Home | About Us | Current Issue | Ahead of print | Archives | Search | Instructions | Subscription | Feedback | Editorial Board | e-Alerts | Login 
Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
 Users Online:1408 
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size

Table of Contents   
Year : 2011  |  Volume : 16  |  Issue : 1  |  Page : 26-28

Covered exstrophy with anorectal malformation and vaginal duplication

Department of Pediatric Surgery, Advanced Pediatric Centre, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India

Date of Web Publication3-Jan-2011

Correspondence Address:
J K Mahajan
Department of Pediatric Surgery, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh - 160 012
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.74519

Rights and Permissions



Covered exstrophy is a rare variant of the exstrophy-epispadias complex. We report a female newborn with covered exstrophy, absent anal opening and duplication of the introitus and the lower vagina. This rare, previously unreported, combination of anomalies highlights the complexity of the embryological events in the caudal area during separation of the hindgut and allantois.

Keywords: Anorectal malformation, covered exstrophy, vaginal duplication

How to cite this article:
Singh JK, Mahajan J K, Bawa M, Rao K. Covered exstrophy with anorectal malformation and vaginal duplication. J Indian Assoc Pediatr Surg 2011;16:26-8

How to cite this URL:
Singh JK, Mahajan J K, Bawa M, Rao K. Covered exstrophy with anorectal malformation and vaginal duplication. J Indian Assoc Pediatr Surg [serial online] 2011 [cited 2023 Jun 8];16:26-8. Available from: https://www.jiaps.com/text.asp?2011/16/1/26/74519

   Introduction Top

Classical exstrophy of the bladder is a rare anomaly with an incidence of 1 in 30,000-50,000 live births. [1] Covered exstrophy is one of the variants of exstrophy-epispadias complex. There are a few case reports of covered exstrophy with anorectal malformation and the complete duplication of bladder, urethra, and the mullerian structures. [2],[3] However, covered exstrophy associated with duplication of introitus and the lower vagina has not been reported earlier in the English literature. We report one such baby.

   Case Report Top

A 2.6 kg, 2-day-old female child, delivered by cesarean section, was brought to us with malformed external genitalia and a swelling over the lower part of the abdomen and the perineum.

Physical examination revealed a lobulated bowel projection with a skin-covered swelling extending over the lower part of the abdomen and the perineum. The umbilicus was low set, situated just above the upper extent of the swelling. The pubic symphysis was widely separated with a bulging urinary bladder occupying the space in between. The introitus was replaced by the swelling which was continuing on to the lower abdomen. There was duplication of the introitus with two openings, each flanked by the single labia majora located laterally, in the groin area [Figure 1]A. The clitoris was bifid. The two orifices on either side were in communication with each other as a catheter introduced from one orifice exited through the other. The urethral orifice could not be seen from the outside; however, both the introitus openings were discharging urine intermittently. The anal opening was absent. An infantogram and cross-prone-lateral X-ray showed a high type of anorectal malformation and the absence of calcification in the area of accessory bowel loop.
Figure 1: (A) Duplicated introitus, absent anal opening and accessory bowel segment. (B) Neoanus after excision of accessory bowel segment

Click here to view

The baby was resuscitated and undertaken for surgery. A midline laparotomy showed that the small bowel and large bowel were normal and in continuity. The internal genitalia were female type and the uterus was unicornuate with normal  Fallopian tube More Detailss and the ovaries. The urinary bladder was intact although subcutaneous in location. The accessory bowel loop was attached to the lower part of the swelling and the lumen was in continuity with lumen of the terminal part of the rectum. The extra piece of the bowel was excised and the terminal part of rectum fashioned as a neoanus [Figure 1]B. A suprapubic cystostomy (SPC) was also inserted as the anatomy of the bladder neck and urethra was still not clear. The rectus sheath in the upper part of the abdomen was closed primarily and the lower part containing the urinary bladder in subcutaneous location was left as such. The histopathologic examination of the sequestered loop revealed colonic mucosa. The baby was put on oral feeds after 48 h and the SPC was clamped. The baby was decompressing well from the perineal openings and was discharged from the hospital after removal of the SPC catheter. Postoperatively, the baby was put on dilatation program for the neoanus. The ultrasonography and radionuclide scans for the renal tract have shown normal upper tracts. The spine and spinal cord were normal radiologically. At the age of 6 months, the baby underwent genitoscopy examination which showed single urethral orifice with duplication of the lower part of the vagina. Bladder was unremarkable. At present, the patient is waiting for the genital reconstruction at a later age and is continent for urine.

