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.

Gorlin syndrome: two unusual cases of recurrent, bilateral, multinodular, calcified ovarian fibromas with conservative surgical treatment.

Gynecologically, NBCCS is variously associated (14%-75% cases) to typically bilateral, multinodular, calcified, ovarian fibromas. We report 2 rare cases of unusually recurrent bilateral ovarian fibromas treated with conservative surgery. Preservation of the normal ovarian tissue is always recommended, even though there is the risk of recurrences, given the benign nature of the lesions and the young age of patients.

Davydov's procedure for the treatment of neovaginal prolapse in Rokitansky syndrome.

A 19-year-old woman with Rokitansky syndrome with neovaginal prolapse following self-dilation showed vaginal vault eversion of a 4- × 3-cm neovagina. A modified Davydov procedure was performed. No complications occurred. Vaginoscopy after 6 months showed an iodine-positive 8- × 3-cm neovagina. Functional results were assessed with the Female Sexual Function Index.

SHOX duplications found in some cases with type I Mayer-Rokitansky-Kuster-Hauser syndrome.

PURPOSE: The Mayer-Rokitansky-Küster-Hauser syndrome is defined as congenital aplasia of müllerian ducts derived structures in females with a normal female chromosomal and gonadal sex. Most cases with Mayer-Rokitansky-Küster-Hauser syndrome are sporadic, although familial cases have been reported. The genetic basis of Mayer-Rokitansky-Küster-Hauser syndrome is largely unknown and seems heterogeneous, and a small number of cases were found to have mutations in the WNT4 gene. The aim of this study was to identify possible recurrent submicroscopic imbalances in a cohort of familial and sporadic cases with Mayer-Rokitansky-Küster-Hauser syndrome. METHODS: Multiplex ligation-dependent probe amplification was used to screen the subtelomeric sequences of all chromosomes in 30 patients with Mayer-Rokitansky-Küster-Hauser syndrome (sporadic, n = 27 and familial, n = 3). Segregation analysis and pyrosequencing were applied to validate the MLPA results in the informative family. RESULTS: Partial duplication of the Xpter pseudoautosomal region 1 containing the short stature homeobox (SHOX) gene was detected in five patients with Mayer-Rokitansky-Küster-Hauser syndrome (familial, n = 3 and sporadic, n = 2) and not in 53 healthy controls. The duplications were not overlapping, and SHOX was never entirely duplicated. Haplotyping in the informative family revealed that SHOX gene duplication was inherited from the unaffected father and was absent in two healthy sisters. CONCLUSIONS: Partial duplication of SHOX gene is found in some cases with both familial and sporadic Mayer-Rokitansky-Küster-Hauser type I syndrome.

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.

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.

Hormonal treatments for adenomyosis.

Like endometriosis and uterine myomas, adenomyosis presents the typical characteristics of oestrogen-dependent diseases. The medical treatment of adenomyosis is based on the hormonal dependency of the disease and its strongly debated similarities with endometriosis. Infact, despite the evident differences between the two conditions, the therapies that treat endometriosis effectively have also been successful for the treatment of adenomyosis. Although the two diseases have distinct epidemiological features, they have the same 'target tissue' for hormonal therapy, namely ectopic endometrium. Recognized approaches are systemic hormonal treatments, which are generally used for endometriosis and are capable of suppressing the oestrogenic induction of the disease, and local hormonal treatment that targets the ectopic endometrium directly. Gonadotropin-releasing hormone agonists, danazol and intrauterine levonorgestrel- or danazol-releasing devices have been used in the treatment of adenomyosis. Despite the solid rational basis for its hormonal treatment, few studies have been performed on medical therapy for adenomyosis.

Radical excision of inguinal endometriosis.

