Short Stature

Short stature
Classification and external resources
ICD-10	E34.3
ICD-9	783.43
DiseasesDB	18756
MedlinePlus	003271

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayesha Javid, MBBS[2]

Overview

Short stature is characterized as a condition in which the height of the person in the 3rd percentile is the average height of the age , sex, and population group. According to Ranke (1996), “Short stature is defined as a condition in which the height of an individual is two standard deviations (SD) below the corresponding mean height of a given age, sex and population group.”

Classification

There are two types of short stature,

Proportionate short stature (PSS)

Proportionate short stature (PSS) is when the person is small, but limbs and trunk height are in proportions.

Disproportionate short stature (DSS)

Whereas in disproportionate short stature (DSS), the normal proportion of limbs and trunk height is absent, and the individual shows a great difference in their sitting and standing height.

Causes

PATHOLOGIC CAUSES OF GROWTH FAILURE

Systemic disorders with secondary effects on growth

• Undernutrition
• Glucocorticoid therapy
• Gastrointestinal disease
• Rheumatologic disease
• Chronic kidney disease
• Cancer
• Pulmonary disease
• Cardiac disease
• Immunologic disease
• Metabolic diseases

Genetic Causes

Several genetic disorders have prominent effects on growth.

• Turner's Syndrome
• SHOX gene variant
• Prader-Willi Syndrome
• Noonan syndrome
• Silver-Russell syndrome
• Skeletal dysplasias/growth plate abnormalities

Endocrine Causes

• Cushing syndrome
• Hypothyroidism
• Growth hormone deficiency

Psychosocial short stature

It is a growth disorder that is observed between the ages of 2 and 15, caused by extreme emotional deprivation or stress.

The symptoms include decreased growth hormone (GH) and somatomedin secretion, very short stature, weight that is inappropriate for the height, and immature skeletal age. This disease is a progressive one, and as long as the child is left in the stressing environment, their cognitive abilities continue to degenerate. Though rare in the population at large, it is common in feral children and in children kept in abusive, confined conditions for extended lengths of time. It can cause the body to completely stop growing but is generally considered to be temporary; regular growth will resume when the source of stress is removed

Differential Diagnosis

• Endocrine disorders: Growth hormone deficiency (GHD), insulin-like growth factor-1 (IGF-1), growth failure, constitutional growth delay, growth failure, stunted growth, structural brain abnormalities, or pituitary lesions, pituitary microadenomas, congenital hypothyroidism, idiopathic short stature, intrauterine growth deficiency, etc.

• Genetic disorders: Down syndrome, Turner syndrome, 3M syndrome, Noonan syndrome, Prader-Willi syndrome, Aarskog syndrome, Silver-Russell syndrome, etc.
• Bone diseases: Dwarfism, Achondroplasia (short-limbed dwarfism), diastrophic dysplasia (short-limbed dwarfism), spondylo-epiphyseal dysplasia (short-trunk dwarfism), rickets, etc
• Chronic disorders: Cystic fibrosis, Crohn disease, juvenile idiopathic arthritis (JIA), anemia, chronic renal insufficiency, inflammatory bowel disorder, chronic malnutrition, etc
• Psychological distress

Epidemiology and Demographics

In the United States, 2.5% of the population is short. A study conducted on the school children age 4-16 years in the South Indian Population shows the overall prevalence of short stature was 2.86%.

Gender

• People of both sexes are affected, but
• A retrospective study in South China showed that more boys were admitted than girls. This could be due to the reason that males are more likely to be brought to medical attention because of the prevalence of social expectations and pressures as compared to the females.
• On the other hand, in Rosario, Argentina, the higher prevalence was found in females (16.4%) than males (8.4%) (p<0.001).[21] The short stature in females was related to age, weight, and abdominal obesity.

Age

• Different ethnic groups have different average statures, which are essential to take into consideration while comparing mean parameters.
• Any individual who has not attained the union of epiphyseal plates, can get affected.

Treatment

Medical Therapy

• The primary management of short stature should be to treat the underlying cause.

• If the short stature is caused due to a hormonal deficiency it should be managed with hormonal treatment.

• These include treating growth hormone deficiencies and constitutional growth delays with gonadotropin-releasing hormone analogues (GnRHa), aromatase inhibitors, recombinant human insulin-like growth factor- 1 (RhIGF-1), low-dose androgen therapy, recombinant human growth hormone (rhGH) etc.

• In addition to treating the underlying cause, patients should be provided with psychosocial counselling and support in order to help them cope with psychosocial distress as a result of their short stature.

Surgery

• Bone lengthening surgery is being performed not only for the treatment of dwarfism and/or skeletal deformities caused by congenital abnormalities, tumors and infections.

• The surgery is performed using Ilizarov method with circular external fixation. This is called cosmetic leg lengthening or symmetrical extended limb lengthening.

Prevention

• There are no primary preventive measures available for short stature.

• Once diagnosed it could be managed, with either medical or surgical options.