A Novel cause of abdominal pain presenting with anuria and renal failure.

A girl in early adolescence with autism presented with 3 months of abdominal pain and 36 hours of anuria. She had recently received treatment for urinary tract infections, anxiety and menorrhagia (she had undergone menarche a few months earlier). Due to the pain, she had pulled out an incisor. Bladder scan showed 923 mL, creatinine was 829 mmol/L but urethral catheter insertion did not drain urine. An unenhanced CT scan revealed an absent left kidney, didelphys uterus and right-sided hydroureteronephrosis caused by haematocolpos in keeping with a diagnosis of OHVIRA syndrome and ureteric obstruction of a single kidney causing acute renal failure. She underwent vaginal septoplasty, drainage of the haematocolpos and right ureteric stent.

Adolescent and Young Adult Patients with Vaginal Graft-vs-Host Disease and Hematocolpos Managed with Vaginal Stents: A Case Series.

STUDY OBJECTIVE: Vaginal stenosis can be acquired as a result of vaginal graft-vs-host disease (GVHD) in patients who have undergone hematopoietic stem cell transplant (HSCT). Little data exist to guide the management of vaginal GVHD, particularly in adolescent and young adult patients. The objective of this study was to detail the management of vaginal stenosis with lysis of adhesions and vaginal stent placement in 3 young patients with vaginal GVHD. METHODS: A retrospective chart review was done for 3 patients with vaginal GVHD causing vaginal stenosis with hematometrocolpos. All 3 were treated using vaginal stent placement. Additionally, a literature review was conducted through PubMed and Google Scholar to identify 21 case reports (with a total of 35 patients) of menstrual obstruction due to GVHD. RESULTS: Obstructive vaginal stenosis secondary to vaginal GVHD occurred in our patients at ages 15, 16, and 24 years. Resolution of hematocolpos was obtained with lysis of vaginal adhesions with vaginal stent placement in all patients, with varying regimens of systemic and topical hormones, topical corticosteroids, and dilator therapy. DISCUSSION: Vaginal stenosis secondary to vaginal GVHD should be considered in patients with a history of allogeneic HSCT presenting with amenorrhea, especially those with a diagnosis of primary ovarian insufficiency. The use of vaginal stents, along with postoperative medical and dilator management as appropriate, may prevent re-stenosis, although more information is needed regarding the efficacy of treatments.

Rol de la laparoscopia en el manejo del síndrome OHVIRA: reporte de un caso y su relación con la endometriosis / Role of laparoscopy in the management of OHVIRA syndrome: a case report and its relation to endometriosis

El síndrome de hemivagina obstruida y anomalía renal ipsilateral (OHVIRA) es producido por una alteración en el desarrollo de los conductos de Müller y Wolff en la vida fetal. El síndrome es poco frecuente, se reporta una prevalencia de 1/2.000 a 1/28.000 casos. La endometriosis se presenta en un 19% de los casos complicando esta patología. El tratamiento del síndrome OHVIRA consiste en resecar el tabique vaginal drenando el hematocolpos. Hasta el momento no existe un consenso en recomendar la realización de una laparoscopia diagnóstica. El objetivo de este estudio es reportar la eventual importancia de la laparoscopia diagnóstica/terapéutica como parte del manejo del síndrome OHVIRA.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is caused by a defect in the development of Müllerian and Wolffian ducts at fetal life. The syndrome is uncommon, with a reported prevalence of 1/2,000 to 1/28,000 cases. Endometriosis is present in 19% of cases complicating this pathology. Treatment of OHVIRA syndrome consists in resecting the vaginal septum and draining the hematocolpos. Until now there isnt an agreement on recommending diagnostic laparoscopy as part of the treatment. The aim of this study is to report the importance of diagnostic/therapeutic laparoscopy in the management of OHVIRA syndrome.

Síndrome De Herlyn ­ Werner ­ Wünderlich. A proposito de un caso / Herlyn ­ Werner ­ Wünderlich Syndrome. About a case

Presentamos el caso de una paciente de 11 años que presento un cuadro clínico de oligomenorrea, leucorrea y dolor pélvico tipo cólico. Se ha pensado por el dolor abdominal en la posibilidad de apendicitis modificada por lo que se realizó ecografía pélvica con los hallazgos de útero didelfo, quiste anexial derecho y agenesia renal izquierda, datos compatibles con síndrome de Herlyn ­ Werner ­ Wünderlich

We present the case of an 11-year-old patient who presented a clinical picture of oligomenorrhea, leucorrhoea and pelvic pain type colic. It has been thought for abdominal pain in the possibility of modified appendicitis so pelvic ultrasound was performed with the findings of didelphic uterus, right adnexal cyst and left renal agenesis, data compatible with Herlyn ­ Werner ­ Wünderlich syndrome.

