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Clinical ShowCase™: Developing a Personalized Treatment Plan for a Patient with Sickle Cell Disease

Clinical Showcase Sickle Cell 

Ms. Hester is Content Specialist with Contemporary OB/GYN and Contemporary Pediatrics.

 evokes images of early adolescence and for most children, that is when they transition to this next stage of growth. However, some children who are going through central precocious puberty (CPP) will enter puberty earlier than their peers, in some cases much earlier. In a webinar from the 

 series, 2 experts discussed a variety of subjects on CPP, including findings from a recent study that examined treatment initiation and duration with height, the need to individualize treatment decisions for girls with CPP, and 2 case reports.

 Karen O. Klein, MD, a pediatric endocrinologist at Rady Children’s Hospital-San Diego in California and an associate clinical professor of pediatrics at the University of California San Diego School of Medicine, led the discussion with Kent L. Reifschneider, MD, a pediatric endocrinologist at Children’s Hospital of The King’s Daughters in Norfolk, Virginia.

CPP occurs when the hypothalamic-pituitary-gonadal axis is activated early on, which leads to an increase in gonadal steroid hormone secretion. The ages of affected children are less than 8 years for girls and less than 9 years for boys. Children affected by CPP develop secondary characteristics early on; have at least 1 Tanner stage progression per year; and have less than 2 standard deviations in bone age (BA) beyond chronological age (CA). If untreated, CPP can lead to early fusing of growth plates, a reduction in adult stature, atypical pubertal physical development, and premature menarche. Gonadotropin-releasing hormone agonists are the standard-of-care treatment.

Klein discussed her recent study coauthored with Marcela Vargas Trujillo, MD; Sanja Dragnic, MD; and Petra Aldridge, MS. The authors performed the investigation to supply data for an area in which data were scarce: the benefit to height outcomes when treatment with leuprolide was initiated after CA of 7 years and continued after CA of 10 years or BA of 12 years. The study was carried out in 48 treatment-naïve girls who were treated with monthly leuprolide, administered intramuscularly every 28 days at doses based on weight. The investigators only included girls so that the results would have statistical power. The patients had visits at weeks 4, 8, 12, 24, 36, and 48 and then every 6 months until leuprolide was discontinued. They then had annual visits until age 21 years. The average treatment duration was 3.9 plus/minus 2.0 years (range, 1.1-9.8). The average CA at the end of treatment was 11.5 plus/minus 0.97 years (range, 9.8-14.1) whereas the average BA was 12.7 plus/minus 0.94 years (range, 11.5-16.4). Throughout the treatment period, the ratio of BA to CA consistently decreased. Fifteen girls achieved a BA that was less than 1 year above the CA. Longer duration of treatment was associated with increased predicted adult height for nearly all the girls, 45 of 48. Those who saw no improvement were either near midparental height when they started the treatment or showed poor growth velocity. A wide range was found in final heights: 162.54 plus/minus 7.35 cm (range, 146.47-176.11) that was similar to the predicted adult height in treatment (162.39 ± 8.38 cm). Midparental height was not available for all the girls, but of the 29 for whom it was available, 17 either met or surpassed it.

Reifschneider joined Klein to discuss nuances in the treatment of CPP. He noted, “With CPP, I think of the limitations of the disease state, which includes the impact of estrogen on growth velocity and BA and how much that should factor into clinical decisions as well as the variability in interpreting BA.”

Klein added, “When examining a patient, a clinician should balance the need for more data with the fact that early commencement of treatment can lead to better height outcomes.” She also noted that estimating predicted adult height is also mostly based on average-sized children who are having average growth patterns. Another consideration for starting treatment is the predicted adult height of the patient and the height of the child’s parents. For example, if the predicted height for a child is 155 cm and the mother is 157.5 cm, treatment may not be considered necessary by the patient’s family.

Klein added that although treatment will pause the tempo of puberty, once treatment ends, the tempo will resume its speed. However, this does not mean that a pubertal growth spurt will occur, and, in fact, data indicate that this typically doesn’t occur. 

The first case Reifschneider and Klein examined concerned a girl, with the following details:

Tanner stage 3 breast development, starting at age 7 years

With the girl’s BA and current height, her predicted final height is roughly 147 cm and her midparental height, 168 cm. The BA maturation was noted as significant by Reifschneider and the patient would meet his criteria for treatment. However, Klein emphasized the patient’s age, noting that she had been trained to only treat children when pubertal onset occurred before age 8 years. Countering that assertion, Reifschneider said, “One has to keep in mind the individual and that a lab [finding] or CA is just a piece of the puzzle. In this case, the fact that the girl started developing breast tissue when she was aged 7 years is important, in addition to growth velocity that is greater than prepubertal [velocity] as well as bones that are considerably advanced.”

When discussing treatment with families, a conversation about outcomes can be important. Many girls with CPP can be among the tallest girls in their class but their BA is often much advanced, and when their non-CPP peers reach puberty, many affected girls will be left behind in terms of height. If he were treating this patient, Reifschneider said, he would ideally initiate treatment and then see her every 4 to 6 months to determine how the treatment has changed the tempo of her puberty. Once the tempo became prepubertal, he would transition to twice yearly maintenance screenings.

The second case was of a girl with CPP who had undergone treatment, with the following details:

Leuprolide acetate treatment initiated at age 6 years

1 incidence of vaginal bleeding following treatment initiation

Tanner stage 3 breast development at age 6 years

The patient’s breast development has stabilized at Tanner stage 3. Her BA is 12 years and her height is currently predicted to reach 60 cm. She is now asking whether it’s time to stop treatment.

Reifschneider emphasized that this was a very successful case and the patient had responded very well to therapy. “When considering the ceasing of treatment, there are a couple of things that need to be thought through, like the limitation of predicted adult heights and not knowing the tempo that will occur when she ceases treatment. Although she is currently predicted to reach 5 feet, there is a likelihood that she would not achieve that height if she ended treatment.” Klein added that a BA of 12 years has often been considered the end point for treatment because of questions about whether continued growth could be achieved, but her study findings showed that 5 to 10 additional cm can be achieved with continued treatment. Reifschneider confirmed that prior to publication of the study’s results, he would have considered ending treatment at BA 12, although he noted that this patient was an outlier who likely would continue to benefit from treatment.

1. Klein KO, Reifschneider K. Between the lines: central precocious puberty. Virtual webinar. Recorded March 4, 2022.

Articles

Two experts discuss a variety of subjects on central precocious puberty, including recent study findings that examined treatment initiation and duration with height, the need to individualize treatment decisions for girls with CPP, and 2 case reports. 

Between the lines: central precocious puberty

The Society for Pediatric Dermatology was founded in 1975. As the only national organization in the United States specifically dedicated to the subspecialty of pediatric dermatology, its annual conference is something its 1400+ members look forward to each year. Meena Julapalli, MD, a board-certified pediatric dermatologist at Bluebird Dermatology in Houston, Texas, eagerly awaits this year's meeting, and talks about the sessions she is most looking forward to attending.

Videos

Meena Julapalli, MD, notes the sessions she is most looking forward to at the Society for Pediatric Dermatology in Indianapolis, Indiana.

Meena Julapalli, MD, gets ready for the Society for Pediatric Dermatology conference

As we head into summer, all eyes will be turning to the Supreme Court in anticipation of its decision in the case of 

, which is challenging a Mississippi law that bans abortions after 15 weeks of gestation. The court heard arguments in the case in December 2021.

