Potter Syndrome Treatment & Management

  • Author: Sushil Gupta, MD; Chief Editor: Craig B Langman, MD   more...
 
Updated: Jun 30, 2010
 

Medical Care

The renal function and respiratory status of neonates born with Potter syndrome must be assessed.[15] 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.

  • 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, Prader-Willi 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.
    • 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.
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Consultations

A neonatologist, pediatric nephrologist, pediatric pulmonologist, geneticist, and pediatric surgeon should be consulted as needed.

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Diet

  • Appropriate restriction of fluid during renal failure is indicated. Nutrition with adequate protein and caloric intake is indicated.
  • Children with hypertension must avoid excessive salt intake.
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Activity

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

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Contributor Information and Disclosures
Author

Sushil Gupta, MD  Fellow, Department of Pediatric Nephrology, University Of Florida College of Medicine

Sushil Gupta, MD is a member of the following medical societies: American Society of Nephrology, American Society of Pediatric Nephrology, Indian Medical Association, and National Kidney Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Carlos E Araya, MD  Clinical Assistant Professor, Division of Pediatric Nephrology, University of Florida College of Medicine; Medical Director, Pediatric Dialysis Unit, Shands Children's Hospital

Carlos E Araya, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Nephrology, and International Pediatric Transplant Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Laurence Finberg, MD  Clinical Professor, Department of Pediatrics, University of California at San Francisco and Stanford University

Laurence Finberg, MD is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Frederick J Kaskel, MD, PhD  Director of the Division and Training Program in Pediatric Nephrology, Vice Chair, Department of Pediatrics, Montefiore Medical Center and Albert Einstein School of Medicine

Frederick J Kaskel, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Pediatric Society, American Physiological Society, American Society of Nephrology, American Society of Pediatric Nephrology, American Society of Transplantation, Eastern Society for Pediatric Research, Federation of American Societies for Experimental Biology, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Renal Physicians Association, Sigma Xi, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Howard Trachtman, MD  Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine

Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD  The Isaac A Abt, MD, Professor of Kidney Diseases, Feinberg School of Medicine, Northwestern University; Division Head of Kidney Diseases, Children's Memorial Hospital, Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology

Disclosure: Amgen Grant/research funds None; Genzyme Grant/research funds None; Merck Grant/research funds None; NIH Grant/research funds None; Raptor Pharmaceuticals, Inc Grant/research funds None; Alexion Pharmaceuticals, Inc. Grant/research funds None

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Sonogram obtained before second-trimester amnioinfusion. This fetus has bilaterally absent kidneys consistent with a diagnosis of Potter syndrome. The cystic structures in the renal fossae are most likely the adrenal glands.
 
 
 
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