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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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Table of Contents   
CASE REPORT
Year : 2019  |  Volume : 24  |  Issue : 2  |  Page : 141-143
 

Female hypospadias-need for clarity in definition and management


Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi, India

Date of Web Publication1-Mar-2019

Correspondence Address:
Dr. Yogesh Kumar Sarin
Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi - 110 002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_69_18

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   Abstract 


We report a 6-year-old girl with female hypospadias and discuss the definition and management of the entity.


Keywords: Cloaca, female hypospadias, urogenital sinus


How to cite this article:
Sarin YK, Kumar P. Female hypospadias-need for clarity in definition and management. J Indian Assoc Pediatr Surg 2019;24:141-3

How to cite this URL:
Sarin YK, Kumar P. Female hypospadias-need for clarity in definition and management. J Indian Assoc Pediatr Surg [serial online] 2019 [cited 2019 Jul 24];24:141-3. Available from: http://www.jiaps.com/text.asp?2019/24/2/141/253348





   Introduction Top


In contrast to male hypospadias, female hypospadias (FH) or “hypospadia feminis” is such a rare anomaly of the lower urogenital system that most textbooks fail even to mention it. Isolated FH, with no associated genitourinary abnormalities, is even rarer. We report this anomaly in a 6-year-old girl and briefly highlight the confusion and controversies that surround its definition and management.


   Case Report Top


A 6-year-old girl was admitted to our department with urinary incontinence since birth. On examination of the external genitalia, there was a solitary wide orifice in the vestibule [Figure 1].
Figure 1: Urethral orifice was not visualized on initial examination

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No other anomaly of the genitalia or the upper urinary tract was noted on preoperative workup; urine culture and renal function tests were normal.

Examination of the vestibule under anesthesia revealed that the urethral orifice opened widely at the anterior vaginal wall about 0.5 cm proximal to its vestibule [Figure 2]. Continuous leakage of urine was noted from both the urethral and vaginal orifices. These orifices could be individually catheterized. Urethrocystoscopy revealed a short urethra measuring 1 cm in length. There were no abnormalities detected in the bladder though the bladder neck appeared rather wide. A modification of Hendren's technique for the external urethral lengthening was employed.[1],[2] The modification included performing the surgery in lithotomy position instead of “sky-diver” position and using “advancement flap” of anteromedial part of the thigh just lateral to the left labium majus for the reconstruction of anterolateral part of the distal vagina instead of a “rotation flap” of buttock. The postoperative course was uneventful, and the child had the improvement of urinary continence at 6-week review.
Figure 2: Both the urethral and vaginal orifices could be demonstrated on examination under anesthesia; the urethral orifice opened at the anterior vaginal wall just close to the vestibule

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   Discussion Top


There is no unanimity of opinion about the terms “hypospadias” and “urogenital sinus” in females. Most of the surgeons use them interchangeably. Some have used the term “hypospadias” only in “very low anomalies” when the common channel is only 0.5–1.5 cm long. Others believe this abnormality represents the situation, where the urethral meatus is located at “any” site on the anterior vaginal wall from just above the introitus up (proximal to the hymenal ring) to the vaginal fornix. The FH was classified into three varieties by Solov'ev:[3] vestibular (partial), vestibulo-vaginal (subtotal), and vaginal (total). FH is even considered as a variant of cloacal anomaly or mentioned in the context of cloacal anomaly! Classification of anatomic variants of FH given by Derevianko et al. includes low-vaginal ectopia of the external urethral opening; high-vaginal ectopia of the external opening of the urethra; urovaginal (vesicovaginal) fusion of the neck of the urinary bladder with vagina accompanied with enuresis; urogenital sinus in females (ectopia of the external urethral opening in the urogenital sinus).[4]

We believe that the two entities reflect the different degrees of agenesis of the urethrovaginal septum. The term FH should be reserved for very low anomalies that open in the lower 1/3rd of the vagina or its vestibule, and the term “urogenital sinus” should be used for the situations where the urethral orifice opens in the upper 2/3rd of the vagina.

This differentiation has prognostic implications that relate to the caliber of the urethra. A urethra that has its meatus closer to the vestibule of the vagina, that is, FH, is more likely to have a normal caliber. The higher the urethral orifice on the anterior wall of the vagina (closer to the vaginal fornix), the narrower the urethra, and the presentation is more likely to be signs of urinary outflow obstruction, for example, distended urinary tract, urinary tract infection, obstructive nephropathy, and hypertension. This picture is more consistent with patients that we designate as “urogenital sinus anomaly.” Furthermore, the majority of children born with a urogenital sinus have genital ambiguity.

From an embryologic point of view, an arrest of caudal urogenital sinus development would lead to different degrees of hypospadias in males; similarly, in females, it would result in a deficient distal vaginal vestibule and FH. FH occurs due to partial or total agenesis of the urethrovaginal septum, resulting in urinary drainage into the genital tract.

The presentation in a female hypospadias patient is variable. It may be asymptomatic, coming to light only when an attempt to catheterize the patient is made, or it may present with postmicturition incontinence and imperfect control caused by vaginal voiding with the urethral syndrome-frequency, dysuria, urgency, and dyspareunia presenting postpuberty when sexual intercourse has commenced or with recurrent urinary tract infections. They may behave like detrusor sphincter dyssynergia, due to the reflex activation of sphincteric activity postvaginal void.

FH is often associated with other genitourinary anomalies such as cloacal malformation, female pseudohermaphroditism, nonneurogenic neurogenic bladder, and urethral duplication. An isolated FH is very rare.

Various methods have been described for creating a urethra in such patients. These include tubularizing a bladder flap, tubularizing the vaginal wall and covering it with a flap of adjacent vaginal wall, tubularizing the vaginal wall and covering it with a pedicle flap of labial and/or bulbocavernosus muscle, constructing the female urethra from the vaginal wall with a perineal flap[1] or buttock flap,[2] and urethral meatal transposition.[5] We found the “skydiver” position little cumbersome and chose to modify Hendren's technique with a lithotomy position.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Hendren WH. Construction of female urethra from vaginal wall and a perineal flap. J Urol 1980;123:657-64.  Back to cited text no. 1
    
2.
Hendren WH. Construction of a female urethra using the vaginal wall and a buttock flap: Experience with 40 cases. J Pediatr Surg 1998;33:180-7.  Back to cited text no. 2
    
3.
Solov'ev AE. The diagnosis and treatment of hypospadias in girls Urol Nefrol (Mosk). 1993;6:11-3. [Article in Russian].  Back to cited text no. 3
    
4.
Derevianko IM, Derevianko TI, Ryzhkov VV. Hypospadias in females. Urologiia. 2007;3:26-8. [Article in Russian].  Back to cited text no. 4
    
5.
Van Bogaert LJ. Surgical repair of hypospadias in women with symptoms of urethral syndrome. J Urol 1992;147:1263-4.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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    Abstract
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