Based on questions posed at the time and on previous decisions by the majority of the justices, most observers expect the Mississippi law will be upheld, effectively overturning 

 and ending the constitutional right to pregnancy termination in the United States. This expectation is further supported by the recent and unprecedented leak of Justice Samuel Alito’s draft decision, which challenges the validity of


The implications for abortion care of overturning 

 are clear. Each state will be free to restrict or ban abortions as it sees fit. Indeed, many have already passed laws that do this, and 13 have “trigger laws” ready to go into effect as soon as 

 is undone. A variety of approaches are being taken. Like Mississippi, many states are developing time-based restrictions (eg, abortion bans after a given gestation time). Others, anticipating a complete reversal of 

However, restrictions will not be limited to pregnancy termination. If the rationale Justice Alito described in the leaked decision is accepted, the whole framework that has given rise to reproductive and sexual privacy may be at risk. The leaked decision very much sets the stage for challenges to the decisions that protected same-sex marriage, struck down antisodomy statutes, and established the right to contraception.

Fertility care is also at significant risk. Many of the bills being introduced in statehouses around the country (over 500 have already been put forward, according to the Guttmacher Institute) include language defining a fertilized egg as a legal human entity.1 The terminology varies, which makes it difficult to generalize, but the danger is clear.

If the legal status of fertilized eggs or early embryos is codified, in vitro fertilization (IVF) procedures may become legally risky for patients, physicians, and staff.

The danger posed by some legislative proposals is obvious. For instance, Nebraska’s Legislative Bill 933 (the state has a unicameral legislature) asserts that an “unborn child means an individual living member of the species homo sapiens, throughout the embryonic and fetal stages of development from fertilization to full gestation and childbirth” and that the “causing or abetting the termination of life of an unborn child” will be unlawful. This bill clearly classifies an IVF-created embryo as an unborn child.

If you put a 3-year-old child in a freezer, you will kill them and presumably will have committed homicide. But what if you put a blastocyst in a cryopreservation tank? Most blastocysts will survive the thaw. However, the risk of not surviving is approximately 1%. Would that constitute homicide?

This happens every day in fertility clinics. In fact, most children born as a result of IVF procedures in the United States are born from frozen embryos. According to the Society for Assisted Reproduction (SART), 85.5% (n = 62,915) of total live births from embryo transfers in 2020 (N = 73,602) were transfers of thawed embryos.

Sometimes these questions are even more challenging. Oklahoma’s SB 1553 also defines life and legal status as beginning at fertilization—but also bans acts that would “induce an abortion if more than 30 days have elapsed since the probable beginning of the last menstrual cycle of the pregnant woman.”

Obviously, the “probable last menstrual cycle” from which a frozen embryo is derived may have taken place a long time before the embryo is used. How, then, does this wording apply to in vitro procedures? Your guess is as good as ours.

But it’s safe to say that none of the physicians providing infertility services want to be the test case that determines whether they have violated the law by putting a “person” in a tank of liquid nitrogen.

The ability to cryopreserve sperm, eggs, and embryos has revolutionized infertility care over the past 20 years. Contemporary best practices in IVF call for a single embryo transfer to mitigate the risk of multiple births. Some clinics perform “freeze all” cycles, whereas others transfer 1 embryo immediately and freeze the rest for subsequent use. Under some proposed legislation, the legal status of these embryos could be in flux. The use of single frozen embryos has enabled fertility doctors to improve the safety of procedures without compromising the probability of pregnancy from one in vitro fertilization cycle. In fact, SART reports that 88.3% of all live births (fresh and frozen embryo transfers) were singletons, and 89.5% of live births from frozen embryos were singletons.

In addition, the use of preimplantation genetic diagnosis has allowed physicians to determine whether embryos carry genetic diseases, such as Huntington and Tay-Sachs disease, and thus to eliminate these horrible conditions from the blood lines of patients. However, this technique requires the use of frozen embryos. What becomes of embryos that are not transferred because they are known to carry such diseases? Will they remain frozen forever, and who will pay for this?

