Background: Nonsurgical development of nasal cerebrospinal fluid (CSF) leaks may occur in the setting of pituitary adenomas, especially following a favorable response of invasive prolactinomas to initiation of Dopamine Agonist (DA) therapy, but this has not previously described in children.
Case description: A girl of Srilankan origin, aged 13.8 years, whose parents spoke no English, presented with headaches and secondary amenorrhea. Pituitary MRI revealed a large pituitary macroadenoma, invading the sphenoid sinus and elevating the optic chiasm. Thyroid and adrenal reserve was normal while she had growth hormone deficiency. There was no family history of pituitary-related disease and MEN1 and AIP genetic testing was normal. She was commenced on cabergoline at 0.5mg twice a week with prolactin drop to less than 3% of original value and significant tumour size drop. However, she developed a CSF leak, which was managed surgically. Tumour biopsy confirmed a prolactinoma and was low Ki-67 staining. Postoperatively her prolactin levels normalized.
Conclusion: Childhood prolactinomas are often large invasive macroadenomas, and significant tumour response to cabergoline can lead to a CSF leak, if the tumour invaded the sphenoid sinus. This case emphasizes the importance to monitor these patients closely and warn them of sign and symptoms of CSF leak.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology