Recurrence Rates for Pediatric Benign Ovarian Neoplasms.

STUDY OBJECTIVE: To examine the recurrence rates of pediatric benign ovarian neoplasms METHODS: A retrospective review of females up to 21 years of age who underwent surgery for a benign ovarian neoplasm at 8 pediatric hospitals from January 2010 through December 2016 was conducted. Data include primary operation details, follow-up imaging, and reoperation details. RESULTS: Four hundred and twenty-six females were included in our cohort, with a median age of 15 years at the time of the primary operation. Of the patients, 69% had a mature teratoma, 18% had a serous cystadenoma, and 8% had a mucinous cystadenoma. Two-thirds of patients underwent ovarian-sparing surgery. There were 11 pathologically confirmed recurrences (2.6%) at a median follow-up of 12.8 months. The pathologically confirmed recurrence was 10.5 per 100 person-months at 12 months (SE = 5.7) for mucinous cystadenomas and 0.4 months (SE = 0.4) for mature teratomas (P = .001). For half of the patients, the pathologically confirmed recurrences occurred by 12.8 months, and for 75%, they occurred by 23.3 months. There were no differences in reoperation or recurrence on the basis of initial procedure (ovary-sparing surgery vs oophorectomy). CONCLUSION: We measured the pathologically confirmed recurrence rate for pediatric benign ovarian neoplasms in a large cohort. Oophorectomy was not protective against recurrence. Mucinous cystadenomas were at a greater risk of pathologically confirmed recurrence.

Failure of Operative Therapy after Ovarian-Sparing Surgery for Pediatric Benign Ovarian Neoplasms: A Retrospective Review.

STUDY OBJECTIVE: To evaluate failure of initial operative therapy (incomplete tumor removal) of ovarian-sparing surgery for pediatric benign ovarian neoplasms. METHODS: A retrospective review of patients up to 21 years of age who underwent ovarian-sparing surgery for a benign ovarian neoplasm from 2010 to 2016 at 8 pediatric hospitals was conducted. Failure of initial operative therapy is defined as a radiologically suspected or pathologically confirmed ipsilateral lesion with the same pathology as the primary neoplasm within 12 weeks of the initial operation. RESULTS: Forty patients received imaging within 12 weeks of their primary operation. Sixteen (40%) patients had a radiologically identified ovarian abnormality ipsilateral to the primary lesion, and 5 patients were suspected to have the same lesion as their primary neoplasm. Three of the 5 patients (7.5%) underwent reoperation with pathologic confirmation of the same lesion, resulting in a pathologically confirmed failure of therapy rate of 7.5%. The other 2 patients had serial imaging that subsequently demonstrated no recurrence with lesion resolution. Age, race/ethnicity, laparoscopy vs laparotomy, presence of torsion, pathology, size of lesion, and surgeon specialty were not associated with failure of therapy. CONCLUSION: In most patients who received imaging within 12 weeks of the primary operation for resection of a benign ovarian neoplasm, ovarian-sparing surgery was successful in complete tumor removal, with a low failure of therapy rate. Selected patients with suspected failure of therapy on initial imaging could be serially monitored to determine the need for repeat surgical intervention.

Recommendations for Postoperative Surveillance of Pediatric Benign Ovarian Neoplasms.

STUDY OBJECTIVE: To assess postoperative management of pediatric patients with benign ovarian neoplasms, to develop recommendations for postoperative care. DESIGN: A retrospective cohort study. SETTING: Eight pediatric hospitals in the midwestern United States. PARTICIPANTS: Patients up to 21 years of age who underwent surgery for a benign ovarian neoplasm between January 2010 and December 2016 were included. INTERVENTIONS: No prospective interventions were evaluated. MAIN OUTCOME MEASURES: Main outcome measures included postoperative imaging findings, recurrence rates, reoperation rates, and the timing of the aforementioned results. RESULTS: A total of 427 patients met inclusion criteria. After the index surgery, 155 patients (36%) underwent a routine imaging study. Among those with routine imaging, abnormalities were noted in 48 patients (31%); 7 went on to have reoperation (5%), and no malignant pathologies or torsion were identified. Excluding the 7 patients who went on to have a reoperation as a result of routine imaging, 113 patients developed symptoms postoperatively and underwent imaging as a result (27%, 113/420). Abnormalities were noted in 44 (10%); 15 of these patients underwent reoperation (4%), among them 2 with malignancies and 3 with torsion. Of these 44 patients, 23 had initially undergone routine imaging and subsequently went on to have symptomatic imaging, with 17% (4/23) undergoing reoperation. CONCLUSIONS: Routine imaging did not identify malignancy; most lesions identified on routine imaging were incidental findings. Although the study was not powered to appreciate a statistically significant difference, patients with malignancy or torsion were identified in the symptomatic group. This suggests no benefit from routine imaging, and supports symptomatic imaging postoperatively to minimize costs and patient/family burden.