The legislative risk to IVF is not limited to frozen embryos. Problems in an IVF lab can impact embryo health. In fact, only about 60% of fertilized eggs survive to the blastocyst stage in the lab.

At what point will the embryos be considered nonviable? Can a physician be accused of impacting viability? What happens if equipment failure affects the culture of a blastocyst? What if the embryologist accidentally drops an embryo? Although these events are very rare, do they constitute homicide? What if a tank of frozen embryos fails and the embryos are destroyed: Would that be considered mass murder? Will physicians still be allowed to offer patients the best treatments, or will they risk being charged with a felony? For America’s fertility doctors, these are no longer hypothetical questions.

The moral and legal status of the developing fetus is a complicated question, one that science is poorly equipped to answer. Unfortunately, the current political and legal environment in the United States does not lend itself to informed, complicated, or nuanced discussions.

As a result, we will be dealing with blunt policy outcomes. “Life begins at conception” has become an article of faith for some in the antichoice community. For others, however, reality can intrude. Thus, we get laws that allow abortion in cases of rape or incest. Only the most extreme elements of the antichoice movement would want to consign women with ectopic pregnancies to death and, therefore, most legislative proposals allow them to be treated.

Nevertheless, we have seen states seeking to ban such treatments or mandate what we know is scientifically unsound to “save” ectopic pregnancies. Similarly, some state laws attempt to carve out contraception, although these attempts may be more about bowing to political realities than about the appreciation of scientific facts.

IVF is one risk facing reproductive care if 

 is overturned. But it’s far from the only risk. 

, rest in part on a crucial right not explicitly articulated in the first 10 amendments to the Constitution: the right to privacy.

This right was fundamental to the findings in 

, which established the right to contraception originally available only to married women. Should the Supreme Court overturn Roe, that decision might include an explicit rejection of the right to privacy, which could have crucial implications for reproductive medicine.

We have already seen laws enacted that make treating transsexual patients illegal, in most cases based on age, but now we are seeing bills that block the care of adult patients as well.

If there is no constitutional right to privacy, there may be no end to the government’s right to limit reproductive medical procedures. If abortion and caring for trans patients can be made illegal, why should we suppose there will be a meaningful limit to what state legislatures or Congress could ban?

Most antichoice activists, and the politicians doing their bidding, don’t really want to stop would-be parents from receiving the medical treatments they need to raise families. However, they seem to have no problem if the infertile are the collateral damage required to win the abortion wars.

Jared Robins, MD, is executive director of the American Society for Reproductive Medicine (ASRM). Prior to joining ASRM, he was a practicing reproductive endocrinologist working in both academic and private settings.

Sean Tipton serves as the chief policy and advocacy officer for the American Society for Reproductive Medicine. He has more than 20 years experience working on reproductive health issues.

Originally published on our sister brand, 

1. Nash E, Cross L, Dreweke J. 2022 State legislative sessions: abortion bans and restrictions on abortion dominate. Guttmacher Institute. March 16, 2022. Accessed May 4, 2022. https://www.guttmacher.org/article/2022/03/2022-state-legislative-sessions-abortion-bans-and-restrictions-medication-abortion

2. Preliminary national summary report for 2020. Society for Assisted Reproductive Technology. Accessed May 4, 2022. https://www.sartcorsonline.com/rptCSR_PublicMultYear.aspx?reportingYear=2020

With the Supreme Court's overturning of Roe v Wade eliminating the constitutional right to an abortion, there are questions about its impact on fertility care.

Not just abortion: How the demise of Roe v Wade may impact fertility care

Vulvovaginal complaints make up the majority of gynecologic clinic visits by prepubertal girls.

Vulvovaginal symptoms can be caused by infection, trauma, hygiene, dermatologic conditions, and congenital anomalies. Though these conditions also may present after puberty, this article will focus on common vulvar conditions in the prepubertal pediatric population.

