ESPE2018 Poster Presentations Pituitary, Neuroendocrinology and Puberty P3 (38 abstracts)
aDepartment of Pediatric and Adolescent Endocrinology, Chair of Pediatrics, Medical College, Jagiellonian University, Cracow, Poland; bDepartment of Radiology, University Children Hospital, Cracow, Poland
Common cause of hypopituitarism is congenital, genetically determined abnormality called pituitary stalk interruption syndrome (PSIS). It is characterised by hypoplastic anterior pituitary gland with thin or absent infundibulum and ectopic posterior pituitary location in MRI examination. Post-traumatic hypopituitarism may mimic this image. We present 7 years and 10 months old girl who was admitted to Pediatrics Institute due to hypoglycemia during an acute gastroenteritis. Hypoglycemia was not responsive to the infusion of glucose. She had no medical history of any serious diseases or health problems until the age of 3 years and 10 months when she was admitted to Intensive Care Unit because of severe head trauma after being hit by a swing. Computer tomography of the head revealed basilar skull fracture, cerebral edema, subarachnoid hemorrhage. For few days she has been kept in medically induced coma and treated with desmopressin because of transient diabetes insipidus. After the accident the patient did not undergo any routine medical check-ups. On admission to our hospital physical examination revealed skin pallor, orthstatic hypotension and short stature with body weight appropriate for the height and no signs of pubertal maturation. Based on laboratory studies and auxological data secondary hypothyroidism (ft4:6.6 pmol/l N:1025, TSH:2.89 uIU/ml N:0.34.0, ft3:1.8 pmol/l N: 3.08.1), growth hormone deficiency (0.13 ng/ml during hypoglycemia and max. 0.49 ng/ml in twa stimulation tests, IGF1:27.1 ng/ml N:59297, growth on the 90th percentile on the growth chart respectively to mid-parental high with growth retardation since the age of 4 years, bone age delayed 5 years), secondary adrenal insufficiency(morning cortisol 44.8 ng/ml N:50230, ACTH 5.3 pg/ml N:1060, max. cortisol in glucagon stimulation test 55.5 ng/ml, low serum sodium level 135 mmol/l N:135145) were found. Magnetic resonanse imagining of the pituitary gland showed disruption of the pituitary stalk with hiperintense signal of distal axon of the hypothalamus, hypoplastic anterior and ectopic posterior pituitary gland. The introduction of multiple hormonal replacement therapy (hydrocortisone, L-tyroxine, human recombinant GH) caused resolution of hypotonia, hypoglycemia and normalization of the general condition and growth of the child (GV=16 cm/year). Head trauma followed by biochemical or somatic symptoms of pituitary insufficiency together with hyperintense signal of distal axon of the hypothalamus suggest traumatic stalk transection with secondary hypoplasia of anterior pituitary gland.In every case of severe traumatic head injury hormonal evaluation and MRI of hypothalamicpituitary axis should be performed.