Imperforate hymen is an extreme manifestation of hymenal variation occurring in as less as 0.0014 to 0.1% of infants girls. Female infants with imperforate hymen rarely present with urological complications. We would like to present an unusual case of urinary incontinence with hydroureter and hydronephrosis in a 7 month old female child due to a large mucocolpos. This infant was successfully treated surgically.
A mother presented to the Uro-Gynaecology Outpatient department with her 7 month old daughter having complaints of urinary incontinence with dribbling of urine and mass in lower abdomen since last 15-20 days. On examination there was a large cystic mass in the hypogastrium which was extending upto the umbilicus. The lower end of masscould not be reached suggesting that it was arising from the pelvis. Ultrasonography showed a large cystic mass arising from the pelvis with a distended urinary bladder, hydroureter and hydronephrosis.(Fig 1). Differential diagnosis of bladder diverticulum, mesenteric cyst, a tuboovarian mass,or an ovarian cyst was made. Cystourethrogram was performed which showed a distended urinary bladder displaced anteriorly due to some mass behind it(Fig 2,3). Detailed gynecological examination was done under anaesthesia which revealed a bulging IMPERFORATE HYMEN. It was incised by a cruciate incision and ~1500 ml of straw coloured fluid was drained out.
Ambroise Pare first described Imperforate hymen in 1633.1 Most distal form of vaginal outflow obstruction is called “imperforate hymen”. During normal embryological development, the central portion of the hymenal membrane disappears, creating the hymenal opening at the level of the vaginal vestibule.(Fig 4). Persistance of the intact hymenal membrance results in the condition of imperforate hymen. The imperforate hymen is a solid membrane interposed between the proximal uterovaginal tract and the introitus. This vertical fusion defect from other vertical fusion defects in that it is not derived from the mullerian system. This vaginal oulet obstruction leads to entrapment of vaginal and uterine secrection above it forming a cystic collection in the pelvis. Large mucocolpos can cause urethral compression anteriorly leading to bladder outflow obstruction,urinary retention with urnary tract infection, hydroureter and hydronephrosis. Most neonates with imperforate hymen are missed at birth, delaying the diagnosis sometimes upto early adolescence when they present with hematometrocolpos. Imperforate hymen may also present with back pain ,urinary retention (37%- 60% of patients ), and constipation.2 Physical examination may reveal a lower abdominal mass on plapation, or a pelvis mass on bimanual rectal examination. The diagnosis of imperforate hymen is often established during examination when a distended bluish membrane is observed at the introitus. In the absence of this finding, only imaging study by ultrasound or MRI can establish the level of obstruction. The differential diagnoses of uterovaginal obstruction include disorders of vaginal development, such as a transverse vaginal septum or complete vaginal agenesis.which may be associated with other development anomalies(e.g, Rokitansky- Kuster-Maier-Hauersysdrome).
Prenatal diagnosis of imperforate hymen has also been reported. Fetal diagnosis has occurred as early as 25 weeks` gestation. A thin bulging memebrane separating the labia in association with a distended vagina is apparent on ultrasounography,3 these finding are usually noted during an evaluation for fetal ascites and are thought to result from distal urinary tract obstruction, however they can also be related to reflux of uterine contents through the fallopian tubes. Ascites and bladder outlet obstruction are the most common associated finding in the fetal period.4 Intestinal, cardiac and anorectal defects have NOT been reported in conjuction with imperforate hymen. Sometimes polydactyly is associated with imperforate hymen as in Mckusick-Kaufmann sysdrome.5
Careful evaluation of the perineum of the newborn is essential. Female neonate has full labia majora under the influence of maternal estrogens. Inspection of the introitus reveals that hymenal membrane is pink and slightly edematous. In the newborn with an imperforate hymen, the membrane is often bulging because of retained mucoid secretions. A vaginal cyst which fills the introitus but is attatched only to one vaginal aspect should be distinguished from imperforate hymen.
Aspirating secretions beyond the obstruction should be deferred because this procedure may result in iatrogenic pyocolpos. Instead, the diagnosis should be confirmed by performing noninvasive imaging studies (Ultrasonography, MRI) to determine the extent of vaginal outflow obstruction and to diagnose other associated anomalies (i.e imperforate hymen and a transverse vaginal septum) can occur.6
- Wall EM, Stone B, Klein BL. Imperforate hymen: a not-so-hidden diagnosis. Am J Emerg Med. May 2003;21(3):249-50.
- Nazir Z, Rizvi RM, Qureshi RN, Khan ZS, Khan Z. Congenital vaginal obstructions: varied presentation and outcome. Pediatr Surg Int. Sep 2006;22(9):749-53.
- Winderl LM, Silverman RK. Prenatal diagnosis of congenital imperforate hymen. Obstet Gynecol. May 1995;85(5 Pt 2):857-60.
- Ogunyemi D. Prenatal sonographic diagnosis of bladder outlet obstruction caused by a ureterocele associated with hydrocolpos and imperforate hymen. Am J Perinatol. 2001;18(1):15-21.
- El-Messidi A, Fleming NA. Congenital imperforate hymen and its life-threatening consequences in the neonatal period. J Pediatr Adolesc Gynecol. Apr 2006;19(2):99-103.
- Ahmed S, Morris LL, Atkinson E. Distal mucocolpos and proximal hematocolpos secondary to concurrent imperforate hymen and transverse vaginal septum. J Pediatr Surg. Oct 1999;34(10):1555-6.
- Internet website available on http://www.embryology.ch/anglais/ugenital/genitinterne06.html#sug
(This reference has been used in legend 4a and 4b)
Fig 1 : Ultrasonography showing Hydronephrosis and Hydroureter
Fig 2 : Cystourethrogram showing bladder compressed and displaced anteriorly due to some mass behind .(both anteroposterior and posteroanterior view)
Fig 3: Cystourethrogram showing a distended urinary bladder extending upto the ribcage of the baby.
Fig 4a: In females, the development of the SUG (urogenital sinus) begins in the 3rd month, at the same time as the formation of the vagina.7
1=Genital tubercle 2= Vestibule 2a =SUG : phallic part 2b= SUG : lower pelvic part of definitive urogenital sinus 3=Vaginal plate 4=Perineum 5=Rectum 6= Utero-vaginal canal 7 = Urinary bladder 8=Urethra
Fig 4b: The pelvic part of the SUG has shrunk and will be retracted into the phallic part in order to form the definitive vaginal vestibule.7
2= vestibule 3a = uterine cavity 3b =uterine cervix 6a= vagina:lower fourth out of endoderm 6b =vagina :upper 3/4th out of mesoderm 9= hymen
Dr. Baldawa Pratibha S
M.S(Obgyn),D.N.B, D.G.O,F.C.P.S,D.F.P, Assistant Professor .
Correspondence address :
Baldawa Hospital, Budhwar Peth, Near Kasturba Market, Solapur – 413002,
Phone: (+91) 217- 2324762 (home), (+91) 9745306852 [Mobile].
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