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1.
J Drugs Dermatol ; 23(6): 450-455, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38834221

ABSTRACT

BACKGROUND/OBJECTIVES: Lichen sclerosus (LS) is a chronic condition that warrants close follow-up due to the risk of scarring. The optimal long-term management of pediatric vulvar and perianal lichen sclerosus (PVPLS) is unknown. This study aimed to identify diagnostic, treatment, and maintenance regimens among pediatric dermatologists and pediatric/adolescent gynecologists, as well as assess provider confidence and desire for guidance on long-term PVPLS management. METHODS: A cross-sectional 35-question survey was administered through the Pediatric Dermatology Research Alliance (PeDRA) and the North American Society for Pediatric and Adolescent Gynecology (NASPAG) between 7/13/2021 and 8/30/2021 to ascertain PVPLS diagnostic and management regimens. RESULTS: Most responders were attending-level pediatric/adolescent gynecologists (46%) and pediatric dermatologists (41%). Although 85% of participants felt completely or very confident in diagnosing PVPLS, the majority (86%) desired further management guidelines. While the initial treatment was similar among providers, maintenance regimens and follow-up varied considerably, with only 42% recommending lifelong monitoring despite potential persistence into adulthood. CONCLUSIONS: While initial treatment was similar among practitioners, there was variation by specialty in subsequent management and a lack of uniformity in long-term follow-up. Additional studies are needed to clarify the optimal management of PVPLS and to provide evidence-based guidelines regarding long-term follow-up.  J Drugs Dermatol. 2024;23(6):450-455.     doi:10.36849/JDD.8084.


Subject(s)
Dermatologists , Gynecology , Practice Patterns, Physicians' , Humans , Female , Cross-Sectional Studies , Dermatologists/statistics & numerical data , Practice Patterns, Physicians'/statistics & numerical data , Practice Patterns, Physicians'/standards , Child , Gynecology/statistics & numerical data , Gynecology/standards , Adolescent , Surveys and Questionnaires/statistics & numerical data , Male , Vulvar Lichen Sclerosus/diagnosis , Vulvar Lichen Sclerosus/therapy , Vulvar Lichen Sclerosus/drug therapy , Dermatology/methods , Dermatology/standards , Dermatology/statistics & numerical data , Lichen Sclerosus et Atrophicus/diagnosis , Lichen Sclerosus et Atrophicus/therapy , Practice Guidelines as Topic , Gynecologists
2.
Pediatr Ann ; 53(3): e104-e108, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38466333

ABSTRACT

Heavy menstrual bleeding has a high prevalence and is well documented in adult patients with hypermobile-type Ehlers-Danlos syndrome, but there is limited research surrounding work-up and treatment for the adolescent population. Excessive menstrual blood loss can significantly interfere with emotional and physical quality of life. A provider should acquire a comprehensive medical and menstrual history and focused physical examination, as well as baseline laboratory studies, to determine the presence of anemia or underlying bleeding disorder. Use of a pictorial blood assessment chart may be considered to help quantify the amount of bleeding. Treatment to reduce heavy menstrual flow and referral to specialty care should be initiated swiftly to improve quality of life for this population. [Pediatr Ann. 2024;53(3):e104-e108.].


Subject(s)
Ehlers-Danlos Syndrome , Joint Instability , Menorrhagia , Adolescent , Female , Humans , Ehlers-Danlos Syndrome/complications , Ehlers-Danlos Syndrome/diagnosis , Ehlers-Danlos Syndrome/therapy , Joint Instability/complications , Joint Instability/diagnosis , Joint Instability/therapy , Joint Instability/congenital , Menorrhagia/diagnosis , Menorrhagia/etiology , Menorrhagia/therapy , Quality of Life
3.
Clin Endocrinol (Oxf) ; 100(2): 143-148, 2024 02.
Article in English | MEDLINE | ID: mdl-37708074