Pelvic Castleman's Disease Presenting as an Adnexal Mass in an Adolescent.

BACKGROUND: Castleman disease (CD) is a rare lymphoproliferative disorder that might present as an adnexal mass. We report a case of pelvic CD in an adolescent girl who presented with abdominal pain. CASE: A 13-year-old girl presented with severe abdominal pain, nausea, and vomiting, and was found to have a solid adnexal mass. Repeat imaging revealed the mass to be retroperitoneal and in the left pelvic side wall. She underwent surgical removal via an open retroperitoneal approach, and pathology revealed CD, hyaline vascular variant subtype. SUMMARY AND CONCLUSION: Pelvic CD should be considered in the differential diagnosis for an adnexal mass in a young woman. Surgical planning is critical because of the possibility of extension and mass effect. Most pelvic CD is unicentric, hyaline vascular variant subtype, and does not recur after surgical removal.

The Role of Patient Advocacy and the Declining Rate of Clitoroplasty in 46,XX Patients With Congenital Adrenal Hyperplasia.

Prior to the 1990s, most 46,XX infants with clitoromegaly secondary to congenital adrenal hyperplasia were treated with feminizing genitoplasty to make their cosmetic appearance congruent with their genotypic sex. A 2006 consensus statement for the management of intersex disorders accepted input from patient advocates and did not support purely cosmetic surgery for clitoromegaly. This study examined the extent to which the desired change was implemented in practice. Retrospective chart review was performed at a single Midwestern tertiary care medical center for patients born between 1979 and 2013. Of 45 virilized patients, 40 had clitoromegaly and 39 had urogenital sinus or posterior labial fusion. Twenty-seven (67.5%) patients underwent clitoroplasty and 33 (84.6%) underwent perineoplasty, including vaginoplasty, urethroplasty, imperforate vagina repair, and/or posterior labial fusion repair. There was a linear decline in the rate of clitoroplasty over time for the patient cohort. This study demonstrates the power of patient advocacy to improve medical practice.

Genital injuries acute evaluation and management.

Genital trauma may result in external injuries to the labia, vulva or vagina, urethra and anus and internal injuries to the bony pelvis, bladder, bowels and reproductive organs. Worldwide, the most common cause of genital trauma in reproductive age women is injury sustained during childbirth, but in this chapter we will focus on accidental genital injuries as well as those arising from sexual violence, and female genital mutilation. While genital injuries alone rarely result in death; if not properly managed, chronic discomfort, dyspareunia, infertility, or fistula formation may result. Clinicians need to be able to recognize these injuries and provide initial management, and assure that the patient's mental, emotional and physical needs are addressed.

Risk association of congenital anomalies in patients with ambiguous genitalia: A 22-year single-center experience.

