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Anomalía vaginal: a propósito de un caso interesante / Vaginal abnormality: an interesting case reportAnomalía vaginal: a propósito de un caso interesante / Vaginal abnormality: an interesting case reportLas 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.Palabras clave: ANOMALÍAS GENITOURINARIAS, HEMATOCOLPOS, ULTRASONOGRAFÍA, HEMATÓMETRA.ABSTRACTUterovaginal 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. Key words: UROGENITAL ABNORMALITIES, HEMATOCOLPOS, ULTRASONOGRAPHY, HEMATOMATRA.

SUMMARY

ABSTRACTUterovaginal 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. Key words: UROGENITAL ABNORMALITIES, HEMATOCOLPOS, ULTRASONOGRAPHY, HEMATOMATRA.

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Déficit de 21-hidroxilasa: aspectos actuales / Deficiency of 21-hydroxylase: current aspectsDéficit de 21-hidroxilasa: aspectos actuales / Deficiency of 21-hydroxylase: current aspectsLa hiperplasia suprarrenal congénita (HSC) es una de las alteraciones autosómicas recesivas más frecuentes, caracterizada por un defecto enzimático en la síntesis de cortisol, la causa es en el 95% de los casos, la deficiencia de la enzima 21-hidroxilasa (21-OH). La 17-OH progesterona, precursor del cortisol, presenta valores elevados, marcadores del diagnóstico. Esta enfermedad presenta diferentes formas clínicas: las clásicas o graves comienzan desde el período neonatal, con síntomas debidos al exceso de andrógenos suprarrenales como virilidad y ambigüedad de los genitales externos de las niñas afectadas. En más del 70% de los casos se asocia con pérdida salina (deficiencia de aldosterona), potencialmente letal en varones que no se diagnostican precozmente. Resumimos las diferentes formas de presentación de la deficiencia de 21-OH, y describimos el diagnóstico y tratamiento. Los programas de detección precoz evitan la asignación incorrecta de sexo en la recién nacida y pueden salvar la vida de los varones con formas graves y pérdida salina. Comentamos el diagnóstico genético-molecular del CYP21A2 (cromosoma 6p 21.3). Revisamos las directrices futuras para el estudio y el tratamiento de esta enfermedad, incluyendo diversos tratamientos como la hormona de crecimiento, los antagonistas de las gonadotropinas y otros. El diagnóstico y tratamiento prenatales del feto femenino afectado son posibles, y los resultados son alentadores. Comentamos también, el abordaje hacia la transición y edad adulta, y la relevancia del control de la mujer con HAC durante la gestación. Palabras clave: Hiperplasia/diagnóstico/genética, esteroide 21-hodroxilasa/metabolismo, genitales/anomalías. ABSTRACT Congenital adrenal hyperplasia (CAH) is one of the most frequent autosomal recessive disorders. It is characterized by a deficiency of an enzyme involved in cortisol synthesis and in 95% of patients the cause is 21-hydroxylase deficiency. A diagnostic marker is elevated levels of 17-hydroxyprogesterone, a precursor of cortisol. CAH has several clinical forms, and classical or severe forms manifest in the neonatal period with symptoms due to excess adrenal androgen production such as virilization and ambiguity of the external genitalia in affected girls. In more than 70% of patients, there is associated salt wasting (aldosterone deficiency), which can be fatal in males without an early diagnosis. We summarize the various forms of presentation of 21-hydroxylase deficiency and describe diagnosis and treatment. Screening programs avoid incorrect sex assignment in the newborn and can save the lives of males with severe forms and salt wasting. We discuss the genetic-molecular diagnosis of CYP21A2 (chromosome 6p 21.3). We review future recommendations for the study and management of this disease, including several treatments such as, growth hormone, and gonadotrophin antagonists. Prenatal diagnosis and treatment in affected female fetuses are feasible and the results are encouraging. We also discuss the management of the transition to adulthood and the importance of follow-up of women with CAH during pregnancy.Key Words: Hyperplasia/diagnose/genetics, 21-steroid-hydroxylase/metabolism, genitals/anomalies.

Deysi Licourt Otero,Martha María Pérez Martín    



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