Síndrome de Herlyn-Werner-Wunderlich com hematométrio ­ Uma associação incomum / Herlyn-Werner-Wunderlich Syndrome with hematometrium - An unusual association

Contexto: A Síndrome de Herlyn-Werner-Wunderlich (SHWW) é uma variação congênita rara, determinada pelo útero didelfo associado à hemivagina obstruída e agenesia renal ipsilateral, podendo ser associada a situs inversus ou não. Descrição do caso: Paciente do sexo feminino, de 35 anos, com dor lombar que irradia para o hipogástrio há 10 dias. A ultrassonografia de abdome total e pélvica transabdominal não visualizou o rim direito, apresentando pequena efusão líquida na pelve e útero didelfo apresentando cavidade uterina direita distendida por conteúdo hemático ­ hematométrio/hematocolpo. A ressonância magnética demonstra útero didelfo, confirmando o achado de hematométrio/hematocolpo à direita, além da agenesia renal à direita. O conjunto de achados é compatível com a SHWW. Discussão: Portadoras da SHWW costumam ser assintomáticas e têm o ciclo menstrual normal, podendo ou não ser associada a dor abdominal de caráter intermitente. Tal manifestação clínica ocorre pelo fato de apenas um corno uterino estar obstruído, fazendo com que a paciente menstrue pelo outro corno. Na suspeita diagnóstica, o padrão-ouro é a ressonância magnética. Conclusão: O atraso no diagnóstico da SHWW dificulta o tratamento e possibilita o desenvolvimento de complicações. A SHWW pode ser diagnosticada pela ultrassonografia, exame acessível e de menor custo quando comparado aos outros métodos de diagnóstico por imagem.

Hymenal atresia - a rare congenital anomaly with the risk of late diagnosis.

OBJECTIVE: Comprehensive analysis of causes, clinical signs, dia-gnostic process, differential dia-gnosis and therapy of hymenal atresia. METHODS: Literature search using the Web of Science, Google Scholar and PubMed databases with keywords and analysis of articles published in high impact and reviewed journals. RESULTS: Hymenal atresia is a congenital malformation of a womans genitals, which is manifested by complete obstruction of the vaginal introitus by a closed hymen. It should be dia-gnosed in the neonatal period, but clinically it usually manifests itself only during puberty as a result of menstrual blood retention (cryptomenorrhea) with the cyclic abdominal pain at monthly intervals. The therapy is based on optimally timed surgical creation of communication in the hymen (hymenotomy, hymenectomy) enabling free evacuation of menstrual contents. The aim of this simple treatment method is immediate subjective relief from pain and a permanent solution to this congenital anomaly. CONCLUSION: Knowledge of all types of congenital malformations of the female genitalia is a basic condition for an early and effective dia-gnostic process in adolescent girls with abdominal pain. The girl who has not yet menstruated and has cyclic lower abdominal pain and a tumor behind the pubic symphysis should be examined by a specialist in pediatric and adolescent gynecology who will confirm hymenal atresia according to a bluish and closed hymen, and suggest prompt and effective therapy.

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.

Himen imperforado con hematocolpos. A propósito de un caso / Imperforate hymen with hematocolpos. Apropos of a case

RESUMEN Los defectos en la canalización de la membrana himeneal causan obstrucción del tracto genital femenino. Como consecuencia aparece hematocolpos, resultante de la acumulación y retención de secreciones cervicovaginales -sangre en útero y vagina-, ante la imposibilidad de su evacuación por la presencia de un himen imperforado. Clínicamente aparece masa abdominal, asociada con malformaciones vaginales congénitas. Se presentó el caso de una adolescente de 13 años que refirió retención urinaria, disuria, dolor y masa en hipogastrio, y ausencia de la menarquía a pesar de un desarrollo puberal en estadio Tanner III. Al realizar ultrasonido se constató sangre en el útero, debido a imperforación himeneal que se resolvió mediante himenotomía. La patología de himen imperforado con hematocolpos debe estar entre los posibles planteamientos sindrómicos de aquellas pacientes con cuadros similares; puede diagnosticarse en la Atención Primaria de Salud a partir de una detallada anamnesis y exploración física, aunque la comprobación requiere ecografía (AU).

