Encysted hydrocele of the canal of Nuck in an 11-month-old child with a past history of duodenal atresia and Arnold-Chiari malformation: A case report.

RATIONALE: Hydrocele of the canal of Nuck is a rare developmental disorder and represents of a homolog of hydrocele of spermatic cord in males. Hydrocele of the canal of Nuck is a very rare cause of inguinal swelling in female infants and children. It results from the failure of obliteration of the distal portion of evaginated parietal peritoneum within the inguinal canal, which forms a sac containing fluid. PATIENT CONCERNS: We describe a case of hydrocele of the canal of Nuck in an 11-month-old girl with a past medical history of duodenal atresia and Arnold-Chiari malformation. DIAGNOSIS: Physical examination and ultrasound revealed a soft, cystic, noncompressible, and non-fluctuant labial mass measuring approximately 5 cm. INTERVENTIONS: The patient underwent surgical exploration through a right skin crease incision. The cystic lesion was histologically confirmed to be a non-communicated hydrocele of canal of Nuck. OUTCOMES: The child is doing well at 1-year follow-up with no swelling or recurrence on the operated side. LESSONS: Hydrocele of the canal of Nuck is a rare developmental disorder but should be considered in a differential diagnosis in young girls with an inguino-labial swelling.

Pelvic Organ Prolapse and Pregnancy in the Female Bladder Exstrophy Patient.

PURPOSE OF REVIEW: Classic bladder exstrophy (BE) remains one of the most demanding reconstructive challenges encountered in urology. In female BE patients, the long-term sequela of both primary and revision genitoplasty, as well as intrinsic pelvic floor deficits, predispose adult women to significant issues with sexual function, pelvic organ prolapse (POP), and complexities with reproductive health. RECENT FINDINGS: Contemporary data suggest 30-50% of women with BE develop prolapse at a mean age of 16 years. Most women will require revision genitoplasty for successful sexual function, although in some series over 40% report dyspareunia. Current management for pregnancy includes elective cesarean section with involvement of high-risk obstetrics and urologic surgery. This review encapsulates contemporary concepts of etiology, prevalence, and management of POP and pregnancy in the adult female BE patient.

The perinatal origins of major reproductive disorders in the adolescent: Research avenues.

The fetal endometrium becomes responsive to steroid hormones around the fourth month of pregnancy starting with an oestrogenic phase, which is followed late in pregnancy by a secretory phase. Based on post-mortem studies, the endometrium at birth is secretory in only one-third of neonates and proliferative in the remaining cases. Decidual or menstrual changes are rare in fetal endometrium despite high circulating steroid hormone levels, which drop rapidly after birth. Hence, acquisition of progesterone responsiveness appears to be dependent on endometrial maturation and relative immaturity may persist in a majority of girls until the menarche and early adolescence. Two major reproductive disorders have been linked with either advanced or delayed endometrial maturation. First, early-onset endometriosis may be caused by menstruation-like bleeding in the neonate, leading to tubal reflux and ectopic implantation of endometrial stem/progenitor cells. Second, persistence of partial progesterone resistance in adolescent girls may compromise deep placentation and account for the increased risk of major obstetrical syndromes, including preeclampsia, fetal growth retardation and preterm birth. The concept of neonatal origins of common reproductive disorders poses important research challenges but also subsumes potential new preventative strategies.

Uterine duplicity without communication between both hemiuteri and cervicovaginal atresia: importance of a thorough diagnosis before treatment.

The study described diagnostic and therapeutic processes for an exceptional congenital female genital anomaly involving a 15-year-old girl with progressive dysmenorrhoea and intense abdominal pain, and analyses pathogenesis of embryological anomalies associated with uterine duplicity (UD). Diagnostic methodology is analysed; treatment is described; and recommendations are provided. For the study of anomalies such as UD, we recommend the use of an ultrasound, vaginoscopy, MRI and laparoscopy imaging. Keeping in mind that hemihysterectomy should only be performed in cases with endometriosis or other unusual circumstances, surgical treatment should be avoided. A complete study based on gynaecological examination and ultrasound should be performed in adolescents with severe and progressive dysmenorrhoea. Based on the findings, imaging tests such as MRI and, if necessary, laparoscopy coinciding with menstruation, should be conducted before opting for surgical treatment.