ABSTRACT

OBJECTIVE: Serum Anti-Mullerian Hormone (AMH) concentrations have been proposed as a marker of spontaneous puberty and future fertility in Turner syndrome (TS). Gonadotropins during minipuberty may also provide a clue to ovarian function but there is insufficient data to inform utility in the routine clinical management of TS. Our objective was to describe the distribution of AMH in a cross-sectional cohort of patients in a TS specialty clinic, and correlate with spontaneous puberty and karyotype, as well as gonadotropins during the minipuberty of infancy in a smaller subset of patients aged 2-9 months. DESIGN: Retrospective chart review of patients seen in the TS clinic at Children's National Hospital from 1/1/2019 to 8/24/2022, to assess AMH and correlate with karyotype and spontaneous puberty. RESULTS: Among 114 patients (median age 9.6 year, 0.08-22 year), AMH values were detectable in only (40/104) 38%, and higher mean AMH (2.7 ± 0.95 ng/mL) was seen in mosaic 45,X/46,XX karyotype compared to 45,X (0.03 ± 0.14 ng/mL) (p < .001), and structurally abnormal-X karyotype (0.11 ± 0.5) (p = .0003). Mean AMH was higher (1.4 ± 1.6 ng/mL) among those with spontaneous menarche compared with spontaneous thelarche but no menarche. AMH was detectable in 2/10 during minipuberty in those with the lowest luteinizing hormone (LH). CONCLUSIONS: Our institutional data reflects a diverse cohort of patients seen in a TS specialty clinic in the United States, showing correlation of AMH with karyotype and spontaneous menarche, as well as description of gonadotropins during minipuberty highlighting their clinical relevance. Studies in larger, prospective longitudinal cohorts will help determine their predictive value and role in the care of TS.


Subject(s)
Turner Syndrome , Child , Female , Humans , Anti-Mullerian Hormone , Cross-Sectional Studies , Gonadotropins , Prospective Studies , Puberty , Retrospective Studies , Infant , Child, Preschool , Adolescent , Young Adult
4.
Horm Res Paediatr ; 97(1): 62-69, 2024.
Article in English | MEDLINE | ID: mdl-37094554

ABSTRACT

INTRODUCTION: Turner syndrome (TS) is associated with primary ovarian insufficiency (POI) and most adolescents and young adults (AYA) with TS require treatment with hormone replacement therapy (HRT). International consensus guidelines are unclear on the optimal formulation and dosing for HRT after pubertal induction. This study assessed current HRT practice patterns of endocrinologists and gynecologists in North America. METHODS: Email listserv members of the North American Society for Pediatric and Adolescent Gynecology (NASPAG) and the Pediatric Endocrine Society (PES) were invited to complete a 19-question survey to assess HRT treatment preferences for the management of POI after completion of pubertal induction in AYA with TS. Descriptive analysis and multinomial logistic regression to predict factors associated with preferred HRT are presented. RESULTS: 155 providers (79% pediatric endocrinology, 17% pediatric gynecology) completed the survey. Although 87% (135) reported confidence in prescribing HRT, only half (51%, 79) were aware of published guidelines. Factors significantly associated with preferred HRT included specialty (p = 0.032) and number of patients with TS seen every 3 months (p = 0.024). Gynecologists were 4 times less likely than endocrinologists to prefer hormonal contraceptives and 4 times more likely to favor transdermal estradiol dose of 100 µg/day as compared to lower doses. CONCLUSION: Although most endocrinologists and gynecologists report confidence in prescribing HRT to AYA with TS after pubertal induction, there are clear differences in provider preferences based on specialty and higher volume of patients with TS in their practice. Additional studies on comparative effectiveness of the HRT regimens and evidence-based guidelines are necessary for AYA with TS.


Subject(s)
Turner Syndrome , Adolescent , Humans , Young Adult , Child , Turner Syndrome/drug therapy , Hormone Replacement Therapy , Estradiol/therapeutic use
5.
Pediatr Dermatol ; 41(1): 41-45, 2024.
Article in English | MEDLINE | ID: mdl-38057120

