Background
Pelvic and abdominal pain is common in the pediatric and adolescent population. The differential diagnoses are broad and include gynecologic as well as gastrointestinal, urologic, musculoskeletal, neurologic, and psychiatric causes (Table 7B.1). Dysmenorrhea, in particular, is a common symptom among adolescents, affecting almost 70 percent of girls [1,2]. Primary dysmenorrhea, painful menstruation that cannot be attributed to pelvic pathology, is thought to be caused by an overproduction of uterine prostaglandins (PGs), which lead to increased myometrial contractility during menstruation. All women have increased levels of PGs during the luteal phase, and these rise further when the corpus luteum regresses at the onset of menstruation [3]. Typical primary dysmenorrhea lasts for up to 3 days during menstruation; radiates to the lower back or the thighs; and can be accompanied by gastrointestinal symptoms, such as diarrhea or vomiting. The gynecologist needs to assess the likelihood of whether a patient’s pain is related to primary dysmenorrhea or a secondary etiology, including a congenital, physiologic, acquired, or inflammatory process, which may require additional investigation and intervention. The purpose of this chapter is to focus on the conditions that will most likely require gynecologic surgical intervention, which include congenital anomalies, ovarian cysts, and endometriosis.
Table 7B.1 Causes of pelvic pain in pediatric and adolescent females
Initial Evaluation
To begin the evaluation, the patient and her caregivers should be included in the discussion and be asked about the onset of pain (acute, onset less than 3 months, more than 3 months). The patient should be asked where the pain is located and, if possible, she should be asked to point directly to the site of the worst pain. A comprehensive review of systems should be included with specific questions about stool consistency, dysuria, hematuria, rectal pain, anorexia, weight loss, vomiting, fever, back pain, or pain radiating down the legs. The patient should be asked about a history of a large gush of blood; urinary frequency, urgency, or retention; or a sense of needing to defecate without being able to do so, all of which may indicate an obstruction. She should also be asked about any interventions that have alleviated the pain, including specific medications, and any factors that have exacerbated the pain (certain foods, activities, or stress).
A detailed pubertal and menstrual history should be obtained, including age of thelarche, pubarche, and menarche. She should be asked about whether her cycles are regular (every 24–45 days in adolescence), abnormal in length (fewer than 3 days or longer than 7 days), or associated with abnormally heavy flow (passing clots larger than 2–3 cm in diameter or soaking through clothing). She should be asked if she has noted any association with her menstrual cycle (mid-cycle pain, premenstrual pain, or menstrual pain). She should be asked about any precocious or delayed pubertal milestones (early thelarche, delayed menarche), prior ovarian cysts, prior pregnancies, or other medical conditions that may be associated with a decreased pain sensitivity threshold (anxiety, depression, or pain-processing disorder).
A careful past medical history should include specific questions about congenital anomalies including renal, spine, cardiac, anorectal, and ear anomalies. Finally, prior to any medical or surgical intervention, she should be asked about medical conditions with relative or absolute contraindications to menstrual suppression options (migraines, hypertension, and prior arterial or venous thrombosis). Information should be sought about current medications, with particular attention paid to medications that may interact with systemic hormonal treatments. If she has tried hormonal methods for menstrual or ovulation suppression previously, she should be asked whether these medications improved her symptoms and about any side effects that she may have experienced. A detailed history of prior operative interventions should be noted, particularly for patients with anorectal malformations or prior ovarian operations. The family history questions should include questions regarding congenital anomalies, endometriosis, ovarian cancer, thrombophilia, and inflammatory bowel disease.
She should be interviewed privately to discuss her social and sexual health. It is critical that this information be asked without the caregivers present to protect patient confidentiality and improve the ability for the patient to speak freely. She should be asked about her home situation, education, disordered eating, drug use, gender identity and sexuality and whether she is involved in consensual sexual activity or has a history of sexual assault. Her history of current and past contraceptive use as well as prior sexually transmitted infection testing and treatment should be detailed. She should be asked directly about any home or school stress, any current anxiety and depression, and how much the symptoms have limited her ability to attend school and participate in her normal activities.
Physical Examination
At this point, a careful physical exam should be performed. The patient’s vital signs and general appearance should be assessed for evidence of acute discomfort or hemodynamic instability. A breast and pubic hair examination will determine her sexual maturity rating. The abdominal exam should include superficial and deep palpation to assess for evidence of peritonitis or the presence of a large mass or advanced pregnancy. The pelvic exam can be stressful and painful for a young adolescent and should be explained to the patient, so that she understands the purpose. Often examination of the external genitalia will permit adequate visualization of the vestibular glands and the distal vagina. Using downward traction of the labia minora, the distal vagina can be examined to assess for patency of the hymen or distal vaginal atresia. A cotton swab can be used to palpate the vestibular glands for tenderness and then inserted into the vagina until resistance is met to assess the depth of the vagina. If the patient is sexually active and consents to an internal vaginal speculum exam, a smaller speculum, such as the Huffman, may be less uncomfortable than typical adult size speculums. A digital bimanual examination can be performed to assess for cervical motion tenderness or adnexal masses. Finally examination of the anus should include note of perianal skin tags or fissures that may be associated with inflammatory bowel disease or trauma.
