TEALE FENNING

rpwalavalkar · Teale Fenning Administrator Joined: 20 Jul 2006 Posts: 966

potter's syndrome--

Definition typical physical appearances of a fetus or neonate due to severe oligohydramnios, or anhydramnios, usually bilateral renal agenesis.

traditionally when we say potter's syndrome it usually means bilateral renal agenesis and affects 1 in 3000 approx.

Other causes maybe
urinary obstruction,
polycystic or multicystic kidney diseases,
renal hypoplasia
SROM.

classification is divided into 4 subtypes

1. Type I is due to autosomal recessive Polycystic Kidney Disease

2. Type II is due to Renal Adysplasia, characterized by the complete agenesis or absence of one kidney and the remaining solitary kidney being small and malformed. Bilateral renal agenesis is believed to be the most extreme phenotypic variation of this type.

3. Type III is due to autosomal dominant Polycystic Kidney Disease

4. Type IV is due to obstructive pathology

Features

* Potter facies: flattened nose, recessed chin, prominent epicanthal folds, low-set abnormal ears.

* Pulmonary hypoplasia: The degree of pulmonary hypoplasia depends on the degree and duration of oligohydramnios, as well as the stage of lung development at which oligohydramnios occurs.

* Features of Eagle-Barrett (prune belly) syndrome: This is an occasional cause of the Potter sequence. Neonates have a deficient abdominal wall, undescended testes, dilated ureters, and a renal pelvis.

* Skeletal malformations: Hemivertebrae, sacral agenesis, and limb anomalies may be present.

* Ophthalmologic malformations: Cataract, angiomatous malformation in the optic disc area, prolapse of the lens, and expulsive hemorrhage may be present.

* Cardiovascular malformations: Ventricular septal defect, endocardial cushion defect, tetralogy of Fallot, and patent ductus arteriosus may be present.

ref -- emedicine / potter syndrome web site / medline plus
_________________
Dr Miss. Raj Walavalkar MBBS MRCOG
TealeFenning Administrator
SR O&G Wessex Region