnosis and revealed the presence of 2 rectourinary fistulae allowing opacification of 2 bladders (Fig. 3). Ultrasonographic examination showed a bilateral ureteral dilatation and 2 bladders with no presacral mass. The intravenous urography showed duplicated bladder and urethra and confirmed bilateral ureteral dilatation. These findings were confirmed by an endoscopic examination, which revealed 2 rectourethral fistulae. There was only 1 vagina, which was atretic at its distal portion. Surgical correction was planned, but unfortunately, the patient died before this could be implemented.
2. Discussion Gastrointestinal duplication is an infrequent congenital abnormality. The reported incidence is 1 in 5000 live births [2,4]. It represents only 0.1 % to 0.3 % of all malformations [5,6]. Colonic localization accounts only for 4% to 18 % of all gastrointestinal duplication [7]. Duplications of the colon are most commonly isolated cystic structures, but rarely are they complete, side-by-side duplications of the entire colon, including the rectum. They can occur at any age, but most are discovered during the neonatal period or infancy particularly when they are associated with anorectal or urogenital malformation [6,8,9]. In our series, no case of CTCD was
Perinatally discovered complete tubular colonic duplication
E21
Fig. 2 A and B, Contrast enema studies (case 2) showed a CTCD that communicated proximally at the level of the ileocecal junction. Distally both segments were not fused resulting in a cul-de-sac twin limbs with both segments ending as fistulas to the urethras.
diagnosed in antenatal period. The 3 cases (2 girls and 1 boy) were diagnosed at the neonatal period because of their associated anal atresia. The precise etiology of these anomalies is still unclear. Proposed theories to explain duplications of the gastrointestinal tract are failure of regression of embryonic diverticula, sequestration of bowel during development, atavism, median septum development, partial twinning theory, and split notochord syndrome [8]. As noted, duplications that affect the large intestine are commonly associated with genitourinary and anorectal malformations. A more severe condition termed caudal duplication syndrome has also been described for cases in which there are associated gastrointestinal, genitourinary, and distal neural tube defects as found in our third case [10-13]. Total tubular colonic duplications may be asymptomatic and may remain undiagnosed for years or diagnosed later incidentally (60%-80 %). In cases of isolated colonic duplication, the diagnosis is invariably delayed until symptoms alert clinicians and prompt diagnostic evaluation is undertaken [14]. If symptomatic, they manifest by obstruction, bleeding, constipation, and perforation. Few cases revealed by malignancy are also reported [15]. In cases where associated congenital anomalies are present, the duplication is usually detected early. Female patients have a high prevalence of rectogenital fistulas and associated duplications of internal or external genital structures, whereas male patients have a high prevalence of rectourinary fistulas and duplication of external genitalia. Colorectal tubular duplications are commonly associated with vertebral anomalies as well as other gastrointestinal and skeletal malformations regardless of the patient's sex [8,16]. Yousefzadeh et al [17] described 57 patients with colon duplications of which 80% had associated anomalies. Our
series demonstrates imperforate anus in all 3 cases. In addition, CTCD was associated with a duplicated vagina in the first case, an appendicular duplication in the second case, and a caudal duplication syndrome in the third case. The neighboring colon in complete colonic duplication either terminates with a blind pouch or opens via rectourinary or rectovaginal fistulas [18,19]. Rectourinary fistulas were noted in cases 2 and 3. Barium contrast enema studies are considered essential for the diagnosis of tubular colonic duplication with opacification of 2 colons being the diagnostic sign [17]. In male infants who have a rectourethral fistula, improved
Fig. 3 Contrast study via the distal colostomy orifices (case 3): CTCD with both limbs ending distally separately in 2 enterourinary fistulae allowing opacification of 2 bladders.
