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.

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.

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.

Herlyn-Werner-Wunderlich Syndrome: A Mini-review

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

Síndrome de Hemivagina Obstruida, Útero Didelfo y Agenesia Renal Ipsilateral (OVHIRA), Drenado Parcialmente por Fístula Útero-Uterina Espontánea / Obstructed Hemivagina Syndrome, Uterus didelphys and Ipsilateral Renal Agenesis (OVHIRA), Partially Drained by Spontaneous Uterine-Uterine Fistula

RESUMEN Antecedentes: El síndrome de hemivagina obstruida, útero didlefo y anomalía renal ipsilateral (OVHIRA) es una malformación mülleriana infrecuente. Objetivo: Se presenta un caso con una complicación no antes descrita y se hace una revisión de la literatura hasta la fecha. Caso clínico: Mujer de 12 años de edad con antecedentes de diagnóstico de útero bicorne y agenesia renal derecha en la infancia. Veintidós meses post menarquia consulta por algia pelviana y dismenorrea premenstrual. Ecografía revela hematómetra en cuerpo uterino izquierdo, de 142 x 69 x 61 mm. Resonancia nuclear magnética (RNM) describe además un hematocolpos de 7 x 10 cm y un tabique vaginal transverso de hemivagina izquierda. Se diagnostica OHVIRA y se planifica cirugía para resecar el tabique. Días antes del la cirugía, la paciente tiene episodio de metrorragia. Ecográficamente se constata una disminución considerable del volumen de hematómetra. En la cirugía se pesquisa fístula uterina que comunica útero obstruido izquierdo con útero no obstruido derecho por donde había drenado espontáneamente la hematómetra. Al resecar el tabique vaginal izquierdo se termina de drenar hematómetra y hematocolpos. Al alta, paciente se maneja con dilatación vaginal progresiva por seis meses para evitar la estenosis del tabique. Conclusión: Se presenta una complicación no descrita, fístula útero uterina, de una malformación mülleriana infrecuente (OVHIRA). El proceso diagnóstico meticuloso, la cirugía bien planificada y la dilatación vaginal prolongada permitieron tener éxito en su manejo.

ABSTRACT Background: Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome (OHVIRA) is an uncommon Müllerian anomaly. Objective: A case is described and the main complications related to the syndrome are reviewed. Case report: Female, 12 years old, with a medical history of a bicornuate uterus and right renal agenesis diagnosed at birth. Twenty-two months post menarche she seeks medical attention due to pelvic pain, menometrorrhagia and premenstrual dysmenorrhea. An ultrasound revealed a hematometra, of 142 x 69 x 61 mm, in the left uterus. Magnetic resonance imaging also described a 7 x 10 cm hematocolpos and a transverse vaginal septum of the left hemivagina. OHVIRA is diagnosed and surgery is planned to resect the septum. Two days before the surgery, the patient has an episode of abundant metrorrhagia. Ultrasonographically a considerable decrease in the volume of the hematometra is observed. In surgery, a uterine fistula is discovered which communicates the left obstructed uterus with the right unobstructed uterus, where the hematometra had been partially drained. During the procedure, the left vaginal septum is resected, which completes the drainage of the hematometra and hematocolpos. At discharge, the patient undergoes progressive vaginal dilation for six months to avoid stenosis of the septum. Conclusion: An utero-uterine fistula has not been previously described as a complication of OHVIRA. The meticulous diagnostic process, the well planned surgery and the prolonged vaginal dilation allowed for a successful management.

Síndrome de Herlyn-Werner-Wunderlich. Síndrome OHVIRA. Reporte de Caso / Herlyn-Werner-Wunderlich Syndrome or OHVIRA Syndrome. Case Report

Paciente Femenina de 11 años con el síndrome de Herlyn-Werner-Wunderlich, manejado multdisciplinariamente y resuelto endoscópicamente con una septotomía y dilataciones vaginales.

This case is about a female patent, 11 years old, with Herlyn-Werner-Wunderlich Syndrome, who received multdisciplinary approach and was endoscopically resolved with septotomy and vaginal dilatatons.

Herlyn werner wunderlich syndrome with hematocolpos: an unusual case report of full diagnostic approach and treatment

Herlyn-Werner-Wunderlich [HWW] syndrome is an uncommon combined miillerian duct anomalies [MDAs] and mesonephric duct malformation of female urogenital tract characterized by uterus didelphys and obstructed hemi-vagina and ipsilateral renal agenesis [OHVIRA] syndrome

We present a rare and unusual case of this syndrome in a 19 year-old female who suffered from hypomenorrhoea and abdominal pain. She had an obstructed hemi-vagina on right side which led to marked distention of ipsilateral cervix, while proximal hemi-vagina compressed the contralateral side causing its partial obstruction resulting in hypomenorrhoea. Understanding the imaging findings of this rare condition is important for early diagnosis in order to prevent complications which may lead to infertility.

