Surgical Management of OHVIRA and Outcomes.

STUDY OBJECTIVE: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) includes uterine didelphys, unilateral obstructed hemivagina, and ipsilateral renal anomaly. Surgical management of this condition relies on accurate diagnosis to excise the obstructed longitudinal vaginal septum (OLVS). Vital considerations involve identifying the side affected, ipsilateral renal anomaly (IRA), thickness of the septum (TS), septal axis (SA), and distance of the septum to perineum (DSP). The study aimed to evaluate the preoperative characteristics, imaging findings, and surgical outcomes of OHVIRA. METHODS: Institutional review board approval was obtained for this retrospective chart review. ICD-10 codes identified OHVIRA cases between 2012 and 2019 at a single children's hospital. Patient demographic characteristics, magnetic resonance imaging findings, surgical management, outcomes, and complications were reviewed. Descriptive statistics were utilized. RESULTS: Twenty-six patients met inclusion criteria. Most were diagnosed at puberty (92%). Abdominal pain (50%) was the most common presenting complaint. The mean age of diagnosis was 13.2 years overall and 11.2 years for those with regular cycles vs 13.4 years for those with irregular cycles. Preoperative imaging showed predominant right-sided OLVS (50%), IRA (77%), and oblique SA (65%). All patients underwent vaginoscopy, septum resection, and vaginoplasty, except 1 who was managed with an abdominal drain as a neonate. Four required postoperative vaginal stent or Foley, with DSP greater than 5 cm in all cases. One intraabdominal abscess complication occurred. No instances of hematocolpos re-accumulation or reoperation were observed during the 3-year follow-up period. CONCLUSION: This study demonstrates that detailed preoperative planning and a systematic surgical approach lead to favorable outcomes in OHVIRA irrespective of the OLVS laterality, TS, SA, or DSP.

Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series.

INTRODUCTION: There are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly. STUDY OBJECTIVE: To describe another variant of UD and compare the presentation and management across different institutions METHODS: This was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used. RESULTS: Five patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain. CONCLUSION: Diagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.

Critical elements in the operative management of pediatric malignant ovarian germ cell tumors.

Performance of the appropriate operation is highly important to ensure that any patient with a suspected ovarian germ cell tumor receives optimal therapy that prioritizes cure while simultaneoulsy minimizing risk of short and long-term toxicities of treatment. The following critical elements of any operative procedure performed for a suspected pediatric or adolescent ovarian germ cell tumor are reviewed: 1. Complete resection of the tumor via ipsilateral oophorectomy while avoiding tumor rupture and spillage, and 2. Performance of complete intraperitoneal staging at the time of initial tumor resection.

Laparoscopic salvage procedures for adnexal torsion in pediatric and adolescent patients during the COVID-19 pandemic: a retrospective cohort study.

BACKGROUND: Early management for adnexal torsion increases likelihood of ovarian/tubal salvage. The Coronavirus disease of 2019 (COVID-19) pandemic poses delays from symptom-onset to intervention. The primary objective was to evaluate rates of ovarian salvage and tubal salvage following ovarian torsion and adnexal torsion during the COVID-19 pandemic in a pediatric and adolescent gynecology population. METHODS: This was a retrospective quality improvement cohort study of pediatric and adolescent gynecology patients at a single children's hospital who underwent laparoscopy for suspected ovarian torsion/adnexal torsion between March 2020 to March 2021. Descriptive statistics and t-tests were utilized. RESULTS: There were 50 suspected adnexal cases in 47 patients. All underwent laparoscopy, revealing 39 adnexal torsion occurrences in 36 patients and 1 patient with recurrent adnexal torsion three times. All underwent pre-operative COVID-19 testing. Mean age was 13.9 ± 2.6 years for adnexal torsion cohort. Menarche was achieved in 88% (n = 44) and 12% (n = 6) were pre-menarchal. The primary outcome was ovarian salvage and tubal salvage rates, which were 97.4% (n = 38) and 89.7% (n = 35), respectively. Secondary outcomes assessed factors contributing to the primary outcome or operative delays. The mean age of menarche was 11.2 years (salvaged) and 12.5 years (non-salvaged) (p = 0.04). There were no differences in mean pain duration or mean COVID-19 testing time between groups. Left, right and bilateral adnexal torsion occurred in 42% (n = 21), 32% (n = 16), and 4% (n = 2) respectively. The most common pathologies were paratubal cyst (n = 17, 34%) and benign ovarian cyst (n = 16, 32%). CONCLUSIONS: Ovarian salvage and tubal salvage rates were 97.4% and 89.7%, respectively during the time frame studied. These salvage rates during the study period are comparable to previous rates in a pre-COVID cohort at our institution. Institutional and departmental quality and safety initiatives likely contributed to this outcome.

