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.

OHVIRA-syndrome (Obstructed hemivagina with ipsilateral renal anomaly) as differential diagnosis of acute lower abdominal pain.

INTRODUCTION: OHVIRA-syndrome (obstructed hemivagina, ipsilateral renal agenesis/anomaly) is a rare Mullerian duct anomaly that can lead to complications in pubescent children. CASE REPORT: We report a case of a 13-year-old patient with acute right-sided lower quadrant abdominal pain who was referred for exclusion of appendicitis. As a result of the examination (transvaginal ultrasound scan and gynecological examination), a female genital tract anomaly was suspected in the form of obstructed hemivagina with hematocolpos and hematometra. The MRI scan showed hematocolpos and hematometra on the right side, uterus didelphys accompanied by right-sided renal agenesis, consistent with OHVIRA-syndrome. Excision of the vaginal septum was performed and the accumulated old menstrual blood, as represented by hematocolpos and hematometra, was evacuated. Postoperative recovery was uneventful. CONCLUSION: The early surgical management of this rare Mullerian duct anomaly is important in order to prevent longterm complications. This malformation should be considered in the differential diagnosis of acute lower abdominal pain in pubescent girls. KEY WORDS: Abdominal Pain, Genital Anomaly, Obstructed Hemivagina, Renal Anomaly.

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.

Levonorgestrel-Releasing Intrauterine System: A Promising Choice after Uterovaginal Anastomosis in Patients with Cervical Dysgenesis.

BACKGROUND: Cervical dysgenesis is categorized into cervical fragmentation, cervical fibrous cord, and cervical obstruction. The definitive management for cervical dysgenesis is either uterovaginal anastomosis (UVA) or hysterectomy. OBJECTIVE: To compare the prevalence of dysmenorrhea, hematometra, and need for dilatation after UVA with and without postprocedural placement of a levonorgestrel intrauterine system (LNG-IUS). METHODS: This was a retrospective cohort study in which 14 patients with cervical dysgenesis were included. Patients had undergone UVA between May 2015 and January 2022 at the Department of Obstetrics and Gynecology of the Cairo University Teaching Hospital. Six patients who had an LNG-IUS inserted after UVA were included in group A, and 8 patients who had undergone UVA without LNG-IUS insertion were included in group B. Transabdominal and/or transvaginal ultrasound was performed monthly for the first 3 months after LNG-IUS insertion in group A and after UVA in group B. Thereafter, the patients were followed up every 6 months. The primary outcomes were dysmenorrhea, hematometra, and need for dilatation of the anastomosis site. RESULTS: The number of patients who developed hematometra was significantly lower in group A than in group B (0 [0%] vs 6 [75%], P = .01). The number of patients who required dilatation was significantly lower in group A than in group B (0 [0%] vs 6 [75%], P = .01). There was no significant difference in the incidence of dysmenorrhea between the 2 groups. CONCLUSION: We recommend offering LNG-IUS after UVA for adolescents who present with cervical dysgenesis. LNG-IUS decreases the recurrence of hematometra and subsequent surgical interventions.

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.

Unicornuate Uterus with Noncommunicating Rudimentary Horn (Class U4aC0V0/ESHRE/ESGE Classification) and a Communicating Bladder Endometriotic Nodule.