A case of Herlyn-Werner-Wunderlich syndrome: a rare, congenital genitourinary anomaly in a 12-year-old girl

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare, congenital genitourinary anomaly involving the Müllerian and Wolffian structures, and is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in adolescent girls in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Here, we report a case of a 12-year-old girl who presented to the emergency department with lower abdominal pain and mass that had lasted for 2 weeks. After the confirmation of HWW syndrome with magnetic resonance imaging, hysteroscopic septostomy was carried out as a definitive treatment. When we evaluate adolescent girls with lower abdominal pain and mass, we should consider the possibility of HWW syndrome.

Complete vaginal outlet stenosis in a patient with Sheehan’s syndrome

We present a case of complete vaginal stenosis in a woman diagnosed with Sheehan’s syndrome. The patient delivered at full-term 5 months prior, and experienced massive postpartum bleeding at that time. During evaluation of persistent amenorrhea, we found that her vaginal orifice was completely adhesive and obstructed. Prior to corrective surgery, we managed the patient with an oral contraceptive to induce uterine bleeding into the vaginal outflow tract. After three cycles of an oral contraceptive, we could confirm that there was no stenotic lesion in the vaginal cavity as a hematocolpos was created. Adhesiolysis with scar revision for the vaginal stenosis was successfully performed; it was found that the lesion was limited to only the distal part of the vaginal outlet. Complete vaginal stenosis in reproductive age women with hypopituitarism has not been reported. The artificial induction of hematometrocolpos before surgery was useful in determining the extent of the stenotic lesion, and assured safety.

Dolor abdominal como síntoma de himen imperforado / Abdominal pain as a symptom of imperforate hymen

El himen imperforado es una anomalía congénita del desarrollo genital femenino. A pesar de ser la anomalía obstructiva más frecuente, presenta una incidencia estimada del 0,1% de las recién nacidas. Su diagnóstico es principalmente clínico, habitualmente ante la presencia de dolor abdominal cíclico en una adolescente que no ha presentado la menarquia. Presentamos el caso de una paciente de 12 años de edad que acudió al Servicio de Urgencias por dolor abdominal intenso, acompañado de alteración del hábito intestinal de dos días de evolución. Tras una detallada anamnesis y exploración física, es diagnosticada de himen imperforado con el apoyo de las pruebas complementarias. El cuadro clínico se resolvió tras la realización de una himenectomía.

Imperforate hymen is a congenital anomaly of female genital development. Although it is the most common obstructive anomaly has an estimated incidence of 0.1% of newborn. The diagnosis is primarily clinical, usually in the presence of cyclic abdominal pain in an adolescent who has not submitted menarche. We report the case of a 12 years old woman came to the emergency department by abdominal intense pain accompanied by altered bowel habit in two days. After a detailed history and physical examination, was diagnosed with imperforate hymen with the support of additional tests. The clinical symptoms resolved after performing a hymenectomy.

Imperforate Hymen Causing Hematocolpos and Acute Urinary Retention in a 14-Year-Old Adolescent

We report the case of a 14-year-old girl who visited the emergency room because of suprapubic discomfort and sudden acute urinary retention. She did not have any significant medical and surgical history, and her neurological examinations were all normal. Urinary catheterization led to the passage of 500 mL urine. Abdominal ultrasonography showed a hematocolpos that was compressing the urinary bladder. Gynecologic history taking revealed that the patient has not had menarche yet. Therefore, a cruciate incision was performed and her urination became normal. As the surgical outcome after adequate hymenotomy for imperforate hymen is usually good, the diagnosis of imperforate hymen is important. However, this condition is easily missed in the clinic because the first physician visited by the patient rarely takes a detailed gynecologic history or performs appropriate physical examinations. Although rare, imperforate hymen should be considered as a cause of acute urinary retention in the adolescence period. If an adolescent girl presents with abdominal pain and voiding dysfunction, a detailed gynecologic history and appropriate physical examinations of the genital introitus should be performed.

Himen imperforado como causa de dolor abdominal en la adolescencia: A propósito de un caso / Imperforate hymen as a cause of abdominal pain in adolescents: a case report

El himen imperforado es una anomalía congénita del desarrollo genital femenino. Es una patología poco frecuente, con una incidencia estimada del 0,1% de los recién nacidos del sexo femenino. En muchas ocasiones, el diagnóstico pasa inadvertido hasta la pubertad y debuta con dolor abdominal cíclico en adolescentes que no han presentado la menarquia. El diagnóstico se basa en la anamnesis y la exploración física, aunque las pruebas complementarias, en especial la ecografía, permiten la comprobación del diagnóstico clínico y la exclusión de otras malformaciones genitales. Se presenta el caso clínico de una paciente de 13 años que fue diagnosticada tras consultar en varias ocasiones por dolor abdominal recurrente.

The imperforate hymen is a congenital anomaly of the female genital development. This is a rare pathology with an estimated incidence of 0.1% in female newborns. In many cases, the diagnosis goes unnoticed until puberty, debuting with cyclical abdominal pain in adolescents who have not submitted menarche. Diagnosis is based on history and physical examination, although additional tests, especially the ultrasound that allows to confrm clinical diagnosis and to exclude other genital malformations. We report a case of a 13-year-old that was diagnosed after consulting several times for recurrent abdominal pain.

