Reconstrucción cervical con conexión vaginal secundaria a agenesia cervical. Reporte de caso / Cervical reconstruction with vaginal conexion secundary to cervical agenesia. Case report

Resumen ANTECEDENTES: La agenesia cervical congénita es una anomalía poco común del sistema de Müller, que generalmente se reconoce durante la menarquia. Hasta hace poco, el tratamiento de primera elección era la histerectomía, debido a que no se consideraba una enfermedad compatible con la fertilidad. Hoy en día, gracias a las técnicas de reproducción asistida, se han desarrollado procedimientos quirúrgicos menos agresivos que sí preservan la fertilidad de las pacientes. OBJETIVO: Revisar un caso de agenesia cervical a partir de su tratamiento quirúrgico y seguimiento de la reconstrucción cervical con la conexión entre la vagina y el útero, revisar la bibliografía y cómo establecer el pronóstico a largo plazo. CASO CLÍNICO: Paciente de 12 años llevada a consulta ante la ausencia de menstruación. El procedimiento diagnóstico transcurrió sin que se encontraran alteraciones; sin embargo, en la laparoscopia diagnóstica operatoria efectuada a los 14 años, indicada por dolor pélvico cíclico, se advirtieron la ausencia del cuello del útero, un hematometra y endometriosis grado IV. En el segundo tiempo quirúrgico, practicado en conjunto con el cirujano pediatra, se practicó, por laparotomía, la comunicación entre el útero y la vagina. En la actualidad, la paciente tiene 19 años y sus menstruaciones son cíclicas, no dolorosas. En el último ultrasonido se visualizó la conexión entre el útero y la vagina. CONCLUSIONES: Hoy en día, la anastomosis útero vaginal es un procedimiento capaz de mantener la función del útero y permitir el embarazo, con los cuidados adecuados.

Abstract BACKGROUND: Congenital cervical agenesis is a rare anomaly of the Müllerian system, usually recognized during menarche. Until recently, the treatment of first choice was hysterectomy, because it was not considered a disease compatible with fertility. Today, thanks to assisted reproductive techniques, less aggressive surgical procedures have been developed that do preserve the fertility of patients. OBJECTIVE: To review a case of cervical agenesis from its surgical treatment and follow-up cervical reconstruction with the connection between the vagina and the uterus, review the literature and how to establish the long-term prognosis. CLINICAL CASE: A 12 years old female patient was brought for consultation due to absence of menstruation. The diagnostic procedure was carried out without finding any alterations; however, in the operative diagnostic laparoscopy performed at the age of 14, indicated by cyclic pelvic pain, the absence of the cervix, a hematometra and endometriosis grade IV were noticed. In the second surgery, performed in conjunction with the pediatric surgeon, the communication between the uterus and the vagina was performed by laparotomy. At present, the patient is 19 years old and her menses are cyclical, not painful. In the last ultrasound the connection between the uterus and the vagina was visualized. CONCLUSIONS: Today, utero-vaginal anastomosis is a procedure capable of maintaining the function of the uterus and allowing pregnancy, with proper care.

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

Diagnóstico y tratamiento del síndrome de Herlyn-Werner-Wünderlich: reporte de dos casos

Las malformaciones müllerianas son anomalías congénitas que suelen manifestarse después de la menarquia. Entre ellas, el síndrome de Herlyn-Werner-Wünderlich (SHWW) se caracteriza por útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica del SHWW suele ser asintomática hasta la menarquia; la mayoría es diagnosticada entre 2 meses y 1 año después de la primera menstruación. Los síntomas que se presentan son dismenorrea, dolor abdominal cíclico recurrente, menstruación irregular y, a veces, masa pélvica palpable. Ocasionalmente, el hematocolpos o la hematometra pueden desarrollar piocolpos, piosálpinx y pelviperitonitis, y algunas pacientes solo manifiestan infertilidad o pérdida recurrente del embarazo. Presentamos los casos de dos pacientes de 11 y 13 años que acudieron con dolor abdominal cíclico y describimos la historia clínica, las imágenes y el tratamiento específico que se realizó en cada una de las pacientes.

