Müllerian Aplasia with Severe Hematometra: A Case Report of Diagnosis and Management in a Low Resource Setting.

BACKGROUND: Müllerian aplasia or Mayer-Rokitansky-Küster-Hauser syndrome is described as congenital absence of the proximal vagina with or without absence of the cervix and uterus, most often recognized after the onset of primary amenorrhea. CASE: An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam was significant for uterus palpable superior to the umbilicus, absence of vagina, and rectal exam without palpable vagina or cervix. Transabdominal and transperineal ultrasound examinations did not reveal hematocolpos. Exploratory laparotomy revealed severe endometriosis with bilateral hematosalpinx, markedly distended uterus, no proximal vagina, and normal ovaries. Uterine specimen was filled with blood and no clear cervix was present. SUMMARY AND CONCLUSION: Diagnosis of vaginal and cervical agenesis is complicated in low-resource settings and treatment must be modified when subspecialty care and consistent follow-up are not available.

McKusick Kaufman syndrome, complications arising at puberty.

BACKGROUND: McKusick Kaufman Syndrome (MKS), a rare genetic condition, presents in the neonatal period with a classic triad of postaxial polydactyly, congenital heart disease, and hydrometrocolpos. The diagnosis is typically clinical, based on the presence of polydactyly and hydrometrocolpos. CASE: We report the case of a 13-year-old female, who was diagnosed with MKS in infancy and underwent vaginal reconstructive surgery for a urogenital sinus. She was lost to follow-up thereafter. She presented to our institution at age 13 with pyometra, pyosalpinx, and tubo-ovarian abscess due to a stenotic cervix obstructing menstrual outflow. SUMMARY AND CONCLUSION: Gynecologic follow-up is imperative in patients with history of vaginal reconstruction to monitor for hematometra from outflow obstruction to prevent life threatening secondary bacterial infections.

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 (AU)

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 (AU)

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.

Hematocervix y sinequia cervical: caso clínico / Hematocervix and cervical adhesion: clinical case

We report a case of hematocervix diagnosed by ultrasonography in a perimenopausal patient with no history of cervical pathology. We discuss the etiology of this alteration, usefulness of ultrasound diagnosis, its surgical treatment and its relationship with pathologies affecting some other organs and systems.

Se presenta un caso clínico de hematocervix diagnosticado por ultrasonografía en una paciente perimenopáusica sin antecedentes de patología cervical. Se discuten las causas de esta alteración, la utilidad del diagnóstico por ultrasonido, su tratamiento quirúrgico y su relación con patologías en otros órganos y sistemas.

[The imperforate hymen. A clinical case]. / Himen imperforado. Caso clínico.

A clinical case of imperforate hymen is presented. The echography rule is emphasized.