E22 visualization of the duplication is achieved during cystourethrography or a retrograde urethrogram. In our series, colonic opacification was performed after surgical colostomy, showing CTCD in all cases with signs of enterourinary fistulas demonstrated in 2 cases. Other imaging modalities such as ultrasound, computed tomography, and magnetic resonance imaging may be helpful with cystic duplications, but in instances of tubular duplication ultrasonography computed tomography, or magnetic resonance imaging may be used in the investigation of the commonly occurring associated anomalies [8,20,21]. Endoscopy can have an interesting role for both the diagnosis and the treatment. The endoscopic examination under general anesthesia carried out in the third patient showed a complex cloacal malformation and revealed 2 rectourethral fistulae as well as a partial vaginal atresia. Indications for surgical intervention often arise in an acute setting, in the form of complications. [21-24]. The possibility of malignant degeneration within the duplication was reported [25-28]. The methods of surgical management are dependent primarily on size, location, communication with the gastrointestinal tract, and involvement of mesenteric vasculature [29,30]. Surgery is not indicated for an uncomplicated colonic duplication alone. It must be remembered that the treatment of this benign lesion must aim to eliminate symptoms and prevent complications; therefore, the surgical procedure should not be more radical than necessary. In most cases, resection of the supernumerary colon or microcolon is impossible because the 2 colons often share a common blood supply and, in most cases, there is only a single mesentery. The simplest approach is to perform a single anastomosis between the duplications, and it was first described by Yucesan et al [18]. Complete resection of the duplication is possible if both colic vascular supplies are separated, but this is exceptional (7% of cases). In our series, for the first 2 cases, treatment consisted of excision of the distal common septum with creation of a common channel followed by the lowering (pull through) of 2 duplicated colons by the technique of Stephens in the first case and Pena and Devries (posterior sagittal anorectoplasty) in the second case. The third patient died before the definitive surgical treatment could be implemented. Surgical treatment by excision of the distal common septum with creation of a common channel should prevent accumulation of feces in the false lumen and hopefully avoid constipation [31]. Mortality of CTCD is low: 4% to 8%. It is mainly seen in forms associated with severe malformations or with malignant transformation. Total tubular colonic duplication treated before the onset of complications generally have a favorable prognosis, hence the importance of an early diagnosis. Complete colonic duplications are very rare and usually associated with genitourinary duplications and complex congenital anomalies. Our series of 3 cases illustrates that
M.A. Jellali et al. CTCD presents perinatally in association with anal atresia, with or without other associated anomalies.
References [1] Kaur N, Nagpal K, Sodhi P, et al. Hindgut duplication—case report and literature review. Pediatr Surg Int 2004;20:640-2. [2] Craigie RJ, Abbaraju JS, Ba'ath ME, et al. Anorectal malformation with tubular hindgut duplication. J Pediatr Surg 2006;41:e31-4. [3] Peña A, Devries PA. Posterior sagittal anorectoplasty: important technical considerations and new applications. J Pediatr Surg 1982;17 (6):796-811. [4] Kokoska ER, Steinhardt GF, Tomita SS, et al. Prostatorectal fistula asscoaited with tubular colorectal duplication. J Pediatr Surg 1999;34: 1546-8. [5] Becmeur F, Viville B, Langer B, et al. Prenatal and neonatal management of digestive tract duplications. Diagnostic difficulties and therapeutic implications. J