Rare variant enrichment analysis supports GREB1L as a contributory driver gene in the etiology of Mayer-Rokitansky-Küster-Hauser syndrome.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by aplasia of the female reproductive tract; the syndrome can include renal anomalies, absence or dysgenesis, and skeletal anomalies. While functional models have elucidated several candidate genes, only WNT4 (MIM: 603490) variants have been definitively associated with a subtype of MRKH with hyperandrogenism (MIM: 158330). DNA from 148 clinically diagnosed MRKH probands across 144 unrelated families and available family members from North America, Europe, and South America were exome sequenced (ES) and by family-based genomics analyzed for rare likely deleterious variants. A replication cohort consisting of 442 Han Chinese individuals with MRKH was used to further reproduce GREB1L findings in diverse genetic backgrounds. Proband and OMIM phenotypes annotated using the Human Phenotype Ontology were analyzed to quantitatively delineate the phenotypic spectrum associated with GREB1L variant alleles found in our MRKH cohort and those previously published. This study reports 18 novel GREB1L variant alleles, 16 within a multiethnic MRKH cohort and two within a congenital scoliosis cohort. Cohort-wide analyses for a burden of rare variants within a single gene identified likely damaging variants in GREB1L (MIM: 617782), a known disease gene for renal hypoplasia and uterine abnormalities (MIM: 617805), in 16 of 590 MRKH probands. GREB1L variant alleles, including a CNV null allele, were found in 8 MRKH type 1 probands and 8 MRKH type II probands. This study used quantitative phenotypic analyses in a worldwide multiethnic cohort to identify and strengthen the association of GREB1L to isolated uterine agenesis (MRKH type I) and syndromic MRKH type II.

The Effect of the Rate of Increase of Estrogen Replacement Therapy on Bone Mineral Density Accrual in Young Patients with Turner Syndrome.

BACKGROUND: Turner syndrome (TS) is caused by partial/complete X-chromosome monosomy with variable phenotypes, characterized by hypogonadism and short stature. To achieve pubertal changes, up to 50%-79% of patients with TS require estrogen replacement therapy (ERT), and 80% have low bone mineral density (BMD). Studies show that pubertal delays are associated with decreased BMD. Currently, guidelines suggest that ERT start at 12 years, increasing slowly, simulating pubertal progression. Many studies show that ERT increases BMD in adolescents with TS, but uncertainty remains as to how the rate of increase in ERT affects BMD. METHODS: Institutional review board approval was obtained from our institution for this retrospective chart review from 1991 to 2020. Charts were requested for the database using ICD 9-10 codes for TS and patients undergoing dual-energy X-ray absorptiometry. Biometric data and medical and treatment histories were extracted from charts. Multilevel random effects models were constructed to assess the time-dependent associations between ERT and bone density parameters. The primary independent variable of interest was the rate at which patients went from initiating ERT to reaching final doses. The primary dependent variables measured were total body BMD (tbBMD) and corresponding z-scores, calculated using dual-energy X-ray absorptiometry techniques. Analyses were done with SAS software (version 9.4, Cary, NC). RESULTS: Twenty-eight patients met the inclusion criteria. The mean age at TS diagnosis was 6.9 years; 8 patients had monosomy X, 16 had mosaic karyotypes, and 4 had unknown karyotypes. The average age for starting hormone replacement therapy was 14.1 years. Thirteen patients had spontaneous pubertal onset before starting hormone replacement therapy. tbBMD increased significantly with age (P = .03). However, change in BMD by age did not vary between patients who reached final adult doses of ERT within 0-2.5 years compared with patients who took 2.5-5.5 years (P = .7). Patients who took 2.5-5.5 years to reach final adult doses of ERT had a more negative trend in z-scores (-2.144) in comparison with patients who took 0-2.5 years (-1.776), although this difference did not reach statistical significance (P = .15). Future larger studies are needed to better understand the relationship between duration of ERT use and tbBMD. CONCLUSION: BMD in adolescents with TS increases with age. Neither absolute tbBMD values nor tbBMD z-scores increased faster when ERT doses were maximized within 2.5 years. This study identified a cohort of children under 12 years with TS who had not had any ERT or BMD measurements, a potential population for future larger prospective studies.

