Bruno Splavski

Pediatric and adult Chiari malformation Type I surgical series 1965–2013: a review of demographics, operative treatment, and outcomes

OBJECT Chiari malformation Type I (CM-I) is a hindbrain disorder associated with elongation of the cerebellar tonsils, which descend below the foramen magnum into the spinal canal. It occurs in children and adults. Clinical symptoms mainly develop from alterations in CSF flow at the foramen magnum and the common subsequent development of syringomyelia. METHODS The authors reviewed English-language reports of pediatric, adult, and combined (adult and pediatric) surgical series of patients with CM-I published from 1965 through August 31, 2013, to investigate the following: 1) geographical distribution of reports; 2) demographics of patients; 3) follow-up lengths; 4) study durations; 5) spectrum and frequency of surgical techniques; 6) outcomes for neurological status, syrinx, and headache; 7) frequency and scope of complications; 8) mortality rates; and 9) differences between pediatric and adult populations. Research and inclusion criteria were defined, and all series that contained at least 4 cases and all publications with sufficient data for analysis were included. RESULTS The authors identified 145 operative series of patients with CM-I, primarily from the United States and Europe, and divided patient ages into 1 of 3 categories: adult (>18 years of age; 27% of the cases), pediatric (≤18 years of age; 30%), or unknown (43%). Most series (76%) were published in the previous 21 years. The median number of patients in the series was 31. The mean duration of the studies was 10 years, and the mean follow-up time was 43 months. The peak ages of presentation in the pediatric studies were 8 years, followed by 9 years, and in the adult series, 41 years, followed by 46 years. The incidence of syringomyelia was 65%. Most of the studies (99%) reported the use of posterior fossa/foramen magnum decompression. In 92%, the dura was opened, and in 65% of these cases, the arachnoid was opened and dissected; tonsillar resection was performed in 27% of these patients. Postoperatively, syringomyelia improved or resolved in 78% of the patients. Most series (80%) reported postoperative neurological outcomes as follows: 75% improved, 17% showed no change, and 9% experienced worsening. Postoperative headaches improved or resolved in 81% of the patients, with a statistical difference in favor of the pediatric series. Postoperative complications were reported for 41% of the series, most commonly with CSF leak, pseudomeningocele, aseptic meningitis, wound infection, meningitis, and neurological deficit, with a mean complication rate of 4.5%. Complications were reported for 37% of pediatric, 20% of adult, and 43% of combined series. Mortality was reported for 11% of the series. No difference in mortality rates was seen between the pediatric and adult series. CONCLUSIONS Before undergoing surgical treatment for CM-I, symptomatic patients and their families should be given clear information about the success of treatment and potential complications. Furthermore, surgeons may benefit from comparing published data with their own. In the future, operative CM-I reports should provide all details of each case for the purpose of comparison.

Association of increased body mass index with Chiari malformation Type I and syrinx formation in adults

OBJECT In this paper the authors describe an association between increased body mass index (BMI) and Chiari malformation Type I (CM-I) in adults, as well as its relationship to the development of syringomyelia. METHODS In the period between January 2004 and December 2011, the senior author reviewed the data for all CM-I patients with or without syringomyelia and neurological deficit. Analyzed factors included clinical status (headaches and neurological signs), radiological characteristics of syringomyelia (diameter and vertical extent of syrinx), BMI, and relationship of age to BMI, syrinx diameter, and vertical extent of syrinx. RESULTS Sixty consecutive adults had CM-I, 26 of whom also had syringomyelia. The mean BMI among all patients was 30.35 ± 7.65, which is Class I obesity (WHO), and was similar among patients with or without syringomyelia. Extension of the vertical syrinx was greater in overweight patients (p = 0.027) than in those with a normal body weight. Evidence of de novo syrinx formation was found in 2 patients who gained an average BMI of 10.8 points. After repeated decompression and no change in holocord syrinx width or vertical extent, a reduction in the syrinx was seen after BMI decreased 11.7 points in one individual. No correlation was found between patient age and BMI, age and vertical extension of the syrinx, and age and diameter of the syrinx. CONCLUSIONS An association between increased BMI and CM-I in adults was recognized. Gaining weight may influence the de novo creation of a syrinx in adults who previously had minimally symptomatic or asymptomatic CM-I, and reducing weight can improve a syrinx after unsuccessful surgical decompression. Therefore, a reduction in body weight should be recommended for all overweight and obese patients with CM-I.

Simple Tumor Localization Scale for Estimating Management Outcome of Intracranial Meningioma

OBJECTIVE Intracranial meningiomas are typically benign extracerebral tumors derived from dural arachnoid cells. Predominantly, these tumors are effectively managed surgically, but the prognosis is heavily dependent on a tumors histologic heterogeneity and its location. It is known that reproductive and hormonal factors increase the risk for meningioma in adult females, but their prognostic value still remains controversial. This studys aim was to examine possible correlation between tumor location and outcome, as well as to evaluate the prognostic value of the proposed simple tumor localization scale in predicting the management outcome of intracranial meningioma. METHODS In a retrospective, cross-section survey, a single-institution cohort of 243 patients operated on due to intracranial meningioma was analyzed. The investigated parameters were age and gender, tumor location, and extent of surgery (Simpson grading). The patients were divided into 2 groups allocating tumors to central and peripheral position within the cranial vault. The outcome was estimated by Karnofsky Performance Score (KPS) at discharge from hospital and Glasgow Outcome Scale at 1 year after surgery. RESULTS A statistically significant correlation between tumor location and outcome, as well as negative correlation between age and outcome, were observed (P < 0.001). CONCLUSIONS When estimating the management outcome of intracranial meningiomas, we propose a simple localization scale dividing the tumors into central and peripheral positions. Central tumor location might be prognostically unfavorable due to involvement of major neurovascular structures. Among the independent factors predicting favorable tumor response to surgery, more effective outcomes associated with peripheral tumor location and younger age were observed.

Hemangioblastomas of the Posterior Cranial Fossa in Adults: Demographics, Clinical, Morphologic, Pathologic, Surgical Features, and Outcomes. A Systematic Review

BACKGROUND Posterior cranial fossa (PCF) hemangioblastomas are benign, highly vascularized, and well-differentiated tumors with well-described histopathologic features. Although relatively rare, this tumor is the most prevalent primary tumor of the cerebellum in adults. OBJECTIVE Because the demographics of patients with such a tumor (as well as the clinical, morphologic, pathologic, surgical features, and outcomes) are not fully understood, we systematized characteristic patient and tumor features. METHODS We undertook a systematic review of the English-language literature in PubMed for PCF hemangioblastomas in adults published in the past 31 years. We analyzed geographic distribution and year of publication of articles; demographic data of patients; presenting symptoms and clinical signs; tumor location and morphology; histopathologic features, extent of tumor resection, perioperative blood loss, and postoperative complications; length of hospital stay; and outcomes. RESULTS We reviewed 207 articles describing 1759 infratentorial hemangioblastomas in a cohort of 1515 adult patients. We found female predominance in patients with Von Hippel-Lindau disease (VHLD) compared with male predominance in the general patient group. Symptoms of intracranial hypertension were more common in the VHLD group compared with the general group of patients. The cerebellar location was more common in the VHLD group and solid (parenchymatous) tumor was the most common type. Most patients underwent total resection but rate of resection did not differ between the general and VHLD groups. Most patients had a favorable outcome. CONCLUSIONS The literature of adult PCF hemangioblastomas is limited and general surgical experience with such tumors is scarce because of their rarity. Rates of postoperative complications and mortality remain higher than expected. However, prognosis and surgical outcomes are generally favorable. Nevertheless, surgery of adult PCF hemangioblastomas is a demanding and challenging task.

Primary Central Nervous System Anaplastic Large T-cell Lymphoma -

Introduction: Primary central nervous system lymphoma (PCNSL) of T-cell origin is an exceptionally rare, highly malignant intracranial neoplasm. Although such a tumor typically presents with a focal mass lesion. Case report: Past medical history of a 26-year-old male patient with a PCNS lymphoma of T-cell origin was not suggestive of intracranial pathology or any disorder of other organs and organic systems. To achieve a gross total tumor resection, surgery was performed via osteoplastic craniotomy using the left frontal transcortical transventricular approach. Histological and immunohistochemical analyses of the tissue removed described tumor as anaplastic large cell lymphoma of T-cells (T-ALCL). Postoperative and neurological recovery was complete, while control imaging of the brain showed no signs of residual tumor at a six-month follow-up. The patient, who did not appear immunocompromized, was referred to a hematologist and an oncologist where corticosteroids, the particular chemotherapeutic protocol and irradiation therapy were applied. Conclusion: Since PCNS lymphoma is a potentially curable brain tumor, we believe that proper selection of the management options, including early radical tumor resection for solitary PCNS lymphoma, may be proposed as a major treatment of such a tumor in selected patients, resulting in a satisfactory outcome.

Management and Prognosis of Primary Cerebral Melanocytic Tumors. A Case Report and Systematic Review

Primary melanocytic cerebral tumors form a spectrum of different lesions that range from benign to extremely malignant, such as an intracranial melanoma. This article points out some characteristics of primary melanocytic cerebral tumors and discusses their management and prognosis, based on a systematic literature review and a case of primary cerebral malignant melanoma.Because the biological characteristics of primary melanocytic cerebral tumors remain unclear, it appears that total surgical resection of the tumor followed by oncologic treatment is the mainstay of the management. The prognosis still depends on the degree of malignancy.