Orkide KUTLU, İBRAHİM ŞAHİN, ABDULLAH SAKİN, Bahri EVREN

Amenore ile seyreden De Morsier Sendromu: Olgu sunumu

Septa Optik Displazi, De Morsier Sendromu olarak da bilinen nadir bir konjenital durumdur. Sendromun klasik triadındaki bulgular; beyin orta hat yapılarında anomaliler, optik sinir hipoplazisi ve hipofiz hormon disfonksiyonlarıdır. Triaddaki bulguların en az ikisinin bulunması ile tanı konulur. Burada primer amenore ile gelen ve de Morsier sendromu tanısı konulan bir olguyu sunduk. Optik sinir hipoplazisi, hipofizer yetmezlik veya beyin orta hat yapılarda anomali saptanması durumunda triada ait diğer bulguları araştırmalıdır ve gereğinde hormon replasman tedavileri başlanmalıdır.

De morsier syndrome presenting with amenorrhea: A case report

Septo-optic dysplasia, also known as de Morsier Syndrome is a rare congenital condition. The classic triad features of the syndrome are; midline brain abnormalities, optic nerve hypoplasia and pituitary endocrine dysfunction. The diagnosis of Septo -optic dysplasia is made when two or more features of the classic triad are present. Here, we presented a case with de Morsier Syndrome. The physician diagnosed optic nerve hypoplasis or midline brain abnormalities should be searched for the other findings of the triad of Septo -optic dysplasia a nd scheduled proper replacement therapy and follow the patient.

Tam Metin

Kaynakça

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