NASPAG Pediatric and Adolescent Gynecology Surgery Compensation Survey.

INTRODUCTION: Over the last decade, the field of pediatric and adolescent gynecology (PAG) has rapidly expanded on the basis of a need for this specialized training to care for children and adolescents with gynecologic concerns. There are 18 PAG fellowship programs within the United States and Canada. Since 2017, which marked the beginning of the American Board of Obstetrics and Gynecology (ABOG) Focused Practice Examination in PAG and PAG Maintenance of Certification program, there has been a growing recognition of this specialty, given the unique population served. However, there is a paucity of information related to compensation in PAG. As the field has grown and more PAG fellow graduates are sought by children's hospitals throughout the United States and Canada, there is an urgent need to ensure that salary is equitable for these specialty PAG providers given the unique skills acquired during fellowship training, beyond that of obstetrics and gynecology (OBGYN) residency. This initial survey aimed to focus on compensation for PAG surgeons in the United States. OBJECTIVES: To survey NASPAG PAG surgeons in the United States about current benefits, practice metrics, and compensation METHODS: A 15-question anonymous survey was sent to PAG surgeons in the United States in February 2022, with 3 reminders sent by email to engage voluntary participation. The survey aimed to understand practice characteristics, current compensation, and relative value unit (RVU) benchmarks. Descriptive statistics were utilized. Compensation means and quartiles were calculated in U.S. dollars for the following categories: assistant professor, associate professor, and full professor. The compensation mean was also calculated for private/hybrid and instructor categories. RESULTS: Among 255 eligible members, 88 completed the survey, for a participation rate of 34.5%. Sixty-three point six percent reported having completed a PAG fellowship, whereas one-third reported specializing in this area with no fellowship available at the time. Three-fourths reported having achieved ABOG Focused Practice PAG certification. Most providers were academic (75%) and working full time (82.9%). Among academicians, most were assistant professors (48%) and on the non-tenure track (50.6%). RVU benchmarks varied, with 40.2% reporting OBGYN generalist targets and only 18.3% reporting PAG-specific targets, despite most physicians practicing only PAG (62.5%) and less than 30% practicing PAG plus some adult OBGYN. Salary support varied, with 57% employed by a hospital. Incentives were common, with most receiving a bonus at the end of the year (52.9%). As expected, full professors reported higher mean compensation ($345k) as compared with less senior colleagues ($248k and $302k for assistant and associate professors, respectively). Private practice/hybrid practitioners reported compensation ($251k) similar to that of assistant professors ($248k). Only 2 instructor-level physicians completed the survey. The mean number for this level is skewed and is not a reliable predictor for this academic level. Quartiles could not be calculated for this category. CONCLUSION: This is the first survey addressing compensation in the field of PAG in the United States. There is an ongoing need to collect this information to prepare PAG fellow graduates for the job market. Additional surveys, including an understanding of the compensation landscape in Canada, are needed in the future to address specific questions related to compensation for those who have less than 50% clinical time.

Imperforate Hymen: Clinical Pearls and Implications of Management.

Imperforate hymen is a common condition that with classic appearance and presentation in puberty. This may be diagnosed in a neonate when mucocolpos is noted. There are many great pretenders of this condition, but the hallmarks include a bulging hymen with blue hue at puberty. If this is not seen on external genital exam, the provider should proceed with magnetic resonance imaging to better assess the reproductive tract anatomy before engaging in surgery. For providers uncomfortable in managing conditions often confused with an imperforate hymen, referral to a Pediatric and Adolescent Gynecologist or specialist comfortable managing these conditions, is recommended.

Functional characteristics of a broad spectrum of TBX6 variants in Mayer-Rokitansky-Küster-Hauser syndrome.

