- Email: [email protected]
Unique Case of Imperforate Hymen
Case Report Unique Case of Imperforate Hymen Lynn Coppola MD, MPH * Baptist Medical Centre, Nalerigu, Ghana
a b s t r a c t Background: Imperforate hymen typically presents in adolescence with pain, hematocolpometra and primary amenorrhea. This case documents a previously unreported etiology for an atypical presentation with a history of recent menstruation. Case: A female adolescent presented with symptoms of urinary retention and leg pain. She reported a history of irregular, painful menses. Clinical examination revealed a pelvic mass and imperforate hymen. Sonography was consistent with hematocolpometra. Before a planned hymenectomy, the patient began to pass dark blood through a fistulous opening in her vulva. Hymenectomy resulted in complete resolution of the pain and hematocolpometra. Summary and Conclusion: Identification of the fistulous tract explained the patient's history of menstrual bleeding despite an imperforate hymen. Spontaneous rupture of hematocolpometra through a fistulous tract to the vulva is a previously unreported atypical presentation of imperforate hymen in a “menstruating” adolescent with pain and a pelvic mass. Key Words: Imperforate hymen, Adolescent, Amenorrhea, Hematocolpometra
Introduction
Imperforate hymen is the most common cause of congenital vaginal outlet obstruction. The result of failed embryologic canalization of the most caudal portion of the vaginal plate at its juncture with the urogenital sinus, it occurs in 0.05%-0.1% of female newborns. Its early recognition and appropriate surgical management are necessary to prevent complications such as pain, retrograde menstruation, hemoperitoneum, endometriosis, urinary tract obstruction, and infertility. Although an imperforate hymen might be detected using antenatal ultrasonography and is ideally diagnosed before the onset of puberty, most patients present during adolescence with cyclic lower abdominal and/or pelvic pain, hematocolpometra, and primary amenorrhea.1 Rarely, an adolescent who reports previous menstrual bleeding might have an imperforate hymen.2,3 This case report is important because it documents a previously unreported etiology for such an uncharacteristic presentation. Recognition of vaginal outlet obstruction despite atypical clinical presentation is requisite to timely surgical intervention for alleviation of pain and establishment of a functional genital tract. Case
A female adolescent presented to a rural Ghanaian hospital with symptoms of urinary retention and leg pain. Her exact age was unknown. She reported a history of three The author indicates no conflicts of interest. * Address correspondence to: Lynn Coppola, MD, MPH, Department of Obstetrics and Gynecology, University of Arizona Medical Center, PO Box 245078, 1501 North Campbell Ave, Tucson, AZ 85742; Phone: þ1 (520) 626-5998 E-mail address: [email protected]
menstrual periods, the most recent of which had been two months before presentation. Previous episodes of bleeding had occurred at intervals of 2-3 months and were associated with significant pain. She was not sexually active and denied vaginal insertion of any local traditional medicines. She had no family history of adolescent gynecologic problems. On examination, the patient appeared phenotypically normal and had Tanner stage III-IV pubertal development. Her urinary retention resolved spontaneously shortly after her arrival, but she persisted to have a tender, firm pelvic mass to the level of her umbilicus. A taut membrane completely occluded the vaginal canal at the level of the hymen. A mixture of herbs (traditional medicine) filled her anus and lower rectum. She had no extragenital anomalies. Ultrasonography of the pelvic mass revealed a hypoechoic fluid-filled, sagittally bilobed structure with thick walls consistent with hematocolpometra (Figure 1). No free fluid was seen within the abdomen. Bilateral renal appearance and location were normal. Although the patient did not report a history of amenorrhea, her clinical findings were very consistent with an imperforate hymen. She was provided with pain relief and scheduled for hymenectomy the next morning. Incongruity between her reported menstrual history and clinical findings was initially attributed to a local sociocultural emphasis on fertility (with significant ramifications for future marriage in girls with suspected subfertility). On the morning of her scheduled hymenectomy, however, the patient reported the painful onset of another menstrual cycle. Because pain and overall poor visualization precluded adequate examination at the bedside, the patient was taken to the operating theatre for examination under anesthesia. Under spinal anesthesia, examination confirmed occlusion of the vaginal canal at the introitus, consistent with the
1083-3188/$ - see front matter Ó 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2015.07.004
2
L. Coppola / J Pediatr Adolesc Gynecol xxx (2015) 1e3
Figure 1. Sagittal ultrasound image showing distension of uterus and vagina by fluid, consistent with hematocolpometra.Ă
