Imperforate Hymen |
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INTRODUCTION | ¡@ |
Imperforate hymen is at the extreme of a spectrum of variations in hymenal configuration. Variations in the embryologic development of the hymen are common and result in fenestrations, septa, bands, microperforations, anterior displacement, and differences in rigidity and/or elasticity of the hymenal tissue. Inspection of the external genitalia and anus are important components of the physical examination of the female neonate. While this examination can and should be accomplished by the pediatrician, the observant delivering obstetrician can learn much about the normal variations in genital configuration by examining the female neonate in the delivery room, keeping in mind the influence and structural changes induced by maternal estrogens. Under this influence, the labia majora are plump, the hymen is elastic and often lax and fimbriated, and the mucosal surfaces (ie, introitus, fossa navicularis) are pale pink.
Problem: In spite of the recommendations for early inspection of the external genitalia, variations in hymenal anatomy typically escape diagnosis until the time of menarche. Different normal variants in hymenal configuration are described, varying from the common annular, to crescentic, to navicular (with an anteriorly displaced hymenal orifice). Rarely, hymenal variations may be clinically significant before menarche. In the case of a navicular configuration, urinary complaints (eg, dribbling, retention, urinary tract infections [UTIs]) may result. Sometimes, a cribriform or navicular configuration to the hymen can be associated with retention of vaginal secretions and prolongation of the common condition of a mixed bacterial vulvovaginitis.
Imperforate hymen in infancy or childhood
An infant or child may be thought to have an imperforate hymen. Careful examination with pressure applied to the fourchette may reveal a microperforation, sometimes with an anteriorly placed opening just beneath the urethra. Capraro described a surgical technique similar to a perineotomy to correct such a defect; however, in the asymptomatic patient, waiting until puberty is suggested before deciding whether such a technique is necessary. The hymenal changes that result from estrogenization (increased elasticity and fimbriation) may preclude the need for surgery.
Sexual abuse
Accurate description of the morphology and integrity of the hymen is critical in the diagnosis of female sexual abuse. Concerns about hymenal disruption and lacerations associated with sexual abuse with digital or penile penetration have led to discussions of the normal hymenal diameter. At one time, the diameter of the hymenal opening (measured within the hymenal ring) was proposed to be approximately 1 mm for each year of age. Clearly, this guideline does not apply in the neonatal stage, when maternal estrogens lead to an elastic hymen; however, in the prepubertal stage, marked enlargement, according to this guideline, should prompt consideration of the possibility of abuse. An important difficulty with this "rule of thumb" is that the degree of the child's relaxation and comfort with both the examination and the examiner clearly affects measurements, as does the type of measuring device used.
Experts in sexual abuse assessment have used unaided visual examination and colposcopy to examine the integrity of the hymenal ring. Lacerations through the hymen into the fossa navicularis and introitus suggest a penetrating injury. Frequently, sexual abuse evaluations are conducted at some time remote from the immediate injury; thus, healed or healing lacerations are noted.
Muram concluded that the use of the colposcope by an experienced examiner adds little to the evaluation. In addition, Muram proposed a scale that the examiner can use to evaluate physical findings as normal, abnormal and nonspecific, abnormal and suggestive of abuse, and definitive for abuse. The latter category includes only the situation in which sperm are found on examination.
Anatomic anomalies
Consider anatomic anomalies that can be confused with imperforate hymen in the differential diagnosis. These anomalies include the following:
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Frequency: Imperforate hymen probably is the most frequent obstructive anomaly of the female genital tract, but estimates of its frequency vary from 1 case per 1000 population to 1 case per 10,000 population. McCann et al (1990) examined 93 girls, aged 10 months to 10 years, to collect normative data on genital anatomy in nonabused prepubertal girls and found 1 child with an imperforate hymen (1.2%) and 2 children with hymenal septa (2.5%).
Etiology: Imperforate hymen and related genital tract anomalies result from abnormal or incomplete embryologic development.
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Pathophysiology: The genital tract develops during embryogenesis, from 3 weeks?gestation to the second trimester. The initial development of both the male and female genital tracts occurs concurrently and is referred to as the indifferent stage of development.
