CASE REPORT SUPRAPUBIC DISCHARGING SINUS ASSOCIATED WITH CLITORAL
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ABSTRACT SUPRAPUBIC DISCHARGING SINUS ASSOCIATED WITH CLITORAL CLEFT AND PUBIC DIASTASIS: A VARIANT OF BLADDER EXSTROPHY Key words Bladder exstrophy, Exstrophy variants, Duplicate bladder exstrophy. INTRODUCTION: Variants of classic bladder exstrophy are extremely rare, making up 8% of all exstrophy/epispadias complex. 1 Another name for these lesion is “split symphysis” variants. 2 These have all the usual musculoskeletal findings of classic exstrophy, however the urinary bladder is closed with varying degrees of skin and subcutaneous cover, and the urethra and sphincter mechanism may be intact. 1,3 Three main variants are superior vesical fissure, covered exstrophy and duplicate exstrophy. CASE REPORT: Seven months old girl presented in a private clinic with complaint of small opening in the suprapubic area which was noted at birth. Occasionally pus discharged from the opening. She voided from the normal urethral opening with a normal stream. On examination there was a moist, soft mucosal area about 2-cm in diameter in the suprapubic area. Below it was cleft mons pubis and cleft clitoris. Umbilicus was normally placed (Figure Ia & b). A pelvic x-ray showed a 4-cm pubic symphysis diastsis. A voiding cystourethrogram demonstrated a normal bladder and urethra without reflux or any outside communication (Figure II). Contrast x-ray Correspondence: Dr. Yaqoot Jahan Department of Paediatric Surgery Civil Hospital and DUHS, Karachi through the opening showed irregular shaped tubular structure, not communicating with bladder (Figure III). Cystoscopy could not be done. Surgical excision of the sinus was done through an elliptical incision. The tract extended through the rectus sheath and found lying blindly over the bladder surface .There was no communication with bladder. Bladder was accidentally opened and repaired. Postoperative recovery was smooth. Histopathologic examination of the exstrophic mucosal area revealed transitional epithelium with focal chronic inflammatory infiltrate and fibromuscular layer with frequent nerve bundles and adipose tissue consistent with soft tissue of urinary bladder. DISCUSSION: Duplicate exstrophy is one of the rarest variant and only 23 cases have been reported in the English literature till 2009. 4 Duplicate bladder exstrophy consists of an exposed patch of exstrophic bladder with a normal or smaller than normal intact bladder deep to the exstrophic bladder, a musculoskeletal defect, and an occasional epispadias. There is no urinary communication to the exstrophied component. 3 The possible embryological cause behind exstrophy anomalies is thougth to be incomplete closure of the infraumbilical abdominal wall. A low lying umbilicus, separated pubic rami, extroversion and nonclosure of the bladder, with or without epispadias, are proposed to result from abnormal persistence of cloacal membrane. 5 A variant of the exstrophic urinary bladder (duplicate bladder exstrophy) presenting with a discharging sinus in suprapubic region in a seven months old continent girl is being reported. The infant also had cleft mons pubis, cleft clitoris and pubic diastasis. VCUG was normal. Contrast x-ray through discharging sinus showed a blind tract which was excised at surgery. It extended through the rectus sheath and ended blindly at bladder surface, which itself was intact. Histopathology showed transitional epithelium with fibromuscular layer. Journal of Surgery Pakistan (International) 15 (2) April - June 2010 CASE REPORT YAQOOT JAHAN 115
Transcript of CASE REPORT SUPRAPUBIC DISCHARGING SINUS ASSOCIATED WITH CLITORAL
A B S T R A C T
SUPRAPUBIC DISCHARGING SINUSASSOCIATED WITH CLITORAL CLEFTAND PUBIC DIASTASIS: A VARIANT
OF BLADDER EXSTROPHY
Key words Bladder exstrophy, Exstrophy variants, Duplicate bladder exstrophy.
INTRODUCTION:Variants of classic bladder exstrophy are extremelyrare, making up 8% of all exstrophy/epispadiascomplex.1 Another name for these lesion is “splitsymphysis” variants.2 These have all the usualmusculoskeletal findings of classic exstrophy,however the urinary bladder is closed with varyingdegrees of skin and subcutaneous cover, and theurethra and sphincter mechanism may be intact.1,3
Three main variants are superior vesical fissure,covered exstrophy and dupl icate exstrophy.
CASE REPORT:Seven months old girl presented in a private clinicwith complaint of small opening in the suprapubicarea which was noted at birth. Occasionally pusdischarged from the opening. She voided from thenormal urethral opening with a normal stream. Onexamination there was a moist, soft mucosal areaabout 2-cm in diameter in the suprapubic area. Belowit was cleft mons pubis and cleft clitoris. Umbilicuswas normally placed (Figure Ia & b).