Most pediatric vulvar conditions can be clinically diagnosed with history and physical exam alone, although certain conditions may require additional testing. History taking often requires gathering information from both the child and parent and should focus on the timing, duration, and characterization of the symptoms, including itching, pain, burning, and associated genitourinary and gastrointestinal symptoms such as dysuria, urinary retention, constipation, and stooling habits.

Vulvar hygiene should be elicited, including the use of perfumed soaps, bubbles, detergents, or wipes applied to the external and internal genitalia, as well as who performs these vulvar hygiene practices (child or parent).

A specific inquiry should address whether the child wipes front to back, has inserted any foreign objects into the vagina, or has experienced any inappropriate touching of their genitals. Symptom triggers and irritants should be investigated, as well as the outcomes of prior palliative measures and interventions. A report of vaginal bleeding, discharge, or malodor is nonspecific but may raise concern for tissue injury, infection, or abuse.

External pelvic examination should be performed to assess the patient’s pubertal development, including Tanner staging and documentation of the degree of estrogenization of the vestibule and vaginal mucosa.

Genital examination of a prepubertal child can be accomplished by asking the child to make a frog leg or butterfly position, a cannonball position (to better assess the perianal region), or a hands and knees position.

 Skin changes and tissue injuries such as excoriations, abrasions, or bruising are often immediately apparent.

Of note, the vestibule, hymen, and vaginal mucosa of prepubertal girls commonly appear erythematous, thin, and atrophic due to the poor estrogenization of the tissues. Careful attention should be paid to deviations from normal vulvar anatomy. The patient should always be verbally prepared for the anticipation of touching of the skin and tissue and be reassured that the examination will not hurt.

Most pediatric gynecology exams will be tailored to the complaints and not require speculum examination. Gentle traction of the labia majora outward allows assessment of hymenal patency and visualization into the lower vagina 

 Examination of the vulva of a prepubertal child. (Reported in: Emans SJ, et al. 

Emans, Laufer, Goldstein's Pediatric Gynecology.

 7th ed. Figure 1-6 (B), page 5, Philadelphia, Wolters Kluwer, 2020).

If necessary, further assessment of vaginal patency and length may be performed with a saline-moistened cotton-tipped applicator, which can be used to obtain bacterial cultures if indicated.1 If further intravaginal evaluation is indicated, a vaginoscopy in the office or under anesthesia may be performed.

Nonspecific vulvovaginitis is the most common (75%) cause of vaginitis in young girls.

 Pediatric patients typically describe vulvar discomfort, pruritus, malodor, or vaginal discharge that may be purulent or tinged with blood.

Signs include erythema, mucoid vaginal discharge, and poor hygiene—or there may be no findings. The prepubertal vulva and vagina are hypoestrogenic and susceptible to irritants such as soaps, detergents, bath bombs, urine, stool, and perfumed products.

In the absence of purulent discharge, antibiotics are rarely indicated. Most symptoms resolve within 2 to 3 weeks after vulvar hygiene measures are instituted, including soaking in plain water without soap, standing up to shower, patting dry, wiping front to back, wearing cotton underwear and loose clothing, and avoiding irritants.

 Barrier emollients such as petroleum jelly or zinc oxide can be applied externally for symptomatic relief.

Additional testing with bacterial vaginal culture and vaginoscopy may be indicated to evaluate for specific causes of vulvovaginitis if symptoms persist or vaginal bleeding or purulent discharge is present, raising suspicion for a specific etiology.

Mixed vaginal flora is a typical culture result in nonspecific vulvovaginitis. Respiratory (eg, 

Streptococcus pyogenes, Staphylococcus aureus, Streptococcus pneumoniae, Haemophilus influenzae, Neisseria meningitidis, Moraxella catarrhalis

coli, Enterococcus faecalis, Klebsiella pneumoniae, Proteus mirabilis, Pseudomonas, Shigella, Yersinia

) pathogens are commonly isolated on bacterial vaginal swabs in patients with vaginitis as well as those who are asymptomatic. Therefore they do not always represent an infection.

