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    Russell-Silver syndrome

    Silver-Russell syndrome; Silver syndrome

    Russell-Silver syndrome is a disorder present at birth involving poor growth. One side of the body also will appear to be larger than the other.

    Causes

    Up to 1 in 10 children with this syndrome have a problem involving chromosome 7. In other patients,the syndrome may affect chromosome 11.

    Most of the time, it occurs in people with no family history of the disease.

    The estimated number of people who develop this condition varies greatly. Some say it affects about 1 in 3,000 people. Other reports say it affects 1 in 100,000 people. Males and females are equally affected.

    Symptoms

    • Birthmarks that are the color of coffee with milk (cafe-au-laitmarks)
    • Curving of the pinky toward the ring finger
    • Delayed bone age
    • Failure to thrive
    • Gastroesophageal reflux disease
    • Kidney problems, such as:
      • Horseshoe kidney
      • Hydronephrosis
      • Posterior urethral valves
      • Renal tubular acidosis
    • Low birth weight
    • Large head for body size
    • Poor growth
    • Short arms
    • Short height (stature)
    • Short, stubby fingers and toes
    • Delayed stomach emptying, and constipation
    • Wide forehead with a small triangle-shaped face and small, narrow chin

    Exams and Tests

    The condition is usually diagnosed by early childhood. The doctor will perform a physical exam. Signs include:

    • Pointed chin that is not fully developed
    • Thin, wide mouth
    • Triangle-shaped face with broad forehead

    There are no specific laboratory tests to diagnose Russell-Silver syndrome. Diagnosis is usually based on the judgment of your child's pediatrician. However, the following tests may be done:

    • Blood sugar (some children may have low blood glucose)
    • Bone age testing (bone age is often younger than the child's actual age)
    • Chromosometesting (may detect a chromosomal problem)
    • Growth hormone (some children may have a deficiency)
    • Skeletal survey (to rule out other conditions that may mimic Russell-Silver syndrome)

    Treatment

    Growth hormone replacement may help if this hormone is lacking. Other treatments include:

    • Making sure the person gets enough calories, to prevent low blood sugar and promote growth
    • Physical therapy, to improve muscle tone
    • Special education, to address learning disabilities and attention deficit problems the child may have

    Many specialists may be involved in treating this condition:

    • A doctor specializing in genetics can help diagnose Russell-Silver syndrome.
    • A gastroenterologist or nutritionist can help develop the proper diet to enhance growth.
    • An endocrinologist may prescribe growth hormone, if it is needed.
    • Genetic counselors and psychologists may also be involved.

    Outlook (Prognosis)

    Older children and adults do not show typical features as clearly as infants or younger children. Intelligence may be normal, although the patient may have a learning disability.

    Possible Complications

    • Chewing or speaking difficulty if jaw is very small
    • Learning disabilities

    When to Contact a Medical Professional

    Call your health care child's provider if signs of Russell-Silver syndrome develop. Make sure your child's height and weight is measure during each well child visit. The doctor may refer you to:

    • Agenetic professional for a full evaluation and chromosome studies
    • A pediatric endocrinologist for management of your child's growth problems

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              Tests for Russell-Silver syndrome

                Review Date: 11/2/2012

                Reviewed By: Chad Haldeman-Englert, MD, FACMG, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, and Stephanie Slon.

                The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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