preoperative diagnosis of an imperforate hymen. The patient's perineum and inner thighs were heavily stained with dark blood. Her left labium minus was enlarged and darkly discolored, with a medial fistulous opening at the approximate level of the urethral orifice (Figure 2). Dark blood could be easily expressed through the opening. The size and ultrasonographic nature of the pelvic mass, however, remained grossly unchanged. Hymenectomy was performed with the use of stellate incisions.1 The obstructing membrane measured no more than 1 mm in thickness, and the procedure resulted in an immediate release of approximately 500 mL of dark blood followed by a more gradual reduction in the size of the pelvic mass. Further physical examination revealed a single cervix and no evidence of a longitudinal vaginal septum. Hemostats did not readily pass beyond a 2-cm depth within the fistulous tract, and the tips were not directly palpable in the vaginal canal. No further invasive attempt was made to
Figure 2. Imperforate hymen and fistulous opening on left labium minus.Ă
determine the origin of the fistula. The left vaginal wall was irregular in texture. The patient has been seen once in follow-up, two weeks after her hymenectomy. She reported complete resolution of her pain. Her left labia remained minimally larger than the right, with some dimpling at the site of the previous fistulous opening. The former pelvic mass was no longer palpable abdominally. Repeat ultrasonography revealed complete resolution of the uterine and vaginal distension. Summary and Conclusion
This case documents spontaneous rupture of hematocolpometra via a fistulous vulvar tract as a previously unreported explanation for perceived menstrual bleeding despite complete distal genital tract obstruction. The differential diagnosis for distal genital tract obstruction includes an imperforate hymen and a low transverse vaginal septum; however, despite absence of the characteristic bulging, bluish membrane that separates the labia minora, clinical examination in this patient was most consistent with an imperforate hymen. Although recent menstruation and distal genital tract obstruction are seemingly incongruous, previous case reports have documented several other rare etiologies. Patients with uterine didelphys and an associated obstructed hemivagina and ipsilateral renal anomaly (or HerlynWerner-Wunderlich syndrome) typically present with postmenarchal onset of pelvic pain, a vaginal or pelvic mass, and normal menses.2 Adolescents with an imperforate hymen might rarely present with secondary amenorrhea. For example, secondary closure of the hymen has been reported in the settings of sexual abuse and previous surgical incision of the hymen at an early age.3 Spontaneous closure due to hymenal reorganization during pregnancy has also been
L. Coppola / J Pediatr Adolesc Gynecol xxx (2015) 1e3
3
Figure 3. Fistulous tract from hematocolpometra to vulva. The left panel depicts the distended uterine and vaginal cavities, with the arrow showing the path of egress of menstrual blood through the fistulous tract. The right panel depicts the occluded vaginal introitus. The arrow identifies the fistulous opening in the medial portion of the left labium minus at the approximate level of the urethral orifice.
reported.4 More common in developing nations, acquired genital tract obstruction due to the use of caustic vaginal herbal pessaries might result in secondary amenorrhea.5 Such acquired gynatresia, however, is typically not at the level of the vaginal introitus. € llerian structures When the distensile capacity of the Mu are overwhelmed, the patient with a distal genital tract obstruction might experience rupture of the hematocolpometra. Previous case reports have documented rupture into the peritoneal cavity and through the obstructing hymenal membrane.6,7 In a patient with obstructed hemivagina and ipsilateral renal anomaly and a thin vaginal septum, prolonged pressure within the obstructed hemivagina might result in a microperforate opening between the 2 vaginas.8 This case is the first report of rupture via a fistulous tract to the vulva. Although no invasive attempt was made to definitively document the origin of the fistula, it is presumed to have originated from the distended vagina (Figure 3). Anatomic proximity, palpation of roughened vaginal mucosa along the left € llerian duplication despite vaginal sidewall, absence of Mu diligent examination and excellent clinical response to standard hymenectomy support this presumption. Despite a classic association between imperforate hymen and primary amenorrhea, this case uniquely demonstrates
that a history of recent genital bleeding does not preclude a diagnosis of imperforate hymen in adolescents with a painful pelvic mass. For such patients, fistula formation should be recognized as a possible etiology for apparently discordant menstrual history and clinical findings. Early identification of the obstruction enables timely surgical intervention.
