Dr. Rosen is an assistant professor of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, University of Michigan, Ann Arbor, MI.

Monica Rosen, MD

According to the Centers for Disease Control and Prevention (CDC), there are over 12 million children and adolescents aged 3 to 17 years with a diagnosed developmental disability.

This number is growing, with the CDC reporting an increased incidence of developmental disabilities in children and adolescents from 16.2% between 2009 and 2011 to 17.8% between 2015 and 2017.

 Although adolescents with disabilities make up a significant portion of obstetricians and gynecologists’ patient panels, specific training on the best care for this population is often lacking.

The reproductive health care of adolescents with disabilities has been fraught with a painful history—including forced sterilization, which has lasting impacts on patients to this day. A recent study found that 15- to 40-year-old females with physical or sensory disabilities are at an increased risk of sterilization (odds ratio, 1.36; CI, 1.03-1.79) after adjusting for age, race, ethnicity, marital status, insurance, parity, and self-reported health.

 Additionally, the disproportionate incidence of sexual violence against children with disabilities is staggering.

 Recent research suggests that children with disabilities are 3 times as likely to experience sexual violence as those without disabilities, with the highest rates of violence occurring between aged 12 and 15 years.

Furthermore, adolescents with disabilities receive inadequate sexual education and counseling regarding contraception and experience higher rates of sexually transmitted infection and unplanned pregnancy.

 Finally, even in the past 2 decades, it has been well documented that patients with disabilities are less likely than their peers to receive appropriate breast and cervical cancer screening.

 provides a comprehensive description of the sexual and reproductive health disparities that affect adolescents with disabilities. It is imperative that providers better understand how to care for this patient population so that all patients are treated with the dignity, respect, and compassion they deserve.

Reproductive Health Disparities in Adolescents

General approach to patients with disabilities

According to the American College of Obstetricians and Gynecologists (ACOG), “excellent gynecologic health care for women and adolescents with disabilities is comprehensive; maintains confidentiality; is an act of dignity and respecttoward the patient; maximizes the patient’s autonomy; avoids harm; and assesses and addresses the patient’s knowledge of puberty, menstruation, sexuality, safety, and consent.”9As demonstrated in 

, adolescent women with disabilities face several interrelated barriers to this excellent gynecologic health care.

Barriers to Accessing Health Care for Adolescents With Disabilities

Many of the most critical aspects of providing quality care to adolescents with disabilities are not groundbreaking techniques but rather, common-sense practices that empower patients to be active participants in their own health care. It is imperative that the provider assess the patient’s competence and ability to communicate without making assumptions that caregivers should speak on behalf of the patient. This can sometimes be met with discomfort from well-intentioned families who are accustomed to having a large amount of control over an adolescent’s life. However, it is essential to provide health care in a way that gives maximal autonomy to the patient based on age and level of competence—not the comfort of family members.

Speaking directly with patients in an adult tone and working to establish familiarity and rapport can also improve care in patients with disabilities. Furthermore, it is beneficial to ask adolescents how they communicate best and be willing to engage with assistive devices. It is crucial to provide an environment that minimizes sensory strain for patients with intellectual disabilities to promote engagement. It is also necessary to assess the physical disability of adolescents and pursue creative options to ensure that they have equitable access to gynecologic exams and procedures if they are indicated.

The physical, mental, and emotional changes that adolescents with disabilities experience during puberty will be largely similar to those of their nondisabled peers. It is important to normalize and anticipate these changes with patients and their families in addition to understanding the special considerations discussed below.

Special considerations in this population

For most adolescents with disabilities, the process and pattern of menstruation vary little from those of their peers. No matter the timing of puberty, ideal sexual health care of adolescents with disabilities involves anticipatory guidance that begins before menarche, when breast development begins.

Specific disabilities that can be anticipated to affect the tempo and timing of puberty include Prader-Willi syndrome and spina bifida. Patients with Prader-Willi syndrome often experience delayed or incomplete puberty with sustained irregular menstruation.

 Patients with spina bifida, especially those with myelomeningocele, may experience central precocious puberty and require prompt referral to a pediatric endocrinologist to minimize unwanted side effects.

 There is conflicting evidence on the timing of puberty in adolescents with autism spectrum disorder (ASD), with a recent study suggesting that there is no statistically significant difference in pubertal timing for adolescents with ASD compared with the rest of the population.

During puberty, patients may have an increased sense of separation from their peers, which can result in challenges with friendships and social isolation.

 Normal mood changes during puberty may be especially challenging for patients with disabilities, who may already be struggling with emotional regulation, communication, and age-appropriate behavior.

