Potter syndrome

Potter Syndrome

Potter Syndrome (pronunciation: /ˈpɒtər/), also known as Potter's Sequence or Oligohydramnios Sequence, is a rare congenital disorder characterized by a group of features that can occur in a newborn when there is little or no amniotic fluid surrounding them in the womb. The term "Potter" is derived from the name of the American pathologist, Edith Potter, who first described the condition in 1946.

Definition

Potter Syndrome is a condition that results from renal agenesis or other severe kidney malformations. The lack of amniotic fluid, known as oligohydramnios, leads to a sequence of events including underdeveloped lungs (pulmonary hypoplasia) and distinctive facial features (Potter facies).

Symptoms

The symptoms of Potter Syndrome can vary but often include:

Pulmonary hypoplasia (underdeveloped lungs)
Distinctive facial features (Potter facies) such as wide-set eyes, flattened nose, receding chin, and low-set ears
Limb deformities
Renal agenesis (absence of one or both kidneys)

Causes

Potter Syndrome is caused by a lack of amniotic fluid in the womb. This can occur due to bilateral renal agenesis (both kidneys fail to develop), severe obstructive uropathy (blockage of normal urine outflow), or cystic kidney disease.

Treatment

Treatment for Potter Syndrome is supportive and depends on the severity of the condition. It may include respiratory support for underdeveloped lungs, dialysis for kidney failure, and surgery for urinary tract abnormalities.

Prognosis

The prognosis for Potter Syndrome is generally poor, as it is often associated with stillbirth or neonatal death due to pulmonary hypoplasia. However, some individuals with milder forms of the condition may survive with appropriate treatment and management.

External links

Medical encyclopedia article on Potter syndrome
Wikipedia's article - Potter syndrome

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