A 73-year-old woman presented with one month of progressive pain and swelling in her right breast. She had a past medical history of right breast cancer sixteen years prior, which had been treated with lumpectomy and chemoradiation in Colombia, followed by bilateral textured silicone breast implant placement.
Breast MRI showed that the right breast implant had been deformed by a complex effusion within the fibrous implant capsule, giving it the appearance of rupture on ultrasound. Extending superiorly from the right implant capsule was a mass, measuring up to 8 cm and invading both the chest wall and pleura. While some simple fluid extended across the midline to the medial aspect of the left breast implant, there was no suspicious enhancement to suggest left breast involvement.
A core needle biopsy of the mass was performed. Sections showed neoplastic infiltrate comprised of large malignant cells with round, oval and irregular nuclei, finely stippled chromatin, conspicuous nucleoli and abundant pale, vacuolated cytoplasm. Tumor cells were associated with a rich mixed inflammatory infiltrate comprised of small T and B lymphocytes, many eosinophils and histiocytes. Tumor cells were noted to infiltrate skeletal muscle and other soft tissues. Immunohistochemistry showed diffuse positivity for CD45, CD30, CD43, CD4, MUM-1, and very weak positivity for CD2. The Ki67 proliferative index in tumor cells was high, approaching 90%. Tumor cells were negative for CD79a, PAX5, CD20, CD8, CD56, CD3, EMA, CD34, CD5, ALK-1, pan-keratin (AE1/AE3/PCK26), CK5/6, CK818, CK903, CD31, Factor VIII, CD15, D2-40, EBER (in situ hybridization), CD163, and CD68.
The patient underwent bilateral explantation of her prostheses, followed by full-body staging CT scans. There was a right supraclavicular lymphadenopathy, with three nodes measuring up to 4.1 cm in diameter. The chest wall and axilla showed extensive inflammation with subcutaneous gas. The tumor mass was associated with a right pleural effusion, which was found to contain only benign inflammatory cells and reactive mesothelial cells by pathologic examination. A bone marrow biopsy was negative for ALCL, but incidentally positive for a kappa clonal plasma cell population comprising 20% of the cellularity.
The patient underwent palliative radiation therapy for persistent breast pain. Her case was presented to a multidisciplinary tumor board, and she subsequently began monotherapy with brentuximab vedotin (BV). Her dosing regimen was initiated at 1.8 mg per kilogram of body weight for 18 cycles at three-week intervals. Her treatment was interrupted after her first cycle of BV due to a 12-day hospital admission for multilobular pneumonia, colitis, and associated septic shock. Upon recovery, she resumed BV treatment and a subsequent PET/CT scan after her 5th cycle showed that the chest wall mass and supraclavicular adenopathy had resolved and a large seroma had formed in the anterior chest wall (Figure ). She developed a peripheral neuropathy in her hands and feet after the 9th cycle, which was successfully treated with duloxetine and no dose reduction was required. At the writing of this case report, the patient is 20 months postcompletion of her 18-cycle regimen and subsequent PET/CT scans continue to demonstrate complete remission.


A 25-year old woman was admitted to a psychiatric hospital due to psychotic mania. During the preceding month she had experienced elevated mood, decreased sleep, increased thought pressure, excessive spending, distractibility and expansive ideas. One week prior to admission she had been exposed to a traumatic event by proxy, which triggered intense anxiety, loss of energy and motor agitation. She subsequently started experiencing referential thoughts, which were felt as increasingly convincing, and she developed grandiose delusions raising concern in her family. She was escorted for medical and psychiatric evaluation, and was admitted into a psychiatric hospital for treatment. By the time of admission she had also experienced frequent referential delusions to surrounding events, a Fregoli-delusion, visual illusions and auditory speech hallucinations. Her behaviour and speech were disorganized, and affects changed from elation to agitated anxiety.
According to a structured clinical interview for DSM-IV Axis I disorders, the patient was initially diagnosed as suffering from a manic episode with mood congruent psychotic symptoms. Peroral treatment with aripiprazole 15 mg/day and lorazepam 3 mg/day was started. Rorschach comprehensive system revealed sensory distractibility and distorted reality testing []. Neuropsychological testing indicated above average performance in psychomotor speed, word fluency, social cognition and working memory and average performance in executive functioning and visual learning performance []. Routine clinical and laboratory assessments at admission were normal. There was no evident pathology in brain MRI taken at 29 days from admission. Due to intermittent bouts of low blood pressure, and tachy- or bradycardia, aripirazole was changed to quetiapine 300 mg/day, which was rapidly switched to olanzapine 20 mg/day. Olanzapine was then combined with lithium 300 mg/day due to increasingly volatile affects, novel paranoid delusions and an apparent lack of therapeutic effect.
