Potter sequence
| Potter sequence | |
|---|---|
| Synonyms | Potter syndrome, Oligohydramnios sequence |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Pulmonary hypoplasia, renal agenesis, limb deformities, facial anomalies |
| Complications | Respiratory distress, renal failure |
| Onset | Prenatal |
| Duration | Lifelong |
| Types | N/A |
| Causes | Oligohydramnios, renal agenesis, obstructive uropathy |
| Risks | Genetic factors, maternal conditions |
| Diagnosis | Ultrasound, amniocentesis, genetic testing |
| Differential diagnosis | Meckel-Gruber syndrome, VACTERL association |
| Prevention | Prenatal care, genetic counseling |
| Treatment | Supportive care, neonatal intensive care, renal transplantation |
| Medication | N/A |
| Prognosis | Poor, often fatal |
| Frequency | Rare |
| Deaths | N/A |
Potter sequence (also known as Potter's Syndrome or Potter's Sequence) is a rare condition characterized by a lack of amniotic fluid in the womb during pregnancy, leading to fetal complications. It is named after Edith Potter, a pathologist who first described the condition in 1946.
Causes
Potter sequence is typically caused by bilateral renal agenesis (BRA), a condition in which the kidneys fail to develop in the fetus. Other causes can include chronic placental insufficiency, ruptured membranes, and other conditions that lead to a decrease in amniotic fluid.
Symptoms
The primary symptom of Potter sequence is oligohydramnios, a condition characterized by a deficiency of amniotic fluid. This can lead to a number of complications for the fetus, including pulmonary hypoplasia (underdeveloped lungs), limb deformities, and characteristic facial features (Potter's facies) such as low-set ears, a broad, flat nose, and a receding chin.
Diagnosis
Potter sequence is usually diagnosed through prenatal ultrasound, which can detect the absence of kidneys and a lack of amniotic fluid. Postnatal diagnosis can be made based on the characteristic physical features of the baby.
Treatment
There is currently no cure for Potter sequence. Treatment is supportive and depends on the severity of the condition. This may include interventions to support breathing and kidney function.
Prognosis
The prognosis for Potter sequence is generally poor, with most babies dying shortly after birth due to respiratory failure. However, some babies with less severe forms of the condition may survive with intensive medical support.
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Contributors: Prab R. Tumpati, MD