Physical interventions required for monthly menstruation, such as pads or tampons, might be difficult or impossible for adolescents with certain disabilities to use. Menstrual suppression is therefore one of the most common reasons patients with disabilities seek gynecologic care. In a retrospective study of 300 adolescents with developmental disabilities ranging in age from 7 to 19 years, the main reason patients sought care was menstrual suppression, followed by hygiene and caregiver burden14 

.Patients with cognitive or physical impairments who make appointments for pubertal anticipatory guidance typically present 13.5 months prior to menarche,

 with more than half of families describing potential behavior changes as their main concern.

Concerns Surrounding Menstrual Suppression in Adolescents With Disabilities

It is important to note that menstrual suppression is not recommended prior to menarche, according to ACOG’s Committee on Adolescent Healthcare.9 As with all patients, menstrual suppression plans should be individualized following discussion of all options (pill, patch, ring, shot, implant, intrauterine device [IUD]) and elucidation of adolescent preference. Families will sometimes ask about surgical options for menstrual suppression (hysterectomy) but these should be reserved only for medically indicated reasons such as cancer or when all other treatment options have been exhausted. According to ACOG, endometrial ablation is never recommended for menstrual suppression in adolescents. 

Setting expectations with families is a crucial component of menstrual management for adolescents with disabilities. Families may be unaware that surgical treatment to permanently end menses will not affect the mood changes that occur with menses. Additionally, it is important to emphasize that complete amenorrhea may be difficult to achieve with medical management. All menses management methods have benefits and drawbacks; see 

 for an overview of factors to consider when deciding on the best method to use.

Considerations in Menses Management Options for Adolescents with Disabilities

Previously, the most popular contraceptive method for adolescents with disabilities was depot medroxyprogesterone acetate (DMPA) because of its injection form and possible resultant amenorrhea with long-term use.

However, because DMPA is associated with loss of bone mineral density, other forms of contraception without this side effect are often considered first line today. Oral contraceptive pills (OCPs), hormonal transdermal patches, and levonorgestrel IUDs are safe and common agents for menstrual suppression in adolescents with disabilities.

 Amenorrhea is often achieved via continuous hormone therapy (with OCPs or transdermal patches) or levonorgestrel IUD placement. In a 2021 study, of 30 patients with disability who desired hormonal management of menses, 30% had an IUD placed, 30% chose OCPs, and 20% chose oral progestin. Ninety-six percent were satisfied with their final bleeding pattern and 50% achieved amenorrhea.

 The remaining 20% of patients who chose not to begin hormonal management of menses demonstrate that providing education and resources prior to menarche can equip patients and their families to manage menses without medication.

It is important to mention the small but well-supported increased risk of venous thromboembolism (VTE) with estrogen-containing contraceptive methods. The population of adolescents with disabilities includes many with limited mobility, wheelchair dependence, or other baseline risk factors for VTEs. Data from 2019 suggest that patients with cerebral palsy are at an increased risk of VTE whether they are ambulatory or not.

 Although VTE is incredibly rare in adolescents (estimated 0.07/10,000 children < aged 18 years),

 we recommend weighing each patient’s specific risk factors and opting for progestin-only options for menses management in patients with multiple risk factors.

According to ACOG’s practice bulletin on noncontraceptive uses of hormonal contraceptives, data show that levonorgestrel IUD placement and endometrial ablation have similar reductions in menstrual blood loss up to 24 months, supporting the effectiveness of IUDs in menses suppression.

 IUD insertion can prove more challenging in adolescents with disabilities, however, sometimes requiring intravenous (IV) sedation or general anesthesia in the operating room (OR) for placement. A recent study looking at 159 females under 22 years who had disabilities found that of 189 IUDs placed in this population over 10 years, 96% of insertions were done in the OR. Continuation rates were high, with 95% of patients keeping their IUD at 1 year and 73% at 5 years.15 It is also not uncommon to combine IUD insertion with other medical procedures requiring IV sedation or the OR. Cervical cancer screening (Papanicolaou test) at the time of insertion is recommended
if appropriate.


Sexual health education can be challenging and uncomfortable for all adolescents, and this is no different for patients with disabilities. For centuries, incorrect beliefs about adolescents with disabilities, including a presumed lack of sexual desire/activity, and lifelong infantilization led to neglect of sexual health in this population. Sexuality education is still often overlooked in this population, and adolescents with disabilities receive far less of it than their peers despite being at an increased risk of poor sexual health.