Müllerian malformations are congenital anomalies that usually manifest after menarche. Among them, Herlyn-Werner-Wünderlich syndrome (SHWW) is characterized by didelphic uterus, obstructed hemivagina and ipsilateral renal agenesis. The clinical presentation of SHWW is usually asymptomatic until menarche; most are diagnosed 2 months to 1 year after first menstruation. Presenting symptoms are dysmenorrhea, recurrent cyclic abdominal pain, irregular menses, and sometimes palpable pelvic mass. Occasionally, hematocolpos or hematometra may develop pyocolpos, pyosalpinx and pelviperitonitis, and some patients manifest only infertility or recurrent pregnancy loss. We report the cases of two patients aged 11 and 13 years who presented with cyclic abdominal pain and describe the clinical history, imaging and specific treatment performed in each patient.

Unicornuate uterus with functional non communicating rudimentary horn: a refractory cause of dysmenorrhea

Mullerian duct anomalies are rare. Unicornuate uterus with a non-communicating rudimentary horn is a rare type of mullerian duct anomaly which occurs due to defective fusion of malformed duct with contralateral duct. The incidence is approximately 1:100000. Patient usually remain asymptomatic due to the absence of functional endometrium in most of the cases. If the rudimentary uterine horn has an endometrium lined uterine cavity and doesn’t communicate externally then the signs and symptoms of obstructed menstruation appears, as soon as menarche begins. It will be associated with severe dysmennorhoea and hematometra. Other complications may be abdominal lump, chronic pelvic pain, infertility, endometriosis, adenomyosis and ectopic pregnancy in rudimentary horn. Authors are presenting a case of refractory dysmenorrhea with lump abdomen in a patient with unicornuate uterus with functional non communicating horn. In a patient with refractory dysmenorrhea mullerian duct anomaly should be kept as differential diagnosis.

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.

A Rare Case Of Mullerian Anomaly- (HEMATOMETRA With Cervical Agenesis)

Cervical Agenesis is a relatively infrequent mullerian anomaly. Sixteen years old, unmarried girl presented to ELMC&H Lucknow, with complaints of primary amenorrhoea and cyclical abdominal pain for 1 year. On Per-rectal examination, tense cystic mass was felt on right side, approximately 5x5 cm in size, tender and freely mobile. Cervix could not be palpated. Small knob like structure was felt on left side, 1.5x1.5 cm in size, firm in consistency and freely mobile. USG showed uterus of size 5x4x3 cm with collection in endrometrial cavity with? hypoplastic cervix suggestive of- Hematometra with cervical agenesis. On laparotomy, tense unicornuate uterus on right side and solid rudimentary horn on left side was found, which was attached to the uterus by peritoneal fold. Cervix was absent. Both ovaries and tubes were normal. Right cornua of uterus was completely excised leaving both the tubes and ovaries.

Achados histeroscópicos na cavidade endometrial após ablação endometrial / Hysteroscopic appearance of the endometrial cavity after endometrial ablation

OBJETIVO: Verificar o aspecto da cavidade uterina após a ablação endometrial histeroscópica, a prevalência de sinéquias após o procedimento e, com isso, avaliar a importância da histeroscopia realizada no pós-operatório dessas pacientes. MÉTODOS: Foram avaliados, retrospectivamente, os laudos dos exames de 153 pacientes que haviam sido submetidas à histeroscopia ambulatorial após ablação do endométrio devido a sangramento uterino anormal de causa benigna, no período entre janeiro de 2006 e julho de 2011. As pacientes foram divididas em dois grupos: HIST≤60 (n=90), com pacientes submetidas ao exame no período de 40 a 60 dias após o procedimento, e grupo HIST>60 (n=63), das que foram examinadas entre 61 dias e 12 meses. RESULTADOS: No grupo HIST≤60, 30% das pacientes apresentavam algum grau de sinéquia; aderências grau I foram descritas em 4,4%; grau II em 6,7%; grau IIa em 4,4%; grau III em 7,8%; e 2,2% apresentavam grau IV. No HIST>60, sinéquias foram descritas em 53,9% dos casos, 3,2% tinham sinéquias grau I; 11,1%, grau II; 7,9%, grau IIa; 15,9%, grau III; e 4,8%, grau IV. Hematometra foi descrito em 2,2% dos casos do HIST≤60 e em 6,3% no HIST>60. CONCLUSÕES: A cavidade uterina de pacientes submetidas à histeroscopia ambulatorial até 60 dias após a ablação endometrial mostrou menor número de sinéquias quando comparada com as cavidades uterinas de pacientes que foram submetidas ao exame após 60 dias. Acompanhamento em longo prazo é necessário para avaliar plenamente o impacto da histeroscopia ambulatorial após a ablação endometrial. .