Elbow rigidity, gait abnormalities and tremors had been absent at admission. After 1 month of antipsychotic treatments olanzapine was discontinued due to difficulties in focusing vision, joint rigidity and shuffling gait. Extrapyramidal and motor symptoms continued to worsen nevertheless. Joint rigidity increased gradually, and at the same time the patients speech became slower, verbal content poor, and affects blunted. The poor clinical response to antipsychotics prompted a neurology consultation. An EEG examination was normal but oligoclonal bands of IgG were found in cerebrospinal fluid (CSF) when blood and CSF samples for autoimmune encephalitis were drawn 8 weeks after admission to psychiatric hospital. Mood stabilizing medications were discontinued as neurologic symptoms worsened with opisthotonus posturing, retrocollis, swallowing difficulties, increased salivation, nocturnal incontinence, autonomic instability and periodic unresponsiveness. Malignant neuroleptic syndrome was previously ruled out in an emergency consultation. In the context of fluctuating psychotic, manic and disorganized behavior, a clinical picture of catatonia started to emerge including mutism, mannerisms and incoherence. The patient received a series of three sessions of electroconvulsive therapy without clinical response.
At this time, 82 days after admission to psychiatric hospital, the serum and CSF sample results for NMDA receptor antibodies were reported as positive. IgG antibodies were detected using indirect immunofluorescence of 1:10 diluted serum or undiluted CSF incubated on cerebellar tissue (Clinical Immunological Laboratory, Prof. Dr. med. Winfried Stöcker, Lübeck, Germany). The patient was transferred to a neurology ward and intravenous (i.v.) methylprednisolone 1 g/day and i.v. immunoglobulins 20 g/day were started and commenced for 5 days. Peroral lorazepam was continued at 4 mg/day, and prednisone was started perorally after i.v. treatments at 50 mg/day. Ovarian teratomas and tumors were excluded with a pelvic MRI and whole body computer tomography. Positive titers of serum NMDA receptor antibodies were still measured at 166 days and 242 days after admission to psychiatric hospital. One infusion of rituximab 500 mg i.v. was given at 220 days due to persistently elevated antibody titers.
The premorbid level of functioning of our patient had been excellent, with a highest global assessment of functioning (GAF) level of 91 during the past year. At the time of admission to psychiatric hospital GAF was 43, and at the start of neurologic rehabilitation GAF was 21. Prior to illness, the patient had been working to complete a university degree after a lifetime history of academic success at international schooling. Prejuvenile and juvenile social adjustments were good, [] and there were no history of neurological illness, chronic medical illness, perinatal complications, head trauma, nor history of psychiatric disorders or substance use disorders in the family.
After 50 days of treatment in the neurological ward the patient was discharged. She received 15 sessions of neuropsychological rehabilitation, speech therapy and psychological counseling during outpatient treatment and further functional recovery was gained. Disregarding subjective concentration difficulties, objectives for neuropsychological recovery were attained. Medical status was assessed again at 290 days after admission to psychiatric hospital. All medications had been discontinued 3 months prior to this assessment. The patients weight and abdominal circumference changed marginally between assessments at 14 and 290 days from 55 kg to 54 kg, and 78 cm to 76 cm respectively. At this time GAF was rated at 70, which was the highest level of functioning during the past year. She had only mild subjective experiences of difficulties in verbal expression, distractibility, concentration difficulties, slowed-down thinking and lack of thought energy. Auditory working memory performance was solely reduced in repeated neuropsychological testing compared to baseline, but remained within normal limits. Positive symptoms, catatonia, joint rigidity or gait disturbances were no longer present. There was no detectable pathology or longitudinal change in conventional brain MRI. Serum NMDAR antibody titers were still positive at 408 days from admission The patient has subsequently completed her university studies, and has been since employed in full time work. All subjective neuropsychological symptoms have disappeared and no symptomatic relapses have occurred during a 946 day follow-up.
We further wanted to explore for subclinical morphological changes between the first and latest brain T1 weighted MRI using Freesurfer image analysis suite () for cortical reconstruction and volumetric segmentation in a longitudinal design []. The patient, and eight healthy female controls of similar age, underwent the same structural MRI scan with the Philips 3 T Ingenuity PET/MR hybrid scanner at baseline, and after 9 months with same imaging sequences. We compared regional volume changes between time points with changes measured from the reference sample. Values deviating from the lower or upper quartile for more than 1.5 times the interquartile range (IQR) was chosen as a cutoff for outlier status from reference population. See Additional file : Supplementary methods for details.