Discussions with patients can help providers assess patient understanding, knowledge, and attitude toward sex and sexual development. Providers should educate adolescents on a wide variety of topics, including the anatomic and physiologic changes of puberty, sex and sexual development, gender identity, healthy relationships, safe online practices, consent, and sexual abuse 

Although these topics can be time-consuming to discuss, they can have a profound positive impact on adolescents whose sexuality is often ignored. Help from other providers (nurse practitioners, social workers, sexual health educators) can ensure that patients get adequate age- and development-appropriate teaching. It is imperative to assess an adolescent’s level of cognition, and have at least some discussions of sexual health privately, without the presence of family, if deemed appropriate. In today’s era of virtual visits, it can be challenging to ensure this level of true privacy. Office visits, where both patients and providers can be confident in the privacy of the exam room, may be superior for visits regarding sexual health education.

Teens with disabilities can be vulnerable to abuse and exploitation because of social isolation and restrictions on their reproductive rights. If apt, it is important to discuss with patients what types of touching are inappropriate and to develop a plan they can carry out if they do experience inappropriate touching. In the era of constant online connectedness, both patients and families must be involved in discussing ways to ensure online communication is consensual, safe, and appropriate.

As health care providers, we are uniquely positioned to be involved with and improve the reproductive health care and education of patients with disabilities. It is imperative that we treat all patients equally, offering all sexual and reproductive services regardless of whether a patient has a disability. 

1.Disability and health information for women with disabilities. Centers for Disease Control and Prevention. September 16, 2020. Accessed May 12, 2022. https://www.cdc.gov/ncbddd/disabilityandhealth/women.html

2.Increase in developmental disabilities among children in the United States. Centers for Disease Control and Prevention. May 16, 2022. Accessed May 16, 2022. https://www.cdc.gov/ncbddd/developmentaldisabilities/features/increase-in-developmental-disabilities.html

3.Smeltzer SC, Mitra M, Long-Bellil L, Iezzoni LI, Smith LD. Obstetric clinicians' experiences and educational preparation for caring for pregnant women with physical disabilities: a qualitative study. 

. 2018;11(1):8-13. doi:10.1016/j.dhjo.2017.07.004

4.Wu JP, McKee MM, McKee KS, Meade MA, Plegue M, Sen A. Female sterilization is more common among women with physical and/or sensory disabilities than women without disabilities in the United States. 

2017;10(3):400-405. doi:10.1016/j.dhjo.2016.12.020

5.Legano LA, Desch LW, Messner SA, et al. Maltreatment of children with disabilities. 

. 2021;147(5):e2021050920. doi:10.1542/peds.2021-050920

6. Iezzoni LI, Mitra M. Transcending the counter-normative: sexual and reproductive health and persons with disability.

. 2017;10(3):369-370. doi:10.1016/j.dhjo.2017.04.001

7. Ballan MS, Freyer MB. The sexuality of young women with intellectual and developmental disabilities: a neglected focus in the American foster care system. 

. 2017;10(3):371-375. doi:10.1016/j.dhjo.2017.02.005

8Wisdom JP, McGee MG, Horner-Johnson W, Michael YL, Adams E, Berlin M. Health disparities between women with and without disabilities: a review of the research. 

. 2010;25(3):368-386. doi:10.1080/19371910903240969

9.American College of Obstetricians and Gynecologists’ Committee on Adolescent Health Care. Committee Opinion No. 668: Menstrual manipulation for adolescents with physical and developmental disabilities. 

. 2016;128(2):e20-e25. doi:10.1097/AOG.0000000000001585

10.Crinò A, Schiaffini R, Ciampalini P, et al. Hypogonadism and pubertal development in Prader-Willi syndrome. 

. 2003;162(5):327-333. doi:10.1007/s00431-002-1132-4

11. Almutlaq N, O'Neil J, Fuqua JS. Central precocious puberty in spina bifida children: Guidelines for the care of people with spina bifida. J Pediatr Rehabil Med. 2020;13(4):557-563. doi:10.3233/PRM-200728

12.May T, Pang KC, O'Connell MA, Williams K. Typical pubertal timing in an Australian population of girls and boys with autism spectrum disorder. 

. 2017;47(12):3983-3993. doi:10.1007/s10803-017-3281-3

13.Patel DR, Greydanus DE, Calles JL Jr, Pratt HD. Developmental disabilities across the lifespan. 

. 2010;56(6):304-397. doi:10.1016/j.disamonth.2010.02.001

14.Kirkham YA, Allen L, Kives S, Caccia N, Spitzer RF, Ornstein MP. Trends in menstrual concerns and suppression in adolescents with developmental disabilities. 