PURPOSE: To examine the aspect of the uterine cavity after hysteroscopic endometrial ablation, to determine the prevalence of synechiae after the procedure, and to analyze the importance of hysteroscopy during the postoperative period. METHODS: The results of the hysteroscopic exams of 153 patients who underwent outpatient hysteroscopy after endometrial ablation due to abnormal uterine bleeding of benign etiology during the period from January 2006 to July 2011 were retrospectively reviewed. The patients were divided into two groups: HIST≤60 (n=90) consisting of patients undergoing the exam 40-60 days after the ablation procedure, and the group HIST>60 (n=63) consisting of patients undergoing the exam between 61 days and 12 months after the procedure. RESULTS: In the HIST≤60 group, 30% of the patients presented some degree of synechiae: synechiae grade I in 4.4% of patients, grade II in 6.7% , grade IIa in 4.4%, grade III in 7.8%, and grade IV in 2.2%. In the HIST>60 group, 53.9% of all cases had synechiae, 3.2% were grade I, 11.1% grade II, 7.9% grade IIa, 15.9% grade III, and 4.8% grade IV. Hematometra was detected in 2.2 % of all cases in group HIST≤60 and in 6.3% of all cases in group HIST>60. CONCLUSIONS: The uterine cavity of the patients submitted to diagnostic hysteroscopy up to 60 days after endometrial ablation showed significantly fewer synechiae compared to the uterine cavity of patients who underwent the exam after 60 days. Long-term follow-up is necessary to fully evaluate the importance of outpatient hysteroscopy after endometrial ablation regarding menstrual patterns, risk of cancer and prevalence of treatment failure. .

Cervical Stenosis: A Rare Complication of Cesarean Section.

Deliveries through cesarean section are steadily increasing since the last two decades. Although rare, complications do occur after cesarean section. A very rare complication of cesarean section is cervical stenosis, leading to hematometra and hematosalpinx. We report a case of a 38-year-old woman who presented with pain in abdomen and secondary amenorrhea since 15 years, after her last delivery which was conducted by cesarean section. On examination, a provisional diagnosis of hematometra due to cervical stenosis was made, which was confirmed at laparotomy. Due to adhesions and pinpoint stenosed cervix, she was managed by total abdominal hysterectomy with right salpingo-oophorectomy and left salpingectomy was done.

Mullerian Agenesis: An Unusual Presentation as Hematometra and Bilateral Hematosalpinx.

A case of hematometra with bicornuate uterus and bilateral hematosalpinx in a 15-year-old girl complicated by vaginal agenesis and absent cervix is presented. She was managed by abdominal hysterectomy and bilateral salpingectomy. Bilateral ovaries were conserved.

Anomalía vaginal: a propósito de un caso interesante / Vaginal abnormality: an interesting case report

Las malformaciones uterovaginales (MUV), se deben a una alteración en el proceso de embriogénesis del sistema reproductor femenino. La ecografía tiene un rol importante en el estudio inicial de las mismas, la RMN tiene ventajas importantes, es un estudio no invasivo, con una sensibilidad de casi 100%. La histerosalpingografía es la técnica clásica para el estudio de las MUV que proporciona información sobre el canal cervical, endometrial y la permeabilidad tubárica. Se demuestra la utilidad de la ecografía transabdominal en el manejo imagenológico de una MUV y sus complicaciones principales al presentar el caso de una paciente de 12 años de edad que acudió a consulta con fiebre elevada, dolor hipogástrico y oliguria/disuria sin menarquia. Al examen físico se palpó tumoración suprapúbica, abombamiento perineal e himen no tunelizado, se constató edema facial e hipertensión arterial. La ecografía reveló una moderada dilatación de los sistemas excretores de ambos riñones, la cavidad vesical notablemente distendida, visualizándose masa hipoecoica retrovesical en fosa ilíaca derecha, sin septos ni contenido, relacionada con hematometra secundaria a himen imperforado, causando hidronefrosis obstructiva bilateral que condujo a retención urinaria y de fallo renal agudo. Se realizó corrección quirúrgica de la MUV obteniéndo una evolución clínico imagenológica satisfactoria.

