Chronic Hematic Cyst of the Right Maxillary Sinus and Orbit Presenting as Proptosis.

A 28-year-old man presented to the emergency room complaining of right proptosis. He also manifested eye pain and facial fullness and redness in this side. Seven years ago, he had right hemifacial gunshot trauma treated with surgical reconstruction. The CT scan showed expansion of the right maxillary sinus due to a hypodense nonenhancing lesion extending to the nasal cavity, masticatory space, and extraconal space of the orbit. MRI was performed showing high signal intensity of the lesion on T2-weighted images indicating a cystic nature. T1-weighted images also demonstrated high signal intensity of the lesion suggesting hemorrhage. At endoscopic maxillary antrostomy, the diagnosis of a chronic hematic cyst was confirmed. Chronic hematic cysts of the orbit should be included in the differential diagnosis of proptosis, especially if there is clinical history of past trauma. Due to the fact that physical examination is nonspecific, radiologic evaluation is useful to confirm the diagnosis and for presurgical planning.

Impressive and durable response to nivolumab in a patient with metastatic type 2 papillary renal cell carcinoma: On-label but without evidence.

Nivolumab is a treatment option for patients with metastatic renal cell carcinoma (RCC) previously treated with targeted antiangiogenic therapy. Papillary renal cell carcinoma (PRCC) comprises 10-15% of RCC cases but non-clear cell subtypes were excluded from the immunotherapy trials. We report the case of a woman with recurrent metastatic PRCC who had an impressive therapeutic response to nivolumab with no significant adverse events. She had previously been treated with sunitinib and pazopanib with no response. She showed a remarkable clinical improvement after only the first 2 immunotherapy cycles and subsequent radiographic studies demonstrated a marked decrease in tumor burden. At present, she continues to show a durable benefit after 8 months of treatment. Her tumor had <1% positivity for PD-L1 staining and a low tumor mutational burden with no actionable mutations on genomic sequencing. Considering its high genetic variation, checkpoint blockade immunotherapies (CBIs) are attractive treatment options in PRCC. This is the third case that reports objective responses of nivolumab in PRCC. We believe our patient's experience supports the inclusion of non-clear cell RCC on clinical trials using CBIs. PD-L1 status and TMB may not serve as predictive biomarkers for response.

Fetal Magnetic Resonance Imaging Findings in Prenatal Zika Virus Infection.

Brain lesions and malformations have been described on ultrasonography of prenatal Zika infection; however, there are scarce reports about fetal magnetic resonance (MR) findings. We report 3 cases of fetuses with confirmed intrauterine Zika virus infection evaluated by ultrasound and fetal MR. Various morphometric measurements were assessed and brain maturation was calculated with the fetal total maturation score. Fetuses with prenatal Zika virus infection showed retardation in brain maturation indexes evaluated by fetal MR. Brain calcifications were demonstrated by neurosonography in all cases, while fetal MR characterized the specific type of cortical development malformation.

Contrast-enhanced T2-FLAIR MR imaging in patients with uveitis.

To report MRI findings which reflect a pathological inflammatory condition of the uveal tract. This study includes single-center retrospective case series of five patients with clinical diagnosis of uveitis. There were 1 male (20 %) and 4 female patients (80 %). The average age was 29.6 years (range 25-38 years). Patients and 50 age-range-matched control subjects were scanned using a 1.5 T scanner. Ten additional control subjects scanned at 3 T were evaluated to have reference images at that high field. All patients (n = 5, 100 %) presented uveal tract enhancement on post-contrast T2-FLAIR fat-suppressed images and only 2 (40 %) had enhancement on T1-weighted images. The enhancement was anterior in 2 (40 %), pan-uveal in 2 (40 %), and posterior in 1 patient (20 %). Two patients (40 %) had unilateral increased vitreous signal on T2-FLAIR. One patient (20 %) had bilateral retrobulbar fat enhancement in both post-contrast T2-FLAIR and T1-weighted images. Post-contrast T2-FLAIR images can reveal abnormal enhancement of the uveal tract and retrobulbar fat as well as increased vitreous signal in patients with uveitis. In our small series, the sensitivity of post-contrast T2-FLAIR was higher than the conventional post-contrast T1-weighted images. Nonetheless, when bilateral uveal tract enhancement is present, there should be discretion before calling uveitis because the finding has been reported in different eye conditions as well as in a small percentage of healthy subjects at 1.5 T. In addition, it should be noted that post-contrast T2-FLAIR enhancement of the uveal tract is a normal finding at 3 T imaging.

