Dwarfism

Dwarfism – Restricted Growth/Short Stature

Dwarfism is restricted growth, a condition which is characterised by short stature and could be caused due to several various medical conditions. It is generally defined as an adult height of 4 feet 10 inches or less and the average adult height with people of dwarfism is 4 feet.

This disorder is divided into two categories namely: proportionate short stature – PSS, which is a general lack of growth and disproportionate short stature – DSS, a problem with the way some bones tend to grow. In the case of Proportionate shorty stature the length of the trunk, which is the abdomen and chest, will have a normal proportion with the legs though they will have a general lack of height. Common cause of this disorder is being born to small parents and can also occur when the body is not capable of producing enough growth hormone.

There are certain types of genetic syndrome which may also cause PSS. For instance, girls with Turner syndrome and children have genetic abnormality like SHOX, gene haploinsufficiency may have a mild form of PSS. Proportionate dwarfism could be due to medical conditions that may be present at birth or appear in the early stage of childhood which limits the overall growth and development where the head, trunk and the limbs are small though proportionate to each other. The outcome of these disorders could affect the overall growth, most of which could end in poor development of one or more body systems.

Disproportionate Short Stature – DSS

Disproportionate short stature – DSS is when a problem occurs with the way joints and bones grow where certain limbs tend to be shorter, or the trunk could be particularly short. There could be a severe lack of height. Conditions related to DSS are often caused by faulty gene and several children born with this disorder have parents of average height while the faulty gene occurs by chance.

These people are treated based on the cause of short stature which is likely to involve several different forms of healthcare professionals. Should the legs be particularly short, DSS could be treated with a leg lengthening procedure though it is uncertain regarding the effectiveness as well as the safety of this and certain children who have restricted growth could be eligible for treatment with human growth hormone.

Most of the people with disproportionate dwarfism tend to have normal intellectual potentials with only rare exceptions to the rule which are the result of a secondary factor like excess fluid around the brain known as hydrocephalus. Latest study shows that Dwarfism gives protection from diabetes and cancer.

This disorder is due to achondroplasia that causes disproportionate short stature resulting in an average size trunk, short fingers, with wide separation between the middle and ring fingers, short arms and legs, particularly short upper arms and upper legs, disproportionately large head, having a prominent forehead with a flattened bridge of the nose, progressive development of bowed legs/swayed lower back and limited mobility at the elbows.

Surgical/Hormone Therapy

Surgical procedure which could resolve problems with disproportionate dwarfism comprises of correcting the direction the way the bones tend to grow, stabilizing and correcting the shape of the spine, increase the size of the opening in bones of the spine, to alleviate pressure on the spinal cord and placing of a shunt to eliminate excess fluid if present, around the brain.

Hormonetherapy is for those with hormone deficiency which involves treatment with injection, a synthetic version of the hormone which could increase the overall height. Regular check-ups with the physician are essential which could help in improving the quality of life in dwarfism.