Silver-Russell Syndrome Treatment & Management

Updated: May 31, 2017
  • Author: Sunil Kumar Sinha, MD; Chief Editor: Luis O Rohena, MD, FAAP, FACMG  more...
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Treatment

Medical Care

Growth

Significant effort must be undertaken to optimize caloric intake.

Growth hormone therapy should be considered in a child with Silver-Russell syndrome (SRS) who has not manifested adequate catch-up growth by age 2 years. Growth hormone was approved in the United States in 2001 by the US Food and Drug Administration for use in children born small for gestational age who have not yet manifested adequate catch-up growth by age 2 years. Recombinant human growth hormone (rhGH) is given via daily subcutaneous injections. Recommended dose is 0.48 mg/kg/wk.

Development

An early intervention program, including physical therapy, is beneficial.

Special education courses are needed when the child is older.

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Surgical Care

See the list below:

  • Consider enteral feeding if the patient does not tolerate oral feeding and has severe failure to thrive. Nasogastric or percutaneous endoscopic gastrostomy (PEG) feeds are needed to facilitate growth and maintenance.
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Consultations

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  • Consult a clinical geneticist to differentiate Silver-Russell syndrome from etiologies of growth retardation, asymmetry, and other clinical findings.
  • Consult a gastroenterologist or nutritionist to optimize caloric intake and enhance growth and feeding therapy.
  • Developmental evaluation places the patient in early intervention, special education programs, physical therapy, and occupational therapy as early as possible.
  • Consult a pediatric endocrinologist to consider the use of recombinant human growth hormone (rhGH) in an infant nearing age 2 years who has not manifested adequate catch-up growth.
  • Consult an ophthalmologist to identify refractive errors and other abnormalities of vision commonly associated with Silver-Russell syndrome.
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Diet

See the list below:

  • Patients should have a nutritional evaluation to provide optimal calories for growth. As stated previously, nasogastric or PEG feeding may be required.
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