In the baseline MRI scan of this case conventional neuroradiological assessment revealed no major pathology. A more detailed volumetric analysis revealed lower volumes in multiple brain regions, but these did not clearly deviate from the control subjects when corrected for intracranial volume. However, the patient had more volume reduction between baseline and the follow-up time point than any subject in the reference group in primarily frontal cortical regions (Fig. ). Movement artefact was minimal, but slightly more prominent in the patient baseline MRI scan compared to the follow-up scan. Movement during a functional MRI series from the same scan session was higher in baseline for the patient, but there was no association of change in movement to change in volume in the complete study sample.


The patient is a 70-year-old man, with a history of nonvalvular atrial fibrillation (NVAF) with prior stroke, chronic obstructive lung disease, hypertension, diabetes mellitus, chronic foot ulcers, and frequent falls, who initially presented with a fall 5 days after Watchman™ device placement. His history of NVAF was complicated by multiple episodes of syncope, despite various strategies including antiarrhythmic therapy. He was previously on dabigatran which resulted in severe bruising and rivaroxaban which was intolerable due to headaches. After discussion with his cardiologist, a Watchman™ device was placed given his high thrombotic risk and prior complications with oral antithrombotic therapy (CHADS2-VASC2 score of 6 and HAS-BLED score of 4) [, ]. After device placement, he was started on apixaban 5 mg twice daily plus aspirin 81 mg daily, with the plan for continuation for the following 45 days. However, 5 days after device placement, he presented to the emergency room after a fall, with lethargy, fever, and hypotension. He was found to be in septic shock from methicillin-resistant staphylococcus aureus (MRSA) bacteremia. His hemodynamics and mental status initially improved in the intensive care unit (ICU) on vasopressors, stress dose steroids, and broad-spectrum antibiotics, which were subsequently narrowed to intravenous vancomycin. In addition, his apixaban was transitioned to intravenous unfractionated heparin upon presentation to the ICU. In spite of hemodynamic improvement, his mental status worsened. Magnetic resonance imaging (MRI) of the brain revealed multiple acute small punctuate infarcts in the left corona radiata, right occipital cortex, and right frontal deep white matter. These multifocal strokes were thought to be cardioembolic, not septic in origin. Upon further questioning, the patient admitted to missing doses of apixaban post LAOO device placement. A transesophageal echocardiogram (TEE) was obtained; no thrombus in the left atrial appendage was noted, and there were no vegetations or patent foramen ovale. A well-positioned 27 mm Watchman™ device was visualized occluding the LAA orifice with a 1.3 mm paradevice leak, stable from prior. Given high concern for potential seeding of the newly implanted Watchman™ device, a six-week course of intravenous vancomycin plus oral rifampin was recommended by infectious disease consultants as a means of targeting MRSA and additionally preventing biofilm formation and device seeding. This was to be followed by a prolonged suppressive course of oral antibiotics.
The patient improved clinically with recovery of mental status and was deemed ready for transition from unfractionated heparin to an oral anticoagulant. Given the concerns about significant drug interactions with either warfarin or a DOAC with concomitant rifampin, a strong cytochrome (CYP) 3A4 inducer, strong permeability glycoprotein (Pgp) inducer, and moderate CYP2C9 inducer, a shared decision was reached to use weight-based LMWH with enoxaparin to complete the 45 days post Watchman™ device implantation.
The patient refused repeat TEE at 45 days, and anticoagulation therapy was continued with enoxaparin until the device could be evaluated. Two months after Watchman™ device placement, a repeat TEE was performed, revealing improvement in the paradevice leak from 1.3 mm to 1 mm, and the antithrombotic therapy was transitioned to DAPT with aspirin 81 mg daily and clopidogrel 75 mg daily. A repeat brain MRI obtained 4 months post LAOO device placement noted a right cerebellar chronic hemorrhage with numerous susceptibility foci distributed in the bilateral supratentorial and infratentorial compartment predominantly in the periphery of the brain parenchyma. Given the short interval since the prior MRI, the presence of these foci was concerning for microemboli of likely cardiac origin. A repeat TEE did not reveal a thrombogenic focus; however, there was persistence of paradevice leak at 1.3 mm. Anticoagulation therapy with apixaban was resumed, and DAPT was discontinued, due to the persistent device leak. The patient continues to struggle with worsening frequent falls; however, he remains functional at home with continued rehabilitation with physical therapy. He continues to take apixaban, living with the risks of associated bleeding that Watchman™ device placement was intended to reduce.


A 16-year-old female with a history of OCD, MDD, and suicidal thoughts, with no past psychiatric hospitalizations, was brought to the psychiatric emergency department for two months of constant suicidal thoughts. She reported that these thoughts occurred “at night when I go to sleep and when I wake up; I am always wanting to die”. However, she felt strongly that these were not her own thoughts and that she did not actually want to commit suicide. On admission, she endorsed dysphoria, anhedonia, initial insomnia, hopelessness, worthlessness, and feelings of guilt. She also expressed “wanting to strangle the ghost out of her throat” in addition to other bizarre, intrusive suicidal and self-harming thoughts.