Colonization is thought to occur by autoinoculation, with group A streptococci accounting for 20% of infectious cases. It should be treated with a 10-day course of oral amoxicillin.

Other respiratory and enteric flora isolates often resolve with hygiene measures alone but should be treated based on sensitivities if symptoms are persistent or if specific infectious causes such as 

Though approximately 3% to 4% of prepubertal girls are colonized with candida, vulvar candidiasis most frequently occurs in patients wearing diapers.8 Pathogenic vaginal candidiasis is uncommon in healthy, toilet-trained prepubertal girls. Therefore, empiric treatment for vaginal yeast infection should not be performed unless a positive candida test is present in the setting of immunosuppression, recent antibiotics, or diaper use.

 can be isolated even in the absence of sexual abuse, and treatment should be based on culture results and symptoms—vaginal pH testing and microscopy are not reliable testing measures in prepubertal girls.

Sexually transmitted infection testing for chlamydia, gonorrhea, trichomonas, and herpes simplex (if ulcerative lesions present) should be performed if there are concerns for sexual abuse. Empiric treatment for pinworms may be indicated in patients with significant perianal and vulvar itching that does not respond to hygiene measures.

Chronic vulvovaginitis with vaginal discharge, malodor, and bleeding is often secondary to a foreign body such as toilet paper, toys, beads, or other small objects 

 Toilet paper within the vagina of a prepubertal child

 (Reported in: Emans SJ, et al. Emans, Laufer, Goldstein’s Pediatric & Adolescent Gynecology. 7th ed. Figure 14-5, page 185, Philadelphia, Wolters Kluwer, 2020).

If the patient is able to cooperate, visualization and foreign body removal in the office can often be accomplished with the application of topical anesthetic if needed and use of a swab or vaginal irrigation with warmed fluid.3 Small batteries are a rare but serious cause of vaginal burns.

 Batteries are radio-opaque on x-ray imaging and require emergent removal under sedation, followed by vaginoscopy to assess the vaginal mucosa for depth of burns and possible damage to the urinary tract or rectal mucosa.

Labial adhesions commonly occur in girls aged 3 months to 6 years due to the prepubertal hypoestrogenic genital tissue 

 (Reported in: Emans SJ, et al. Emans, Laufer, Goldstein’s Pediatric & Adolescent Gynecology. 7th ed. Figure 14-13 (B), page 189, Philadelphia, Wolters Kluwer, 2020).

Other contributing factors may include genital trauma, infection, inflammation, or lichen sclerosus. Adhesions may be asymptomatic or cause vulvar discomfort and urinary symptoms such as postvoid dribbling, difficulty with urination, and recurrent urinary tract infections.

Small, asymptomatic labial adhesions can be expectantly managed. Most will resolve with endogenous estrogen production at the onset of puberty.

Symptomatic or extensive labial adhesions warrant therapy with topical estrogen (0.01%) cream or high potency (betamethasone 0.05% cream) steroid applied sparingly to the fusion line and continued until resolution.

These topical treatments have similar efficacy, and the choice of therapy is dependent on the acceptability of potential adverse effects (eg, breast budding, skin atrophy), which can often be mitigated by directed focal application for the minimum duration needed.

Resolution often occurs within 6 weeks of treatment and should be followed by vulvar hygiene measures and daily application of barrier emollients such as petroleum jelly for several months to prevent recurrence. Manual or surgical separation is rarely indicated and reserved for cases of complete urinary obstruction and retention.

Vulvar lichen sclerosus is an inflammatory dermatologic condition affecting the anogenital region with a bimodal incidence in postmenopausal women and prepubertal girls.

Common symptoms include vulvar pruritus, pain, bleeding, and urinary and bowel complaints such as dysuria and constipation or pain with defecation.

