Dermatologic care of patients with differences of sex development.

Background: Differences of sex development (DSD or disorders of sex development) are uncommon congenital conditions, characterized by atypical development of chromosomal, gonadal, or anatomic sex.

Objective: Dermatologic care is an important component of the multidisciplinary care needed for individuals with DSD. This article discusses the most common primary dermatologic manifestations of DSD in addition to the cutaneous manifestations of hormonal and surgical therapies in individuals with DSD.

Reproductive late effects after hematopoietic stem cell transplant in pediatric, adolescent, and young adult cancer survivors.

Reproductive late effects after hematopoietic stem cell transplant can have a significant impact on cancer survivors' quality of life. Potential late effects include gonadal insufficiency, genital graft-versus-host disease, uterine injury, psychosexual dysfunction, and an increased risk of breast and cervical cancer in patients treated with total body irradiation. Despite guidelines, screening and treatment are not standardized among at-risk patients.

Management of Hematometra and Hematocolpos in Obstructive Mullerian Anomalies by Image-Guided Drainage with Interventional Radiology: A Multi-Institutional Case Series.

Study Objective: To describe cases of image-guided drainage of symptomatic hematometrocolpos from obstructive Müllerian anomalies as a temporizing measure to manage acute pain symptoms and delay definitive management of the obstructive Müllerian anomalies that require complex reconstruction METHODS: Institutional Review Board exemption from all included institutions was obtained. A retrospective case series from 3 academic children's hospitals of 8 females under the age of 21 with symptomatic hematometrocolpos due to obstructive Müllerian anomalies drained by image-guided percutaneous transabdominal vaginal or uterine drainage with interventional radiology was reviewed and described.

Results: Eight pubertal patients with obstructive Müllerian anomalies (6 patients with distal vaginal agenesis, 1 patient with an obstructed uterine horn, and 1 patient with a high obstructed hemi-vagina) and symptomatic hematometrocolpos are reported.

Elevated von Willebrand factor levels during heavy menstrual bleeding episodes limit the diagnostic utility for von Willebrand disease.

Background: Heavy menstrual bleeding (HMB) is often the first bleeding symptom for female individuals with inherited bleeding disorders. Guidelines recommend performing the hemostatic evaluation at HMB presentation. Von Willebrand factor (VWF) levels increase with stress, making it unclear if VWF studies during acute bleeding are beneficial in diagnosing von Willebrand disease (VWD).

The effect of early puberty suppression on treatment options and outcomes in transgender patients.

In the past 10-15 years, paediatric transgender care has emerged at the forefront of several general practice and subspecialty guidelines and is the topic of continuing medical education for various medical disciplines. Providers in specialties ranging from family medicine, paediatrics and adolescent medicine to endocrinology, gynaecology and urology are caring for transgender patients in increasing numbers. Current and evolving national and international best practice guidelines recommend offering a halt of endogenous puberty for patients with early gender dysphoria, in whom impending puberty is unacceptable for their psychosocial health and wellness.

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.

Fetal exposure to capecitabine and temozolomide during the first trimester: A case report.

Literature on the outcome of pregnancy after exposure to capecitabine and temozolomide during the first trimester is scarce. Chemotherapy administration in the first trimester is generally not recommended due the potential risks to the fetus including fetal death and major congenital malformations. Capecitabine and temozolomide are oral chemotherapy agents and pregnancy category D medications, thus the use of these agents in pregnancy is not recommended.

Compensatory Ovarian Hypertrophy after Unilateral Oophorectomy: Evaluation of Ovarian Volumes in Pediatric and Adolescent Populations.

Study Objective: Limited data exist on the morphologic and physiologic effect on the remaining ovary after unilateral oophorectomy, especially in the pediatric population. Our aim is to evaluate ovarian volumes following unilateral oophorectomy to determine whether compensatory ovarian hypertrophy occurs in the remaining contralateral ovary.

Design: This was a retrospective chart review of ovarian volume measured on ultrasounds that were completed after unilateral oophorectomy.

Vaginal Yolk Sac Tumor: A Case Series and Review of the Literature.

