• Stoyan Kostov, Stanislav Slavchev, Deyan Dzhenkov, Strahil Strashilov, and Angel Yordanov.
• Department of Gynecology, Medical University Varna, 9000 Varna, Bulgaria.
• Medicina (Kaunas). 2020 Mar 4; 56 (3).

IntroductionPotter's syndrome, also known as Potter's sequence, is an uncommon and fatal disorder. Potter's sequence in a multiple pregnancy is uncommon, and its frequency remains unknown. Worldwide in a diamniotic twin pregnancy, there are only a few cases described.Case ReportWe present an unusual case discordance for Potter's syndrome in a dichorionic diamniotic twin pregnancy. Twin A had the typical physical and histological Potter's findings. Twin B had normal respiratory function and normal physical examination findings. There are many controversies about this condition in diamniotic twin pregnancy. One case report concluded that that the presence of a normal co-twin in diamniotic pregnancy prevented the cutaneous features seen in Potter's syndrome and ameliorated the pulmonary complications, whereas two other case studies reported that the affected twin had extrarenal features typical of the syndrome.ConclusionWe performed an autopsy and calculated lung weight/body weight ratio to diagnose pulmonary hypoplasia. Histopathologic examination of lungs and kidneys was performed. We concluded that the appearance of extrarenal features in the affected twin depends on the amniocity.

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