Utility of Ultrasound and Magnetic Resonance Imaging in Patients with Disorders of Sex Development Who Undergo Prophylactic Gonadectomy.

STUDY OBJECTIVE: To evaluate ultrasonography and magnetic resonance imaging (MRI) in identifying gonads in patients with disorders of sex development (DSD) who undergo prophylactic gonadectomy, and to assess the capacity of preoperative imaging to detect premalignant and malignant transformation. DESIGN, SETTING, AND PARTICIPANTS: Retrospective cohort at a tertiary referral center of 39 patients with DSD who underwent MRI and/or ultrasonography before prophylactic gonadectomy. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Identification of gonads on preoperative imaging. RESULTS: Thirty-three patients underwent ultrasonography, which identified 54% (35/65) of gonads and 14 patients had MRI, which identified 41% (11/27) of gonads. There was no significant difference between imaging modalities in the proportion of gonads identified (P = .25). The proportion of pathology-confirmed dysgenetic gonads identified was higher on ultrasound compared with MRI (51% vs 8%; P = .02). There was no difference in the proportion of pathology-confirmed testes identified on ultrasound and MRI (54% vs 71%; P = .33). Eleven out of 39 patients (28%) were diagnosed with a premalignant lesion, and there were no distinguishing characteristics documented on imaging reports to suggest transformation. The only diagnosed malignancy in this series had imaging describing a "normal-sized ovary." CONCLUSION: Ultrasonography and MRI identified 40%-50% of gonads in patients with DSD who underwent prophylactic gonadectomy, with no significant difference between the 2 modalities. Clinicians should, therefore, consider ultrasonography as a first-line imaging modality. Premalignant lesions were not detected on either imaging modality. The only malignancy was described as a "normal-sized ovary" which should raise concern in a patient with complete gonadal dysgenesis expected to have streak gonads.

Adolescents with Disorders of Sex Development (DSD)--Lost in Transition?

Children with chronic illnesses face multiple challenges as they mature into adulthood, which relate to independence, access to care, and changes in care providers. The scope and magnitude of these problems is amplified in children with disorders of sex development (DSD). In these children, the normal progression of pubertal events can be early, late, contrasexual, pharmacologically assisted, or some combination of these features. The diagnosis of DSD can occur in childhood, which gives the family some time to prepare for future events, but in other cases, the diagnosis is made during adolescence as the condition becomes apparent. This article discusses the difficulties these children and their families face during adolescence, provides an overview of the transitioning process, and uses a few conditions as examples to illustrate particular aspects.

Menopause and post-polio symptoms as predictors of subjective sleep disturbance in poliomyelitis survivors.

OBJECTIVES: Sleep disturbance in polio survivors is a common complaint, yet little is known about the effects of the interaction of physical disability and menopause on sleep. The purpose of this study was to understand the relative contribution of menopause factors and disability to subjective sleep disturbance. METHODS: Participants were 465 women aged 50-65 years who had physical disabilities due to poliomyelitis. Hierarchical regression modeling was used to examine menopause (symptoms, status, hormone replacement use, ovarectomy status) and disability factors (post-polio symptoms) in sleep disturbance. RESULTS: In the final model, 19% (frequency) and 17% (severity) of sleep disturbance variance was explained. Psychological symptoms exerted the most influence (for both outcomes) followed by post-polio symptoms, vasomotor symptoms, an interaction of vasomotor and post-polio symptoms and estrogen use. For women with fewer post-polio symptoms, vasomotor symptoms exerted greater influence on sleep disturbance than for women with greater post-polio symptoms. CONCLUSIONS: Psychological symptoms had the strongest association with sleep disturbance in these women. Controlling for the influence of various menopause factors, our findings show that vasomotor symptoms were only one of several influences on sleep disturbance.

Clobetasol propionate in the treatment of premenarchal vulvar lichen sclerosus.

