Emergency Decompressive Surgery Research Articles

Thoracic giant cell tumor after two total en bloc spondylectomies including one emergency surgery: A case report

BACKGROUND For patients with acute paraplegia caused by spinal giant cell tumor (GCT) who require emergency decompressive surgery, there is still a lack of relevant reports on surgical options. This study is the first to present the case of an acute paraplegic patient with a thoracic spinal GCT who underwent an emergency total en bloc spondylectomy (TES). Despite tumor recurrence, three-level TES was repeated after denosumab therapy. CASE SUMMARY A 27-year-old female patient who underwent single-level TES in an emergency presented with sudden severe back pain and acute paraplegia due to a thoracic spinal tumor. After emergency TES, the patient's spinal cord function recovered, and permanent paralysis was avoided. The postoperative histopathological examination revealed that the excised neoplasm was a rare GCT. Unfortunately, the tumor recurred 9 months after the first surgery. After 12 months of denosumab therapy, the tumor size was reduced, and tumor calcification. To prevent recurrent tumor progression and provide a possible cure, a three-level TES was performed again. The patient returned to an active lifestyle 1 month after the second surgery, and no recurrence of GCT was found at the last follow-up. CONCLUSION This patient with acute paraplegia underwent TES twice, including once in an emergency, and achieved good therapeutic results. TES in emergency surgery is feasible and safe when conditions permit; however, it may increase the risk of tumor recurrence.

Emergency decompressive surgery in patients with transtentorial brain herniation and pupillary abnormalities: the importance of improved pupillary response after osmotherapy and surgery.

The predictors of survival and functional recovery following emergency decompressive surgery in patients with transtentorial brain herniation, particularly those with pupillary abnormalities, have not been established. In this study, the authors aimed to assess the outcome of patients with intracranial mass lesions, transtentorial brain herniation, and nonreactive mydriasis, following emergency surgical decompression. A retrospective chart review was performed of all patients with transtentorial herniation and pupillary abnormalities who underwent craniotomy or craniectomy at two trauma and stroke centers between 2016 and 2022. The functional outcome was determined using the modified Rankin Scale (mRS). Forty-three patients, 34 men and 9 women with a mean age of 47 years (range 16-92 years), were included. The underlying etiology was traumatic brain injury in 33 patients, hemorrhagic stroke in 8 patients, and tumor in 2 patients. The median preoperative Glasgow Coma Scale score was 3 (range 3-8), and the median midline shift was 9 mm (range 1-29 mm). Thirty-two patients (74.4%) had bilaterally fixed and dilated pupils. The median time to surgery (from pupillary changes) was 133 minutes (mean 169 minutes, range 30-900 minutes). Eighteen patients (41.9%) died postoperatively. After a median follow-up of 12 months (range 3-12 months), 11 patients (26.8%) had a favorable functional outcome, while 10 remained severely disabled (mRS score 5). On univariate analysis, younger age (p < 0.001), less midline shift (p = 0.049), and improved pupillary response after osmotic therapy (p < 0.01) or decompressive surgery (p < 0.001) were associated with favorable outcomes at 3 months. With aggressive medical and surgical management, patients with transtentorial brain herniation, including those with bilaterally fixed and dilated pupils, may have considerable rates of survival and functional recovery. Young age, less midline shift, and improved pupillary response following osmotic therapy or decompressive surgery are favorable prognosticators.

Salmonella Epidural Abcess with Acute Paraplegia: Report of a Rare Vertebral Infection

Salmonella Vertebral osteomyelitis (SVO) is an uncommon entity. In this article, we reported a rare case of a 39-year-old man admitted with low backache for three days which progressed to paraparesis within 24 hours. Urgent CEMRI revealed Spondylodiscitis D7/D8 associated with an epidural abscess causing significant cord compression. He was initially diagnosed as thoracic Potts Spine and underwent emergency decompressive surgery. He was started on Antitubercular drugs, however, tissue and wound culture grew Salmonella Enterica. USG abdomen revealed hepatosplenomegaly. He was started on 6 weeks intravenous antibiotics accordingly; the patient showed postoperative improving neurological deficits with significant symptomatic relief and radiological resolution.

