Conservative Treatment of Didelphyc Uterus with Obstructed Hemivagina and Ipsilateral Renal Agenesis.

STUDY OBJECTIVE: To show a conservative surgical treatment for a female adolescent affected by Wunderlich syndrome with didelphys uterus and obstructed hemivagina. DESIGN: Stepwise demonstration of the technique with narrated video footage. SETTING: In the context of obstructive congenital müllerian anomalies, involving a stagnation of menstrual blood, the Wunderlich syndrome is the most common and constantly characterized by the duplicity of the uterine body, by the presence of an imperforate hemivagina, and by renal agenesis ipsilateral to the obstructed hemivagina. The imperforate hemivagina leads to dysmenorrhea and abdominal pain, owing to the hematocolpos and the hematometra, which arose immediately after the menarche. This is the case of a 14-year-old female adolescent affected by Wunderlich syndrome referred to the San Raffaele Hospital adolescent center (Milan, Italy) for dysmenorrhea and abdominal pain. At vaginal exploration, a right imperforated hemivagina and hematocolpos were highlighted. A diagnostic magnetic resonance imaging (MRI) found the duplicity of the uterine body, the hematometra, and the right renal agenesis. INTERVENTIONS: A combination of explorative laparoscopy and surgical vaginal time leads to the excision of the vaginal septum that allows the drainage of the hematocolpos and of the hematometra. Three surgical steps are described: 1. First laparoscopic step: exploration of the abdominal cavity with the detection of a double uterine body, an enlarged hemiuterus, and an enlarged hemivagina caused by the hematometra and the hematocolpos. Careful evaluation of adnexa for eventual presence of hematosalpinx. 2. Vaginal step divided into the following: (A) puncture of the vaginal tumescence (corresponding to the imperforated hemivagina) with a 19-gauge needle mounted on a syringe. Aspiration results in thick creamy black material (old menstrual blood). (B) In correspondence with the needle puncture, a full-thickness incision of the vaginal wall widely opening the second uterine cervix and (C) stabilization of the opening by the marsupialization of the edge of the obstructed hemivagina were performed. 3. Second laparoscopic step: having emptied the hematocolpos of the left hemiuterus, the didelphys uterus and the disappearance of the hematocolpos can be clearly seen. CONCLUSION: Here, we demonstrate a conservative surgical approach for the treatment of Wunderlich syndrome. This rare malformation is characterized by an extreme variability of the anatomic presentation, and the precise identification of the variety together with the early diagnosis is of fundamental importance for the surgical correction.

Diagnosis and Surgical Treatment of Uterine Isthmus Atresia: A Case Report and Review of the Literature.

Uterine isthmus atresia is a rare Müllerian duct anomaly occasionally diagnosed in adolescents with primary amenorrhea and cyclic abdominal pain. A case is presented of a 14-year-old female with monthly cyclic lower abdominal pain of a 2-year duration. Magnetic resonance imaging and 3-dimensional ultrasound showed separation of a 10-mm fibrotic tissue between the cervical canal and the endometrial cavity. In an attempt to preserve reproductive ability, an end-to-end anastomosis was laparoscopically performed between the cervical canal and uterine cavity. A platinol stent (WallFlex Biliary RX; Boston Scientific, Boston, MA) was placed by hysteroscopy to avoid stenosis after surgery. Laparoscopic end-to-end anastomosis is proposed to treat congenital uterine isthmus atresia.

Laparoscopy-assisted Neocervicovaginal Reconstruction in a Rare Case of Müllerian Anomaly: Cervicovaginal Aplasia with Unicornuate Uterus.

