Department of Gynecology, Medical University Varna, 9000 Varna, Bulgaria.
Department of General and Clinical pathology, Forensic Medicine and Deontology, Medical University Varna, 9002 Varna, Bulgaria.
Medicina (Kaunas). 2020 Mar 4;56(3):109. doi: 10.3390/medicina56030109.
Potter'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.
We 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.
We 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.
波特氏综合征，又称波特氏序列，是一种罕见且致命的疾病。多胎妊娠中的波特氏序列并不常见，其频率尚不清楚。在全世界的双羊膜囊双胎妊娠中，仅有少数病例被描述。
我们报告了一例在双羊膜囊双胎妊娠中出现波特氏综合征不一致表现的罕见病例。胎儿 A 具有典型的体格和组织学波特氏发现。胎儿 B 呼吸功能正常，体格检查正常。对于这种情况在双羊膜囊妊娠中存在许多争议。一份病例报告得出结论，正常的同卵双胎的存在可以预防在波特氏综合征中所见的皮肤特征，并改善肺部并发症，而另外两份病例研究报告称，受影响的胎儿有典型的肾脏外综合征特征。
我们进行了尸检并计算了肺重/体重比以诊断肺发育不良。对肺和肾进行了组织病理学检查。我们得出结论，受影响胎儿的肾脏外特征的出现取决于羊水量。
