Background: A child with slow growth rate (<−2 SDS) polyuria and polydipsia warrants urgent investigation for hypothalamicpituitary tumors.
Objective and hypoyhesis: To present the case of a boy with slow growth rate, polyuria NAD polydipsia due to remnant craniopharyngeal duct.
Method: Boy 11 years old, was evaluated because of short stature and slow growth rate documented for 2 years. He reported no headaches but he reported polyuria, polydipsia, fatigue and earache. Targuet height was on the 75th percentile. He had no dysmorphic features, height was on the 3rd percentile and weight on the 50th percentile. His physical exam was unremarcable, with normal proportions, unpalpable thyroid, and prepubertal external genitalia. MRI of the hypothalamuspituitary area as well as hormonal investigations were scheduled. IGF1: 115 ng/ml, TSH: 4.28 μIU/ml and fT4:0.72 ng/dl. GH response to stimulation was low, as well as cortisol response to glucagon. Morning cortisol was within normal range. Bone age was 2.5 years delayed. MRI revealed a bony defect of the sphenoid bone, 2.8 mm large and 4 mm long, at the level of sella turcica. There was apparent prolapse of the anterior pituitary lobe through the duct, reaching to the upper part of the rhinopharynx. The intense signal of the posterior pituitary lobe was not recognized. The patient is under replacement therapy with GH and thyroxine.
Conclusion: Remnant craniopharyngeal duct is a rare congenital disorder. To our knowledge there are no previous report of a child with a large craniopharyngeal duct and hormonal deficiencies.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology