Publications by authors named "Jennifer E Dietrich"

96 Publications

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

J Pediatr Adolesc Gynecol 2021 Jun 10. Epub 2021 Jun 10.

Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas; Texas Children's Hospital Pavillion for Women, Houston, Texas.

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/m vs 23.44 kg/m, 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.
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http://dx.doi.org/10.1016/j.jpag.2021.05.014DOI Listing
June 2021

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

Am J Hum Genet 2021 02;108(2):337-345

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.

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.
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http://dx.doi.org/10.1016/j.ajhg.2020.12.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7896104PMC
February 2021

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

J Pediatr Adolesc Gynecol 2021 Jun 14;34(3):400-403. Epub 2020 Dec 14.

Baylor College of Medicine, Houston, Texas; Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas; Division of Pediatric and Adolescent Gynecology, Baylor College of Medicine, Houston, Texas; Department of Pediatrics, Baylor College of Medicine, Houston, Texas. Electronic address:

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.
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http://dx.doi.org/10.1016/j.jpag.2020.12.010DOI Listing
June 2021

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

J Pediatr Adolesc Gynecol 2021 Apr 23;34(2):154-160. Epub 2020 Nov 23.

Division of Pediatric and Adolescent Gynecology, Department of Gynecology, Baylor College of Medicine, Houston, TX.

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.
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http://dx.doi.org/10.1016/j.jpag.2020.11.015DOI Listing
April 2021

Rates of Intrauterine Device Expulsion Among Adolescents and Young Women.

J Pediatr Adolesc Gynecol 2021 Jun 13;34(3):362-365. Epub 2020 Nov 13.

Baylor College of Medicine, Houston, Texas; Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas; Division of Pediatric and Adolescent Gynecology, Baylor College of Medicine, Houston, Texas.

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.
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http://dx.doi.org/10.1016/j.jpag.2020.11.003DOI Listing
June 2021

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

J Pediatr Adolesc Gynecol 2020 Dec 8;33(6):658-666. Epub 2020 Aug 8.

Division of Pediatric and Adolescent Gynecology, Departments of Obstetrics and Gynecology and Pediatrics, Baylor College of Medicine, Houston, Texas; Pediatric and Adolescent Gynecology, Texas Children's Hospital, Houston, Texas.

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.
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http://dx.doi.org/10.1016/j.jpag.2020.08.004DOI Listing
December 2020

HMB in the Adolescent: A Review of the Modern Approach to Diagnosis and Management.

Clin Obstet Gynecol 2020 09;63(3):553-560

Department of OBGYN, Division of Pediatric and Adolescent Gynecology, Baylor College of Medicine, Houston, Texas.

Heavy menstrual bleeding in the adolescent is a cause for concern whether occurring acutely or chronically. There are a number of important considerations during the initial presentation that will help guide the practitioner during workup, which ultimately guides management strategies. The cornerstone of management in the adolescent is that of medical therapy (hormonal and nonhormonal), with avoidance of invasive and irreversible measures, as maintenance of fertility is paramount. Ultimately, the majority of adolescents can be successfully managed in the acute setting and transitioned to maintenance therapy for long-term control of heavy menses. Here, we will review the modern approach to this condition as well as tips and tricks for the practitioner.
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http://dx.doi.org/10.1097/GRF.0000000000000523DOI Listing
September 2020

Paratubal Cyst Recurrence in Children and Adolescents.

J Pediatr Adolesc Gynecol 2020 Dec 24;33(6):649-651. Epub 2020 Jul 24.

Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas; Division of Pediatric and Adolescent Gynecology, Baylor College of Medicine, Houston, Texas; Department of Pediatrics, Texas Children's Hospital, Houston, Texas; Department of Obstetrics and Gynecology, Division of Pediatric and Adolescent Gynecology, Houston, Texas; Department of Pathology and Immunology, Department of Pediatrics, Houston, Texas.

Study Objective: To examine the association between patient characteristics and risk for recurrence risk of paratubal cysts (PTC) in children and adolescents.

Design: Retrospective chart review at a single institution.

Setting: Single academic children's hospital.

Participants: Pediatric patients presenting to Texas Children's Hospital between July 2007 and March 2019. Patients were identified for the study by reviewing pathology reports and were included if they met inclusion criteria of a pathologic diagnosis of a paratubal or paraovarian cyst removed during any surgical procedure between July 2007 and March 2019.

Interventions: Subjects with pathologic diagnoses of a paratubal cyst during the study period underwent chart review for the following data points: age at presentation, ethnicity, pathologic recurrence of paratubal cysts, pubertal status, body mass index (BMI), diagnosis of polycystic ovary syndrome (PCOS), size of cyst, laterality of cysts, and number of cysts.

Main Outcome Measure(s): Recurrence, Pathology types.

Results: Of the 627 patients that met inclusion criteria, the incidence of recurrence was 11.3%. Group 1 included those with recurrence of PTC (N = 70). Group 2 was identified as those without recurrence of PTC (N = 557). There were no differences related to age, BMI, ethnicity, history of PCOS, cyst size, laterality or number of cysts present. PTC NOS and serous PTC occurred most frequently. Of the unique cases involving recurrence, 70.1% recurred on the ipsilateral side. There were no cases of paratubal cyst malignancies in this cohort. The range of pathology diagnoses included pathologies that may occur in ovarian cysts. This is particularly interesting, given the known origins of ovarian cancer from fallopian tube transformations. Rare pathology diagnoses likely did not occur with frequency to determine definitive risks of recurrence in these cases.

Conclusions: There appears to be a baseline recurrence risk for PTC, for which patients can be counseled. Recurrence does not appear to be associated with any particular pathology type, cyst size, number of cysts, BMI, PCOS, or puberty stage. Recurrence, should it occur, appears to occur more commonly on the ipsilateral side.
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http://dx.doi.org/10.1016/j.jpag.2020.07.008DOI Listing
December 2020

The spectrum and severity of bleeding in adolescents with low von Willebrand factor-associated heavy menstrual bleeding.

Blood Adv 2020 07;4(13):3209-3216

Department of Medicine, University of Rochester, Rochester, NY.

Low von Willebrand factor (VWF) in adults is associated with significant bleeding, most notably heavy menstrual bleeding (HMB) and postpartum hemorrhage, although this has not been characterized in adolescents. The objectives of this analysis of a multicenter single arm observational cohort study in adolescents with low VWF-associated HMB were to describe the bleeding phenotype, HMB severity, and related complications. Eligibility criteria included postmenarchal females <21 years of age with HMB (Pictorial Blood Assessment Chart [PBAC] score >100) and low VWF (2 values of VWF activity ≥30 and ≤50 IU/dL). Patients diagnosed with other bleeding disorders were ineligible. Clinical phenotype data, including PBAC and Bleeding Assessment Tool (BAT) scores, laboratory data, and HMB management/outcome details, were extracted. Patient demographics and clinical characteristics were summarized as medians with minimum/maximum values or frequencies with percentages. Groups were compared using a Wilcoxon rank-sum test or Fisher's exact test. A total of 113 patients met inclusion criteria, and 2 were excluded. Ninety four percent had a significant bleeding phenotype (BAT score >2), with predominantly mucocutaneous bleeding (32%-44%), postprocedural/surgical bleeding (15%), and severe HMB (BAT HMB domain score ≥2; 90%). Bleeding complications included iron deficiency (60%), anemia (21%), transfusion (12%), and hospitalization (10%). Desmopressin challenge response in subjects tested was good and sustained. Several (48%) required combined therapy for HMB (hormonal/hemostatic), and one third did not show improvement despite therapy. Our results suggest that adolescent females with low VWF have a significant bleeding phenotype and resultant complications warranting a focus on prompt diagnosis, appropriate therapy, and prevention of complications.
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http://dx.doi.org/10.1182/bloodadvances.2020002081DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7362347PMC
July 2020

Development of a multidisciplinary colorectal and pelvic health program: Program implementation and clinical impact.

J Pediatr Surg 2020 Nov 6;55(11):2397-2402. Epub 2020 May 6.

Texas Children's Hospital, Baylor College of Medicine, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX. Electronic address:

Introduction: Pediatric patients with complex colorectal and genitourinary conditions often require coordinated multidisciplinary care; however, this coordinated care can be hard to structure and deliver. The purpose of this paper is to review the development and implementation of a multidisciplinary colorectal and pelvic health program, one year after the program's initiation.

Methods: This is an observational retrospective 1-year study (10/1/2017 to 9/30/2018). In fiscal year (FY) 2018, a multidisciplinary colorectal and pelvic health program was initiated. The program development incorporated bimonthly team meetings, educational conferences, and initiation of three clinics: a complex colorectal and genitourinary reconstruction clinic, a bowel management clinic, and a colonic motility clinic. Conditions treated included complex anorectal and cloacal malformations, Hirschsprung disease, and idiopathic constipation. The fiscal year was selected to provide comparative administrative data after program implementation.

Results: During the study period, 121 patients underwent comprehensive collaborative evaluation of which 58 (47%) were new to the institution compared to 12 (19%) new patients in the previous year (p < 0.001). In FY 2018, there were 130 procedures performed and 512 collaborative visits with an average of 47 visits per month. This was a 3.4-fold increase in visits compared to FY2017 (171 visits). Of the new patients, 60% (35/58), traveled a median of 181 miles, representing 33 statewide counties, and 4 states compared to a median of 93 miles in the previous fiscal year (p = 0.004).

Conclusion: The development of a colorectal and pelvic health program is feasible and requires a collaborative approach, necessitating multiple service lines within an institution. Program creation and implementation can result in rapid institutional clinical growth by filling a local and regional need through coordinated multidisciplinary care.

Level Of Evidence: IV.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.05.002DOI Listing
November 2020

Prevention of Recurrent Mucinous Borderline Ovarian Tumor with Aromatase Inhibitor.

J Pediatr Adolesc Gynecol 2020 Oct 3;33(5):610-612. Epub 2020 Apr 3.

Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas.

Background: Aromatase inhibitors (AIs) are used for estrogen-modulated conditions. Some borderline ovarian tumors (BOT) express estrogen receptors. We present 2 cases of progression from mucinous cystadenoma to mucinous BOT (mBOT) after prior cystectomies in whom an AI was used with recurrence prevention.

Cases: Two patients underwent laparoscopic ovarian cystectomy for mucinous cystadenoma. Serial imaging demonstrated recurrent ovarian cysts for which both underwent fertility sparing surgery (FSS) with ovarian cystectomy for mBOT. Both patients were initiated on an AI and have been without recurrence.

Summary And Conclusion: BOT predominantly occur in reproductive aged females. FSS with cystectomy is an option, but recurrence occurs in 12-36% of cases. The use of AI in prevention of recurrent BOT shows promise, and more studies are needed to explore this treatment.
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http://dx.doi.org/10.1016/j.jpag.2020.03.011DOI Listing
October 2020

Abnormal Uterine Bleeding in the Adolescent.

Obstet Gynecol 2020 03;135(3):615-621

Division of Pediatric and Adolescent Gynecology, Departments of Obstetrics and Gynecology and Pediatrics, Baylor College of Medicine, Houston, Texas; and the Department of Obstetrics and Gynecology, UCSF Fresno, Fresno, California.