Congenital and Acquired Conditions of the Mesial Temporal Lobe: A Pictorial Essay.

PURPOSE: Our goal is to pictorially review a wide spectrum of congenital and acquired conditions affecting the medial aspect of the temporal lobe. CONCLUSION: After completing this article, the reader will have knowledge of the imaging appearance of diverse developmental, malformative, and acquired lesions of the mesial temporal lobe, which will be useful when evaluating pathology in this location.

Alpha-methyl acetyl-coA racemase deficiency. Magnetic resonance imaging findings of three patients with encephalopathy, epilepsy, and stroke-like episodes.

Alpha-methyl acyl-CoA racemase deficiency (AMACRD) is a rare peroxisomal disorder that results in the accumulation of pristanic acid and 16 cases have been reported in the literature. Here, we present three additional patients, two confirmed by genomic study and one suspected. Three siblings who were born to healthy unrelated parents developed recurrent episodes of encephalopathy, seizures, and behavioral disturbances. In all 3, brain MRI showed lesions in the thalami, cerebral peduncles, and mesencephalic tegmentum, as well as brain volume loss. In addition, one patient had a chronic hemispheric infarct and an acute contralateral infarct, and another had a subacute infarct involving multiple vascular territories without abnormalities on MR angiography.

Association between peripheral blood cytopenia and cancer mortality: A race-specific risk factor for cancer death.

BACKGROUND: Cytopenia is associated with cancer through mechanisms including clonal hematopoiesis and chronic inflammation. Cytopenia is more prevalent in Black people but its relationship with racial disparities in cancer mortality is unknown. METHODS: Cytopenia was defined in 19,028 Black and White participants recruited between 2003 and 2007 for the REasons for Geographic and Racial Differences in Stroke cohort, based on age-, sex-, and race-adjusted ranges for blood counts. Cancer death was ascertained from Social Security Death and National Death Indexes. Multivariable Cox models estimated the risk of cancer mortality associated with cytopenia, adjusting for demographics (model1), anemia and cancer risk factors (model2), and socioeconomics (model3). Racial differences in the cytopenia-cancer death association were tested by cross-product interaction terms. RESULTS: Cytopenia was identified in 383 (2%) participants, 250 (65%) White, and 113 (35%) Black people. With median follow-up 11.3 years, 1,224 (6.4%) cancer deaths occurred. Cytopenia was associated with increased risk of cancer mortality in model1 (HR = 1.57, 95%CI 1.15-2.24), model2 (HR = 1.67, 95%CI 1.22-2.30), and model3 (HR = 1.59, 95%CI 1.17-2.17). Participants with cytopenia had twofold increased cumulative incidence of cancer death (13% vs. 6.5%, p < 0.01). Race by cytopenia interaction terms showed higher HR for cancer death in Black compared to White participants: 2.01 versus 1.41 (pinteraction  = 0.016, model1), 2.12 versus 1.45 (pinteraction  = 0.009, model2), and 1.82 versus 1.44 (pinteraction  = 0.04, model3). CONCLUSION: In this large, observational biracial prospective study, cytopenia was a risk factor for cancer death, with stronger association in Black than White people. Though race impacted the association of cytopenia with cancer mortality, cytopenia was not a mediator of the racial disparity in cancer mortality.

Unrelated Allogeneic Stem Cell Transplant in Relapsed/Refractory AML: Widening the Yellow Brick Road.