. 2013;53(3):407-412. doi:10.1016/j.jadohealth.2013.04.014

15Fei YF, Ernst SD, Dendrinos ML, Quint EH. Preparing for puberty in girls with special needs: a cohort study of caregiver concerns and patient outcomes.

. 2021;34(4):471-476. doi:10.1016/j.jpag.2021.03.008

16.Daunov M, Rondinelli J, Ahuja SP, Stehouwer NR. Prevalence of venous thromboembolism in ambulatory and non-ambulatory patients with cerebral palsy. 

. 2019;134(suppl 1):2428. doi:10.1182/blood-2019-130622

17Spentzouris G, Scriven RJ, Lee TK, Labropoulos N. Pediatric venous thromboembolism in relation to adults. 

. 2012;55(6):1785-1793. doi:10.1016/j.jvs.2011.07.047

18ACOG Practice Bulletin No. 110: noncontraceptive uses of hormonal contraceptives. 

. 2010;115(1):206-218. doi:10.1097/AOG.0b013e3181cb50b5

19Michielsen K, Brockschmidt L. Barriers to sexuality education for children and young people with disabilities in the WHO European region: a scoping review. 

. 2021;21(6):674-692. doi:10.1080/14681811.2020.1851181

Articles

Despite a painful history, equitable care is imperative

Reproductive health care for adolescents with disabilities requires special consideration

Part 1 of this series on pediatric adolescent gynecologic emergencies focuses on cases about premenarchal bleeding, urethral prolapse, and the cause of a mysterious vaginal discharge. 

Monica Woll Rosen, MD, is a clinical assistant professor of obstetrics and gynecology at the University of Michigan Medical School in Ann Arbor. She specializes in pediatric and adolescent gynecology.

Elisabeth H. Quint, MD, is a clinical professor in obstetrics and gynecology at the University of Michigan Medical School in Ann Arbor. She is also fellowship director of the Pediatric and Adolescent Gynecology Fellowship and assistant dean for clinical faculty.

Young patients with gynecologic concerns may present to a general obstetrician-gynecologist as their first local contact. But prior to surgical intervention in a child, consultation with or referral to a pediatric and adolescent gynecologist in an academic medical center may be indicated. This review presents several cases of adolescents with genital and pelvic pain, with guidance for work-up and treatment.

You are called to evaluate a 12-year-old girl who fell on the balance beam during a gymnastics class. Her legs landed on either side of the beam. She is unable to void and is in tremendous pain. She was given several doses of pain medication but she continues to experience severe pain (

Figure 1. A 12-year-old girl with vulvar hematoma 

Credit: Collection of Monica Woll Rosen, MD, University of Michigan

This patient has extensive vulvar hematoma. Nonobstetric vulvar hematomas are rare, accounting for only 3.7% of all vulvar hematomas.

 They can occur from blunt trauma due to a fall, foreign body insertion, sexual assault, saddle injury, or intercourse. The rich vascular supply from the pudendal vessel branches makes this site vulnerable to hematoma formation.

A hematoma can be managed conservatively unless the hematoma is expanding, the skin is necrotic, or the patient is in too much pain.

 Ultrasound, CT, or MRI can be used to investigate the size, site, and expansion of the hematoma. Cases requiring surgical management involve an incision, evacuation of blood, and ligation of bleeding vessels.

Because this patient’s hematoma prohibited her from voiding, she needed a Foley catheter placed. Her urine was evaluated for hematuria. Because of her extensive hematoma and continued pain despite medication, she required an exam under anesthesia to assess the extent of her injury, insertion of the Foley catheter, and incision and drainage of the hematoma.

A 13-year-old girl presents to the emergency department with several days of worsening vulvar pain that is now severe. A few days ago, she developed a runny nose, fever, and sore throat. She otherwise has a negative review of systems. She is having trouble urinating because of the vulvar pain and has dysuria when she does urinate. Today she noticed a thick, yellow discharge in her underwear. On exam you notice several large painful ulcerations (

Aphthous or Lipshütz ulcers. Credit: Collection of Monica Woll Rosen, MD, University of Michigan

Aphthous or Lipschütz ulcers are acute vulvar ulcers that occur mainly in non–sexually active girls and are thought to be viral in nature.

 They commonly appear during or following a prodromal viral illness.

 have been associated with the ulcers, the causative virus often cannot be identified. There have been recent case reports of vulvar ulcers following a COVID-19 infection6 or after receiving the COVID-19 vaccine.

A patient may have black, necrotic, shallow, or deep ulcers in the labia minora or majora.

