Children and Youth with Special Health Needs
Health Condition Fact Sheet
REDUCTION DEFORMITY: LOWER LIMBS
A reduction deformity is the congenital absence of a limb or part of a limb, including a shortened limb. This is sometimes caused by prenatal exposure to certain drugs, such as thalidomide, though often the cause is unknown. It is sometimes detected before birth on prenatal ultrasound. Although there are many causes of limb reduction, women who take folic acid during pregnancy have a 30-40% reduced risk of having a child with a limb deformity.
Lower limbs are affected less often than upper limbs, occurring in 14 of 100,000 live births.
COMMON ASSOCIATED CONDITIONS
Many mothers will have the condition called oligohydramnios in the first half of pregnancy where there is less than a normal amount of amniotic fluid surrounding the growing baby. Other associated conditions occur in 30-50% of children who have limb reduction deformities. These include congenital heart disease, eye malformations, intestinal atresia, kidney malformations, unusual ear shape or placement, and birthmarks.
SHORT-TERM TREATMENT OUTCOMES
Treatment for limb reduction will be unique to each child since no two children will have the same issues. Surgery may be needed, to obtain stability of the lower limb for good weight-bearing and in order to create a good fit for a prosthesis. Since many children have other problems besides the limb reduction, the treatment plans may be relatively straight-forward, or may involve multiple subspecialists also treating the associated conditions. The Shriner's Hospitals nationwide are very focused on assisting children and families with limb reduction conditions and can be an excellent source of resources and support.
LONG-TERM TREATMENT AND OUTCOMES
As children grow they will need to be evaluated regularly to be sure that they are receiving the best possible assistance through surgery, prosthetics or other adaptive equipment to live a full and active life. They may need to have further surgeries on their affected limb, or perhaps they will be fitted with an electronic prosthesis that will give them new options for their activities.
Complications would include those that occur in any surgical procedure, such as infection, delayed wound healing, or pneumonia from the anesthetic. There can be difficulties in prostheses fitting well with children's rapid growth, and thus they need to be frequently assessed to be sure that there is not skin breakdown and ulceration, which can be very serious and difficult to treat.
IMPLICATIONS FOR CHILDREN'S DEVELOPMENT
With any visible birth defect, children and families need special reassurance and guidance to cope with the comments of others who will inquire about the difference in appearance. The family needs to be reassured that they could not have prevented this defect and nothing that they did caused it.
Raising the child to be self-confident will be very important so that the child's self-image is positive. The children should be able to fully participate in most activities, even if using a prosthesis. They may have to do things in a slightly different manner, but children can be very resourceful in adapting activities to their individual circumstances. It is therefore important for parents not to restrict them in their activities or their life goals.
Support groups might be very helpful for children and families, as well as reading about people who have led very normal and active lives. Many online resources can be found for children and families, such as those describing adapted skiing and other sport accommodations. Physical therapy, occupational therapy, and psychological counseling might be very helpful to the child and family.
Of course, any child who has to have repeated surgeries will be impacted by the pain, discomfort and limitations this imposes, as will the family. Families may feel guilty and grieve this occurrence that makes life somewhat more difficult for their child as it is natural to long for a “perfect child”. With time and support and perhaps professional help, families can find other families who are dealing well in similar circumstances and realize they are not facing this problem alone.
Focusing on the many strengths that the child has is very important, but a period of grieving is to be expected.
Families must be encouraged to provide lots of touch to the child, especially touching the affected limb so that they and the child will come to accept the whole child, including that part that is different. Adults who have grown up with limb differences speak of people (sometimes including their parents) being reluctant to look or touch the limb that is different, and thus it is important to give this guidance to families.
Coming up with script of what to say to people who ask questions that might be uncomfortable to the child and family is important. In the past children and adults with disabilities might have been hidden away rather than fully integrated into all life activities. Encountering people who are intrusive with questions or comments can be very difficult for the child and family but this will be a fact of life, so preparing a response for those situations is very helpful.
Prepared for Children and Youth with Special Health Needs by:
Linda L. Lindeke, Ph.D., R.N., C.N.P.
Associate Professor University of Minnesota
School of Nursing & Department of Pediatrics