| Identifier |
walsh_2020_s3_c4-pdf |
| Title |
Bones of the Past (PDF) |
| Creator |
Bart Chwalisz; Konstantinos Douglas; Vivian Douglas; Otto Rapalino |
| Affiliation |
(BC) (OR) Massachusetts General Hospital / Harvard Medical School, Boston, Massachusetts; (KD) (VD) Massachusetts Eye & Ear / Harvard Medical School, Boston, Massachusetts |
| Subject |
6th Nerve Palsy, Skull Base, Pituitary Surgery (Transphenoidal Adenomectomy), PET |
| History |
A 44-year-old woman presented with two episodes of binocular horizontal diplopia within one year. She had a history of Cushing's syndrome status post transsphenoidal resection and bilateral adrenalectomy 4 years prior, hypertension, and secondary diabetes mellitus. Nine months prior she developed severe right-sided headache and a right abducens nerve palsy, which improved over several weeks. At presentation, she described left-sided headache and neck pain, followed by development of horizontal diplopia worst on left gaze. She had normal visual acuity, color vision, visual fields, pupillary function, and fundi. There was an isolated left abduction deficit with incomitant esotropia. MRI of the brain with contrast showed stable residual postoperative findings in the sella, with unchanged residual contrast-enhancing pituitary tissue, and some contact of the left AICA with the left abducens nerve but no enhancement or other abnormality of the abducens nerves or extraocular muscles. A second radiologic opinion noted previously missed confluent but heterogeneous T1-hypointense marrow in the clivus with diffusion restriction, which in retrospect was progressively worsening over serial scans from preceding years. Lumbar puncture yielded normal cerebrospinal fluid. A CT PET of the skull base showed abnormal enhancing soft tissue within the sella and extending along the epidural surface of the clivus, and progression of bony sclerosis within the clivus compared to prior scans; there was hypermetabolism of the clivus. Follow-up MRI demonstrated progression of the changes in the sella and clivus compared to her postoperative imaging. Serum adrenocorticotropic; hormone (ACTH) levels were significantly higher than prior. A transsphenoidal biopsy revealed tumor with surrounding fibrotic reaction in the sella, sphenoid sinus and superior clivus. Pathology was consistent with corticotroph adenoma with elevated; proliferation index and scattered mitoses. A diagnosis of Nelson's syndrome was made. The patient underwent radiation therapy, with full resolution of her diplopia and headache. |
| Disease/Diagnosis |
Sequential abducens palsies secondary to Nelson's syndrome (regrowth of corticotroph pituitary adenoma into the clivus). |
| Date |
2020-03 |
| References |
1. Patel J, Eloy JA, Liu JK. Nelson's syndrome: A review of the clinical manifestations, pathophysiology, and treatment strategies. Neurosurg Focus. 2015;38(20:E14. doi:10.3171/2014.10.FOCUS14681. 2. Garg M, Brar K, Bharwaj R, et al. Nelson′s syndrome presenting as bilateral oculomotor palsy. Indian J Endocrinol Metab. 2013;17(6):114-6.doi:10.4103/2230-8210.122643. 3. Gazioglu N, Kadioglu P, Ocal E, et al. An unusual presentation of Nelson's syndrome with apoplexy and subarachnoid hemorrhage. Pituitary. 2002;5(4):267-74. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
2020 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2020 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2020. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6jf07kg |
| Setname |
ehsl_novel_fbw |
| ID |
1546639 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6jf07kg |
