A Rare Complication of Pituitary Adenoma Surgery in a Patient with Multiple Endocrine Neoplasia 1 Syndrome with Two Novel Genetic Mutations

Pituitary adenoma surgeries are common in neurosurgical setup. Majority are tackled by a transsphenoidal route either by endoscopic or microscopic aid. Complications such as cerebrospinal fluid (CSF) leak, meningitis, diabetes insipidus, hematoma, and loss of vision are known, but midbrain infarct is rarely reported. We report and discuss the possible mechanism of this rare and unusual complication in transsphenoidal surgery. A 36-year-old nulliparous female with no comorbidities suffering from primary infertility presented with intermittent headache for 9 months with bitemporal vision disturbances for 3 months, pigmentation around the neck and nape, oligomenorrhea with an established diagnosis of plurihormonal secreting pituitary tumor (predominantly adrenocorticotropic hormone and prolactin), hyperparathyroidism, and diabetes. The whole symptom fitted into the diagnosis of multiple endocrine neoplasia 1 (MEN-1) syndrome. Molecular genetic testing was done with full gene sequencing analysis of MEN-1 gene using polymerase chain reaction. Furthermore, Sanger DNA sequencing was done, and two novel variations, namely IVS 9c.1364 + 99C>G and EXON 10 c.1813 C>T (p. L605 L), were detected. Radiology detected a microadenoma in the right lobe of the pituitary with mild deviation of the pituitary stalk on dynamic contrast-enhancing magnetic resonance imaging. Gross total excision of the tumor was done through transsphenoidal approach. The surgery was uneventful other than some blood-mixed CSF leak. Post excision, fat and tissue glue was packed. The patient did not wake up from anesthesia and had bilateral dilated pupil with no oculocephalic reflex. Investigations revealed bilateral thalamic and midbrain infarct. The patient subsequently expired. This case is reported in view of its unusual complication and to create awareness for such a fatal complication following transsphenoidal surgery for pituitary microadenoma and the importance of diligent approach to transsphenoidal surgery and to report novel genetic mutation of MEN-I gene.