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. All patients with distal vaginal agenesis had greater than 3 cm lower vaginal agenesis, which would usually require complex vaginoplasty and use of postoperative stents. Given their immaturity and inability to use stents or dilators postoperatively or medical complexity, they subsequently underwent ultrasound-guided drainage of hematometrocolpos with interventional radiology to relieve pain symptoms, followed by menstrual suppression. The patients with obstructed uterine horns had complex medical and surgical histories requiring perioperative planning; they also underwent ultrasound-guided drainage of hematometra as a temporizing measure to manage acute symptoms. CONCLUSION: Patients presenting with symptomatic hematometrocolpos due to obstructive Müllerian anomalies might not be psychologically mature enough to undergo definitive complex reconstruction, which requires vaginal stent or dilator use postoperatively to prevent stenosis and other complications. Image-guided percutaneous drainage of symptomatic hematometrocolpos serves as a temporizing measure by offering pain relief until patients are ready to undergo surgical management and/or to allow time for complex surgical planning.

Pediatric, adolescent, and young adult breast and reproductive tumors.

Tumors of the breast and reproductive organs that occur in children, adolescents, and young adults (AYA) have different biological features and can present special challenges. Although prognosis for these tumors is generally favorable, the long-term effects of treatment can be debilitating. Treatments are often multimodal and may include surgery as well as chemotherapy and/or radiation, which can cause considerable distress and anxiety related to loss of femininity or masculinity, concern over future fertility, or sexual dysfunction. Thus, tumors of the reproductive organs in pediatric/AYA patients require special consideration of the treatment effects beyond the intended oncologic outcome. Multidisciplinary teams should be involved in their care and address issues of fertility, sexual dysfunction, and psychosexual concerns before treatment begins. This review addresses histology, risk factors, prognosis, staging and treatment of gynecologic, breast and testicular cancers in pediatric and AYA patients.

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. To facilitate the recognition and management of these conditions, we provide a review of Mullerian anomalies including the embryology, classifications, syndromes, evaluation, and treatments with attention to their urologic applicability.

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. PARTICIPANTS: Female patients less than 21 years of age diagnosed with vaginal YST. RESULTS: Two cases of vaginal YST at our institution are outlined. Both patients presented within the first 2 years of life with vaginal bleeding and were treated successfully with chemotherapy alone. After review of the literature, 137 cases of vaginal YST were found. The mean age at diagnosis was 11 months, and all patients presented with vaginal bleeding. Before 2000, more radical treatments were pursued, and 40% resulted in death. Since the year 2000, treatment has shifted toward chemotherapy and more conservative surgical management, with 51% of vaginal YST cases treated with chemotherapy alone with 92% of patients alive at time of publication. CONCLUSION: Our cases contribute to the limited literature demonstrating the efficacy of conservative management of rare cases of vaginal YST with chemotherapy alone. This case series and review of the literature provide mounting evidence that vaginal YST should be in the differential diagnosis in young girls with vaginal tumors, and conservative management of vaginal YST has excellent outcomes.

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.

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. Postoperative ovarian volumes were compared to established radiologic standards. SETTING: Large tertiary care academic children's hospital in Atlanta, GA. PARTICIPANTS: Female patients less than 21 years old who underwent unilateral oophorectomy. MAIN OUTCOME MEASURES: Ovarian volumes measured on postoperative ultrasounds. RESULTS: A total of 93 patients met inclusion criteria for this study. Serial ultrasounds were performed in slightly more than half of the patients (n = 48, 51.6%), totaling 193 postoperative ovarian volumes. The average age of oophorectomy was 10.8 years. Prior to surgery, the majority of patients presented with abdominal pain (n = 51, 54.8%) or pelvic mass (n = 51, 54.8%), and most (n = 77, 82.8%) had benign final pathology. Ovarian volumes were compared to 4 published radiologic ultrasound standards. More than 62.2% of ovarian volumes from girls who had previously had unilateral oophorectomy were larger than age-matched standard ovarian volumes. CONCLUSION: Ovarian enlargement occurs in the contralateral ovary following unilateral oophorectomy in the pediatric and adolescent population. This supports the concept of compensatory ovarian hypertrophy. This knowledge provides valuable information for interpretation of radiologic images in young female individuals who have undergone oophorectomy, and can assist with counseling on the risk of adnexal complications due to ovarian hypertrophy after unilateral oophorectomy.