BACKGROUND: Ambiguous genitalia refers to a form of differences of sex development (DSD) wherein the appearance of the external genitalia is atypical. This rare condition presents challenges in decision-making and clinical management. Review of historical data may reveal areas for clinical research to improve care for patients with ambiguous genitalia. OBJECTIVE: This chart review was performed to identify patients with ambiguous genitalia, and to classify them as having 46,XX DSD, 46,XY DSD, or sex chromosome DSD. Within these categories, we looked at establishment of specific diagnoses, type and frequency of other congenital anomalies and neoplasms, and gender assignment, as well as incidence of gender reassignment and transition. METHODS: We performed a retrospective chart review of patients diagnosed with DSD conditions from 1995 to 2016 using ICD9 codes. For the purpose of this study, review was limited to individuals assessed to have neonatal "ambiguous genitalia" or "indeterminate sex." RESULTS: Review identified 128 patients evaluated for ambiguous genitalia from 22 years of experience (Figure). Approximately half of these (53%) had 46,XY karyotype, 35% had 46,XX, and the remaining 12% had sex chromosome aberrations. Diagnostic rate for 46,XX DSD was higher at 64%, all of which were congenital adrenal hyperplasia, while diagnostic rate for 46,XY DSD was 11.7% for a molecularly confirmed diagnosis and 24% if clinical diagnoses were included. The most common anomalies included cardiac anomalies in 28/128 (22%), skeletal anomalies in 19/128 (15%), and failure to thrive or growth problems in 19/128 (15%). Additional congenital anomalies were found in 53 out of 128 patients (41%). There were three reported neoplasms in this group: gonadoblastoma, hepatoblastoma, and myelodysplastic syndrome with monosomy 7. Gender assignment was consistent with chromosomes in approximately 90% of XX and XY patients. There were three recorded gender reassignments or transitions. DISCUSSION: Diagnostic rate for ambiguous genitalia is low, especially in 46,XY DSD. Most neonates were assigned gender consistent with their chromosomes. Given the high rate of associated anomalies, screening for cardiac or other anomalies in patients with ambiguous genitalia may be beneficial. CONCLUSION: Patients with ambiguous genitalia often have additional congenital anomalies. Establishment of a specific diagnosis is uncommon in 46,XY patients. A few patients have gender reassignment outside of the newborn period. Ongoing collection of clinical data on this population may reveal new information regarding long-term health, quality of life, and establishment of more diagnoses with improved molecular techniques.

Surgical Management of Benign Adnexal Masses in the Pediatric/Adolescent Population: An 11-Year Review.

STUDY OBJECTIVE: The purpose of this study was to compare ovarian conservation rates and surgical approach in benign adnexal surgeries performed by surgeons vs gynecologists at a tertiary care institution. DESIGN: A retrospective cohort review. SETTING: Children's and adult tertiary care university-based hospital. PARTICIPANTS: Patients 21 years of age and younger who underwent surgery for an adnexal mass from January 2003 through December 2013. INTERVENTIONS: Patient age, demographic characteristics, menarchal status, clinical symptoms, radiologic imaging, timing of surgery, surgeon specialty, mode of surgery, rate of ovarian conservation, and pathology were recorded. Patients were excluded if they had a uterine anomaly or pathology-proven malignancy. MAIN OUTCOME MEASURES: The primary outcome was the rate of ovarian conservation relative to surgical specialty; secondary outcome was surgical approach relative to surgical specialty. RESULTS: Of 310 potential cases, 194 met inclusion criteria. Gynecologists were more likely than surgeons to conserve the ovary (80% vs 63%; odds ratio, 2.28; 95% confidence interval, 1.16-4.48). After adjusting for age, body mass index, mass size, and urgency of surgery, the difference was attenuated (adjusted odds ratio, 1.84; 95% confidence interval, 0.88-3.84). Surgeons and gynecologists performed minimally invasive surgery at similar rates (62% vs 50%; P = .11). A patient was more likely to receive surgery by a gynecologist if she was older (P < .001) and postmenarchal (P = .005). CONCLUSION: Results of our study suggest that gynecologists are more likely to perform ovarian-conserving surgery. However, our sample size precluded precise estimates in our multivariable model. Educational efforts among all pediatric and gynecologic surgeons should emphasize ovarian conservation and fertility preservation whenever possible.

Physical examination of the female cancer patient with sexual concerns: What oncologists and patients should expect from consultation with a specialist.

Answer questions and earn CME/CNE Sexual concerns are prevalent in women with cancer or cancer history and are a factor in patient decision making about cancer treatment and risk-reduction options. Physical examination of the female cancer patient with sexual concerns, regardless of the type or site of her cancer, is an essential and early component of a comprehensive evaluation and effective treatment plan. Specialized practices are emerging that focus specifically on evaluation and treatment of women with cancer and sexual function problems. As part of a specialized evaluation, oncologists and their patients should expect a thorough physical examination to identify or rule out physical causes of sexual problems or dysfunction. This review provides oncology professionals with a description of the physical examination of the female cancer patient with sexual function concerns. This description aims to inform anticipatory guidance for the patient and to assist in interpreting specialists' findings and recommendations. In centers or regions where specialized care is not yet available, this review can also be used by oncology practices to educate and support health care providers interested in expanding their practices to treat women with cancer and sexual function concerns. CA Cancer J Clin 2016;66:241-263. © 2016 American Cancer Society.

