Sarah H. Milton
History of present illness
A 13-year-old adolescent girl presented to the emergency department with a complaint of cramping and lower abdom- inal pain that was increasing in severity. Her pain was constant, located in the midline, did not radiate, and was not relieved by acetaminophen. Further questioning revealed a history of similar type pain that had been occurring cyclically for the last three months. She reported mild constipation and lower back pain, but denied nausea, vomiting, fever, chills, or urinary symptoms. She had no prior medical or surgical his- tory, was premenarchal and denied prior sexual activity. She is accompanied by her mother who reports no other changes in her daughter’s school performance or social interactions.
Physical examination
General appearance: Thin adolescent woman in moderate distress
Vital signs:
Temperature: 37.1°C Pulse: 120 beats/min
Blood pressure: 92/46 mmHg Respiratory rate: 22 breaths/min Oxygen saturation: 100% on room air
Cardiovascular: Tachycardia, with regular rhythm Breast: Tanner stage IV breast development Respiratory: Chest clear to auscultation bilaterally
Abdomen:Soft, nondistended, normal bowel sounds, with tenderness to palpation suprapubically. There is a midline mass palpable which was smooth, mobile and tender. The mass extended approximately 4 cm above pubic symphysis.
No rebound or guarding
Genitourinary: Normal labia with tense, bulging, bluish translucent membrane just cephlad to the vaginal introitus.
Tanner stage IV pubic hair development Laboratory studies:
WBCs: Normal Hb: Normal
How would you manage this patient?
This 13-year-old adolescent has an imperforate hymen with resultant hematocolpos and hematometria. The diagnosis was suspected based on the classic presentation of an adolescent patient with cyclic abdominal pain and amenorrhea. This
suspicion was confirmed by the genitourinary examination which revealed the classic tense bluish, translucent membrane (indicative of an imperforate hymen) with proximal hemato- colpos. The patient was given narcotic pain medications, and the gynecology team was consulted. She was taken to surgery the following day where she underwent successful excision of the imperforate hymen with evacuation of the hematocolpos and hematometria. She recovered well and was discharged the day of her surgery without incident.
Imperforate hymen
Pathologic conditions of the hymen largely result from failed canalization of the lumen of the vaginal canal and the vaginal vestibule during embryonic life [1]. The hymen is usually patent at birth; however, several pathologic variations in the development of the hymen can occur including septation, microperforation or complete occlusion termed“imperforate.”
Imperforate hymen is the rarest of these variations and is found in 0.05–0.10% of newborn girls [1]. Although the majority of cases are sporadic, genetic transmission has also been reported with both dominant and recessive inheritance patterns [2].
An imperforate hymen most commonly comes to clinical attention in adolescence when menarche occurs and the distal vaginal occlusion results in painful hematocolpos (menstrual blood accumulated in the vagina) [3]. These young girls give a history of several months of cyclic pelvic pain. They may also experience back pain, nausea, vomiting, urinary frequency, and constipation as a result of the mass effect of the hemato- colpos and hematometria [4]. Although less common, these patients may present with peritoneal signs as a result of retro- gradeflow of menstrual blood through fallopian tubes and into the peritoneal cavity [1]. A microperforate hymen or a septate hymen will not obstruct menstruation. These conditions commonly come to clinical attention when patients encoun- ter difficulty with insertion of a tampon or with vaginal intercourse [4].
A careful examination of the external genitalia is recom- mended by the American Academy of Pediatrics [5]. Despite this recommendation, the incidental diagnosis of imperforate hymen in the newborn period is uncommon [3]. In this cir- cumstance, there is not proximal distension of the vagina by blood orfluid which makes visualization and diagnosis more difficult. Very infrequently, a newborn examination may reveal a thin, white bulging membrane at the vaginal introitus. This pathologicfinding is the result of accumulation of genital tract Acute Care and Emergency Gynecology, ed. David Chelmow, Christine R. Isaacs and Ashley Carroll. Published by Cambridge University Press.
© Cambridge University Press 2015.
secretions produced secondary to in-utero exposure to mater- nal estrogen [4]. The result of these secretions in a newborn with an imperforate hymen is the development of a mucocol- pos, which is generally asymptomatic, but more readily identi- fied on routine newborn examination.
An imperforate hymen is a clinical diagnosis. A character- istic history of cyclic pelvic pain in an adolescent patient, in conjunction with a genital examination revealing a tense bulging membrane at the vaginal introitus is sufficient for diagnosis as was the case in this patient. On occasion, the diagnosis may be more difficult if the thickness of the obstruct- ing membrane cannot be readily determined by physical exam- ination (Fig. 74.1). In these circumstances, the differential diagnosis includes Mullerian anomalies, including a transverse or longitudinal vaginal septum, labial adhesions, androgen insensitivity syndrome, or complete Mullerian agenesis. Cor- rect diagnosis is essential prior to operative intervention as the management of the aforementioned conditions varies widely and is not universally surgical. If the diagnosis of imperforate hymen is in question, a MRI is the best test to evaluate the pelvic anatomy prior to intervention [6,7]. Particularly in newborn girls, ultrasound may also be of use to determine the thickness of the obstructing tissue and identify pelvic structures [8].