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. We present the case of a 17-year-old female who while receiving cancer treatment, had unintentional exposure to capecitabine and temozolomide during the first trimester of pregnancy, and subsequently delivered a healthy infant.

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. DESIGN: As a quality improvement measure in perioperative care, an ERAS protocol including preoperative, intraoperative, and postoperative components and a follow-up patient telephone call for pain assessment was implemented for all intra-abdominal gynecologic procedures. A retrospective study on implementation of ERAS components, outcomes, and patient satisfaction was then performed in participants meeting inclusion criteria. SETTING: Large academic children's hospital. PARTICIPANTS: Patients <25 years of age who underwent laparoscopic (LSC) or open abdominal (XLAP) gynecologic surgery using an ERAS protocol by the PAG service over a 12-month period. INTERVENTIONS: An ERAS protocol including preoperative, intraoperative, and postoperative components and follow-up patient telephone call for pain assessment was implemented for all major gynecologic surgeries performed by the PAG service. MAIN OUTCOME MEASURES: Patient satisfaction with the perioperative ERAS protocol along with components including pain management, narcotic use, LOS, RTS, and postoperative complications for various intra-abdominal gynecologic procedures. RESULTS: A total of 40 participants met inclusion criteria for the study. Thirty-four (85%) participants underwent LSC procedures and six (15%) underwent XLAP. Of the LSC patients, 95% were discharged on postoperative day 0, and all XLAP patients and one LSC patient were discharged on postoperative day 1. In all, 95% of patients were discharged from the hospital requiring only non-narcotic ERAS medications. There were no readmissions or postoperative complications. All patients were satisfied with their postoperative pain control at their follow-up telephone call and clinic visit. CONCLUSION: Implementation of a pediatric-specific ERAS protocol in children and adolescents undergoing gynecologic surgery is feasible and safe, and leads to less narcotic use without an increase in complications or decrease in patient satisfaction.

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. Demographics, presenting symptoms, medical history, laboratory studies, imaging, and treatment were reviewed. RESULTS: Sixteen NSA females met inclusion criteria, with one patient presenting with two separate TOAs. Mean age at diagnosis was 14.6 ± 1.8 years; all were menarchal. Presenting symptoms included abdominal pain (88%), fever (76%), and vomiting (53%). Eleven patients (65%) received transabdominal pelvic ultrasound, 2 (12%) had magnetic resonance imaging of pelvis, and 11 (65%) had computed tomography of abdomen/pelvis. All patients had negative gonorrhea and chlamydia testing. Thirteen (76%) had comorbid disease including obstructed hemivagina ipsilateral renal agenesis, active or recent appendicitis, or genitourinary tract anomalies. Sixteen cases underwent drainage via interventional radiology or surgery. Twelve cases (71%) had TOA fluid cultured; five (29%) grew Escherichia coli species. All cases received intravenous antibiotics, and 88% of cases continued outpatient oral antibiotics. CONCLUSIONS: Although uncommon in NSA females, patients with underlying comorbidities may be at increased risk for TOA formation. Providers should consider the diagnosis of TOA even in NSA females.

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). The role of minimally invasive surgery is still being explored but guidelines support this approach as a viable option for early stage ovarian cancers when performed by skilled surgeons. Research continues to address settings in which adjuvant chemotherapy for ovarian cancer can be avoided. Treatment for genitourinary RMS has transitioned from radical surgery to a conservative multimodal approach with FSS, systemic chemotherapy, and targeted radiation. SUMMARY: FSS and ovarian conservation are emerging concepts in the management of gynecologic malignancies in young women. Conservative surgical approaches and avoidance of gonadotoxic chemotherapy and radiation to minimize acute and long-term complications while maintaining efficacy are important considerations. Fertility preservation techniques continue to evolve and offer young women the opportunity for future fertility and preservation of ovarian function.