FRI572 Teprotumumab In Unexplored Presentation Of Thyroid Eye Disease

Abstract Disclosure: M.T. Torres Torres: None. A.N. Gonzalez Bossolo: None. M.M. Mangual Garcia: None. Thyroid eye disease (TED) is an autoimmune disease of the orbital soft tissue associated with facial disfigurement, diminished quality of life, and potentially vision-threatening consequences that can occur in 40% of patients with Graves’ disease (GD). All the therapies for this condition were off-label with questionable long-term effects until 2020 when the Federal Drug Administration approved teprotumumab, a human monoclonal antibody against insulin-like growth factor-1 receptor, for the treatment of TED. Randomized placebo-controlled trials have demonstrated that teprotumumab reduces proptosis, diplopia, and Clinical Activity Score (CAS) in patients with active TED of less than 1.3 years of duration who were treatment-naïve or previously treated with &amp;lt; 1g of methylprednisolone equivalent therapy. Data on the efficacy of teprotumumab on cases of longer TED duration or previously treated with eye surgery or &amp;gt; 1g of methylprednisolone equivalent therapy for TED is limited as these patients were excluded from the trials. A 76-year-old nonsmoker male with long-standing hyperthyroidism due to GD, glaucoma, and type 1 diabetes mellitus was referred by ophthalmologist due to TED. He received radioactive iodine (RAI) for treatment of GD after failure to Methimazole therapy. After RAI, he reported progressively worsening bilateral eye edema, redness, pain, vision loss, and diplopia requiring hospitalization for an emergent orbital decompressive surgery and intravenous steroid therapy due to sight-threatening Grave’s orbitopathy. He was discharged home with oral prednisone taper down until it was discontinued 6 months later with mild improvement of symptoms. Physical exam was remarkable for bilateral strabismus, orbital chemosis, conjunctival injection and redness, eyelid swelling, limited eye motion, severely impaired visual acuity and color vision, no proptosis, and a CAS of 5 suggesting active TED. Laboratories showed biochemical euthyroidism and controlled glycemia. Teprotumumab was started 2 years and 8 months after symptom onset with marked improvement of inflammatory signs and symptoms, eye motion, visual acuity, diplopia, intraocular pressure, and CAS score decrease to 1 point suggesting inactive disease within 4 weeks of therapy without adverse side effects. This case describes the positive effects of teprotumumab in a patient with characteristics not previously studied such as active TED of longer duration than in clinical trials, previous treatment with orbital surgery or &amp;gt; 1g of methylprednisolone equivalent therapy, and increased IOP. Rather than drawing conclusions, our goal is to create awareness and promote further research of teprotumumab and novel agents in patients with TED with previously uncharacterized presentations as it may have important implications for their management including significant improvement in their quality of life. Presentation: Friday, June 16, 2023

Outcomes of orbital decompression for thyroid eye disease over a 10-year period at a tertiary eye care referral center

