Abstract
Potter sequence is a rare congenital malformation that primarily affects male fetuses and is characterized by pulmonary hypoplasia, skeletal malformation, and kidney abnormalities. The pressure of the uterine wall due to oligohydramnios leads to an unusual facial appearance, abnormal limbsor limbs in abnormal positions or contractures. The fetus generally dies soon after birth due to respiratory insufficiency. We presented a male baby of 35 wks gestation with birth weight 1200gms delivered by primi mother. She had severe oligohydramnios and virtually there was no liquor during birth. The baby had severe perinatal depression at birth requiring resuscitation. Multiple congenital anomalies like absence of left eye, congenital cataract on the right eye, right-sided choanal atresia, micrognathia, low set ears, beaked nose, bilateral clubbed foot with hip deformity were noted. After 2 hours of life,baby developed fast breathing and cyanosis and died due to respiratory failure.
Highlights
Potter sequence was first described by Edith Potter 1946 at the Chicago Lying Hospital in the U.S.A that consisted of facial characteristics in infants with bilateral renal agenesis.[1]
The resulting oligohydramnios is the cause of the typical facial appearance of the fetus, which is known as “Potter’s facies” which consists of a flattened nose, recessed chin, epicanthal folds and low-set abnormal ears.[4]
Despite the amniotic fluid index being zero from the 18th week of gestation, pregnancy was continued till 35 weeks and a male baby weighing 1.2kg was born with typical Potter’s facies that died shortly after birth
Summary
Potter sequence was first described by Edith Potter 1946 at the Chicago Lying Hospital in the U.S.A that consisted of facial characteristics in infants with bilateral renal agenesis.[1]. Potter sequence was first described by Edith Potter 1946 at the Chicago Lying Hospital in the U.S.A that consisted of facial characteristics in infants with bilateral renal agenesis.[1] The sequence with an incidence of 1 in every 2,000 to 5,000 fetuses is associated with a recurrence risk of 3-6% and is found in 0.2-0.4% of autopsies in dead new-borns or those who die immediately after birth.[2] It includes clubbed feet, pulmonary hypoplasia and cranial anomalies related to oligohydramnios.
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