She endorsed a history of OCD, diagnosed in high school, and severe hypochondriasis since childhood. She has “always” had fears of being ill or contaminated and engaged in cleaning rituals. Her mother reported that she tended to phrase her obsessions in an “odd” way, for example, stating that she was “scared of breathing the air because it is heavy and dirty and full of sickness” or that she wanted to wash “slimy eels” off her hands after she touched a public handrail. This patient also had a history of depressive symptoms starting at age 7, including periodic irritability, anhedonia, difficulty in concentrating, insomnia, and worthlessness. Despite these difficulties, she was doing well in school and succeeded at playing the violin at a high level. The previous two months had been her first time having such severe, persistent suicidal thoughts.
We began treating this patient with 35 mg fluoxetine q.d. for depression and obsessive thoughts, 300 mg gabapentin q.d. for anxiety, and 150 mg quetiapine q.d. for intrusive and bizarre thoughts. In other words, this was a comprehensive treatment regimen with the goal of addressing our patient's OCD and MDD in addition to her suicidality. Her two-month hospital course was complicated by numerous episodes of heightened anxiety and suicidal thoughts, during which she was “terrified” she was going to “need to kill herself to get this ghost out”. She was worried she would act on this thought, reacting with tearfulness, feelings of hopelessness, difficulty in redirecting her emotions, and inability to concentrate on anything else. Some of her depressive symptoms such as dysphoria and insomnia decreased over her stay, but her suicidality and recurrent thoughts continued to be present. Added to the complex nature of this case was the patient's difficulty in expressing the feelings underlying her suicidal thoughts. She continuously said that these thoughts of strangling herself “came from nowhere”, and she could not pinpoint any correlation of these thoughts with her emotional state, level of distress, or feelings of hopelessness. She sometimes stated: “I want to tie something around my neck and I want to die”, or “life is so pointless”, while continuously denying to staff that she had any desire to commit suicide. The patient's family history was notable for OCD in a maternal grandfather. Of interest, the patient's identical twin sister has not had any psychiatric illnesses.
We sent this patient for neuropsychiatric testing upon recommendation from a child specialist. Neuropsychological examination found deficits in executive functioning, as assessed by a Key Search Task and the Virtual Continuous Performance Test, in addition to decreased verbal cognitive performance as assessed by the Verbal Fluency Test, Verbal Fluency Controlled Oral Word Association Test, and the Digit Span and Letter-Number sequencing subtests of the WAIS-III. The other portions of the neuropsychological exam included the Test of Variables of Attention, to assess attention; the Wechsler Memory Scale, to assess memory; the Rey-Osterrieth Complex Figure task, to assess visuospatial skills; and the Wechsler Adult Intelligence Scale, to assess general cognitive ability. The results of these other tests were all within normal limits.
The results of this testing led firstly to increased understanding by the patient's family of her mental illness; given that she had been high functioning, her parents had not understood why she was so “crazy”. The relatively objective results of this testing alleviated this pressure on our patient from her family. In addition, further treatment focused more on supporting and encouraging expression of emotion, with more time allotted for the patient to think through responses. The patient and her family were advised to increase levels of external support to help with communication and planning upon discharge.
The patient attended a psychiatric day program before returning home to be followed closely as an outpatient for the past several months. During this time, she has received standard-of-care treatment for MDD and OCD, including fluoxetine q.d. for depression, clomipramine q.d. for OCD, and cognitive behavioral therapy. Concurrently, we have worked in particular to improve her executive function and verbal abilities. The patient completes computerized working-memory training tasks daily and is being coached in mindfulness meditation, two methods that have been shown to increase executive functioning in children []. She also has engaged in auditory training exercises to improve verbal processing and memory. Currently, after more than 3 months of this additional training targeted towards neuropsychological deficits in addition to medications and therapy, this patient reports significant improvement. She has more self-efficacy and hope surrounding her mental illness and feels that she “knows what to do” if she feels that way again in the future.


A 36-year-old female was discovered to have an astrocytoma after she presented with new onset seizures. She was started on levetiracetam to control the seizures. Seven weeks later, she presented with break-through seizures while the serum levetiracetam level was therapeutic. Lamotrigine was then added to her medications. Four weeks later, she presented with a rash, right upper quadrant abdominal pain, and fever for four days. She denied any nausea, vomiting, diarrhea, or joint pains. Her medical history was significant for allergies to nuts and pollen. The family history was not significant. She denied having traveled out of the city in the last 18 months. She was a stay-at-home mother of one child.