STUDY OBJECTIVE: To demonstrate a technique of laparoscopy-assisted neocervicovaginal reconstruction in a case of cervicovaginal aplasia. DESIGN: Step-by-step demonstration of the surgery in an instructional video. SETTING: Cervicovaginal aplasia is a rare congenital anomaly that occurs in 1 in 80 000 to 100 000 live births [1]. Occasionally, there can be other associated anomalies. Epigenetic modifications of homeobox and Wnt genes (wingless-type mouse mammary tumor virus integration site family) are hypothesized to cause defects in the development of the müllerian reproductive tract by interfering with cell migration during organogenesis [2]. Our patient was a 15-year-old girl who had a unicornuate uterus along with cervicovaginal aplasia (American Society for Reproductive Medicine class Ia, Ib, and IIc and European Society of Human Reproduction and Embryology class U4a, C4, and V4). The most common approach to treat this particular anomaly is hysterectomy, but there are many reports of neocervicovaginal reconstruction with good results [3]. INTERVENTIONS: Laparoscopic assessment showed a right unicornuate uterus with hematometra, right hematosalpinx, and a left noncavitary rudimentary horn with endometriosis. A vertical incision was made over the most prominent bulging part of the uterine fundus, and the hematometra was drained. Laparoscopic inspection of the uterine cavity showed an irregular cavity with thickened endometrium. The cervical canal could not be identified. The inspection of the external genitalia revealed complete vaginal aplasia. An inverted T incision was made over the vestibule and neovagina created by blunt digital dissection. The bladder was laparoscopically mobilized down. A Maryland dissector was inserted into the uterine cavity through the incision in the fundus and directed toward the neovagina. The myometrium was then punctured while simultaneously visualizing the neovagina to create a neocervix. A 16-F Foley's catheter was then pulled into the uterine cavity with the Maryland dissector from the vaginal end. The neocervix was enlarged around the catheter and then sutured to the vestibular epithelium with 6 interrupted 1-0 polyglactin sutures. The Foley's catheter was anchored to the myometrium laparoscopically, and the uterine incision was closed with interrupted 1-0 polyglactin sutures. The right hematosalpinx was then excised. The intravaginal plastic mold was removed after 4 days. The patient was advised to use the bulb of a plastic pipette to help maintain vaginal patency. She resumed her menses 3 weeks after the surgery. Follow-up at 8 months revealed a normal-sized uterus on transabdominal ultrasound and a vaginal length of 5.5 cm on speculum examination. She currently reports regular cyclic menstruation with mild dysmenorrhea and has not yet begun sexual activity. CONCLUSION: Cervicovaginal aplasia can be successfully treated by laparoscopy-assisted neocervicovaginal reconstruction as demonstrated in the video.

Endometriosis and Glanzmann’s thrombasthenia.

Glanzmann’s thrombasthenia (GT) is a rare bleeding syndrome characterized by deficiency or defect of platelet aggregation complex. The pathogenesis of endometriosis is controversial but the strongest evidence leans towards retrograde menstruation. GT probably predisposes to endometriosis. The management of women affected by this disease can be difficult due to the risk of bleeding complications, especially during surgical treatment. We describe the cases of three sisters affected by endometriosis and GT, referred to our Department, who received different therapeutic management.

Isolated Recurrent Hematotrachelos After Abdominal Myomectomy A Case Report.

BACKGROUND: Isolated hematotrachelos is an ex- tremely rare condition. More commonly it is associated with hematometra and/or hematocolpos. It may devel- op secondary to congenital anomalies of the reproductive tract or may be an acquired condition following cervical surgery or manipulation. No case has been reported fol- lowing abdominal myomec- tomy. CASE: A 40-year-old, nul- ligravid woman was diag- nosed with isolated hemato- trachelos when she presented with severe abdominal pain following ovulation induction with clomiphene citrate. After the diagnosis was obtained, she recounted 2 similar presentations occurring months earlier. Both episodes had been attributed to gastroenteritis, but each had re- solved with "explosive" onset of menses. All 3 episodes plus 1 later recurrence happened within a 36-month period following an abdominal myomectomy. CONCLUSION: Acquired hematotrachelos is a rare condition, and the diagnosis is often missed. To our knowledge, this is the first reported case of isolated, recurrent hematotrachelos following the use of a uterine manipulator cannula in association with an abdominal myomectomy.

Case of early postoperative adhesion in a patient with molimina due to transverse vaginal septum concomitant with imperforate hymen.