If diagnosis of an imperforate hymen is suspected in an adolescent patient, gynecologic consultation should be obtained prior to any attempt to alleviate obstruction. While management of an imperforate hymen is surgical, it is not a surgical emergency. Initial emphasis should be placed on anal- gesia with a plan for surgical decompression in the operating room by a trained gynecologic surgeon. Once adequate anes- thesia is obtained in the operating room, the distended hymenal tissue can be injected with a local anesthetic, and a cruciate incision is made in the hymen to evacuate the hematocolpos. The excess hymenal tissue is then excised, and the mucosal margins are reapproximated to prevent scarring and provide hemostasis [1].
In a newborn girl with an imperforate hymen (with or without a mucocolpos present), surgical excision of the hymen is encouraged. This can be accomplished in the operating room and is identical to the procedure described above. If the hymenectomy is deferred in the newborn period and performed at puberty, one must be mindful of the risk of a painful hematocolpos forming with initiation of menses.
Because hymenectomy is facilitated by well-estrogenized tissue, it should be deferred until after puberty if the diagnosis is made outside of the newborn period [4].
Microperforate and septate hymens should only be surgically managed if they are symptomatic [4]. The most common complaints in patients with these conditions are inability to insert a tampon and difficulty with sexual inter- course. Surgical procedures are individualized and should be performed by a gynecologic surgeon with experience in hymenal pathology.
Complications of hymenectomy are rare. Most patients recover well and do not have any long-term reproductive consequences from the surgery [9]. Because familial associ- ations have been described, any offspring of a woman with a history of an imperforate hymen should be carefully examined in the newborn period for evidence of the condition [2].
Key teaching points
Imperforate hymen is a relatively rare condition that should be considered in all adolescent patients presenting with cyclic abdominal pain and
amenorrhea.
All newborn girls should have a thorough pediatric examination of their external genitalia shortly after birth, which can facilitate early diagnosis and repair of an imperforate hymen.
In most cases, a thorough history and examination should be sufficient for diagnosis of an imperforate hymen and for surgical planning. If, however, the diagnosis is uncertain, the best imaging study to clarify and identify pelvic structures is
an MRI.
Obstruction of menstruation resulting from an imperforate hymen is not a surgical emergency. Emphasis should be
Fig. 74.1 Genital examination.
Case 74: Worsening cyclic pain and amenorrhea in a 13-year-old girl
placed on analgesia while obtaining timely consultation by a gynecologist trained to confirm the diagnosis and plan a nonemergent surgical intervention.
Surgical repair of an imperforate hymen by hymenectomy is best performed when tissue is well estrogenized, either in the immediate newborn period or after puberty.
References
1. Rock JA, Jones HW.TeLinde’s Operative Gynecology, 10th edn.
Philadelphia, PA, Lippincott Williams & Wilkins, 2008.
2. Sakalkale R, Samarakkody U. Familial occurrence of imperforate hymen.
J Pediatr Adolesc Gynecol2005;18(6):
427–9.
3. Posner JC, Spandorfer PR. Early detection of imperforate hymen prevents morbidity from delays in diagnosis.Pediatrics2005;115(4):
1008–12.
4. Emans SJ, Laufer MR.Pediatric and Adolescent Gynecology, 6th edn.
Philadelphia, PA, Lippincott Williams & Wilkins; 2012.
5. McInerny TK.American Academy of Pediatrics Textbook of Pediatric Care.
Washington, DC, American Academy of Pediatrics, 2009.
6. Church DG, Vancil JM, Vasanawala SS.
Magnetic resonance imaging for uterine and vaginal anomalies.Curr Opin Obstet Gynecol2009;21(5):379–89.
7. American College of Obstetricians and Gynecologists. Müllerian agenesis:
diagnosis, management, and treatment.
Committee Opinion No. 562.Obstet Gynecol2013;121:1134–7.
8. Blask AR, Sanders RC, Rock JA.
Obstructed uterovaginal anomalies:
demonstration with sonography. Part II. Teenagers.Radiology1991;179(1):
84–8.
9. Rock JA, Zacur HA, Dlugi AM, et al.
Pregnancy success following the surgical correction of imperforate hymen as compared to the complete transverse vaginal septum.Obstet Gynecol1982;59:448–51.