[Uterovaginal agenesis and polycystic ovary syndrome: psychological disturbance in adolescence].

Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS) is characterized by complete or partial absence of the vagina, uterus and proximal fallopian tubes and diagnosis is usually made in late adolescence, when primary amenorrhea appears as the major symptom. Polycystic Ovary Syndrome (PCOS), which is the most common endocrine disorder among women of reproductive age, includes a variety of clinical manifestations (menstrual irregularities, hirsuitism, acne, alopecia, obesity and infertility), due to androgen hypersecretion, insulin resistance and chronic anovulation. Both MRKHS and PCOS have been studied concerning the psychological aspects and have been associated with emotional distress as well as self-esteem, body image, identity and femininity impairment. The purpose of this study was to assess psychological functioning in adolescents with uterovaginal agenesis and primary amenorrhea due to MRKHS and those with hyperandrogenism and oligomenorrhea due to PCOS, compared with healthy adolescents. The participants were 70 adolescent girls, of whom 24 with MRKHS, 22 with PCOS and 24 healthy eumenorrheic adolescents (control group) matched by age and school grade. Psychological assessment included self report questionnaires, standardized in Greek population sample. Particularly, the "Beck Depression Inventory" (BDI), the "State-Trait Anxiety Inventory" (STAI-Gr) and the "Youth Self Report" (YSR) were used to measure depression, anxiety and psychopathology respectively, while the "Symptom Checklist-90-R" was used to measure psychopathology for the patients >18 years old. The results showed significantly higher scores on the state - anxiety scale for the MRKHS group compared with the control group. The MRKHS patients in late adolescence (18-20 years old) presented also significantly higher scores in depression and psychopathology scales (symptoms of anxiety, aggressive behavior and phobic disorder) than PCOS patients of the same age. On the contrary, regarding PCOS patients, age was negative correlated with attention problems and PCOS patients >18 reported significantly more somatic complaints compared with age-mate MRKHS patients and controls. PCO syndrome's clinical manifestations, including menstrual disorders, hirsuitism, acne, alopecia, obesity and infertility, may cause significant emotional distress. Nevertheless, they appear in great variety and our sample is characterized by mild features of hyperandrogenism and oligomenorrhea. This may explain findings of milder psychological disturbance associated with PCOS in this sample in comparison to other studies. As far as MRKHS is concerned, diagnosis and loss of reproductive ability, especially in late adolescence, obstruct emotional stability, physical maturity and sexual identity development ending that are expected in this period of life. Undoubtedly, the management of MRKHS in adolescence constitutes a complex multidisciplinary issue and psychological support of patients is needed in order to prevent possible psychological consequences and to achieve a normal transition to adulthood. Among the limitations of this study is the small sample size, which limits the generalisability of the reported results, especially in "Youth Self Report" and in "Symptom Checklist-90-R" questionnaires, where the sample was divided according to the age. Nevertheless, the very low incidence of MRKHS (1/5000) emphasize the value of the present results, which support the need for further investigation.

A novel approach to cervical reconstruction using vaginal mucosa-lined polytetrafluoroethylene graft in congenital agenesis of the cervix.

OBJECTIVE: To report a novel technique for cervical reconstruction in a patient with congenital agenesis of the cervix. DESIGN: Case report. SETTING: University-based tertiary care hospital. PATIENT(S): A woman with congenital absence of the cervix, functional vagina, and adenomyosis uteri. INTERVENTION(S): Laparoscopic-assisted creation of an uterovaginal fistula followed by placement of a polytetrafluoroethylene graft lined with vaginal mucosa graft to create the neocervix. MAIN OUTCOME MEASURE(S): Follow-up examinations with the hysteroscope confirmed unobstructed menstrual outflow through the patent neocervix. RESULT(S): The neocervix was completely mucosalized on the inner surface by 12 days postoperatively. The patient had return of menstrual bleeding at 4 weeks postoperatively. She has resumed normal sexual activities and had maintained regular menstrual cycles at the 6-month clinical follow-up. There were no complications related to the graft. CONCLUSION(S): Prosthetic reconstruction of the cervix using vaginal mucosa-lined polytetrafluoroethylene is easy to perform and effective. It offers an alternative treatment option for patients with cervical agenesis to preserve their reproductive potential.

Contemporary pediatric gynecologic imaging.