ABSTRACT The defects in the channeling of the himeneal membrane cause obstruction in the female genital tract. As consequence hematocolpos appears, resulting from the accumulation and retention of cervicovaginal secretions -blood in the uterus and vagina-, due to the impossibility of its evacuation because of the presence of an imperforate hymen. Clinically, abdominal mass appears associated to congenital vaginal malformations. We presented the case of teenager aged 13 years that referred urine retention, dysuria, pain, had a mass in the hypogastrium, and absence of menarche in spite of a Tanner III pubertal development. An ultrasound showed blood in the uterus due to hymeneal imperforation solved through hymenotomy. The pathology of imperforate hymen with hematocolpos should be among the possible syndrome considerations in those patients with the same characteristics; it can be diagnosed in the primary health care from anamnesis and physical exploration, although the verification requires an ultrasound (AU).

Hematocolpos secundario a himen imperforado / Hematocolpos secondary to imperforate hymen

RESUMEN El hematocolpos es la acumulación de sangre menstrual en la vagina, se produce por imperforación del himen o de la parte inferior de la vagina. El himen imperforado es una rara malformación congénita que ocurre en el 0,1 % de los recién nacidos del sexo femenino; este es un trastorno del desarrollo embriológico del himen que carece del orificio central por el que drenan las secreciones cervicales y uterinas. Se presentó una paciente de 15 años de edad, con antecedentes de haber nacido de parto eutócico a término, con dolor en bajo vientre y en la parte baja de la espalda hacia el lado derecho. El dolor abdominal es la forma de presentación clínica más frecuente de esta afección. El diagnóstico es clínico y se apoya en exámenes complementarios (ultrasonido abdominal y ginecológico). Esta afección se resuelve con la realización de una himenectomía.

ABSTRACT Hematocolpos is the accumulation of menstrual blood in the vagina; it is produced by imperforation of the hymen or the lower part of the vagina. Imperforate hymen is a rare congenital malformation that occurs in 0.1% of female newborns; this is an embryological developmental disorder of the hymen that lacks the central orifice through which uterine and cervical secretions drain. We present a 15-year-old patient with a history of having been born from eutocic delivery at term, pain in lower abdomen and in lower back towards the right side. Abdominal pain is the most common clinical presentation of this condition. Its diagnosis is clinical and is supported by complementary tests (abdominal and gynecological ultrasound). This condition is resolved by means of a hymenectomy.

Mimics of malignancy caused by concurrent imperforate hymen and transverse vaginal septum: an instructive case and review of the literature.

The coexistence of imperforate hymen and vaginal septum is rare and their ability to mimic malignant manifestations have not been frequently reported. This current case report describes a 13-year-old girl that presented with cyclic abdominal pain for 6 months. She was found to have a huge mass via abdominal plain film X-ray and sonography, with inexplicably high levels of serum carcinoembryonic antigen, cancer antigen (CA)-19-9 and CA-125. Pelvic computed tomography imaging disclosed two huge cystic lesions in the uterine and upper vaginal cavities. Surgical intervention conformed the diagnosis of a concurrent imperforate hymen and transverse vaginal septum, echoing the imaging findings of haematocolpometra. Her tumour marker levels gradually returned to normal after surgery. This rare case of concomitant imperforate hymen and transverse vaginal septum highlights that haematocolpometra, a benign disease that might mimic malignancy, should be taken into consideration in any adolescent females with an abdominal mass and amenorrhoea to ensure an early diagnosis and timely appropriate management.

A Novel Technique for the Reconstructive Formation of an Annular Hymen in Cases of Postpubertal Imperforate Hymen.

