Rachel F. Spitzer

Müllerian Duct Anomalies and Mimics in Children and Adolescents: Correlative Intraoperative Assessment with Clinical Imaging

Müllerian duct anomalies (MDAs) are congenital entities that result from nondevelopment, defective vertical or lateral fusion, or resorption failure of the müllerian (paramesonephric) ducts. MDAs are common, although the majority are asymptomatic, and have been classified by the American Society of Reproductive Medicine according to clinical manifestations, prognosis, and treatment. Accurate diagnosis of an MDA is essential, since the management approach varies depending on the type of malformation. In females, when a müllerian duct becomes obstructed, the patient may present with an abdominal mass and dysmenorrhea. If the patient is not treated in a timely fashion, the consequences can be severe, extending even to infertility. When an MDA is suspected, ultrasonography (US) should be performed initially to delineate any abnormalities in the genital tract. However, US cannot help identify the type of MDA. In contrast, magnetic resonance imaging is a valuable technique for noninvasive evaluation of the female pelvic anatomy and accurate MDA classification. If obstruction is present, surgical correction of the MDA may be required, and further counseling of the patient with regard to reproductive possibilities becomes important. Supplemental material available at http://radiographics.rsnajnls.org/cgi/content/full/29/4/1085/DC1.

Trends in menstrual concerns and suppression in adolescents with developmental disabilities.

PURPOSE Demonstrate changes in methods of menstrual suppression in adolescents with developmental disabilities in a recent 5-year cohort compared with an historical cohort at the same hospital. METHODS Retrospective cohort study of patients with physical and cognitive challenges presenting for menstrual concerns at an Adolescent Gynecology Clinic between 2006 and 2011 compared with a previous published cohort (1998 to 2003). RESULTS Three hundred patients with developmental disabilities aged 7.3 to 18.5 years (mean 12.1 ± 1.6) were analyzed. Caregiver concerns included menstrual suppression, hygiene, caregiver burden, and menstrual symptoms. Ninety-five percent of patients had cognitive disabilities, 4.4% had only physical impairments. Thirty-two (31.7) percent of patients presented premenarchally. The most commonly selected initial method of suppression was extended or continuous oral contraceptive pill (OCP) (42.3%) followed by patch (20%), expectant management (14.9%), depot medroxyprogesterone acetate (DMPA) (11.6%), and levonorgestrel intrauterine system (LNG-IUS) (2.8%). Published data from 1998 to 2003 indicated a preference for DMPA in 59% and OCP in 17% of patients. The average number of methods to reach caregiver satisfaction was 1.5. Sixty-five percent of initial methods were continued. The most common reasons for discontinuation were breakthrough bleeding, decreased bone mineral density, or difficulties with patch adherence. Second-choice selections included OCP (42.5%), LNG-IUS inserted under general anesthesia (19.2%), DMPA (17.8%), and patch (13.7%). CONCLUSIONS Since identification of decreased bone mineral density with DMPA and emergence of new contraceptive options, use of extended OCP or patch has surpassed DMPA for menstrual suppression in our patient population. LNG-IUS is an accepted, successful second-line option in adolescents with developmental disabilities.

Maternal luteoma of pregnancy presenting with virilization of the female infant.

BACKGROUND Virilization in female newborns typically results from congenital adrenal hyperplasia, requiring immediate diagnosis and treatment. We report a rare cause of virilization, maternal pregnancy luteoma, responsible for virilization of both a newborn and the mother. Luteomas are usually asymptomatic tumour-like ovarian lesions of pregnancy that secrete androgens in only 25% of cases. Many female infants born to masculinized mothers will also be virilized. CASE A term infant born with ambiguous genitalia was transferred to a referral centre for investigation, diagnosis, and treatment. Assessment identified Prader II-III genitalia, an elevated serum testosterone level, a normal serum 17-hydroxyprogesterone level, and a normal female karyotype (46,XX). The mother had had virilization from the second trimester and was found to have an elevated serum testosterone level. Pelvic ultrasound assessment in the mother showed a complex right ovarian mass. Laparotomy was performed, and the mass was excised. Histopathology examination confirmed a luteoma. CONCLUSION High maternal serum testosterone levels due to a luteoma can result in virilization in the female newborn. This report emphasizes the need to consider possible underlying maternal pathology in evaluating a virilized female infant.

Characteristics and surgical success of patients presenting for repair of obstetric fistula in western Kenya

To carry out a large‐scale retrospective review of patients who had undergone surgical repair of obstetric fistula in Kenya to determine patient characteristics and determinants of successful surgical repair.

Retrospective Review of Unintentional Female Genital Trauma at a Pediatric Referral Center

Unintentional female genital trauma is a complaint commonly seen and managed through the emergency department. The purpose of this study was to review all unintentional female genital trauma evaluated at The Hospital for Sick Children for 3.5 years to determine the factors associated with gynecologic consultation and need for operative repair. Methods: One hundred five patients were identified by health record coding. Data were extracted to study factors associated with gynecologic consultation and operative repair. Statistical analyses were performed to evaluate the significance of these associations. Surgical choices were also evaluated. Results: Mean age was 5.60 years. Mean time to presentation was 7.05 hours. Straddle injury was the most common mechanism (81.90%), and only 4.76% injuries were penetrating. Of the 105 patients, 48.57% consulted the gynecology section, 19.05% were taken to the operating room, and 6.66% were treated under conscious sedation. Overall, 20.95% required surgical repair. The most common complication was dysuria. Six patients had other injuries, the most common of which were pelvic fractures related to trauma. Factors significantly associated with gynecologic consultation and operative management included older age, transfer to our institution, shorter time to presentation, laceration-type injury, hymenal injury, and larger size of injury. Straddle injuries were significantly less likely to be taken to the operating room. When cases were stratified by a surgeon, there were no significant differences in management. Conclusions: Unintentional female pediatric genital traumas most commonly result from straddle injuries. Most injuries are minor, and in this cohort, only 48.57% received gynecologic consultation and 19.05% required operative management. Future prospective studies would be useful to better evaluate the efficacy of surgical choices.

