Atkinson, John L. D.

AbstractBackgroundCushing’s syndrome (CS), due to multiple etiologies, is a disorder associated with the ravages of cortisol excess. The purpose of this review article is to provide a historical synopsis of surgery for CS, review a recent 10‐year period of operative management at a tertiary care facility, and to outline a practical approach to diagnosis and management.Materials and MethodsFrom 1996 to 2005, 298 patients underwent 322 operative procedures for CS at Mayo Clinic, Rochester, Minnesota. A retrospective chart review was carried out. Data was gathered regarding demographics, preoperative assessment, procedures performed, and outcomes. Data are presented as counts and percentages. Five‐year survival rates were calculated where applicable by the Kaplan‐Meier method. Statistical analysis was carried out with SAS, version 9 (SAS Institute, Inc., Cary, NC).ResultsTwo‐hundred thirty‐one patients (78%) had ACTH‐dependent CS and 67 patients (22%) had ACTH‐independent CS. One‐hundred ninety‐six patients (66%) had pituitary‐dependent CS and 35 patients (12%) had ectopic ACTH syndrome. Fifty‐four patients (18%) had cortisol‐secreting adenomas, 10 patients (3%) had cortisol‐producing adrenocortical carcinomas, and 1% had other causes. Cure rates for first time pituitary operations (transsphenoidal, sublabial, and endonasal) were 80% and 55% for reoperations. Most benign adrenal processes could be managed laparoscopically. Five‐year survival rates (all causes) were 90%, 51%, and 23% for adrenocortical adenomas, ectopic ACTH syndrome, and adrenocortical carcinomas, respectively.ConclusionsSurgery for CS is highly successful for pituitary‐dependent CS and most ACTH‐independent adrenal causes. Bilateral total adrenalectomy can also provide effective palliation from the ravages of hypercortisolism in patients with ectopic ACTH syndrome and for those who have failed transsphenoidal surgery. Unfortunately, to date, adrenocortical carcinomas are rarely cured. Future successes with this disease will likely depend on a better understanding of tumor biology, more effective adjuvant therapies and earlier detection. Clearly, IPSS, advances in cross‐sectional imaging, along with developments in transsphenoidal and laparoscopic surgery, have had the greatest impact on today’s management of the complex patient with CS.

The transseptal/transsphenoidal approach to the pituitary gland has been the most commonly used approach for resection of pituitary adenomas for the last 50 years. This procedure has a low morbidity and provides direct midline access to the sella and pituitary gland. Recent advancements in endoscopic surgery, however, suggest that a lower morbidity approach to the sella would be possible via transnasal endoscopic route. Prior reports have confirmed effectiveness of this approach to the pituitary gland and we report here an early series of endoscopic transnasal pituitary surgery from our institution. We report seven cases of transnasal endoscopic pituitary surgery. Our technique consists of endoscopic exposure of the sphenoid ostium unilaterally, excision of the posterior septum anterior to the rostrum of the sphenoid sinus with resection of the sphenoid rostrum for bilateral exposure of the sphenoid sinus. A specially designed nasal speculum is positioned to displace the posterior septum and lateralize the middle turbinates, permitting direct midline exposure of the sphenoid sinus and sella. We have progressively modified the technique over the seven cases that we present and will discuss our specific instrumentation, indications, and technique for this procedure. We have encountered one cerebrospinal fluid leak in this series. Patient satisfaction has been high and hospitalization is less than with the conventional transseptal approach, averaging 1 day. Our impression is that the transnasal endoscopic approach to pituitary adenomas is a safe technique with reduced morbidity permitting shortened hospital stay.

The increasing use of endonasal techniques and a new 3-dimensional CT-guided imaging system have allowed us to develop a combined, minimally invasive endonasal approach to the pituitary gland. Thus far, more than 30 patients have undergone an endonasal transsphenoidal approach to the sella using a combined endoscopic approach, with additional selective use of the Instatrak CT-guided imaging system for realtime imaging. Our current technique involves obtaining a preoperative CT using a plastic head frame with registration markers. By using this head frame intraoperatively, real-time localization with CT images in axial, coronal, and sagittal planes can be performed. Using endoscopic techniques and a Papavero-Caspar speculum, the sella is exposed. A combined approach using endoscopes, the operating microscope, and real-time localization is undertaken to expose and resect tumors. We have encountered minimal associated complications in our series, and this method has been progressively modified to improve exposure and safety. Because we are able to visualize the sella without a sublabial incision or septal resection, we have nearly eliminated the need for nasal packing, reduced the average hospital stay to 2.5 days, and improved patient satisfaction.