Ultrasound is the primary screening modality for the evaluation of pediatric gynecologic maladies. Magnetic resonance imaging (MRI) is used progressively more in this field, particularly for the evaluation of complex pelvic masses and congenital anomalies. However, ultrasound remains the preferred modality due to the excellent visualization, the dynamic nature of the examination modality, lack of ionizing radiation and sedation risks, and comparatively lower cost. MRI is generally a second-line examination and is preferred over computed tomography as it does not involve the use of ionizing radiation. Additionally, visualization of the female reproductive system anatomy with MRI is superior to computed tomography, the latter being reserved generally for tumor staging. Fluoroscopic examinations and abdominal radiographs can provide additional information that may support a diagnosis.

Estrogen receptor ESR1 is indispensable for the induction of persistent vaginal change by neonatal 5alpha-dihydrotestosterone exposure in mice.

Development of the reproductive organs can be strongly affected by the hormonal environment. In the mouse, exposure to estrogens and androgens during the critical developmental period induces estrogen-independent cell proliferation and differentiation in the adult vaginal epithelium, which often results in cancerous lesions later in life. In the present study, we assessed the contributions of estrogen receptor 1 (alpha) (ESR1) to the developmental effects of the nonaromatizable androgen 5alpha-dihydrotestosterone (DHT) on female mouse vagina and external genitalia. The vagina of Esr1(-/-) mice treated neonatally with DHT showed atrophic epithelium, whereas the vaginal epithelium of Esr1(+/+) mice was stratified and keratinized even after ovariectomy. In addition, neonatal treatment with DHT led to persistent phosphorylation of ESR1 in the vaginae of 60-day-old ovariectomized mice. We infer from these data that ESR1 is obligatory for the induction and maintenance of persistent vaginal epithelial changes induced by neonatal administration of DHT. Neonatal DHT treatment also induced hypospadias in both Esr1(-/-) and Esr1(+/+) mice. In contrast, DHT-induced formation of an os penis-like large bone in the clitoris was found in Esr1(-/-) mice but not in Esr1(+/-) or Esr1(+/+) mice. These results shed light on mechanisms of the induction of developmental effects elicited by sex steroid hormones on the developing animals.

Concurrent imperforate hymen, transverse vaginal septum, and unicornuate uterus: a case report.

A 14-year-old premenarcheal adolescent girl presented with lower abdominal discomfort and urine retention. After clinical and imaging examination, an imperforate hymen and a large hematocolpos along the upper part of the vagina was diagnosed. Incision of the imperforate hymen did not lead to drainage of blood or fluid. A complete transverse vaginal septum in the middle third of the vagina was identified and when incised drained approximately 200 mL of a dense brownish fluid. Laparoscopy showed a small unicornuate uterus, confirmed by hysteroscopy. This is the first case in the literature of concurrent imperforate hymen, transverse complete vaginal septum, and unicornuate uterus, and it highlights the potential of a multifactorial embryologic genetic etiology.

Spontaneous lesions in the reproductive tract and mammary gland of female non-human primates.

Because of their close phylogenic relationship with humans, the use of non-human primates (NHP) as experimental subjects has a long history in biomedical research. Although research topics have shifted focus and species used have changed, NHP remain vital as models in basic and applied research. While there is a wealth of information available on the spontaneous lesions of NHP, most of this information is fragmented, dated, or narrow in focus, often limited to single case reports. This review attempts to integrate this information to illustrate and enumerate the spectrum of spontaneous pathology of the reproductive tract and mammary gland of NHP. Although not the focus of this review, steroid-related changes are inextricably linked to these tissues, and brief consideration is given to this subject as well.

Outcome of bilateral adrenalectomy in congenital adrenal hyperplasia: one unit's experience.

OBJECTIVE: The use of bilateral adrenalectomy in the management of congenital adrenal hyperplasia (CAH) is controversial. We set out to review the outcome of 5 cases of CAH who have undergone adrenalectomy in our unit. DESIGN: A retrospective case note review and subject interview of the experience of adrenalectomy in the setting of a tertiary adult CAH clinic. METHODS: Subjects who had undergone adrenalectomy were reviewed at a routine clinic visit with particular reference to clinical and biochemical outcomes after adrenalectomy. RESULTS: Two subjects underwent surgery for the sole indication of desire for fertility with successful outcome and without subsequent adrenal crises. Three women suffered from the common clinical management problem of unsuppressible hyperandrogenism and worsening obesity. Whilst the outcome of improved appearance and weight loss was achieved in these subjects, all three experienced significant complications including pigmentation and acute episodes of adrenal insufficiency. CONCLUSION: We present a mean follow-up of 4.2 patient years and conclude that this procedure may be suitable for selected women with CAH. Outcomes for those pursuing fertility were positive; however, complications were noted in those women for whom the procedure was performed for symptom control. Accepting that the number of subjects is small, it is clear that more data are required before widely recommending this procedure.