Background: Orbital decompression is frequently indicated to treat exophthalmos and compressive optic neuropathy, among other indications for thyroid eye disease (TED). This study aimed to evaluate the outcomes of orbital decompression and compare the results by urgency and type of surgery in patients with TED.&#x0D; Methods: In this cross-sectional study, we recruited patients with TED who had undergone emergency or elective orbital decompression surgery at a tertiary eye care referral center in Tehran, Iran, between 2010 and 2020. Ophthalmic examination findings, demographic and clinical profiles, and types and outcomes of surgical interventions were reviewed and analyzed.&#x0D; Results: Fifty-one orbits of 35 patients with a mean (standard deviation [SD]) age of 36.2 (12.0) years and male-to-female ratio of 23 (66%)/12 (34%) were included. The mean (SD) duration from the diagnosis to the surgery was 41.0 (39.0) months. The surgical method was fat decompression in 1 (2%) orbit; fat and inferior wall decompressions in 2 (4%) orbits; fat, inferior, and medial wall (two-wall) decompressions in 43 (84%) orbits; and fat, inferior, medial, and lateral wall (three-wall) decompressions in five (10%) orbits. Three-wall decompression surgery resulted in significantly lower exophthalmometry readings than those associated with two-wall surgery at all postoperative follow-ups (P &lt; 0.05). Ten (20%) orbits required emergency decompression because of sight-threatening conditions and revealed comparable exophthalmometry readings with electively decompressed orbits at the 1-year visit (P &gt; 0.05). Thirty-seven (73%) orbits required other surgeries within the 1-year follow-up. The mean (SD) exophthalmometry readings before and 1-year after surgery were 26.3 (4.0) and 18.3 (2.7) mm, respectively, with a significant decrease and significant 5.5 (3.3)-mm change from baseline in decompressed orbits (both P &lt; 0.001). Diplopia was reported in 29% (n = 10) of patients less than 2 months postoperatively.&#x0D; Conclusions: Emergency or elective orbital decompression significantly reduced exophthalmos in patients with TED within 1 year postoperatively. Three-wall orbital decompression produced the more immediate impact, while two-wall orbital decompression showed the higher effect at a later timepoint. The most common complication was diplopia, while other serious complications occurred infrequently. Further prospective comparative studies involving more participants and longer postoperative follow-up periods are required to verify these preliminary findings.

Position of surgical treatment in step-up management of severe acute pancreatitis.

The incidence of acute pancreatitis has been increasing over the past twenty years and there is still no causal treatment available. Although cases of severe acute pancreatitis account for only about a fifth of all cases of acute pancreatitis, high morbidity and lethality call for an optimization and unification of treatment procedures. We operated on 27 patients suffering from severe acute pancreatitis in the past five years. We compared selected parameters such as gender, age, body mass index, aetiology, presence of type 2 diabetes, BISAP score, previous minimally invasive treatment and presence of the intraabdominal compartment syndrome. The average age of men and women was similar in our group. Most patients were overweight or obese. Alcoholic aetiology was more common in men while biliary aetiology prevailed in women. The mortality rate was 26% in our group. The intra-abdominal compartment syndrome followed by emergency decompression surgery was present in one fourth of the patients. A minimally invasive approach was used in approximately in one half of the patients, and surgical treatment was used only in cases where the minimally invasive approach failed. After each surgical revision, clinical deterioration of the patient´s condition occurs during the first two to three days in response to operative stress. Therefore, the current trend in the treatment of acute pancreatitis is to proceed as conservatively as possible, or using the minimally invasive approach, and surgical treatment should be reserved only for conditions that cannot be managed otherwise. If surgical treatment is used, it is advisable to perform cholecystectomy, whatever the aetiology of the pancreatitis.

Are Bilaterally Fixed and Dilated Pupils the Kiss of Death in Patients with Transtentorial Herniation? Systematic Review and Pooled Analysis

Bilaterally fixed and dilated pupils (BFDP) in the setting of transtentorial herniation due to a space-occupying lesion have traditionally been considered a sign of futility. As a result, such patients may be denied life-saving decompressive surgery, resulting in very high mortality rates. We sought to determine the survival rate and functional outcomes in patients with transtentorial herniation and BFDP following emergency decompressive surgery. This was a systematic review of MEDLINE, Embase, Cochrane, and Google Scholar databases, using a combination of 15 prespecified keywords, according to Preferred Reporting Items for Systematic reviews and Meta-Analyses methodology. Individual patient data were extracted, pooled, and analyzed. Twenty-two studies totaling 503 patients were included. Study designs were as follows: prospective cohort (n= 1), retrospective cohort (n= 15), and case report (n= 6). Nearly two thirds of patients (67.7%) were male. The mean age was 41years (range= 3-82). The median preoperative Glasgow coma scale was 3 (range= 3-6). Nearly two thirds (66.9%) underwent surgical decompression within 2hours of pupillary changes. The mean follow-up was 7months (range= 1-40). Two thirds (67%) died. Among survivors, 50.5% had severe disability (Glasgow outcome scale= 2-3), while 49.5% had a good outcome (Glasgow outcome scale 4-5), representing 17% of the whole population. Given the methodological limitations, the prognostic value of age, Glasgow comascale, and time to surgery could not be determined. The literature suggests a rate of favorable recovery approaching 17% following decompressive surgery in patients with transtentorial herniation and BFDP, secondary to space-occupying lesions. In the setting of stroke or trauma, the clinical finding of BFDP should not be solely relied on as an indicator of futility. Prospective studies are warranted.

