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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., (Copyright © 2023. Published by Elsevier Inc.)

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.

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., (Copyright © 2022 AAGL. Published by Elsevier Inc. All rights reserved.)

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., Competing Interests: Competing interests: None declared., (I, Pamela Stratton, The Corresponding Author has the right to grant and do so grant on behalf of all Contributors a licence to all intellectual property rights for all content within the submitted case report or as later submitted (which includes without limitation any diagrams photographs, other illustrative material, video, film or any other material howsoever submitted by any of the Contributors at any time and related to this article) other than as agreed with the BMJ Publishing Group Ltd) (“BMJ”)) has the right to grant on behalf of all Contributors and does grant on behalf of all Contributors , a licence to BMJ in any media known now or created in the future, and permit this case report (if accepted) to be published on BMJ Case Reports website and /or within any other BMJ products in any media and to be fully exploited within the remit of the licence which has been read.)

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.

Imperforate hymen is a rare condition that presents with amenorrhea, cyclical abdominal pains and urine retention among pubertal girls. A 14 year old girl with imperforate hymen underwent hymenotomy for hematocolpometra, having presented with abdominal pains and tenesmus.

Background: Transverse vaginal septum is a rare cause of primary amenorrhea. It has a reported incidence of 1:2,100-1:72,000 and a variety of clinical presentations., Case: A 16-year-old patient presented with primary amenorrhea and a large abdominal mass that was palpated on clinical exam and confirmed on MRI imaging. A diagnosis of transverse vaginal septum was ultimately made and the patient underwent a successful surgical excision of the septum and vaginal reconstruction., Conclusion: The presentation, etiology, diagnosis, and surgical treatment of a transverse vaginal septum are discussed.

Radiation therapy is often used to achieve local control of pelvic Ewing sarcoma in children. The effects of radiation on the female reproductive tract have been well documented in adults with gynecological malignancies, but the long-term consequences of pelvic radiation in pre-pubertal or adolescent girls are not as well described. We report a case of hematometrocolpos developing in an adolescent previously treated with chemotherapy and radiation therapy for pelvic Ewing sarcoma. We describe the clinical presentation, radiographic features, gross pathology, treatment strategies, outcome, as well as putative predisposing factors and preventative interventions., ((c) 2007 Wiley-Liss, Inc.)

Background: Malformations of the mullerian-duct-system occur with an incidence of about 0.001-0.005% of the population, and they are frequently associated with unilateral agenesis of the kidney., Case: A 12-year-old girl presented to our emergency department with cyclic abdominal pain which increased with each of her menses. Ultrasound investigation showed a uterus duplex with obstructed hemivagina and hematometrocolpos as well as an ipsilateral absence of the kidney. Additionally, a cystic expansion in the small pelvis was detected which required further investigations (MRT and MR-angiography) to establish the diagnosis of a hematosalpinx. Excision of the vaginal septum resulted in drainage of the hemato-metrocolpos and the hematosalpinx., Conclusion: Unilateral hematometrocolpos should be considered as an important differential diagnosis in female adolescents with relapsing lower abdominal pain, even if normal menstruation occurs.

Objective: To describe a technique for treating hematocolpos and hematometra in patients with uterus didelphys and unilateral imperforate vagina involving the use of resectoscopy under ultrasonographic control., Design: Case report., Setting: University hospital., Patient(s): A 13-year-old girl with uterus didelphys with unilateral hematometra, hematocolpos, and ipsilateral renal agenesis. The girl complained of severe abdominal pain, which appeared with each of her menses., Intervention(s): The intervention was performed by a vaginoscopic approach to preserve the integrity of the hymen. The first incision on the vaginal wall was performed in correspondence with the hematocolpos under continuous ultrasonographic guidance with the use of a straight resectoscopic loop. Resection of the vaginal septum was continued with the use of an angled resectoscopic loop until almost complete excision of the septum was achieved., Main Outcome Measure(s): Clinical, echographic, and vaginoscopic findings before the operation and 2 and 6 months after the operation., Result(s): The surgical procedure was easy to perform. Almost complete excision of the septum was achieved with just a few passages of the resectoscope. Complete drainage of both the hematocolpos and the hematometra was confirmed by ultrasonography. The postoperative period was completely uneventful. Clinical and vaginoscopic evaluations 6 months after the operation confirmed the integrity of the hymen, the complete resolution of clinical symptoms, and the persistence of a large communication between the two vaginas., Conclusion(s): Resectoscopic excision under ultrasonographic guidance of the vaginal septum in a girl with uterus didelphys with unilateral hematometra and hematocolpos was effective and easy to perform, and it fully respected the integrity of the reproductive system.

Background: Transperineal sonography has been used to detect incompetent cervices, placenta previa, cervical pregnancy, and vaginal and cervical atresia., Case: Transperineal sonography was used to investigate primary amenorrhea in a 14-year-old girl. A mid-transverse septum was found and confirmed surgically., Conclusion: Transperineal or translabial sonography assisted in the differentiation of primary amenorrhea, when adequate vaginal access was prohibited.

Uterus didelphys with double vagina and hemi-vaginal atresia is a rare syndrome of congenital anomalies. A 17-year-old girl had a right blind-ending ectopic ureter, the proximal end of which communicated with the ipsilateral uterine cervix of uterus didelphys. The patient presented with vaginal urinary incontinence after incision of the vaginal wall for right hemi-hematocolpometra. Following various examinations, the ipsilateral kidney was found to be absent. The ectopic ureter and communicating duct were resected, and the fistula was closed. The genesis of malformation of the female genitalia and urinary tract resulting in such a unique communication is discussed. The importance of preoperative meticulous examinations, including cysto-genitography, pelvic magnetic resonance imaging and panendoscopy with the patient under anesthesia, is emphasized.

Although this anomaly is seen more frequently, the present case is unique in that the genital anomaly (imperforate vagina with the subsequent hematocolpos and hematometra) was associated with contralateral renal agenesis, whereas the cases reported in the literature have all been ipsilateral. Like most of the cases, the presenting symptom was acute urinary retention arising from extrinsic compression of the genital mass on the lower urinary tract. The embryological aspects of the genitourinary system concerning the present case are discussed, as well as some of the hypotheses that have been put forward to explain the etiology of these anomalies, although these have been developed on the basis of the associated ipsilateral anomalies observed.

A late complication of separation of conjoined twins, hematometra-hematocolpos, may appear with sexual maturation. An obstructed genital outflow tract can cause significant urologic and reproductive tract morbidity.

Complete or incomplete transverse vaginal septum is a rare malformation of the female genital tract. Usually the complete congenital type occurs at puberty because of the collection of menstrual blood above the septum with amenorrhea and cyclic lower abdominal pain as presenting symptoms. On the contrary, in the case shown by the authors, the subacute epilogue occurred in the perimenopausal phase: a very large colpohematometra is reported in a 49 years old woman, with an incomplete vaginal septum resulting in progressive obstruction. The association between this malformation and the presence of endometriotic localizations in the genital tract, as reported by other authors, is interesting. In this case, endometriosis can be secondary to the presence of the septum or could have determined the impairment of the obstruction in consequence of the associated status of chronic flogosis.