References 1. Dominguez CE, Rock JA, Horowitz IR: Surgical conditions of the vagina and urethra. In: Rock JA, Jones HW III, editors. Te Linde's operative gynecology, (10th ed.). Philadelphia, PA, Lippincott Williams & Wilkins, 2011, pp 508e511 2. Mandava A, Prabhakar RR, Smitha S: OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol 2012; 25:e23 3. Khan ZA, Rajesh U, Rastogi P, et al: Imperforate hymen: a rare case of secondary amenorrhoea. J Obstet Gynecol 2011; 31:91 4. Onan MA, Turp AB, Taskiran C, et al: Spontaneous closure of the hymen during pregnancy. Am J Obstet Gynecol 2005; 193:889 5. Ugburo AO, Fadeyibi IO, Oluwole AA, et al: The epidemiology and management of gynatresia in Lagos, southwest Nigeria. Int J Gynecol Obstet 2012; 118:231 6. Bakos O, Berglund L: Imperforate hymen and ruptured hematosalpinx: a case report with a review of the literature. J Adolesc Health 1999; 24:226 7. Kurdoglu Z, Kurdoglu M, Kucukaydin Z: Spontaneous rupture of the imperforate hymen in an adolescent girl with hematocolpometra. ISRN Obstet Gynecol 2011; 2011:520304 8. Zurawin RK, Dietrich JE, Heard MJ, et al: Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of the literature. J Pediatr Adolesc Gynecol 2004; 17:137
a b s t r a c t Background: Imperforate hymen typically presents in adolescence with pain, hematocolpometra and primary amenorrhea. This case documents a previously unreported etiology for an atypical presentation with a history of recent menstruation. Case: A female adolescent presented with symptoms of urinary retention and leg pain. She reported a history of irregular, painful menses. Clinical examination revealed a pelvic mass and imperforate hymen. Sonography was consistent with hematocolpometra. Before a planned hymenectomy, the patient began to pass dark blood through a fistulous opening in her vulva. Hymenectomy resulted in complete resolution of the pain and hematocolpometra. Summary and Conclusion: Identification of the fistulous tract explained the patient's history of menstrual bleeding despite an imperforate hymen. Spontaneous rupture of hematocolpometra through a fistulous tract to the vulva is a previously unreported atypical presentation of imperforate hymen in a “menstruating” adolescent with pain and a pelvic mass. Key Words: Imperforate hymen, Adolescent, Amenorrhea, Hematocolpometra
Introduction
Imperforate hymen is the most common cause of congenital vaginal outlet obstruction. The result of failed embryologic canalization of the most caudal portion of the vaginal plate at its juncture with the urogenital sinus, it occurs in 0.05%-0.1% of female newborns. Its early recognition and appropriate surgical management are necessary to prevent complications such as pain, retrograde menstruation, hemoperitoneum, endometriosis, urinary tract obstruction, and infertility. Although an imperforate hymen might be detected using antenatal ultrasonography and is ideally diagnosed before the onset of puberty, most patients present during adolescence with cyclic lower abdominal and/or pelvic pain, hematocolpometra, and primary amenorrhea.1 Rarely, an adolescent who reports previous menstrual bleeding might have an imperforate hymen.2,3 This case report is important because it documents a previously unreported etiology for such an uncharacteristic presentation. Recognition of vaginal outlet obstruction despite atypical clinical presentation is requisite to timely surgical intervention for alleviation of pain and establishment of a functional genital tract. Case
A female adolescent presented to a rural Ghanaian hospital with symptoms of urinary retention and leg pain. Her exact age was unknown. She reported a history of three The author indicates no conflicts of interest. * Address correspondence to: Lynn Coppola, MD, MPH, Department of Obstetrics and Gynecology, University of Arizona Medical Center, PO Box 245078, 1501 North Campbell Ave, Tucson, AZ 85742; Phone: þ1 (520) 626-5998 E-mail address: [email protected]
menstrual periods, the most recent of which had been two months before presentation. Previous episodes of bleeding had occurred at intervals of 2-3 months and were associated with significant pain. She was not sexually active and denied vaginal insertion of any local traditional medicines. She had no family history of adolescent gynecologic problems. On examination, the patient appeared phenotypically normal and had Tanner stage III-IV pubertal development. Her urinary retention resolved spontaneously shortly after her arrival, but she persisted to have a tender, firm pelvic mass to the level of her umbilicus. A taut membrane completely occluded the vaginal canal at the level of the hymen. A mixture of herbs (traditional medicine) filled her anus