POTTER SYNDROME

Course overview

POTTER SYNDROME IS A FATAL CONGENITAL DISORDER CHARACTERIZED BY THE CHANGES IN PHYSICAL APPEARANCES OF NEONATE DUE TO OLIGOHYDRAMNIOS CAUSED BY RENAL AGENESIS AND IMPAIRMENT. IT IS INCOMPATIBLE WITH LIFE AS NEONATES WITH POTTER SYNDROME HAVE PULMONARY HYPOPLASIA THAT LEADS TO RESPIRATORY DISTRESS WITHIN AN HOUR OF BIRTH. POTTER SEQUENCE AND OTTER SYNDROME ARE USED INTERCHANGEABLY BECAUSE THE SEQUENCE OF EVENTS LEADING TO OLIGOHYDRAMNIOS IS CONSISTENT. POTTER SYNDROME IS THE CONSEQUENCE OF A MULTIFACTORIAL INHERITANCE PATTERN. IT INVOLVES AUTOSOMAL DOMINANT AND RECESSIVE FORMS AS WELL AS SOME SPORADIC CASES. AUTOSOMAL DOMINANT IS THE MOST SEVERE FORM WITH INCOMPLETE PENETRANCE AND VARIABLE EXPRESSIBILITY.