Síndrome de Mayer-Rokitansky-Kuster-Hauser (MRKH) a propósito de un caso
DOI:
https://doi.org/10.18041/2665-427X/ijeph.1.8663Palabras clave:
Síndrome de Mayer-Rokitansky-Kuster-Hauser, MRKH, resección septum, preservación de la fertilidad, trastorno congénito , anomalia, sindromeResumen
Antecedentes: El Síndrome de Mayer-Rokitansky-Küster-Hauser (MRKH) se da en 1:5,000 nacimientos. Es un trastorno congénito en la formación de los conductos de Müller y por ello presentan una amenorrea primaria, debido a ausencia de vagina y útero, con ovarios funcionales. Se desarrollan los caracteres sexuales secundarios, teniendo un cariotipo femenino normal (46, XX). Las mujeres con la afección sufren de infertilidad por factor uterino que forma parte de la infertilidad multifactorial en un 50% de casos; pero, considerado como factor responsable único, su incidencia está entre 5-12%.
Objetivo: Reportar anomalía de MRKH de paciente pediátrico con descripción de opciones de manejo.
Presentación de caso: Femenina de 15 años, desarrollo mamario Tanner IV, vagina ausente y amenorrea. Estradiol 56 pg/ml, FSH 5.7 mUI/ml, LH 10 mUI/ml, cariotipo 46,XX. Ecografía pélvica muestra ovarios presentes, restos uterinos. Resonancia magnética simple pélvica: restos uterinos, ovarios presentes, agenesia de vagina. Ecografía renal y radiografía de tórax normales. Cirugía vaginoplastia, retiro de restos uterinos. Vida sexual actual activa normal.
Conclusión: El diagnóstico del síndrome MRKH tipo 1 a menudo ocurre en la adolescencia tardía, cuando consulta porque no ha comenzado su período menstrual. Un diagnóstico de MRKH Tipo 2 puede ser provocado por un período ausente o una amplia gama de otros síntomas o anomalías relacionados con los órganos, a veces a una edad más temprana. Existen opciones quirúrgicas y no quirúrgicas para tratar la MRKH. Dependiendo de su condición individual, puede beneficiarse. Estos pacientes requieren un manejo integral e interdisciplinar para favorecer su rehabilitación total.
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