OBJECTIVE: To assess the effectiveness of treating premenarchal vulvar lichen sclerosus with clobetasol propionate. METHODS: A retrospective chart review was performed of girls presenting to the University of Michigan Pediatric and Adolescent Gynecology Clinic from January, 1995, to July, 2000, with premenarchal lichen sclerosus. Subjects in the study were treated with topical clobetasol propionate ointment 0.05% for 2-4 weeks, and then tapered to a less potent steroid. Information was extracted concerning age at onset, symptoms, vulvar examination, previous treatments, effectiveness of clobetasol, follow-up, and complications. The parents were contacted for a follow-up telephone survey. RESULTS: Fifteen girls averaging 5.7 years at the start of symptoms met criteria. The diagnosis of lichen sclerosus was made visually in 11 and by biopsy in four. Follow-up ranged from 2 months to 6 years. Fourteen girls had good improvement within 4-7 weeks. One girl developed a yeast superinfection and one developed transient erythema. At least 1 year of follow-up by clinic visit or telephone interview was available in 11 girls. Of these 11, two girls had no further vulvar symptoms after the initial treatment, five had one or two total flares, three reported three to eight flares per year, and one girl continues to be unresponsive to therapy. CONCLUSION: Clobetasol propionate was an effective treatment of premenarchal vulvar lichen sclerosus in this small group; however, recurrences were common and required additional steroid treatment. Furthermore, complications of treatment were infrequent, minor, and easily treatable.

Comparison of microscopic examination and human papillomavirus DNA subtyping in vulvar lesions of premenarchal girls.

STUDY OBJECTIVE: The purpose of this study is to compare the microscopic examination and human papillomavirus (HPV) DNA subtyping of vulvar specimens from premenarchal girls clinically diagnosed with condyloma to determine whether DNA subtyping aids in the diagnostic process. DESIGN: A retrospective chart review was performed on all premenarchal girls who underwent surgical treatment of clinically diagnosed condyloma between 1993 and 1999 at the University of Michigan Medical Center by the Pediatric and Adolescent Gynecology Service. Tissue was sent for pathologic evaluation and in 10 patients the specimens also underwent DNA subtyping. One patient had prior DNA subtyping. All the other lesions were surgically ablated. The microscopic slides were reviewed by a single pathologist blinded to the study. SETTING: The study was performed in a tertiary care university hospital. PARTICIPANTS: The study group included 11 premenarchal girls with an average age of 2.3 yr. MAIN OUTCOME MEASURES: The charts were reviewed for previous HPV treatment, maternal history of HPV, history of sexual abuse, microscopic diagnosis, and HPV DNA subtyping. RESULTS: Four patients had prior surgical treatment and two patients had undergone prior medical treatment. The microscopic diagnosis was condyloma in 8 patients, chronic dermatitis in 2 patients, and 1 patient had VIN 2-3. All 11 specimens tested positive for HPV DNA, 10 specimens contained at least one of the low-risk subtypes (6, 11, 42, 43, 44), and 1 tested positive for low-risk as well as intermediate/high-risk HPV subtypes (16, 18, 31, 33, 35, 45, 51, 52, 56). CONCLUSIONS: Although all the patients with a clinical diagnosis of condyloma tested positive for HPV DNA, only 9 of 11 were definitely diagnosed with HPV-related pathology by microscopic examination. Therefore, in premenarchal patients with verrucous lesions in the anogenital area, microscopic evaluation alone may be inadequate as a confirmatory test when a positive clinical diagnosis has been made, and HPV DNA subtyping should be considered to avoid confusion with the diagnosis.

The treatment of cyclical behavioral changes in women with mental disabilities.

