Pediatric vulvar lichen sclerosus: Does it resolve or does it persist after menarche?

BACKGROUND AND OBJECTIVES: Although data regarding the rates of remission and progression of the disease are still scarce, it is generally now acknowledged that pediatric vulvar lichen sclerosus (pVLS) can persist beyond puberty. Recent studies reveal that this condition may persist in as many as 75% of cases. The present study aims to answer the following query: how does pVLS evolve after menarche? METHODS: This observational retrospective study conducted on premenarchal girls diagnosed with pVLS in our institution between 1990 and 2011 describes 31 patients who returned for multidisciplinary clinical evaluation following menarche. RESULTS: The mean follow-up time was 14 years. At the post-menarche clinical examination, patients were classified as follows: 58% were still affected by VLS, 16% presented with a complete remission of disease, and 26% were completely asymptomatic although with persistent clinical signs of VLS. CONCLUSIONS: In our series, pVLS persists following menarche in the majority of patients. These findings suggest the importance of a long-term follow-up even among patients who report resolution of symptoms following menarche.

Psychological impact and health-related quality-of-life outcomes of Mayer-Rokitansky-Küster-Hauser syndrome: A systematic review and narrative synthesis.

Mayer-Rokitansky-Küster-Hauser syndrome causes absence or underdevelopment of uterus and vagina, but women's subjective experience remains understudied. This systematic review was conducted to examine the psychological and health-related quality-of-life outcomes of Mayer-Rokitansky-Küster-Hauser syndrome. In total, 22 articles identified through electronic search matched the inclusion criteria and were included in our review. Mayer-Rokitansky-Küster-Hauser syndrome may be associated with psychological symptoms and impaired quality of life, but especially with poor sexual esteem and genital image. Women may experience difficulties managing intimacy and disclosing to partners. Mothers may be perceived as overinvolved, with consequent negative emotions in women with the disease.

Dysmorphologic assessment in 115 Mayer-Rokitansky-Küster-Hauser patients.

Mayer-Rokitansky-Küster-Hauser (MRKH) patients are characterized by congenital aplasia of the uterus and the upper part of the vagina, with normal secondary sexual characteristics. This disorders affects one in 4000-5000 females and it is classified as typical, type I or isolated, and as atypical, type II, manifesting additional malformations. To date, no specific study has addressed the question of facial features in MRKH patients. The aim of this study is to perform a dysmorphological assessment of a large cohort of patients. We studied 115 women referred to our center from 2008 to 2012. Seventy-two percentage (83/115) of our patients showed isolated uterovaginal aplasia (MRKH type I); 32/115 (28%) had other abnormalities including kidney and cardiac defects, skeletal anomalies, and hearing impairment. Auxologic investigations comprised measurements of height, weight, BMI, head circumference, arm span, span to height ratio, hand length, middle finger length, foot length, inner and outer intercanthal distance, and auricle length. All patients had normal measurements, except for the outer canthal distance-inner canthal distance ratio, which was consistent with elongated eyelids. Women with MRKH syndromes do not present a typical facial feature and a dysmorphological examination of all patients seems unnecessary. However, a multidisciplinary approach is useful with respect to explaining the etiology, interpreting test results, and counseling.

A uterovaginal septum and imperforate hymen with a double pyocolpos.

The presence of both a uterovaginal septum and imperforate hymen is described in a young patient presenting with ongoing chronic pelvic pain and a double pyocolpos. Ultrasound and magnetic resonance imaging scans were performed. The patient underwent laparoscopic adesiolysis, hymenotomy with drainage of 200 mL of pus, and excision of a complete longitudinal vaginal septum. Over the past 5 years of regular follow-up examinations, the patient has always reported regular menstrual cycles and an absence of pelvic pain.

Creation of a neovagina in Rokitansky syndrome: comparison between two laparoscopic techniques.