Diagnostic Evaluation
Pelvic imaging is useful to evaluate for congenital causes of pain as well as the presence of ovarian masses. In most young adolescents and postpubertal females, transabdominal ultrasound is adequate to assess the appendix, uterus, and ovaries for evidence of a cyst, tumor, or obstruction. Transvaginal ultrasound is more sensitive for a patient who is pregnant to determine the location of the pregnancy, if there is a concern for ectopic pregnancy or spontaneous abortion. Doppler assessment may provide information about the vascular flow to the adnexa, but the presence of flow does not exclude ovarian torsion as the etiology for pain [4]. Three-dimensional ultrasound is also an effective imaging modality for Mullerian anomalies [5,6].
Magnetic resonance imaging (MRI) is the modality of choice for Mullerian anomalies and can also evaluate renal and spinal anomalies. Of note, a detailed protocol to assess the cervix and upper vagina can increase the sensitivity of evaluation of obstructive anomalies [7]. If there is a known congenital anomaly, such as unilateral renal agenesis or cloacal anomaly, MRI evaluation can determine the presence of a uterovaginal anomaly, including a transverse septum or a noncommunicating functioning uterine horn. If the imaging is normal, then the most likely etiology for secondary dysmenorrhea in adolescents is endometriosis. Due to expense, MRI should not be considered a first-line radiologic examination.
Computerized tomography (CT) may be ordered for patients presenting with acute pain to assess for appendicitis or nephrolithiasis. The scan may then demonstrate findings of an ovarian cyst, tumor, or tubo-ovarian abscess. Although there are findings on CT that may be associated with adnexal torsion including Fallopian tube thickening and uterine deviation to the affected side, it is not the preferred imaging technique for evaluation of adnexal torsion [8]. Due to the radiation exposure, it should not be considered a first-line radiologic examination for patients with concern for ovarian cysts, torsion, or uterine anomaly.
Laboratory evaluation for pelvic pain may include urine pregnancy test and urinalysis to evaluate for hematuria and urinary tract infection. A complete blood count will assess the white blood cell count and hematocrit for infection or anemia; the erythrocyte sedimentation rate may be useful as a marker of inflammation. For any patient with a positive urine pregnancy test, a blood type to assess for Rh status and quantitative human chorionic gonadotropin (hCG) should be performed. Serum tumor markers may be helpful to assess the likelihood of an ovarian malignancy, if imaging reveals an ovarian mass with solid elements or if the tumor is large. Epithelial malignancies in adolescence are rare and therefore tumor markers associated with non-epithelial ovarian cancer, such as serum hCG, inhibin, alpha fetoprotein (AFP), testosterone, and estradiol may be collected [9]. If there is concern for an obstruction with a known renal anomaly, a creatinine can assess for renal insufficiency. Finally, gonorrhea and chlamydia testing should be performed in any patient with a history of sexual activity by vaginal swab or urine testing.
Condition-specific Management Strategies
Primary Dysmenorrhea
Relief of primary dysmenorrhea is usually accomplished with the use of a nonsteroidal anti-inflammatory drug (NSAID) initiated at the onset and, ideally, 24 hours prior to the onset of menstruation if predictable, and continued for the first 2–3 days of the period. Prostaglandin inhibitors have been shown not only to decrease pain but also to decrease the amount of menstrual bleeding. Methods include naproxen, mefenamic acid, ibuprofen, or alternately celecoxib.
Primary dysmenorrhea may also be alleviated with combined oral, transvaginal, or transdermal contraceptives [10]. Combined contraceptive methods can be prescribed in a continuous versus cyclic manner to suppress pain and bleeding. Patients should be assessed for relative and absolute contraindications to treatment prior to initiation, including history of thrombosis, thrombophilia, hypertension, or migraines.
If estrogen is contraindicated, progesterone only methods are also effective, including depot medroxyprogesterone acetate (150 mg every 3 months), oral norethindrone or desogestrel, or the levonorgestrel intrauterine system. The etonogestrel implant may decrease pain but is less likely to achieve oligo or amenorrhea and has not been studied for its benefits in the management of dysmenorrhea.
A secondary cause of dysmenorrhea in adolescents should be suspected when the patient reports onset of symptoms at menarche or soon thereafter with worsening, debilitating pain, refractory to NSAIDs. When dysmenorrhea worsens and is not responding to a trial of a menstrual suppressive method, then an acquired cause of dysmenorrhea, including endometriosis, should be ruled out.
Endometriosis
Endometriosis, was long thought to be a condition primarily diagnosed during the third or fourth decade of life but has now been increasingly identified as a cause of dysmenorrhea in adolescents. Nevertheless, it remains an under-recognized entity in this age group, with the majority of adolescents having a several year delay in diagnosis, often after seeing multiple specialists [11]. More than half of adolescents with pelvic pain not responding to menstrual suppression or NSAIDs and approximately half of those complaining of gastrointestinal and urinary symptoms are found to have endometriosis at laparoscopy [11,12]. Risk factors for the development of endometriosis include a family history of the disease, the presence of a congenital uterine anomaly, and early menarche [13,14].
