Dwarfism

Dwarfism and Short Stature Syndromes

Definitions:

Dwarfism ( n ): a genetic condition resulting in short stature

Dwarf ( n ): a diminutive human being

An extremely little person who is of proportionate stature.
A small or miniature version of something
A class of small objects

The Little People of America (LPA) define dwarfism as an adult height of 4’10" or shorter, among both men and women, as the result of a medical or genetic condition.

Medical Prognosis: The medical criteria for dwarfism vary. Short stature in dwarfs is due to a genetic condition. The majority of little people demonstrate normal intelligence, have an average life span, and enjoy good health. Conditions and severity do vary among individuals.

Terminology: Today individuals with short stature generally prefer to be described as 'Dwarfs' or 'Little People.' However, it is more important to respect the human choice, and ask them their preference, or better yet, just use their name. The term midget is very offensive and no longer used.

Intelligence: Incidence of mental retardation among little people is the same as "average-size" population. Three conditions of short stature that are linked to learning disabilities and/or mental retardation are Turner syndrome, Noonan syndrome, and Morquio syndrome.

Categories of Dwarfism:

Disproportionate - The individual has an average-sized torso with short arms and legs. It is usually caused by skeletal dysplasia or chondrodystrophy (the failure of cartilage to develop into bone).

Proportionate - In this form, the body parts are proportionate, but abnormally short. The primary cause is a growth hormone deficiency or dysfunction with the pituitary gland. These disorders are now often treatable.

Types of Dwarfism:

There are over 200 types of dwarfism. A compilation of the types of dwarfism and dysplasia types can be found at Dwarfism.org ?Types of Dwarfism page. The most common types are described below.

Achondroplasia: Achondroplasia is an autosomal dominant condition which means that there is a 50% chance of having children with achondroplasia. Eighty percent of all Little People have achondroplasia. Incidence ranges from 1 in 10,000 to 1 in 40,000. Characteristics include:

Disproportionate, average trunk with short limbs and large head.

Facial features include a large head with a prominent forehead.

The midface is often small with a flat nasal bridge and narrow nasal passage.

Lumbar lordosis

Waddling gait

Bowed legs

Reduction of elbow extension

Delayed motor milestones

Otitis media

During infancy and childhood, middle ear infections are common due to the small nasal passages, and "ear tubes" may be required to treat multiple ear infections.

Occasional airway obstruction or difficulty breathing until the airways grow

Respiratory problems can occur in infants and children which may limit aerobic fitness.

Average height is 4 ft. 0 inches.

Diastrophic Dysplasia: Diastrophic dysplasia is an autosomal recessive condition, and parents have a 25% chance of having a child with it. This is the most disabling of the types. Incidence is 1 in 110,000 births. Characteristics include:

Spinal deformity

Cleft palate

Clubfoot

Ears have cauliflower appearance.

Hand deformities

Broad fingers

Hip and knee dislocations

Orthopedic problems are frequent and ambulatory assistance required.

Usually caused by inefficient nourishment during prenatal bone growth and failure of nerve centers.

Respiratory complications cause increased infant mortality, but individuals who survive infancy have a normal life span.

The average height of adults is 118 cm (males: 86-127 cm; females 104-122 cm).

Spondyloepiphyseal Dysplasia: Incidence is one per 95,000 births. Characteristics include:

	Abnormal development of the growth plates in the vertebrae causing a short trunk and spinal and limb irregularities.
	Spinal fusion may be necessary at the C1 and C2 area.
	Clubfeet
	Cleft palate
	Barrel chest
	Short neck
	Eyesight complications.
	Average height between 3 ft. and 4 ft.

Hypochondroplasia is the term for the tallest dwarfs.

	Average adult height is 125-160 cm.
	Extremities and fingers are short but the head is of normal size.
	Many cases are overlooked because short stature can be mild.

Possible Complications in Infancy

Bran stem compression - Top of the spine is to small to accommodate the spinal cord. Symptoms can include failure to thrive and central apnea (stop breathing while sleeping). Condition is treatable through surgery.

Hydrocephalus - excess fluid on the brain. If needed, can be treated with a shunt.

Obstructive apnea - Airways too small or irregularly shaped.

	May wake frequently at night or fail to thrive.
	Child may outgrow the condition, or require supplemental oxygen or a tracheotomy until the airways grow.

Physical Education and Sport Program Considerations:

Disproportionately short limbs can limit ball handling, and athletic performance in racquet sports and certain track events, like the long jump, high jump, and triple jump. However the short limbs can be advantageous for power lifting. Additionally, swimming and bicycling are recommended due to limited impact on joints.

Modification Suggestions:

	Height adjustments for goals, volleyball nets, targets
	Modified rules to promote inclusive participation and fair scoring in a mainstream environment
	Modified equipment such as smaller balls and racquets
	Lowered swings to facilitate independence on playground

The Dwarf Athletic Association of America (DAAA, established in 1985) supports sports for little people. Common sports are basketball, volleyball, powerlifting, track, field, swimming, bowling, and boccie. National events are held in conjunction with with the annual conference of Little People of America. Qualifying athletes (4'10" or less with choldrodystrophy or related causes) also compete in the Paralympic Games and other events sponsored by the International Paralympic Committee.

Medical Considerations

For little people with nonachondroplasia, atlantoaxial instability is associated and a neck x-ray is recommended. If positive, contraindications include any undue pressure to the head or neck (heading a soccer ball, diving, gymnastics). Little people can be subject to joint defects, limiting range of motion and increasing chance of dislocations. Strenuous running may cause hip and knee joint trauma.

Little People of America

Little People of America was founded in 1957 by Billy Barty. There are almost 6,000 members, and 1 out of 2 members are of average size. LPA reaches an estimated 10 percent of the little people community.

Links

Little People of America

Dwarf Athletic Association of America

Dwarfism.org

Information Sheet Home

Information on this sheet contains only suggested guidelines. Each person must be considered individually, and in many cases, a physician's written consent should be obtained.

This web page was created by Beatrice Darden and Kristin Roth, Ph.D. students in Adapted Physical Education, Doctoral Seminar, Spring, 2002.