Abnormal uterine bleeding is a common problem in adolescents. The differential diagnosis varies from pregnancy and infection to anovulation and coagulopathy. Careful history and examination can help elucidate the best next steps for workup and management. Heavy menstrual bleeding is particularly worrisome in this group not only when it occurs at menarche, but also anytime afterward when bleeding lasts longer than 7 days, blood loss is greater than 80 mL per cycle, or other warning signs that indicate a history of heavy bleeding such as anemia. Once conditions of pregnancy, infection, structural abnormalities, and hormonal causes have been ruled out, first-line treatment is medical management with hormonal therapy or nonhormonal options. Invasive measures are a last resort in this patient population, because maintenance of fertility is of critical importance.
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http://dx.doi.org/10.1097/AOG.0000000000003693DOI Listing
March 2020

Gynecologic Management of Pediatric and Adolescent Patients with Ehlers-Danlos Syndrome.

J Pediatr Adolesc Gynecol 2020 Jun 25;33(3):291-295. Epub 2019 Dec 25.

Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas; Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

Study Objective: Ehlers-Danlos syndrome (EDS) is a connective tissue disorder resulting in abnormal collagen synthesis leading to skin, joint, ligament, blood vessel, and organ abnormalities. Studies in adult women show an association with heavy menstrual bleeding, dysmenorrhea, and pelvic organ prolapse. We aimed to evaluate gynecologic complaints in pediatric and adolescent patients with EDS and their management by pediatric and adolescent gynecology (PAG) Providers. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Charts of female individuals less than 21 years of age with an International Classification of Diseases (ICD) Ninth or Tenth revision diagnosis of EDS who were evaluated between July 1, 2007, and July 31, 2017, were reviewed for menstrual history, gynecologic complaints, and interventions prescribed.

Results: A total of 156 patients met inclusion criteria, and 26 (16.7%) were seen by PAG providers. The mean age was 14.5 ± 1.9 years. Fifteen (57.7%) reported dysmenorrhea, 13 (50%) complained of heavy menstrual bleeding (HMB), 10 (38.5%) reported irregular menses, and 7 (26.9%) sought contraception. Concurrent medical problems were reviewed, as this affected hormone choice. The cohort was stratified into 2 groups: patients whose menstrual cycles were well controlled on a single method (group A), and patients who tried multiple medications (group B). Progesterone-only pills were most commonly used in froup A. Eleven (73%) patients in group B tried depot medroxyprogesterone acetate (DMPA), but ultimately a levonorgestrel intrauterine device (IUD) was the most popular final choice of treatment and was used by 4 (27%) patients.

Conclusion: Few adolescents with EDS are referred to PAG providers despite the prevalence of gynecologic complaints and potential for obstetric and gynecologic complications. In this population, early entry to gynecologic care would be beneficial.
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http://dx.doi.org/10.1016/j.jpag.2019.12.011DOI Listing
June 2020

Physiologic Ovarian Cysts versus Other Ovarian and Adnexal Pathologic Changes in the Preadolescent and Adolescent Population: US and Surgical Follow-up.

Radiology 2019 07 21;292(1):172-178. Epub 2019 May 21.

From the Department of Radiology, Texas Children's Hospital, 6701 Fannin St, Suite 470, Houston, TX 77030 (E.K.S., A.R.M.R., R.C.O.); Department of Surgery, Children's Hospital of Michigan, Detroit, Mich (P.I.A.); Department of Radiology, Geisinger Wyoming Valley Medical Center, Wilkes Barre, Pa (M.C.P.); and Department of Pediatric and Adolescent Gynecology, Texas Children's Hospital, Houston, Tex (J.E.D.).

Background Ovarian and adnexal cysts are frequently encountered at US examinations performed in preadolescent and adolescent patients, yet there are few published studies regarding the outcomes of cysts in this population. Purpose To identify characteristics at US that help to distinguish physiologic ovarian cysts from nonphysiologic entities. Materials and Methods Female patients who underwent pelvic US with or without Doppler from January 2009 through December 2013 were identified by using a centralized imaging database. Patients older than 7 years and younger than 18 years with ovarian or adnexal cysts at least 2.5 cm were included. Demographic characteristics, date of surgery, surgical notes, and pathologic reports were extracted from the electronic medical record. Initial and follow-up dates of US, cyst size and complexity, imaging diagnosis, and change on subsequent US images were recorded. Statistical analysis was performed with the Wilcoxon rank sum and Kruskal-Wallis tests for continuous variables and the Fisher exact test for categorical variables. Results Of 754 patients who met inclusion criteria (age, 8-18 years; mean age, 14.6 years ± 1.9 [standard deviation]; mean cyst size, 5 cm ± 3.3), 409 patients underwent complete follow-up that included resolution at imaging ( = 250) or surgery ( = 159). In the patients with complete imaging follow-up, mean time to US documentation of resolution was 194 days ± 321; 59.6% (149 of 250) patients had nonsimple cyst characteristics. One-hundred fifty-nine patients underwent surgical intervention (mean cyst size, 8.5 cm ± 5.3), and 69.8% (111 of 159) of the cysts had simple characteristics. Of the 159 cysts, 100 (62.8%) were defined in the pathologic report as paratubal cysts. Of 409 patients, no malignancies were encountered in this study population with surgical or imaging resolution. Conclusion No malignancies were encountered in the study population and the majority of cysts resolved at follow-up imaging. Large size, persistence, and separability from the ovary were most helpful for identification of nonphysiologic paratubal cysts. © RSNA, 2019.
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http://dx.doi.org/10.1148/radiol.2019182563DOI Listing
July 2019

Can We Improve Vaginal Tissue Healing Using Customized Devices: 3D Printing and Biomechanical Changes in Vaginal Tissue.

Gynecol Obstet Invest 2019 28;84(2):145-153. Epub 2018 Sep 28.

Division of Pediatric Adolescent Gynecology, Baylor College of Medicine, Houston, Texas, USA.

Background: Determining biomechanical changes in vaginal tissue with tissue stretch is critical for understanding the role of mechanotransduction on vaginal tissue healing. Noncontact dynamic optical coherence elastography (OCE) can quantify biomechanical changes in vaginal tissues noninvasively. Improved vaginal tissue healing will reduce postoperative complications from vaginal surgery.

Aims: (1) To complete dimensional assessments (DAs) of the vaginal tract. (2) To elucidate biomechanical properties (BMP) of porcine vaginal tissues (PVT). (3) Compare BMPs of piglet and adult PVTs after placement of customized vaginal dilators (VD) by OCE and uniaxial mechanical testing (MT).

Methods: Pilot study using adult nulliparous pig and piglet PVTs (n = 20 each). DA of PVTs was performed using silicone molding. 3D-printed VDs were used to achieve different Relative Diameter Change (RDC) of the PVTs (no dilatation, and -50%, 0%, 50% RDC). Elastographic testing using OCE and MT.

Results: Using OCE, no significant differences (SD) were noted between adult and piglet PVT (p = 0.74) or by stretch direction (p = 0.300). SD was noted with increasing RDC (p = 0.023). Using MT, there were SD in tissue stiffness between adult and piglet PVT (p = 0.048), but no SD as a function of RDC (p = 0.750) or stretch direction (p = 0.592).

Conclusions: This study quantified biomechanical changes in PVT with customized stretching by 3D printed VD using both OCE and MT. This work has implications for the mechanotransduction of vaginal wound healing and noninvasive assessment of vaginal diseases.
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http://dx.doi.org/10.1159/000491696DOI Listing
May 2019

Adnexal Torsion in Children and Adolescents: A Retrospective Review of 245 Cases at a Single Institution.

J Pediatr Adolesc Gynecol 2019 Feb 12;32(1):64-69. Epub 2018 Nov 12.

Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas; Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas.

Study Objective: Adnexal torsion (AT) requires urgent surgical intervention to preserve ovarian function. Historically, treatment for AT was oophorectomy because of concerns related to leaving a nonviable ischemic ovary. No published studies support these theoretical concerns and current literature supports conservative management. The aim of this study was to review the institutional outcomes for AT cases, including salvage rates and complications. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: This study was approved by the institutional review board at Baylor College of Medicine. A retrospective chart review on cases of AT from 2007 to 2016 at a single Children's hospital was performed on the basis of International Classification of Diseases, 10th revision and Current Procedural Terminology codes. A standardized chart review form was used in data extraction. Statistical analysis was performed using SAS version 9.4 (SAS Institute).

Results: Chart review identified 245 torsion cases in 237 patients. The mean age was 12.4 ± 3.29 years. Of the participants, 230 (94%) underwent minimally invasive laparoscopy with ovarian preservation in 233 (95%) of the cases. There were no complications due to detorsion of the affected adnexa. Intraoperatively, the right adnexa was affected in 134 (55%) cases and a lesion was noted in 193 (79%) cases, most commonly paratubal cysts and mature teratomas. The malignancy rate was low, noted only in 4/245 patients at (1.2%). Pediatric gynecology performed most of the cases (n = 214; 87%).

Conclusion: The findings of our study continue to support the conservative management of patients with AT.
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http://dx.doi.org/10.1016/j.jpag.2018.07.003DOI Listing
February 2019

Gynecologic Bleeding Complications in Postmenarchal Female Adolescents Receiving Antithrombotic Medications.

J Pediatr Adolesc Gynecol 2018 Jun 20;31(3):242-246. Epub 2017 Dec 20.

Department of Pediatrics, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas.

Study Objective: The prevalence, clinical features, and management of gynecologic bleeding complications and health care provider awareness of these in postmenarchal adolescents receiving antithrombotic medications has rarely been addressed in the literature. We sought to address these issues in a review of our experience in a pediatric tertiary care center.

Design, Setting, And Participants: A retrospective chart review was conducted with institutional review board approval from 2004 to 2014, on eligible postmenarchal adolescents receiving antithrombotic medications. Descriptive statistics were used.

Results: Sixty-eight adolescents received antithrombotic medications (thromboembolism in 67 of 68; 99%; cardiac causes in 4 of 68; 6%), which included enoxaparin, warfarin, unfractionated heparin, alteplase, fondaparinux, and aspirin. Heavy menstrual bleeding (HMB) screening questions were documented by treating hematologists in 52 of 68 patients (76%; 95% confidence interval, 65%-86%). Adolescent gynecology consult was requested for 25 of 68 patients (37%). After antithrombotic medications were started, 13 of 68 (19%) developed HMB. Anemia was found in 43% of patients tested (18 of 42); 50% (9 of 18) and 78% patients (14 of 18) received packed red blood cell transfusion and iron therapy, respectively. Five patients (5 of 68; 7%) developed hemorrhagic ovarian cysts, 40% (2 of 5) were treated with surgery, 16% (1 of 5) received transfusions, and 100% (5 of 5) received or continued progesterone-only therapy with no recurrence.

Conclusion: Adolescents receiving antithrombotic medications are at risk of developing gynecologic bleeding complications, which can result in anemia, hospitalization, transfusions, or surgery. Provider awareness/screening of HMB as a bleeding complication of antithrombotic medications is less than optimal. Future prospective studies in adolescents receiving antithrombotic medications are needed to better evaluate provider awareness and the prevalence of gynecologic bleeding complications, which can lead to effective management.
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http://dx.doi.org/10.1016/j.jpag.2017.12.007DOI Listing
June 2018

Borderline Ovarian Tumor in the Pediatric and Adolescent Population: A Case Series and Literature Review.

J Pediatr Adolesc Gynecol 2018 Feb 9;31(1):48-54. Epub 2017 Sep 9.

Division of Hematology-Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

Study Objective: To determine the diagnosis, management, and outcome for children and adolescents with borderline ovarian tumor (BOT), and to provide a review of the literature on BOT in children and adolescents.

Design: A retrospective cohort study of female adolescents younger than age 21 years diagnosed with BOT between January 2001 and May 2016.

Setting: Texas Children's Hospital, Houston, Texas.

Participants: Fourteen patients (ages 12 to 18 years) diagnosed with BOT.

Main Outcome Measures: Clinical presentation, preoperative characteristics, surgical technique, cancer stage, histology, treatment, and recurrence.

Results: Median age at diagnosis was 15.5 years, with most postmenarchal. Abdominal mass/pain were the most common presenting symptoms. Median tumor size was 16.6 cm (range, 4-32 cm). Preoperative cancer antigen 125 (CA 125) was elevated in 54% (7/13) of cases. All patients had fertility-preserving surgery, either cystectomy (CY) or unilateral salpingo-oophorectomy (USO): 5 via laparoscopy (LSC) and 9 via laparotomy. Most were stage I with 5 serous and 9 mucinous BOT histology. No one received adjuvant chemotherapy. Two patients had recurrence. One had ipsilateral recurrence 2 months after LSC CY for FIGO stage IC1 mucinous BOT. The second had contralateral recurrence 15 months after laparotomy, right USO for FIGO stage IIIC serous BOT treated with LSC CY, then a second recurrence treated with USO after oocyte cryopreservation for fertility preservation. All patients were alive at last follow-up, 1 with disease.

Conclusions: BOT in children and adolescents can be treated conservatively with fertility-preserving techniques and surveillance with good outcome. The role of adjuvant therapy is not known.
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http://dx.doi.org/10.1016/j.jpag.2017.09.001DOI Listing
February 2018

Evaluation and management of heavy menstrual bleeding in adolescents.

Curr Opin Obstet Gynecol 2017 Oct;29(5):328-336

Baylor College of Medicine, Houston, Texas, USA.

Purpose Of Review: Heavy menstrual bleeding (HMB) is a common condition in women of reproductive age; however, adolescents with this issue present unique challenges in both diagnosis and management. Much of the research into this topic focuses on the adult population, with variable applicability to adolescents. There are currently no standard guidelines for the work up and treatment of adolescents with HMB.

Recent Findings: Current research into this topic has explored the utilization of standardized protocols in the evaluation of HMB in adolescents, the efficacy of various hormonal, nonhormonal, and surgical treatment modalities, and the benefits of a multidisciplinary approach. Recent literature has focused on adolescents found to have an underlying bleeding disorder, recommending more comprehensive bleeding disorder work up to identify these patients in a timely manner and initiate effective treatment plans.

Summary: Providers in the primary care setting should be aware of the definitions for normal menses, and be able to recognize abnormal bleeding and HMB. Early recognition of HMB in adolescents can then lead to appropriate diagnosis of underlying disorders, and current research has proposed standard protocols to assist with the evaluation, ultimately leading to effective long-term management into adulthood.VIDEO ABSTRACT.
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http://dx.doi.org/10.1097/GCO.0000000000000394DOI Listing
October 2017

A Multidisciplinary Approach to Puberty and Fertility in Girls with Turner Syndrome.

Pediatr Endocrinol Rev 2016 Sep;14(1):33-47

Department of Obstetrics and Gynecology. Baylor College of Medicine. Texas Children's Hospital, Houston, TX, USA.

Women with Turner Syndrome (TS) have a variety of medical needs throughout their lives; however, the peripubertal years are particularly challenging. From a medical perspective, the burden of care increases during this time due to growth optimization strategies, frequent health screenings, and puberty induction. Psychologically, girls begin to comprehend the long-term implications of the condition, including their diminished fertility potential. Unfortunately, clear guidelines for how to best approach this stage have not been established. It remains to be determined what is the best age to begin treatment; the best compound, dose, or protocol to induce puberty; how, when or what to discuss regarding fertility and potential fertility preservation options; and how to support them to accept their differences and empower them to take an active role in their care. Given the complexity of this life stage, a multidisciplinary treatment team that includes experts in endocrinology, gynecology, and psychology is optimal.
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http://dx.doi.org/10.17458/PER.2016.CCALM.MultidisciplinaryApproachDOI Listing
September 2016

Paratubal Cyst Size Correlates With Obesity and Dysregulation of the Wnt Signaling Pathway.

J Pediatr Adolesc Gynecol 2017 Oct 27;30(5):571-577. Epub 2017 Apr 27.

Center for Reproductive Medicine, Baylor College of Medicine, Houston, Texas; Department of Urology, Baylor College of Medicine, Houston, Texas. Electronic address:

Study Objective: Paratubal cysts (PTCs) occur in 7%-10% of women, regardless of age. Although common, PTCs often are found incidentally because of the potential for these cysts to be asymptomatic. The specific aims of the study were to determine if PTC number and size correlated with signs of hyperandrogenism and obesity, as well as to investigate the molecular profiles of these PTCs in samples derived from female adolescents. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: A prospective cohort study was performed in a single children's hospital. Girls 18 years of age or younger who underwent surgery for PTC suspected on the basis of the presence of a persistent adnexal cyst on imaging or a concern for adnexal torsion involving a cyst were consented to participate in the study.

Results: Nineteen patients met enrollment criteria with a mean age at menarche of 11.2 ± 1.3 years. Most of the patients (84%; n = 16/19) had adnexal torsion at the time of diagnosis of PTC. Irregular menses and hirsutism was found in 52.6% (n = 10/19) of the patients, among whom 36.8% (n = 7/19) were obese. The mean PTC size was 10.4 ± 4.3 cm with 57.9% (n = 11/19) of the cohort having more than 1 PTC. When patients were compared on the basis of their body mass index, the size of PTCs was significantly larger in the overweight/obese group. The wingless-type (WNT) signaling members catenin beta 1 (CTNBB1) and wingless-type MMTV integration site family, member 7A (WNT7A) were upregulated in 86% (n = 12/14) and 79% (n = 11/14) of the patients, respectively. WNT7A was significantly upregulated in girls with 1 cyst and low body mass index.

Conclusion: A correlation exists between obesity, cyst size, and hyperandrogenism. Activation of the WNT/CTNBB1 pathway via WNT7A might play a role in PTC development.
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http://dx.doi.org/10.1016/j.jpag.2017.04.002DOI Listing
October 2017

Intraoperative Rupture of Ovarian Dermoid Cysts in the Pediatric and Adolescent Population: Should This Change Your Surgical Management?

J Pediatr Adolesc Gynecol 2017 Dec 21;30(6):636-640. Epub 2017 Mar 21.

Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas.

Study Objective: (1) To determine the incidence of chemical peritonitis after cystectomy for ovarian dermoid cysts with intraoperative cyst rupture in the pediatric and adolescent population; and (2) to examine the intraoperative and postoperative outcomes of cystectomies performed for ovarian dermoid cysts using laparoscopy and laparotomy, especially those with intraoperative cyst rupture.

Design: A retrospective cohort study of female patients who underwent ovarian cystectomy with proven ovarian dermoid pathology between July 2007 and July 2015.

Setting: Texas Children's Hospital, Houston, Texas.

Participants: One hundred forty-four patients between the ages of 1 and 21 years who underwent an ovarian cystectomy with proven benign ovarian dermoid histology on the basis of pathology reports.

Interventions And Main Outcome Measures: Occurrence of spill of cyst contents, chemical peritonitis, postoperative complications, and hospital readmissions.

Results: One hundred forty-four female patients underwent cystectomy (38 laparotomy and 106 laparoscopy) resulting in benign ovarian dermoid histology. Their mean age was 12.4 ± 4.1 years (range, 1-21), mean cyst size was 9.2 ± 6.4 cm (range, 1-30 cm), no patients had elevated tumor markers, 42 (29.1%) presented with torsion, 73 (50.7%) had cyst fluid spill, and there were no cases of chemical peritonitis. Few significant differences were found between cases performed via laparoscopy and laparotomy and those with and without intraoperative cyst rupture resulting in spill of contents. Laparotomy cases were found to have larger mean cyst size (P < .001), estimated blood loss (P = .003), and presence of bilateral cysts (P = .017) compared with laparoscopic cases. Cyst fluid spill occurred in more laparoscopic cases ([67/106] 63.2% vs [6/38] 15.8%; P < .001), and risk increased with cyst size greater than 5 cm (P < .001). In the laparoscopy group, cyst size greater than 5 cm was more likely to present with torsion (P < .001). There was no significant difference in the repeat cystectomy rate between the laparoscopy and laparotomy groups even with cyst rupture (P = .394). Only 5 cases presented to the emergency room postoperatively and 2 were admitted postoperatively for umbilical port site dehiscence and pyelonephritis.

Conclusion: Laparoscopic cystectomy of ovarian dermoid cysts is associated with greater intraoperative cyst rupture. However, cyst rupture is rarely associated with complications, therefore minimally invasive surgical management of ovarian dermoid cysts is a reasonable surgical approach.
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http://dx.doi.org/10.1016/j.jpag.2017.03.139DOI Listing
December 2017

Assessing the Experience of Vaginal Dilator Use and Potential Barriers to Ongoing Use among a Focus Group of Women with Mayer-Rokitansky-Küster-Hauser Syndrome.

J Pediatr Adolesc Gynecol 2017 Aug 16;30(4):491-494. Epub 2017 Feb 16.

Department of Pediatrics, and the Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas.

Study Objective: To assess the experience of dilator use for neovagina creation in women diagnosed with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and identify barriers affecting patient compliance with dilator use.

Design And Setting: This was a qualitative study using a structured questionnaire to investigate perception and identify barriers regarding vaginal dilator use. A questionnaire was administered after participants viewed a 2-minute patient educational video to additionally examine the usefulness of a patient education video tool as an adjunct to counseling in clinic on vaginal dilator use.

Participants, Interventions, And Main Outcome Measures: We enrolled 13 adolescent girls and women diagnosed with MRKH, aged 14-48 years, recruited from clinic and during a conference geared toward women affected by MRKH.

Results: The mean age of participants was 26 (±11.4) years with most diagnosed between the ages of 12 and 18 years. Of all participants, 69% (9/13) reported ever being sexually active with a mean age of coitarche of 16 (±2.6) years. Seventy-seven percent (10/13) reported current or past use of vaginal dilators, and most had some previous education on dilator use. Only 15% (2/13) reported no previous exposure to an educational tool. After viewing the 2-minute educational video, all participants rated it very helpful using a Likert scale (strongly agree to strongly disagree). Major themes that emerged as perceived barriers to dilator use included viewing dilation as a negative experience, uncertainty about success, and lack of motivation.

Conclusion: Education is an integral part of improving compliance with vaginal dilator use among adolescent girls and women affected by MRKH. Technology can be used to reinforce face-to-face counseling in the clinic. To our knowledge, this is the first study to specifically investigate patient experience with dilator use and opinion regarding the use of video technology to demonstrate self-directed neovagina creation in an MRKH population. The barriers identified in this study provide new insights to inform future research and clinical management of dilator use.
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http://dx.doi.org/10.1016/j.jpag.2017.02.002DOI Listing
August 2017

Pediatric medical device development by surgeons via capstone engineering design programs.

J Pediatr Surg 2018 Mar 6;53(3):493-498. Epub 2017 Feb 6.