Gynecologic complication of chronic graft-versus-host disease: Vaginal obstruction

Allogenic peripheral blood stem cell transplantation (Allo-PBSCT) is being used to treat hematological malignancies with increasing frequency. Graft-versus-host disease (GvHD) is a complex complication of PBSCT. A 43-year-old woman came to the gynecology clinic for amenorrhea. She had been diagnosed with acute myeloid leukemia 2 years earlier and treated with induction and consolidation chemotherapy. After developing complete remission, she underwent Allo-PBSCT. When she started chemotherapy, her menstrual cycle completely disappeared. Fourteen months after menopausal hormone replacement therapy, it was discovered that her upper vaginal canal was completely obstructed. The lower vagina had an atrophic appearance. We report a rare case of partial vaginal obstruction as a complication of chronic GvHD and review the literature. We expect that this case report provides an opportunity to remind clinician of the gynecologic complications of GvHD.

Cervical agenesis: a case report

A 24 year-old patient, single, with cervical agenesis and imperforate hymen is presented. She underwent hymenectomy for hematosalpinx and right oophorectomy for endometriosis, before finally undergoing hysterectomy. Cervical agenesis is a rare anatomic developmental abnormality and proposed guidelines in managing similar conditions are reviewed in this report. The management of choice will defend on the condition of the patient and the availability of resources. The patient underwent multiple surgeries in the past. However, despite all efforts to address her medical condition, due to the complications, the small chance of getting pregnant, and the absence of an experienced surgeon capable of performing a reconstructive procedure, she was eventually convinced to undergo hysterectomy. With cervical agenesis, the patient is faced with psychological difficulties that should be addressed. One can only hope that the future will hold a more optimistic outlook for young girls with this rare congenital abnormality.

Hematocolpos secundario a síndrome de Rokitansky / Hematocolpos secondary to Rokitansky syndrome

El hematocolpos es una entidad que puede estar presente en adolescentes con amenorrea primaria que acuden con dolor pélvico cíclico, y al reconocerlos se palpa una masa en dicha región. En este trabajo se presenta un caso portador de síndrome de Rokitansky, con agenesia vaginal, que fue diagnosticado por los antecedentes personales de malformaciones asociadas (hipoplasia renal), manifestaciones clínicas propias del hematocolpos, examen ginecológico y estudios imagenológicos. Se decide tratamiento quirúrgico y posterior reconstrucción cérvico- vaginal. La paciente tuvo una evolución favorable

The hematocolpos is an entity that could be present in adolescents with amenorrhea came presenting with cyclic pelvic pain and in physical examination it is possible to palpate a mass in such region. In this paper authors present the case of a carrier of Rokitansky syndrome with vaginal agenesia diagnosed according the personal history of associated manifestations (renal hypoplasia), clinical manifestations typical of hematocolpos, gynecological examination and imaging studies. Authors decide to perform the surgical treatment and latter cervicovaginal posterior reconstruction. Patient had a favorable evolution

A life threatening complication of an imperforate hymen : a case report

A not so benign disease of imperforate hymen in a 20 year old who presented with acute hypogastric pain will be discussed. The diagnosis of an imperforate hymen should be made during routine neonatal pediatric examination. However, it is not unusual to see a teenage girl present with typical findings of cyclic adominal pain, urinary retention, constipation and a bulging introital mass with hematocolpos and hematometra on ultrasounds examination. Additional findings may include hematosalpinges and formation of endometrial cysts with severe pelvic adgesions. Conservative management generally includes hymenectomy with drainage of the blood and antibiotic therapy. Patients are generally discharged after 24 hours or the procedure is done as an outpatient basis with oral pain medications given. However in our case, the dilemma in terms of management of conservative versus aggressive surgery was encountered. Our patient was only 20 years old presenting with primary amenorrhea and cyclic dysmenorrhea for 7 years with the possibility of a pelvic "clean up" and the problems of premature menopause and infertility were at the back of our minds. A greater awareness of this complication should prompt clinicians to consider this entity when dealing with cases such as this one.

The pitfalls in the diagnosis and management of distal vaginal agenesis: a report of three cases

Vaginal agenesis is an uncommon congenital anomaly and such complex malformations are usually incorrectly identified. Deviations from the normal embryologic events in the reproductive system may result to malformations which may exhibit with the same clinical presentation. A complete history and physical examination coupled with appropriate imaging techniques are necessary to arrive at a correct diagnosis. Physicians must be aware of the differential diagnosis and management options available. A correct diagnosis must be made prior to any surgical intervention to prevent inappropriate surgeries with their anticipated complications. In cases with vaginal outlet obstruction, an accurate diagnosis with the adequate treatment reduces the need for re-operations and subsequent complications. Reported are three misdiagnosed cases of distal vaginal agenesis resulting to unwarranted surgical interventions.