BACKGROUND: The diagnosis of inguinal endometriosis can be complex, and patients are often first operated by a general surgeon for a hernia. We present five cases of inguinal endometriosis in which primary surgery resulted in recurrence and a second correct procedure resulted in a cure. CASES: Five patients with inguinal endometriosis, operated between 1996 and 2002, were seen for the recurrence of symptoms. All underwent excision of the extraperitoneal portion of the round ligament and pelvic exploration. In all cases, both pelvic and round ligament endometriosic lesions were confirmed at histology. No complications or recurrence of inguinal endometriosis occurred. CONCLUSION: The complete excision of inguinal endometriosis must also include the extraperitoneal portion of the round ligament.

Laparoscopic findings and pelvic anatomy in Mayer-Rokitansky-Küster-Hauser syndrome.

OBJECTIVE: To describe analytically the anatomic variety and laparoscopic findings observed in patients with Rokitansky syndrome throughout an 11-year span. METHODS: We analyzed the laparoscopic and chart records of 106 consecutive patients who underwent surgery for the creation of a neovagina, according to the modified laparoscopic Vecchietti procedure. RESULTS: A hypoplastic vagina was observed in 61 women. Müllerian remnants, laterally displaced in the pelvis, were identified in 92 cases; 42 (25.9%, 95% confidence interval [CI] 19.2-33.3) of 162 müllerian remnants were cavitated and contained endometrial mucosa. Ovaries were extrapelvic in 17 (16.0%, 95% CI 9.6-24.4) cases. Anomalies of the urinary tract were identified in 32 (30.2%, 95% CI 21.7-39.9) patients; unilateral renal agenesis was the most frequent finding (18 cases; 18%, 95%CI 10.4-25.5). CONCLUSION: Rokitansky syndrome has a wide variability of anatomic presentations, and, as the do other congenital anomalies of the female genital tract, it represents a continuum of embryonic malformations, which occur at different stages of development.

Evidence for asymmetric distribution of sciatic nerve endometriosis.

OBJECTIVE: To investigate if a lateral asymmetry exists in the distribution of endometriotic lesions of the sciatic nerve. DATA SOURCES: All articles on sciatic nerve endometriosis identified by MEDLINE and EMBASE database searches were retrieved, and additional reports were collected by systematically reviewing all references. Monographs on endometriosis published in the last 15 years were consulted. METHODS OF STUDY SELECTION: We considered articles in which the presence of an endometriotic lesion of the sciatic nerve and the affected side were assessed. We also included reports lacking histological examination of sciatic nerve specimens but with a surgical diagnosis of pelvic endometriosis. Two authors abstracted data independently on standardized forms. The number of women and the side of the lesion were obtained from individual studies, and the combined frequency of left- and right-side sciatic nerve endometriosis in published reports was computed. TABULATION, INTEGRATION, AND RESULTS: Thirty-two reports including 63 subjects were selected. Endometriosis of the sciatic nerve was on the right side in 41 patients, on the left in 20, and bilateral in two. Considering only patients with unilateral sciatic nerve endometriosis, the observed proportion of right-side lesions (41 of 61 [67.2%]; 95% confidence interval 54.0%, 78.7%) significantly differed from the expected proportion of 50% (chi(2)(1) 7.23, P =.007). Among the 16 cases of histological demonstration of endometriosis infiltrating sciatic nerve roots or fibers, ten had it on the right side (62.5%) and six on the left. Twenty-six of the 38 subjects (68.4%) with surgical demonstration of pelvic endometriosis but without histopathologic evidence of direct sciatic nerve involvement were affected by right cyclic sciatica. CONCLUSION: The finding that two thirds of patients with sciatic nerve endometriosis had right-side lesions constitutes further evidence against the coelomic metaplasia theory. The interposition of the sigmoid colon between the regurgitated endometrial cells implanted on the left posterolateral pelvic peritoneum seems to protect the left lumbosacral plexus and sciatic nerve.

Continuous use of an oral contraceptive for endometriosis-associated recurrent dysmenorrhea that does not respond to a cyclic pill regimen.