OBJECTIVES: Imperforate hymen (IH) is a common genital tract anomaly in women which usually presents after puberty. However, surgical treatment is often considered controversial in religious or conservative communities for sociocultural reasons. This study therefore aimed to assess the efficacy of a novel reconstructive technique involving the preservation of the annular hymen. METHODS: This prospective interventional study was performed between July 2013 and October 2019 at the minimally invasive surgery unit of a tertiary university hospital in Egypt. A total of 36 women presenting with primary amenorrhoea and haematocolpus were diagnosed with postpubertal IH. A circular hymenotomy was performed on each patient using a 10 mm laparoscopy trocar tip and sleeve to form a new annular hymen under general anaesthesia while preserving the annular hymen. The primary outcome measure was the persistence of hymenal patency and integrity at follow-up. The secondary outcome measure included post-operative patient satisfaction and pain relief. RESULTS: The reported technique was feasible in all cases without intraoperative complications. Patency of the reconstructed annular hymen was confirmed at follow-up in all cases; moreover, no intraoperative complications were reported. There was a significant post-operative improvement in pain scores (P <0.001). Both the patients and their parents/guardians reported a high level of satisfaction with the technique. CONCLUSION: This novel technique for the correction of IH involving the reconstruction of an annular hymen was found to be a safe, minimally invasive and effective procedure. This technique should be considered a feasible alternative to a conventional hymenotomy as it allows for the resumption of normal hymenal anatomy without overtreatment.

Laparoscopic Colpo-Pneumo Occluder as a Vaginal Mold for Vaginal Reconstruction.

BACKGROUND: Currently, there is no commercially available soft vaginal mold designed for reconstructive surgeries for congenital vaginal anomalies. Stricter operating room regulations discourage the use of makeshift molds from foams and gloves. A colpo-pneumo-occluder balloon is designed to maintain pneumoperitoneum after colpotomy in laparoscopic hysterectomies and is approved for use in vaginal surgeries. CASE: A 17-year-old girl with a congenital transverse vaginal septum experienced recurrent obstruction and hematocolpos. We successfully used a colpo-pneumo-occluder balloon as a vaginal mold during postoperative care. Its size and design make this device ideal for use in vaginal reconstructive surgeries in adolescents. SUMMARY AND CONCLUSION: The laparoscopic colpo-pneumo-occluder, a sterile vaginal device, is appropriate to use as an adjustable, soft vaginal mold for correction of congenital and acquired vaginal anomalies.

Herlyn-Werner-Wünderlich syndrome: An unusual case with presentation of menorrhagia.

OBJECTIVE: Herlyn-Werner-Wünderlich (HWW) syndrome is a rare condition in which patients present with a palpable pelvic mass and pain caused by an obstructed hemivagina. Here we present a case of HWW syndrome characterized by prolonged menstrual bleeding. CASE REPORT: A 19-year-old nonsexually active unmarried women experienced irregular menstrual cycles and menorrhagia. The duration of menstrual bleeding was 10-14 days. She also suffered from mild dysmenorrhea since menarche at the age of 13. Transabdominal sonography revealed a double uterus and a heterogeneous myoma-mimicking mass over the left cervical region. The left kidney was absent. Magnetic resonance imaging revealed a double uterus, a double vagina with an unperforated left hemivagina, and ipsilateral renal agenesis. The patient underwent cervicovaginal orifice reconstruction surgery. CONCLUSION: Left hematocolpos compression, a partially obstructed right vaginal channel, and an orifice with local venous drainage abnormalities resulted in prolonged menstrual bleeding. In HWW syndrome, the occurrence of a pelvic mass and pain is common; however, prolonged menstrual bleeding is rare.

Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management of Asymptomatic Herlyn-Werner-Wunderlich Syndrome.

INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare variant of Mullerian ductal anomaly associated with ipsilateral renal agenesis. Most patients are diagnosed after menarche with complications of uterovaginal obstruction, genitourinary infection and pelvic adhesions. Patients often undergo additional operations for misdiagnosis or treatment of complications. Our institution manages several HWWS patients diagnosed before symptoms by screening for antenatally-diagnosed renal agenesis. This study aims to improve the presymptomatic management of HWWS patients. METHODS: We carried out retrospective case review of patients diagnosed with HWWS from 2010 to 2017 on patient demographics, symptoms, clinical course and operative management and summarize the sparse literature published to date. RESULTS: There were 8 patients with HWWS but only 2 symptomatic patients presented acutely with hematocolpos requiring urgent vaginal surgery. The other six patients had early diagnosis through postnatal ultrasound screening. No patient required further operation for diagnosis or complications related to obstructed hemivagina. CONCLUSION: Our case series and literature review show that the majority of prepubertal patients with HWWS do not require early gynecological surgery. We recommend that female babies with renal agenesis should be screened for HWWS syndrome with ultrasound. Early diagnosis and presymptomatic elective surgery may prevent urogynecological complications that cause fertility and renal impairment. STUDY DESIGN: Case series, level IV evidence.