Abstract Objective: Spontaneous intracranial hypotension is typically caused by a spontaneous spinal cerebrospinal fluid (CSF) leak. The configuration of the related dural defects can be complex. We describe our experience with the surgical anatomy of these defects. Methods: Thirteen consecutive patients with spontaneous spinal CSF leaks who underwent surgical exploration at Mayo Clinic between 1994 and 2003 were studied. All patients' records, imaging studies, detailed intraoperative findings, and outcomes were reviewed. Results: There were four men and nine women with an average age of 40 years (range, 12–62 yr). Preoperative imaging studies revealed a single site of CSF leak in eight patients, two sites in three patients, and multiple sites in two patients. Intraoperatively, the exact site of leakage could not be found in four patients. Among the other nine patients, primary closure of a meningeal diverticulum was achieved in one patient. Significant regional attenuation of the dura prevented primary repair of the leak site in eight patients. Muscle, fibrin glue, and Gelfoam (Upjohn Co., Kalamazoo, MI) soaked in patient's own blood were commonly used to pack the epidural space in an attempt to seal the site of the leak. Ligation of two nonappendicular nerve roots allowed closure of the leak in one of these patients. Postoperatively, resolution of symptoms occurred in eight patients, significant improvement was noted in three patients, and only transient resolution in two. The mean duration of follow-up was 20.5 months. Conclusion: Surgery for closure of spontaneous spinal CSF leaks may not be straightforward. Even when extradural CSF leakage is discovered preoperatively by imaging studies, it may not always be possible to identify the exact site of the leakage intraoperatively. Furthermore, the anatomy of the dural defects may be complex and not amenable to primary closure. In such cases, the use of adjuvant techniques during surgical exploration may be effective.

Background and Purpose— The aim of this study was to analyze the distribution patterns of delayed cerebral ischemia after subarachnoid hemorrhage (SAH) and the factors that determine their occurrence. Methods— We analyzed clinical and radiological data of 143 consecutive patients with aneurysmal SAH. Computed tomography scan revision was blinded to clinical information. Superficial infarctions were defined as territorial lesions with cortical involvement. Perioperative infarctions were excluded. Results— Fifty-six patients (39%) had cerebral infarctions. They were unilateral in 34 patients (61%) and involved a single territory in 29 (52%). Location was cortical in 34 patients (61%), deep in 10 (18%), and combined cortical and deep in 12 (21%). Single infarctions were cortical in 23 of 28 cases (79%). Deep territory ischemia was more common with multiple lesions (16/28, 57% versus 6/29 with single lesions, 21%; P <0.01). Single infarctions occurred frequently in the territory of the ruptured aneurysm (22/28 patients; 79%), whereas multiple infarctions were often distant to the site of rupture (21/28 cases, 75%). History of diabetes ( P =0.05), early hydrocephalus ( P =0.05), and requirement of external ventricular drainage ( P =0.02) were associated with the occurrence of multiple infarctions on univariate analysis. On multivariable analysis, this association only remained significant for the requirement of external ventricular drainage. Conclusion— The 2 most common patterns of delayed cerebral ischemia after aneurysmal SAH are single cortical infarction, typically near the ruptured aneurysm, and multiple widespread lesions including subcortical locations and often unrelated to the site of aneurysm rupture. These 2 patterns may represent different pathophysiological mechanisms or different degrees of severity of the same vascular process.

Abstract OBJECTIVE AND IMPORTANCE A case of acutely symptomatic epidural hematoma caused by metastatic hepatocellular carcinoma (HCC) to the cranium is reported. This is a rare case of metastatic HCC without known primary presenting as an epidural hematoma. CLINICAL PRESENTATION The patient presented with an acute onset of headache, aphasia, and right hemiparesis 2 weeks after he experienced minor trauma to the cranium. An emergency computed tomographic scan of the head revealed the presence of a left parietal epidural hematoma. INTERVENTION An emergency evacuation of the epidural hematoma was performed, and metastatic HCC was diagnosed. CONCLUSION The patient's neurological deficits were reversed with surgical intervention, and he is now undergoing palliative chemotherapy. This was the first clinical manifestation of HCC in this patient. This case reaffirms the neurosurgeon's role in the complex, multidisciplinary care of patients with craniospinal metastasis.

Abstract OBJECTIVE AND IMPORTANCE We report the first case of combined oligodendroglioma and pleomorphic xanthoastrocytoma. CLINICAL PRESENTATION A female college student and competitive cross-country runner presented to the Mayo Clinic at age 18 years with complaints of progressive headache and visual disturbances. Neuroimaging revealed a heavily calcified left parieto-occipital mass with focal enhancement and remodeling of the overlying calvarium. INTERVENTION A histological examination of the nearly gross total resection specimen disclosed a low-grade oligodendroglioma discretely abutting a superficially situated focus of pleomorphic xanthoastrocytoma. Ten months thereafter, a recurrence was suspected on the basis of the detection of a new focus of nodular enhancement. This lesion stabilized after radiation therapy, and the patient is well, without apparent residual disease 2.8 years after surgery. CONCLUSION This unique neoplasm is presumed to represent a collision tumor, its biological behavior being similar to that of oligodendroglioma alone. However, the possibility of an unusual, mixed oligoastrocytoma with pleomorphic xanthoastrocytoma as the astrocytic component cannot be excluded entirely. Potentially, both components have the capacity for recurrence and anaplastic transformation.