On physical examination, she was in mild distress due to abdominal pain, febrile to 38 degree Celsius, pulse of 110 per minute, respiratory rate of 18 breaths per min, blood pressure of 124/77 mm Hg in the right upper limb in the supine position, and oxygen saturation of 98% on room air. A reticulated macular erythematous rash was noted on the upper extremities (Figure ) and trunk (Figure ). The head and neck exam did not demonstrate any localized swelling, lymphadenopathy, or icterus. There was no oral ulceration or conjunctival injection. Cardiac and lung exam were within normal limits. The abdominal exam revealed right upper quadrant tenderness with Murphy’s sign. Initial lab data demonstrated an eosinophil count of 4 x 10^5/ml, elevated white cell count, alanine aminotransferase (ALT) of 1231 units/L, and aspartate aminotransferase (AST) of 1026 units/L. Alkaline phosphatase was 362 units/L. Lamotrigine was stopped as DRESS was considered. Over the next 48 hours. there was no improvement, against expectations. An ultrasound of the abdomen followed by magnetic resonance cholangiopancreatography confirmed the presence of acalculous cholecystitis. Antibiotics were administered for cholecystitis. Two days later, her abdominal pain subsided but she remained febrile (T-max of 39.3 Deg C). By now, the reticulated erythema spread to her face and lower extremities (Figure ). Her face became edematous, and she developed enlarged salivary glands and lower lip edema (Figure ). Her pharynx was noted to be erythematous and her tonsils were enlarged and covered with exudates. Cervical and submandibular lymphadenopathy developed as well. At this time, an alternate diagnosis was entertained while she did not improve over the next one week. She was immune to hepatitis A and B. Antibody to hepatitis C was negative. The antinuclear antibody, anti-smooth muscle antibody, antimitochondrial antibody, and liver-kidney microsomal assay were negative. Serum ceruloplasmin was within normal limits. Serum toxicology showed a salicylate and acetaminophen level within normal limits. Urine toxicology was negative for substances of abuse. Serum titers for rubella, rubeola, mumps, Epstein Barr virus, cytomegalovirus, varicella zoster, and human herpesvirus-6 were negative. Anti-streptococcal titers, throat culture for Streptococcus, and diphtheria culture were sent as well and reported no growth or negative growth. The cell count peripheral smear did not demonstrate mononucleosis or atypical lymphocytosis. A chest radiograph demonstrated a newly developed right-sided pleural effusion without evidence of consolidation. A skin biopsy was done, which confirmed perivascular dermatitis such as that seen in drug hypersensitivity (Figures -).
About 10 days after presentation, the levetiracetam was discontinued because of the lack of improvement in symptoms with the cessation of lamotrigine. With that, she began to improve gradually with the disappearance of the skin rash over the next few days, resolution of the facial and the lymph node swelling, as well as a return of transaminases to the baseline over the next few weeks.


A male patient aged 49 years with no previous medical history presented with neck pain from a foreign object. The patient was accidentally struck by a projectile from a nail gun on a construction site. The patient complained of pain along the left side of the neck and inability to swallow. On initial evaluation, patient was neurologically intact with absent Horner's syndrome. There were no signs of active bleeding or hemodynamic instability. The head of the nail was visible behind and above the angle of the mandible at the level of the skin - neck zone 3 (Figure , Table )
The point of the nail was not visible in the oral cavity but was palpable along the oral mucosa along the left side of the oropharynx. The patient was intubated and sedated for airway protection without complications in an outlying emergency room (ER) and triaged to the local Level One trauma center for further management. Upon arrival to our trauma center, a computed tomography (CT) angiography of the neck was obtained which demonstrated the nail traversing in very close proximity to the left internal carotid artery close to the skull base with preserved flow proximal and distal to the nail. There was no active contrast extravasation or definitive presence of a pseudoaneurysm although the evaluation was limited by the beam-hardening artifact from the nail (Figure ).
The patient was emergently transported to the interventional radiology suite to determine the status of the left carotid artery and possible endovascular repair. Left common carotid angiography in the neck demonstrated preserved flow through the left internal carotid artery but significant vessel narrowing at the level of the nail (Figure ).
Further investigation with three-dimensional (3D) rotational angiography demonstrated small areas of contrast stagnation proximal and distal to the nail with a small dissection flap proximal to the nail, consistent with at least a partial vessel injury (Figure ).