 There can be overlying purulent discharge. When assessing any ulcers it is important to evaluate for underlying sexually transmitted infections, such as herpes simplex. Other considerations include Crohn disease with multiple tracks and ulceration or Behçet disease, which presents with recurrent vulvar ulcers in conjunction with recurring oral ulcers.

Treatment for aphthous ulcers is supportive, as it is a self-limiting condition. Topical lidocaine gel can be used for pain control, and a topical clobetasol ointment can be used twice daily.

If a patient is severely affected, an oral steroid taper may be considered, as well as an admission for intravenous pain control. If the patient cannot void because of dysuria, a Foley catheter should be placed.

An 11-year-old girl presents after track practice with severe, unilateral right lower quadrant pain. Her last menstrual period was 3 weeks ago. The pain is sharp and radiates to her back. She has associated nausea and vomiting. On exam, she has rebound tenderness and guarding of her lower abdomen. On a pelvic ultrasound, she has a 6-cm simple cyst and a normal appendix. What do you do next?

Arrow shows torsed infundibulopelvic ligament.

Credit: Collection of Monica Woll Rosen, MD, University
of Michigan

This patient most likely is experiencing ovarian and/or tubal torsion 

. Ovarian torsion accounts for 2.7% of gynecologic emergencies.

 It occurs when the ovary twists on its attachments, which causes decreased blood flow and eventually leads to necrosis. Ovarian and paraovarian cysts, increased length of ovarian ligaments, and enlarged ovaries are risk factors for this condition. A patient is typically evaluated with an ultrasound to assess for a mass and blood flow to the ovary.

Ovarian torsion can be difficult to diagnose preoperatively—the differential includes appendicitis, hemorrhagic cyst, ovarian rupture, and other bowel etiologies. Absent Doppler flow to the ovary suggests torsion, but torsion may be present even with normal flow. 

A recent study of 87 girls with suspected ovarian torsion found that a preoperative diagnosis was confirmed in 48% of postmenarchal patients.

Treatment involves detorsion of the ovary when possible and cystectomy to prevent recurrence.

 There is limited indication for oophorectomy. Detorsion with cystectomy can almost always be done, regardless of ovarian size. There is insufficient evidence to dictate oophoropexy, although this may be considered, particularly in the case of recurrent torsion.

A 14-year-old girl presents to the emergency department with significant abdominal pain. She is premenarchal but has had pelvic cramping for the past 6 to 9 months. She has Tanner stage V breasts and genitalia. On pelvic exam, there is a bulging hymen with a blue hue (

On rectal exam, there is a large anterior bulge immediately inside her rectal opening. On ultrasound, you see a large mass and have difficulty distinguishing the uterus or ovaries. What is your diagnosis?

This presentation and exam are consistent with an imperforate hymen, which occurs in 0.05% to 0.1% of girls.

Abdominal pain at the time of expected menarche is the most common presenting symptom of this condition (54.2%), followed by urinary retention (20.3%) and dysuria (9.7%).

Of note, this condition can and should potentially be diagnosed in the neonatal nursery, as maternal estrogen allows easy visualization of the vaginal introitus. If the neonate is asymptomatic and voiding without difficulty, surgical management can be deferred until puberty. 

More typical is a missed diagnosis until after menarche. Surgical incision of the hymen is then indicated to relieve the backup of menstrual blood. 

Traditionally, this procedure was performed using a cruciate incision, but many providers now use a circular incision to open the hymen. It is important to suture with interrupted stitches so as not to constrict the hymen. This heals very well and should not recur or cause stenosis. No postoperative dilation is needed. Hormonal suppression of menses may be considered until surgery can be performed. This condition, although urgent, is not emergent, particularly if the diagnosis is in question.

Vaginal agenesis also can present with primary amenorrhea and occasional pain due to obstructed uterine remnants. However, there will usually be some hymenal remnants visible on exam and no bulge will be felt on rectal examination. Further imaging with ultrasound followed by MRI is the next step in distinguishing this condition. Other rare obstructing anomalies include a transverse vaginal septum or cervical agenesis.

A 13-year-old girl presents with significant cyclical abdominal pain and amenorrhea. She has Tanner stage V breasts and genitalia. Her external genital exam is completely normal with an appropriate hymenal opening. There is no bulge on the perineum. A small cotton swab placed through the hymen stops at 4 cm. On rectal exam you feel a bulge anteriorly, approximately 4 cm into the rectal canal. Ultrasound reveals a large blood-filled cavity, so you obtain an MRI (

. MRI of a 13-year-old girl with transverse vaginal septum. 

This scenario is most consistent with a transverse vaginal septum. The septum can be located at different levels of the vagina, but external genitalia appear normal. Septa can vary in thickness, with whole segments of the vagina undeveloped.