STUDY OBJECTIVE: This study aims to evaluate the frequency of occurrence of cyclical behavioral changes in women with mental retardation, as well as the effectiveness of several treatment modalities for this type of behavior. DESIGN: Retrospective chart analysis. SETTING: University of Michigan clinic for reproductive healthcare of women with mental disabilities. PARTICIPANTS: All clinic patients of reproductive age, presenting with cyclical behavioral changes in our clinic from November 1985 to October 1992. INTERVENTIONS: Medical treatment of cyclical behavioral changes. MAIN OUTCOME MEASURES: Presence and documentation of cyclical behavioral changes, level of retardation, type of behavior, treatment modalities and results. RESULTS: Ninety-three of 522 reproductive-age patients (18%) presented with cyclical behavioral changes. Good documentation with behavior charts was present in 46% of these records. Level of retardation among the women included: 57 (61%) severely, 12 (13%) moderately, and 1 (1%) mildly retarded. No level of retardation could be detected in 23 (25%) women. Aggressive behavior was noted in 35 (38%) women, self-mutilation in 20 (22%) a combination of both in 10 (10%) and other behavior in 28 (28%) women. Primary treatment was by physician preference. Of 46 patients who received nonsteroidal antiinflammatory drugs (NSAID), 30 (65%) showed improvement, 10 (21%) showed no improvement, and 2 patients (4%) showed worsening symptoms. Birth control pills were used in 15 patients; there were signs of improvement in 6 (40%), no improvement in 3 (20%), and worsening in 3 (20%). The use of depomedroxyprogesterone injections was successful in treating 6 of 9 patients (66%), and showed no improvement in 2 women. No statistically significant differences were found between these treatment modalities. CONCLUSIONS: Cyclical behavioral changes in women with mental retardation is a common problem (18%) and may be related to pain- possibly due to menstrual cramps, since 65% of the patients responded to NSAID. If treatment with NSAID is unsuccessful, birth control pills and depomedroxyprogesterone improved behavior in 40% to 66% of patients. Documentation is a key issue.

Vulvar disorders in adolescent patients.

This article describes the most commonly encountered vulvar disorders in adolescents and refers to some less common entities. The categories are based upon appearance of the vulvar lesions: solid, cystic, ulcerative and infectious.

Gynecological health care for adolescents with developmental disabilities.

The gynecological health care of adolescents with developmental disabilities presents a unique challenge to care providers. Adolescence is a time of turmoil for many girls and for adolescents with special needs it may seem overwhelming. Patients, their parents and their caregivers need special attention during these changes in their lives. This chapter focuses on how to perform a reproductive health evaluation focusing on the history and physical exam, especially the pelvic exam. Menstrual hygiene and menstrual abnormalities are discussed in detail. Other topics include abuse and abuse prevention, contraception, and cyclical behavior changes. Patience, persistence, and adaptation of the usual examination techniques can lead to a thorough and balanced assessment of the patient and help her, her parents, and her caregivers with the challenges of adolescence.

Ovarian surgery in premenarchal girls.

STUDY OBJECTIVE: This study was undertaken to assess the indications, procedures, and pathology in premenarchal girls undergoing ovarian surgery. DESIGN: Retrospective chart review. SETTING: University of Michigan Medical Center 1980-1996. PARTICIPANTS: Premenarchal girls, who underwent ovarian surgery. INTERVENTIONS: None. MAIN OUTCOME MEASURES: All available charts (n = 52) had information extracted concerning age at time of surgery, presenting symptoms, preoperative and postoperative diagnosis, procedure, and pathology report. RESULTS: Of the 52 patients, 50% were less than 1 year old, 31% were between 1 and 8 years old, and 19% were between 8 and 12 years old. Presenting complaints in 31 patients included structural and or endocrinologic abnormalities, and the other 21 patients presented with abdominal or systemic complaints. The most common preoperative diagnosis was an abdominal/pelvic mass (n = 24). The postoperative diagnoses revealed 18 torsions and 16 ovarian masses without torsion, 8 chromosomal abnormalities, 5 hernias, and 5 malignancies. Procedures included 37 salpingo-oophorectomies (28 unilateral and 9 bilateral), 7 oophorectomies, and 7 cystectomies. One patient underwent a staging procedure. Pathology reports confirmed hemorrhagic infarctions (n = 19), dysgenic gonads (n = 8), simple cysts (n = 7), teratomas (n = 6), theca lutein cysts (n = 4), fibroma (n = 1), stromal tumor (n = 1), mucinous cystadenoma (n = 1), granulosa cell tumor (n = 1), uterine neuroblastoma (n = 1), mixed germ cell neoplasm (n = 1), metastatic Wilms' tumor (n = 1), and gonadoblastoma (n = 1). CONCLUSIONS: Torsion was the most common diagnosis in our study group and was usually unsuspected. Premenarchal ovarian surgery usually included removal of the entire ovary. However, because malignancies are uncommon in this population (9.6%), a cystectomy should be considered when appropriate and technically feasible.

