Hormones and their Structural and Functional Effects on the Brain: How Can We Change our Practice Moving Forward?

Is hormone treatment an invasive procedure? In this paper, we discuss aspects related to the choice of treating disorders of sex development (DSD) using hormones. Specifically, we focus on some of the challenging issues related to this treatment and the need to establish a standard of care for the use of hormone therapy in this patient population. The objectives of this paper are to: 1) Enhance understanding of the uncertainties in the decision-making process regarding hormonal interventions to treat patients with DSD. 2) Recognize that the effects of hormonal interventions might require a consent process similar to that applied for surgical procedures. 3) Emphasize the need to establish treatment algorithms that could form the basis of a standard of care for this patient population.

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.

Evidence-Based Management of Patients with 45,X/46,XY Gonadal Dysgenesis and Male Sex Assignment: from Infancy to Adulthood.

45,X/46,XY gonadal dysgenesis is a disorder of sexual differentiation with a wide clinical presentation, ranging from Turner-like females to individuals with genital ambiguity to azoospermic but otherwise normal-appearing males. Hence, patients can be assigned female or male sex. Female patients are managed according to the Turner Syndrome Guidelines, whereas males are managed on a case-by-case basis. Male patients present with multiple medical challenges: undervirilization, hypogonadism, gonadoblastoma risk, and short stature. Many require surgeries and hormonal treatments that are time-sensitive and irreversible. Nonetheless, these therapeutic decisions are made without evidence-based guidelines. This review describes the medical concerns and possible interventions in male patients with 45,X/46,XY dysgenesis for each stage of development. Interventions should be addressed within a patient-centered framework by a multidisciplinary team and after thorough discussion with the family. We use the GRADE system to appraise the existing evidence and provide recommendations based on the available evidence.

Surgical Management of OHVIRA and Outcomes.

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

Rates of Intrauterine Device Expulsion Among Adolescents and Young Women.

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

A Rare Case of Vulvar Superficial Angiomyxoma in a Pediatric Patient.

BACKGROUND: Superficial angiomyxoma (SAM) is a rare, benign cutaneous tumor. Originally described as a component of Carney complex, it is now recognized as a sporadic condition. CASE: A 7-year-old girl was referred for management of a 2.5-cm mass arising from the right labia majora. Key pathologic features included lobules of spindle-shaped cells in a myxoid matrix and prominent neutrophilic infiltrate. The cells were positive for CD34 and negative for desmin, progesterone receptor, and estrogen receptor staining. SUMMARY AND CONCLUSION: This case is that of the youngest described patient with vulvar SAM. Patients should be carefully examined for manifestations of Carney complex to avoid potentially life-threatening complications. It is critical to distinguish SAM from aggressive angiomyxoma. The patient was referred to genetics and will be followed for local recurrence.

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

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.

Intrauterine Devices: Effective Contraception with Noncontraceptive Benefits for Adolescents.

Although adolescent pregnancy and birth rates have been declining since the early 1990s, the rate of intrauterine device (IUD) use in adolescents remain low. IUDs are a highly effective contraceptive method with a failure rate of less than 1%. There are currently 5 IUDs available and marketed in the United States: the nonhormonal copper-containing IUD (Paragard Copper T380A; Ortho-McNeil) and 4 hormonal levonorgestrel-releasing intrauterine systems (LNG-IUDs). IUDs can be used in adolescents, and the LNG-IUD has many noncontraceptive benefits including the treatment of heavy menstrual bleeding, dysmenorrhea, pelvic pain/endometriosis, and endometrial hyperplasia/endometrial cancer. In addition, the LNG-IUD is an effective tool for suppression of menses.

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.

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.

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

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.

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.

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

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.

Regression of Adenomyosis on Magnetic Resonance Imaging after a Course of Hormonal Suppression in Adolescents: A Case Series.

STUDY OBJECTIVE: To demonstrate that adenomyosis is a rare cause of dysmenorrhea or chronic pelvic pain (CPP) in the adolescent population that can be identified with magnetic resonance imaging (MRI) and to report resolution of adenomyosis by MRI after a course of hormonal suppression in 4 adolescents. DESIGN, SETTING, AND PARTICIPANTS: Retrospective case series of 4 adolescents with adenomyosis on pelvic MRI at Texas Children's Hospital. INTERVENTIONS: Continuous oral contraceptive (COC) therapy or leuprolide acetate. MAIN OUTCOME MEASURES: Lesions on pelvic MRI after treatment. METHODS: We reviewed medical records of 4 adolescents with CPP and adenomyosis on T2-weighted pelvic MRI. All patients had initial diagnostic pelvic MRI and then definitive hormonal intervention. Repeat imaging was obtained after a symptom-free interval. RESULTS: Patient ages ranged from 12 to 16 years. One patient had resolution of symptoms with COC therapy. MRI performed 3 years later showed no adenomyosis. Three patients failed COC therapy. All were symptomatically improved after therapy with a gonadotropin-releasing hormone agonist. Follow-up MRI performed at intervals between 6 months and 3 years showed resolution of adenomyosis. CONCLUSION: MRI can raise suspicion for the diagnosis of adenomyosis in adolescents with refractory CPP. Subsequent MRI can show regression of lesions after symptom resolution with hormonal therapy.

Clinical recommendation: pediatric lichen sclerosus.

Lichen sclerosus is a chronic inflammatory condition affecting the anogenital region that may present in the prepubertal or adolescent patient. Clinical presentations include significant pruritus, labial adhesions, and loss of pigmentation. Treatment includes topical anti-inflammatory agents and long-term follow-up as there is a high risk of recurrence and an increased risk of vulvar cancer in adult women with history of lichen sclerosus. These recommendations are intended for pediatricians, gynecologists, nurse practitioners and others who care for pediatric/adolescent girls in order to facilitate diagnosis and treatment.