Transverse vaginal septum is a residual vaginal plate composed of the Müllerian duct and urogenital sinus. Imperforate hymen results from failure of perforation of the membrane between the urogenital sinus and vaginal cavity. We report a rare case of concurrence of these two conditions. A 16-year-old girl had been treated with puncture several times for hematometra and hematocolpos from 13 years of age because of monthly occurrence of lower abdominal pain without menstrual bleeding and was referred to our hospital. Magnetic resonance imaging demonstrated hematometra, hematocolpos and expansion of the vaginal fornix. The imperforate hymen was incised and a slight adhesion at the lower vaginal cavity was detached. After that, a complete transverse vaginal septum, which was 5 mm thick, was identified. It was excised after ultrasonography-guided puncture. Although two cycles of menstrual bleeding took place, molimina recurred. Re-operation was performed 6 months after the first operation, and recurrence of adhesion in the lower vaginal cavity was identified. A silicon dilator was inserted, but she could not use it at home and instead used a tampon. Cyclic menstrual bleeding is observed 4 months after the second operation.

Hemivaginal septum resection in a patient with a rare variant of Herlyn-Werner-Wunderlich syndrome.

Herlyn-Werner-Wunderlich syndrome is characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The syndrome should be suspected in any young woman with abdominal pain or cyclic dysmenorrhea, and radiologic evidence of müllerian duct and renal anomalies. Herein is presented the case of a 25-year-old woman with a rare variant of Herlyn-Werner-Wunderlich syndrome, characterized by an anomalous connection between the 2 endocervical canals, who underwent hemivaginal septum resection to relieve progressively worsening dysmenorrhea. The right hemivaginal septum was resected medially from the left cervix and laterally off the right vaginal wall. Hydrodissection between the hemivaginal septum and right cervix facilitated surgical resection. After resection of the hemivaginal septum there was complete resolution of dysmenorrhea and no recurrence of hematometra or hematocolpos.

A postconization hematometra revealed a rare case of endocervical bone metaplasia.

BACKGROUND: Hematometra is an unusual occurrence in young women undergoing conization; moreover, osseous metaplasia of the uterine cervix is a very rare event, with 7 cases in the literature. A postconization hematometra due to endocervical ossification is a unique occurrence. The authors report such an event. CASE: A young woman undergoing conization developed progressive hypoamenorrhea with pelvic pain. Pregnancy test was negative and a transvaginal ultrasound showed an image of suspected hematometra. Diagnostic hysteroscopy showed an endocervical obstruction due to a bone formation of the uterine cervix, which was removed with an office operative hysteroscopy. After surgery, the patient restored normal menstrual cycle. Histological examination revealed a cervical bone metaplasia. CONCLUSIONS: In premenopausal women undergoing conization, the appearance of a progressive hypoamenorrhea with pelvic pain could suggest a cervical mechanical obstruction that could be an uncommon stenosis. Despite this case represents a very rare event, a postconization hematometra due to an endocervical ossification can be managed with an office operative hysteroscopy.

[Primary amenorrhea by transverse vaginal septum: a case report and review of the literature]. / Amenorrea primaria por tabique transverso vaginal inferior: reporte de un caso y revisión de la bibliografía.

The transverse vaginal septum is one of the rarest anomalies of the reproductive tract classified depending on location. It is manifested by primary amenorrhea, cyclic pain and progressive mass growth at abdominopelvic level, ultrasound and magnetic resonance imaging provides the diagnosis, location and thickness of a transverse vaginal septum, treatment is surgical. We present a case of a teenage patient with primary amenorrhea due to lower transverse vaginal septum with surgical resection and satisfactory follow-up with successful vaginal patency.

Uterine lithiasis, management by hysteroscopy: Case report and literature review.

The presence of calculi inside the uterine cavity is an uncommon finding, with few cases described in the literature. Here, we present a clinical case of a 30-year-old patient with uterine lithiasis. The patient presented with 3 months of evolution, characterized by abdominal pain in the hypogastrium, with a report of transvaginal ultrasound of hematometra with suggestive images of endometrial polyps. For this reason, she was scheduled for hysteroscopy, where three lesions of hard consistency were found, with the largest 1.5 cm. Currently, due to the few cases of uterine lithiasis described, there is no explanation of how the stones are formed in the uterine cavity. The age of the patient in this case report and the absence of predisposing factors for uterine calcifications reported in the literature is striking, which leads us to propose a hypothesis of the origin of uterine lithiasis, which is related to the history of cesarean section and the presence of hematometra, such hematic retention could have predisposed to the development of calculi in this patient. Uterine lithiasis is an extremely rare entity with few published cases; therefore, further studies are necessary.