OBJECTIVE: To ascertain whether long-term reduction of pain is obtained by continuous administration of an oral contraceptive (OC) in women with endometriosis-associated recurrent dysmenorrhea that does not respond to cyclic OC use. DESIGN: Prospective, therapeutic, self-controlled clinical trial. SETTING: A tertiary care and referral center for patients with endometriosis. PATIENT(S): Fifty women who underwent surgery for endometriosis in the previous year and experienced recurrent dysmenorrhea despite cyclic OC use. INTERVENTION(S): Continuous use of an OC containing ethinyl estradiol (0.02 mg) and desogestrel (0.15 mg) for 2 years. MAIN OUTCOME MEASURE(S): Dysmenorrhea variation during cyclic and continuous OC use, evaluated with a 100-mm visual analog scale and a 0- to 3-point verbal rating scale, and degree of satisfaction with continuous OC treatment. RESULT(S): In the study period, amenorrhea, spotting, and breakthrough bleeding were reported by 19 (38%), 18 (36%), and 13 (26%) women. The mean +/- SD number of >7-day bleeding episodes with consequent 7-day OC suspension was 5.5 +/- 2.1. The mean +/- SD dysmenorrhea visual analog scale and verbal rating scale scores were 75 +/- 13 and 2.4 +/- 0.5 at baseline and 31 +/- 17 and 0.7 +/- 0.6 at 2-year follow-up, respectively. Moderate or severe side effects were reported by 7/50 (14%) women. At final evaluation, 13 (26%) women were very satisfied, 27 (54%) were satisfied, 1 (2%) was uncertain, 8 (16%) were dissatisfied, and 1 (2%) was very dissatisfied. CONCLUSION(S): Long-term continuous OC use can be proposed to women with symptomatic endometriosis and menstruation-related pain symptoms.

Comparison of a levonorgestrel-releasing intrauterine device versus expectant management after conservative surgery for symptomatic endometriosis: a pilot study.

OBJECTIVE: To determine whether the frequency and severity of dysmenorrhea are reduced in women with symptomatic endometriosis in whom a levonorgestrel-releasing intrauterine device (Lng-IUD) is inserted after operative laparoscopy compared with those treated with surgery only. DESIGN: Open-label, parallel-group, randomized, controlled trial. SETTING: A tertiary care and referral center for patients with endometriosis. PATIENTS(S): Parous women with moderate or severe dysmenorrhea undergoing first-line operative laparoscopy for symptomatic endometriosis. INTERVENTION(S): Randomization to immediate Lng-IUD insertion or expectant management after laparoscopic treatment of endometriotic lesions. Proportions of women with recurrence of moderate or severe dysmenorrhea in the two study groups 1 year after surgery and overall degree of satisfaction with treatment. Moderate or severe dysmenorrhea recurred in 2 of 20 (10%) subjects in the postoperative Lng-IUD group and 9/20 (45%) in the surgery-only group. Thus, a medicated device inserted postoperatively will prevent the recurrence of moderate or severe dysmenorrhea in one out of three patients 1 year after surgery. A total of 15/20 (75%) women in the Lng-IUD group and 10/20 (50%) in the expectant management group were satisfied or very satisfied with the treatment received. CONCLUSION(S): Insertion of an Lng-IUD after laparoscopic surgery for symptomatic endometriosis significantly reduced the medium-term risk of recurrence of moderate or severe dysmenorrhea.

Endometriosis: preoperative and postoperative medical treatment.

The quality of the evidence that supports the use of medical treatment before conservative surgery for endometriosis is manifestly poor, and no recommendations can be made based on the results of the published studies. There are practical advantages inherent to this schedule, but whether this translates into better conception rates and reduced pain recurrence rates is unproven. The effect of drug therapy after surgery can be assessed better as data from seven true randomized, controlled trials are available. The results of the current review do not support the notion that suppressing ovarian activity postoperatively increases the long-term pregnancy rate. As far as pelvic pain is concerned, more data are needed to verify the reduced symptoms recurrence rate found in four trials in women who were allocated to postoperative medical therapy, particularly in view of the different results obtained in some of the considered studies. The observed differences among various drugs used before or after surgery are limited in clinical terms and, in the absence of formal randomized comparisons, are difficult to interpret. Because of their tolerable side effects and limited cost, progestins with or without estrogens should be considered strongly as first-line postoperative medical treatment if and when suppression of ovulation after conservative surgery is deemed opportune.

