Sharma Neeraj, Bhattacharjee Suchanda, Anne Beatrice
Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
Department of Endocrinology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
Asian J Neurosurg. 2020 Dec 21;15(4):1020-1023. doi: 10.4103/ajns.AJNS_100_20. eCollection 2020 Oct-Dec.
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 + , 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.
垂体腺瘤手术在神经外科手术中很常见。大多数手术通过经蝶窦途径，借助内镜或显微镜进行。脑脊液漏、脑膜炎、尿崩症、血肿和视力丧失等并发症是已知的，但中脑梗死很少被报道。我们报告并讨论经蝶窦手术中这种罕见且不寻常并发症的可能机制。一名36岁未生育、无合并症的原发性不孕女性，间歇性头痛9个月，双颞侧视力障碍3个月，颈部和项部色素沉着，月经过少，确诊为多激素分泌型垂体瘤（主要为促肾上腺皮质激素和催乳素）、甲状旁腺功能亢进和糖尿病。所有症状符合多发性内分泌肿瘤1型（MEN - 1）综合征的诊断。使用聚合酶链反应对MEN - 1基因进行全基因测序分析，进行了分子遗传学检测。此外，进行了桑格DNA测序，检测到两个新的变异，即 + 。影像学检查在垂体右叶发现微腺瘤，动态对比增强磁共振成像显示垂体柄轻度偏移。通过经蝶窦入路对肿瘤进行了全切除。除了一些血性脑脊液漏外，手术过程顺利。切除后，填充了脂肪和组织胶。患者术后未从麻醉中苏醒，双侧瞳孔散大，无眼头反射。检查发现双侧丘脑和中脑梗死。患者随后死亡。鉴于其不寻常的并发症，报告此病例是为了提高对垂体微腺瘤经蝶窦手术后这种致命并发症的认识，以及强调经蝶窦手术谨慎操作的重要性，并报告MEN - I基因的新基因突变。