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By the 12th week, the paired müllerian ducts have fused into a single tube (ie, primitive uterovaginal canal). Two solid evaginations form from the distal aspects of the müllerian tubercle from the sinovaginal bulbs (of urogenital sinus origin) or vaginal plate. The initial or cephalad portion of the müllerian ducts forms the fimbria and fallopian tubes; the more distal segment forms the uterus and upper vagina. The canalization of the paramesonephric ducts and/or upper vagina joins with the vaginal plate, which canalizes beginning caudally and creates the lower vagina. By the fifth month of gestation, the canalization of the vagina is complete. The hymen itself is formed from the proliferation of the sinovaginal bulbs, becoming perforate before birth.
Gonadal development
The development of the gonads occurs from the migration of primordial germ cells to the genital ridge, while the genital tract itself develops from the müllerian ducts (paramesonephric ducts), urogenital sinus, and vaginal plate. Thus, anomalies of the vagina, hymen, and uterus are not accompanied by abnormalities of ovarian development, and hormonal and endocrinologic function is without abnormality, leading to expected pubertal breast development.
Because the mesodermal layer contributes to the development of the kidneys, gonads, and ductal structures, defects or insults in embryologic development may result in congenital defects of the kidneys that accompany abnormalities of the vagina and uterus.
The lining of the urethra and urinary bladder derives from endoderm, and the urogenital sinus forms the urethra and vestibule in females. The ectoderm fuses with the endoderm to contribute to the patency and canalization of the genital tract. Defects in this process lead to fusion failures and imperforate and obstruction defects.
Familial occurrence
Familial occurrence is reported, and screening by history or examination of family members is warranted. Dominant transmission (either sex-linked or autosomal) and sibships suggesting a recessive mode of inheritance are described. The inheritance of müllerian defects likely is polygenic or multifactorial, although some syndromes of heritable disorders are described with associated genital and nongenital anomalies.
Anomalies of the female reproductive tract
Anomalies of the female reproductive tract can result from agenesis or hypoplasia, vertical fusion and/or canalization defects, lateral fusion and/or duplication abnormalities, or failure of resorption, resulting in septa.
Clinical:
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Occasionally, diagnosis can occur in infancy. The patient presents with a bulging yellow-gray mass at or beyond the introitus. The presence of an abdominal mass has been described in association with urinary obstruction.
Diagnosis has been made in utero with obstetrical ultrasonography. Ultrasonography is an essential first step in diagnosis, precluding unwise and unplanned surgical intervention with resultant injury to the urethra.
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Observation with a planned hymenotomy during puberty is a reasonable course of action in most cases, assuming no urinary symptoms or obstruction is present. More typically, a mucocele is not present. If a patient is diagnosed with an asymptomatic imperforate hymen in infancy or childhood, the optimal time for surgical repair is during puberty and prior to menarche. During this time, surgical repair prevents the typical situation in which a young woman presents with intermittent abdominal pelvic pain, which can become severe over the course of several months.
Urinary pressure and even retention with dilation of upper urinary tracts from obstruction is not uncommon. Frequently, vaginal and rectal pressure is present. Severe constipation and low-back pain are described as presenting symptoms. The laborlike menstrual cramps may be severe and cyclic, although the cyclic nature of the symptoms may not be apparent easily or immediately to the young woman or her family.
Unfortunately, the typical findings at diagnosis include a large collection of blood within the uterus (hematometra) and an even larger collection of blood within the distensible vagina (hematocolpos). Additional findings may include blood-filled fallopian tubes (hematosalpinges) and signs of retrograde menses, occasionally to the point of the development of intra-abdominal endometriosis and severe adhesions. The classic teaching is that endometriosis associated with obstructive anomalies resolves spontaneously and does not cause problems with subsequent pain and infertility compared to endometriosis arising spontaneously; however, this assertion is anecdotal rather than evidence based.
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The differential diagnosis of an imperforate hymen includes many conditions, some rare and others relatively common.
