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Potter Syndrome: Bilateral Renal Agenesis
Definition of Potter Syndrome
Bilateral renal agenesis (Potter syndrome) is a rare and always fatal malformation with bilateral absent kidneys (Potter, 1965).
Epidemiology of Potter syndrome:
- Incidence: 3–15/100.000 births for bilateral renal agenesis, 3–20/100.000 births for bilateral renal dysgenesia. In 75%, boys are affected.
Etiology of Potter Syndrome:
Recessive inheritance is likely, siblings of children with bilateral renal agenesis are also affected in 3.5%. The absence of a ureteric bud or the absence of metanephric blastema results in the complete absence of renal tissue.
The oligohydramnios and/or the lack of renal proline production leads to pulmonary hypoplasia, respiratory insufficiency and Potter facies of the children.
Signs and Symptoms of Potter syndrome
- 40% stillbirth, 90% die within 48 h.
- Oligohydramnios, low birth weight
- Potter facies: curved skin fold below the eye, low-set ears, dry wrinkled skin.
- Leg deformity (excessive flexion of hip and knees)
- Respiratory insufficiency leading to death
- Anuria and increasing retention parameters
Diagnosis of Potter Syndrome
- Maternal ultrasound screening reveals oligohydramnios and fails to detect renal tissue.
- Postnatal ultrasound, in doubtful cases static renal scintigraphy
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- Potter 1965 POTTER, E. L.:
- Bilateral Absence of Ureters and Kidneys: A Report of 50 Cases.
In: Obstet Gynecol
25 (1965), S. 3–12
Deutsche Version: Bilaterale Nierenagenesie