Case Series: Vaginal Rupture Injuries after Sexual Assault in Children and Adolescents.

BACKGROUND: Vaginal rupture after sexual assault is a rare but life-threatening occurrence requiring prompt recognition and treatment. Herein, we describe four such cases in children. Our purposes are to increase clinicians' awareness of the physical trauma that a sexual abuse victim can suffer and increase recognition that these victims require immediate trauma services. CASES: Each patient had obvious hymenal and vaginal lacerations with a vaginal apical rupture injury and secondary acute blood loss. None of the four victims sustained infectious sequelae. SUMMARY AND CONCLUSION: Providers should have a low threshold for managing sexual abuse victims as trauma cases when they have obvious hymenal and vaginal lacerations and genital bleeding, proceeding expeditiously to examination using general anesthesia when appropriate.

Renal Remnant Presenting as a Large Retroperitoneal Mass in a Teenage Girl.

BACKGROUND: Adolescents commonly present with a symptomatic pelvic mass. The vast majority of these masses are benign and gynecological in nature. Nonetheless, it is important to consider less common etiologies including retroperitoneal and enteric cysts. CASE: A 17-year-old female patient with chronic pelvic pain due to a large 9-cm cystic pelvic mass underwent laparoscopy. Operative findings indicated a retroperitoneal mass, which was excised in a minimally invasive fashion. Final pathology was suggestive of a remnant of a developmental renal anomaly. SUMMARY AND CONCLUSION: This case highlights the importance of a broad differential when considering etiologies of a cystic pelvic mass. Clinical diagnosis and management are often challenging. Therefore, it is important to increase awareness of this diagnosis within the gynecological community.

Invasive Ductal Carcinoma in a 46,XY Partial Androgen Insensitivity Syndrome Patient on Hormone Therapy.

BACKGROUND: The hormonal management of patients with androgen insensitivity can be challenging. CASE: An illustrative case is presented of a newborn with ambiguous genitalia who was raised female. She was diagnosed as 46,XY Disorder of Sexual Development with partial androgen insensitivity. To induce puberty, conjugated equine estrogens were administered beginning at age 12. At age 13, she instead began taking combined oral contraceptives for maternal concerns about height and continued taking them for social reasons. Invasive ductal carcinoma was diagnosed at age 27, and the patient was treated with chemotherapy, radiation therapy, bilateral mastectomies, and endocrine therapy. SUMMARY AND CONCLUSION: The current literature is reviewed, and hormonal management and other risks for breast cancer are discussed.

Massive Hydronephrosis from Ureteropelvic Junction Obstruction Masquerading as a Paratubal Cyst in an 11-Year-Old Girl.

BACKGROUND: The differential diagnosis of a cystic pelvic mass in an adolescent girl is broad, and includes gastrointestinal, urologic, and gynecologic conditions. CASE: A premenarchal 11-year-old girl presented with abdominal pain. On transabdominal ultrasonography, abutting the lateral side of the left ovary was a large mass with an appearance compatible with a large paratubal cyst measuring 16.7 × 11.9 cm. On exploratory laparotomy, the patient's uterus, tubes and ovaries were normal and a massively dilated and displaced left kidney due to an ureteropelvic junction obstruction was ultimately diagnosed. SUMMARY AND CONCLUSION: A cystic pelvic mass in an adolescent girl may not always be of ovarian or müllerian origin. Urinary tract obstruction is often silent; an incidental finding of hydronephrosis on ultrasonography may be the first clue of the possibility of ureteropelvic junction obstruction as the underlying diagnosis. It is always best to know which organ system is involved prior to surgery, so that the correct surgical team is present and the correct surgical approach is utilized.

Rope swing injuries resulting in vulvar trauma.