Obstructive anomalies of the female reproductive tract.

Obstructive anomalies of the female reproductive tract preclude the outflow of menstruation and may allow the collection of blood in the uterus and/or vagina and increase the likelihood of retrograde flow. These conditions occur in young women and may result in pelvic masses, endometriosis and/or pain. In addition, the long-term sequelae of endometriosis may influence fertility. Pediatricians, primary care physicians and gynecologists need to maintain a high index of suspicion in order to aggressively diagnose these congenital anomalies in young adolescents. In order to maintain this vigilance and assist in maintaining future fertility, it is important to review the embryologic sources of these anomalies, understand the modes of diagnosis and recognize the treatment options currently available.

Seno pre-púbico congénito / Congenital prepubis sinus

Niña de raza negra de 2 1/2 meses, hospitalizada en Cirugía Infantil para evaluación por historia de descarga de secreción intermitente en la región suprapúbica. La paciente fue producto de parto y nacimiento normales; no solo hablan antecedentes de infección del tracto urinario. En el examen físico destacaba presencia de pequeño orificio en la región suprapública que al comprimirlo, descargaba secreción de aspecto blanquizco-filante. Se envió muestra de secreción a cultivo resultando este estéril

[Colpoplasty for vaginal aplasia by rectovesical dissection and thin skin graft with adjustable stent. Review of the literature apropos of 4 cases of surgically treated Mayer-Rokitansky-Küster-Hauser syndrome]. / La colpoplastie pour aplasie vaginale par clivage recto-vesical et greffe de peau mince avec conformateur gonflable. Revue de la littérature à propos de quatre cas opérés de syndrome de Mayer-Rokitansky-Küster-Hauser.

The congenital absence of the vagina has a low incidency but it is a very invalidating condition. The Mayer-Rokitansky-Küster-Hauser syndrome represents 90% of all cases of vaginal aplasia. The authors review the anatomical classification, etiopathogenesis, epidemiology, diagnosis and treatment. Between 1981 and 1991, four cases were treated by recto-vesical dissection and split thickness skin graft technique with adjustable stent. The results are good and correspond to those of the literature. Their quality and duration depend on maintaining the stent for a sufficient length of time. Frequent post-operative follow-up is necessary to prevent graft retraction and to give psychological support when it is needed.

[Elephantiasis of the external genitalia in children]. / Slonovost' naruzhnykh polovykh organov u detei.

This study presents an experience with diagnosis and management of external genital elephantiasis in 18 children in the age range of 3 to 15 years. All patients underwent surgical treatment. The type and scope of surgery depended on the extent and severity of elephantiasis. A total of 21 operations were done. Long-term results were evaluated in these 13 patients at 1 to 22 years. The evaluation included physical appearance of the genitalia, scars, secondary deformities, penile and testicular size, the presence of erections and menstruation. Adults of mature ages were evaluated for fertility and reproductive status. Long-term results were good in all 13 patients. This analysis suggests that early radical surgical treatment of children with congenital elephantiasis of the external genitalia enables a normal anatomic and functional genital development and averts severe local and systemic disorders.

Paraurethral cysts in the newborn.

I have reported three case of paraurethral cysts in female neonates. These are uncommon lesions, and urologic evaluation is required to differentiate them from ectopic ureteroceles, urethral diverticula, or paraurethral tumors. Histologic appearance of the cyst epithelium identifies the embryologic origin. The cysts either rupture spontaneously or require simple marsupialization.

Ultrasound contribution to the diagnosis of neonatal hydrometrocolpos with proximal vaginal atresia.

The value of ultrasound examination in a case of neonatal hydrometrocolpos is presented. Ultrasound proved the pelvic origin and the cystic nature of the mass, determinations essential to a correct diagnosis.