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The information below is summarised from the booklet
"Intrauterine Growth Retardation (IUGR) including Russell Silver
Syndrome - A Guide for parents and patients" available from the CGF.
Russell Silver Syndrome is very rare occurring in
approximately 1/75000 births. Little is known about the cause of this
condition, in the majority of families only one child is affected but
very occasionally families do have more than one affected child, This
may suggest a genetic basis for this condition and this is the subject
of much research. In some children with the milder forms of IUGR a
genetic irregularity has been found.
Diagnosis - Intrauterine Growth Retardation
A low
birth weight is defined as a baby born with a
weight that is inappropriately low for the duration of the pregnancy
- for a baby born at term this would be a birth weight less than
2.5kg. The inappropriately low weight indicates that the growth of
the baby in the womb has been unsatisfactory and this is why is is
called Intrauterine Growth Retardation.
The majority of
babies born small for their gestational age show catch-up growth
over the first two or three years of life. However, in about a third
complete catch-up growth does not occur. These children remain small
and fail to reach their genetic potential as defined by their
parental heights.
The diagnosis of IUGR is based on comparison of the
babies weight centile at birth with standards which take into account
he gestational age of the baby, ideally infant length should also be
taken into account.
Diagnosis - Russell Silver Syndrome
The diagnosis of RSS is based on that of IUGR. In
addition some of the following symptoms are often present
- Feeding problems - early feeding
problems are common. The baby is often disinterested in feeding and
takes only small amounts with difficulty
- Sweating - the babies sweat a lot, especially at
night, and have a greyness or pallor of the skin. In some infants,
this is a symptom of a low level of sugar in the blood
(hypoglycaemia). As they get older, these infants/children may have
altered behaviour, such as hyperactivity or, conversely, tiredness,
as a symptom of their low blood sugar level
- Face - the face is triangular shaped with a small
jaw and a pointed chin. The mouth tends to curve down
- Eyes - a blue tinge to the whites of the eyes in
younger children
- Head - the head circumference may be of normal
size, which means it can appear large in comparison to a small body
size
- Fontanelle - the opening between the bones of the
skull, the fontanella, may be very wide and late to close in babies
- Clinodactaly - the little finger on each hand may
be small and curve inwards.
- Body asymmetry - one side of the body grows more
slowly than the other
- Continued poor growth - with no "catch up" into
the normal centile lines on the growth chart
- Puberty - may commence at an early age.
Overview
The term IUGR probably represents
a spectrum of conditions (some of which are described as Russell
Silver syndrome) resulting from abnormal foetal growth. As with any
syndrome, not every child will have all the features described. The
child who has IUGR, but has not experiences 'catch-up' growth during
the first year of life, will remain small for their age and probably
very thin. Their final height may be in the region of 157cm (5'2")
for a boy and 144cm (4'9") for a girl.
There are many features of IUGR
which may require medical help and support and it is important that
you discuss any concerns that you have with your growth specialist
who can then refer your child to another appropriate specialist if
necessary. This may be especially relevant when considering leg
asymmetry and the referral to an orthopaedic surgeon with experience
in the procedure of limb lengthening (and not shortening).
There is no definitive way to help
with achieving weight gain although it often causes much concern.
These children are healthy and active, and confrontation over food
should be avoided if possible. There is no treatment at present that
can be of certain long-term benefit for growth. The use of growth
hormone appears to improve the rate of growth in the first five
years of treatment, but longer-term benefit is as yet uncertain and
clinical studies are still ongoing. The estimates of adult height
after growth hormone treatment will not be known until these studies
are complete.
Further Reading
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