Crystallin Alpha-B Overexpression as a Possible Marker of Reactive Astrogliosis in Human Cerebral Contusions

The pathophysiology of traumatic brain injury (TBI) has not yet been fully elucidated. Crystallin alpha-B (CRYAB) is a molecular chaperone that apparently tries to stabilize the rapid thickening of the intermediate filaments of glial fibrillary acidic protein (GFAP) during the process of reactive astrogliosis in response to TBI. Previous analyses of the gene expression profile in human brain contusion tissue showed us an exacerbated CRYAB overexpression. Here, we used 3, 3’-diaminobenzidine (DAB) immunohistochemistry and immunofluorescence to verify CRYAB overexpression and to describe its expression and distribution in samples of contused cortical tissue derived from emergency decompressive surgery after severe TBI. The histological expression of CRYAB was mainly seen in subcortical white matter astrocytes of injured tissue. Most of the cells that overexpressed GFAP in the analyzed tissue also overexpressed CRYAB, a finding corroborated by the co-localization of the two markers. The only difference was the presence of a few pyramidal neurons that expressed CRYAB in layer V of the cerebral cortex. The selective vulnerability of layer V of the cerebral cortex during TBI could explain the expression of CRYAB in neurons of this cortical layer. Our results indicate a parallel behavior in the cellular expression of CRYAB and GFAP during the subacute response to TBI. These results lead us to postulate CRYAB as a possible marker of reactive astrogliosis in contused cortical tissue.

Is Cauda Equina Surgery Safe Out-of-Hours? A Single United Kingdom Institute Experience

Cauda equina syndrome (CES) can have devastating neurological sequelae if surgical treatment is delayed. However, out-of-hours surgery (weekdays from 6:00 pm to 8:00 am and all weekend operations) can potentially result in higher rates of intraoperative complications, resulting in worse outcomes. In the present study, we have described our outcomes for patients with CES during an 8-year period (December 2011 to October 2019) with the aim of assessing the risk of out-of-hours surgery. We performed a retrospective analysis of inpatient events and outcomes at 6 months of follow-up. Patient demographics, symptoms, and management data were extracted, and a risk factor analysis was performed using logistic regression. The outcome measures were the incidence of complications and symptom changes at follow-up. Symptom outcome changes between 2 time points were analyzed using repeated measures. A total of 278 patients were included in the present study. Surgery out-of-hours (P= 0.018) and prolonged operations (P= 0.018) were significant risk factors for intraoperative complications. Improved outcomes at 6 months of follow-up were found for lower back pain, sciatica, altered saddle sensation, and urinary sphincter disturbance, with no significant changes for the remaining symptoms. Out-of-hours surgeries did not significantly affect individual symptom outcomes. Our analysis has suggested that emergency decompressive surgery for patients with CES does not result in worsening of outcomes with out-of-hours surgery compared with in-hours. However, our findings also showed that no clear benefit exists to expediting surgery for those with severe presentations. Thus, decompressive surgery should be undertaken at the earliest possible time to safely do so.

Implementation of a cauda equina service in a medium-sized district general hospital in the UK.

This project explored how the implementation of national guidance for investigation and management of patients with suspected cauda equina syndrome impacted on service provision. Retrospective analysis of patients with suspected cauda equina syndrome during 12 months before the implementation of the national guidelines were compared with data from the 21 months following. Monthly mean numbers of referrals for suspected cauda equina syndrome increased from 10.1 to 18.9 (P<0.001). Statistically significant increases were also seen in the total number of magnetic resonance imaging scans for suspected cauda equina syndrome, and the number of magnetic resonance imaging scans performed out of hours. The mean time interval, from magnetic resonance imaging scan confirming cauda equina syndrome to starting emergency decompressive surgery, reduced from 14.87 hours to 9.57 hours. Compliance with the national guidance for suspected cauda equina syndrome is imperative for patients to receive optimal treatment. However, this project has demonstrated challenges related to increased pressure on resources.

Cauda equina syndrome in a postoperative patient of total hip replacement surgery: Possible exacerbation due to extreme intraoperative manipulation

Cauda equina syndrome (CES) is a rare but devastating complication, which warrants an urgent or emergency decompressive surgery. To diagnose it early and also, a timely intervention can halt neurological sequelae. More common causes for CES include chronic disc diseases, spinal injury, and fractures. However, there are less common causes such as spinal manipulation, tuberculosis, and spinal tumors. Here, we report a rare cause of CES precipitated by extreme manipulation of the spine during total hip replacement surgery done under neuraxial blockade. This condition was diagnosed by the acute pain service team at an early stage and successfully treated with emergency decompressive surgery to avert any further neurological complications.

Protocolo de actuación y manejo de la compresión medular aguda en urgencias

Acute spinal injuries caused by spinal trauma are frequent damage in patients suffering from high-energy trauma. A correct clinical evaluation and initial management (administration of ABCD of basic life support) are of vital importance. Whenever, anybody suffering from high-energy trauma has to be suspected of spinal column damage. Thus, patient transfer demands total cervical and dorsolumbar spine immobilization with orthopedic collar and right board. Thorough neurological examination has to be performed accompanied by complementary imaging tests. The goal of treatment of emergency will be to achieve mean high blood pressure (85-90mmHg) for a week. If clinical progression is observed, emergency decompressive surgery (in less than 24hours) will be required.

Myofascial self-release law

Osteofascial compartment syndrome (OFCS) is clinically common and is well known to orthopedic surgeons. Clinicians attach great importance to OFCS because of its severe clinical consequences, and decompression of fascial compartment is often performed in emergency treatment. This article reviews the literature on the threshold of fascial compartment decompression proposed by many scholars in the past and discusses the problems in the clinical diagnosis of acute compartment syndrome, especially the inconsistent pressure thresholds as the indication for emergency decompression surgery. By observing calf fractures patients with tension blister, we found that the pressure of fascia decreased sharply upon the appearance of blisters. Meanwhile, the swelling gradually subsided as well as the clinical manifestations of pain and parasthsia. In view of the uncertainty of various thresholds of fascial decompression and self-decompression, the concepts of myofascial self-release law and muscle-swelling syndrome were first proposed. The author believes that when intracompartmental pressure rises to a point, some unknown mechanisms of fascia can achieve self-decompression. Therefore, no compartment syndrome will take place. We also emphasize that the 'muscle-swelling syndrome’ should be strictly distinguished from the soft tissue necrosis caused by crush syndrome and acute limb vascular injury, so as to provide more precise treatment. We believe that without external restrictions such as casts, splints and compression bandages, the muscle-swelling syndrome can achieve self decompression by releasing the pressure in the compartment through tension blisters, and there is no need for fasciotomy. Key words: Compartment syndromes; Blister; Myofascial self-release law

Ewing sarcoma of the mobile spine; predictive factors for survival, neurological function and local control. A Scandinavian sarcoma group study with a mean follow-up of 12 years