PURPOSE: Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is characterized by congenital absence of the uterus, cervix, and the upper part of the vagina in females. Whole-gene deletion and loss-of-function variants in TBX6 have been identified in association with MRKHS. We aimed to expand the spectrum of TBX6 variants in MRKHS and explore the biological effect of the variant alleles. METHODS: Rare variants in TBX6 were called from a combined multiethnic cohort of 622 probands with MRKHS who underwent exome sequencing or genome sequencing. Multiple in vitro functional experiments were performed, including messenger RNA analysis, western blotting, transcriptional activity assay, and immunofluorescence staining. RESULTS: We identified 16 rare variants in TBX6 from the combined cohort, including 1 protein-truncating variant reported in our previous study and 15 variants with unknown effects. By comparing the prevalence of TBX6 variants in the Chinese MRKHS cohort vs 1038 female controls, we observed a significant mutational burden of TBX6 in affected individuals (P = .0004, odds ratio = 5.25), suggesting a causal role of TBX6 variants in MRKHS. Of the 15 variants with uncertain effects, 7 were shown to induce a loss-of-function effect through various mechanisms. The c.423G>A (p.Leu141=) and c.839+5G>A variants impaired the normal splicing of TBX6 messenger RNA, c.422T>C (p.Leu141Pro) and c.745G>A (p.Val249Met) led to decreased protein expression, c.10C>T (p.Pro4Ser) and c.400G>A (p.Glu134Lys) resulted in perturbed transcriptional activity, and c.356G>A (p.Arg119His) caused protein mislocalization. We observed incomplete penetrance and variable expressivity in families carrying deleterious variants, which indicates a more complex genetic mechanism than classical Mendelian inheritance. CONCLUSION: Our study expands the mutational spectrum of TBX6 in MRKHS and delineates the molecular pathogenesis of TBX6 variants, supporting the association between deleterious variants in TBX6 and MRKHS.

Diagnosis and Management of Mullerian Anomalies Across Differing Resource Settings: Worldwide Adaptations.

BACKGROUND: Mullerian anomalies affect 7% of reproductive age women. It is important to have a basic understanding of these conditions, given they can affect women at any stage of life and have potential impacts on fertility and pregnancy. This article seeks to review these anomalies as well as specific diagnostic pitfalls and strategies to approach these conditions in both high- and low-resource environments. METHODS: This review was undertaken with a PubMed focused search, using terms related to the diagnosis and management of Mullerian anomalies in many worldwide settings. Consideration was made to assess the medical resources available in low- and middle-income countries (LMICs), which could impact diagnostic and management decisions, compared with high-income countries (HICs). Concurrent medical conditions and both gynecologic and obstetric outcomes were also searched. Practice recommendations from international societies were also reviewed and compared. Finally, 4 conditions were evaluated more closely to assess management differences based on geographic locations and whether the countries were LMICs or HICs; specifically, those evaluated were lower vaginal atresia, uterovaginal agenesis, bicornuate uterus, and septate uterus. DISCUSSION: Mullerian anomalies encompass a wide variety of conditions, ranging from subtle anatomic changes without concurrent anomalies to complex conditions, associated with anomalies of the kidney or spine, which could impact the ability to manage certain conditions based on medical resources available geographically. A systematic approach and provider expertise is important for appropriate diagnosis and management, independent of geographic location. Counseling is critical for medical and surgical decision-making and might be limited or guided by the resources available in certain settings or even by existing laws. CONCLUSIONS: Limited outcomes are available among patients with Mullerian anomalies in LMICs; however, the management varies based on the basic tools available to address acute needs, as well as long-term fertility and obstetric concerns. More research is needed in this population, which could help drive the importance of early diagnosis and management not only in HICs but also in LMICs, where individualization strategies are key.

Review of Surgical Neovagina Techniques and Management of Vaginal Stricture.

STUDY OBJECTIVE: To review the currently available methods for surgical and nonsurgical neovaginal creation with an additional focus on situations requiring vaginal stretching, replacement, or augmentation and the management of vaginal stenosis or stricture. DESIGN: Review of the existing literature METHODS: PUBMED search with key words vaginal agenesis, neovaginal creation, vaginoplasty, vaginal stretching, vaginal augmentation, vaginal stenosis, outcomes of vaginoplasty, Mullerian anomalies, vaginal anomalies RESULTS: The need to perform vaginal stretching, replacement, or augmentation will vary depending on the underlying reproductive tract condition. CONCLUSIONS: A variety of techniques have been described to performed vaginal stretching, replacement, or augmentation. There are risks and benefits to each technique. Vaginal stricture and stenosis remain difficult problems given the high rates of these complications in patients undergoing these procedures. Although several techniques can alleviate this problem, they rely on the thickness of the stenosis and the location of the stenosis within the vagina.