Menorrhagia in adolescents requiring hospitalization.

STUDY OBJECTIVE: This study was undertaken to assess the causes and treatments of menorrhagia in adolescents hospitalized for this menstrual disorder. DESIGN: A retrospective chart review was performed of all adolescents < or =20 years of age with menorrhagia admitted at the University of Michigan from 1979 to 1995. Information regarding medical history, hematologic parameters, treatment, and diagnosis was extracted from each chart. Pregnant and premenarchal patients were excluded. RESULTS: Thirty-seven adolescents with 46 admissions for menorrhagia were identified. The average age of menarche was 12.9 years and the average age at admission was 15.9 years. Nineteen adolescents had significant medical diseases. For the 46 admissions, causes of menorrhagia were anovulation (21), hematologic disease (15), chemotherapy-related (5), and infections (5). Transfusions of blood products were performed in 28 of the admissions. Treatments included oral contraceptive pills or progestins (30), intravenous conjugated estrogens (8), antibiotics (4), immune gammaglobulin (3), DDAVP (3), and prednisone (1). Twelve surgical procedures were performed, including eight dilatation and curettages (D&Cs), three laparoscopies, and one hysterectomy. CONCLUSIONS: Sixty-one percent of admissions for adolescent menorrhagia were in adolescents with significant medical problems. The patients with menorrhagia who required admission had severe anemia and were transfused in 63% of cases. The predominant causes for these admissions included anovulation in 46%, hematologic disease in 33%, chemotherapy in 11%, and infection in 11%. Hormonal regulation or suppression of menses should be considered in adolescents with significant medical disease.

Recurrent benign müllerian papilloma of the cervix.

A 4-year-old girl with a recurrent benign cervical müllerian papilloma is presented. Benign müllerian papillomas are rare cervical and vaginal lesions of childhood, with only two recurrences previously reported. This lesion was originally biopsied and later rebiopsied and completely fulgarated with electrocautery. One year later, the lesion recurred and was biopsied but not completely excised because of concern that complete excision of this benign, asymptomatic lesion would cause significant damage to her cervix. A review of the literature on this subject is presented.

Cervical cytology in women with mental retardation.

OBJECTIVE: To examine our experience with performing pelvic examination and obtaining cervical cytology in women with mental retardation. METHODS: From November 1985 to October 1992, 658 women were seen in our clinic for women with mental disabilities. A standardized clinic visit form was completed for each woman. Of these records, 574 were available for analysis. All charts were reviewed for data on the level of retardation, incidence of sedation, success in performing pelvic examination and Papanicolaou smear, technique used for cervical smear, and pathology results of the cervical cytology. RESULTS: Of the 574 women, 289 (50%) had severe, 69 (12%) moderate, and 31 (5%) mild retardation. In 185 (32%), the exact level of retardation was not established. During the study period, 1235 pelvic examinations were analyzed. Within this group, 845 examinations could be completed initially, 177 required the use of sedation (14%), and 213 (17%) examinations could not be completed. Of the 845 examinations, 706 cervical smears were available for analysis. Of these, only 243 (34%) contained endocervical cells. Two abnormal cytology results were found (0.3%). Of the 177 examinations done with sedation, 44 women (25%) still could not be examined. Cytology specimen results were obtained in 124 of the examinations, and 40 (32%) of these specimens contained endocervical cells. No cytologic abnormalities were present. In a cervical smear technique comparison, endocervical cells were present in 58% of 161 standard speculum examinations and in 18% of 93 cotton-swab tests (P < .001). CONCLUSION: Cytology specimens can be obtained without sedation in most mentally retarded women. Only one-third of Papanicolaou smears contained endocervical cells from examinations with sedation as well as those without. The cotton-swab technique has a significantly lower endocervical cell collection rate than the standard speculum technique. The incidence of abnormalities on cytologic examination appears to be low compared with other populations of women. Whether this is due to suboptimal smears, lower prevalence of human papillomavirus, or a combination of both remains to be resolved.