Knowledge of sexually transmitted infections among adolescents in the Houston area presenting for reproductive healthcare at Texas Children's Hospital.

OBJECTIVE: Studies indicate a deficiency in knowledge of sexually transmitted infections (STIs) among adolescents, yet adolescents comprise 25% of the sexually active (SA) population and account for 48% of STIs acquired annually. This survey assesses knowledge of STIs among adolescent females. The goal of this study was to assess knowledge of STIs and how it relates to safe sex behaviors and educational access. DESIGN: A confidential 10-question STI survey was administered to a convenience sample of female adolescents. Data analysis included descriptive statistics, chi-square analysis, and linear regression analysis. SETTING: Texas Children's Hospital Pediatric and Adolescent Gynecology Clinic. INTERVENTIONS: None. PARTICIPANTS: Seventy-five female participants between the ages of 10-21 years. MAIN OUTCOME MEASURES: Age, sexual activity, educational access, preferred methods of risk reduction and questions answered correctly on the STI survey. RESULTS: The mean age of participants was 14.9 ± 2.4 years; mean age of menarche was 10.9 ± 2.9 years. Based on survey responses, all adolescents demonstrated similar knowledge of specific STIs regardless of demographic factors. However, middle and late adolescent groups had increased awareness of STIs. SA participants (36%) were more likely to choose 2 or more methods of risk reduction compared to non-SA participants (P = 0.014). There was no correlation between educational access and preferred methods of risk reduction even though 92% of respondents reported receiving STI education from school, parents, or peers. CONCLUSIONS: Current efforts at STI education are not effective. Different approaches to STI education are necessary to increase knowledge and motivate adolescents to reduce high risk behaviors.

The incidence, attitudes and practices of the removal of pubic hair as a body modification.

OBJECTIVE: To evaluate the incidence, attitudes, and practices of the removal of pubic hair as a body modification. DESIGN: A voluntary, anonymous survey was conducted among adolescents and young women 12-20 years of age seen in gynecology clinics in Houston, Texas. RESULTS: There were a total of 171 adolescent participants surveyed, of whom 70.4% of reported routinely shaving or waxing their pubic hair. More traditional body modification methods were evaluated, and 3.5% reported tattoos and 57.1% reported piercings. Sexually active participants were more likely to remove pubic hair routinely. The areas of the body where the participants felt there was "too much" hair included the genital area, upper lip, and abdomen. Of total respondents, 14.7% reported feeling "pressure" from friends or family to participate in body modification such as tattooing, piercing, or removal of pubic hair. The decision to participate in body modification was mostly influenced by friends and family. CONCLUSION: Based on this study, removal of pubic hair as a body modification is common. The results of this study demonstrated that removal of pubic hair was more common in sexually active participants compared to non-sexually active participants. Therefore, providers should additionally be aware that removal of pubic hair may be a warning sign of sexual behaviors or impending sexual activity.

Diagnosis and management of precocious puberty in atypical presentations of McCune-Albright syndrome: a case series review.

BACKGROUND: McCune-Albright syndrome is a rare syndrome, classically defined as the triad of precocious puberty, fibrous dysplasia of bone, and café au lait lesions. Partial or atypical presentations of McCune-Albright syndrome, with only one or two of the classic symptoms, have been described in the literature and remain particularly challenging due to lack of diagnostic phenotype. In these patients, the utility of analysis of mutations in the gene of the α subunit of the stimulatory G-protein is limited and so the diagnosis is often based on clinical judgment. Three atypical cases of suspected McCune-Albright syndrome with gonadotropin-independent precocious puberty are presented. CASES: Case #1: A 5-year-old female presented with painlesss vaginal bleeding and was found to have café au lait lesions. She was diagnosed with gonadotropin independent precocious puberty with eventual progression to gonadotropin dependent precocious puberty which was successfully treated with both letrozole and gonadotropin-releasing hormone agonist therapy. Case #2: A 3-year-old female presented with painless vaginal bleeding and was found to have café au lait lesions. She was diagnosed with gonadotropin independent precocious puberty and was successfully treated with letrozole. Case #3: A 5-year-old female presented with fibrous dysplasia and was found to have evidence of uterine and ovarian enlargement on ultrasound. She was diagnosed with gonadotropin-independent precocious puberty and was successfully treated with letrozole. SUMMARY AND CONCLUSION: Although different in presentation, all three atypical cases of suspected McCune-Albright syndrome with gonadotropin-independent precocious puberty were successfully treated with aromatase inhibitors. This small case series shows the utility and efficacy of aromatase inhibitors in the setting of atypical cases of suspected MAS with gonadotropin-independent precocious puberty.

Recurrent menorrhagia in an adolescent with a platelet secretion defect.

BACKGROUND: Although von Willebrand disease is the most common inherited bleeding disorder, platelet function disorders are less well recognized as a cause of bleeding. We report a case of menorrhagia caused by an unsuspected platelet secretion defect. CASE: A 13-year-old Asian female, with unknown family history, presented with menorrhagia not responsive to intravenous conjugated estrogens, requiring transfusion of 7 units of packed red blood cells. Initial screening tests for bleeding disorders were normal; however, due to high clinical suspicion, further specific testing with platelet aggregometry was performed, which revealed a platelet secretion defect. SUMMARY AND CONCLUSION: The prevalence of platelet secretion defects in adolescents with menorrhagia is unknown, but may be higher than currently recognized. When screening tests are normal, yet suspicion remains high for an underlying hemostatic disorder, platelet aggregometry must be performed.