Prevalence of Polycystic Ovary Syndrome in Patients With McCune Albright Syndrome.

STUDY OBJECTIVE: To identify polycystic ovarian syndrome (PCOS) in a population of female patients with McCune-Albright syndrome (MAS) by retrospective chart review. DESIGN: Retrospective study. SETTING: Academic setting. PARTICIPANTS: All female patients with a prior diagnosis of MAS who were more than 12 years of age at the time of chart review. Only complete medical records from January 2009 to January 2020 were included in the analysis. INTERVENTIONS: None. MAIN OUTCOME MEASURE(S): Diagnosis of PCOS based on the Rotterdam 2003 criteria. RESULTS: Seventeen female patients with MAS were included in the analysis. PCOS appeared to be more prevalent in MAS patients than expected based on population estimates (exact binomial test = 0.353; CI = 0.142-0.617, P = .01). The average body mass index was not significantly different between MAS patients with and without PCOS (23.38 kg/m2 vs 23.44 kg/m2, 2-sample Wilcoxon rank-sum test with continuity correction, W = 29, P = 0.733). The majority of patients (71%) were treated with an aromatase inhibitor and/or a gonadotropin-releasing hormone (GnRH) agonist. CONCLUSIONS: The results of this study suggest that female individuals with MAS have a statistically higher prevalence of PCOS. These findings warrant further studies to determine whether the increased risk of PCOS may be associated with precocious puberty, treatment of precocious puberty, or other factors.

Whole-exome analysis of adolescents with low VWF and heavy menstrual bleeding identifies novel genetic associations.

Adolescents with low von Willebrand factor (VWF) levels and heavy menstrual bleeding (HMB) experience significant morbidity. There is a need to better characterize these patients genetically and improve our understanding of the pathophysiology of bleeding. We performed whole-exome sequencing on 86 postmenarchal patients diagnosed with low VWF levels (30-50 IU/dL) and HMB and compared them with 660 in-house controls. We compared the number of rare stop-gain/stop-loss and rare ClinVar "pathogenic" variants between cases and controls, as well as performed gene burden and gene-set burden analyses. We found an enrichment in cases of rare stop-gain/stop-loss variants in genes involved in bleeding disorders and an enrichment of rare ClinVar "pathogenic" variants in genes involved in anemias. The 2 most significant genes in the gene burden analysis, CFB and DNASE2, are associated with atypical hemolytic uremia and severe anemia, respectively. VWF also surpassed exome-wide significance in the gene burden analysis (P = 7.31 × 10-6). Gene-set burden analysis revealed an enrichment of rare nonsynonymous variants in cases in several hematologically relevant pathways. Further, common variants in FERMT2, a gene involved in the regulation of hemostasis and angiogenesis, surpassed genome-wide significance. We demonstrate that adolescents with HMB and low VWF have an excess of rare nonsynonymous and pathogenic variants in genes involved in bleeding disorders and anemia. Variants of variable penetrance in these genes may contribute to the spectrum of phenotypes observed in patients with HMB and could partially explain the bleeding phenotype. By identifying patients with HMB who possess these variants, we may be able to improve risk stratification and patient outcomes.

Surgical Approach to Pediatric Ovarian Growing Teratoma Syndrome: A Case Report.

BACKGROUND: Growing teratoma syndrome is defined as conversion of a metastatic immature tumor to a mature tumor after adjuvant chemotherapy and remains an area of investigation because of its unclear pathogenesis. Because of its risk of malignant transformation, the primary treatment strategy for pediatric patients is surgical resection. CASE: In this report we present a case of a pediatric patient with recurrent growing teratoma syndrome who was treated with chemotherapy, debulking procedures, and cryoablation for the growing nodules throughout her abdominal cavity. The patient has had a good clinical outcome without recurrent malignant tumor. SUMMARY AND CONCLUSION: These masses do not always regress with chemotherapy and complete surgical excision or ablation should be attempted when possible.