Vulvar Inflammation as the Only Clinical Manifestation of Crohn Disease in an 8-Year-Old Girl

Recognition of Crohn disease in children who present with cutaneous vulvar lesions as their initial clinical manifestation is often difficult. We report here the case of an 8-year-old girl with chronic vulvar edema, which on biopsy revealed granulomatous inflammation of the vulva. Further investigation and biopsy of the terminal ileum demonstrated similar findings and was consistent with a diagnosis of Crohn disease.

Case series of laparoscopically resected noncommunicating functional uterine horns.

BACKGROUND Unicornuate uteri occur in 1 in 5400; 74-90% are associated with a rudimentary horn. If the noncommunicating horn contains functional endometrium, it often requires surgical resection. There are fewer than 35 published cases describing laparoscopic resection of uterine horns. This series reviews the preoperative assessment, operative management, and postoperative imaging of the laparoscopic management of 5 functional noncommunicating uterine horns. CASES Our patients presented with pelvic pain, dysmenorrheal, and dyspareunia. All patients underwent preoperative imaging with ultrasound and MRI. The diagnosis in all cases was unicornuate uterus with functional, noncommunicating uterine horn. SUMMARY AND CONCLUSIONS Mean age was 19.5 years. Preoperative assessment with MRI was found to be useful for surgical planning and anticipation of the amount of myometrial connection. Average operating time was 2:24. Mean length of stay was 0.8 nights. No procedures were converted to laparotomy and all patients recovered well. Postoperative MRIs indicate no myometrial abnormalities.

Retrospective Review of Predisposing Factors and Surgical Outcomes in Obstetric Fistula Patients at a Single Teaching Hospital in Western Kenya

OBJECTIVE We examined success rates and complications of obstetric fistula (OF) surgical repairs in association with patient and fistula characteristics, including sociocultural and socioeconomic determinants of health. A better understanding of these associations will help guide surgical management and prevent predisposing factors. METHODS We reviewed the medical records of 86 patients who underwent OF repair at Moi Teaching and Referral Hospital in Kenya between 1999 and 2007. RESULTS Women with OF presented for repair with a variety of concurrent conditions. Seventy-eight percent had laboured for at least 24 hours; 29% had undergone previous unsuccessful surgery. Of the women who presented at postoperative follow-up, 54% still complained of incontinence. Persistent incontinence was associated with larger, more complicated fistulas and having had previous failed attempts at surgical repair. CONCLUSION The association of factors such as duration of labour with OF reflects the limited availability of obstetrical care in Western Kenya. There is a significant difference in postoperative success of fistula repair between women with large fistulas or those who had previous failed surgery and other patients. This reflects the importance of primary and secondary prevention.

A randomized, double-blind, placebo-controlled trial of combined nevirapine and zidovudine compared with nevirapine alone in the prevention of perinatal transmission of HIV in Zimbabwe.

BACKGROUND A single dose of nevirapine (sdNVP) administered to both mother and infant can decrease mother-to-child transmission of human immunodeficiency virus (HIV) by 47%, compared with ultra-short course zidovudine therapy (usZDV). There is limited data about the benefit of usZDV added to sdNVP to prevent mother-to-child transmission. METHODS We performed a double-blind, randomized, placebo-controlled trial to determine whether usZDV combined with sdNVP improved neonatal outcome, compared with sdNVP alone. Mothers were randomized to 1 of 2 treatment groups. Mothers in the usZDV/sdNVP group received a loading dose of zidovudine (600 mg administered orally) and continued to receive 300-mg doses of zidovudine orally every 3 h while in labor, and their infants received zidovudine at a dosage of 2 mg per kg of body weight 4 times per day orally for 72 h. Mothers and infants in the sdNVP group received zidovudine placebo dosed in the same manner. All mothers also received nevirapine at a dosage of 200 mg orally while in labor, and all infants received nevirapine 2 mg per kg of body weight orally within 72 h of delivery. RESULTS The study was stopped on the basis of futility, because interim data showed that, at present trends, superiority would not be demonstrated. Results at 6 weeks of age were available for 609 infants. The primary end point of HIV RNA positivity or death occurred in 21.8% of infants in the usZDV/sdNVP arm and 23.6% of the infants in the sdNVP arm. CONCLUSION usZDV, when added to a standard 2-dose regimen of sdNVP, did not demonstrate a clinically important decrease in the combined end point of mother-to-child transmission or infant death. High rates of adverse maternal and infant outcome in both study arms suggest that improved approaches are necessary.

Menstrual suppression for adolescents with developmental disabilities.

The approach to menstrual suppression for adolescents with developmental disabilities has evolved considerably over the years due to changing philosophies and evolving treatment options. We review the medical management options available for menstrual suppression with a focus on the needs and treatment of adolescents with developmental disabilities.