ABSTRACT

BACKGROUND/OBJECTIVES: Pediatric vulvar disease has not been widely explored in the medical literature. Few studies focus on vulvar disease in skin of color. The vulvar disease can be distressing for young patients given the sensitive location, and providers may lack experience in diagnosing and managing vulvar dermatoses. We sought to characterize the conditions seen, diagnostic challenges encountered, and the racial and ethnic factors associated with vulvar diseases in our multidisciplinary pediatric dermatology-gynecology vulvar clinic at Children's National. METHODS: Medical records of 220 patients who presented to our multidisciplinary pediatric dermatology-gynecology clinic were reviewed retrospectively. RESULTS: Lichen sclerosus (LS) (36%, n = 80), inflammatory vulvitis (11%, n = 23), and vitiligo (9%, n = 19) were the three most frequent conditions observed. These conditions were often misdiagnosed as one another. There was a mean delay in diagnosis after symptom onset in LS patients of 16.43 months. CONCLUSIONS: LS, inflammatory vulvitis, and vitiligo are common vulvar diseases among pediatric patients. Accurate diagnosis is important because LS must be treated aggressively to prevent sequelae. Further studies are warranted to help differentiate LS and vitiligo with consideration of skin tone.


Subject(s)
Dermatology , Lichen Sclerosus et Atrophicus , Vitiligo , Vulvar Diseases , Vulvar Lichen Sclerosus , Vulvitis , Female , Child , Humans , Retrospective Studies , Vitiligo/diagnosis , Lichen Sclerosus et Atrophicus/diagnosis , Vulvar Diseases/diagnosis , Vulvar Diseases/therapy , Vulvar Diseases/complications , Vulvitis/complications , Vulvar Lichen Sclerosus/complications
6.
Am J Med Genet A ; 194(2): 311-319, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37827984

ABSTRACT

Inspiring New Science to Guide Healthcare in Turner Syndrome (InsighTS) Registry is a national, multicenter registry for individuals with Turner syndrome (TS) designed to collect and store validated longitudinal clinical data from a diverse cohort of patients with TS. Herein, we describe the rationale, design, and approach used to develop the InsighTS registry, as well as the demographics of the initial participants to illustrate the registry's diversity and future utility. Multiple stakeholder groups have been involved from project conceptualization through dissemination, ensuring the registry serves the priorities of the TS community. Key features of InsighTS include recruitment strategies to facilitate enrollment of participants that appropriately reflect the population of individuals with TS receiving care in the US, clarity of data ownership and sharing, and sustainability of this resource. The registry gathers clinical data on diagnosis, treatment, comorbidities, health care utilization, clinical practices, and quality of life with the goal of improving health outcomes for this population. Future directions include multiple patient-centered clinical-translational research projects that will use the InsighTS platform. This thorough and thoughtful planning will ensure InsighTS is a valuable and sustainable resource for the TS community for decades to come.


Subject(s)
Turner Syndrome , Humans , Turner Syndrome/diagnosis , Turner Syndrome/epidemiology , Turner Syndrome/therapy , Quality of Life , Delivery of Health Care , Registries , Patient Acceptance of Health Care
7.
J Pediatr Adolesc Gynecol ; 36(5): 497-500, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37301425

ABSTRACT

BACKGROUND: Vulvodynia involves vulvar discomfort that occurs in the absence of an identifiable cause. Because vulvodynia is often accompanied by myofascial pain and pelvic floor tension, transvaginal botulinum toxin (BT) injection into the pelvic floor has been proposed as a possible treatment. METHODS: Retrospective case series RESULTS: Three adolescents with vulvodynia had a suboptimal response to treatment with several interventions, including neuromodulators (oral and topical), tricyclic antidepressants (oral and topical), and pelvic floor physical therapy. Subsequently, these patients underwent BT injections to the pelvic floor as treatment with varying responses. CONCLUSION: In select adolescent patients with vulvodynia, transvaginal BT injection into the pelvic floor can be an effective treatment. Further studies are needed to assess the optimal dosing, frequency, and sites of BT injections in the treatment of vulvodynia in pediatric and adolescent patients.