Perturbations of genes essential for Müllerian duct and Wölffian duct development in Mayer-Rokitansky-Küster-Hauser syndrome.

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is associated with congenital absence of the uterus, cervix, and the upper part of the vagina; it is a sex-limited trait. Disrupted development of the Müllerian ducts (MD)/Wölffian ducts (WD) through multifactorial mechanisms has been proposed to underlie MRKHS. In this study, exome sequencing (ES) was performed on a Chinese discovery cohort (442 affected subjects and 941 female control subjects) and a replication MRKHS cohort (150 affected subjects of mixed ethnicity from North America, South America, and Europe). Phenotypic follow-up of the female reproductive system was performed on an additional cohort of PAX8-associated congenital hypothyroidism (CH) (n = 5, Chinese). By analyzing 19 candidate genes essential for MD/WD development, we identified 12 likely gene-disrupting (LGD) variants in 7 genes: PAX8 (n = 4), BMP4 (n = 2), BMP7 (n = 2), TBX6 (n = 1), HOXA10 (n = 1), EMX2 (n = 1), and WNT9B (n = 1), while LGD variants in these genes were not detected in control samples (p = 1.27E-06). Interestingly, a sex-limited penetrance with paternal inheritance was observed in multiple families. One additional PAX8 LGD variant from the replication cohort and two missense variants from both cohorts were revealed to cause loss-of-function of the protein. From the PAX8-associated CH cohort, we identified one individual presenting a syndromic condition characterized by CH and MRKHS (CH-MRKHS). Our study demonstrates the comprehensive utilization of knowledge from developmental biology toward elucidating genetic perturbations, i.e., rare pathogenic alleles involving the same loci, contributing to human birth defects.

The Utility of Blind Pap Tests in Young Adults with Developmental Delay: A Pilot Study.

STUDY OBJECTIVE: There are no guidelines specific to women with developmental delay who may not be able to tolerate a Papanicolaou (Pap) test in the office setting. The purpose of this study was to assess the utility of a less invasive method, a blind Pap for women with developmental delay, compared to the traditional Pap test performed in pediatric and adolescent gynecology (PAG) patients. DESIGN: Retrospective cohort pilot study. SETTING: Outpatient PAG clinics and the inpatient PAG service at a single institution. PARTICIPANTS: PAG patients who underwent a traditional Pap test and developmentally delayed PAG patients who underwent a blind Pap test. Patients were 26 years of age or less between July 2007 and March 2019. INTERVENTIONS AND MAIN OUTCOME MEASURES: Charts were reviewed to identify those who underwent a traditional Pap test (with a speculum and direct visualization) versus a blind Pap test (with a vaginal swab without a speculum). Descriptive statistics and Wilcoxon rank and Fisher exact tests compared specimen adequacy, presence of the endocervical/transformation zone (EC/TZ), and cytology results. RESULTS: Of 328 PAG patients identified, 314 patients had a traditional Pap test (control), and 14 patients had a blind Pap test (4.3%). The majority of Pap tests were satisfactory in both groups. The EC/TZ component was present in 279 (90%) specimens within the traditional Pap test group and 8 (57%) in the blind Pap test group (P = .002). The traditional Pap test group results varied, with most (81.9%) being negative for intraepithelial lesion or malignancy. All BP group pathology findings were negative for intraepithelial lesion or malignancy. CONCLUSION: Our study demonstrated an ability to perform a Pap test in a less invasive manner than a traditional Pap test. Although all blind Pap test specimens were adequate, only in 57% was the EC/TZ component reported to be present, compared to 90% of the traditional Pap test specimens.

Rates of Intrauterine Device Expulsion Among Adolescents and Young Women.