A sizable proportion of patients with acute myeloid leukemia (AML) fail to achieve remission. Hematopoietic stem cell transplantation (HSCT) is the only intervention with potential of long-term survival. A recent Acute Leukemia Working Party (ALWP)/European Society for Blood and Marrow Transplantation (EBMT) analysis reports substantial posttransplant survival gains for patients with active disease who received transplants. Decreased relapse was the largest contributor to survival, a cause for optimism in this challenging population. See related article by Nagler et al., p. 4258.

Endovascular treatment of penetrating traumatic injuries of the extracranial carotid artery.

PURPOSE: To describe the clinical and angiographic results of endovascular therapy for traumatic injuries of the extracranial carotid artery. MATERIALS AND METHODS: The clinical and angiographic features of 36 traumatic injuries of the carotid artery during a 12-year period were reviewed. There were 35 male patients (97.2%) and 1 female patient (2.8%) with an average age of 28.8 years (range 13-60 years). Of the 36 lesions of the carotid artery, 29 (80.6%) were the result of gunshot injury, and 7 (19.4%) were secondary to stab wounds. In 24 (66.7%) instances, the injury resulted in a pseudoaneurysm; in 7 (19.4%), in an arteriovenous fistula (AVF); in 4 (11.1%), in a dissection; and in 1 (2.8%), in inactive bleeding. All patients were treated with an endovascular approach using different techniques (balloon occlusion, embolization, or stent deployment). RESULTS: Endovascular therapy resulted in documented lesion occlusion in 34 (94.4%) patients. Two patients declined any follow-up postprocedural imaging; however, they have remained asymptomatic. Clinical improvement was documented in 35 (97.2%) patients, and there was one procedure-related complication with fatal consequences. CONCLUSIONS: In this series, endovascular techniques were an effective method of treatment. It was possible to use different endovascular reconstructive techniques or parent artery occlusion depending on the degree of vessel damage, with resolution of clinical symptoms and avoidance of surgery in most cases.

Ruxolitinib in CMML: A Case Study of Innovative Trial Design in a Rare Cancer.

Alternative statistical designs cannot fully mitigate the limitations of traditional clinical trials in rare cancers. Creative study designs that integrate early clinical data and correlative outcomes from concomitant translational and laboratory models to evaluate the efficacy of druggable targets can potentially expedite access to novel therapies for these patients.See related article by Hunter et al., p. 6095.

Delayed complication after embolotherapy of a vertebral arteriovenous fistula: spinal cord ischemia.

Previous reports suggest a generally successful experience with embolotherapy of vertebral arteriovenous fistulas of the neck. However, potential complications do exist, as shown by this report documenting spinal cord ischemia secondary to compromise of a dominant spinal artery arising from the proximal aspect of the right vertebral artery.

Incidence of deep venous thrombosis after temporary joint spanning external fixation for complex lower extremity injuries.

OBJECTIVE: The purpose of this study was to determine the incidence of deep venous thrombosis (DVT) in a prospective protocol of early spanning external fixation with the concurrent use of low-molecular weight heparin (LMWH) in patients with high-energy lower extremity trauma. SETTING: Three level I trauma centers. DESIGN: Prospective observational study. PATIENTS: One hundred thirty-six consecutive patients with 151 complex lower extremity injuries were treated with a protocol of immediate joint spanning external fixation application and LMWH administration within 24 hours of admission. A total of 143 external fixators were applied. Early patient mobilization was encouraged and possible due to the skeletal stability provided by the external fixator. There were 87 men and 49 women with a mean age of 43 years. There were 62 proximal tibia fractures (Orthopaedic Trauma Association [OTA] Fracture Classification 41), 4 tibial shaft fractures (OTA 42), 49 distal tibia-fibula fractures (OTA 43, 44), 14 femur fractures (OTA 32, 33), 8 calcaneus fractures (OTA 73), 10 knee dislocations, and 4 talus fracture dislocations. Forty-eight injuries (32%) were open. INTERVENTION: Temporary joint spanning external fixator placement, LMWH administration, and early mobilization within 24 hours of admission. Duplex ultrasonography of the bilateral lower extremities within 1 day to 3 days before fixator removal and definitive fixation procedure. MAIN OUTCOME MEASUREMENTS: Presence of DVT on duplex ultrasound examination. RESULTS: Duplex ultrasonography was negative for DVT in all but three patients for an incidence of 2.1% (3 of 143 fixators). There were no bleeding complications secondary to the use of LMWH while the temporary external fixator was in place. CONCLUSION: The incidence of DVT in patients treated with a protocol of early joint spanning external fixation and LMWH administration does not exceed historical controls. The early restoration of limb length, alignment, and stability allows early mobilization, which may contribute to the prevention of DVT.