Subject(s)
Botulinum Toxins , Vulvodynia , Female , Adolescent , Humans , Child , Vulvodynia/drug therapy , Retrospective Studies , Treatment Outcome
8.
J Pediatr Adolesc Gynecol ; 35(6): 614-623, 2022 Dec.
Article in English | MEDLINE | ID: mdl-35830928

ABSTRACT

Reproductive tract bleeding is an underappreciated health care problem among adolescent and young adult (AYA) females with inherited bleeding disorders (IBDs) comprising von Willebrand disease, platelet disorders, hemophilia carriership, and rare factor deficiencies. IBDs are prevalent in women of all ages and have been detected in about 50% of women with menorrhagia or heavy menstrual bleeding (HMB) and about 20% of women with postpartum hemorrhage (PPH). The clinical spectrum of gynecologic and obstetric bleeding in AYA with IBDs ranges from HMB, ovulation bleeding, and surgical bleeding to miscarriages and life-threatening PPH. Reproductive tract bleeding adversely affects the quality of life of this patient population, in addition to causing substantial morbidity and mortality. Early diagnosis of IBDs offers the opportunity for timely intervention with hormones, hemostatic agents, and prophylaxis with factor concentrates, thereby improving outcomes. This review summarizes the epidemiology, pathophysiology, clinical manifestations, diagnostic approach, management, and prophylaxis for reproductive tract bleeding in AYA with IBDs. This review provides a multidisciplinary approach to the problem, which is critical to improve the outcomes of this patient population.


Subject(s)
Blood Platelet Disorders , Menorrhagia , Postpartum Hemorrhage , von Willebrand Diseases , Pregnancy , Female , Adolescent , Young Adult , Humans , Quality of Life , von Willebrand Diseases/complications , von Willebrand Diseases/diagnosis , von Willebrand Diseases/therapy , Menorrhagia/etiology , Blood Platelet Disorders/complications , Postpartum Hemorrhage/etiology
9.
Am J Obstet Gynecol ; 227(6): 862-870, 2022 12.
Article in English | MEDLINE | ID: mdl-35841934

ABSTRACT

Initially provided as an alternative to evaluation of serum analytes and nuchal translucency for the assessment of pregnancies at high risk of trisomy 21, cell-free DNA screening for fetal aneuploidy, also referred to as noninvasive prenatal screening, can now also screen for fetal sex chromosome anomalies such as monosomy X as early as 9 to 10 weeks of gestation. Early identification of Turner syndrome, a sex chromosome anomaly resulting from the complete or partial absence of the second X chromosome, allows medical interventions such as optimizing obstetrical outcomes, hormone replacement therapy, fertility preservation and support, and improved neurocognitive outcomes. However, cell-free DNA screening for sex chromosome anomalies and monosomy X in particular is associated with high false-positive rates and low positive predictive value. A cell-free DNA result positive for monosomy X may represent fetal Turner syndrome, maternal Turner syndrome, or confined placental mosaicism. A positive screen for monosomy X with discordant results of diagnostic fetal karyotype presents unique interpretation and management challenges because of potential implications for previously unrecognized maternal Turner syndrome. The current international consensus clinical practice guidelines for the care of individuals with Turner syndrome throughout the lifespan do not specifically address management of individuals with a cell-free DNA screen positive for monosomy X. This study aimed to provide context and expert-driven recommendations for maternal and/or fetal evaluation and management when cell-free DNA screening is positive for monosomy X. We highlight unique challenges of cell-free DNA screening that is incidentally positive for monosomy X, present recommendations for determining if the result is a true-positive, and discuss when diagnosis of Turner syndrome is applicable to the fetus vs the mother. Whereas we defer the subsequent management of confirmed Turner syndrome to the clinical practice guidelines, we highlight unique considerations for individuals initially identified through cell-free DNA screening.


Subject(s)
Cell-Free Nucleic Acids , Chromosome Disorders , Turner Syndrome , Female , Pregnancy , Humans , Turner Syndrome/diagnosis , Turner Syndrome/genetics , Turner Syndrome/therapy , Prenatal Diagnosis/methods , Placenta , Chromosome Disorders/diagnosis , Sex Chromosome Aberrations
10.
J Pediatr Adolesc Gynecol ; 35(5): 552-557, 2022 Oct.
Article in English | MEDLINE | ID: mdl-35472468

