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.

Laparoscopic resection of functional, noncommunicating uterine horn in close approximation to functional hemiuterus.

OBJECTIVE: To review the presentation of unicornuate uterus with a functional noncommunicating rudimentary uterine horn and a laparoscopic method of management, highlighting laparoscopic surgical techniques. DESIGN: A video review of unicornuate uterus with a functional noncommunicating rudimentary uterine horn and a laparoscopic approach to treatment in a 13-year-old pubertal female with severe menstrual pain. SETTING: Tertiary care facility. PATIENT(S): A 13-year-old G0 was referred to the clinic for severe cyclic right lower quadrant pain during menses. Transvaginal ultrasonography revealed a left unicornuate uterus with a right-sided noncommunicating rudimentary horn measuring 4.8 × 4.7 × 4.6 cm, containing blood consistent with hematometra. Her kidneys were bilaterally present and normal by ultrasonography. INTERVENTION(S): Because of the patient's worsening pain and the presence of hematometra, we proceeded with diagnostic laparoscopy and removal of the rudimentary uterine horn. The entire procedure was performed laparoscopically, with an estimated total blood loss of 20 mL. Included are tips for laparoscopic resection and suturing. MAIN OUTCOME MEASURE(S): Pathologic features and postoperative course. RESULTS: The patient's pathologic features were benign, and her severe menstrual pain was resolved. She had no complications or readmissions. CONCLUSION(S): In patients with severe menstrual pain from outflow obstruction from a noncommunicating rudimentary uterine horn with functional endometrium, laparoscopic resection can be a safe and effective method of treatment.

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.

Pre-, intra-, and postoperative management of Robert's uterus.

OBJECTIVES: To demonstrate a minimally invasive approach and management in three different types of Robert's uterus. DESIGN: Video presentation of surgical and ultrasound techniques. SETTING: University hospital and two private centers. PATIENTS: Patients with three types of Robert's uterus; a rare form of septate uterus consisting of non-communicating hemicavity, a contralateral unicornuate uterine cavity in a single uterine body with normal fundus: with large hematometra in the blind hemi-cavity and acute pelvic pain; with an inactive blind hemi-cavity without hematometra and recurrent miscarriages; and with small hematometra in the blind hemi-cavity. INTERVENTIONS: Three-dimensional ultrasound with saline infusion sonohysterography and automatic volume calculation software (SonoHySteroAVC) were used for differential diagnosis and surgical planning. Transrectally guided hysteroscopic metroplasty, a incision of myometrium between two parts of cavities by resectoscope and Collin's electrode, were performed and recorded. Sequentional balloon anti-adhesion therapy and three-dimensional ultrasound with saline infusion sonohysterography with SonoHysteroAVC were used in post-operative management. MAIN OUTCOME MEASURES: Pre-, intra- and postoperative findings regarding uterine morphology, feasibility of surgery and anatomical and clinical outcomes. RESULTS: A successful unification of non-communicating and communicating uterine cavity parts during surgery, better shape and several times higher volume of uterine cavity, and total elimination of pain associated with obstruction after healing period were recorded. CONCLUSIONS: Three-dimensional ultrasound techniques seem to be the best tool for complex pre- and postoperative management of Robert's uterus. Minimally invasive ultrasound-guided hysteroscopic metroplasty should be considered as the first choice of treatment because of the potential for normalization of uterine morphology and function.

Novel management of vaginal chronic graft-versus-host disease causing haematometra and haematocolpos.

Genital chronic graft-versus-host disease (GVHD) in women posthaematopoietic cell transplantation may cause vaginal mucosal sclerosis. Human papillomavirus (HPV) reactivation, also common post-transplantation, limits local immunosuppressive, but not oestrogen treatment. A 36-year-old nulliparous woman developed coincidental genital chronic GVHD and HPV 22 months after transplant for aplastic anaemia. Topical immunosuppression for GVHD led to an eruption of warts successfully treated with laser surgery and cone biopsy. She maintained normal ovarian function and used extended cycle combined hormonal contraception. A vaginal oestrogen ring used continuously limited most scarring for 8 years. Progressive apical vaginal scarring obstructed menstrual flow leading to haematocolpos and haematometra. Normal anatomy was restored with a cruciate incision in the cervicovaginal scar performed during menses. When HPV disease limits use of topical immunosuppression in women with vaginal GVHD, the local scar-reducing effect of a vaginal oestrogen ring is limited, and surgery may be needed and can be successful in treating haematocolpos.This study was registered in ClinicalTrials.gov with trial registration number of NCT00003838.