A pelvic x-ray showed a 4-cm pubic symphysisdiastsis. A voiding cystourethrogram demonstrateda normal bladder and urethra without reflux or anyoutside communication (Figure II). Contrast x-ray
Correspondence:Dr. Yaqoot JahanDepartment of Paediatric SurgeryCivil Hospital and DUHS,Karachi
through the opening showed irregular shapedtubular structure, not communicating with bladder(Figure III). Cystoscopy could not be done. Surgicalexcision of the sinus was done through an ellipticalincision. The tract extended through the rectus sheathand found lying blindly over the bladder surface.There was no communication with bladder. Bladderwas accidentally opened and repaired. Postoperativerecovery was smooth.
Histopathologic examination of the exstrophicmucosal area revealed transitional epithelium with
focal chronic inflammatory infiltrate and fibromuscularlayer with frequent nerve bundles and adipose tissueconsistent with soft tissue of urinary bladder.
DISCUSSION:Duplicate exstrophy is one of the rarest variant andonly 23 cases have been reported in the Englishliterature till 2009.4 Duplicate bladder exstrophyconsists of an exposed patch of exstrophic bladderwith a normal or smaller than normal intact bladderdeep to the exstrophic bladder, a musculoskeletaldefect, and an occasional epispadias. There is nour inary communicat ion to the exst rophiedcomponent.3 The possible embryological causebehind exstrophy anomalies is thougth to beincomplete closure of the infraumbilical abdominalwall. A low lying umbilicus, separated pubic rami,extroversion and nonclosure of the bladder, withor without epispadias, are proposed to resultfrom abnormal persistence of cloacal membrane.5
A variant of the exstrophic urinary bladder (duplicate bladder exstrophy) presenting witha discharging sinus in suprapubic region in a seven months old continent girl is beingreported. The infant also had cleft mons pubis, cleft clitoris and pubic diastasis. VCUG wasnormal. Contrast x-ray through discharging sinus showed a blind tract which was excisedat surgery. It extended through the rectus sheath and ended blindly at bladder surface,which itself was intact. Histopathology showed transitional epithelium with fibromuscularlayer.
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This infraumbilical cloacal membrane acts as awedge and prevents the lateral mesoderm fromprogressing medially between its ectodermal and
endodermal layers. Rupture of this unstablemembrane results in an absent lower abdominalwall and an exposed bladder with epispadias.Exstrophy variants are explained by incompleterupture or persistence of the abnormal cloacalmembrane.6
Treatment is gratifying, because the patients havenormal internal urogenital structures. The difficultproblems with urinary continence with bladderexstrophy may not exist with variants of the exstrophycomplex.7 Careful examination with fluoroscopy andcystoscopy is helpful in defining the anatomy inthese variants.
REFERENCES:
1. Turner WR, Ransley PG, Bloom DA, et al.Variants of the exstrophy complex. Urol ClinNorth Am 1980;7:493-501.
2. Williams DI. Split symphysis variants, inWilliams DI (Ed): Encyclopedia of Urology.,Ch 15, New York, NY, Springer Verlag, 1974,p 2779.
3. Marshall VF, Muecke EC. Variations inexstrophy of the bladder. J Urol 1962;88:766-96.
4. Kumar B, Sherma C, Sinha DD. Trueduplicate bladder exstrophy: A rare lesion.Indian J Pediatr 2009;76;852-3.
5. Jeffs RD: Exstrophy of the urinary bladder,in Welch KJ, Randolph JG, Ravitch MM, etal(eds):Pediatric Surgery. Chicago, IL, YearB o o k M e d i c a l 1 9 8 6 , p p 1 2 1 6 - 4 1 .
6. Boemers TML, de Jong TPVM, RovekampMH, et al. Covered exstrophy associatedwith an anorectal malformation; A rare variantoF classic bladder exstrophy. Pediat SurgInt 1994; 9:438-40.
7. Gearhart JP, Mathews R. Exstrophy-epispadiascomplex. In: Wein AJ, Kavoussi LR, Novick AC,et al, (editors), Campbell-Walsh urology. 9th ed.Philadelphia: Saunders Elsevier; 2007.pp 3348-50.
Journal of Surgery Pakistan (International) 15 (2) April - June 2010
Suprapubic Discharging Sinus Associated with Clitoral Cleft and Pubic Diastasis: A Variant of Bladder Exstrophy
Fig I a & b: Cleft mons pubis and cleft clitons withexstrophied bladder mucosa.
Fig II: Normal VCUG with pubic diastasis.
Fig III: Sinogram showing blind tract.
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