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.

Endometriosis in young women: the experience of GISE.

STUDY OBJECTIVE: To analyze the clinical manifestations of endometriosis in adolescents. DESIGN: prospective clinical study. SETTING: University and General hospitals. PARTICIPANTS: A total of 38 females aged < or = 21 years who had a first surgically confirmed diagnosis of endometriosis during the period including years 2005-2006 at 12 participating centers were identified. Endometriotic lesions were staged according to the 1985 revised American Fertility Society [renamed American Society for Reproductive Medicine (ASRM)] classification. RESULTS: The mean age at diagnosis was 18.6 years, except in 3 cases (7.9%) in which it was made at < or = 15 years of age. None of the patients had a prior diagnosis of genital malformations, nor were any cases of diagnosed familiarity for endometriosis. Pelvic pain was present in all cases, although in 3 cases the presence of a pelvic mass was the indication for surgery. CONCLUSIONS: The main clinical finding emerging from this analysis suggests that pelvic pain is the main symptom. In our series pain was present in all cases and an ovarian endometrioma was present in three cases. With regard to the stage and site of the disease, the frequency of minimal-mild endometriosis was lower than in adult cases observed in the experience of GISE.

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.

Pelvic endometriosis and hydroureteronephrosis.

OBJECTIVE: To assess whether routine renal ultrasonography may be recommended in all patients with pelvic endometriosis, in order to avoid silent ureteral involvement of the disease. DESIGN: Retrospective descriptive study. SETTINGS: Tertiary center for the treatment of endometriosis at the Department of Obstetrics and Gynecology of the State University of Milan, Milan, Italy. PATIENT(S): Seven-hundred-fifty patients with a primary diagnosis of endometriosis, between January 2005 and July 2007. INTERVENTION(S): Routine urinary ultrasound; recording of patient history, signs, and symptoms; gynecologic examination; blood and urinary analyses; magnetic resonance imaging; spiral multislice computerized tomography. MAIN OUTCOME MEASURE(S): Symptoms and signs of ureterohydronephrosis; diagnosis of ureterohydronephrosis. RESULT(S): Twenty-three patients (3%) of all 750 patients with endometriosis had associated ureterohydronephrosis diagnosed at renal ultrasound. Symptoms secondary to ureteral and renal involvement were present in 10 patients (43.5%); 6 reported lumbar pain (26.1%) and 4 patients (17.4%) had renal colic. CONCLUSION(S): In our study, the high number (56.5%) of asymptomatic ureteral involvement in patients with known pelvic endometriosis seems to warrant the need for further investigations regarding the possibility to avoid the high percentage of silent renal losses. Unfortunately there appears to be no specific risk factor to allow for early suspicion nor a validated preventive diagnostic and therapeutic program. It remains to be evaluated whether urinary ultrasound ensures a beneficial cost-benefit ratio if employed on a routine basis.

Umbilical endometriosis: a radical excision with laparoscopic assistance.

BACKGROUND: Umbilical endometriosis represents the most common site of cutaneous endometriosis. Although its treatment is typically surgical, in literature the approach used is variable and extends from diathermocoagulation to omphalectomy. Such superficial treatments for umbilical endometriosis can predispose the patient to a relapse of the disease. We here present seven cases of umbilical endometriosis treated with radical surgery with a laparoscopically-assisted approach, with a complete and long-term disease-free follow-up. CASES: Seven cases of umbilical endometriosis, four of which relapsing from a prior superficial treatment, were treated radically with a laparoscopically-assisted approach, with a long-term disease-free follow-up. CONCLUSION: Although a medical treatment can be considered, the treatment of choice in these patients should be that of excisional surgery so as to avoid lesion relapse and the risk of oncogenic transformation. Despite umbilical endometriosis is a rare finding, this relatively small case series treated by laparoscopically-assisted omphalectomy shows a complete resolution of the lesion and symptoms along with good aesthetic results at a long-term follow-up.