and lower rectum. She had no extragenital anomalies. Ultrasonography of the pelvic mass revealed a hypoechoic fluid-filled, sagittally bilobed structure with thick walls consistent with hematocolpometra (Figure 1). No free fluid was seen within the abdomen. Bilateral renal appearance and location were normal. Although the patient did not report a history of amenorrhea, her clinical findings were very consistent with an imperforate hymen. She was provided with pain relief and scheduled for hymenectomy the next morning. Incongruity between her reported menstrual history and clinical findings was initially attributed to a local sociocultural emphasis on fertility (with significant ramifications for future marriage in girls with suspected subfertility). On the morning of her scheduled hymenectomy, however, the patient reported the painful onset of another menstrual cycle. Because pain and overall poor visualization precluded adequate examination at the bedside, the patient was taken to the operating theatre for examination under anesthesia. Under spinal anesthesia, examination confirmed occlusion of the vaginal canal at the introitus, consistent with the
1083-3188/$ - see front matter Ó 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2015.07.004
2
L. Coppola / J Pediatr Adolesc Gynecol xxx (2015) 1e3
Figure 1. Sagittal ultrasound image showing distension of uterus and vagina by fluid, consistent with hematocolpometra.Ă
preoperative diagnosis of an imperforate hymen. The patient's perineum and inner thighs were heavily stained with dark blood. Her left labium minus was enlarged and darkly discolored, with a medial fistulous opening at the approximate level of the urethral orifice (Figure 2). Dark blood could be easily expressed through the opening. The size and ultrasonographic nature of the pelvic mass, however, remained grossly unchanged. Hymenectomy was performed with the use of stellate incisions.1 The obstructing membrane measured no more than 1 mm in thickness, and the procedure resulted in an immediate release of approximately 500 mL of dark blood followed by a more gradual reduction in the size of the pelvic mass. Further physical examination revealed a single cervix and no evidence of a longitudinal vaginal septum. Hemostats did not readily pass beyond a 2-cm depth within the fistulous tract, and the tips were not directly palpable in the vaginal canal. No further invasive attempt was made to
Figure 2. Imperforate hymen and fistulous opening on left labium minus.Ă
determine the origin of the fistula. The left vaginal wall was irregular in texture. The patient has been seen once in follow-up, two weeks after her hymenectomy. She reported complete resolution of her pain. Her left labia remained minimally larger than the right, with some dimpling at the site of the previous fistulous opening. The former pelvic mass was no longer palpable abdominally. Repeat ultrasonography revealed complete resolution of the uterine and vaginal distension. Summary and Conclusion
This case documents spontaneous rupture of hematocolpometra via a fistulous vulvar tract as a previously unreported explanation for perceived menstrual bleeding despite complete distal genital tract obstruction. The differential diagnosis for distal genital tract obstruction includes an imperforate hymen and a low transverse vaginal septum; however, despite absence of the characteristic bulging, bluish membrane that separates the labia minora, clinical examination in this patient was most consistent with an imperforate hymen. Although recent menstruation and distal genital tract obstruction are seemingly incongruous, previous case reports have documented several other rare etiologies. Patients with uterine didelphys and an associated obstructed hemivagina and ipsilateral renal anomaly (or HerlynWerner-Wunderlich syndrome) typically present with postmenarchal onset of pelvic pain, a vaginal or pelvic mass, and normal menses.2 Adolescents with an imperforate hymen might rarely present with secondary amenorrhea. For example, secondary closure of the hymen has been reported in the settings of sexual abuse and previous surgical incision of the hymen at an early age.3 Spontaneous closure due to hymenal reorganization during pregnancy has also been
L. Coppola / J Pediatr Adolesc Gynecol xxx (2015) 1e3
3
Figure 3. Fistulous tract from hematocolpometra to vulva. The left panel depicts the distended uterine and vaginal cavities, with the arrow showing the path of egress of menstrual blood through the fistulous tract. The right panel depicts the occluded vaginal introitus. The arrow identifies the fistulous opening in the medial portion of the left labium minus at the approximate level of the urethral orifice.