Achilles tendon allograft reconstruction of the fibular collateral ligament and posterolateral corner.

PURPOSE: The purpose of this study was to investigate the functional and clinical outcomes of a consecutive series of patients who underwent fibular collateral ligament (FCL) and posterolateral corner (PLC) reconstruction by use of a single Achilles tendon allograft construct. METHODS: Patients were identified through prospective sports medicine databases at 2 academic institutions. Only patients who had undergone FCL and PLC reconstruction (as opposed to repair) were included. All patients followed a standard postoperative rehabilitation protocol. Various patterns of combined ligament injuries were included and divided into 2 groups (2 ligament v multiligament). Functional and clinical outcomes were assessed by clinical examination, Lysholm scores, and International Knee Documentation Committee subjective scores. Statistical analysis was performed by use of Wilcoxon paired rank-sum tests and multivariate regression. RESULTS: We identified 16 knees in 16 patients, with a minimum of 2 years' follow-up. There were 13 men and 3 women. The mean age was 30 years (range, 19 to 61 years). The mean length of clinical follow-up was 30 months (range, 24 to 75 months). The mean International Knee Documentation Committee subjective scores were 80 points and 80 points (P = .79) in the 2-ligament and multiligament groups, respectively, and the mean Lysholm scores were 90 points and 89 points (P = .96), respectively. Age (P = .41), gender (P = .84), and interval between injury and surgery (P = .72) did not affect the clinical and functional outcomes between the 2 groups. Arthrofibrosis requiring manipulation developed in 1 patient. Residual varus laxity (1+) was noted in 4 patients, none of whom displayed functional instability. No patient has required revision reconstruction to date. CONCLUSIONS: We describe a novel technique that takes into account the main static PLC stabilizers (FCL, popliteofibular ligament, and posterolateral capsule) that has not been previously reported. Our series showed no significant differences in clinical and functional outcomes between 2-ligament and multiligament PLC-based reconstructions. However, given the heterogeneity and small sample size of our study group, it is difficult to draw qualitative conclusions. LEVEL OF EVIDENCE: Level IV, therapeutic case series.

Imaging findings of rhinocerebral mucormycosis.

BACKGROUND AND OBJECTIVES: The purpose of this study was to describe common radiographic patterns that may be useful in predicting the diagnosis of rhinocerebral mucormycosis. METHODS: We retrospectively evaluated the imaging and clinical data of four males and one female, 3 to 72 years old, with rhinocerebral mucormycosis. RESULTS: All the patients presented with sinusitis and ophthalmological symptoms. Most of the patients (80%) had isointense lesions relative to brain in T1-weighted images. The signal intensity in T2-weighted images was more variable, with only one (20%) patient showing hyperintensity. A pattern of anatomic involvement affecting the nasal cavity, maxillary sinus, orbit, and ethmoid cells was consistently observed in all five patients (100%). Our series demonstrated a mortality rate of 60%. CONCLUSION: Progressive and rapid involvement of the cavernous sinus, vascular structures and intracranial contents is the usual evolution of rhinocerebral mucormycosis. In the context of immunosupression, a pattern of nasal cavity, maxillary sinus, ethmoid cells, and orbit inflammatory lesions should prompt the diagnosis of mucormycosis. Multiplanar magnetic resonance imaging shows anatomic involvement, helping in surgery planning. However, the prognosis is grave despite radical surgery and antifungals.