Because cases of transverse septa are rare and difficult to repair, they should be managed at tertiary centers by experienced pediatric and adolescent gynecologists or other gynecological surgeons. 

Surgery is usually performed vaginally, with assistance abdominally or laparoscopically. Surgery involves resection of the septum, which relieves the obstruction, followed by either a simple pull-through vaginoplasty or by using a skin or buccal graft to cover the defect.

It is imperative that patients perform patient-led vaginal dilation after surgery. A recent study showed that of 21 patients who underwent transverse septum resections and had a postoperative complication of vaginal stenosis or scarring, 14 (56%) either did not dilate afterward or did not dilate correctly.

Therefore, when a transverse septum is diagnosed, menstrual suppression is usually initiated until a patient is old enough to dilate on her own.

The cases above outline gynecological situations and emergencies that are unique to children and adolescents. Pediatric patients may need an exam under anesthesia to thoroughly evaluate anatomy or vaginal complaints. When uncertainties exist, and especially prior to surgical intervention in a child, consider consulting with a gynecologist experienced in pediatric and
adolescent gynecology.

1. Burnham A, Cooke-Barber J, Thacker S, Evans D, Carney D, Boswell W. Non-obstetric vulvar/paravaginal hematoma in an adolescent female with scleroderma: an opportunity for intimate partner violence intervention. 

. 2020;1(4):211-215. doi:10.36518/2689-0216.1043

2.Kim MS, Lee HJ, Joo E, Kang S, Lee MH, Kim HC. Management of non-obstetric traumatic vulvar haematoma: a retrospective review of 33 cases.

. 2021:1-4. doi:10.1080/01443615.2021.1960289

3.Vismara SA, Lava SAG, Kottanattu L, et al. Lipschütz’s acute vulvar ulcer: a systematic review. 

. 2020;179(10):1559-1567. doi:10.1007/s00431-020-03647-y

4.Lorenzo CV, Robertson WS. Genital ulcerations as presenting symptom of infectious mononucleosis. 

. 2005;18(1):67-68. doi:10.3122/jabfm.18.1.67

5.Martin JM, Godoy R, Calduch L, Villalon G, Jordá E. Lipschütz acute vulval ulcers associated with primary cytomegalovirus infection. 

. 2008;25(1):113-115. doi:10.1111/j.1525-1470.2007.00597.x

6.Krapf JM, Casey RK, Goldstein AT. Reactive non-sexually related acute genital ulcers associated with COVID-19. 

. 2021;14(5):e242653. doi:10.1136/bcr-2021-242653

7.Wojcicki AV, O’Flynn, O’Brien KL. Vulvar aphthous ulcer in an adolescent after Pfizer-BioNTech (BNT162b2) COVID-19 vaccination. 

. 2022;35(2):167-170. doi:10.1016/j.jpag.2021.10.005

8. Rosman IS, Berk DR, Bayliss SJ, White AJ, Merritt DF. Acute genital ulcers in nonsexually active young girls: case series, review of the literature, and evaluation and management recommendations. 

. 2012;29(2):147-153. doi:10.1111/j.1525-1470.2011.01589.x

9. Bandow GD. Diagnosis and management of vulvar ulcers. 

. 2010;28(4):753-763. doi:10.1016/j.det.2010.08.008

10. Childress KJ, Dietrich JE. Pediatric ovarian torsion. 

. 2017;97(1):209-221. doi:10.1016/j.suc.2016.08.008

11.Melcer Y, Maymon R, Pekar-Zlotin M, Pansky M, Smorgick N. Clinical and sonographic predictors of adnexal torsion in pediatric and adolescent patients.

. 2018;53(7):1396-1398. doi:10.1016/j.jpedsurg.2017.07.011

12.Dasgupta R, Renaud E, Goldin AB, et al. Ovarian torsion in pediatric and adolescent patients: a systematic review.

. 2018;53(7):1387-1391. doi:10.1016/j.jpedsurg.2017.10.053

13.Lee KH, Hong JS, Jung HJ, et al. Imperforate hymen: a comprehensive systematic review. 

14.Brander EPA, Vincent S, McQuillan SK. Transverse vaginal septum resection: technique, timing, and the utility of dilation. A scoping review of the literature. 

. 2022;35(1):65-72. doi:10.1016/j.jpag.2021.09.002

15.Wancura M, McCracken JM, Steen E, Cosgriff-Hernandez E, Keswani S, Hakim JCE. Emerging technologies in pediatric gynecology: new paradigms in women’s health care. 

