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Jan27
Solitary median maxillary central incisor, a clinical predictor of hypoplastic anterior pituitary, ectopic neurohypophysis and growth hormone deficiency
Clinical evaluation of a 5-year-old boy seeking medical
advice for micropenis was detected to have solitary
median maxillary central incisor (SMMCI), short stature[height: 95.2 cm; < 3rd percentile; standard deviation score
(SDS): – 2.57; target height SDS: – 0.63], small face, low set
ears, depressed nasal bridge, prominent forehead and
stretched penile length of 2 cm . He was born
at term of breech delivery with an uneventful perinatal
history. Investigations were significant for delayed bone
age (3.5 years; Greulich-Pyle), low insulin like growth
factor-1 (IGF-1) (41 ng/mL; normal, 50 – 286), GH deficiency
(post clonidine 100 μ g peak growth hormone: 1.2 ng/mL;
normal > 10 ng/mL), hypoplastic anterior pituitary (white
arrow; partial empty sella), stalk agenesis and ectopic
neurohypophysis (EN) located in tuber cinereum (black
arrow) on MRI. Optic nerve, olfactory bulbs,
corpus callosum and septum pellucidum were normal. GH
replacement resulted in an 11 cm height gain in 1 year.


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