Russell-Silver Syndrome

Russell-Silver syndrome is a growth disorder characterized by slow growth before and after birth. Babies with this condition have a low birth weight (small for gestational age – SGA) and often fail to grow and gain weight at the expected rate (failure to thrive). Head growth is normal, however, so the head may appear unusually large compared to the rest of the body. Affected children are thin and have poor appetites, and some develop low blood sugar (hypoglycemia) as a result of feeding difficulties. Adults with Russell-Silver syndrome tend to be short. The average height for affected males is about 4 feet, 11 inches, and the average height for affected females is about 4 feet, 7 inches.

Many children with Russell-Silver syndrome have a small, triangular face with distinctive facial features including a prominent forehead, a narrow chin, a small jaw, and down-turned corners of the mouth. Other features of this disorder can include an unusual curving of the fifth finger (clinodactyly), asymmetric or uneven growth of some parts of the body, and digestive system abnormalities. Russell-Silver syndrome is also associated with an increased risk of delayed development and learning disabilities.

The calorific intake of children with RSS must be carefully controlled in order to provide the best opportunity for growth, if the child is unable to tolerate oral feeding then enteral feeding may be used.
In children with limb-length differences, or scoliosis, physical therapy can help alleviate the problems caused by these symptoms. In more severe cases surgery to lengthen limbs may be required. To prevent aggravating posture difficulties, children with leg length differences may require a raise in their shoe.

Growth hormone therapy is often prescribed as part of the treatment of RSS. The hormones are given by injection typically daily from the age of 2 years old through teenage years. It may be effective even when the patient does not have a Growth Hormone deficiency. Growth Hormone therapy has been shown to increase the rate of growth in patients and also prompts 'catch up' growth. This may enable the child to begin their education at a normal height, improving their self-esteem and interaction with other children. The effect of growth hormone therapy on mature and final height is as still under investigation. There are some theories suggesting that growth hormone therapy may also assist with muscular development and help control hypoglycemia in these patients.

Useful Links:

The MAGIC Foundation – Russell-Silver Syndrome
(http://www.magicfoundation.org/www/docs/112.111/russell_silver_syndrome)

Russell-Silver Syndrome Support Network
(http://russell-silversupport.org/)

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