STUDY OBJECTIVE: The objective of our study was to determine the rate of intrauterine device (IUD) expulsion and risk factors for expulsion among adolescents and young adults. DESIGN: Retrospective chart review. SETTING: IUD insertions were performed at a single children's hospital. PARTICIPANTS: Eligible adolescent and young adult patients who underwent IUD insertion between August 2009 and March 2019. INTERVENTIONS: IUD insertion. MAIN OUTCOME MEASURES: Primary outcome was the incidence of IUD expulsion in adolescents and young women. Secondary outcomes were risk factors for IUD expulsion including heavy menstrual bleeding, abnormal uterine bleeding (AUB), anemia, or a bleeding disorder diagnosis. RESULTS: Six hundred forty-two eligible patients underwent IUD insertion. The incidence of first IUD expulsion in this population was 58/642 (9.03%). Among those who chose to have a second IUD placed (n = 29), 8/29 (27.6%) had a second expulsion. Patients who expelled their IUD were more likely to have a history of AUB, heavy menstrual bleeding, anemia, or a bleeding disorder. When controlled for body mass index and age at insertion, history of AUB and anemia remained significant risks for IUD expulsion. CONCLUSION: This study similarly showed a higher risk of primary and secondary IUD expulsion in adolescents and young women. A history of AUB, anemia, bleeding disorder, and elevated body mass index are associated with higher risk for IUD expulsion. This population should be counseled that these conditions might place them at higher risk for expulsion.

The Prevalence of Müllerian Anomalies in Women with a Diagnosed Renal Anomaly.

STUDY OBJECTIVE: To characterize the prevalence of Müllerian anomalies (MAs) among patients with renal anomalies (RAs). DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: A retrospective chart review of female patients with RAs who presented to an academic pediatric hospital between 2007 and 2019 was performed. Patients were identified using International Classification of Diseases 9th and 10th revision codes. Data collected included the type of RA, presence and type of MA, method of diagnosis, and associated anomalies. RA subtype analysis was performed. RESULTS: We identified 5590 cases of RA for the years 2007 through 2019. A random, retrospective chart review was performed resulting in a study population of 363 RA patients. The prevalence of any MA in the overall RA population was 104/363 (29%) (95% confidence interval, 24% - 33%). The prevalence of MA for patients with renal agenesis was 59/182 (32%) compared with 45/181 (25%) for patients with renal dysgenesis. The most common MA were failures of Müllerian duct fusion. Only 73/352 (21%) of patients received screening for a MA at the time of RA diagnosis. Of patients without a diagnosed MA 187/259 (72%) were unscreened and either not yet menarchal or had unknown menarchal status. CONCLUSIONS: Of all RA patients, 29% (n = 104/363) had an underlying MA. No difference was found in the prevalence of MA in patients with renal agenesis vs dysgenesis. Limitations noted are that some patients might be of an age at which assessment of the Müllerian structures is suboptimal or who might not have been screened. These results suggest the need for a prospective study to determine evidence-based guidelines for screening for MA among patients diagnosed with any RA to avoid complications from an unrecognized MA.

Patterns of Genital Examination and Vulvovaginal Graft-Versus-Host Disease in a Pediatric Post-Hematopoietic Stem Cell Transplant Population.

STUDY OBJECTIVE: To determine vulvovaginal graft-versus-host disease (vvGVHD) incidence among pediatric patients who have received hematopoietic stem cell transplantation (HSCT) and who already have graft-versus-host disease (GVHD) involving any organ system and characterize patterns of genital examination and referral to pediatric and adolescent gynecology (PAG) in the post-HSCT population. DESIGN: Retrospective chart review. SETTING: Large tertiary children's hospital in Texas. PARTICIPANTS: Eighty-six post-HSCT female patients 21 years old and younger with GVHD involving any organ system. INTERVENTIONS: None. MAIN OUTCOME MEASURES: vvGVHD among post-HSCT children, referrals to PAG, genital examinations documented by any clinician. RESULTS: Eighty-six patients met inclusion criteria. Most HSCTs were bone marrow transplants, typically for leukemia. Median ages of indication diagnosis and HSCT were 5.1 and 7.5 years, respectively. Median time from HSCT to first GVHD diagnosis (eg, skin, intestine) was 96 days. Nearly all patients had at least 1 genital exam documented in the first 2 years post-HSCT, with a median of 17 exams. Twenty-eight patients were seen by PAG post-HSCT, with 7 of these patients seen within the first 2 years post-HSCT. Four symptomatic patients were diagnosed with vvGVHD. Median time from HSCT to vvGVHD was 398 days. CONCLUSION: The small number of vvGVHD cases in our study population is likely because of lack of symptom reporting from patients and families and difficulty with vvGVHD diagnosis. Further training for non-PAG physicians, including pediatricians and oncologists, in identifying and managing vvGVHD might prevent delayed diagnosis and severe sequelae. Earlier referral to PAG or a gynecologist versed in post-HSCT survivorship is also recommended.