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Jeffrey W. Cozzens and James P. Chandler

✓ The authors describe a relationship between the presence of distal shunt catheter side-wall slits and distal catheter obstruction in a single-surgeon series of ventriculoperitoneal (VP) shunt revisions. Between 1985 and 1996, 168 operations for VP shunt revision were performed by the senior author (J.W.C.) in 71 patients. Indications for shunt revision included obstruction in 140 operations; overdrainage or underdrainage requiring a change of valve in 17 operations; inadequate length of distal shunt tubing resulting in the distal end no longer reaching the peritoneum in five operations; the ventricular catheter in the wrong ventricle or space, requiring repositioning in five operations; and a disconnected or broken shunt in one operation.

Of the 140 instances of shunt obstruction, the blockage occurred at the ventricular end in 108 instances (77.1%), the peritoneal end in 17 (12.1%), the ventricular and the peritoneal end in 14 (10%), and in the valve mechanism (not including distal slit valves) in one (0.8%). Thus, the peritoneal end was obstructed in 31 (22.1%) of 140 cases of shunt malfunction. In every case in which the peritoneal end was obstructed, some form of distal slit was found: either a distal slit valve in an otherwise closed catheter or slits in the side of an open catheter. No instances were found of distal peritoneal catheter obstruction when the peritoneal catheter was a simple open-ended tube with no accompanying side slits (0 of 55).

It is concluded that side slits in the distal peritoneal catheters of VP shunts are associated with a greater incidence of distal shunt obstruction.

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Ania G. Pollack, MaryAnne H. Marymont, John A. Kalapurakal, Alan Kepka, Vythialingam Sathiaseelan and James P. Chandler

✓ The authors describe an acute facial and acoustic neuropathy following gamma knife surgery (GKS) for vestibular schwannoma (VS). This 39-year-old woman presenting with tinnitus underwent GKS for a small right-sided intracanalicular VS, receiving a maximal dose of 26 Gy and a tumor margin dose of 13 Gy to the 50% isodose line. Thirty-six hours following treatment she presented with nausea, vomiting, vertigo, diminished hearing, and a House—Brackmann Grade III facial palsy. She was started on intravenous glucocorticosteroid agents, and over the course of 2 weeks her facial function returned to House—Brackmann Grade I. Unfortunately, her hearing loss persisted. A magnetic resonance (MR) image obtained at the time of initial deterioration demonstrated a significant decrease in tumor enhancement but no change in tumor size or peritumoral edema. Subsequently, the patient experienced severe hemifacial spasms, which persisted for a period of 3 weeks and then progressed to a House—Brackmann Grade V facial palsy. During the next 3 months, the patient was treated with steroids and in time her facial function and hearing returned to baseline levels. Results of MR imaging revealed transient enlargement (3 mm) of the tumor, which subsequently returned to its baseline size. This change corresponded to the tumor volume increase from 270 to 336 mm3. The patient remains radiologically and neurologically stable at 10 months posttreatment.

This is the first detailed report of acute facial and vestibulocochlear neurotoxicity following GKS for VS that improved with time. In addition, MR imaging findings were indicative of early neurotoxic changes. A review of possible risk factors and explanations of causative mechanisms is provided.

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Bradley A. Gross, Stefan A. Mindea, Anthony J. Pick, James P. Chandler and H. Hunt Batjer

✓ In Cushing disease, a pituitary corticotroph neoplasm causes secondary adrenal hypercortisolism. This condition has known morbidity and mortality, underscoring the need for an efficient and accurate diagnostic approach. An 11 p.m. salivary cortisol level is a modern, simple initial screening tool for the diagnosis of Cushing syndrome. Confirmation with a 24-hour urinary free cortisol test and/or a low-dose dexamethasone suppression test may subsequently be performed. Patients with repeatedly equivocal results should be reevaluated after several months or undergo a corticotropin-releasing hormone (CRH) stimulation test following low-dose dexamethasone suppression to help rule out pseudo-Cushing states. The presence of low morning serum adrenocorticotropic hormone (ACTH) levels then distinguishes primary adrenal hypercortisolism from Cushing disease and the ectopic ACTH syndrome. Patients with moderate ACTH levels can undergo CRH stimulation testing to clarify the underlying disease because those with an ACTH-independent disorder have blunted subsequent ACTH levels. Once ACTH-dependent hypercortisolemia is detected, magnetic resonance (MR) imaging of the pituitary gland can be performed to detect a pituitary neoplasm. Normal or equivocal MR imaging results revealing small pituitary lesions should be followed up with inferior petrosal sinus sampling, a highly specific measure for the diagnosis of Cushing disease in experienced hands. If necessary, body imaging may be used in turn to detect sources of ectopic ACTH.

