Potter Sequence

Overview


Plain-Language Overview

Potter Sequence is a condition that affects babies before birth due to problems with the kidneys. It causes a lack of amniotic fluid, which is the fluid surrounding the baby in the womb. This fluid is important for protecting the baby and helping the lungs develop properly. Without enough fluid, the baby may have distinctive facial features, such as a flat nose and low-set ears, and the lungs may not grow well, leading to breathing difficulties after birth. The condition mainly involves the urinary system and the lungs. It can also cause problems with the baby's limbs and overall growth. The severity of the condition depends on how much amniotic fluid is missing and the underlying kidney problem.

Clinical Definition

Potter Sequence is a constellation of findings resulting from severe oligohydramnios caused by bilateral renal agenesis or other causes of impaired fetal urine production. The core pathology is the absence or dysfunction of the fetal kidneys, leading to decreased amniotic fluid volume, which in turn causes characteristic facial anomalies, limb deformities, and pulmonary hypoplasia. The sequence is clinically significant because the resulting pulmonary hypoplasia is often fatal due to respiratory insufficiency. The condition is a classic example of a sequence, where a single initiating defect (renal agenesis or obstruction) leads to a cascade of secondary malformations. It is important to distinguish this from isolated renal anomalies because of the associated systemic effects and poor prognosis.

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Clinical Presentation


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