Division of Pediatric Urology, Department of Surgery, and the Scott Department of Urology, Texas Children's Hospital/Baylor College of Medicine, Houston, TX. Electronic address:

Background: There is a need for pediatric medical devices that accommodate the unique physiology and anatomy of pediatric patients that is increasingly receiving more attention. However, there is limited literature on the programs within children's hospitals and academia that can support pediatric device development. We describe our experience with pediatric device design utilizing collaborations between a children's hospital and two engineering schools.

Methods: Utilizing the academic year as a timeline, unmet pediatric device needs were identified by surgical faculty and matched with an engineering mentor and a team of students within the Capstone Engineering Design programs at two universities. The final prototypes were showcased at the end of the academic year and if appropriate, provisional patent applications were filed.

Results: All twelve teams successfully developed device prototypes, and five teams obtained provisional patents. The prototypes that obtained provisional patents included a non-operative ureteral stent removal system, an evacuation device for small kidney stone fragments, a mechanical leech, an anchoring system of the chorio-amniotic membranes during fetal surgery, and a fetal oxygenation monitor during fetoscopic procedures.

Conclusions: Capstone Engineering Design programs in partnership with surgical faculty at children's hospitals can play an effective role in the prototype development of novel pediatric medical devices.

Levels Of Evidence: N/A - No clinical subjects or human testing was performed.
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http://dx.doi.org/10.1016/j.jpedsurg.2017.01.067DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5545169PMC
March 2018

Heavy Menstrual Bleeding in Adolescents.

J Pediatr Adolesc Gynecol 2017 Jun 17;30(3):335-340. Epub 2017 Jan 17.

Baylor College of Medicine, Houston, Texas. Electronic address:

Heavy menstrual bleeding (HMB) is a very common gynecological condition in female adolescents and a frequent presenting complaint of those with bleeding disorders. Recommendations have been established to screen for bleeding disorders in this age group where appropriate. The purpose of this document is to impart clinical recommendations regarding HMB in adolescents. Specifically, in this article we provide a description of the epidemiology, clinical presentation, diagnostic approach, and treatment of HMB in adolescents.
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http://dx.doi.org/10.1016/j.jpag.2017.01.002DOI Listing
June 2017

Pediatric Ovarian Torsion.

Surg Clin North Am 2017 Feb;97(1):209-221

Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, Baylor College of Medicine, 6651 Main Street, Suite F1020, Houston, TX 77030, USA. Electronic address:

Adnexal torsion is the fifth most common gynecologic emergency. The vague clinical presentation and variable imaging findings make the diagnosis difficult. If suspicion for adnexal torsion is high enough based on clinical symptoms and imaging findings, prompt intervention should include conservative management with laparoscopic adnexal detorsion and ovarian preservation even in the event of a necrotic-appearing ovary because studies persistently show follicular development and ovarian function after a short time period and no increased patient morbidity.
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http://dx.doi.org/10.1016/j.suc.2016.08.008DOI Listing
February 2017

Assessment of an Electronic Intervention in Young Women with Heavy Menstrual Bleeding.

J Pediatr Adolesc Gynecol 2017 Apr 15;30(2):243-246. Epub 2016 Oct 15.

Division of Pediatric Hematology and Oncology, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.

STUDY OBJECTIVE, DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Bleeding disorders (BD) occur in up to 50% of adolescents with heavy menstrual bleeding (HMB). This presents unique challenges to health care providers because of the complexity of treating the condition and such complexity can result in difficulty with patients understanding basic information about their condition, limit communication with medical providers, and patient compliance. The aim of the study was to use an electronic approach to enhance patient compliance with medications used to treat their HMB, and to provide educational access to adolescents with BD. This was a prospective cohort study involving patients in a Young Women's Bleeding Disorder Clinic at a single children's hospital. Subjects were given an iPod Touch (Apple Inc, Cupertino, CA) device (ITD), preloaded with the iPeriod (Winkpass Creations) application. Participants recorded information about their BD that they learned about on BD Web sites, and menses, and medications. Electronic and charted data were collected to monitor compliance with prescribed treatment regimens. All ITD allowed Wi-Fi access to allow teens to explore BD Web sites and knowledge was assessed.

Results: Twenty-three of 45 subjects completed the study. The mean age was 14.1 ± 1.9 years. Subjects who were compliant with the ITD (group 1), charted on baseline symptoms, menstrual flow (83.3%), cramps (100%, 23/23), breakthrough bleeding (95.6%, 22/23), mood (95.6%, 22/23), and medication use (91.7%) for a mean of 9.3 ± 3.1 months. Subjects who were nonusers (group 2) did not report on symptoms, their condition, or medication use in the device (n = 22). More than 75% (17/23) of subjects in group 1 used hormones alone or hormones with antifibrinolytic agents to control HMB. No subjects stopped or missed medications who were in group 1 intentionally, and also there were 9 enrollees within this same group who missed a medication related to awaiting the prescription to be filled from pharmacy. In group 2, 17 enrollees missed medications, resulting in 19% (4/22) of these enrollees being admitted to hospital for 1-2 days. In addition, enrollees in group 2 missed more medications on average compared with group 1. No subjects in group 1 required admission for HMB treatment failure during the study period, compared with those in group 2 (P = .006). All subjects in group 1 reported accessing Web sites using their ITD to learn about their BD. Groups 1 and 2 did not differ in the number of medications that were prescribed during the time frame (P = .77) or the number of follow-up clinic visits (P = .49). Furthermore, those in group 1 reported fewer breakthrough bleeding episodes than those in group 2 according to clinic notes (P = .03). Users of the ITD were given a set of knowledge questions. Group 2 subjects were not consistent users of the ITD use and did not complete the knowledge questions. Group 1 and 2 could not be compared with regard to knowledge as a result.

