RT Journal Article
SR Electronic
T1 Pituitary gigantism: a rare learning opportunity
JF Archives of disease in childhood - Education & practice edition
JO Arch Dis Child Educ Pract Ed
FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
SP 111
OP 116
DO 10.1136/archdischild-2018-316282
VO 105
IS 2
A1 Owen Martyn Bendor-Samuel
A1 Aparna Pal
A1 Simon Cudlip
A1 Gemma Anderson
A1 Sanjay Salgia
A1 Tafadzwa Makaya
YR 2020
UL http://ep.bmj.com/content/105/2/111.abstract
AB Introduction Pituitary gigantism is a rare but significant paediatric condition with complexities surrounding diagnosis and management. Transsphenoidal surgery (TSS) is the treatment of choice; however, medical treatment is often considered as adjuvant therapy.Case A 10½ -year-old boy presented with tall stature and a height velocity of 11 cm/year. His height was 178.7 cm (+5.8 SD above mean) and insulin-like growth factor-1 (IGF-1) was elevated. An oral glucose tolerance test demonstrated non-suppression of growth hormone (GH). Initial contrast MRI was inconclusive, but C-11 methionine functional positron emission tomography CT identified a 6 mm pituitary microadenoma. A multidisciplinary team clinic held with the family allowed discussion about medical and surgical treatment options. Due to a number of factors including the patient’s young age, prepubertal status, a wish to allow him to settle into his new high school and his desire to reach a final height taller than his father’s height, it was decided to try medical therapy first with a somatostatin analogue. Pubertal induction was also commenced and bilateral epiphysiodesis surgery performed. Initial response to octreotide was positive; however, 4 months into therapy his IGF-1 was climbing and a repeat GH profile was not fully suppressed. The patient therefore proceeded to have successful TSS excision of the adenoma.Conclusion Rare cases such as this require sharing of knowledge and expertise, so the best possible care is offered. It is often necessary to work across sites and disciplines. Each case requires an individual approach tailored to the patient and their family.