Labial adhesions
The presence of acquired labial adhesions in a prepubertal girl is a common situation that often is confused with absence of the vagina. Labial adhesions are not congenital and result from agglutination due to inflammation. Small areas of labial adhesions can be managed expectantly. Extensive labial adhesions or those associated with such symptoms as recurrent UTIs, urinary dribbling, or recurrent vulvovaginitis can be managed easily using the topical application of estrogen cream for 2-6 weeks. Such treatment results in the marked thinning of the adhesions, often with spontaneous resolution. Adhesions that do not resolve completely over this interval but are thin and translucent almost always can be managed in the physician's office with gentle manual separation, after the application of topical lidocaine jelly or prilocaine (eutectic mixture of local anesthetics [EMLA]) cream.
Separation of thick adhesions is possible to accomplish in the office with a child who can be restrained; however, this procedure ultimately is counterproductive because the examination frequently is difficult. General anesthesia in an operative setting may be required.
The biggest problem in managing labial adhesions is that recurrent adhesions are common because the epithelial surfaces are damaged during separation. Parents or caretakers must be instructed on how to ensure that the child maintains excellent perineal hygiene and avoids vulvovaginitis. The application of a topical emollient on a daily basis helps to prevent recurrences until the endogenous pubertal estrogen stimulation alleviates the risk.
Labial adhesions may be confused with posterior labial fusion encountered in congenital adrenal hyperplasia and may be differentiated by careful physical examination with attention to the presence or absence of clitoromegaly.
Hymenal obstruction
In the case of incomplete hymenal obstruction due to a cribriform hymen or hymenal band, the typical presenting complaint is difficulty inserting a tampon. Anatomic variations must be distinguished from the involuntary vaginismus or contraction of the perineal musculature or levator ani muscles, which can be associated with the learning process of tampon insertion, becoming a vicious cycle when persistent insertion is attempted without success and causing pain.
Hymenotomy occasionally may be indicated in the case of a rigid inelastic hymen, particularly for young women who are athletes (eg, swimmers, divers, gymnasts, cheerleaders). A reasonable alternative to surgical correction involves the use of progressive self-digital dilation in a motivated young woman. In athletes with a rigid hymen, an evaluation for possible hypoestrogenism associated with vigorous physical activity should be considered; if present, estrogen replacement improves the hymenal characteristics and increases hymenal elasticity.
Hymenal bands
This condition typically is amenable to division using a local anesthetic in the office; however, the young woman's age and tolerance of such an office procedure must be predicted and judged. Her degree of motivation for tampon use or intercourse impacts the timing at which she requests such a procedure. A typical presenting history of an individual with a hymenal band is the ability to insert a tampon but extreme difficulty in removing it (eg, patient in whom the tampon string became wrapped around the hymenal band, leading to marked edema and pain when removal was attempted).
Obstructing longitudinal or transverse septa
These conditions require careful preoperative evaluation to define the anatomy. The repair of such complicated anomalies usually should be left to gynecologists at a tertiary care center where these cases are not a rarity. MRI usually is the criterion standard for defining the anatomy.
Vaginal agenesis or androgen insensitivity
The evaluation and management of vaginal agenesis or androgen insensitivity syndrome is beyond the scope of this article, but these conditions should be considered in the differential diagnosis. These patients should be referred to a gynecologist who specializes in adolescents. The options for creation of a neovagina are operative, such as a McIndoe procedure with the creation of a neovagina, or nonoperative, using progressively larger Lucite dilators.
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INDICATIONS | ¡@ |
An imperforate hymen must be corrected surgically. Surgical decision-making should focus on appropriate diagnosis and timing of surgical repair.
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RELEVANT ANATOMY AND CONTRAINDICATIONS | ¡@ |
Relevant Anatomy: An imperforate hymen is visible on examination as a translucently thin membrane that bulges with Valsalva maneuver just inferior to the urethral meatus. If a hematocolpos is present, a bluish discoloration is visible behind the translucent membrane.
Depending on the size of the hematometra, hematocolpos, or hematosalpinges, a pelvic or abdominal mass may be palpable on abdominal or rectal examination.
Radiographic documentation must demonstrate that the true diagnosis is not an obstructing transverse vaginal septum or other anomaly. Pelvic ultrasound via the transabdominal, transperineal, or transrectal route is indicated as the initial diagnostic test, followed by MRI if any question about the anatomy exists. Because renal and urologic abnormalities are associated with müllerian abnormalities, imaging of the upper urinary tract can help to diagnose ipsilateral renal agenesis, duplex collecting systems, and other complex anomalies.
The incidence of renal agenesis is estimated at 1 case per 600-1200 persons on the basis of autopsy studies. As many as 25-90% of women with renal anomalies are suggested to have concurrent genital anomalies; thus, abdominal and pelvic imaging of these patients also is warranted.
Contraindications: The only contraindications for a surgical repair of an imperforate hymen relate to the surgeon's inexperience with this condition, failure to adequately consider the alternative diagnoses, or failure to carefully define the anatomy.
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WORKUP | ¡@ |
Lab Studies:
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Imaging Studies:
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Diagnostic Procedures:
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TREATMENT | ¡@ |
Medical therapy: Medical therapy has no place in the management of an imperforate hymen. Nonoperative therapy using progressive vaginal dilators may be the treatment of choice for patients with vaginal agenesis and androgen insensitivity syndrome.
Surgical therapy: The timing of surgical therapy is based on the presence of symptoms. A symptomatic mucocele presenting in the neonatal time period should be managed expediently. If an asymptomatic imperforate hymen without mucocele is diagnosed during childhood, it can be managed during puberty and prior to the development of a hematometra or hematocolpos. The presence of estrogen stimulation in puberty facilitates the surgical repair and healing.
While the treatment of an imperforate hymen is a surgical urgency when it presents in an adolescent with hematometra and hematocolpos, the procedure should not be performed on an emergency basis without appropriate preoperative evaluation. Surgical correction should be definitive. A diagnostic technique (eg, needle aspiration in the office setting) should not be used to confirm the diagnosis because this can allow the introduction of bacteria into what had been a sterile hematocolpos or hematometra, setting the stage for pyocolpos or pyometrium, with the potential to adversely affect fertility.
Preoperative details: Preoperative pelvic and abdominal ultrasound (to image the kidneys and urinary tract) should be performed, with MRI reserved for the evaluation of questionable anatomy or the possibility of müllerian abnormalities.
The patient and family should be prepared for the surgical procedure, which can be described as a hymenotomy (opening up the hymenal membrane). A concurrent laparoscopy also is suggested in a young woman presenting with hematocolpos because severe pelvic adhesions and extensive intra-abdominal endometriosis may be present. The potential risks and benefits of this component of the surgical procedure should be explained to the young woman and her parents in an effort to facilitate informed decision-making and consent.
Intraoperative details: The objective of a hymenotomy procedure is to open the hymenal membrane in such a way as to leave a normally patent vaginal orifice that does not scar. Infiltration of the membrane prior to the incision with a long-acting local anesthetic (eg, 0.25% bupivacaine) provides preemptive analgesia.
If a large hematocolpos is present, it typically is under pressure, and the surgeon should be prepared to dodge the pressure-driven stream of old blood and to evacuate the hematocolpos and hematometra using one or more suction tubes. Often, the revision of the incision in the hymenal membrane must await the evacuation of the hematocolpos.
The hymenal orifice is enlarged using a circular incision following the lines of the normal annular hymenal configuration. Alternatively, a cruciate incision along the diagonal diameters of the hymen, rather than anterior to posterior, avoids injury to the urethra directly anteriorly and can be enlarged by removal of excess hymenal tissue. In either approach, the vaginal epithelium then is sutured to the hymenal ring using interrupted stitches with fine absorbable suture (eg, 4-0 polyglycolic acid suture). The application of 2% lidocaine jelly to the suture line to provide postoperative analgesia is suggested.
Aspiration or puncture of the mucocolpos or hematocolpos without definitive enlargement of the vaginal orifice should be avoided because a pyocolpos or ascending infection may develop.