Object: Many patients with Ewing sarcoma (ES) of the mobile spine present with neurologic symptoms leading to emergency decompressive surgery. Only rarely is optimal treatment involving neo-adjuvant chemotherapy followed by en bloc excision possible. The purpose of this study was to study treatment, neurologic and oncologic outcome in patients with ES of the mobile spine.Methods: Twenty-four patients diagnosed between 1986 and 2012 were identified through the Scandinavian Sarcoma Group registry. Charts were reviewed in order to assess details in patient characteristics, neurologic status, treatment and outcome. Prognostic factors were analyzed with respect to local control, disease-free survival and overall survival.Results: Neurologic symptoms were frequently observed at presentation, being present in 19/23 patients with documented neurologic status. Most (13/19) patients had a complete neurologic recovery regardless of whether or not emergency decompressive surgery was performed. The majority (18/24) of patients were treated with definitive radiotherapy. However, only 9/17 received the recommended dose of ≥ 50.4 Gy. The disease-free and overall survival rates at 10 years were 48% and 57%, respectively. The local recurrence rates were 19% and 27% at 5 and 10 years, respectively. Only year of diagnosis, categorized into periods with significant changes in chemotherapy protocols, was a significant factor for local recurrence, but there was a trend (p = 0.06) for an increased risk of a local recurrence if emergency decompressive surgery was performed.Conclusion: Patients with ES of the mobile have a relatively favorable prognosis. Nonetheless, local recurrence rate is high for this group of patients for which local treatment mainly relies on definitive radiotherapy.Emergency decompressive surgery may increase the risk for local recurrence.

Early mobilisation does not increase the complication rate from unintended lumbar durotomy

Background: Unintended durotomy is a well-recognised complication of lumbar spine surgery. Reported complications include headaches, intracranial haematomata, pseudomeningocoele and infection. Methods of intraoperative repair vary and although post-operative flat bed rest is advocated by some, there is no consensus on duration. We reviewed a series of unintended durotomies that occurred in our institution and reviewed them to compare management strategies and outcome.Methods: A retrospective analysis was conducted of adult patients who experienced an unintended durotomy during surgery for lumbar degenerative disease in our neurosurgical unit over a 15-month period. Post-operative complications were followed up for a minimum of 3 months.Results: 1125 patients underwent elective or emergency decompressive lumbar spine surgery. 45 (4%) dural tears were identified; all were repaired intra-operatively with suturing, Tisseal thrombin glue or both. Absence of leakage was confirmed on Valsalva manoeuvre for all cases, before wound closure. 28 patients were mobilised within 24 hrs of surgery, 16 patients between 24–48 hours and 1 patient after 48 hours. Seven patients (16%) with a dural tear experienced a complication. There was no statistically significant relationship between time to post-operative mobilisation and complication rate (p = .76). There was a significantly longer inpatient stay when patients were on bed rest for longer (2 tailed test significant at the 2% level).Conclusion: Duration of post-operative bed rest was not related to complication rate but led to delays in discharge. We did not find evidence that early mobilisation lead to increased likelihood of complications.

Effect of decompressive surgery on spine balance in congenital lumbar kyphoscoliosis: anatomical aspect with a case report

The lumbar vertebral column is a complex structure formed by the fusion of intervertebral joints. It protects the spinal cord by wrapping around it, carries most of the body’s weight and creates a flexible structure for the body. As individuals with congenital deformities of the lumbar vertebral column age, they develop some changes in the anatomy of the spine and with some consequences. In this case, a 35-year-old male patient had a deteriorated posture together with back pain since childhood. He had walking difficulty and numbness in two legs during walking for the last three years. He fell due to a sudden brake while he was travelling afoot by bus in 2015. He was admitted to the emergency service with loss of strength in two legs. Lumbar CT and MRI showed a severe narrowing of the spinal canal due to lumbar kyphoscoliosis. The patient had neurological deficits after hyperflexion trauma. The patient underwent emergency decompressive surgery due to marked paraparesis and narrow spinal canal. At the end of the first post-operative month, his clinical complaints were almost completely resolved, and his biomechanical balance did not show radiological deterioration. While the anatomical change that occurred during the natural course of congenital kyphoscoliosis increased the stability of the lumbar spine, it severely narrowed the spinal canal and affected the spinal nerves and thereby caused severe neurological deficits.

Drug reaction with eosinophilia and systemic symptoms after daclizumab therapy in MS.