. 2019;31(5):309-316. doi:10.1097/GCO.0000000000000563

Part 2 in a series on emergencies in pediatric and adolescent gynecology

Managing genital and pelvic pain in young patients

Gynecologic conditions that are common in girls and adolescents are often not seen in adult patients or can present differently, creating a dilemma for the provider.

 Because these are relatively rare occurrences, this review is designed to guide a general obstetrician-gynecologist through several case-based scenarios of premenarchal vaginal bleeding. These scenarios are intended to give the general gynecologist guidance, but consultation with or referral to a pediatric and adolescent gynecologist in an academic medical center may be indicated.

: A 4-year-old presents with 1 day of spots of blood in her underwear that the mother noticed when helping her daughter change. The child denies any falls; does not complain of pain, dysuria, or itching; and has been supervised by her mother all day. 

Discussion: In this patient, the exam shows whitening of her vulva (classically, in a “figure of 8” distribution that can surround the anus) in addition to several areas of subepithelial hemorrhage (“blood blisters”) and scarring around the clitoral hood, consistent with lichen sclerosus (LS)—a chronic inflammatory skin disease that primarily presents in the anogenital region. It most commonly affects females and may occur premenarche or after menopause, likely due to lower estrogen levels.

 The disease is estimated to occur in 1 in 900 prepubertal girls.

 The pathogenesis of LS is multifactorial and can be associated with other autoimmune conditions including thyroiditis, alopecia, pernicious anemia, and vitiligo.

 Occasionally, LS may look like significant bruising and has been mistaken for abuse.

 The whiteness of the surrounding skin and the presence of scarring should alert the provider to the correct diagnosis.

 Although the association of vulvar cancer has been made in adults, this has not been described in children. Treatment is indicated to alleviate symptoms—usually itching or pain—and to prevent further scarring. Ultrapotent topical corticosteroids are often used initially with tapering to lower-potency ointment for maintenance; tacrolimus may be used as second-line therapy.

It is important to note that, besides trauma-related bleeding (both accidental and nonaccidental/abuse), most of the possible reasons for premenarchal vaginal bleeding are not true emergencies; however, they often present as such due to anxiety in the patient
and caregiver.

The differential diagnosis list for premenarchal vaginal and vulvar bleeding is long 

, with trauma and precocious puberty as the diagnoses that initially need to be ruled out. Vulvar concerns and urologic factors are the most encountered etiologies.

The physical exam of the premenarchal girl who presents with bleeding should always include a breast exam, as any breast development prior to age 7 is cause for concern for precocious puberty.

After an abdominal exam, a vulvar exam should be attempted. This can be more complicated than it would be in an adolescent or adult patient. First, the provider must explain the plan to the patient and parent, including that only an external exam will likely be performed. A speculum examination of a prepubertal girl in the office is never indicated. For a very anxious girl, it is helpful to consider having a child life specialist at the exam if feasible.

The child should sit in a lithotomy position, either on the parent’s lap or in frog-leg position on the exam table, with the desired parent or caregiver by her side. Stirrups are usually not used, as this can make the child uncomfortable. Gentle, lateral traction on the labia majora should be used for vulvar visualization. Gently gripping the labia majora and pulling anteriorly allows the edges of the hymen to be seen.

 Deep breathing should be encouraged, as this helps to open the vagina and further enables the hymenal edges to be more easily appreciated.

 The child should always be informed of the examiner’s actions prior to every maneuver.

If there is a need to look deeper into the vagina or if the child is more comfortable on her abdomen, the child can lie in a prone knee-chest position. In this position, the child lies on her knees with her buttocks elevated and her head resting on the table. Her arms can be positioned above her head or by her side. An otoscope can be used to see inside the lower vagina. Topical lidocaine jelly may be helpful with this exam.

With a similar story but different exam, this patient could also have had a urethral prolapse 

, which has an incidence of 1 in 3000 premenarchal girls.10 The most common presentation is vaginal bleeding, as the prolapsed tissue can be friable; pain may also occur.

 This happens when there is a lack of urethral support, enabling the urethral mucosa to prolapse and form a friable mass, and is more common in Black girls. The distinction between urethral prolapse and a vaginal mass or tumor can be facilitated by observing the child voiding on a bedpan; urine exits through the center of the prolapsed urethra, and anterior to a vaginal mass. Sitzbaths and topical estrogen are the mainstays of treatment; surgical intervention is rarely necessary if necrosis is present.