STUDY OBJECTIVE: To describe the diagnostic workup and laparoscopic management of a noncommunicating left uterine rudimentary horn (class U4aC0V0 European Society of Human Reproduction and Embryology/European Society of Gastrointestinal Endoscopy Classification) with communicating endometriotic bladder nodule. DESIGN: Step-by-step description of the surgical treatment. PATIENT: A 33-year-old woman with unicornuate uterus and a left-side noncommunicating rudimentary horn affected by primary infertility, mild dysmenorrhea (visual analog scale score 6), severe catamenial dysuria (visual analog scale score 10), and catamenial hematuria. SETTING: Noncommunicating rudimentary horns are rare Müllerian anomalies present in 20% to 25% of women with a unicornuate uterus. It is associated with severe dysmenorrhea, pelvic pain, subfertility, and poor obstetric outcomes and usually presents with cyclic pelvic pain that starts early after the menarche. Endometriotic bladder nodules are present in 1% to 2% of patients with endometriosis. In the literature, there are no reported cases of noncommunicating rudimentary horn with communicating endometriotic bladder nodules. Surgical excision of the rudimentary horn is the treatment of choice. In our case, the 2-dimensional/3-dimensional ultrasound revealed a right unicornuate uterus with a left noncommunicating rudimentary horn with hematometra. The uterine fundus presented «gamma sign¼ vascularization. In addition, a bladder endometriotic nodule (16 × 15 mm) communicating with hematometra was displayed. Magnetic resonance imaging demonstrated no additional malformations. Diagnostic hysteroscopy revealed a single cervix without vaginal malformation and small right uterine cavity with single tubal ostium. At laparoscopy, using hysteroscopic transillumination, a clear plane of dissection was identified between the rudimentary horn and the uterus confirming the presence of a noncommunicating horn. Evaluation of the abdominal cavity showed bilateral normal adnexa with normal ovaries. Chromopertubation showed a patent right Fallopian tube and obstructed left tube. INTERVENTIONS: A left salpingectomy using bipolar and the ultrasonic energy was performed. The utero-ovarian ligament was transected, and the left ovary was preserved. The left ovary was suspended at the pelvic wall, the retroperitoneum was opened, the ureter was identified, and the left uterine artery was temporary occluded. The left round ligament was transected and the left paravesical space was developed. With a lateromedial approach, we opened the vesicouterine septum to dissect the bladder from the rudimentary horn. The endometriotic bladder nodule was gently detached from the uterine horn with a lateromedial approach. The left uterine artery was coagulated and dissected at level of the cervix. A solution of vasopressin was injected between the uterine horn and the uterus. Resection of the rudimentary horn was performed. The peritoneum was closed. The temporary occlusion of the uterine artery was removed. The specimen was placed in a bag and removed using an extracorporeal tissue extraction technique. CONCLUSION: The late clinical presentation of our patient with only mild dysmenorrhea could be explained by the drainage of the hematometra from the noncommunicating horn into the endometriotic bladder nodule. The bladder symptoms in patients with Müllerian anomalies should be carefully investigated. The laparoscopic removal of rudimentary horn with mobilization of communicating bladder nodule when present is a safe and feasible method to improve symptomatology.

Intrauterine adhesions combined with Robert's uterus: a case report and literature review.

PURPOSE: To summarize the clinical characteristics and surgical option of Robert's uterus. METHODS: We reported a rare case of Robert's uterus with severe uterine adhesion with successive laparoscopic and hysteroscopic surgery. To our knowledge, such a case has not been reported previously. We also performed a systematic literature review from the PubMed, Embase, and Cochrane databases. RESULTS: Our patient with Robert's uterus with severe uterine adhesions was successfully treated with hysteroscopic septal resection and hysteroscopic adhesiolysis, and the intractable dysmenorrhea disappeared after the hysteroscopic septal resection. In our study, we analyzed the selected 22 reported cases, 10/22 cases (45.5%) were diagnosed before age 20; 20/22 cases (90.91%) experienced dysmenorrhea, 19/22 cases (86.36%) were with hematometra. 5/22 cases (22.73%) underwent re-operation or a third surgery before diagnosis and management. CONCLUSION: Robert's uterus, a rare congenital abnormality of Mullerian duct development, consists of an oblique septum and non-communicating asymmetrical uterine hemi-cavity. The main symptoms are the presence of hematometra and severe dysmenorrhea. Septal resection is the main surgical procedure; however, the rarity and difficulty obtaining a pre-operative diagnosis lead to a high rate of misdiagnosis and second surgery.

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.

Inappropriate surgery in a patient with misdiagnosed Robert's uterus.

BACKGROUND: Robert's uterus is a rare Mullerian anomaly, which can be described as an asymmetric, septate uterus with a non-communicating hemicavity. Herein, we present the case of a misdiagnosed Robert's uterus, resulting in an invasive and disadvantageous surgery. CASE PRESENTATION: A 16-year-old woman was referred to our department because of dysmenorrhea and suspicion of uterine malformation. We misdiagnosed Robert's uterus as a unicornuate uterus with a non-communicating rudimentary horn and hematometra, and performed laparoscopic hemi-hysterectomy. Although the patient's symptoms were relieved, our surgical procedure left the lateral uterine wall weak, making the patient's uterus susceptible to uterine rupture in any future pregnancy. CONCLUSIONS: Although the early diagnosis of Robert's uterus is challenging, it is important in order to determine appropriate surgical interventions and management for maintaining the quality of life and ensuring safety in future pregnancies.

