Till Burkhardt

Surgical Strategies in Childhood Craniopharyngioma

Craniopharyngiomas are biologically benign lesions (WHO Grade 1) of the sellar and suprasellar region, associated with a serious morbidity. About 50% of these tumors become clinically apparent during childhood. Clinical symptoms include headaches, chiasm syndrome, hydrocephalus, pituitary insufficiencies, and obesity. Growth arrest is a typical symptom in children. The treatment of craniopharyngiomas includes surgery as well as radiotherapy. The goal of surgery varies according to the tumor location and extension and may range from complete resection to biopsy. Surgical complications are well known and cause constant evaluation of surgical strategies. Diencephalic obesity is related to surgical manipulation of hypothalamic tissue. Therefore, a classification system for craniopharyngiomas based on preoperative MRI is suggested by the authors. Recurrences are frequent in craniopharyngiomas, even after complete or gross-total resection. Radiotherapy is therefore recommended to patients with incomplete resections. However, the ideal time for radiotherapy after surgery is under discussion. The treatment of craniopharyngiomas requires an interdisciplinary and multimodal approach. Each patient should receive an individually tailored treatment. Surgically, different approaches as well as different degrees of resection can be considered, depending on tumor location and tumor extension.

Dynamics of neurological deficit after surgical decompression of symptomatic vertebral metastases.

Study Design. We conducted a retrospective study to examination the influence of preoperative duration of symptoms on the clinical outcome of patients that underwent surgical decompression because of neurologic deficit in metastatic disease of the spine. Objectives. Our aim was to investigate possible correlations between the duration of neurologic deficit before surgery and postoperative outcome with respect to neural recovery in patients with spinal metastases, and second, based on those results, propose criteria for the timing of surgery in these patients. Summary of Background Data. It has not yet been determined whether the duration of preoperative symptoms has an influence on the postoperative outcome of patients with vertebral metastases. A standardized treatment or protocol defining a strategy of surgical treatment has yet to be designed. Methods. This study includes 194 patients. The duration of symptoms before surgical treatment and the neurologic status before and after operation were determined and classified according to the Frankel score. Results. Of 401 patients, who underwent surgery due to metastases to the spine, 194 suffered from neurologic deficit. Analyzing the postoperative neurostatus in these patients revealed an improvement in 78 patients (40%), impairment in 13 patients (7%), and in 103 patients it did not change. The relation of duration of neurologic symptoms before surgery, and the outcome after an operation was highly significant (P < 0.001). In patients with less than 3 days of neurologic deficit, the probability of improvement in neurostatus was highly significantly higher (P < 0.001) than in patients with neurologic deficit existing for more than 15 days. Conclusion. Patients with neurologic deficit because of spinal bone metastases benefit from early operative intervention. Urgent surgery is indicated in patients with less than 3 days of neurologic deficit.

Surgery for pediatric craniopharyngiomas: is less more?

Abstract Surgery for craniopharyngiomas, especially in childhood and adolescence, has evolved from an era of aggressive strategies – with the primary goal of gross total removal and accepting an impaired functional outcome – to a more individually tailored therapy that avoids immediate treatment-related and long-term morbidity. Modern imaging techniques and a wider understanding of hypothalamic risk factors have led to surgical strategies adapted to the localization of these inhomogenously grown pathologies. Whereas purely infradiaphragmatic as well as supradiaphragmatic/infrachiasmatic tumors have a favorably surgical outcome with higher gross total resection rates in experienced hands, lesions within the third ventricle extending beyond the mammillary bodies remain a problem. The same is valid for lesions beyond 3 cm in diameter, more or less independent of their localization. Aside from the traditional microscopic approach via the subfrontal or pterional craniotomy, transsphenoidal approaches and other minimal invasive surgical methods, e.g., catheter implantation into cystic formations of the tumor have become popular. Radiotherapy, with its risks and limitations, can effectively be added to avoid recurrences. Nowadays, surgery as part of an interdisciplinary treatment strategy is still the typical first choice. However, taking the patient’s long-term prognosis into considertaion, the surgical complication rates have to be minimized.

