TALL STATURE

Tall Stature Definition: Tall stature is defined as height of the child is greater than 2 SD above the population mean for age & sex, using growth charts based on local reference standards. If there is significant discrepancy between the mid – parental centile & child’s height, detailed assessment is indicated. History · A detailed history of early growth including birth weight & length should be obtained. · Birth weight & length are usually above 2 SD in children with primary growth disorders like Sotos, Beck with – Widemann & Marfan Syndromes. They continue to show accelerated growth velocity in infancy & early childhood. · Children with constitutional tall stature are within normal range for birth weight & length & demonstrate accelerated growth velocity in early childhood. · Neonatal hypoglycemia can be a feature of Beck with - Wiedemann syndrome. · An underlying endocrine disorder is suspected when growth initially follows a low centile & then crosses up growth chart centiles. · History of eating habits, weight gain & pubertal changes should be taken in all cases. · Simple obesity can lead to tall stature & advanced skeletal age. · Symptoms like palpitation, muscle weakness, diarrhea, weight loss, heat intolerance suggest hyperthyroidism. · History of ophthalmic problems like lens subluxation, glaucoma, retinal detachment suggest Marfan syndrome or Homocystinuria. · Pituitary tumor, secreting growth hormone may present with impairment of vision. · Developmental delay & learning difficulties are associated with Klinefelter syndrome, Homocystinuria, Sotos syndrome etc. Physical Examination · Serial measurement of height, weight, head circumference, upper segment / lower segment ratio, span & BMI (Body Mass Index) & plotting them on standard growth chart is an important step for the evaluation. · Measuring the height of each biological parent, sibling & other family members is necessary. It is essential to calculate mid - parental height centile range for final adult height. · A significant discrepancy between the height of the child & mid – parental centile indicates a growth disorder than constitutional tall stature. · Measure sitting height & arm span is essential. · Marfan & Klinefelter syndromes are associated with disproportionate tall stature. · Arm span is more than height in Marfan & Sotos syndromes. · Head circumference is more than 2 SD in most patients with Sotos syndrome. · Precocious puberty is associated with an early pubertal growth spurt, but results in a shorter than expected final height due to the pre mature closure of the epiphyses. · Tachycardia, tremors, diarrhea, hair loss & exophthalmos are signs of hyperthyroidism. · Growth hormone secreting pituitary adenoma may present with bitemporal hemianopia. · Sotos syndrome is characterized by dysmorphic features like prominent forehead, hypertelorism, large ears & high arched palate. · Look for other dysmorphic & syndromic features. · Macroglossia & earlobe creases are features of Beckwith – Wiedemann syndrome. image.png Constitutional (Familial) Tall Stature · Birth weight & length normal · Parents are tall. · Predicted adult height with mid – parental target range & normal height velocity. · Tall stature becomes apparent from 3 to 4 years of age · Clinical examination normal. · Onset of puberty within normal range. Obesity · Exogenous obesity is becoming a global epidemic. · May be associated with tall stature & advanced skeletal age. · May mimic Cushing syndrome. · Associated with elevated levels of insulin. Klinefelter Syndrome · 47, XXY (other variants 47, XXX, 48 XXXY, 48 XX YY, 49 XX XX Y). · Tall stature with eunuchoid body proportions (increased arm span to height ratio). · Hypogonadism (small testes). · Pubertal failure or arrest. · Gynecomastia. · Behavioral problems & learning difficulties. Marfan Syndrome · Autosomal Dominant · Skeletal features × Long arms (Arm span > 5 cm than height). × US/LS ratio reduced. × Arachnodactyly. × Joint laxity. × Wrist & thumb sign. × Scoliosis & chest deformities. × High arched palate. × Flat feet. · Cardiovascular anomalies × Mitral & Aortic valve incompetence. × Pulmonary stenosis. × Aortic root dilatation & dissection. · Eyes abnormalities × Myopia. × Dislocation of lens. × Hypoplastic iris. · Hernias. Hamocystinuria · Autosomal recessive IEM. · Disproportionate tall stature. · Dislocation of eye lens. · Severe myopenia. · Thromboembolic & cardiovascular complications. Sotos Syndrome (Cerebral Gigantism) · Autosomal Dominant. · Excessive prenatal & postnatal growth. · Increased birth weight, · Large head (> head ʘ). · High arched palate. · Down slanting palpebral fissures. · Flushed cheeks. · Large chin. · Large hands & feet. · Scoliosis. · They are tall during childhood but attain normal adult height due to an advanced skeletal age. Beckwith – Wiedemann Syndrome · Autosomal dominant. · Neonatal hypoglycemia (Hyperinsulinism due to pancreatic hyperplasia). · Macrosomia. · Macroglossia. · Hemi hypertrophy. · Transverse ear crease. · Organomegaly. · Omphalocele. · Wilm’s tumor, Hepatoblastoma & Germ cell tumors are common. Growth Hormone Excess (Acromegaly) · Excessive GH production prior to fusion of epiphyses. · GH producing pituitary adenoma or GHRH excess from hypothalamic tumors. · McCune Albright syndrome can present as excessive GH secretion & tall stature. · Multiple endocrinal neoplasia is another cause. · Prominent soft tissues, prognathism, frontal bossing, large hands & feet etc are other features. · May be signs & symptoms of compression of optic chiasma due to pituitary tumor.