Hematometra y hematocervix en paciente postmenopáusica: reporte de caso / Hematometry and hematocervix in postmenopausal patient: case repor

El hematometra es la retención de sangre en el útero y comúnmente se presenta en mujeres jóvenes con anomalías mullerianas pero puede aparecer también en mujeres postmenopausicas por causas secundarias como traumas, tumores, terapia de remplazo hormonal, estenosis cervical, entre otras. En esta presentación de caso interesante se describe una mujer postmenopáusica bajo terapia de remplazo hormonal. Dicha mujer inicia con hemorragia uterina anormal por lo que se le realiza ultrasonido evidenciando hematómetra y hematocervix. Como método diagnóstico y terapéutico de la hemorragia postmenopáusica se le realiza histerectomía abdominal en la cual la patología evidencia leiomiomatosis uterina con endometrio secretor

Hematometra is the retention of blood in the uterus and commonly occurs in young women with Mullerian abnormalities but can also appear in postmenopausal women due to secondary causes such as trauma, tumors, hormone replacement therapy, cervical stenosis, among others. In this presentation an interesting case is described a postmenopausal woman under hormone replacement therapy. She said woman began with abnormal uterine bleeding, so an ultrasound was performed showing hematometer and hematocervix. As a method diagnosis and treatment of postmenopausal hemorrhage, abdominal hysterectomy is performed in which the pathology shows uterine leiomyomatosis with secretory endometrium

[Urinary Retention Due to Hematocolpos].

Hematocolpos is an obstruction of the menstrual flow due to an anomaly of the genital tract, with imperforate hymen being the most common one. It is an uncommon condition, with a prevalence of 1:1000 - 1:16 000. It is usually an asymptomatic condition until the onset of menarche, when there is accumulation of blood in the vagina (hematocolpos) or in the uterus (hematometra). A rare symptom of hematocolpos could be urinary retention. Our case report is about a 12-year-old girl, with no menarche and Tanner stage M4/P5, who was admitted to our emergency department with abdominal pain and urinary retention. A brief review of this rare condition is presented, in order to remind clinicians about its existence, so that it can be diagnosed and treated as quickly as possible.

O hematocolpos consiste na obstrução do fluxo menstrual por uma anomalia do trato genital, sendo a mais frequente o hímen imperfurado, uma patologia pouco comum (prevalência de 1:1000 ­ 1:16 000). Geralmente é uma condição assintomática até a menarca, quando ocorre acumulação de sangue na vagina (hematocolpos) ou no útero (hematometra). Um sintoma raro de hematocolpos é a retenção urinária. Apresentamos o caso de uma adolescente de 12 anos, sem menarca e com estadio sexual de Tanner M4/P5, que recorreu ao nosso serviço de urgência com dor abdominal e retenção urinária. Com o intuito de sensibilizar para esta patologia rara, fizemos uma breve revisão teórica orientada para o rápido diagnóstico e tratamento da mesma.

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.

Descenso vaginal vía perineal asistido por laparoscopia en paciente con atresia vaginal distal / Laparoscopically assisted pull-through surgery in a patient with distal vaginal atresia

La atresia vaginal distal es una anomalía infrecuente que deriva de la falta de desarrollo de los 2/3 distales de la vagina a partir del seno urogenital. Presentamos una paciente de 14 años con amenorrea primaria, abdominalgia, masa palpable en hipogastrio y ausencia de orificio vaginal. La ecografía abdominal y la RMN evidenciaron hematometrocolpos secundario a agenesia vaginal distal, con una distancia al periné de 5cm, sin otras malformaciones. Se realiza un drenaje vaginal transuretral y descenso vía perineal asistido por laparoscopia. Evolución favorable con calibre vaginal adecuado y normalización de la menstruación a los 6 meses

Distal vaginal atresia is an uncommon disorder that results from the lack of development of the distal 2/3 of the vagina from the urogenital sinus. The case is presented on a 14 year-old patient with primary amenorrhoea, abdominal pain, a palpable mass in the hypogastrium, and an absent vaginal opening. The abdominal ultrasound and magnetic resonance imaging showed haematometrocolpos secondary to distal vaginal agenesis, with a distance of 5cm to the perineum, with no other malformations. A transurethral vaginal drainage and laparoscopic assisted pull-through were performed. Her follow-up was favourable with an adequate vaginal calibre and normal menstruation after 6 months