Hippocampal and cerebellar atrophy in patients with Cushing’s disease

OBJECT Cushings disease (CD) may cause atrophy of different regions of the human brain, mostly affecting the hippocampus and the cerebellum. This study evaluates the use of 3-T MRI of newly diagnosed patients with CD to detect atrophic degeneration with voxel-based volumetry. METHODS Subjects with newly diagnosed, untreated CD were included and underwent 3-T MRI. Images were analyzed using a voxelwise statistical test to detect reduction of brain parenchyma. In addition, an atlas-based volumetric study for regions likely to be affected by CD was performed. RESULTS Nineteen patients with a mean disease duration of 24 months were included. Tumor markers included adrenocorticotropic hormone (median 17.5 pmol/L), cortisol (949.4 nmol/L), and dehydroepiandrosterone sulfate (5.4 μmol/L). The following values are expressed as the mean ± SD. The voxelwise statistical test revealed clusters of significantly reduced gray matter in the hippocampus and cerebellum, with volumes of 2.90 ± 0.26 ml (right hippocampus), 2.89 ± 0.28 ml (left hippocampus), 41.95 ± 4.67 ml (right cerebellar hemisphere), and 42.11 ± 4.59 ml (left cerebellar hemisphere). Healthy control volunteers showed volumes of 3.22 ± 0.25 ml for the right hippocampus, 3.23 ± 0.25 ml for the left hippocampus, 50.87 ± 4.23 ml for the right cerebellar hemisphere, and 50.42 ± 3.97 ml for the left cerebellar hemisphere. CONCLUSIONS Patients with untreated CD show significant reduction of gray matter in the cerebellum and hippocampus. These changes can be analyzed and objectified with the quantitative voxel-based method described in this study.

Dexamethasone PONV-prophylaxis alters the hypothalamic-pituitary-adrenal axis after transsphenoidal pituitary surgery.

Background: Postoperative nausea and vomiting (PONV) is common after general anesthesia and are reported by approximately 20% to 25% of all patients and up to 39% of patients undergoing neurosurgical procedures. The most common standard prophylaxis is a single application of 4 mg of dexamethasone before initiating anesthesia. Dexamethasone is known to suppress adreno-corticotroph hormone and cortisol levels. The objective was to find out whether this prophylaxis has an effect on the postoperative levels of cortisol in patients undergoing transsphenoidal pituitary surgery, and therefore simulates pituitary deficiency. Patients and Methods: A retrospective analysis of the files of 136 consecutive patients who were operated during a course of 6 months were included. Nineteen patients with a known history of PONV received a standard dose of 4 mg of dexamethasone perioperatively. Blood tests were drawn at the first postoperative day and were compared with blood tests of patients who had no history of PONV and therefore received no prophylaxis. Results: Patients who were treated with a dexamethasone PONV prophylaxis showed no significant changes in cortisol levels; preoperative median of 93 &mgr;g/L (range, 39 to 427) and a postoperative median of 87 &mgr;g/L (range, 10 to 733; P=0.798) opposed to patients who did not receive such treatment; preoperative cortisol 114 &mgr;g/L (range, 10 to 387) and postoperative levels of 273 &mgr;g/L (range, 10 to 1352; P<0.001). Conclusions: As early postoperative blood checks of the cortisol levels yield important information about potential pituitary sufficiency after transsphenoidal surgery, the probability that dexamethasone PONV prophylaxis suppresses postoperative cortisol levels should be considered.

Intramedullary spinal cavernoma: clinical presentation, microsurgical approach, and long-term outcome in a cohort of 48 patients

OBJECT Intramedullary spinal cavernoma (ISC) is a rare entity and accounts for approximately 5%-12% of all spinal vascular pathologies. The purpose of the present study was to examine the influence of clinical presentation, localization, and different surgical approaches on long-term outcome in patients treated for ISC. METHODS The authors performed a retrospective single-center study of 48 cases of ISC treated microsurgically over the past 28 years. Analyzed factors included preoperative clinical history, microsurgical strategies, neurological outcome (American Spinal Injury Association [ASIA] grade, Epstein and Cooper grade), and the occurrence of postoperative spinal ataxia. Univariate analysis was performed to identify factors influencing long-term outcome. RESULTS Preoperatively, 18.8% of all patients experienced a slow, progressive decline in neurological function and 33.3% suffered repetitive episodes of acute neurological deterioration over a time frame of months to years. Moreover, 16.7% noted the sudden onset of a severe neurological deficit, whereas 25% experienced the sudden onset of symptoms with a subsequent gradually progressive decline in neurological function. On long-term follow-up after treatment (mean ± SD, 79.3 ± 35.2 months), 70.8% of patients showed no change in neurological function, 6.3% suffered from a decline, and 22.9% improved neurologically. Thoracolumbar localization (p = 0.043), low preoperative Epstein and Cooper grade for the lower extremities (p < 0.001), and a low preoperative ASIA grade (p < 0.001) were identified as factors associated with an unfavorable outcome (ASIA Grade A-C). The rate of spinal ataxia related to surgical approach was 16.7%. CONCLUSION Postoperative neurological function in ISC patients is determined by the preoperative neurological status. On long-term follow-up after microsurgical treatment, 93.7% of patients presented with a stable or improved condition (ASIA grade); thus, definite microsurgical treatment should be considered as long as patients present with only mild symptoms after the diagnosis of symptomatic ISC.