Conclusion: ITD is an excellent tool for adolescents with HMB and BD to allow self-monitoring, provider monitoring, and improve educational access through engaging technology; compliance with device use was associated with several parameters suggestive of improved clinical outcomes.
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http://dx.doi.org/10.1016/j.jpag.2016.10.006DOI Listing
April 2017

Juvenile Granulosa Cell Tumor of the Ovary: A Clinicopathologic Study.

J Pediatr Adolesc Gynecol 2017 Feb 1;30(1):138-143. Epub 2016 Oct 1.

Texas Children's Cancer Center, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

Study Objective: To report on the clinical characteristics and outcome of pediatric patients with juvenile granulosa cell tumor (JGCT) of the ovary. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Patients with histopathologically confirmed ovarian JGCT diagnosed between 1990 and 2016 were identified. Data on the clinical presentation, surgical management, oncologic management, laboratory investigation, follow-up, and outcome were collected. Tumors were staged according to the International Federation of Gynecology and Obstetrics criteria.

Results: Eight patients were diagnosed with ovarian JGCT during the study period. The median age at presentation was 3 years (range, 0.7-14 years). Precocious puberty was the presenting symptom in all five prepubertal children; abdominal distension due to mass effect was the presenting symptom in three children older than 9 years of age. In patients who had preoperative serologic testing, estradiol (n = 3) and inhibin (n = 3) levels were elevated. Five patients had stage I disease, and three had stage III. All stage I patients underwent salpingo-oophorectomy as the only treatment. Stage III patients received adjuvant chemotherapy. After a median follow-up of 6.2 years, six patients (75%) were alive without evidence of disease. One stage I patient with germline p53 mutation and phosphatase and tensin homolog mutation, died because of subsequent liposarcoma. One patient with stage IIIB disease developed recurrence detected according to an elevated inhibin serum level, and died due to progressive disease despite receiving multiple chemotherapy regimens.

Conclusion: Juvenile granulosa cell tumor has a favorable prognosis in patients with stage I disease after surgical resection alone. Adjuvant chemotherapy might be indicated in patients with higher-stage tumors.
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http://dx.doi.org/10.1016/j.jpag.2016.09.008DOI Listing
February 2017

Longitudinal Vaginal Septum Resection Using the Ligasure Device.

J Pediatr Adolesc Gynecol 2016 Dec 16;29(6):e95-e96. Epub 2016 Jun 16.

Division of Pediatric and Adolescent Gynecology, Department of Obstetrics and Gynecology, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas. Electronic address:

Background: A longitudinal vaginal septum might present as a nonobstructive or obstructive type. Both can result in dyspareunia, difficult tampon insertion, persistent vaginal bleeding despite tampon placement, and dysmenorrhea. Surgical correction is warranted for symptomatic cases.

Case: We present a 16-year-old female adolescent with a complex cloacal anomaly, uterine didelphys, longitudinal vaginal septum, and other congenital anomalies. The patient was unable to use tampons during menses because of the small caliber on both vaginal canals. We performed a longitudinal vaginal septum resection using the Ligasure (Medtronic, Inc, Doral, FL) device. We were able to resect the vaginal septum without difficulty.

Summary And Conclusion: Our case introduces an innovative and safe alternative to the surgical management of a longitudinal vaginal septum, particularly useful in a confined surgical space.
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http://dx.doi.org/10.1016/j.jpag.2016.06.002DOI Listing
December 2016

Levonorgestrel-Releasing Intrauterine Device Use in Female Adolescents with Heavy Menstrual Bleeding and Bleeding Disorders: Single Institution Review.

J Pediatr Adolesc Gynecol 2017 Aug 21;30(4):479-483. Epub 2016 Apr 21.

Department of Pediatrics, Baylor College of Medicine, Houston, Texas; Texas Children's Cancer and Hematology Centers, Houston, Texas.

Study Objective: To identify complications and efficacy of the levonorgestrel-releasing intrauterine device (LNgIUD) in adolescents with heavy menstrual bleeding (HMB) and bleeding disorders (BD).

Design, Setting, And Participants: A retrospective chart review of 13 postmenarchal adolescent girls with HMB/BD who underwent placement of an LNgIUD.

Interventions: Placement of an LNgIUD.

Main Outcome Measures: Primary outcome was to identify complications from placement of an LNgIUD. Secondary outcome was to evaluate the efficacy of the LNgIUD in adolescents with BD.

Results: Thirteen patients met study criteria. The mean age of diagnosis of HMB was 14.08 ± 1.75 years. BD or bleeding risk factor diagnoses included low von Willebrand (VW) activity in 5, type I VW disease in 5, type IIM VW disease in 1, low VW activity and factor 7 deficiency in 1, and acquired VW disease and factor 7 deficiency in 1. Before LNgIUD placement, other hormonal therapy (n = 13) and hemostatic therapy (antifibrinolytic agents, desmopressin acetate; n = 8) yielded poor control of HMB. The LNgIUD was placed using anesthesia with periprocedure hemostatic therapy with no complications. All patients reported significant improvement in HMB after LNgIUD placement and 60% achieved amenorrhea, with mean time to improvement of 94 ± 69 days. Mean hemoglobin and ferritin levels increased after LNgIUD placement compared with before LNgIUD placement values (P = .02, P = .0085, respectively). Use of supplemental hormonal and hemostatic agents decreased (n = 4) after LNgIUD placement. None required LNgIUD removal; 1 spontaneously expelled the LNgIUD with subsequent replacement.

Conclusion: Study results indicated the LNgIUD is an effective therapeutic option in postmenarchal adolescents with HMB due to BD/bleeding risk factor with minimal complications, high compliance rate, improvement in HMB and anemia, and no periprocedural bleeding with hemostatic management.
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http://dx.doi.org/10.1016/j.jpag.2016.04.001DOI Listing
August 2017