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A possible genetic disorder characterized by oligohydramnios secondary to renal diseases such as bilateral renal agenesis. Other possible renal defects include obstructive uropathy, autosomal recessive polycystic kidney disease, medullary dysplastic kidney, and renal hypoplasia. Clinical features include Potter facies (i.e., flattened nose, recessed chin), pulmonary hypoplasia, skeletal anomalies, and congenital heart defects.

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Oligohydramnios Sequence; Potter Sequence. Potter's disease; Potter facies.

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Renal agenesis occurs in 1:3000 live births and is responsible for 20% of Potter cases. Males have an increased incidence.

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Unclear. Different reports suggests an autosomal recessive, autosomal dominant or X-linked recessive. Male:female ratio of 2.5:1.

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The possible mechanism of renal agenesis or hypoplasia may be failure of the ureteric bud formation, failure of the bud to reach the metanephric blastema, or failure of the bud and the metanephric blastema to influence each other. The ensuing decreased or absent intrauterine urine production results in oligoor anhydramnios. This results in compression of the fetus and the typical anomalies found. Other causes of oligohydramnios (obstructive uropathy, polycystic kidneys, chronic leakage of amniotic fluid) produce a similar spectrum of anomalies.

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Based on ultrasonographic evidence of renal agenesis in addition to the typical clinical picture. Prenatal diagnosis of renal agenesis or hypoplasia is possible after 18 weeks of gestation.

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Potter facies: mild hypertelorism, prominent inner canthus, flattened nose, mild micrognathia, large low-set ears. Lungs: severe pulmonary hypoplasia similar to that of congenital diaphragmatic hernia. Skeletal: malformation of spine, bowing of legs, club feet, large hands. Cardiovascular: Ventricular septal defects, tetralogy of fallot, patent ductus arteriosus, endocardial cushion defect. Prognosis is very poor. Death usually shortly after birth as a consequence of pulmonary hypoplasia.

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Unlikely to present for surgery in view of poor prognosis. Assess pulmonary status. Evaluate renal function: absent in case of renal agenesis or hypoplasia, variable in other causes of oligohydramnios.

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Severe pulmonary hypoplasia requiring special techniques of mechanical ventilation. Endotracheal intubation may be difficult because of micrognathia. Renal agenesis may affect management. The association of cardiovascular malformations will influence the anesthetic management.

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According to renal function.

Jones K: Oligohydramnios sequence, in Jones KL (ed): Smith's Recognizable Patterns of Human Malformation. 5th ed. WB Saunders, Philadelphia, 1997, p 632.
Zerres K, Völpel M, Weiss H: Cystic kidneys: Genetics, pathologic anatomy, clinical picture, and prenatal diagnosis. Hum Genet 68:104, 1984.  [PubMed: 6500563]

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