BACKGROUND: River tree rope swings are popular for recreation along inland lakes and rivers, but not without hazard. In a comprehensive review of injuries related to rope swings, genital injuries accounted for nearly 3 percent. CASE: We describe significant genital injuries, with laceration and avulsion of the labia and a large vulvar hematoma in 2 young women as a consequence of rope swing use. SUMMARY AND CONCLUSIONS: Visitors to inland waterways need to be cognizant of the hazards of rope swings and health care professionals should consider this mechanism of injury when confronted with vulvar trauma.

The course of lichen sclerosus diagnosed prior to puberty.

STUDY OBJECTIVE: To help determine the long-term course of girls diagnosed with lichen sclerosus before puberty. DESIGN: Retrospective chart review and follow-up interview. SETTING: Washington University pediatric gynecology and dermatology clinics. PARTICIPANTS: Premenarchal girls diagnosed with lichen sclerosus from 1989-2010. INTERVENTIONS: Telephone interview. MAIN OUTCOME MEASURES: Resolution of symptoms, specifically pain and/or pruritus. RESULTS: Follow-up was available for 36 premenarchal girls. The mean age at lichen sclerosus (LS) diagnosis was 7 years (range: 3-14 years). The mean duration of follow-up was 5.3 years (range: 2 months-15 years). Treatment with topical steroids (primarily 0.05% clobetasol propionate ointment) resulted in improvement in symptoms within an average of 14 weeks (range: 2 weeks-2 years) in 33 girls. Eighty-three percent of patients (n = 30) experienced remission after initial treatment. Sixteen patients reported relapses requiring an average of 3.1 years of intermittent maintenance therapy. The mean length of remission to date was 3.6 years (range 1 months-10 years). 72% of patients reported remission at the time of the phone interview. Of note, 7 out of 9 patients in our study who continue to report symptoms are still premenarchal. One postmenarchal patient was asymptomatic but had signs of LS on physical exam. CONCLUSION: The prognosis and long term course of LS diagnosed prior to puberty is unclear. Although remission may occur prior to menarche in some cases, once children reach menarche with active disease, complete remission may be less likely. Treatment duration of LS in our study had a wide range, but 3 months appears to be adequate for most patients to obtain remission.

The auto-amputated adnexa: a review of findings in a pediatric population.

STUDY OBJECTIVE: To quantify our experience and that of the literature with diagnosis and management of the auto-amputated adnexa in a pediatric population. DESIGN: Case series and literature review. SETTING: Tertiary care medical center. PARTICIPANTS: Case series of pediatric patients (<18 years of age) with surgically documented adnexal auto-amputation collected from our medical center and the literature. INTERVENTIONS: None. MAIN OUTCOME MEASURE: Auto-amputated adnexa. RESULTS: In addition to the 3 cases discussed from our institution, 91 cases of auto-amputated adnexa were identified in the literature dating back to 1943, for a total of 94 cases. Forty-nine percent (46/94) of the cases involved girls in a pediatric population (<18 years of age). Of these, the majority (n = 26) were identified in a subgroup of girls who were diagnosed with an adnexal cyst by antenatal ultrasound. Most of these neonates were asymptomatic at birth or had a palpable abdominal mass (n = 6) and at the time of surgical exploration were found to have an auto-amputated adnexa. 34 out of 46 cases were analyzed in detail. The right adnexa were involved in 56% of the cases. The most common presenting complaint verbalized by the older girls was pain; however, 8 cases were identified in asymptomatic girls undergoing unrelated diagnostic testing. CONCLUSION: The auto-amputated adnexa is a rare finding in the pediatric population, but it must be considered as a possible explanation for the incidental finding of absence of the fallopian tube or ovary in the subgroup of patients who undergo surgery for any reason. Patients with an antecedent history of pelvic pain either chronic or intermittent in nature may be diagnosed with torsion or less frequently auto-amputation of the adnexa. A fetal "pelvic mass" or "ovarian cyst" may predispose the adnexa to torsion and subsequent auto-amputation either in-utero or post-delivery. Many of these antenatally diagnosed cysts and even subsequent auto-amputations are completely asymptomatic, however, and do not compromise fertility assuming the contralateral adnexa are normal. Thus expectant management is appropriate for small (less than 4 cm), asymptomatic simple cysts and even suspected auto-amputated adnexa in an asymptomatic patient.

Acute genital ulcers in nonsexually active young girls: case series, review of the literature, and evaluation and management recommendations.

