Potter

Potter sequence
Synonyms	Potter syndrome, Oligohydramnios sequence
Pronounce	N/A
Specialty	N/A
Symptoms	N/A
Complications	N/A
Onset	N/A
Duration	N/A
Types	N/A
Causes	N/A
Risks	N/A
Diagnosis	N/A
Differential diagnosis	N/A
Prevention	N/A
Treatment	N/A
Medication	N/A
Prognosis	N/A
Frequency	N/A
Deaths	N/A

Potter sequence is a rare congenital disorder characterized by a distinct set of physical features and complications resulting from oligohydramnios, a condition where there is a deficiency of amniotic fluid surrounding the fetus during pregnancy.

Etiology

Potter sequence is primarily caused by renal agenesis, where the kidneys fail to develop. This leads to oligohydramnios, as the fetal kidneys are responsible for producing a significant portion of the amniotic fluid. Other causes include obstructive uropathy, polycystic kidney disease, and chronic leakage of amniotic fluid.

Clinical Features

The lack of amniotic fluid results in compression of the fetus, leading to characteristic physical features known as Potter facies, which include:

• Flattened nose
• Recessed chin
• Low-set ears
• Limb deformities

Additionally, the lack of amniotic fluid can lead to pulmonary hypoplasia, a condition where the lungs are underdeveloped, often resulting in severe respiratory distress at birth.

Diagnosis

Diagnosis of Potter sequence is typically made through prenatal ultrasound, which can reveal oligohydramnios and associated anomalies. Genetic testing and amniocentesis may be performed to identify underlying genetic causes.

Management

Management of Potter sequence is challenging and primarily supportive. In cases where pulmonary hypoplasia is severe, neonatal intensive care and ventilatory support may be required. Long-term management may involve renal replacement therapy or kidney transplantation if the child survives the neonatal period.

Prognosis

The prognosis for infants with Potter sequence is generally poor, especially when associated with bilateral renal agenesis and severe pulmonary hypoplasia. Survival beyond the neonatal period is rare without significant medical intervention.

See Also

• Oligohydramnios
• Renal agenesis
• Pulmonary hypoplasia

External Links

• Potter Sequence - NCBI Bookshelf
• Potter Sequence - Genetic and Rare Diseases Information Center