ß-Tubulinopathy caused by a mutation of the TUBB2B gene: magnetic resonance imaging findings of the brain.

Patients with mutations in tubulin-related genes usually present with brain malformations, intellectual disability, epilepsy, microcephaly and ocular abnormalities. In these patients the diagnosis can be suggested by neuroimaging findings. We report a 5-year-old patient with characteristic magnetic resonance imaging findings including malformation of cortical development, fused basal ganglia, large head of the caudate nuclei, absent anterior limbs of the internal capsules, corpus callosum dysgenesis and dysplastic cerebellar vermis. Sequencing of the TUBB2B gene confirmed a heterozygous mutation: c. 260C>A (p. Pro87Gln).

Response of Leptomeningeal Metastases in EGFR-Mutated Non-Small-Cell Lung Cancer to Afatinib in the Absence of Radiotherapy.

Palliative radiotherapy is currently the medical standard of care for non-small-cell lung cancer (NSCLC) patients with symptomatic CNS and leptomeningeal disease. We report the case of a 62-year-old male patient with EGFR mutation (del19+) NSLC with symptomatic lymph node, bone, CNS, and leptomeningeal metastases. Taking into account on one hand the response to tyrosine kinase inhibitors (TKIs) and on the other hand the short- to medium-term side effects of radiotherapy and the lack of timely availability in our healthcare system, the patient was treated with afatinib (40 mg daily) and exhibited a rapid response with improvement of neurological symptoms. The patient presented partial response of extracranial, CNS, and leptomeningeal lesions at 3, 6, and 12 months of treatment, currently completing 16 months of progression-free survival despite presenting mild dermatological and gastrointestinal toxicities. Afatinib is an effective and safe option in patients with NSLC EGFR mutation del19+ with CNS and leptomeningeal compromise avoiding or delaying radiotherapy and its side effects, especially in countries where there is a lack of access to this kind of therapy.

The medial approach for arthroscopic-assisted fixation of lateral tibial plateau fractures: patient selection and mid- to long-term results.

Arthroscopy has been used in the fixation of tibial plateau fractures for more than 20 years. Although not proved in randomized trials, several authors using the arthroscopic technique have demonstrated results comparable to traditional methods, using open reduction and internal fixation with standard buttress plates. Improved rehabilitation, greater range of motion, and a shortened hospital stay for patients who have undergone this technique have also been reported.

Treatment of acute distal femur fractures above a total knee arthroplasty: systematic review of 415 cases (1981-2006).

BACKGROUND: There is no consensus on the best treatment for periprosthetic supracondylar fracture. MATERIAL AND METHODS: We systematically summarized and compared results of different fixation techniques in the management of acute distal femur fractures above a total knee arthroplasty (TKA). Several databases were searched (Medline, Cochrane library, OTA and AAOS abstract databases) and baseline and outcome parameters were abstracted. RESULTS: We extracted data from 29 case series with a total of 415 fractures. The following outcomes were noted: a nonunion rate of 9%, a fixation failure rate of 4%, an infection rate of 3%, and a revision surgery rate of 13%. Retrograde nailing was associated with relative risk reduction (RRR) of 87% (p = 0.01) for developing a nonunion and 70% (p = 0.03) for requiring revision surgery compared to traditional (non-locking) plating methods. Point estimates also suggested risk reductions for locking plates, although these were not statistically significant (57% for nonunion, p = 0.2; 43% for revision surgery, p = 0.23) compared to traditional plating. RRRs for nonunion and revision surgery were also statistically significantly lower for retrograde nailing and locking plates compared to nonoperative treatment. INTERPRETATION: Modern-day treatment methods are superior to conventional treatment options in the management of distal femur fractures above TKAs. The results should be interpreted with caution, due to the lack of randomized controlled trials and the possible selection bias in case series.