Dermatologic findings include thin, parchment paper–like skin with hypopigmentation frequently in a figure-8 distribution around the anus and vestibule. Lichen sclerosus also can have diverse and subtle presentations including erythema, bruising, punctate hemorrhages, telangiectasias, excoriations, fissures, and hyperkeratotic lesions 

 (Reported in: Emans SJ, et al. Emans, Laufer, Goldstein’s Pediatric & Adolescent Gynecology. 7th ed. Figure 14-4, page 184, Philadelphia, Wolters Kluwer, 2020).

Untreated, severe lichen sclerosus can cause scarring and loss of the vestibular architecture leading to loss of the labia minora, labial agglutination, introital stenosis, and burying of the clitoris under the prepuce.

Of note, vitiligo of the vulva will demonstrate hypopigmentation but without changes in skin texture or architectural changes.

 Vaginal involvement is uncommon in lichen sclerosus and, if present, should raise concern for an alternative diagnosis such as lichen planus. The etiology of lichen sclerosus is unclear but has been associated with familial inheritance, local trauma, and systemic autoimmune disorders.

Although lichen sclerosus is associated with the development of vulvar carcinoma in postmenopausal women, there is no evidence of this association in young girls.

Skin biopsy is rarely indicated in the pediatric population, and clinical diagnosis based on examination should prompt initiation of topical therapy with a super high potency steroid such as clobetasol propionate 0.05% ointment twice daily.

 Medical management is continued until symptoms and skin findings resolve, and then it is tapered over 4 to 8 weeks.

After resolution, maintenance therapy with a potent topical steroid such as mometasone furoate 0.1% ointment is recommended.

 As lichen sclerosus commonly recurs in 44% to 82% of patients and can persist beyond puberty, repeat treatment courses are often required and long-term follow-up with annual genital exams is recommended.

Vulvar ulcers are distressing for young girls and their parents and can cause significant discomfort. The differential diagnosis for vulvar ulcers is broad and should include testing to exclude infection.

 Herpes simplex virus is characterized by clustered vesicles and painful ulcerations confirmed via viral culture or polymerase chain reaction assay.

Primary syphilis causes a painless chancre characterized by an isolated ulcer with raised borders with or without regional lymphadenopathy and is diagnosed by dark field microscopy of the chancre fluid.

Less common infectious etiologies include chancroid (

). These can be diagnosed with bacterial culture of the ulcerative lesion. A positive result of any of these sexually transmitted infections warrants prompt treatment and investigation of suspected
sexual abuse.

Beçhet syndrome is a systemic autoimmune disorder that manifests with arthritis, uveitis, and recurrent ulcerative oral and vulvar lesions.

 Genital Crohn disease can present with “knife cut” genital ulcers along the inguinal or labial folds. A careful history of associated symptoms may prompt referral to specialists to rule out these systemic etiologies.

Lipschütz ulcers are a common cause of acute-onset non–sexually transmitted vulvar aphthous ulcers in both pre- and postmenarchal girls and adolescents.

 Lesions are characterized by large, often bilateral “kissing” ulcers with a necrotic-appearing base and gray exudate on the medial aspect of the labia minora 

 (Reported in: Emans SJ, et al. Emans, Laufer, Goldstein’s Pediatric & Adolescent Gynecology. 7th ed. Figure 17-9, page 267, Philadelphia, Wolters Kluwer, 2020).

They are often accompanied by significant edema, pain, dysuria, and occasionally urinary retention due to discomfort. Prodromal symptoms such as fever, malaise, myalgias, lymphadenopathy, and pharyngitis are common, and these ulcerative lesions have been associated with viral and bacterial infections such as Epstein-Barr virus, cytomegalovirus, influenza, mycoplasma, and strep throat, though serologic confirmation of these infections is not required for this diagnosis of exclusion.

The pathogenesis of these ulcers is unclear but it is thought to be secondary to a hypersensitivity reaction to pathogens and has even been reported after vaccinations.