: A 5-year-old girl presents with foul-smelling bloody vaginal discharge that she has had for 1 week. Based on a normal vulvar exam, except for purulent discharge, you diagnose vulvovaginitis and treat her with oral antibiotics for 7 days. The discharge disappears but returns when the antibiotic course is complete. What is the most likely diagnosis and treatment?

: When discharge persists despite antibiotics, there is suspicion for a foreign body. Foreign bodies occur in up to 18% of girls with bleeding and discharge.

 Until the foreign body is removed, the discharge or bleeding will likely continue. The most common foreign body is toilet paper, although all manner of objects may be intentionally placed in the vagina by the child. It may be hard to see the foreign body in clinic on the exam, even in a knee-chest position. If the foreign body can be easily seen at the introitus, it can be carefully removed with a small instrument. A rectal exam may aid in palpating a firm object, and the object can sometimes be removed by “milking” it toward the introitus. Again, lidocaine jelly may be helpful.

Options for removal of a deeper or nonvisible foreign body include a lavage or irrigation in an office setting, office vaginoscopy or irrigation with an office hysteroscope (Endosee; CooperSurgical). This choice is based on patient anxiety, tolerance of the initial examination, and family preference. If the decision is a clinic lavage, child life assistance can be invaluable. Before performing a lavage, the gynecologist can apply topical lidocaine jelly to the hymen for comfort. With the patient in a frog-leg position, a small pediatric feeding tube or foley is attached to a 20-mL syringe of warm saline. The catheter tube is passed through the hymenal opening and irrigation of the vagina is initiated. Some water pressure may be required to help loosen the foreign body. During irrigation of the vagina, touching the hymen should be avoided. A small foreign object may exit the vagina with this technique.

If an office exam is not desired, pelvic imaging can be obtained, although this does not always show the foreign body.

 A vaginoscopy under general anesthesia using a pediatric cystoscope can be performed to better visualize the vagina and the foreign body. With vaginoscopy, the labia majora can be held closed to allow for vaginal distension and better visualization. Common culprits are toilet paper or cotton balls 

With premenarchal vaginal bleeding, it is important to remember a rare reproductive system malignancy: embryonal rhabdomyosarcoma—also known as sarcoma botryoides.

 This malignancy involves the vagina, uterus, bladder, and urethra and typically presents with the passage of grape-like lesions and/or vaginal bleeding or discharge. Ninety percent of these tumors occur before a child turns 5 years of age, with most occurring before age 2. Growth is rapid and treatment involves a combination of surgery, chemotherapy, and radiation, decided upon using a multidisciplinary team approach.

 Fortunately there are high cure rates, so early detection is imperative.

You are paged urgently to the emergency department to see an 8-year-old girl who reports falling on the side of the bathtub an hour ago. She reports pain in her genital area after straddling the tub, along with some vaginal spotting. What do you do next?

Discussion: This girl has suffered a straddle injury. Straddle injuries are the most common gynecologic injury in this age group and are usually the result of blunt trauma,

 which occurs when soft tissue is compressed against bone, causing bruising and superficial lacerations. Hymenal involvement
is rare.

In this case, the provider should first assess the patient’s stability, then take a history. There may be concern for nonaccidental trauma, so this should include the exact circumstances of how the trauma occurred and if it was witnessed. If possible, the provider should interview the child alone if she is of an appropriate age. On examination of the vulva, it is important to specifically evaluate whether the injury fits the history that was given. The hymen should be assessed to see if it is involved. In a typical straddle injury on a nonsharp surface, a hymenal laceration is uncommon; therefore further evaluation of the circumstances of this trauma must occur, possibly with the help of a child protection team or social worker.

In findings from recent studies, 1 in 5 girls are estimated to experience sexual abuse during childhood.

 It is important to remember, however, that most children who are evaluated for suspected sexual abuse will not have any signs of injury; minor injuries can heal quickly, and bruising, abrasions, and lacerations are rare in children examined more than 72 hours after experiencing abusive trauma.

If there is significant bleeding, irrigating with warm water may help delineate the extent of the trauma. Applying topical lidocaine prior to the exam can also be helpful, although it may sting on an open wound. An exam under anesthesia with possible vaginoscopy should be considered for any of the following: the patient is unable to tolerate an exam, vital signs are unstable, the extent of the injury or laceration cannot be appreciated at the bedside, there is active bleeding, or the pain is not responding to appropriate measures.

Part 1 in a series on emergencies in pediatric and adolescent gynecology.

Monica Rosen, MD

Articles

Reproductive health care for adolescents with disabilities requires special consideration

Managing genital and pelvic pain in young patients

Have no fear! Premenarchal vaginal bleeding

Choosing the right pill

Bleeding disorders: When to worry, how to help