In 2012, a 33-year-old woman was diagnosed with relapsing-remitting MS (RRMS) after fulfilling McDonald criteria and careful exclusion of other differential diagnoses. Five years later (EDSS 1), she changed her disease-modifying therapy after side effects to betaferon (flu-like symptoms) and dimethyl fumarate (erythema and pruritus) to daclizumab. Two months after 2 applications of 150 mg daclizumab, she developed a self-limiting rash (figure 1A), followed by fever, headaches, meningismus, photophobia, nausea, paraesthesia, and itching of her upper body 1 month later. Blood test showed decreased lymphocyte counts of 1.7/μL (17.1%) and increased eosinophil granulocytes of 1.5/μL (15.1%). CSF analysis revealed pleocytosis of 570/μL (1.8% eosinophilic granulocytes) and an initially negative, but 2 weeks later marked intrathecal immunoglobulin synthesis (IgM 85%, IgA 43%, and IgG 38%). MRI showed periventricular contrast-enhancing lesions and a multisegmental cervical myelitis (C1–4). After 10 days, she further deteriorated by severe infratentorial edema with compression of the fourth ventricle and obstructive hydrocephalus (figure 1, B–F). At admission to our hospital, she obtained an external ventricle drainage showing normal opening pressure. Two days later, she had sudden increase in intracranial pressure requiring intubation and emergency decompressive surgery of the posterior fossa. Increased intracranial pressure (up to 40 mm Hg) due to global brain edema and subsequently complicated by a postoperative bleeding into the right frontal lobe after a brain biopsy (see below) was controlled by CSF drainage, deep analgosedation, dexamethasone, and osmotherapy. The atypical clinical picture with brain edema, rhombenecephalitis, and high CSF cell count was incompatible with MS relapse as initially suggested by the periventricular lesions and cervical myelitis. Because careful examinations revealed no other autoimmune or infectious diseases, we initially treated polypragmatically with aciclovir/ganciclovir, ceftriaxone/ampicillin, voriconazole/posaconazole, and IV immunoglobulins. Brain biopsies of the right frontal lobe, cerebellum, and cerebellar leptomeninges showed inflammatory demyelinating lesions with pronounced inflammation, numerous T cells, eosinophils, plasma cells, and an eosinophilic and lymphoplasmacellular meningitis (figure 1, G–J). We diagnosed the patient with CNS manifestation of daclizumab-induced DRESS (drug reaction with eosinophilia and systemic symptoms) since 6 of 7 Registry of Severe Cutaneous Adverse Reaction Criteria (RegiSCAR)1 for DRESS were fulfilled (positive: hospitalization, reaction suspected to be drug-related, fever >38°C, skin rash, internal organ involvement, blood eosinophilia; negative: lymphadenopathy). Accordingly, the patient was treated with high-dose IV methylprednisolone (5 days with 1 g each) and 6 cycles of plasma exchange, followed by 1 mg/kg oral prednisolone with subsequent tapering. Thereafter, the patient improved dramatically and could be extubated 2 weeks after disease exacerbation. Postoperative disease course was complicated by wound infection, bacterial meningitis, persisting CSF effusion, and obstructive hydrocephalus that required a ventriculoperitoneal shunt. Four months later, neurologic syndromes with tetraparesis, choreoathetosis, cerebellar ataxia, and cognitive dysfunctions had regressed substantially (EDSS 3.5). The authors thank the patient for participation in the study.

The inflammatory pseudotumor presenting periodic acid-Schiff-positive inclusions with acute unilateral facial nerve palsy

Although most acute peripheral facial palsies can be attributed to Bell’s palsy, other factors, such as infection, trauma, and neoplasm, can cause facial palsy as well. Among these, facial nerve tumors are rare but should be considered in the differential diagnosis of facial palsy. Palsies due to facial nerve tumors usually present with slow onset but occasionally present as acute episodes. In such cases, facial nerve decompression is the treatment of choice to allow the tumor room to grow without compressing the nerve or its blood supply. We describe a case of severe, acute facial palsy presenting with a spindle-shaped bone erosion on the mastoid portion of the facial canal. Although facial neuroma was suspected preoperatively, emergency decompression surgery revealed that an unusual inflammatory pseudotumor was responsible for the finding. Postoperative histological analysis revealed extensive destruction of the nerve fibers, with extensive infiltration of foamy macrophages containing characteristic, diastase-resistant, periodic acid-Schiff (PAS)-positive inclusions, which are hallmark of the uncommon bacterial infections. This was a case of facial palsy with an unusual etiology. The case shows the benefit of decompression surgery not only as treatment for the palsy but also as exploratory surgery in cases of facial nerve tumor.