Although ultrasonography and MRI have been used to identify endometriosis in older women, the gold standard for the diagnosis remains laparoscopy. Endometriomas are rare, and other ultrasound findings characteristic of severe endometriosis, including diminished ovarian or uterine mobility and tenderness, when exerting pressure on the adnexa or pouch of Douglas are not useful with transabdominal ultrasound. Chronic pain without improvement with a trial of menstrual suppression and NSAIDs in the absence of any other etiology is an indication for laparoscopic evaluation for endometriosis [11].
Operative Management
More than two-thirds of adolescents will be diagnosed with stage I endometriosis, although more severe disease has been reported [11,15]. The macroscopic appearance of endometriosis in adolescents differs from that found in adults, with white, clear, or red lesions; peritoneal defects; or scars being characteristic features (Figure 7B.1). Rarely, advanced blue or blackish lesions are identified [16,17]. To better visualize clear peritoneal lesions, the laparoscopic camera can be submerged in pooled irrigation fluid [17]. Ectopic endometriosis has been reported in previous abdominal scar sites, the lung, and the appendix. Approximately 30 percent of the time, the appendix can have early lesion involvement and be a continued source of pain for some females; therefore, diagnostic laparoscopy should include visualization of the appendix [17,18].
Figure 7B.1 Clear vesicular endometriosis lesions identified in the cul de sac during laparoscopy
If a lesion is identified, it should be biopsied. Small lesions and adhesions can be ablated with cautery and CO2 laser [17,18]. Peritoneal stripping to completely remove all endometriosis is controversial, especially in adolescents, and may not result in long-term symptomatic relief. When no lesions are visible, it is recommended to consider taking a random biopsy within the cul-de-sac, as this is a common location for early endometriosis lesions to be found.
Postoperative Management
Without ongoing medical treatment, endometriosis remains a chronic disease, likely to deteriorate in the future, with detrimental effects on fertility [16]. Therefore, surgery should be followed by long-term menstrual suppression to minimize disease progression. Although highly effective at suppression of endometriosis pain, the depo medroxyprogesterone acetate (DMPA) and gonadotropin-releasing hormone analogues (GnRH agonists) may impair bone mineralization, due to the complete follicular suppression and hypoestrogenism that they induce and are thus not a preferred long-term option in adolescents [20]. GnRH agonists should ideally be used with add-back therapy if treatment is prolonged beyond 6 months [16]. Danazol, a 17-alpha ethinyltestosterone derivative, results in amenorrhea and is considered highly effective in the management of endometriosis, but it is also less favored among adolescents, due to unpleasant androgenic side effects, such as deepening of the voice and hirsutism. The levonorgestrel intrauterine device is now recommended by most scientific societies as a first-line long-acting contraceptive method in adolescence [21,22]. Evidence exists regarding its efficacy in the treatment of dysmenorrhea and endometriosis in adulthood and emerging data are suggesting that these management principles can be extrapolated to adolescents [23,24]. Another alternative may be a combined estrogen-progestin method, such as the combined oral contraceptive pill, which can be used continuously.
Endometriosis is associated with decreased physical and mental well-being. Close collaboration with psychologists, pain specialists, and social workers may be a valuable strategy to improve quality of life in young women with endometriosis [25]. With early identification and aggressive medical treatment, the progression of endometriosis can be minimized. For patients who are desiring pregnancy, early referral to a reproductive endocrinologist is appropriate if conception is delayed.
Congenital Anomalies
Incidence
The incidence of congenital anomalies of the reproductive tract ranges from 3.2 percent in asymptomatic fertile women undergoing tubal ligation to a high of 67 percent of patients with cloacal anomalies [26,27]. The absolute incidence of uterine and vaginal anomalies in adolescents with pelvic pain has not been established. The most common congenital anomalies identified include imperforate hymen, septate hymen, distal vaginal atresia, transverse vaginal septum, and obstructed uterine horn.
Operative Management
Imperforate Hymen
Imperforate hymen is most often a diagnosis that will be made at the time of puberty, when there is bulge of the hymen with proximal hematocolpos (Figure 7B.2). If mucocolpos is identified in infancy, surgical intervention is indicated if the mass effect causes urinary tract obstruction. It is recommended to remove as much hymenal tissue as is possible to prevent restenosis. Due to the risk of ascending infection with inadequate drainage, simple incision and drainage is not advised. For patients with hematocolpos, the hymenal tissue can be removed in a cruciate fashion or circumferentially to allow adequate vaginal width for tampon use, speculum examination, or coitus. If the hymenal tissue is very thin, the hymen may be resected with the cautery alone, followed by use of topical estrogen cream to aid wound healing. Otherwise, absorbable sutures are placed to approximate the mucosal edges to achieve hemostasis [28].