Gynecological and reproductive function after liver transplantation.

Women of reproductive age who underwent orthotopic liver transplantation were surveyed to determine timing and pattern of menstruation, sexual activity, contraception, and incidence of pregnancy and gynecological disorders. Eighty two female recipients of liver transplantation at the University of Michigan between August 1985 and January 1992 were surveyed about menstrual function and gynecological and obstetrical histories before and after transplantation. Additional information was retrieved from medical records regarding their liver disease and details of pregnancies and gynecological care. In the year before transplantation, 27 women (42%) reported regular menstrual cycles, 18 (28%) irregular and unpredictable bleeding, and 19 (30%) amenorrhea. After transplantation, 30 women (48%) experienced regular menses, 16 (26%) irregular bleeding, and 16 (26%) amenorrhea. In women less than 46 years old, 27 (53%) had regular menses before and after transplant. Most women with acute liver disease had regular periods before (82%) and after transplant (73%). A total of 95% of women under the age of 46 had return of menstrual bleeding within the first year after transplantation. Of these women 49% had normal liver function tests at the time of survey, 33% mildly abnormal, and 18% severely abnormal. Liver function was not correlated with menstrual patterns. A total of 72% of women were sexually active after transplantation. Of 24 women under age 46 who had not undergone sterilization or hysterectomy, six women conceived seven pregnancies. Seven women reported abnormal cervical cytology results after transplantation. Six underwent colposcopy and 4 required some form of destructive therapy for cervical dysplasia. In women with liver disease, menstrual patterns may change after orthotopic liver transplantation. This is more common in women with chronic liver disease than in those with acute liver disease. There was no correlation between liver function and menstrual regularity after transplant. Return to sexual activity can be expected and pregnancies are not rare in a population of young women after liver transplantation. Regular cervical cytology is critical due to a recognized increase in cervical neoplasia in immunocompromised patients.

Laparoscopic transillumination for the location of anterior abdominal wall blood vessels.

OBJECTIVE: To determine the efficacy of transillumination for locating abdominal wall vessels prior to trocar placement during laparoscopy. DESIGN: Prospective clinical descriptive study. SETTING: Normal human volunteers in an academic research environment. PATIENTS: Forty-seven white and 21 black women of various weights undergoing laparoscopy for clinical indications unrelated to this study. INTERVENTIONS: None. MAIN OUTCOME MEASURES: The location and number of abdominal wall vessels visible by transillumination were recorded for each patient. RESULTS: In women of normal weight, a single vessel could be seen approximately 5 cm from the midline in > 90% of the patients, and second vessel approximately 8 cm from the midline could also be seen in 51%. The more medial vessels did not correlate with the course of the inferior epigastric vessels seen laparoscopically. The ability to see vessels was decreased significantly by the patients' weight but not by skin color. CONCLUSIONS: Superficial abdominal wall vessels may be located by transillumination in the majority of women of normal weight regardless of skin color, but is of less value in overweight and obese women. However, the deep (inferior) epigastric vessels cannot be effectively located by transillumination, and thus other techniques should be used to minimize the risk of injury to these vessels.

