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James K. Liu, Jimmy Patel, Ira M. Goldstein and Jean Anderson Eloy

The transoral approach is considered the gold-standard surgical route for performing anterior odontoidectomy and ventral decompression of the craniovertebral junction for pathological conditions that result in symptomatic cervicomedullary compression, including basilar invagination, rheumatoid pannus, platybasia with retroflexed odontoid processes, and neoplasms. Extended modifications to increase the operative corridor and exposure include the transmaxillary, extended “open-door” maxillotomy, transpalatal, and transmandibular approaches. With the advent of extended endoscopic endonasal skull base techniques, there has been increased interest in the last decade in the endoscopic endonasal transclival transodontoid approach to the craniovertebral junction. The endonasal route represents an attractive minimally invasive surgical alternative, especially in cases of irreducible basilar invagination in which the pathology is situated well above the palatine line. Angled endoscopes and instrumentation can also be used for lower-lying pathology. By avoiding the oral cavity and subsequently using a transoral retractor, the endonasal route has the advantages of avoiding complications related to tongue swelling, tracheal swelling, prolonged intubation, velopharyngeal insufficiency, dysphagia, and dysphonia. Postoperative recovery is quicker, and hospital stays are shorter. In this report, the authors describe and illustrate their method of purely endoscopic endonasal transclival odonotoidectomy for anterior decompression of the craniovertebral junction and describe various operative pearls and nuances of the technique for avoiding complications.

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Jimmy Patel, Jean Anderson Eloy and James K. Liu

Nelson's syndrome is a rare clinical manifestation that occurs in 8%–47% of patients as a complication of bilateral adrenalectomy, a procedure that is used to control hypercortisolism in patients with Cushing's disease. First described in 1958 by Dr. Don Nelson, the disease has since become associated with a clinical triad of hyperpigmentation, excessive adrenocorticotropin secretion, and a corticotroph adenoma. Even so, for the past several years the diagnostic criteria and management of Nelson's syndrome have been inadequately studied. The primary treatment for Nelson's syndrome is transsphenoidal surgery. Other stand-alone therapies, which in many cases have been used as adjuvant treatments with surgery, include radiotherapy, radiosurgery, and pharmacotherapy. Prophylactic radiotherapy at the time of bilateral adrenalectomy can prevent Nelson's syndrome (protective effect). The most promising pharmacological agents are temozolomide, octreotide, and pasireotide, but these agents are often administered after transsphenoidal surgery. In murine models, rosiglitazone has shown some efficacy, but these results have not yet been found in human studies. In this article, the authors review the clinical manifestations, pathophysiology, diagnostic criteria, and efficacy of multimodal treatment strategies for Nelson's syndrome.