Objective: To report diagnosis, treatment, and outcomes of vaginal yolk sac tumor (YST) cases at a single institution and review literature on vaginal YST to outline advancements in diagnosis, treatment, and survival.

Design: Retrospective chart review of female patients less than 21 years of age with pathologic diagnosis of vaginal YST treated at a large children's hospital, and summary of a 100-year review of the literature on vaginal yolk sac tumor.

Setting: Children's Healthcare of Atlanta, a tertiary center in Atlanta, GA.

2019 Pediatric Initiative Network: Progress, Proceedings, and Plans.

Impairment of fertility and sexual/reproductive health are common after oncologic therapy, and are known to have negative impacts on romantic relationships and psychosocial well-being among childhood cancer survivors. The Pediatric Initiative Network (PIN) is an international, multidisciplinary group of providers within the Oncofertility Consortium dedicated to preserving and protecting the fertility of children and adolescents at risk for infertility due to medical conditions or treatments. The PIN and its Best Practices and Research committees meet virtually throughout the year, with one annual in-person meeting.

A Review of Mullerian Anomalies and Their Urologic Associations.

Structural anomalies of the female reproductive tract, known as Mullerian anomalies, can occur in isolation or in association with anomalies of other organ systems. Due to shared embryology, the most common association in up to 40% of patients is with renal, ureteral, and bladder anomalies. Affected girls can have a wide range of genitourinary symptoms with urologists playing an integral role in their diagnosis and treatment.

Enhanced Recovery After Surgery in Pediatric and Adolescent Gynecology: A Pilot Study.

Study Objective: Enhanced recovery after surgery (ERAS) protocols have been successfully implemented in adult gynecology as well as adult and pediatric colorectal and urologic surgery with reduction in narcotic use, complications, return to the system (RTS), length of stay (LOS), and improved patient satisfaction. There are no studies evaluating the use of ERAS in pediatric and adolescent gynecology (PAG). The goals of this study are to present initial patient outcomes using ERAS in PAG patients undergoing intra-abdominal gynecologic surgery to prove efficacy, patient satisfaction, and decreased narcotic use.

Tubo-Ovarian Abscesses in Nonsexually Active Adolescent Females: A Large Case Series.

Purpose: The aim of the study was to review the incidence, presentation, and management of tubo-ovarian abscesses (TOA) in nonsexually active (NSA) adolescents.

Methods: A retrospective chart review was performed at a single children's hospital. We evaluated self-reported NSA females aged < 21 years diagnosed with TOA.

Ovarian conservation in management of pediatric gynecology malignancies.

Purpose Of Review: The purpose of this review is to outline ovarian conservation and fertility sparing surgery (FSS) in the management of the most common pediatric gynecology malignancies.

Recent Findings: Management of gynecologic malignancies has evolved to have a greater appreciation for adverse late effects from radical surgery, chemotherapy, and radiation. A growing body of literature has led to a paradigm shift toward ovarian conservation and FSS in the management of pediatric ovarian malignancies and genitourinary rhabdomyosarcoma (RMS).

Medical Child Abuse: A Case Presenting as Anogenital Bleeding of Unknown Origin in an Older Child.

Background: Medical child abuse is a challenging diagnosis to make, particularly in older children with unusual presenting symptoms.

Case: A 7-year-old child with complex medical history presented with anogenital bleeding of unknown origin. Extensive laboratory testing, imaging studies, and diagnostic procedures were negative for any etiology.

Inguinal Hyperhidrosis: Case Report of an Uncommon Cause of Vaginitis.

Background: Hyperhidrosis (excessive sweating) affects 1%-3% of the population. Primary focal hyperhidrosis most commonly affects the axilla, palms, and soles. There are few case reports of hyperhidrosis of the genital region, typically described as inguinal hyperhidrosis or Hexsel's hyperhidrosis.

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

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.

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

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.

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

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.

Pediatric Ovarian Torsion.

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.

Lipoblastoma of the Labia: A Case Report.

Background: Vulvar masses are rare in prepubertal girls. Lipoblastomas are benign adipose tumors that arise from embryonic white fat and occur almost exclusively in infants and children.

Case: An 18-month-old female infant presented with a 2-cm mobile mass in the left labia majora.