Giant prolactinomain childhood as a rare cause of blindness: Case report and review of literature

Prolactinomas in childhood and adolescence are rare, and may be confused with other skull base tumors.We report a case of a girl presenting with progressive visual loss due to giant prolactinoma, and her interdisciplinary treatment. We give an overview of the current literature.
The patient was a 14 year old girl who presented with headache and left sided visual deterioration in her mother-country. Plane CCT revealed a large suprasellar with hyper- and hypodens areas, sellar enlargement and likely bilateral cavernous sinus infiltration. Prolactin level was stated not to be elevated. After 3 months the girl could be admitted for neurosurgical treatment. At that time she was blind for weeks, showed panpituitarism and extensive hyperprolactinemia. MRI confirmed the giant tumor with cystic areas and gadolinium uptake in the solid parts.
Treatment consisted of dopamin-agonist therapy which was started urgently with bromocriptin. The prolactin level responded rapidly. However, blindness persisted. Transspenoidal microsurgical, MR-navigated and ultrasound-assisted tumor debulking was performed. Thereafter, light perception was regained for the right eye, whereas blindness persisted for the left. Although the prolactin level progressively declined within 2 weeks, no further improvement to functional visual perception occurred. Right sided transcranial optic nerve decompression resulted in stable visual improvement of the right eye which allowed good spatial orientation, reading, and independence. A psychotic period developed under bromocriptin, which was ceased and replaced by cabergolin. Prolactin decreased to normal levels. After one month the girl was readmitted with meningitis due to CSF-leak. This was treated with another transnasal procedure and lumbar drainage. Back in her mother-country, the girl is stable under cabergolin after one year, with substitution of thyroid hormone, hydrocortisone, and estrogen.

Since giant prolactinoma in childhood is very rare, its diagnosis may be missed due to hook effect. Although dopamine agonist treatment effectively acts on hyperprolactinemia, aggressive surgical treatment has to be considered in cases of visual loss.

No relevant conflicts of interest declared.