Christopher Munoz-Bendix

Early surgical occipitocervical stabilization for plasma cell neoplasms at the craniocervical junction: systematic review and proposal of a treatment algorithm

BACKGROUND CONTEXT Plasma cell neoplasms (PCNs) of the craniocervical junction (CCJ) are rare. Because of their destructive growth, PCNs may induce spinal instability and harbor the risk of sudden death. Therefore, PCNs at the CCJ require special consideration. Although the commonly used primary treatment of PCN is radiotherapy (RT), treatment guidelines are inexistent for CCJ occurrences. PURPOSE This study aimed to conduct a systematic review of the literature, evaluate the benefit of early and extended surgical treatment followed by RT, and outline a treatment algorithm based on the data gathered. STUDY DESIGN/SETTING Case series and systematic review of all reported cases in the English, Spanish and German medical literature were carried out. METHODS CASE SERIES retrospective clinical study, tertiary care center (2004-2014). Patients with a lesion of the CCJ (C0-C2) were identified. Clinical charts, imaging data, operative reports, and follow-up data were analyzed. REVIEW a systematic literature review was performed using PubMed. Further manuscripts were identified by the web search engine Google. RESULTS Our series comprised four patients (one female, three males), mean age 58 years. There was one lesion of C1 and three of C2. Two patients with neck pain received vertebroplasty (C1 and C2, respectively) and RT as primary management. Both developed secondary instability of the CCJ after 12 and 5 months, respectively, and required occipitocervical stabilization (OCS). The other two patients underwent OCS and required no additional surgery and no signs of instability at follow-up. Forty-nine cases of OCS were published previously. Spinal stability was achieved significantly more frequently by OCS than by less invasive or medical interventional treatment options (p=.001; two-sided Fisher exact test). CONCLUSIONS Plasma cell neoplasms are highly radiosensitive. However, at the CCJ, a life-threatening instability may occur early and require surgical treatment. Based on personal experience, we favor OCS in this location. A systematic review of the literature supports this approach. We present a summary of our findings in a concise treatment algorithm for PCN of the CCJ.

Primary bone tumors of the spine revisited: A 10-year single-center experience of the management and outcome in a neurosurgical department

Objective: To report a large clinical series of primary bone tumors of the spine (PBTS) and review the current concepts of management. Materials and Methods: We retrospectively analyzed a clinical series of PBTS treated over the last decade (2004-2014) in the spine unit of a large European tertiary care center. Every PBTS was identified from an electronic medical-record system. Analysis comprised medical records and clinical imaging. Overall survival and outcome was measured using the Glasgow Outcome Scale (GOS) at six weeks, six months and one year postoperatively. Surgical management and adjuvant/neoadjuvant strategies were analyzed. A thorough review of the current literature was performed. Results: A total of 79 patients were included. Of these, 44 (55.7%) were male. The age ranged from 9 to 90 years (mean 55), and most patients were adults (93.6%). Local pain was the most common symptom and was present in 91.1% of the patients. The majority of the tumors occurred in the thoracic spine (52 patients, 65.8%). Overall 86% (68 patients) of PBTS were classified as malignant and at the time of diagnosis, 7 patients (8.9%) presented with non-spinal metastasis. The most common histologic types were hematopoietic tumors (72.2%), followed by chondrogenic ones (12.7%). Within hematopoietic tumors, plasmacytoma was the most frequent type (49 patients, 62%). In 12 patients (15.2%) recurrences were seen during the follow-up period. Overall mean survival of benign PBTS was 100%, malignant non-hematopoietic PBTS 50% and, malignant hematopoietic PBTS 84% at one year, respectively. At six weeks and one year after the initial surgery, 79% and 54% of the patients presented a GOS >3, respectively. Conclusion: PBTS were almost exclusively seen in adults. Malignant tumors were markedly more frequent than benign tumors, with hematopoietic tumors being the most common type. For PBTS, early surgery is important in order to restore spinal stability and decompress the spinal cord. This allows pain reduction and prevention of neurological deficits.

Outcome following surgical treatment of chronic subdural hematoma in the oldest-old population

Dear Editor: We would like to thank Prof. Gelabert-Gonzalez for the interest and comments regarding our manuscript entitled BOutcome following surgical treatment of chronic subdural hematoma in the oldest-old population.^ Additionally, we would respectfully like to address every observation performed. In order to address the first point, we have to clearly state that the focus of our manuscript was on the outcome of this subgroup of patients, not on the preoperative clinical grade described byMarkwalder and Reulen in [1] and not in [2] as mentioned in your letter. It is important to mention that this grading was also developed after studying some historical series [3–7], (1) before computer tomography was a standard diagnostic tool; (2) they also included acute subdural hematomas, involving another spectrum of perioperative complications; (3) they utilized other surgical techniques; (4) it is significantly outdated for the evaluation of a postoperative patient (in our center, we would not wait until the patient presents a grade 4, 3, or even 2 according to [1] to perform a computer tomography); and (5) it is profoundly unsuited for outcome evaluation. Nonetheless, the grading described by Markwalder and Reulen [1] would be suitable for the assessment of the preoperative neurological status. Further, Winn et al. [8] label clinical grading scales in chronic subdural hematomas as irrelevant for the decisionmaking process and define its use as purely scientific. We also do not favor the use of the Glasgow Coma Scale (GCS) for the assessment of outcome, but the Glasgow Outcome Scale (GOS) as described by Jennett et al. [9] and utilized in our study. One of the objectives of our study was to evaluate the complications in this subgroup of patients according to our standard therapy, including general anesthesia. Although the use of local anesthesia for the surgical evacuation of a chronic subdural hematoma is a standard therapy in some centers, it is not established in every center. This also applies to the surgical technique. Our study acknowledges a higher rate of perioperative complications in this subgroup of patients that not necessarily are a direct result of general anesthesia, but causalities of previous decompensated illnesses. Still, we also believe and describe in our manuscript that in specific and selected cases, the use of local anesthesia might be an optimal or even more beneficial option compared to general anesthesia. Controversy regarding the use of corticosteroids, postoperative fluid administration, and early mobilization still remains. It was not the purpose of this study to evaluate these significant issues. However, we advocate the early mobilization of all of our patients, if medically possible. We also favor the use of postoperative intravenous fluids, as well as regular electrolyte controls. Nevertheless, use of corticosteroids is in our center usually reserved only for recurrence cases. It was not our intention to infringe any offence with the term oldest-old. Still, as a result of the demographic shift in western societies, inside the cohort of old patients, age is further stratified into three groups: young-old 65–74 years, middle-old 75–84 years, and oldest-old ≥85 years [10–13]. * Christopher Munoz-Bendix christopher.munoz@med.uni-duesseldorf.de

Recurrent spinal metastasis of a sporadic medullary carcinoma of the thyroid after radiation therapy: a case report and review of the literature

Sporadic Medullary Carcinoma of the Thyroid is a relatively uncommon entity and at the time of diagnosis, most already present loco‐regional metastasis. Therapy should be aggressive to reduce recurrence and mortality. Follow‐up period should continue lifelong and should also include calcium/pentagastrin infusion test, as well as 6‐month interval diagnostic imaging.

Endoscopic Lavage of Extensive Chronic Subdural Hematoma in an Infant After Abusive Head Trauma: Adaptation of a Technique From Ventricular Neuroendoscopy

Subdural fluid collections are frequently encountered in young children after non-accidental injury. In a subset of patients, these collections progress in size and ultimately require permanent drainage, which is commonly achieved with subdural-peritoneal shunts. However, excessive protein and cellular contents in the fluid are potential risk factors for shunt failure. Here, we describe the adaptation of an endoscopic lavage technique established for ventricular endoscopy with the aim of improving fluid condition prior to shunting. We present a case of subdural fluid collections secondary to non-accidental injury, where permanent shunting was required but could not be performed due to excessive protein and cellular levels in the subdural fluid despite conventional burr hole drainage. A two-month-old male infant presented with a bulging and tense fontanel, a reduced level of consciousness, bradycardia, and significant macrocephaly. Computed tomography (CT) demonstrated massive bilateral, low attenuation subdural fluid collections, reaching a diameter of 4.5 cm. Emergency burr hole washout and insertion of subdural drains was performed. Despite prolonged drainage over 10 days, the protein level remained at 544 mg/dl and the mean erythrocyte count at 6,493/µl. Continuous drainage was required to avoid clinical deterioration due to raised intracranial pressure; however, the fluid condition was still considered incompatible with permanent subdural-peritoneal shunting. We, therefore, performed an endoscopic subdural lavage with a careful evacuation of residual blood deposits. No complications were encountered. Postoperatively, mean protein level was 292 mg/dl and mean erythrocyte count was 101/µl. Endoscopic lavage could be safely performed in a case of extensive subdural low attenuation fluid collections, where conventional burr hole drainage failed to improve protein and cellular contents as a prerequisite for successful permanent shunting. We conclude that adaptation of this technique can be helpful in selected cases as an alternative procedure.

Can the combination of hyperthermia, seizures and ion channel dysfunction cause fatal post-ictal cerebral edema in patients with SCN1A mutations?

A 21-year-old male with an SCN1A mutation died of cerebral herniation 3 h after a seizure occurring during physical activity. Cases of fatal cerebral edema in patients with SCN1A mutations after fever and status epilepticus have been recently reported raising the question whether sodium channel dysfunction may contribute to cerebral edema and thereby contribute to the increased premature mortality in Dravet Syndrome. We report on our patient and discuss whether the combination of hyperthermia and ion channel dysfunction may not only trigger seizures but also a fatal pathophysiological cascade of cerebral edema and herniation leading to cardiorespiratory collapse.

Giant cell tumor of the lumbar spine with intraperitoneal growth: case report and review of literature

Giant cell tumors of the spine are uncommon. Usually they are benign and solitary, but locally very aggressive. Most of them occur at the sacral spine. There are only 26 reported cases in the literature involving this type of tumor in the lumbar spine, in particular exhibiting an intraperitoneal growth. We present the case of a woman with a primary tumor of the lumbar spine (giant cell tumor) with intraperitoneal growth, the outcome as well as a review of the literature. Furthermore, after reviewing all spinal cases in the literature above the sacral spine, we carefully suggest a management algorithm.