ESPE Abstracts

Introduction: Pituitary gigantism in children is a rare condition that results from excessive production of growth hormone (GH). The most common cause of gigantism is GH secreting pituitary adenomas, thart arise from excessive proliferation of somatotropic cells in the anterior portion of the pituitary gland. The described pediatric incidence is 3-8 cases per 1 million population. There are genetic syndromes associated with GH excess, especially in pediatric patients.

Objective: We describe a series of 19 pediatric cases (<18 years old) with GH excess who were evaluated and treated in a referral center of our country during the years 1992-2023.

Methods: The clinical records of 19 patients with GH excess were reviewed, including symptoms at diagnosis, hormonal levels, imaging, visual assessment, type of initial treatment and follow up.

Results: Of the 19 patients, 73.7% were female, the average age at diagnosis was 17.4 ± 5.5 (6-26) years and the average diagnostic delay 4.5 (1-13) years, with a follow up of 4.2 ± 4.5 (0,5-12) years. The most frequent symptom at diagnosis was acral growth (89.5%), followed by prognathism (68.4%), and excessive sweating, headache and tall stature (36.8%). Fifty percent of the women presented menstrual alterations. A family history of second degree relatives with pituitary tumors or acromegaly was found in 10.5% of the patients. Two patients presented clinical signs compatible with McCune Albright syndrome. Ten patients presented at least one pituitary dysfunction, being the thyroid axis the most frequently altered (31%). Molecular study was performed in 4%. Fifteen corresponded to a macroadenoma with 36.8% of them presenting invasion of the cavernous sinus and 15.8% involvement of the optic chiasm. At the visual assessment, 36% presented visual field alteration, and 21% of the optic nerve. In 94.7% transsphenoidal surgery was used as first line treatment, with 22.2% having complete remission after this surgery, and 44.4% partial remission. The most frequent complications were arginine-vasopressin deficiency (10%) and cerebrospinal fluid fistula (10%). A second or third line treatment was required in 10 patients, 50% were reoperated, and 36% were given a somatostatin analogue.

Conclusion: Growth hormone excess is a rare condition, with high life-quality impact in pediatric patients, affecting their growth and development in the long term. Our center has a well established pituitary specific multidisciplinary team. The data here presented offers a contribution to national and international epidemiology for the continuous improvement and optimization of the management of these patients.