Cavernous sinus sampling in patients with Cushing's disease

OBJECT Correct diagnosis and precise localization of adenomas in patients with Cushings disease are essential for avoiding unsuccessful transsphenoidal pituitary exploration. In addition to the well-established inferior petrosal sinus sampling, preoperative cavernous sinus sampling (CSS) was introduced as a potentially improved way to predict adenoma lateralization. The authors present their results with CSS in a consecutive series of patients with Cushings disease. METHODS During 1999-2014, transsphenoidal surgeries were consecutively performed in 510 patients with Cushings disease. For most patients, suppression of cortisol in high-dose dexamethasone tests and stimulation of adrenocorticotropic hormone and cortisol after administration of corticotropin-releasing hormone were sufficient to prove the diagnosis of adrenocorticotropic hormone-dependent hypercortisolism. Of the 510 patients, 67 (13%) were referred to the department of neuroradiology for CSS according to the technique of Teramoto. The indications for CSS were unclear endocrine test results or negative MRI results. Data for all patients were retrospectively analyzed. RESULTS A central/peripheral gradient was found in 59 patients; lateralization to the left or right side was found in 51. For 8 patients with a central/peripheral gradient, no left/right gradient could be determined. For another 8 patients with equivocal test results, no central/peripheral gradient was found. No severe CSS-associated complications were encountered. Of the 51 patients who underwent transsphenoidal surgery, the predicted lateralization was proven correct for 42 (82%). CONCLUSIONS As MRI techniques have improved, the number of potential candidates for this invasive method has decreased in the past decade. However, because detecting minute adenomas remains problematic, CSS remains a useful diagnostic tool for patients with Cushings disease.

Pituicytoma-An outlook on possible targeted therapies

Pituicytoma is a rare neoplasm of the sella region. Tumor resection is the primary treatment option, but remains subtotal due to excessive bleeding in many cases. The search for alternative or additional treatment regimens is necessary.

Ocular Ultrasound as an Easy Applicable Tool for Detection of Terson's Syndrome after Aneurysmal Subarachnoid Hemorrhage

Introduction Intraocular hemorrhage in patients suffering from aneurysmal subarachnoid hemorrhage is known as Tersons syndrome and is an underestimated but common pathology. We therefore designed a prospective single-blinded study to evaluate the validity of ocular ultrasound compared to the gold standard indirect funduscopy in the diagnosis of Tersons syndrome. Material and Methods Fifty-two patients (104 eyes in total) suffering from aneurysmal subarachnoid hemorrhage were enrolled in this study. Two investigators independently performed a single-blinded ocular ultrasound using a standard intensive care ultrasound system to detect an intraocular hemorrhage. Indirect funduscopy following iatrogenic mydriasis served as the gold standard for confirmation or exclusion of an intraocular hemorrhage. Statistical analyses were performed to evaluate the sensitivity and specificity, positive and negative predictive values of the method as well as the learning curve of ocular ultrasound. Results Indirect funduscopy detected Tersons syndrome in 11 of 52 (21.2%) respectively in 21 of 104 (20.2%) eyes in patients suffering from subarachnoid hemorrhage. Sensitivity and specificity increased with the number of ocular ultrasound examinations for both investigators, reaching 81.8% and 100% respectively. Positive and negative predictive values were different for both investigators (63.6% vs. 100% positive and 100% vs. 95.7% negative) but were both correlated to the amount of intraocular hemorrhage. A low Glasgow Coma scale (p = 0.015) and high Hunt & Hess grade (p = 0.003) was associated with a higher rate of Tersons syndrome. Conclusions Ocular ultrasound using standard ultrasound equipment has been confirmed as a reliable, easy-to-handle bedside screening tool for detecting Tersons syndrome. Nevertheless funduscopy remains the gold standard to detect Tersons syndrome.

Symptomatic Vasospasms as a Life-Threatening Complication After Transsphenoidal Surgery

OBJECTIVE To identify symptomatic vasospasms as a rare complication after transsphenoidal surgery, with emphasis on management and outcomes. METHODS In this retrospective study, the medical records of 1997 patients who underwent microscopic transsphenoidal surgery at our hospital between 2008 and 2016 were analyzed regarding postoperative vasospasm events, clinical management, and neurologic outcomes. RESULTS Four patients (0.2%) were identified who developed neurologic deficits in the postoperative phase caused by proven vasospasms due to subarachnoid hemorrhage (SAH). All 4 patients were treated according to current state-of-the-art recommendations for SAH-triggered vasospasms and, as ultima ratio, intra-arterial spasmolysis. Nonetheless, all patients developed multilocular ischemic infarctions. Three patients recovered with no or only slight neurologic deficits (2 with a Glasgow Outcome Score [GOS] of 5; 1 with a GOS of 4), and 1 patient died, at 24 days after surgery. CONCLUSIONS Although a rare complication, vasospasms after transsphenoidal surgery can lead to severe and multilocular ischemic infarctions with a wide variety of neurologic impairments. This rare complication should be considered in patients with unexpected postoperative neurologic deficits. Computed tomography (CT)/magnetic resonance imaging and (contrast-enhanced) CT/magnetic resonance angiography are appropriate diagnostic tools. Treatment of vasospasms, including the option of intra-arterial spasmolysis, should not be delayed.