Unilateral hydrocephalus

see Hydrocephalus classification

The septum pellucidum is a bilateral thin membranous structure representing the border between the frontal horns of the lateral ventricles. Its most examined components are the septal veins due to their surgical importance during endoscopic septum pellucidotomy (ESP), which is a well-accepted method for surgical treatment of unilateral hydrocephalus.

Although pituitary macroadenomas often cause mass effects on surrounding structures, it is extremely rare for pituitary lesions to disturb cerebrospinal fluid circulation. Sellar gangliocytoma-pituitary neuroendocrine tumors (SGPAs) (pituitary neuroendocrine tumor with gangliocytoma) are also extremely rare.

Ryder et al., reported the unique case of a man with the unusual combination of acromegaly from an SGPA, who presented with unilateral hydrocephalus. A 60-year-old man presented with rapid neurological deterioration, bitemporal hemianopia, and acromegalic features. Neuroimaging revealed a large sellar lesion extending superiorly into the left foramen of Monro, causing acute obstructive unilateral hydrocephalus. External ventricular drain placement improved consciousness immediately. The biochemical assessment confirmed acromegaly. Following trans-sphenoidal debulking, histology revealed a mixed gangliocytoma/sparsely-granulated somatotrophinoma. Despite the residual disease, his vision recovered remarkably, low-dose cabergoline controlled residual excess growth hormone (GH) secretion, and the residual tumour has remained extremely stable over 2 years. Hydrocephalus is an extremely rare complication of pituitary lesions, and unilateral hydrocephalus has never been reported previously. GH secretion in SGPAs is more common than for pituitary neuroendocrine tumors in general, raising questions regarding the aetiology and therapeutic approach to this rare combination tumour 1).

Ryder S, Robusto J, Robertson T, Alexander H, Duncan EL. Unilateral hydrocephalus from a gangliocytoma-somatotrophinoma: first reported case. Endocrinol Diabetes Metab Case Rep. 2021 Jul 1;2021:EDM210037. doi: 10.1530/EDM-21-0037. Epub ahead of print. PMID: 34236040.
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