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ORIGINAL ARTICLE
Year : 2019  |  Volume : 24  |  Issue : 2  |  Page : 104-108
 

Radiologic evaluation of uterovaginal abnormalities in girls with congenital pouch colon


1 Department of Pediatric Surgery, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, New Delhi, India
2 Department of Radiology, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, New Delhi, India

Date of Web Publication1-Mar-2019

Correspondence Address:
Dr. Rajiv Chadha
G-123 Vikaspuri, New Delhi - 110 018
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_33_18

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   Abstract 


Background/Purpose: The radiologic investigations of 25 girls with congenital pouch colon (CPC), managed over 17 years, were retrospectively reviewed. In 13 girls who form the study group, the investigations provided information about the anomalous uterovaginal (UV) anatomy and these findings were studied.
Materials and Methods: Age at presentation was 2 months to 10 years. The subtypes of CPC were Type I (n = 2), Type II (n = 9), and not recorded (n = 2). All patients had a double vagina and a unicornuate uterus on each side in the pelvis. The radiologic studies, performed at varying periods after surgery, included an intravenous urogram (IVU) (n = 4), micturating cystourethrogram (MCU) (n = 3), distal ileostogram/colostogram (n = 6), and magnetic resonance imaging (MRI) (n = 7).
Results: IVU and MCU showed retrograde filling of the vaginas with contrast during micturition with a small-capacity urinary bladder and a relatively open bladder neck. The two vaginas were quite apart, but symmetrical in appearance and position. A distal dye study showed filling of the colonic pouch, its terminal fistula, and the two vaginas in six patients. Opacification of the bladder was seen in 3/6 girls, including one girl with left-sided Grade IV vesicoureteral reflux. MRI (n = 7) showed a monocornuate uterus on each side in the pelvis. The upper vaginas on each side were apart, being widely apart (n = 5) and somewhat closer (n = 2). The lower vaginas were closer with an intervaginal septum. Other findings were a widely open bladder neck and urethra in two girls with urinary incontinence and visualization of the terminal fistula of the colonic pouch (n = 2). The lumbosacral spine was normal in all patients.
Conclusions: In girls with CPC, retrograde reflux of contrast into the vaginas during a distal dye study or an IVU/MCU may provide useful details of the anatomy of the vaginas. An MRI scan is recommended as essential for comprehensive evaluation of the anomalous UV anatomy.


Keywords: Anorectal malformation, congenital pouch colon, monocornuate uterus, vaginal duplication


How to cite this article:
Khan NA, Pant N, Gupta A, Anand R, Yadav PS, Chadha R, Choudhury SR. Radiologic evaluation of uterovaginal abnormalities in girls with congenital pouch colon. J Indian Assoc Pediatr Surg 2019;24:104-8

How to cite this URL:
Khan NA, Pant N, Gupta A, Anand R, Yadav PS, Chadha R, Choudhury SR. Radiologic evaluation of uterovaginal abnormalities in girls with congenital pouch colon. J Indian Assoc Pediatr Surg [serial online] 2019 [cited 2019 Jul 24];24:104-8. Available from: http://www.jiaps.com/text.asp?2019/24/2/104/253342





   Introduction Top


Congenital pouch colon (CPC) is an uncommon malformation, most commonly reported from North or North-Western India, in which a pouch-like dilatation of a shortened colon is associated with an anorectal malformation.[1] CPC has been categorized into four subtypes (Types I–IV) based on the length of normal colon proximal to the colonic pouch.[2] The anomalous clinical anatomy of CPC in girls, especially that of the genitourinary tract, has been described in detail in two earlier reports from our center.[3],[4] Important findings included an essentially normal appearance of the external genitalia with a wide posterior margin of the vestibule and double vaginas with a wide intervaginal bridge.[3],[4] The vaginas often appeared tilted, meeting the openings of the urethra and the terminal fistula of the colonic pouch medially at a more cranial point of confluence. The fistula opened either in the urethra or in the vestibule, usually just posterior to the urethral opening, in an intervaginal position.[3],[4] There was a high incidence of urinary incontinence (81%) associated with a small-capacity urinary bladder, an open bladder neck and a short, wide urethra.[4] Surgery showed a monocornuate uterus on each side in the pelvis, flanking the terminal portion of the colonic pouch.[3],[4] Similar findings, although not in large series of patients, have also been reported by other authors.[5],[6],[7],[8],[9],[10] However, radiologic imaging for a more detailed study of the uterovaginal (UV) anatomy has not been described in these reports[3],[4],[5],[6],[7],[8],[9],[10] or elsewhere in the literature.

In the present study, the records of 13 girls with CPC in whom radiologic studies provided information regarding the UV anatomy were reviewed and analyzed to confirm the clinical findings described earlier[3],[4] as well as obtain a more complete picture of the anomalous anatomy. A detailed knowledge of the UV anatomy is essential as these girls would necessarily be requiring gynecologic and obstetric interventions later in life.


   Materials and Methods Top


The results of the radiological investigations of 25 girls with CPC, managed during a 17-year period (January 2000 to January 2017), were retrospectively reviewed. The patients had undergone a variety of preliminary and definitive surgical procedures, and the relevant radiological studies had been performed at varying periods after the initial or definitive surgery. Our protocol is to perform a micturating cystourethrogram (MCU) in patients with ureteric dilatation on abdominal ultrasound (US) or else for evaluation of the lower urinary tract in girls with urinary incontinence. A distal dye study is performed in patients with a diverting stoma proximal to the colonic pouch for studying the anatomy of the pouch and its terminal fistula. A magnetic resonance imaging (MRI) scan of the abdomen and pelvis is performed in older girls. However, due to logistical reasons, especially lack of facilities, it was often not possible to perform all required radiologic investigations in each case. An intravenous urogram (IVU) had been performed only in patients managed during the early years of the study or had already been performed in patients referred after initial surgery at another center. The radiological studies included an IVU (n = 8), MCU (n = 12), a distal ileostogram/colostogram (n = 11), and MRI scan (n = 7). A review of these studies showed details of the anomalous UV anatomy on an IVU in 4/8 (50%) patients, an MCU in 3/12 (25%) patients, a distal dye study in 6/11 (54.5%) patients, and MRI in all 7 patients. The total number of patients in whom these studies showed important findings was 13 and these patients form the study group. These findings were analyzed, and other relevant findings from the case files of the 13 patients were also recorded.

The girls in the study group had either been referred to our center with a definitive diagnosis of CPC after preliminary surgery performed elsewhere (n = 2) or been presented initially at our institution (n = 11). The age at presentation at our center ranged from 2 months to 10 years. The subtypes of CPC were Type I (n = 2) and Type II (n = 9), while, in two cases, the subtype had not been recorded. The records showed that the terminal fistula of the colonic pouch opened in the proximal urethra just distal to the bladder neck (n = 2), the distal urethra close to the urethral opening (n = 3), and the vestibule just posterior to the urethra (n = 5), while, in four patients, the fistulous opening could not be identified.


   Results Top


On plain X-rays, the sacrum was normal in all 13 patients. Abdominal US for evaluation of the urinary tract was normal in 12 girls while mild unilateral dilatation of the renal pelvis was noted in one patient. Gas foci within the bladder were seen in one girl.

Intravenous urogram and micturating cystourethrogram studies

In four girls, an IVU showed a small-capacity urinary bladder, a relatively open bladder neck, and retrograde filling up of the two vaginas during micturition [Figure 1]. The two vaginas appeared quite apart, especially cranially, but were symmetrically placed on each side of the midline. Additional findings included a unilaterally bifid renal pelvis in two patients. Similar findings were noted in three girls in whom MCU revealed a small bladder, a funnel-shaped bladder neck, and retrograde filling of the vaginas with contrast.
Figure 1: Intravenous urogram in a 4-year-old girl showing small capacity urinary bladder, wide urethra, and filling of vaginas with contrast

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Distal ileostogram/colostogram (n = 6)

In five patients, an ileostomy and in one patient a colostomy had been constructed proximal to the colonic pouch. In two of the six patients, the colonic pouch had been tubularized earlier but had not been disconnected from its terminal fistula. In three patients, the dye study showed filling up of the colonic pouch, its terminal fistula, and the two vaginas with contrast. In another three patients, the colonic pouch, the two vaginas as well as the urinary bladder were visualized [Figure 2] and [Figure 3] with, in one of these patients, evidence of left-sided Grade IV vesicoureteral reflux [Figure 2]. The typical appearance on an anteroposterior (AP) view was of a large colonic pouch. Where the vaginas were visualized, two widely separated vaginal shadows on either side of the midline were seen, flanking the colonic pouch which tapered in the midline between the vaginas [Figure 2]. Due to the overlapping the bladder shadow by the contrast-filled vaginas in lateral films, the anatomical picture was clearer on AP films.
Figure 2: Distal ileostogram showing a Type II colonic pouch with filling of the vaginas and the urinary bladder with left-sided Grade IV vesicoureteral reflux. V-vagina

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Figure 3: Distal colostogram in a girl with a tubularized Type II colonic pouch without disconnection of the vestibular fistula. Filling of the tubularized colon, the bladder, and the vaginas seen. U Bl-urinary bladder; V-vagina

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Magnetic resonance imaging (n = 7)

MRI was performed for assessment of the UV anatomy in seven girls above the age of 10 years. In all patients, there was a monocornuate uterus on each side in the pelvis. The upper vaginas on each side were apart in all patients, being widely apart in five patients [Figure 4] and somewhat closer in two patients [Figure 5]. The lower vaginas were closer together with an intervaginal septum [Figure 4] and [Figure 5]. Coronal cuts suggested a widely open bladder neck and wide urethra in two patients, both of whom had urinary incontinence. The terminal remnant of the fistula from the colonic pouch could be identified in two patients in whom it had been ligated just above the peritoneal reflexion [Figure 6]. The lumbosacral spine was normal in all patients and there was no intraspinal abnormality.
Figure 4: Magnetic resonance imaging scan of a 10-year-old girl. (a) Coronal section showing a monocornuate uterus each side with widely separated upper vaginas, and (b) transverse T2-weighted image showing the bladder, the two uteruses, and posteriorly the previously tapered colon (arrowhead). Ut-uterus; V-vagina

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Figure 5: Coronal section on magnetic resonance imaging scan of a 7-year-old girl. The upper vaginas are closer together. Ut-uterus; V-vagina

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Figure 6: Sagittal section of magnetic resonance imaging scan showing the distal remnant of the colonic pouch and its terminal fistula (arrow) just posterior to the urinary bladder and urethra. Note the normal sacrum

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   Discussion Top


The results of the present study confirm the clinical and operative findings reported earlier[3],[4] and provide important additional information about the anomalous UV anatomy in girls with CPC.

An interesting finding on a distal ileostogram/colostogram in patients with a proximal diverting stoma was that, in addition to filling the colonic pouch that tapered distally to the midline fistula, retrograde reflux of contrast delineated the widely separated vaginas in six patients, including three patients in whom the urinary bladder was also opacified. In these three patients, therefore, the study provided a very useful complete picture of the anatomy of the lower genitourinary tract. Due to the presence of widely separated lateral but symmetrically placed vaginas overlapping the bladder in lateral films, the anatomical picture was clearer on AP films. A similar retrograde filling of the vaginas was also seen in four patients during an IVU study and in three patients during an MCU. These findings can be explained by the anomalous anatomy, especially the presence of the fistula just posterior to the urethra, the tilt of the vaginas, and the high or cranial confluence of the perineal openings.[3],[4] This would allow reflux of contrast into the vaginas during the micturition phase of an IVU/MCU study as well as during a distal ileostogram or colostogram.

