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Discordance for Potter's Syndrome in a Dichorionic Diamniotic Twin

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Discordance for Potter's Syndrome in a Dichorionic Diamniotic Twin medicina Case Report Discordance for Potter’s Syndrome in a Dichorionic Diamniotic Twin Pregnancy—An Unusual Case Report Stoyan Kostov 1 , Stanislav Slavchev 1, Deyan Dzhenkov 2, Strahil Strashilov 3 and Angel Yordanov 4,* 1 Department of Gynecology, Medical University Varna, 9000 Varna, Bulgaria; [email protected] (S.K.); [email protected] (S.S.) 2 Department of General and Clinical pathology, Forensic Medicine and Deontology, Medical University Varna, 9002 Varna, Bulgaria; [email protected] 3 Department of Plastic Restorative, Reconstructive and Aesthetic Surgery, Medical University Pleven, 5800 Pleven, Bulgaria; [email protected] 4 Department of Gynecologic Oncology, Medical University Pleven, 5800 Pleven, Bulgaria * Correspondence: [email protected]; Tel.: +359-98-8767-1520 Received: 15 December 2019; Accepted: 24 February 2020; Published: 4 March 2020 Abstract: Introduction: 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. Case report: 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. Conclusion: 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. Keywords: potter’s sequence; dichorionic; oligohydramnios; extrarenal features; pulmonary hypoplasia 1. Introduction Potter’s syndrome (PS) is an uncommon fatal disorder with an incidence of 1 in 4000 singleton pregnancies. Edith Potter first described it in 1946. The sequence is associated with bilateral renal agenesis, oligohydramnios, and pulmonary hypoplasia (PH). Renal abnormalities, which can include bilateral renal agenesis, severe hypoplasia, dysplasia, polycystic kidney, or obstructive uropathy, are the primary defect [1–3]. The incidence of PS in multiple pregnancies remains unknown. We report a case of PS in one of a twin pair in a dichorionic diamniotic twin pregnancy. Case studies suggest that in monoamniotic pregnancy, the affected twin has no extrarenal features of this syndrome, whereas, in diamniotic pregnancy, there are controversial reports. There are very few previous cases describing this condition in dichorionic twin pregnancy. Medicina 2020, 56, 109; doi:10.3390/medicina56030109 www.mdpi.com/journal/medicina Medicina 2020, 56, 109 2 of 9 Medicina 2020, 56, x FOR PEER REVIEW 2 of 10 2. Case Report 2. Case Report A 21-year-old female multigravida (gravida 2, parity 2, abortion 0) at 34 weeks of twin pregnancy wasA admitted 21-year-old to our female hospital multigravida with preterm (gravida prelabor 2, parity rupture 2, ofabortion membranes 0) at followed 34 weeks by of uterine twin pregnancycontractions. was There admitted was no to historyour hospital of smoking, with pret alcohol,erm prelabor toxemia, rupture exposure of to membranes drugs, radiation, followed or any by uterinediseases contractions. during pregnancy. There The was pregnancy no history was of not sm bookedoking, and alcohol, without toxemia, regular followexposure up. Thereto drugs, was radiation, or any diseases during pregnancy. The pregnancy was not booked and without regular no history of birth defects in her family members. The patient had had two previous singleton vaginal follow up. There was no history of birth defects in her family members. The patient had had two deliveries four and two years ago. The first vaginal delivery was at 35 weeks of pregnancy. The fetus previous singleton vaginal deliveries four and two years ago. The first vaginal delivery was at 35 was at cephalic presentation, female with birth weight—2055 g. The neonate had PS (absence of weeks of pregnancy. The fetus was at cephalic presentation, female with birth weight—2055 g. The kidneys, lung hypoplasia, PS facies, congenital bilateral talipes equinovarus) and died twenty minutes neonate had PS (absence of kidneys, lung hypoplasia, PS facies, congenital bilateral talipes equinovarus)after birth due and to respiratorydied twenty distress minutes syndrome after birth (Figure due to1). respiratory distress syndrome (Figure1). Figure 1. An autopsy of the first first neonate with Potter’ Potter’ss syndrome (PS). The autopsy was performed 4 years ago: ( A)) Lung Lung hypoplasia; hypoplasia; ( B) absence of left kidney; ( C) absence of right kidney. A genetic study was refused. A lung weig weightht/body/body