The intracranial left anterior circulation imaged normally without abnormal vessel dropout. Upon discussion among trauma surgery, oral and maxillofacial surgery and neurointerventional teams, the decision was made to proceed with endovascular repair in conjunction with nail removal. The patient was given 600 mg of clopidogrel and 325 mg of aspirin via orogastric tube and was heparinized to an activated clotting time (ACT) greater than 250. The short 6F sheath was exchanged for 6F Terumo Destination sheath (Terumo Medical, Somerset, New Jersey, USA) and positioned in the distal left common carotid artery. The injury site was crossed with a Synchro-2 0.014 microwire (Stryker, Fremont, California, USA) under the fluoroscopic roadmap. A 5 x 25 mm Gore Viabahn covered stent (W.L. Gore & Associates, Flagstaff, Arizona, USA) was positioned across the level of injury. The nail was removed by gentle traction by trauma surgery with the simultaneous deployment of the Gore Viabahn covered stent into the left internal carotid artery spanning the injured segment. Follow-up angiography demonstrated excellent flow through the stent but with active extravasation secondary to a proximal endoleak related to incomplete apposition of the proximal stent to the vessel wall (Figure ).
At this time, the anaesthesia team noted blood pooling in the oropharynx. We introduced a 5 x 30 mm Aviator Plus balloon (Cordis, Milpitas, California, USA) and performed angioplasty of the proximal end of the stent. Follow-up angiography demonstrated no active extravasation and excellent flow through the stent (Figure ).
Cerebral angiography demonstrated no abnormal vessel dropout in the left anterior circulation. There was no further bleeding in the oropharynx or through the tract. The patient was transferred to intensive care unit (ICU) for further care.
On a postoperative day one, the patient was extubated without incident. No neurological deficits were noted. The patient was continued on 325 mg of Aspirin and 75 mg of clopidogrel daily. The patient was cleared for an oral diet. Follow-up CT angiogram demonstrated patency of the left internal carotid stent and no pseudoaneurysm formation (Figure ). The patient did receive tetanus boosters and antibiotics as part of the initial management to prevent secondary infection. The patient was discharged to home on hospital day five. We planned to follow up the patient with CT angiogram at six weeks after the procedure. Unfortunately, we were not able to obtain the imaging as the patient was out of state and was uninsured. Per phone conversation the patient was doing very well and did not report any symptoms consistent with stroke or transient ischemic attack (TIA) at two months after the procedure. The patient stopped his clopidogrel one week after the procedure due to financial reasons but continues on the aspirin regimen.


A 67-year-old man was admitted to our hospital with intermittent headache for 10 days and hypomnesis for a week. The patient had no B symptoms but was generally in poor condition (Eastern Cooperative Oncology Group (ECOG) performance status =2). Neuroimaging revealed a homogeneously enhancing mass with peripheral signal hyperintensity on the right temporal. Serum lactate dehydrogenase (LDH) level (630 U/L, reference range: 135–215 U/L) and cerebrospinal fluid (CSF) protein concentration (954 mg/L, reference range: 150–450 mg/L) were elevated in the patient. Involvement of deep structures of the brain was not found. The International Extranodal Lymphoma Study Group (IELSG) score [] was 4 and belonged to the high-risk group. This patient received high-dose methotrexate (HD-MTX) (3.5 g/m2) and the concomitant chemotherapy drug idarubicin after surgery. The patient achieved a partial remission according to the response criteria [] after therapy but died 5 months after the onset of disease.


A 54-year-old man was admitted to our hospital with a history of right limb weakness for 1 year. The patient had no B symptoms, and the general condition was good (ECOG performance status =0). Neuroimaging showed a noncalcified homogeneously enhancing mass with peripheral signal hyperintensity around the ventricles with associated edema and multiple damaged parts. Serum LDH level (375 U/L) and CSF protein concentration (625 mg/L) were elevated. Involvement of deep structures of the brain was found, and the IELSG score was 4 and belonged to the high-risk group. This patient received HD-MTX (3.5 g/m2) and the concomitant chemotherapy drug cytarabine after surgery followed by consolidative whole-brain radiotherapy (40 Gy). He achieved a PR after therapy but died 8 months after diagnosis.


A 55-year-old woman was admitted to our hospital with history of dizzy and headache for 1 year. The patient had no B symptoms and her general condition was poor (ECOG performance status =3). Neuroimaging revealed a homogeneously enhancing mass with peripheral signal hyperintensity on the interventricular septum and the corpus callosum with associated obstructive hydrocephalus. Serum LDH level (780 U/L) and CSF protein concentration (863 mg/L) were elevated. Involvement of deep structures of the brain was found, and the IELSG score was 4 and belonged to the high-risk group. This patient received HD-MTX (3.5 g/m2) and the concomitant chemotherapy drug idarubicin after surgery. She had progressive disease after therapy and died within 2 months of diagnosis.