OBJECTIVE: To compare Vecchietti's and Davydov's laparoscopic techniques for creation of a neovagina in patients with Rokitansky syndrome. DESIGN: Comparative retrospective study. SETTING: Tertiary referral center for the treatment of Rokitansky syndrome. PATIENT(S): Eighty patients with Rokitansky syndrome. INTERVENTION(S): Patients underwent surgical creation of a neovagina. Fifteen patients who underwent the Vecchietti procedure from October 2003 to December 2004 and 30 patients who underwent the Davydov procedure from June 2005 to August 2008 were also included from two previously published studies. Follow-up lasted at least 12 months. MAIN OUTCOME MEASURE(S): Intraoperative parameters and anatomic results were compared. Functional results were compared through the Female Sexual Function Index. Epithelization of the neovagina was assessed in both groups through vaginoscopy and Schiller's test. RESULT(S): No major intraoperative complications were encountered in either group. Mean (±SD) duration of surgery was 30 ± 9.6 and 134 ± 24 minutes in Vecchietti's and Davydov's approach, respectively. At 12 postoperative months, length and width of the neovagina in the two groups were 7.5 ± 1.1 and 2.8 ± 0.6 cm, and 8.5 ± 1.6 and 2.8 ± 0.65, respectively. Epithelization of the neovagina at 6-month follow-up was 60% and 80%, respectively, and 100% in both groups at 12 postoperative months. CONCLUSION(S): Anatomic and functional outcomes of the two approaches tend to be comparable at 12-month follow-up; the only significant difference seems to be in greater length for the neovagina obtained by Davydov's approach.

Creation of a neovagina by Davydov's laparoscopic modified technique in patients with Rokitansky syndrome.

OBJECTIVE: The purpose of this study was to assess anatomic and functional results after the laparoscopic Davydov procedure for the creation of a neovagina in Rokitansky syndrome. STUDY DESIGN: Thirty patients with Rokitansky syndrome underwent the laparoscopic Davydov technique from June 2005-August 2008. Mean follow-up time lasted 30 months (range, 6-44 months) and included clinical examinations and evaluation of the quality of sexual intercourse; vaginoscopy, Schiller's test, and neovaginal biopsies were performed after 6 and 12 months. Functional results were assessed with the use of Rosen's Female Sexual Function Index and were compared with age-matched normal control subjects. RESULTS: No perioperative complications occurred. At 6 months, anatomic success was achieved in 97% of the patients (n = 29); functional success and optimal results for the Female Sexual Function Index questionnaire were obtained in 96% of patients. Vaginoscopy and biopsy results showed a normal iodine-positive vaginal epithelium. CONCLUSION: The Davydov technique seems to be a safe and effective treatment for vaginal agenesis in patients with Rokitansky syndrome.

Creation of a neovagina in Rokitansky patients with a pelvic kidney: comparison of long-term results of the modified Vecchietti and McIndoe techniques.

OBJECTIVE: To evaluate perioperative data and long-term results of Rokitansky patients with a pelvic kidney that underwent the McIndoe and modified Vecchietti procedures. DESIGN: Retrospective descriptive study. SETTING: A tertiary referral center for the study and treatment of Rokitansky syndrome. PATIENT(S): Eleven patients with Rokitansky syndrome. INTERVENTION(S): Two and nine patients, respectively, underwent the McIndoe and Vecchietti modified techniques. MAIN OUTCOME MEASURE(S): Anatomic success was defined as a neovagina > or =6 cm long allowing easy introduction of two fingers within 6 months postoperatively. Functional success was considered achieved when the patient reported satisfactory sexual intercourse starting from 6 months postoperatively. RESULT(S): Surgery was performed with no complications in all 11 patients. The mean duration of surgery was 190 +/- 14.1 minutes in the first group and 32 +/- 6.4 minutes in the second group. At 14 years of follow-up, both patients who underwent McIndoe vaginoplasty had a mean +/- SD length and width of 8.2 +/- 0.4 cm and 5 cm and negative Schiller's test 24 months postoperatively. At 4 years of follow-up, eight out of the nine patients who underwent the Vecchietti procedure (89%) had a mean +/- SD length and width of the neovagina of 7.4 +/- 0.6 cm and 4.2 +/- 0.5 cm and iodine-positive vaginal-type epithelium coating 100% of the neovagina 24 months postoperatively. CONCLUSION(S): While appearing to be safe, effective, and with optimal functional results, the modified Vecchietti approach also seems to yield good anatomical and aesthetic results along with shorter surgical and hospitalization times.