weight ratio was not performed. The secondsecond pregnancy pregnancy went went without without any any complications, complications, and the and baby the was baby in goodwas healthin good condition. health Aftercondition. hospitalization, After hospitalization, ultrasound ultrasound examination examination revealed diamniotic revealed diamniotic twin pregnancy twin withpregnancy an absence with anof amnioticabsence of fluid amniotic in twin fluid A and in twin oligohydramnios A and oligoh inydramnios twin B. Twin in twin A and B. twinTwin BA were and intwin cephalic B were and in cephalicin breech and presentation, in breech presentation, respectively. respectively Both placentas. Both were placentas in fundal were position in fundal and position Grade and II. Vaginal Grade II.examination Vaginal examination revealed cervical revealed dilatation cervical of dilatation 5 cm. Although of 5 cm. the Although amniotic the membrane amniotic inmembrane twin A was in twinintact, A leakage was intact, of amniotic leakage fluidof amniotic during fluid examination during examination was noticed. was noticed. Twin A exhibitedexhibited signssigns ofof fetalfetal distress.distress. External cardiotocography (CTG) showed variable decelerations. WeWe decided decided to performto perform a cesarean a cesare sectionan due section to variable due decelerations,to variable anhydramnios,decelerations, anhydramnios,and prematurity and of prematurity twins. Twin of A twins. had an Twin intact A had amniotic an intact membrane amniotic and membrane an absence and ofan amniotic absence offluid. amniotic The neonate fluid. The had neonate extrarenal had manifestations extrarenal manifestations of PS and growth of PS weight—1690and growth weight—1690 g, length 32 cm, g, lengthhead circumference 32 cm, head 31circumference cm. Twin A 31 died cm. 3 hTwin after A birth died due 3 h to after respiratory birth due distress to respiratory secondary distress to PH. secondary to PH. The Apgar score at one and five minutes was two and three, respectively. There was a preterm prelabor rupture of membranes of twin B. This twin had a growth weight—1350 g, length—30 cm, head circumference—30 cm, and no visual anomalies. The Apgar score at one and Medicina 2020, 56, 109 3 of 9 The Apgar score at one and five minutes was two and three, respectively. There was a preterm prelabor rupture of membranes of twin B. This twin had a growth weight—1350 g, length—30 cm, Medicina 2020, 56, x FOR PEER REVIEW 3 of 10 head circumference—30 cm, and no visual anomalies. The Apgar score at one and five minutes was fivethree minutes and five, was respectively. three and Twin five, B respectively. had a normal Twin neonatal B had course a normal and was neonatal discharged course 14 daysand afterwas dischargeddelivery. Both 14 days babies after were delivery. male with Both two babies separate were placentas, male with amniotic two separate sacs, andplacentas, umbilical amniotic cords. Theirsacs, and umbilical umbilical cords cords. contained Their two umbilical arteries cords and a contained vein. The pregnancytwo arteries was and dizygotic a vein. asThe neonates pregnancy had wasdifferent dizygotic blood as types—Twin neonates had A—“AB” different/+/; blood Twin B—“A”types—Twin/+/. There A—“AB”/+/; was an extremely Twin B—“A”/+/. small chance There of wasmosaicism an extremely or blood small chimerism, chance whichof mosaicism is more likelyor blood to occurchimerism, in monochorionic which is more twins. likely An to autopsy occur in of monochorionictwin A was performed twins. (FigureAn autopsy2). He hadof twin typical A facialwas performed features for (Figure PS—a flattened 2). He had nose, typical low set facial ears, featuresa specific for suborbital PS—a crease,flattened and nose, a short low neck. set Anotherears, a specific facial anomaly suborbital was acrease, preauricular and a sinus,short whichneck. Anotheris not typical facial for anomaly this condition. was a Limbpreauricular malformations sinus, which were alsois not present—congenital typical for this condition. bilateral talipes Limb malformationsequinovarus. Heart,were liver,also present— spleen, anus,congenital and genitals bilateral were talipes normal. equinovarus. Further examination Heart, liver, revealed spleen, polycysticanus, and kidneysgenitals andwere lung normal. hypoplasia—lung
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