reported.4 More common in developing nations, acquired genital tract obstruction due to the use of caustic vaginal herbal pessaries might result in secondary amenorrhea.5 Such acquired gynatresia, however, is typically not at the level of the vaginal introitus. € llerian structures When the distensile capacity of the Mu are overwhelmed, the patient with a distal genital tract obstruction might experience rupture of the hematocolpometra. Previous case reports have documented rupture into the peritoneal cavity and through the obstructing hymenal membrane.6,7 In a patient with obstructed hemivagina and ipsilateral renal anomaly and a thin vaginal septum, prolonged pressure within the obstructed hemivagina might result in a microperforate opening between the 2 vaginas.8 This case is the first report of rupture via a fistulous tract to the vulva. Although no invasive attempt was made to definitively document the origin of the fistula, it is presumed to have originated from the distended vagina (Figure 3). Anatomic proximity, palpation of roughened vaginal mucosa along the left € llerian duplication despite vaginal sidewall, absence of Mu diligent examination and excellent clinical response to standard hymenectomy support this presumption. Despite a classic association between imperforate hymen and primary amenorrhea, this case uniquely demonstrates
that a history of recent genital bleeding does not preclude a diagnosis of imperforate hymen in adolescents with a painful pelvic mass. For such patients, fistula formation should be recognized as a possible etiology for apparently discordant menstrual history and clinical findings. Early identification of the obstruction enables timely surgical intervention.
References 1. Dominguez CE, Rock JA, Horowitz IR: Surgical conditions of the vagina and urethra. In: Rock JA, Jones HW III, editors. Te Linde's operative gynecology, (10th ed.). Philadelphia, PA, Lippincott Williams & Wilkins, 2011, pp 508e511 2. Mandava A, Prabhakar RR, Smitha S: OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol 2012; 25:e23 3. Khan ZA, Rajesh U, Rastogi P, et al: Imperforate hymen: a rare case of secondary amenorrhoea. J Obstet Gynecol 2011; 31:91 4. Onan MA, Turp AB, Taskiran C, et al: Spontaneous closure of the hymen during pregnancy. Am J Obstet Gynecol 2005; 193:889 5. Ugburo AO, Fadeyibi IO, Oluwole AA, et al: The epidemiology and management of gynatresia in Lagos, southwest Nigeria. Int J Gynecol Obstet 2012; 118:231 6. Bakos O, Berglund L: Imperforate hymen and ruptured hematosalpinx: a case report with a review of the literature. J Adolesc Health 1999; 24:226 7. Kurdoglu Z, Kurdoglu M, Kucukaydin Z: Spontaneous rupture of the imperforate hymen in an adolescent girl with hematocolpometra. ISRN Obstet Gynecol 2011; 2011:520304 8. Zurawin RK, Dietrich JE, Heard MJ, et al: Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of the literature. J Pediatr Adolesc Gynecol 2004; 17:137