A case of Herlyn-Werner-Wunderlich syndrome with exacerbation of hematometra after adnexectomy.

A 27-year-old nulliparous woman presented with a feeling of fullness in the lower abdomen and abdominal pain. A left ovarian tumor, uterus didelphys, left renal agenesis, and left vaginal atresia were observed on imaging. The ovarian tumor was presumed to have caused the abdominal pain, and an abdominal left adnexectomy was performed. After 3 months, she reported severe lower abdominal pain during menstruation. Transvaginal ultrasonography revealed uterine enlargement. After 17 days, the patient presented with abdominal pain and fever. She was diagnosed with peritonitis due to infection and left uterine hematometra. Because she did not improve with antibiotic treatment, left laparoscopic hysterectomy was performed. Subsequently, she did not experience the lower abdominal pain. Appropriate diagnosis and treatment based on the morphology of the reproductive tract and symptoms must be considered in patients with Herlyn-Werner-Wunderlich syndrome. Treatment must permit the outflow of menstrual blood.

Herlyn-Werner-Wunderlinch: An unusual presentation in a patient with Prader-Willi syndrome.

Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear.

An unusual case of cervical diverticulum identified by presence of 3 cavities at diagnostic hysteroscopy.

Herein is presented the case report of a patient who had severe dysmenorrhea since menarche, known right unicornuate uterus with a left rudimentary horn, and recurrent hematometra. Previous hysteroscopic drainage of the hematometra temporarily alleviated the symptoms. At subsequent hysteroscopy, 3 cavities were identified, 2 corresponding to the uterine horns and the other to a cervical diverticulum. Hysteroscopic metroplasty with drainage of the rudimentary horn hematometra provided long-term relief of the symptoms. The diagnosis was verified at diagnostic laparoscopy.

Severe gynecologic sequelae of Stevens-Johnson syndrome and toxic epidermal necrolysis caused by ibuprofen: a case report.

BACKGROUND: Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a serious, drug-induced, life-threatening condition characterized by an epidermal blistering rash with necrosis, desquamation and mucosal surface involvement. This patient represents the youngest and most significant case report in the literature of gynecologic damage due to TEN. CASE: A 31/2-year-old girl developed TEN involving 90% of her body surface area after exposure to pediatric ibuprofen. After onset of puberty she required surgery to treat vulvar, vaginal and cervical adhesions, stenosis and hematometra. CONCLUSION: While delaying evaluation and treatment of the extremely young child with this disorder until puberty has been the standard, consideration should be given to earlier evaluation and intervention.

[Uterus Didelphys, obstructed hemivagina and ipsilateral renal agenesis as a presentation of a case of the Herlyn-Wemer-Wünderlich syndrome. Literature review]. / Utero didelfo, hemivagina obstruida y agenesia renal ipsilateral como presentación de un caso de síndrome de Herlyn-Werner-Wünderlich. Revisión de la bibliografía.

UNLABELLED: The Herlyn-Werner-Wünderlich syndrome is a rare congenital anomaly of the Müllerian ducts. Müllerian malformations result from defective fusion of the Müllerian ducts during development of the female reproductive system. These malformations are associated with an increased probability of adverse obstetric events. The syndrome is characterized by uterus didelphys with obstructed hemivagina, and ipsilateral renal agenesis. Patients with this syndrome are asymptomatic until menarche, and can follow other complications. CASE REPORT: A 12 year-old female patient presented with a clinical picture of acute abdomen and a previous history of cyclical dysmenorrhea. A diagnosis of uterus didelphys associated with right hematometracolpos was made following an exploratory laparotomy. Resection of the right obstructed hemivagina was subsequently performed using a vaginal approach. A subsequent computed tomography seen identified renal agenesis ipsilateral to the obstructed hemivagina. IN CONCLUSION: The Herlyn-Werner-Wünderlich syndrome is a rare Müllerian anomaly. The characteristics of the syndrome may result in missed diagnosis until detailed evaluation is carried out. We therefore advise clinicians to look for Müllerian duct anomalies whenever a renal malformation is discovered in a fetus or girl postnatal. Early diagnosis of the syndrome and its associated anomalies are essential to provide adequate therapy, and to reduce reproductive complications through radical treatments. Conservative treatment is effective and has a good prognosis for the patient's reproductive function.