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. Forensic testing confirmed the blood in her underwear was a genetic match to the patient. Trial separation from the mother was diagnostic and therapeutic in this case. SUMMARY AND CONCLUSION: Older children who are victims of medical child abuse might present in a variety of ways, and might even collaborate with the perpetrator in falsifying symptoms. It is important to keep medical child abuse on the differential when the patient's symptoms and work-up do not match.

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. CASE: A 17-year-old girl presented with 3 years of copious, clear "vaginal" discharge causing significant emotional distress. After extensive gynecologic and urologic workup was negative, further review of her history was notable for excessive axillary sweating. Inguinal hyperhidrosis was suspected and she was treated with topical aluminum chloride hexahydrate with complete resolution of her symptoms. SUMMARY AND CONCLUSIONS: Inguinal hyperhidrosis, compared with other sites, is not widely described in the literature. Awareness of inguinal hyperhidrosis is important because it causes significant social embarrassment but is a treatable condition.

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. 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.

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. 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.

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. Imaging and examination revealed normal prepubertal gynecologic structures and a 4.5 cm fatty mass in the left labia. Surgical excision revealed a 3.8 cm well circumscribed adipose tissue mass consistent with maturing lipoblastoma on microscopic examination. Cytogenetic analysis revealed 79,XXX [7]/46,XX[13], a near-triploid clone. SUMMARY AND CONCLUSION: The differential diagnosis of vulvar masses in children should include lipoblastoma. Although preoperative imaging has limited ability to differentiate lipomatous tumors, magnetic resonance imaging is the modality of choice for evaluating tumor extension and for surgical planning. Treatment is complete surgical excision with close follow-up for at least 5 years because of the high recurrence rate.

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. 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.

First contact: the intersection of demographics, knowledge, and appraisal of treatment at the initial infertility visit.

OBJECTIVE: To determine the impact of the initial infertility visit on treatment-related knowledge, patient anxiety, and appraisals of treatment. DESIGN: Prospective survey. SETTING: Academic medical center. PATIENT(S): Two hundred thirty-four English-speaking women aged 18-50 years attending their first infertility visit. INTERVENTION(S): Participants completed a survey assessing health literacy, knowledge, anxiety, and appraisals of the treatment process before and after their infertility visit. MAIN OUTCOME MEASURE(S): Knowledge of infertility and treatment and anxiety and appraisal scores. RESULT(S): Most participants were white and earned >$100,000/year and had at least a college education. Baseline knowledge of reproductive anatomy, assisted reproductive technology (ART), and fertility factors was modest but improved after the initial visit. Factors associated with higher knowledge included higher education and income, white or Asian ethnicity, and English as a primary language. Patient appraisals of treatment represented by the positive (Challenge) and negative (Threat and Loss) subscale scores on the Appraisal of Life Events (ALE) scale changed over time Negative appraisals of treatment and anxiety scores decreased and positive appraisals of treatment increased after the initial visit. Lower knowledge was associated with higher positive appraisal scores; lower health literacy was associated with higher anxiety and appraisal scores (positive and negative) after the visit. Black women had higher Challenge scores compared with white and Asian women. Hispanic women had higher anxiety scores than non-Hispanic women. CONCLUSION(S): Infertility patients have modest baseline knowledge of fertility and infertility treatment. The initial infertility visit can improve this knowledge and decrease both negative appraisals of treatment and anxiety levels. Differences in knowledge and appraisal were seen across ethnic groups and other demographic variables. Physicians should individualize patient counseling to improve patients' knowledge and provide realistic treatment expectations while also reducing patient anxiety.