A 66-year-old male was previously admitted to our hospital with a subarachnoid hemorrhage (SAH) secondary to a ruptured anterior communicating artery (Acom) aneurysm. He underwent coil embolization of the aneurysm, which initially showed no residual filling; however, subsequent recanalization of a posterior 2-mm lobe was detected on one-year follow-up (Figure ).
The decision was made to retreat the patient.
The right femoral artery was accessed using the standard fashion. Next, with the catheter in the left internal carotid artery (ICA), a rotational angiogram was performed, revealing an aneurysm remnant, 2 x 1.9 mm in size, with one coil loop (Figure ).
A "Y" stent-assisted coil embolization was utilized for treatment. In the first patient, the operator deemed that there is no safe corridor or technique to place the coils in the recurrent aneurysm. In the second case, the initial case was initially thought that it would be stent assisted coil embolization. The coil was attempted to be placed; however, it kept prolapsing so the decision was to leave the stents.
The diagnostic catheter was replaced with a guiding catheter. A microcatheter was advanced over a microwire into the intracranial ICA, then the left A1 segment and finally across the Acom to the right A2. Then, the wire was removed, and a 2.5 x 23-mm LVIS® Jr. braided coil-assist stent was placed from the right A2 to the left A1. Subsequently, the stent pusher wire was removed and the microwire was replaced and advanced to the ipsilateral A2 segment. With the catheter in this position, the wire was removed, and an LVIS® Jr. 2.5 x 17-mm stent was placed. Follow-up angiographic runs revealed no filling of the aneurysm at this point (Figure ).
A femoral artery closure device was deployed at the end of the case.
Postoperatively, he was transferred to the neurological intensive care unit (ICU) and was continued on aspirin and clopidogrel. He was neurologically intact, and no aneurysm filling was noted on six-month follow-up (Figure ).


A 41-year-old female with a history of a ruptured Acom aneurysm treated with balloon-assisted coiling, presented with worsening headaches and a new Acom aneurysm was found in her three-year follow-up. She was noted to have intermittent residual right-sided weakness after her initial subarachnoid hemorrhage. Her home medications were aspirin and clopidogrel. Surgical and non-surgical options were discussed with the patient, including observation, and she opted for endovascular treatment of her new aneurysm.
The patient was brought to the neurosurgical operative suite. She was placed in the supine position on the operating table; both legs were prepped and draped in sterile fashion. The left common carotid and left ICA were sequentially catheterized, contrast injected, and a rotational digitally subtracted angiogram (DSA) performed, which revealed a wide-necked Acom aneurysm (Figure ), a suitable candidate for stent-assisted coil embolization.
At this point, the diagnostic catheter was swapped out for a guiding catheter and a Headway® microcatheter, (MicroVention Inc., Aliso Viejo, CA, USA) over a microwire, was advanced from the left A1 segment into the right A2. Next, a microcatheter was advanced over a microwire into the aneurysm. An attempt was made to place a 2 mm x 3 mm coil, but it kept prolapsing into the parent artery; therefore, a 2.5 mm x 23 mm LVIS® Jr. stent was placed into the right anterior cerebral artery, extending into the left A1. Again, coil placement was attempted, but it kept herniating this time into the left A2. Therefore, the Headway® microcatheter was reinserted into the guiding catheter and advanced into the A1 on the left and then the A2 on the left. A second 2.5 mm x 23 mm LVIS® Jr. stent was placed.
At this point, an attempt was made to place a 2 mm x 3 mm coil, but it kept prolapsing and the location of these prolapsed loops was unclear, therefore the coil was withdrawn. Next, we tried a 1.5 mm x 3 mm coil and had the same problem. Finally, we tried a 1.5 mm x 2 mm and encountered the same issue. While attempting to adjust this coil, the microcatheter came out of the aneurysm. We were unable to cross the two stents to return to the aneurysm.
A final angiographic run revealed slow filling in bilateral anterior cerebral arteries. Therefore, an intra-arterial dose of abciximab was given, and the patient was started on IV abciximab drip. Follow-up angiogram showed significant improvement, although not back to baseline, and the procedure was terminated.
Postoperatively, the patient was maintained on aspirin and ticagrelor. On postoperative day 2, she complained of worsening unrelenting headache, which she rated as 8/10 in intensity. Computed tomography (CT) scan revealed a 4.0 x 3.5 cm left frontal lobe acute intraparenchymal hemorrhage. She was managed conservatively in the neurologic ICU and made a significant recovery. She was discharged home on postoperative day 7, neurologically intact. A six-month post-operative angiogram showed complete obliteration of the aneurysm (Figure ).


