Herlyn-Werner-Wunderlinch: An unusual presentation in a patient with Prader-Willi syndrome.

Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear.

Tratamiento conservador del acretismo placentario / Conservative management of placenta accreta

Introducción. El acretismo placentario ha aumentado debido al incremento de la cirugía uterina previa (en particular las cesáreas). La conducta ha evolucionado de un abordaje quirúrgico radical a un tratamiento conservador. Caso clínico. Gestante de 26,1 semanas que ingresó por rotura prematura de membranas. A los 3 días se realizó una cesárea por riesgo de pérdida de bienestar fetal apreciando acretismo placentario, dejando un fragmento en el lecho cornual. Dada la ausencia de sangrado se decidió adoptar una conducta expectante. El control clínico posterior fue correcto. Los seguimientos ecográfico e histeroscópico observaron una reducción progresiva del tamaño placentario desapareciendo a los 5 meses posparto. Discusión. El manejo óptimo de la placenta acreta sigue siendo discutido en la literatura médica. En casos seleccionados, deberíamos ofrecer la posibilidad de realizar un tratamiento conservador, reduciendo la morbilidad y preservando la fertilidad de la paciente (AU)

Introduction. Placental accreta has increased because of the greater use of prior uterine surgery, especially cesarean section. Treatment has evolved from a radical surgical approach to conservative management. Case report. A woman at 26.1 weeks of pregnancy was admitted to hospital because of premature rupture of membranes. Three days after a cesarian section was performed for suspected fetal distress, we observed placenta accreta. A fragment of placenta was left in the cornual bed. Given the absence of bleeding, an expectant attitude was adopted. Subsequent follow-up showed no abnormalities. Ultrasound and hysteroscopic monitoring showed a progressive reduction of placental size until its disappearance at 5 months postpartum. Discussion. The optimal management of placenta accreta remains controversial in the literature. In selected cases, we should offer the possibility of conservative treatment, reducing morbidity and preserving the fertility of the patient (AU)

Síndrome de insensibilidad a los andrógenos como causa de amenorrea primaria / Androgen insensitivity syndrome as a cause of primary amenorrhea

El síndrome de insensibilidad a los andrógenos se caracteriza por la presencia de cariotipo 46,XY, fenotipo femenino y presencia de gónadas masculinas. Es la tercera causa más frecuente de amenorrea primaria, tras la disgenesia gonadal y la ausencia congénita de vagina (síndrome de Mayer-Rokitansky-Küster-Hauser). Se trata de una entidad de interés por su relevancia en la identificación sexual y por su posible asociación con tumores malignos de las gónadas masculinas, que hace necesario un correcto diagnóstico y tratamiento quirúrgico. En este artículo se describen dos casos de síndrome de insensibilidad a los andrógenos, con su estudio clínico-genético y tratamiento, así como su seguimiento (AU)

Androgen insensitivity syndrome is characterized by the presence of external female phenotype, 46,XY karyotype and intraabdominal testes. This syndrome is the third most frequent cause of primary amenorrhea, after gonadal dysgenesis and congenital absence of the vagina (Mayer-Rokitansky-Küster-Hauser syndrome). Androgen insensitivity syndrome is of interest due to its role in sexual identification and its possible association with malignant tumors of the male gonads, which require an accurate diagnosis and surgical treatment. We present two cases of androgen insensitivity syndrome. The results of the clinical and genetic examinations, as well as the treatment and follow-up of these two patients, are discussed (AU)