ABSTRACT

STUDY OBJECTIVE: To assess genital symptomatology, characterize the findings of genital examination, and describe the incidence and treatment of vulvovaginal graft-versus-host disease (vvGvHD) in girls and adolescents after allogeneic hematopoietic stem cell transplantation (HSCT). DESIGN: Retrospective cohort. SETTING: Metropolitan-area children's hospital. PARTICIPANTS: Female allogeneic HSCT recipients ages 0 to 22 years. MAIN OUTCOME MEASURES: Genital symptoms, genital examination, diagnosis, and treatment of vvGvHD. RESULTS: A total of 57 participants were included in the analysis. The median age at the time of HSCT was 10 years (range 4 months-23 years). Most (n = 40, 71%) underwent transplant for a nonmalignant condition, most commonly sickle cell anemia (n = 19, 33%). The median time of onset of GvHD post HSCT was 62 days (IQR = 42 to 151 days). The most common initial site of GvHD was skin (n = 21, 64%), followed by GI tract (n = 10, 30%). Three patients (5%) were diagnosed with vvGvHD. The time of onset of vvGvHD post HSCT ranged from 62 to 1565 days. One patient (33%) was asymptomatic at the time of diagnosis. There was no difference in diagnosis of vvGvHD when race (P = 0.15), age at allogeneic HSCT (P = 0.64), nonmalignant vs malignant indication (P = 0.21), source of stem cells (P = 0.25), partial vs full human leukocyte antigens (HLA) donor match (P = 0.34), and GvHD prophylaxis regimen (P = 0.18) were compared. None had isolated vvGvHD. Vulvovaginal GvHD was preceded by skin GvHD in 1 patient, was preceded by lung GvHD in 1 patient, and occurred concurrently with skin GvHD in the third patient. CONCLUSIONS: Pediatric vvGvHD can occur within the first 100 days after transplant and can be asymptomatic. Routine gynecologic evaluation post allogeneic HSCT in children and adolescents should include a thorough review of vulvovaginal symptoms and a gynecologic exam for the detection and treatment of vvGvHD.


Subject(s)
Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Adolescent , Adult , Child , Child, Preschool , Female , Graft vs Host Disease/etiology , Graft vs Host Disease/prevention & control , HLA Antigens , Hematopoietic Stem Cell Transplantation/adverse effects , Humans , Incidence , Infant , Infant, Newborn , Retrospective Studies , Young Adult
11.
J Pediatr Adolesc Gynecol ; 35(4): 429-434, 2022 Aug.
Article in English | MEDLINE | ID: mdl-35272055

ABSTRACT

Turner syndrome (TS) is caused by the absence of a part or whole X chromosome in a phenotypic female and has an estimated prevalence of 25-50/100,000 live-born females. The primary gynecologic manifestation of TS is primary ovarian insufficiency (POI), and the resulting hypoestrogenism and infertility are experienced by most individuals with TS. In this review, we summarize the recommendations for the care of adolescents and young adults with TS, with a focus on primary ovarian insufficiency in TS, hormone replacement therapy, fertility preservation, and pregnancy in TS.


Subject(s)
Fertility Preservation , Gynecology , Infertility , Primary Ovarian Insufficiency , Turner Syndrome , Adolescent , Child , Female , Fertility Preservation/methods , Humans , Pregnancy , Primary Ovarian Insufficiency/genetics , Turner Syndrome/complications , Turner Syndrome/genetics , Young Adult
13.
Contraception ; 110: 42-47, 2022 06.
Article in English | MEDLINE | ID: mdl-35085544