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Sunit Das, James P. Chandler, Ania Pollack, Eileen H. Biggio, Leslie Diaz, Jeffrey J. Raizer and H. Hunt Batjer

✓ The authors describe an oligodendroglioma of the pineal region in a 59-year-old woman. The patient presented with intermittent confusion, memory disturbance, and headache associated with a cystic pineal region mass demonstrated on magnetic resonance imaging. Gross-total resection was performed via a suboccipital supratentorial approach. Pathological and genetic evaluation showed the tumor to be an anaplastic oligodendroglioma. Although the spectrum of tumors arising within the region of the pineal gland is broad, to the authors’ knowledge this is the first report of an oligodendroglioma occurring in this area.

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Bradley A. Gross, Stefan A. Mindea, Anthony J. Pick, James P. Chandler and H. Hunt Batjer

✓Although transsphenoidal excision of the adrenocorticotropic hormone (ACTH)–producing neoplasm is often the treatment of choice in patients with Cushing disease, medical management is itself a useful preoperative temporizing measure, an option for long-term management in nonsurgical candidates, and an option for patients in whom surgery and/or radiotherapy have failed. Three pathophysiologically based approaches exist in the research literature—neuro-modulation to limit ACTH levels, adrenal enzyme inhibition, and glucocorticoid receptor antagonism. Unfortunately, the neuromodulatory approach involving agents such as bromocriptine, cyproheptadine, octreotide, and valproate has yielded only suboptimal results. Glucocorticoid receptor antagonism remains in its infancy but may overall be limited by side effects and a resultant increase in ACTH and cortisol levels. Adrenal enzyme inhibitors, however, offer substantial future promise in the management of Cushing disease but are limited by the potential need to use them indefinitely and by dose-tolerance effects.

Although etomidate is a potential intravenous alternative for acute cortisol level control, ketoconazole has shown efficacy in the long-term treatment of patients with the disease. Metyrapone and/or aminoglutethimide can be added to ketoconazole if additional control is needed. If success is still not achieved, the potent adrenolytic agent often used for adrenocortical carcinomas, mitotane, is another alternative.

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Timothy R. Smith, Rohan R. Lall, Rishi R. Lall, Isaac Josh Abecassis, Omar M. Arnaout, MaryAnne H. Marymont, Kristin R. Swanson and James P. Chandler

Object

Patients with systemic cancer and a single brain metastasis who undergo treatment with resection plus radiotherapy live longer and have a better quality of life than those treated with radiotherapy alone. Historically, whole-brain radiotherapy (WBRT) has been the mainstay of radiation therapy; however, it is associated with significant delayed neurocognitive sequelae. In this study, the authors looked at survival in patients with single and multiple intracranial metastases who had undergone surgery and adjuvant stereotactic radiosurgery (SRS) to the tumor bed and synchronous lesions.

Methods

The authors retrospectively reviewed the records from an 8-year period at a single institution for consecutive patients with brain metastases treated via complete resection of dominant lesions and adjuvant radiosurgery. The cohort was analyzed for time to local progression, synchronous lesion progression, new intracranial lesion development, systemic progression, and overall survival. The Kaplan-Meier method (stratified by age, sex, tumor histology, and number of intracranial lesions prior to surgery) was used to calculate both progression-free and overall survival. A Cox proportional-hazards regression model was also fitted with the number of intracranial lesions as the predictor and survival as the outcome controlling for disease severity, age, sex, and primary histology.