Concurrent diagnostic laparoscopy may be performed in the usual manner to allow lysis of adhesions and excision or cautery of any endometriosis that may be encountered. Copious isotonic irrigation should be used to lavage any retrograde blood in the pelvic and abdominal cavity to prevent future development of adhesions or endometriosis.
Postoperative details: The surgical procedure of hymenotomy and evacuation of hematocolpos is performed on an outpatient basis. The patient and family should be instructed to expect continued drainage of dark, thick old blood for several days to a week after the procedure. Mild cramping may occur as the hematometra resolves and evacuates.
Ibuprofen or other nonsteroidal anti-inflammatory drugs (NSAIDs) may be prescribed for the cramping. Topical lidocaine jelly is recommended for the vaginal orifice. The patient is instructed to apply the jelly sparingly to the area a few minutes prior to urinating and as needed for soreness. Baths are not prohibited and, in fact, may provide some soothing comfort and help to keep the area clean. The use of a hair dryer on the cool setting to dry the area avoids the abrasion of towel drying. Patients and/or parents are instructed to call the physician’s office if the patient experiences severe cramping unrelieved by ibuprofen or if a fever develops. The family also should be informed that all sutures are absorbable and dissolve, sometimes with the observation of the ends of the suture as small threads.
Follow-up care: Schedule a postoperative office visit 1-4 weeks after the surgical procedure. At that visit, inspect the area for signs of inflammation or infection. Topical lidocaine jelly facilitates the examination and helps to relieve the patient's anxiety. A 3- to 6-month course (or longer) of menstrual suppression with oral contraceptives may be indicated and should be discussed at the postoperative visit.
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COMPLICATIONS | ¡@ |
Infectious complications to the procedure are rare, and prophylactic antibiotics are not required or recommended. A careful surgical technique with adequate opening of the vaginal orifice prevents stenosis and reaccumulation of the hematocolpos or mucocele, which carries a risk for pelvic inflammatory disease (PID) with pyocolpos, pyometra, endomyometritis, salpingitis, or tubo-ovarian abscess. The development of PID clearly has implications and risks for subsequent infertility, pelvic pain, and ectopic pregnancy.
Injury to the adjacent urethra, rectum, or bladder is possible if the anatomic defect is not defined clearly and if the actual condition is vaginal agenesis or complicated müllerian abnormality, rather than a simple imperforate hymen.
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OUTCOME AND PROGNOSIS | ¡@ |
Clinical outcome and prognosis generally is good; one study found pregnancy success more likely following surgical correction of an imperforate hymen than following repair of a complete transverse septum. The retrograde menses and endometriosis associated with an obstructed outflow tract behave in a more benign manner than spontaneously occurring endometriosis without obstruction, although this assertion is based primarily on clinical experience rather than specific outcome reports or evidence.
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FUTURE AND CONTROVERSIES | ¡@ |
Management of imperforate hymen essentially is unchanged from initial descriptions of the procedure; thus, improvements in surgical therapy are not expected. Small variations in technique are described, such as the use of the laser or electrosurgery rather than the scalpel to incise the hymenal membrane. The advantages of this technical modification are not demonstrated.
Typically, the condition is not diagnosed until puberty, when the young woman presents with cyclic abdominal pain, pressure symptoms, and often with an abdominal and/or pelvic mass representing a large hematometra and hematocolpos. This morbidity potentially can be avoided if clinicians (eg, pediatricians, obstetrician, gynecologists, family physicians) are trained to examine the genitalia of newborns and young children. Timing of the surgical correction then could be planned more appropriately; while this is not a controversial recommendation, implementation is far from universal in clinical practice. The future should hold a better prognosis for young girls if their gynecologic health is more appropriately addressed in this manner.
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PICTURES | ¡@ |
| Caption: Picture 1. Abdominal mass with imperforate hymen |
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| Picture Type: Photo |
| Caption: Picture 2. Imperforate hymen with bulging membrane |
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| Caption: Picture 3. Imperforate hymen. Diagram of hematometra and hematocolpos with imperforate distal transverse vaginal septum. |
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| Picture Type: Image |
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BIBLIOGRAPHY |
Section 11 of 11 
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