ABSTRACT

OBJECTIVE: The purpose of this study is to use an intersectional approach in which race, insurance, care setting, and disclosure of sexual orientation to a provider are used to assess patterns of contraceptive use in sexual minority women. STUDY DESIGN: This study analyzes cross-sectional data from the 2011-2019 National Survey of Family Growth (NSFG). Sexual orientation of 21,075 respondents' data was used to investigate contraceptive use in sexual minority women, specifically lesbian and bisexual women, as compared to heterosexual women, controlling for variables such as race, age, and socioeconomic factors. RESULTS: Black and Hispanic lesbian women (adjusted odds ratio [aOR] = 0.39 confidence interval [CI] 0.20-0.76 and aOR = 0.44 CI 0.23-0.82, respectively) and Hispanic and Other Race bisexual women use hormonal contraceptive methods less than their White lesbian and bisexual peers (aOR = 0.45 CI 0.29-0.69 and aOR = 0.43 CI 0.20-0.94). Care setting was not correlated with long-acting reversible contraceptive methods (LARC; such as intra-uterine device, hormonal implants) or prescription-based hormonal methods (such as oral contraceptive pills, injectables, vaginal rings, and patches) in lesbian women (aOR = 2.92 CI 0.60-14.2 and aOR = 1.43 CI 0.47-4.38, respectively) or bisexual women (aOR = 0.90 CI 0.48-1.58 and aOR = 0.83 CI 0.37-1.86), but it was for straight women (aOR = 1.28 CI 1.03-1.59 and aOR = 0.68 CI 0.53-0.86). Similarly, insurance status did not correlate with contraceptive patterns in sexual minority women. Importantly, adjusting for nationally representative data did not impact the results; in other words, the odds ratios after adjusting yielded the same results as before adjustment. CONCLUSIONS: Insurance and care setting are important determinants of straight women's contraceptive use patterns with fewer effects seen among sexual minority women. These findings support previous work and indicate that known advantages of insurance coverage or use of public clinics may not positively impact sexual minority women as much as they do straight women. Provider awareness of sexual identity and sexual orientation is important for adequate contraceptive care. IMPLICATIONS: While prior research has presented findings on sexual minority women contraceptive use, to our knowledge there are limited studies that address the social and demographic implications for contraceptive use in this population.


Subject(s)
Homosexuality, Female , Sexual and Gender Minorities , Contraceptive Agents , Cross-Sectional Studies , Ethnicity , Female , Humans , Male , Sexual Behavior
14.
J Clin Endocrinol Metab ; 107(2): 309-323, 2022 01 18.
Article in English | MEDLINE | ID: mdl-34537849

ABSTRACT

CONTEXT: Anti-Mullerian hormone (AMH) was originally described in the context of sexual differentiation in the male fetus but has gained prominence now as a marker of ovarian reserve and fertility in females. In this mini-review, we offer an updated synopsis on AMH and its clinical utility in pediatric patients. DESIGN AND RESULTS: A systematic search was undertaken for studies related to the physiology of AMH, normative data, and clinical role in pediatrics. In males, AMH, secreted by Sertoli cells, is found at high levels prenatally and throughout childhood and declines with progression through puberty to overlap with levels in females. Thus, serum AMH has clinical utility as a marker of testicular tissue in males with differences in sexual development and cryptorchidism and in the evaluation of persistent Mullerian duct syndrome. In females, serum AMH has been used as a predictive marker of ovarian reserve and fertility, but prepubertal and adolescent AMH assessments need to be interpreted cautiously. AMH is also a marker of tumor burden, progression, and recurrence in germ cell tumors of the ovary. CONCLUSIONS: AMH has widespread clinical diagnostic utility in pediatrics but interpretation is often challenging and should be undertaken in the context of not only age and sex but also developmental and pubertal stage of the child. Nonstandardized assays necessitate the need for assay-specific normative data. The recognition of the role of AMH beyond gonadal development and maturation may usher in novel diagnostic and therapeutic applications that would further expand its utility in pediatric care.


Subject(s)
Anti-Mullerian Hormone/blood , Cryptorchidism/diagnosis , Disorder of Sex Development, 46,XY/diagnosis , Ovarian Reserve , Anti-Mullerian Hormone/metabolism , Child , Child Development , Cryptorchidism/blood , Disorder of Sex Development, 46,XY/blood , Female , Gonads/growth & development , Humans , Male , Sexual Maturation
15.
J Pediatr Adolesc Gynecol ; 34(5): 745-748, 2021 Oct.
Article in English | MEDLINE | ID: mdl-33915265

ABSTRACT

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening spectra of mucocutaneous delayed hypersensitivity reactions. Prodromal viral-like symptoms are followed by a characteristic diffuse rash caused by keratinocyte apoptosis and epidermal detachment. CASES: Three adolescents were admitted with SJS/TEN and vulvovaginal involvement following initiation of lamictal, bactrim, and phenobarbital. The patients received intravenous immunoglobulin and intravenous steroids. One patient received etanercept. Topical emollients and strict perineal hygiene were initiated. No permanent sequelae were noted following vaginoscopy. SUMMARY AND CONCLUSIONS: Vulvovaginal involvement in SJS/TEN can occur and may result in permanent architectural changes. Basic management includes withdrawal of causative medication, intravenous steroids, intravenous immunoglobulin (IVIG), and supportive care. Early initiation of perineal hygiene, vaginal barrier creams, and menstrual suppression should be employed. Vaginoscopy may be used to document full recovery.