Results

The median overall follow-up among the 150-person cohort eligible for analysis was 17 months. Patients had an average age of 46.2 years (range 16–82 years), and 62.7% were female. The mean (± standard deviation) number of intracranial lesions per patient was 2.5 ± 2.3. The mean time between surgery and stereotactic radiosurgery (SRS) was 3.2 ± 4.1 weeks. Primary cancers included lung cancer (43.3%), breast cancer (21.3%), melanoma (10.0%), renal cell carcinoma (6.7%), and colon cancer (6.7%). The average number of isocenters per treated lesion was 7.6 ± 6.6, and the average treatment dose was 17.8 ± 2.8 Gy. One-year survival for patients in this cohort was 52%, and the 1-year local control rate was 77%. The median (±standard error) overall survival was 13.2 ± 1.9 months. There was no difference in survival between patients with a single lesion and those with multiple lesions (p = 0.319) after controlling for age, sex, and histology of primary tumor. Patients with primary breast histology had the greatest overall median survival (22.9 ± 6.2 months); patients with colorectal cancer had the shortest overall median survival (5.3 ± 1.8 months). The most common cause of death in this series was systemic progression (79%).

Conclusions

These results confirm that 1-year survival for patients with multiple intracranial metastases treated with resection followed by SRS to both the tumor bed and synchronous lesions is similar to established outcomes for patients with a single intracranial metastasis.

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Andrew S. Little, Daniel F. Kelly, William L. White, Paul A. Gardner, Juan C. Fernandez-Miranda, Michael R. Chicoine, Garni Barkhoudarian, James P. Chandler, Daniel M. Prevedello, Brandon D. Liebelt, John Sfondouris, Marc R. Mayberg and for the TRANSSPHER Study Group

OBJECTIVE

Many surgeons have adopted fully endoscopic over microscopic transsphenoidal surgery for nonfunctioning pituitary tumors, although no high-quality evidence demonstrates superior patient outcomes with endoscopic surgery. The goal of this analysis was to compare these techniques in a prospective multicenter controlled study.

METHODS

Extent of tumor resection was compared after endoscopic or microscopic transsphenoidal surgery in adults with nonfunctioning adenomas. The primary end point was gross-total tumor resection determined by postoperative MRI. Secondary end points included volumetric extent of tumor resection, pituitary hormone outcomes, and standard quality measures.

RESULTS

Seven pituitary centers and 15 surgeons participated in the study. Of the 530 patients screened, 260 were enrolled (82 who underwent microscopic procedures, 177 who underwent endoscopic procedures, and 1 who cancelled surgery) between February 2015 and June 2017. Surgeons who used the microscopic technique were more experienced than the surgeons who used the endoscopic technique in terms of years in practice and number of transsphenoidal surgeries performed (p < 0.001). Gross-total resection was achieved in 80.0% (60/75) of microscopic surgery patients and 83.7% (139/166) of endoscopic surgery patients (p = 0.47, OR 0.8, 95% CI 0.4–1.6). Volumetric extent of resection, length of stay, surgery-related deaths, and unplanned readmission rates were similar between groups (p > 0.2). New hormone deficiency was present at 6 months in 28.4% (19/67) of the microscopic surgery patients and 9.7% (14/145) of the endoscopic surgery patients (p < 0.001, OR 3.7, 95% CI 1.7–7.7). Microscopic surgery cases were significantly shorter in duration than endoscopic surgery cases (p < 0.001).

CONCLUSIONS

Experienced surgeons who performed microscopic surgery and less experienced surgeons who performed endoscopic surgery achieved similar extents of tumor resection and quality outcomes in patients with nonfunctioning pituitary adenomas. The endoscopic technique may be associated with lower rates of postoperative pituitary gland dysfunction. This study generally supports the transition to endoscopic pituitary surgery when the procedure is performed by proficient surgeons, although both techniques yield overall acceptable surgical outcomes.

CLASSIFICATION OF EVIDENCE Type of question: therapeutic; study design: prospective cohort trial; evidence: class III.

Clinical trial registration no.: NCT02357498 (clinicaltrials.gov)