Acute genital ulcers rarely occur in nonsexually active young girls. When present, they can cause significant physical and emotional distress for the patient and her parents, and prompt an evaluation for sexual abuse and sexually transmitted diseases. With this review, we aim to further characterize acute genital ulcers in nonsexually active young girls by reviewing the medical records of patients with this disorder and to offer an approach to the diagnosis, evaluation, and treatment of acute genital ulcers based on our understanding and knowledge of this condition. We retrospectively review our understanding and knowledge of acute genital ulcers in nonsexually active girls at a pediatric hospital. A review of the recent literature on acute genital ulcers and a multidisciplinary approach to the diagnosis, evaluation, and treatment of acute genital ulcers are also presented. Twelve patients presented with acute genital ulcers, 11 of which were hospitalized for evaluation and pain management. Extensive work-up failed to reveal a specific infectious or autoimmune etiology in all but one patient, who was diagnosed with acute mycoplasma pneumonia. Acute genital ulcers in nonsexually active young girls likely represent a form of idiopathic vulvar aphthosis. Evaluation of a first episode of acute genital ulcers with mild prodromal symptoms should be limited. Treatment consists primarily of supportive care and symptom relief.

Genital trauma in prepubertal girls and adolescents.

PURPOSE OF REVIEW: To look critically at recent research articles that pertain to children and adolescents who present with genital injuries. RECENT FINDINGS: Emerging evidence supports links to long-term psychological sequelae of child sexual abuse. Parents should be educated to instruct their children regarding types of child abuse and prevention. 'Medicalization' of female genital mutilation (FGM) by health providers, including 'cutting or pricking', is condemned by international organizations. SUMMARY: Genital injuries whether accidental or intentional need to be reported with standardized terminology to allow for comparisons between reported outcomes. Motor vehicle accidents associated with pelvic fractures may result in bladder or urethral trauma. Adverse long-term psychosocial behaviors may be sequelae of child sexual abuse. FGM is willful damage to healthy organs for nontherapeutic reasons, and a form of violence against girls and women. Healthcare providers should counsel women suffering from the consequences of FGM, advise them to seek care, counsel them to resist reinfibulation, and prevent this procedure from being performed on their daughters.

Anti-NMDA-receptor encephalitis: an adolescent with an ovarian teratoma.

BACKGROUND: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis has been reported to be associated with ovarian teratomas. In many patients, surgical excision has resulted in improvement. CASE: A previously healthy 14-year-old girl presented with confusion which later evolved into a comatose state. Imaging revealed an adnexal mass which was surgically removed and confirmed to be a mature teratoma. Her cerebral spinal fluid was positive for antibodies to NR1/NR2 heteromers of NMDA receptors. COMMENTS: Cystic teratomas are one of the most common benign ovarian lesions in children and young women. While an association of ovarian teratomas and paraneoplastic encephalitis is published in the neurologic literature, this association needs to be clarified for the practicing gynecologist who may be asked to consult on these patients.

Novel use of a tracheobronchial stent in a patient with uterine didelphys and obstructed hemivagina.

OBJECTIVE: To describe a novel use for a tracheobronchial stent to maintain patency after vaginal septum excision in a patient with an obstructed hemivagina and uterine didelphys. DESIGN: Description of a novel technique. SETTING: University-affiliated children's hospital. PATIENT(S): One patient with an obstructed hemivagina and uterine didelphys who presented with hematometria and hematocolpos. INTERVENTION(S): To maintain patency and decrease stenosis risk after vaginal septum excision, a coated tracheobronchial stent was deployed and left in place for 6 weeks. MAIN OUTCOME MEASURE(S): To evaluate ease of stent placement and removal, reepithelialization and patency of the neovagina, and postoperative assessment of pain and recurrent obstruction. RESULT(S): The tracheobronchial stent was easily positioned and deployed with vaginoscopic guidance. Six weeks later it was removed without any tissue ingrowth or granulation tissue noted. The vaginal walls were nicely epithelialized. Twelve months postoperatively, the patient remained pain free with regular cycles and no evidence of obstruction or abnormality on ultrasound. CONCLUSION(S): Use of a coated tracheobronchial stent to maintain patency after septum excision in a patient with an obstructed hemivagina presents a safe, easy, and effective option to diminish stenosis risk and avoid infectious complications or hysterectomy.