Perhaps the most interesting and useful findings were seen in MRI scans of the abdomen and pelvis in older girls. These findings are especially important as the anomalous UV anatomy, especially the degree of separation of the upper vaginas, cannot be assessed with certainty during surgery for the primary anomaly. The monocornuate uterus on each side in all patients with the often widely separated upper vaginas (5/7; 71.4%) seen clearly on MRI scanning appears unique to this malformation and has major obstetric implications.[4] In addition, the anatomy of the lower urinary tract can be evaluated on coronal sections in T2-weighted images, the terminal fistula of the colonic pouch can be delineated, and MRI scanning can also evaluate the sacrum and rule out intraspinal abnormalities. An important conclusion from this study is that, in CPC, the abnormalities of the genital organs appear symmetric and similar on each side with the fistula tapering gradually between the two vaginas in the midline, unlike the more complex UV and urinary tract abnormalities often reported with persistent cloaca.[11],[12]


   Conclusions Top


This study provides valuable confirmation of the clinical and operative findings as well as additional information regarding the anomalous UV anatomy in girls with CPC. Retrograde reflux of contrast into the vaginas during a distal dye study via a proximal stoma or during an MCU may provide useful details of the anatomy and positioning of the vaginas in some patients. Although costs as well as the difficulty of performing MRI scans in younger children are limiting factors, an MRI scan is recommended as essential for comprehensive evaluation of the UV anatomy. This is important for optimal planning of the obstetric and gynecologic counseling and management that would be required at puberty and later in life.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Chadha R, Bagga D, Malhotra CJ, Mohta A, Dhar A, Kumar A, et al. The embryology and management of congenital pouch colon associated with anorectal agenesis. J Pediatr Surg 1994;29:439-46.  Back to cited text no. 1
    
2.
Narasimharao KL, Yadav K, Mitra SK, Pathak IC. Congenital short colon with imperforated anus (pouch colon syndrome). Ann Pediatr Surg 1984;1:159-67.  Back to cited text no. 2
    
3.
Chadha R, Choudhury SR, Pant N, Jain V, Puri A, Acharya H, et al. The anomalous clinical anatomy of congenital pouch colon in girls. J Pediatr Surg 2011;46:1593-602.  Back to cited text no. 3
    
4.
Chadha R, Khan NA, Shah S, Pant N, Gupta A, Choudhury SR, et al. Congenital pouch colon in girls: Genitourinary abnormalities and their management. J Indian Assoc Pediatr Surg 2015;20:105-15.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Trusler GA, Mestel AL, Stephens CA. Colon malformation with imperforate anus. Surgery 1959;45:328-34.  Back to cited text no. 5
    
6.
Demirogullari B, Ozen IO, Afsarlar C, Moralioglu S, Poyraz A, Sonmez K, et al. Congenital pouch colon associated with anorectal malformation: Report of 2 cases. J Pediatr Surg 2007;42:E13-6.  Back to cited text no. 6
    
7.
Gharpure V. Our experience in congenital pouch colon. J Indian Assoc Pediatr Surg 2007;12:22-4.  Back to cited text no. 7
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8.
Patil RT, Gupta RK, Sanghvi B. Unusual variants of cloaca- six cases. J Indian Assoc Pediatr Surg 2009;14:156.  Back to cited text no. 8
    
9.
Kurt G, Celayir AC, Sarika K. The importance of the stoma leveling of pouch colon in cases with persistent cloaca. Presented at the 25th Annual Conference of the Egyptian Pediatric Surgical Association (EPSA) and Turkish Association of Pediatric Surgeons (TAPS) and the South Egypt Cancer Institute (SECI), Luxor, Egypt, 8th - 11th December 2009. CILT 42YIL 2011;1:53-7.  Back to cited text no. 9
    
10.
Uotani C, Ishimaru T, Konishi K, Fujishiro J, Sugiyama M, Komuro H, et al. A case of a girl with congenital pouch colon. JJSPS 2014;6:808-13.  Back to cited text no. 10
    
11.
Hendren WH. Cloaca, the most severe degree of imperforate anus: Experience with 195 cases. Ann Surg 1998;228:331-46.  Back to cited text no. 11
    
12.
Breech L. Gynecologic concerns in patients with cloacal anomaly. Semin Pediatr Surg 2016;25:90-5.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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