The unicornuate uterus with an occult adenomyotic rudimentary horn.

We report 2 case of an atypical variant of unicornuate uterus in 2 adolescent patients with severe dysmenorrhea. Pelvic ultrasonography and magnetic resonance imaging identified a normal uterine contour. On the right side within the uterine fundus, a nodule was detected with a small hypoechogenic content. At laparoscopy the uterus and adnexae appeared to be normal. No endometriotic lesions were identified. Hysteroscopy identified a single regular cervical canal and a uterine cavity resembling that of a left unicornuate uterus, with a single regular left tubal ostium. Complete resection of the right uterine nodule along with an ipsilateral salpingectomy was performed. The nodule contained a small endometrial cavity and hematometra. Histologic study showed a cavitated adenomyotic uterine rudiment. The patients were discharged on the second postoperative day. No intraoperative or postoperative complications or recurrence of pelvic pain occurred.

Multiple extrauterine adenomyomas and uterus-like masses: case reports and review of the literature.

OBJECTIVE: To describe two rare cases of multiple extrauterine adenomyomas, their clinical management, and their response to surgical and hormone therapy with GnRH agonist. DESIGN: Case report and review of the English-language scientific literature. SETTING: Cases presented in a tertiary health care academic institution. PATIENT(S): Two patients without urogenital malformations diagnosed with extrauterine adenomyoma, which is a benign tumor composed of smooth muscle and endometrium, typically originating within the uterus. INTERVENTION(S): Surgical radical excision of adenomyomas followed by a long-term hormone therapy with GnRH agonist. MAIN OUTCOME MEASURE(S): Anatomical and clinical outcomes. RESULT(S): Surgical treatment followed by long-term GnRH agonist therapy appeared effective in keeping the disease stable. After a long-term follow-up of 10 and 4 years, the two patients are still asymptomatic and stable. CONCLUSION(S): Only 19 other cases of extrauterine adenomyomas are reported in the English-language scientific literature, and no cases of multiple masses are described until now. It is probable that these extrauterine adenomyomas arose from a metaplastic transformation of the subcelomic mesenchyme. Long-term GnRH agonist therapy after surgery appeared effective in keeping the disease stable.

The laparoscopic Vecchietti's modified technique in Rokitansky syndrome: anatomic, functional, and sexual long-term results.

OBJECTIVE: The objective of the study was to assess the anatomical and functional long-term follow-up results of the laparoscopic Vecchietti approach for the creation of a neovagina in the Rokitansky syndrome. STUDY DESIGN: One hundred ten patients underwent clinical follow-up visits at 1, 3, 6, and 12 months after surgery and every 6 months thereafter. The following were performed: evaluation of the quality of sexual intercourse, vaginal and rectal examinations, vaginoscopy, Schiller's test, and vaginal cytology with microbiologic testing. Functional results were assessed by using Rosen's Female Sexual Function Index questionnaire, of which the results were analyzed comparing normal age-matched controls. RESULTS: Four patients were lost to follow-up. Anatomic and functional success was achieved in 104 of 106 (98%) and 103 of 106 (97%) patients, respectively. Female Sexual Function Index scores were comparable with those of controls. CONCLUSION: Vecchietti's technique is simple, safe, and effective and allows normal and satisfying sexual intercourse, comparable with that of normal controls.