Potter Syndrome Treatment & Management

Updated: Nov 25, 2020
  • Author: Sushil Gupta, MD; Chief Editor: Craig B Langman, MD  more...
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Treatment

Medical Care

The renal function and respiratory status of neonates born with Potter syndrome must be assessed. [51] Associated anomalies of the GI, cardiovascular, and musculoskeletal systems should also be evaluated. Once the long-term prognosis of survival is determined, resuscitation and management plans should be addressed.

A neonatologist, pediatric nephrologist, pediatric pulmonologist, pediatric urologist, geneticist, and pediatric surgeon should be consulted as needed. Transfer the patient to a center where pediatric subspecialists are available for consultation.

In neonates with bilateral renal agenesis, severe neonatal respiratory distress due to associated pulmonary hypoplasia, and spontaneous pneumothorax, further treatment may not be indicated. The decision should be made after discussion with the parents and all consultants involved.

Children with Potter syndrome due to conditions such as infantile polycystic kidney disease, multicystic dysplastic kidney, hypoplastic kidney, Prune-Belly syndrome, and rupture of membranes during gestation have a higher survival rate than children with Potter syndrome due to other conditions.

Children who survive the disease require management of the following:

  • Pulmonary hypoplasia: Mechanical ventilation and chest tube placement may be indicated for ventilatory support and for the treatment of spontaneous pneumothorax.

  • Renal function: This is assessed with imaging studies and calculation of the glomerular filtration rate (GFR) by using the serum creatinine concentration.

Management of renal failure may be required. The following should be addressed:

  • Nutrition: Adequate nutrition is required. Nasogastric feeding may be indicated in infants.

  • Electrolyte abnormalities such as hypocalcemia and hyperphosphatemia can be treated with medications, including calcium carbonate and vitamin D.

  • Anemia is treated with oral or parenteral iron and erythropoietin stimulating agents.

  • Children may have hypertension from either fluid-related causes or activation of the renin-angiotensin system. Antihypertensives that may be given include diuretics, beta-blockers, calcium channel blockers, and ACE inhibitors.

  • Growth: The use of growth hormone is indicated in children with a low GFR who do not grow at a healthy rate.

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Surgical Care

A peritoneal dialysis catheter or a central venous line may be placed for dialysis, if indicated. In patients with posterior urethral valves, vesicostomy or valve ablation may be indicated. [52]  Nephrectomy may be indicated in the case of a large polycystic kidney. G-tube placement may be required for adequate nutrition.

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Diet and Activity

Appropriate restriction of fluid during renal failure is indicated. In addition, nutrition with adequate protein and caloric intake is indicated. Children with hypertension must avoid excessive salt intake.

No restriction of physical activity is needed for children who survive the neonatal period.

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