Subject(s)
Stevens-Johnson Syndrome , Adolescent , Child , Female , Humans , Immunoglobulins, Intravenous , Retrospective Studies , Stevens-Johnson Syndrome/diagnosis , Stevens-Johnson Syndrome/drug therapy , Stevens-Johnson Syndrome/etiology
16.
J Pediatr Adolesc Gynecol ; 32(5): 555-557, 2019 Oct.
Article in English | MEDLINE | ID: mdl-31279777

ABSTRACT

BACKGROUND: Turner syndrome is a genetic disorder resulting from the absence of or structural abnormality of one X chromosome. The presence of Y chromosome material in girls with Turner syndrome confers an increased risk of benign and malignant germ cell tumor and prophylactic bilateral gonadectomy is recommended. CASE: A 10-year-old Turner mosaic syndrome (45X/46XY) patient underwent prophylactic gonadectomy after unremarkable preoperative pelvic imaging. Histopathology showed a streak right gonad, and left gonad with gonadoblastoma with limited degree of infiltrating germinoma. SUMMARYAND CONCLUSION: Gonadoblastoma and dysgerminoma have been reported in girls with Turner mosaic who carry Y chromosome material. Prophylactic gonadectomy should be considered in these girls without delay.


Subject(s)
Dysgerminoma/genetics , Ovarian Neoplasms/genetics , Turner Syndrome/complications , Castration , Child , Dysgerminoma/surgery , Female , Gonadoblastoma/genetics , Gonadoblastoma/surgery , Humans , Ovarian Neoplasms/surgery
17.
J Pediatr Adolesc Gynecol ; 32(4): 429-431, 2019 Aug.
Article in English | MEDLINE | ID: mdl-31029601

ABSTRACT

BACKGROUND: Untreated symptomatic imperforate hymens at birth can result in renal complications and ascending infection. Although guidelines recommend incision and drainage, little is discussed regarding postoperative management and surveillance. CASE: A 2-day-old infant with symptomatic imperforate hymen (hydrometrocolpos and hydronephrosis) underwent incision and drainage using sterile technique. On postoperative day 19 she developed hymen reclosure, fluid reaccumulation, and concern for sepsis. After stabilization, redrainage was performed in the operating room with interrupted suture placement around an annular incision. She rapidly improved. Serial postoperative follow-up was performed to ensure ongoing patency of the hymen during healing. SUMMARY AND CONCLUSION: Neonatal hymenotomies can have postoperative complications. We recommend consideration of annular suture placement and close follow-up, because of risk for reclosure and rapid deterioration from infection in this age range.


Subject(s)
Colpotomy/methods , Hymen/abnormalities , Menstruation Disturbances/surgery , Postoperative Complications/prevention & control , Congenital Abnormalities , Female , Humans , Hymen/surgery , Infant, Newborn , Postoperative Care/methods , Pregnancy , Suture Techniques
18.
J Pediatr Adolesc Gynecol ; 32(3): 259-263, 2019 Jun.
Article in English | MEDLINE | ID: mdl-30385397

ABSTRACT

STUDY OBJECTIVE: Although various treatment options have been proposed for the treatment of labial adhesions, there are currently no clearly outlined limits on the duration of topical therapy, amount of lateral traction to apply, and methods to decrease the recurrence. This clinical trial was undertaken to assess the need for estrogen for treatment of prepubertal labial adhesions. DESIGN: Randomized, double-blinded, controlled trial. SETTING: Pediatric and Adolescent Gynecology Clinic at a children's hospital in a metropolitan area. PARTICIPANTS: Prepubertal girls ages 3 months to 12 years with labial adhesions. INTERVENTIONS: Lateral traction with topical estrogen or topical emollient. MAIN OUTCOME MEASURES: The primary outcome was resolution of labial adhesions. The secondary outcome was the change in severity of labial adhesions over time between the 2 groups. RESULTS: Forty-three girls were enrolled and 38 (88%) completed the study. The difference in complete resolution between the topical emollient group (19%) and the topical estrogen group (36%) was not statistically significant (P = .21). There was a statistically significant decrease in severity of labial adhesions over time, with the magnitude of improvement favoring the topical estrogen group. CONCLUSION: Although labial adhesion severity decreased when treated with lateral traction and topical emollient or topical estrogen, the magnitude of the effect was significantly greater for topical estrogen.