Management of Hematometra and Hematocolpos in Obstructive Mullerian Anomalies by Image-Guided Drainage with Interventional Radiology: A Multi-Institutional Case Series.

STUDY OBJECTIVE: To describe cases of image-guided drainage of symptomatic hematometrocolpos from obstructive Müllerian anomalies as a temporizing measure to manage acute pain symptoms and delay definitive management of the obstructive Müllerian anomalies that require complex reconstruction METHODS: Institutional Review Board exemption from all included institutions was obtained. A retrospective case series from 3 academic children's hospitals of 8 females under the age of 21 with symptomatic hematometrocolpos due to obstructive Müllerian anomalies drained by image-guided percutaneous transabdominal vaginal or uterine drainage with interventional radiology was reviewed and described. RESULTS: Eight pubertal patients with obstructive Müllerian anomalies (6 patients with distal vaginal agenesis, 1 patient with an obstructed uterine horn, and 1 patient with a high obstructed hemi-vagina) and symptomatic hematometrocolpos are reported. All patients with distal vaginal agenesis had greater than 3 cm lower vaginal agenesis, which would usually require complex vaginoplasty and use of postoperative stents. Given their immaturity and inability to use stents or dilators postoperatively or medical complexity, they subsequently underwent ultrasound-guided drainage of hematometrocolpos with interventional radiology to relieve pain symptoms, followed by menstrual suppression. The patients with obstructed uterine horns had complex medical and surgical histories requiring perioperative planning; they also underwent ultrasound-guided drainage of hematometra as a temporizing measure to manage acute symptoms. CONCLUSION: Patients presenting with symptomatic hematometrocolpos due to obstructive Müllerian anomalies might not be psychologically mature enough to undergo definitive complex reconstruction, which requires vaginal stent or dilator use postoperatively to prevent stenosis and other complications. Image-guided percutaneous drainage of symptomatic hematometrocolpos serves as a temporizing measure by offering pain relief until patients are ready to undergo surgical management and/or to allow time for complex surgical planning.

Pathologic characteristics of hysterectomy specimens in women undergoing hysterectomy after global endometrial ablation.

STUDY OBJECTIVE: To describe uterine pathologic features in women who underwent hysterectomy because of failed global endometrial ablation (GEA). DESIGN: Retrospective cohort study from 1998 through 2005 (Canadian Task Force classification III). SETTING: Tertiary referral center. PATIENTS: Sixty-nine women who underwent hysterectomy because of GEA failure. INTERVENTIONS: Pathology reports were available for 67 patients. Descriptions of hysterectomy specimens after GEA were reviewed. MEASUREMENTS AND MAIN RESULTS: Rates of pathologic findings in hysterectomy specimens after failed GEA were determined. Reasons for hysterectomy in the 67 patients with available pathology reports were bleeding in 34 (51%), pain in 19 (28%), and bleeding and pain in 14 (21%). The pathology reports of these specimens showed leiomyomas in 33 specimens (49%); intramural myomas were present in 15 women (44%) who underwent hysterectomy because of bleeding and 8 women (42%) who underwent hysterectomy because of pain. Hematometra was identified in 7 pathologic specimens (10%). Specifically, hematometra was identified in specimens from 5 of 19 women who underwent hysterectomy because of pain (26%). CONCLUSION: Hematometra was a significant finding in women who underwent hysterectomy because of persistent pain after GEA. A possible pathologic predictor of GEA failure may be intramural leiomyomas.