 Lipschütz ulcers are self-limiting, with most symptoms resolving within 10 days, but lesions may take several weeks to heal. Rarely, bladder catheterization is required to relieve urinary retention secondary to severe discomfort with urination.

Conservative management includes oral pain medications, cleansing with a spray bottle and sitz baths, and application of topical anesthetics and barrier emollients. Antibiotics are reserved for cases with concern for overlying cellulitis.

There is limited retrospective evidence that use of systemic and high-potency topical corticosteroids may lessen inflammation and pain, though no reduction in duration of symptoms has been demonstrated and this intervention has not been investigated in prospective trials.

 Recurrences may occur in 30% to 50% of cases.

Prepubertal children are more susceptible to vulvar trauma due to the vascularity of the vulva and absence of protective fat pads. In addition to excluding sexual trauma, the nature of the injury should be investigated to consider internal or deep trauma.

Blunt trauma to the highly vascular anatomy increases risk of hematoma formation, which is typically conservatively managed with pain medications and cool compresses but may require Foley catheter placement for bladder drainage if obstructive symptoms are present.

Surgical drainage is rarely indicated for large or expanding hematomas because the bleeding vessel is rarely identified and drainage raises risk for infection and bleeding due to loss of natural tamponade by the surrounding tissues.

If vulvar lacerations are small, superficial, and hemostatic, they often can be conservatively managed without repair.

Most injuries are superficial and limited to the mons, clitoral hood, labia, and perineum, and bleeding lacerations can be repaired with a fine absorbable suture with a topical or local anesthetic and sedation. Internal injuries often require examination under sedation with vaginoscopy to assess the depth of the injury and repair.

The majority of vulvar conditions and symptoms in children can be clinically diagnosed with history and examination. Providers should be familiar with these common pediatric vulvar conditions and their initial work-up and management. In cases of unclear diagnosis or failure to respond, referral to a pediatric gynecologist is recommended.

Providers and patients can obtain additional educational information and resources at 

 with specific information relating to clinical presentation, diagnosis, and management of common prepubertal vulvar conditions as well as advice on vulvovaginal hygiene measures.

A thorough history taking and external examination often yield a clinical diagnosis for vulvovaginal conditions. When intravaginal examination is required to rule out a foreign body, vaginoscopy or an examination under anesthesia may be required.

Nonspecific vulvovaginitis is a common complaint that is often responsive to improved vulvar hygiene measures. Antibiotics should be initiated only if a specific pathogen is isolated, if purulent discharge is present, or if symptoms persist despite hygiene measures.

Labial adhesions are common in prepubertal girls due to the hypoestrogenic vulva, and a topical estrogen or steroid should be prescribed if the patient is symptomatic.

Lichen sclerosus is a chronic dermatologic condition that requires treatment with high-potency topical steroids followed by maintenance therapy and long-term follow-up.

Vulvar aphthous ulcers can occur in isolation or after viral illness and require exclusion of infectious causes followed by symptomatic management.

Jessica Y. Shim, MD, is a clinical instructor in the Department of Obstetrics, Gynecology, and Reproductive Biology at Harvard Medical School in Boston, Massachusetts. She is also an attending physician of the Division of Pediatric and Adolescent Gynecology in the Department of Surgery at Boston Children’s Hospital.

Danielle T. Cipres, MD, is a clinical instructor in the Department of Obstetrics, Gynecology, and Reproductive Biology at Harvard Medical School in Boston, Massachusetts. She is also a clinical fellow in the Division of Pediatric and Adolescent Gynecology in the Department of Surgery at Boston Children’s Hospital.

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Emans, Laufer, Goldstein’s Pediatric and Adolescent Gynecology, 7th ed

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Beyitler İ, Kavukcu S. Clinical presentation, diagnosis and treatment of vulvovaginitis in girls: a current approach and review of the literature. 

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Understanding signs, symptoms helps determine the best treatment regimen.

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