Imperforate Hymen Causing Hematocolpos and Urinary Retention.

BACKGROUND: Acute urinary retention is rare in the pediatric population and is typically caused by an obstructing entity. It may result from hematocolpos caused by imperforate hymen. Imperforate hymen is rare, with an associated incidence of 1 in 2000 people. Nonetheless, in a pediatric age female who presents with urinary retention and who has a history of primary amenorrhea or a history of cyclical pelvic pain an examination of the external genitalia should be performed to rule out this condition. CASE REPORT: A 12-year-old girl presented to the emergency department with urinary retention and was discharged with a diagnosis of urinary tract infection. She returned the following day to the emergency department with worsening abdominal pain. A computed tomography scan revealed a fluid-filled vagina measuring 12.5 cm. Her bladder measured 15.4 cm. The patient was taken to the operating room for further evaluation by the consulting gynecologist. At the time of surgery, 1000 mL of urine were evacuated after catheterization. Three hundred milliliters of chocolate-colored fluid, consistent with menstrual blood, were evacuated at time of hymenotomy. Hymenotomy was performed with a cruciate incision. No complications were encountered. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: To date, there have been >40 reported cases of imperforate hymen causing hematocolpos and subsequent urinary retention. However, given the rarity of imperforate hymen, hematocolpos leading to acute urinary retention can still be missed. Untreated urinary retention can lead to a compromise in bladder function and subsequent kidney damage.

Hematocolpos por himen imperforado en una adolescente / Hematocolpus due to non-perforated hymen in an adolescent

Se describe el caso clínico de una adolescente de 12 años de edad, quien había estado ingresada 5 años atrás en el Servicio de Terapia Intensiva del Hospital Pediátrico Docente Sur Antonio María Béguez César de Santiago de Cuba por presentar enfermedad estafilocócica y amenorrea primaria. Esta vez acudió a consulta con dolor abdominal recurrente desde hacía 3 meses, localizado en hipogastrio, acompañado de náuseas y vómitos, que se aliviaba con los analgésicos habituales. Se le realizó la prueba de embarazo cuyo resultado fue negativo y las imágenes ecográficas sugirieron la presencia de hematocolpos. Se le realizó himenotomía y egresó de la institución 48 horas después con evolución favorable.

The case report of a 12 years-old adolescent is described who had been admitted 5 years ago in the Intensive Therapy Service of Antonio María Béguez Caesar Southern Teaching Pediatric Hospital in Santiago de Cuba staphylococcal disease and primary amenorrhoea. This time she attended the service with recurrent abdominal pain for 3 months, located in hypogastrium, accompanied by nauseas and vomits that were alleviated with the habitual analgesic ones. The pregnancy test was carried out with negative result and the echographic images suggested hematocolpus. The himenotomy was practiced and was discharged from the institution 48 hours later with favorable clinical course.

Uterine didelphys with one cervix obscured by blind hemivagina: a lesson in rarity.

A 14-year-old girl presented with increasing cyclical pain, scanty menses, pelvic mass and absence of the left kidney. With both radiological and clinical examinations (examination under anaesthesia), diagnosis of bicornuate uterus with single cervix could be made while on laparotomy, and it turned out to be uterine didelphys, with one cervix obscured by blind hemivagina with haematometra and haematocolpos in the left horn, for which hemihysterectomy was done. Post procedure the patient was relieved of cyclical pain and is menstruating properly.

Resolution of Hematocolpos in Adolescents Affected with Graft vs Host Disease.

BACKGROUND: Bone marrow transplantation is a lifesaving procedure for a range of serious benign or malignant hematological conditions. A proportion of patients, however, will develop graft vs host disease (GVHD), acute or chronic, with serious long-term sequalae. CASES: We present 2 cases of hematocolpos that developed in adolescence because of vaginal synechiae due to GVHD. The condition was initially asymptomatic, resolved spontaneously at first and recurred. In 1 girl blunt lysis of the adhesions was performed with the patient under general anesthesia, followed thereafter by local hydrocortisone and estriol treatment. SUMMARY AND CONCLUSION: Genital symptoms might not be readily reported by adolescents after bone marrow transplantation. Physicians should be aware of possible late effects of GVHD on genitalia, inquire about symptoms, and be acquainted with addressing complications, such as vaginal obstruction.