Extragonadal germ cell tumor of the posterior mediastinum in a child complicated with spinal cord compression: a case report

BackgroundGerm cell tumors (GCTs) in children are rare neoplasms with diverse pathological findings according to the site and age of presentation. The most common symptoms in children with mediastinal GCTs, which are nonspecific, are dyspnea, chest pain, cough, hemoptysis, vena cava occlusion syndrome, and fatigue/weakness. Because of these nonspecific symptoms, it is difficult to suspect a mediastinal mass. A posterior mediastinal tumor causing spinal cord compression is an important example of an oncologic emergency arising from a neurogenic tumor.Case presentationChildren with posterior mediastinum GCTs can be easily mistaken as having a neurogenic tumor because of site of tumor origin. We treated our 7-year-old patient with emergency decompression surgery and high-dose steroid pulse therapy to prevent secondary injury to the spinal cord. Primary injury was a result of spinal cord compression due to the initial manifestation of GCT in the posterior mediastinum. Cisplatin-based chemotherapy was also administered. The patient was followed up regularly for 3 years and is undergoing rehabilitation without any signs of recurrence.ConclusionsWe present an extremely rare case of a child with paraparesis caused by extradural spinal cord compression as the initial manifestation of GCT in the posterior mediastinum. The child was treated with emergency decompression surgery and high-dose pulse steroid therapy to prevent secondary injury to the spinal cord.

Use of Whole Brain Radiation Therapy and Immune Checkpoint Inhibition in the Management of Brain Metastases

Whole brain radiation treatment (WBRT) and immune checkpoint inhibition (ICI) are both important treatment modalities in the management of patients with brain metastases; however, concern exists regarding potential increased toxicity with concurrent and sequential use. A retrospective analysis was conducted of patients treated with WBRT within 6 months of the receipt of ICIs including anti-PD-1 (n=18; 56%), anti-CTLA-4 (n=6; 19%) and combined anti-PD-1/anti-CTLA-4 therapies (n=8; 25%). Concurrent treatment was defined as receipt of ICI within 21 days before or during WBRT. The primary endpoint was neurotoxicity assessed by history/clinical examination and Edmonton Symptoms Assessment Scores (ESAS) within one month before and after WBRT. Secondary endpoints included intracranial progression free survival (PFS) and overall survival (OS). 32 patients received WBRT within 6 months of receiving an ICI between February 2013 and October 2016. Median WBRT dose was 30 Gy in 10 fractions. Predominant histologies included melanoma (n=16; 50%; 6 BRAF mutant) and NSCLC (n=13; 41%). ICIs were delivered concurrently, before, or after WBRT in 6 (19%), 9 (28%), and 17 (53%) patients, respectively. Median follow-up following WBRT was 3 months (range 0-24 months). The majority of patients were on steroids during WBRT (n= 22; 69%). One patient with a history of three prior decompressive surgeries and multiple SRS treatments experienced new seizures following 2 fractions of 300 cGy with concurrent anti-PD-1/anti-CTLA-4 therapy and required emergent surgical resection that revealed disease progression. Three patients, one with ICI before and two after WBRT, experienced grade 2-3 headaches following WBRT, which resolved with dexamethasone. ESAS questionnaires were completed by 15 patients (47%). There were no changes in median scores for pain, drowsiness, nausea, dyspnea, depression, or spiritual domains following WBRT. Modest nonsignificant numerical improvements were noted in anxiety and overall well-being with worsening in fatigue, appetite, and sleep. Median intracranial PFS was 2.8 months (95% CI: 1.0-5.1 months). Median OS was 5.0 months (95% CI: 2.1-5.8 months) with 26% 6 month OS. In this retrospective analysis, WBRT appeared to be well tolerated alongside ICIs. We did not observe any evidence of WBRT related deaths and emergent decompressive surgery was only required in one patient with a prior history of multiple similar surgeries. Further prospective evidence is needed to determine the optimal combination of these two modalities.