Uterovaginal malformations (UVM) are due to an alteration in the process of embryogenesis of the female reproductive system. The imaging has a very important role during initial studies of these abnormalities; magnetic resonance imaging has significant advantages in non-invasive studies with a sensitivity of almost 100%. Hysterosalpingography is the classic technique to the study of UVM that provides information about cervical, endometrial canals and tubal permeability. The usefulness of the transabdominal imaging in the management of a UVM and main complications was showed when presenting a case of a 12 years old patient attending to the office with high fever, hypogastric pain and oliguria/dysuria without menarche. At physical examination a suprapubic tumor was palpated, a perineal dome, a non-tunneled hymen, facial edema and hypertension were observed. Imaging revealed a moderate dilatation of the excretory systems in both kidneys, vesical cavity was notable distended, visualizing a retrovesical hypoecoid mass in the right iliac fossa, without septa or contents related to a hematometra due to an imperforate hymen provoking bilateral obstructive hydronephrosis which led to a urinary retention of acute renal failure. Surgical correction of the UVM was carried out having a satisfactory imaging-clinical progression.

Himen imperforado con hematocolpus y hematometra secundario: presentación de un caso / Imperforate hymen with Hematocolpus and Secondary Ematometra: presentation of a case

El himen imperforado es una patología poco frecuente del tracto genital femenino. Presentamos un caso de himen imperforado, en una paciente virgen de 17 años de edad con amenorrea, dolores en bajo vientre de forma cíclica con intervalos de más o menos 15 días y un aumento de volumen en hipogastrio, la que fue atendida durante nuestra misión médica realizada en el hermano país de Venezuela. Se realizó ecografía abdominopélvica con la vejiga llena y se observaron imágenes sugestivas de colpohematometra. La paciente fue intervenida quirúrgicamente, se le realizó incisión y resección de la porción central de la membrana himeneal, con evacuación del contenido sanguinolento, confirmándose el diagnóstico. No se presentaron otras complicaciones y la paciente fue dada de alta con evolución satisfactoria. Se revisó y actualizó la literatura al respecto.

The imperforate hymen is not a very frequent pathology of the feminine genital tract. A case of imperforate hymen was presented, in a 17 year-old virgin patient with amenorrhea, having pains in low stomach in a recurrent way with intervals of more or less 15 days and an increase of volume in hypogastrium, the one that was assisted during our medical mission carried out in Venezuela, our brother country. An echography was carried out abdominopelvic with the full bladder and suggestive images of colpohematometra were observed. The patient was intervened surgically. An incision and resection of the central portion of the membrane hymeneal was carried out to the patient with evacuation of the bloody content being confirmed the diagnosis. There were not any other complications and the patient was discharged from the hospital with satisfactory evolution. It was revised and it modernized the literature in this respect.

Two Cases of Imperforate Hymen with Hematocolpometra / 대한산부인과학회잡지

Imperforate hymen is a rare congenital malformation. Two cases of imperforate hymen regarded to congenital origin, and formed hematocolpometra. In most instances definite finding is noticed after menarche as retention of menstrual flow results in abdominal pain, distension of the lower abdomen and often in acute urinary retention. Two cases of imperforate hymen associated with hematocolpora, hematometra and abdominal distension at age of 14 and 16 were treated satisfactorily by simple crucial incision of hymen, and drained of about 800 cc and 600 cc of tarry blood respectively. We present two cases of imperforate hymen with review of the literatures.