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.

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.

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.

Management of Cervix Atresia with Hematometra by "Genitoscopic Ultrasound-Guided Cervix Fenestration and Balloon Dilatation": A Novel Approach.

Introduction: Cervix atresia causing hematometra usually presents at puberty with cyclic abdominal pain. The management depends upon the type and severity of cervix atresia. We present a novel technique of managing a case of cervix atresia in a 13 years old girl. Case report: A 13 years old girl presented with severe cyclic abdominal pain. Examination revealed normal external genitalia with a palpable tender mass in the suprapubic area. Ultrasonography and MRI suggested a large hematometra with a 1.5 cm long cervix atresia. Vaginoscopy performed under anesthesia showed a normal-looking vagina and a blind cervix. During vaginoscopy and dynamic ultrasound (US) guidance, a spinal needle was passed through the vagina and center of the atretic cervix into the uterine cavity containing the hemolyzed blood. Aspiration of the hemolyzed blood and US visualization confirmed the location of the needle in the uterine cavity. A three-stage (esophageal) balloon dilator was passed over a guidewire through the atretic area. The balloon dilator was dilated up to 10 mm using pressure-controlled dilatation. A satisfactory dilatation was achieved, and all the retained hemolyzed blood was drained through the newly created channel. A size 24 Foleys catheter was retained in the uterine cavity and removed after 1 week. A repeat ultranography before subsequent periods showed a recurrent collection and obliteration of the cervix. US-guided balloon dilatations were repeated. A third dilatation was required after an interval of few months. The child remained well after the third dilatation, having regular periods, and is symptom free at 8 months follow-up, however, may need more sessions of cervix dilatations. Conclusion: Genitoscopic US-guided cervix fenestration and balloon dilatation is an effective technique for treatment of cervix atresia. It is a safe technique, avoids major surgical reconstructions, is a minimally invasive procedure, and can be used in selected cases of cervix atresia.

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.

Müllerian Aplasia with Severe Hematometra: A Case Report of Diagnosis and Management in a Low Resource Setting.

BACKGROUND: Müllerian aplasia or Mayer-Rokitansky-Küster-Hauser syndrome is described as congenital absence of the proximal vagina with or without absence of the cervix and uterus, most often recognized after the onset of primary amenorrhea. CASE: An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam was significant for uterus palpable superior to the umbilicus, absence of vagina, and rectal exam without palpable vagina or cervix. Transabdominal and transperineal ultrasound examinations did not reveal hematocolpos. Exploratory laparotomy revealed severe endometriosis with bilateral hematosalpinx, markedly distended uterus, no proximal vagina, and normal ovaries. Uterine specimen was filled with blood and no clear cervix was present. SUMMARY AND CONCLUSION: Diagnosis of vaginal and cervical agenesis is complicated in low-resource settings and treatment must be modified when subspecialty care and consistent follow-up are not available.

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.

Cause or Coincidence? Spontaneous Hematometra in Young Women Receiving Depomedroxyprogesterone Acetate: A Small Case Series.

BACKGROUND: Abdominal pain, secondary amenorrhea, and abnormal uterine bleeding are common gynecologic presentations in adolescence. Rarely this can be associated with an acquired hematometra. Hematometra is a condition of retained blood or clot within the uterus. High-dose progestogenic agents in this age group have been implicated in the accumulation of a hematometra without other explanation. CASES: We present 4 cases of hematometra after depomedroxyprogesterone acetate (DMPA) therapy in previously menstruating adolescents. All 4 presented with abdominal pelvic pain and/or persistent abnormal uterine bleeding, with the diagnosis confirmed via ultrasound. Suction dilation and curettage was required in each case. SUMMARY AND CONCLUSION: DMPA is a possible cause of hematometra and should be considered in anatomically normal young women experiencing pain or abnormal bleeding out of character for typical long-term DMPA use.

Uterovaginal Anastomosis for Cases of Cryptomenorrhea Due to Cervical Atresia with Vaginal Aplasia: Benefits and Risks.

STUDY OBJECTIVE: The objective of this study was to assess short-term benefits and risks of utero-vaginal anastomosis done for cases of cryptomenorrhea due to cervical atresia with vaginal aplasia. DESIGN: Prospective study. SETTING: Surgical procedures were done between December 2013 and September 2015 at the department of Obstetrics and Gynecology, Cairo University Hospital. PARTICIPANTS: Five patients who had cryptomenorrhea due to cervical atresia associated with vaginal aplasia were included. INTERVENTIONS: Utero-vaginal anastomoses were performed in 2 stages; a stage of McIndoe vaginoplasty and a stage of excision of the atretic cervical tissue and anastomosing the uterus to the neovagina. Follow-up was done by gynecological and ultrasound examination in a duration ranged from 12 to 36 months. MAIN OUTCOME MEASURES: Occurrence of regular menstrual flow and relief of the severe cyclic pain. RESULTS: All patients had relief of the severe cyclic pain. Four patients had regular menstrual flow. One patient developed occlusion of the track after 1 year and needed dilatation once. Three patients developed low vaginal stenosis without occlusion of the track. One patient had rectal injury repaired without causing postoperative morbidity. CONCLUSION: Uterovaginal anastomosis is a promising conservative management option for cervical atresia with vaginal aplasia, which has benefits but is not free of risks. Long-term follow-up is still needed to judge its feasibility. We recommend performing McIndoe vaginoplasty as a starting stage before the anastomosis preferably in a separate setting.

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.