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.

Endometrial Adenocarcinoma Presenting as Hematometra with Underlying Thickened Endometrial Lining in a Postmenopausal Woman - A Case Report.

BACKGROUND: Although most postmenopausal women diagnosed with endometrial cancer usually present with vaginal bleeding, when complete cervical stenosis is present, this sign may be missing. In these cases, the patient usually complaints for pelvic or abdominal pain while the transvaginal ultrasonography might reveal the presence of an intrauterine fluid collection in association with a thickened endometrial lining. CASE REPORT: We present the case of a 65-year-old patient who presented with association of pelvic pain, enlarged uterine cavity with an underlying hematometra and an irregular, thickened endometrium who was submitted to surgery for total histerectomy, bilateral adnexectomy, pelvic and para-aortic lymph node dissection. CONCLUSION: Histopathological studies revealed the presence of a well-differentiated endometrial adenocarcinoma. At three years of follow-up, the patient is free of any recurrent disease.

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.

McKusick Kaufman syndrome, complications arising at puberty.

BACKGROUND: McKusick Kaufman Syndrome (MKS), a rare genetic condition, presents in the neonatal period with a classic triad of postaxial polydactyly, congenital heart disease, and hydrometrocolpos. The diagnosis is typically clinical, based on the presence of polydactyly and hydrometrocolpos. CASE: We report the case of a 13-year-old female, who was diagnosed with MKS in infancy and underwent vaginal reconstructive surgery for a urogenital sinus. She was lost to follow-up thereafter. She presented to our institution at age 13 with pyometra, pyosalpinx, and tubo-ovarian abscess due to a stenotic cervix obstructing menstrual outflow. SUMMARY AND CONCLUSION: Gynecologic follow-up is imperative in patients with history of vaginal reconstruction to monitor for hematometra from outflow obstruction to prevent life threatening secondary bacterial infections.

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.

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.

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.

[Uterine fibroids complicated by haematometra]. / Hematometra als complicatie bij uterus myomatosus.

BACKGROUND: Uterine fibroids are common, benign tumours of the myometrium. The clinical symptoms include menorrhagia, abdominal pain and subfertility. CASE DESCRIPTION: A 37-year-old black woman known to have uterine fibroids presented at the gynaecology outpatient clinic with abdominal pain. Ultrasonography and an MRI scan revealed haematometra caused by an obstructive myoma, which was drained during a hysteroscopy. The patient was subsequently treated with gonadotrophin-releasing hormone (GnRH) agonist in order to reduce the myoma. Resection of the myoma will take place sometime in the future. CONCLUSION: Haematometra is an accumulation of blood in the uterine cavity. It is a rare complication of uterine fibroids and causes abdominal pain and enlargement of the uterus. Amenorrhoea is often a finding because an adequate outflow of menstrual blood is no longer possible. The primary treatment is to drain the haematometra; a subsequent myomectomy should be performed.

Dysmenorrhea due to a rare müllerian anomaly.

Müllerian duct anomalies may produce reproductive failure like abortion and preterm birth, or obstetric problems like malpresentation, retained placenta, etc., or they may be asymptomatic. Unicornuate uterus with a noncommunicating functional rudimentary horn is a type of müllerian anomaly that results in obstruction to menstrual blood flow, leading to endometriosis and dysmenorrhea. Though the majority of cases of dysmenorrhea in adolescents are primary in nature and require only reassurance and symptomatic management, it is important to be aware of rare causes such as müllerian anomalies so that these cases can be properly managed. Hence, we present this case report, with interesting illustrations, so as to increase awareness regarding these anomalies.

Management of hematocervix after loop electrosurgical excision procedure.

BACKGROUND: Management is not established for the uncommon complication of cervical stenosis secondary to a loop electrosurgical excision procedure resulting in a hematocervix. CASE: A premenopausal woman underwent an electrosurgical procedure performed without complications and subsequently developed amenorrhea. The patient was determined to have developed a hematocervix secondary to the electrosurgical procedure. Increasingly aggressive methods were attempted to drain the hematocervix, finally with successful drainage being accomplished with the use of a manual vacuum aspirator. A catheter was then used to maintain patency of the cervical canal as it re-epithelialized. CONCLUSION: Manual vacuum aspiration and placement of a catheter to allow for re-epithelialization is a successful strategy to deal with hematocervix caused by electrosurgical procedures.