Adrenal axis and hypogonadotropism in the growth-restricted female lamb.

Growth retardation induced by dietary restriction in the lamb results in a decrease in LH pulse frequency and therefore in delayed puberty. Increased circulating cortisol levels have been associated with nutritional restriction in a variety of species. The current study tested the hypothesis that hyperactivity of the adrenal axis sustains hypogonadotropism in the growth-restricted lamb. Our approach was to compare the patterns and levels of circulating cortisol and LH in ovariectomized, growth-restricted (n = 8) and ad libitum-fed (n = 6) lambs. At 37 wk of age, after the growth-restricted lambs had been on the reduced diet for 31 wk, basal cortisol levels were determined hourly for 31 h. In addition, during this period, pulsatile LH and cortisol release was determined during a 4-h period (samples every 12 min). Finally, the cortisol response to a physiologic ACTH stimulus and an audiovisual stimulus (barking dog for 2 min) was determined in frequent samples collected during the last 5 h of the 31-h period. As expected, growth-restricted lambs exhibited a low LH pulse frequency (0.25 +/- 0.10 pulses/h) compared with ad libitum-fed lambs (1.37 +/- 0.07 pulses/h). No diurnal cortisol rhythm was observed in either group, and a similar cortisol pulse frequency occurred in the two groups (1.00 +/- 0.07 pulses/h in growth-restricted lambs and 1.05 +/- 0.10 pulses/h in ad libitum-fed lambs). There was no significant difference between the groups in cortisol pulse amplitude. ACTH administration (i.v.) induced a similar cortisol pulse in 4 of 8 growth-restricted lambs and in 5 of 6 ad libitum-fed lambs.(ABSTRACT TRUNCATED AT 250 WORDS)

Incontinence following rupture of the anal sphincter during delivery.

OBJECTIVE: To determine the frequency with which patients report incontinence of flatus or stool after rupture of the anal sphincter during delivery. METHODS: A chart review and telephone interview were conducted with 70 primiparas, 35 of whom had rupture of the anal sphincter at delivery and 35 of whom did not. All were contacted 9-12 months postpartum and questioned about the development of incontinence of gas or liquid or formed stool, persistent dyspareunia, and perineal pain. RESULTS: Incontinence of gas was reported by six women (17%) in the rupture group and one (3%) in the control group (P < .05). The incidence of incontinence of stool, both liquid and solid, dyspareunia, and persistent perineal pain were similar between the groups. CONCLUSION: Incontinence of flatus was reported six times more often by women who experienced a third- or fourth-degree perineal laceration than by those without anal sphincter rupture.

Laparoscopic injury of abdominal wall blood vessels: a report of three cases.

BACKGROUND: Operative laparoscopy is being used for an increasing number of applications. Many of these innovative techniques require the insertion of large trocars through the anterior abdominal wall at points lateral to the midline. Because of the rich vascular supply of the anterior abdominal wall, the incidence of abdominal wall vessel injuries appears to be increased by these techniques. CASES: Three cases are reported in which abdominal wall blood vessels were injured during operative laparoscopy. Case 1 describes laceration of the inferior epigastric artery above the pubic crest. Despite initial hemostasis with a transabdominal suture ligation, postoperative blood loss necessitated transfusion and reoperation to control bleeding. Case 2 describes hematoma formation after unrecognized injury to one of the epigastric vessels. The hematoma resolved without sequelae with conservative treatment. Case 3 describes hematoma formation under a laparoscopic trocar incision lateral to the umbilicus that progressed to an abscess and was treated with drainage, irrigation, and antibiotics. CONCLUSIONS: Choosing appropriate trocar types and insertion sites based on a thorough understanding of anterior abdominal wall anatomy may minimize the risk of vessel injury during operative laparoscopy. However, because of anatomical variation, strategies for managing vessel injuries and their sequelae are also necessary.