Gynecol Obstet Biol Reprod (Paris) 1999;28:388-92. [6] Noel L, Becmeur F, Jacques C, et al. Multiple gastrointestinal tract duplication: a neonatal case report. J Radiol 2001;82:676-8. [7] Gopal SC, Gangopadhyay AN, Gupta DK, et al. A unique presentation of atypical complete duplication of terminal ileum, colon, rectum, and urinary bladder. J Pediatr Surg 1997;32:1250-1. [8] Macpherson RI. Gastrointestinal tract duplications: clinical, pathologic, etiologic, and radiologic considerations. Radiographics 1993;13: 1063-80. [9] Alahyane A, Echarrab M, Lachkar A, et al. Colonic duplication associated with a common mesenterium revealed by cecal volvulus in a young adult. Gastroenterol Clin Biol 2009;33:499-501. [10] Shah KR, Joshi A. Complete genitourinary and colonic duplication: a rare presentation in an adult patient. J Ultrasound Med 2006;25: 407-11. [11] Banu T, Chowdhury TK, Hoque M, et al. Congenital double anus with total colon duplication: a case report. J Pediatr Surg 2007;42:E1-2. [12] McNeill SA, Rance CH, Stewart RJ. Fecolith impaction in a duplex vermiform appendix: an unusual presentation of colonic duplication. J Pediatr Surg 1996;31:1435-7. [13] McPherson AG, Trapnell JE, Airth GR. Duplication of the colon. Br J Surg 1969;56:138-42. [14] Blickman JG, Rieu PH, Buonomo C, et al. Colonic duplications: clinical presentation and radiologic features of five cases. Eur J Radiol 2006;59:14-9. [15] Bakir B, Gokce S, Dursun M, et al. An unusual cause of diffuse ascites in an infant: colonic duplication associated with bladder duplication. Diagn Interv Radiol 2009;15:210-1. [16] Kumar A, Kumar J, Gadodia A, et al. Multiple short-segment colonic duplications. Pediatr Radiol 2008;38:567-70. [17] Yousefzadeh DK, Bickers GH, Jackson JH, et al. Tubular colonic duplication—review of 1876-1981 literature. Pediatr Radiol 1983;13: 65-71. [18] Yucesan S, Zorludemir U, Olcay I. Complete duplication of the colon. J Pediatr Surg 1986;21:962-3. [19] Hunter TB, Tonkin IL. Complete duplication of the colon in assoication with urethral duplication. Gastrointest Radiol 1979;4:93-5. [20] Berrocal T, Lamas M, Gutieerrez J, et al. Congenital anomalies of the small intestine, colon, and rectum. Radiographics 1999;19:1219-36. [21] Misra S, Chaudhary A, Wakhlu A. Unusual CT sign of colonic duplication in a child. Pediatr Radiol 2006;36:462. [22] Stylianos S, Berdon WE, Hicks BA, et al. Complex colon duplication mimicking an obstructed, non-functioning kidney in a newborn with imperforate anus and spinal dysraphism. Pediatr Radiol 1995;25: 269-71. [23] Soper RT. Tubular duplication of the colon and distal ileum: case report and discussion. Surgery 1968;63:998-1004.
Perinatally discovered complete tubular colonic duplication [24] Schwartz DL, Becker JM, Schneider KM, et al. Tubular duplication with autonomous blood supply: resection with preservation of adjacent bowel. J Pediatr Surg 1980;15:341-2. [25] Birnbaum W. Squamous cell carcinoma and adenoacanthoma of the colon. JAMA 1970;212:1511-3. [26] Heiberg ML, Marshall KG, Himal HS. Carcinoma arising in a duplicated colon. Case report and review of literature. Br J Surg 1973; 60:981-2. [27] Hickey WF, Corson JM. Squamous cell carcinoma arising in a duplication of the colon: case report and literature review of squamous
E23
[28] [29] [30] [31]
cell carcinoma of the colon and of malignancy complicating colonic duplication. Cancer 1981;47:602-9. Lee J, Jeon YH, Lee S. Papillary adenocarcinoma arising in a duplication of the cecum. Abdom Imaging 2008;33:601-3. Grosfeld JL, O'Neill Jr JA, Clatworthy Jr HW. Enteric duplications in infancy and childhood: an 18-year review. Ann Surg 1970;172:83-90. Gross RE, Holcomb Jr GW, Farber S. Duplications of the alimentary tract. Pediatrics 1952;9:448-68. Fuchs JR, Clark K, Breckler FD, et al. Complete colonic duplication— a case report. J Pediatr Surg 2008;43:E11-3.