Subject(s)
Estrogens/therapeutic use , Tissue Adhesions/drug therapy , Vulvar Diseases/drug therapy , Administration, Topical , Child , Child, Preschool , Emollients/therapeutic use , Female , Humans , Infant , Recurrence , Severity of Illness Index , Treatment Outcome
19.
J Pediatr Adolesc Gynecol ; 31(1): 19-22, 2018 Feb.
Article in English | MEDLINE | ID: mdl-28782658

ABSTRACT

STUDY OBJECTIVE: To evaluate the characteristics of girls with accidental genital trauma (AGT) who can be managed in the emergency department (ED) vs the operating suite (OS). DESIGN: Retrospective cohort. SETTING: ED at a children's hospital in a metropolitan area. PARTICIPANTS: Girls aged 0-18 years with AGT. INTERVENTIONS AND MAIN OUTCOME MEASURES: Factors associated with need for evaluation and repair of AGT in the OS. RESULTS: A total of 359 girls were included in the analysis. The mean age was 6 ± 3 years. Most girls presented with pain and bleeding, 321/359 (89%). Straddle injury was the most common mechanism, 258/355 (73%). The most commonly injured site was the labia, 225/358 (63%) and the most common type of injury was laceration, 308/357 (86%). Factors significantly associated with treatment in the OS included older age, transfer from another institution, penetrating injuries, injuries involving the hymen/vagina/urethra/anus, and injuries larger than 3 cm in size. The odds of requiring general anesthesia in the OS were 5.5 times higher for injuries larger than 3 cm (95% confidence interval, 2.8-10.9; P < .0001) and 4.1 times greater if the patient was transferred from another facility (95% confidence interval, 1.3-13.3; P < .02). CONCLUSION: Most AGT can be managed expectantly. Penetrating injuries, injuries to the hymen/vagina/urethra/anus, and injuries with a maximal size of 3 cm should be considered as indications for management in the OS. With adequate procedural sedation, most girls with minor injuries as a result of AGT can undergo a thorough examination and repair of AGT in the ED.


Subject(s)
Emergency Service, Hospital/statistics & numerical data , Genitalia/injuries , Operating Rooms/statistics & numerical data , Wounds and Injuries/epidemiology , Adolescent , Child , Child, Preschool , Cohort Studies , Female , Hospitals, Pediatric , Humans , Infant , Retrospective Studies
20.
Curr Opin Obstet Gynecol ; 29(5): 306-309, 2017 Oct.
Article in English | MEDLINE | ID: mdl-28777193

ABSTRACT

PURPOSE OF REVIEW: The current article addresses recent literature regarding the diagnosis and management of endometriosis in adolescents. RECENT FINDINGS: An increasing body of literature suggests that advanced-stage endometriosis (revised scoring system of the American Society for Reproductive Medicine Stage III or IV) and deeply invasive endometriosis are relatively common in adolescents. There remains limited data on the efficacy of postoperative hormonal management of endometriosis in the adolescent population. SUMMARY: Strong consideration should be made for surgical diagnosis of endometriosis in adolescents with pelvic pain, including noncyclic pain, with a concurrent family history of endometriosis and personal history of atopic disease. More research is needed regarding the benefits of the routine use of hypoestrogenic and other hormonal agents in the prevention of disease progression and long-term sequela in adolescents with endometriosis.


Subject(s)
Endometriosis/diagnosis , Endometriosis/therapy , Adolescent , Contraceptives, Oral/therapeutic use , Endometriosis/complications , Endometriosis/pathology , Female , Gonadotropin-Releasing Hormone/agonists , Humans , Laparoscopy , Menstruation Disturbances/etiology , Pelvic Pain/etiology
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