González García Gonzalo, Bretón Hernández Patricia, Boillos Calvo María J, Puigbert Anna Miralles
Hospital Clínico Universitario Lozano Blesa, Zaragoza, España.
Hospital de Palamós, Gerona, España.
Arch Argent Pediatr. 2017 Jun 1;115(3):e150-e152. doi: 10.5546/aap.2017.e150.
Transverse vaginal septum is a disorder that would arise from a failure in the fusion and/or channeling of the urogenital sinus and the müllerian ducts with low frequency but with a significant impact on the health of patients. The treatment is surgical and it will depend on the location and thickness of the septum. Sometimes, it described a female sex-limited autosomal recessive transmission but few cases of family association have been reported. We present the cases of two sisters who had this rare entity 5 years apart and were diagnosed by the presentation of an intense hypogastric abdominal pain at the age of 11 and 12 years when they had a Tanner stage III pubertal development and primary amenorrhea.
阴道横隔是一种因泌尿生殖窦与苗勒管融合和/或管道形成失败而产生的疾病，发病率较低，但对患者健康有重大影响。治疗方法为手术，具体取决于隔膜的位置和厚度。有时，它被描述为女性特有的常染色体隐性遗传，但很少有家族关联病例的报道。我们报告了两姐妹的病例，她们相隔5年患此罕见疾病，分别在11岁和12岁、坦纳青春期发育III期且原发性闭经时，因剧烈的下腹部疼痛而被诊断出来。