   Discussion Top

Variants of the bladder exstrophy complex are quite uncommon and constitute about 8% of all the cases. These variant lesions, also called "Split symphysis variants", have all the musculoskeletal findings of classical exstrophy, but the bladder is closed with varying degrees of skin and the subcutaneous covering, and the urethra and sphincter mechanism may be intact. [4] Exstrophy variants have been classified into four major subgroups, namely pseudoexstrophy, superior vesical fissure, duplicate exstrophy, and covered exstrophy with visceral sequestration. [4],[5]

Classical bladder exstrophy is believed to result from the persistence or overdevelopment of an abnormal infraumbilical cloacal membrane that acts as a wedge and prevents the medial progression of the lateral mesoderm between its ectodermal and endodermal layers. [1] The exstrophy variants are explained by the incomplete rupture or persistence of this abnormal infraumbilical cloacal membrane. [6] Mildenberger et al., however, explained the occurrence of exstrophy and its variants to be the consequences of persistence of an abnormal caudal insertion of the body stalk on the embryo. [7]

Covered exstrophy seems to differ from the classical exstrophy not only in the degree of exposure of the bladder and urethra, but also by frequent presence of the abnormalities of the external genitalia. A partial diphallus with incomplete urethral duplication and laterally displaced phallus with widely separated scrotal halves associated with covered exstrophy have been reported by Mildenberger et al. [7] Genital abnormalities observed in females with covered exstrophy included bifid clitoris (67%), labial cleft (11%), hypoplastic labia (11%), and stenosed duplicate vagina (11%). [2] Covered exstrophy with complete duplication of the genital and the lower urinary tract with visceral sequestration have also been reported. [8] The embryological process in variants of exstrophy may not affect the development of the lower urinary tract significantly, but the so-called wedge effect may prevent fusion of superficial paired genital swellings as happened in our case too. [7] The process seems to affect only the superficial perineal structures leading to genital anomalies. The vaginal duplication in our case involved the introitus and the lower part of the vagina only, sparing the developmentally different upper vagina and the uterus.

Covered exstrophy differs from pseudoexstrophy variant in that, there is an associated isolated ectopic bowel segment present on the inferior abdominal wall near the genital area. This segment is usually a segment of the colon and has no communication with the underlying gastrointestinal tract. [1],[9],[10] However in the present case, the segment was communicating with the rectum and this has not been described earlier in the literature. It has been suggested that this condition is a result of the secondary closure of the cloacal membrane which isolates ectopic embryological colonic cells. [6]

Other associated gastrointestinal anomalies in covered exstrophy include anorectal malformation (40%). [2] Ten cases (six male and four female) of covered exstrophy with visceral sequestration have been reported in the world literature. [8],[11] None of the cases showed the presence of anorectal malformation.

As the continence in these patients is generally good, the treatment is required only for associated gastrointestinal or genital anomalies. Genital reconstruction in such patients is, however, easier than in classical exstrophy.

   References Top

1.Marshall VF, Muecke EC. Variations in exstrophy of the bladder. J Urol 1962;88:766-96.   Back to cited text no. 1
2.Sahoo SP, Gangopadhyay AN, Sinha CK, Gupta DK, Gopal SC. Covered exstrophy: a rare variant of classical bladder exstrophy. Scan J Urol Nephrol 1997;31:103-6.  Back to cited text no. 2
3.Kluwe W, Mau H. Duplication and Exstrophy of the Bladder with Clitoris and Vagina Duplex as Well as Uterus Bicornis. Urology 2007;70:591.  Back to cited text no. 3
4.Narasimharao KL, Chana RS, Mitra SK, Pathak IC. Covered exstrophy and visceral sequestration: a rare exstrophy variant. J Urol 1985;133:274-5.  Back to cited text no. 4
5.Madhavi R. Covered exstrophy and visceral sequestration with complete double bladder. J Urol 1994;151:455-6.  Back to cited text no. 5
6.Weiss RE, Garden RJ, Cohen EL, Stone NN. Covered exstrophy with sequestrated colonic remnant. J Urol 1993;149:185-7.  Back to cited text no. 6
7.Mildenberger H, Kluth D, Dziuba M. Embryology of the bladder exstrophy. J Pediatr Surg 1988; 23:165-170.   Back to cited text no. 7
8.Kajbafzadeh AM, Aghdas FS, Tajik P. Complete covered duplication of the bladder, urethra, vagina, uterus and visceral sequestration. Int J Urol 2006;13:1129-31.  Back to cited text no. 8
9.Johnston JH. The exstrophic anomalies. In: Williams DI, Johnston JH, editors. Pediatric Urology. London: Butterworth Scientific; 1982. p. 299.   Back to cited text no. 9
10.Sheldon CA, McLorie GA, Khoury A, Churchill BM. Duplicate bladder exstrophy: a new variant of clinical and embryological significance. J Urol 1990;144:334-6.  Back to cited text no. 10
11.Sarin YK, Sinha A, Ojha S. Covered exstrophy with visceral sequestration: case report and review of literature. Eur J Pediatr Surg 2005;15:437-40.  Back to cited text no. 11


  [Figure 1]

This article has been cited by
1 New insights into the pathogenesis of bladder exstrophy–epispadias complex
Istiak Mahfuz,Tom Darling,Simon Wilkins,Stefan White,Wei Cheng
Journal of Pediatric Urology. 2013;
[Pubmed] | [DOI]


Print this article  Email this article


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (741 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

    Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded175    
    Comments [Add]    
    Cited by others 1    

Recommend this journal

Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer | Privacy Notice

 © 2005 - Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow 

Online since 1st May '05