Patient #1
The patient is a 70-year-old male with type-2 diabetes mellitus (DM) and chronic kidney disease (CKD) with a history of chronic abdominal discomfort who presented with a missed posterior congenital right Bochdalek hernia. Abdominal computed tomography (CT) revealed a posterior right diaphragmatic hernia containing loops of non-incarcerated bowel without obstruction. The operation was performed via a midline laparotomy and bowel loops were pulled from the hernia inadvertently creating a small enterotomy which was repaired primarily. A pleural sac covering the contents was identified but not opened. The hernia orifice was 8 x 6 cm with a muscular posterior rim. After reducing the orifice to 4 x 4 cm using sutures at the angles, the GORE® BIO-A® Tissue Reinforcement patch was used to close the defect without tension using multiple interrupted nonabsorbable 4/0 prolene sutures. The patient’s associated comorbidities and the enterotomy that was encountered increased the likelihood of surgical infection which led us to use the aforementioned technique and tissue reinforcement patch.
Patient #2
The patient is a 6-year-old male with no significant past medical history who was admitted with a strangulated Morgagni hernia, sepsis and reactionary pericardial effusion. A midline laparotomy was performed and bowel was pulled out of the hernial sac. The ischemic small bowel was resected with the primary end to end anastomosis. The rim of the defect was identified and dissected circumferentially. The defect was repaired using a GORE® BIO-A® Tissue Reinforcement patch with the same technique as mentioned above. In the setting of an infected field, this patient was deemed to be at increased risk of infection and therefore a good candidate for GORE® BIO-A® Tissue Reinforcement patch.
Patient #3
The patient is a 15-year-old male who presented with the recurrence of a posterior left Bochdalek hernia previously repaired ten years ago via laparotomy. The previous repair was done by direct suturing without patch placement. A left posterior lateral thoracotomy was done to avoid abdominal adhesions. The bowel was released from lung and pleura and the hernia rim was dissected and identified. After reducing bowel, two serosal tears were identified which were closed primarily. The 6 x 5-cm hernia was reduced to 3 x 3 cm without tension and the residual defect was closed using GORE® BIO-A® Tissue Reinforcement patch using the same technique as mentioned above. Multiple operations, which are associated with bowel perforation, placed this patient at high risk for surgical infection.


A 9 day old 3.5 kg boy was referred to our tertiary care hospital with diagnosed of PRS. Other than atrial septal defect (ASD), aspiration pneumonia and unilateral complete cleft palate with a maximum width of about 0.8 cm. There are no cleft lip or alveolar cleft or any other comorbidity. Upon admission, he presented with cyanosis with venous carbon dioxide pressure (PvCO2) 87.8 mmHg, multiple bedside direct laryngoscopy and GlideScope (UE Medical, China) attempts were made however none were successful. His saturation was improved to 95% by facial mask. The next morning he had thin sliced Computed Tomography (CT, Philips) with craniofacial as well as airway reconstruction (Fig. a, b).
The same night he deteriorated again. We attempted intubation with GlideScope which revealed grade 4 view. Next we tried a blind intubation with endotracheal tube loaded with stylet, however, this failed as well. Then we tried size 1 laryngeal mask airway (LMA, Well Lead Medical, China), however, we felt the LMA was blocked by an occupying lesion at the left side of tongue’s base so we decided not to force it through for fear it might further aggravate his airway. His respiratory distress was improved after we placed a NPA and saturation returned to 100%.
The third morning he was brought to operating room for MDO placement. After giving Penehyclidine to dry his secretion, we slowed dialed Sevoflurane to 6% then back to 3% to maintain his spontaneous breathing. Placement of a glidescope revealed no identifiable glottic structures. Fiberoptic scope (Olympus, Japan) revealed the epiglottis lying on the posterior pharynx, which could not be maneuvered beneath. Size 1 LMA and lighted wand (CLARUS Medical, MN) cannot be placed in the right place, multiple attempts with high-tech equipment failed to establish his airway. Since NPA could maintain his saturation, we decided to abort the procedure. Upon arrival in surgery intensive care unit (SICU), his PvCO2 was 119.4 mmHg. A TLA procedure was performed with sedation. The fourth night his PvCO2 was elevated to 183.8 mmHg. We reviewed his airway CT again with a different radiologist. We found he had large lesion with size of 21.1 mm X 11.7 mm occupying his base of tongue extending from left all the way to middle. Most likely it was thyroglossal cyst per the second radiologist. (Fig. a, b).
Knowing his hypercarbia could get even worse, on day 5 we brought him back to the operating room. After inducing patient with ketamine and sevoflurane, operator gloved then advanced nondominant middle finger along the tongue, once patient’s epiglottis was touched, middle finger was bent slightly to lift epiglottis, dominant hand then passed the lubricated and bent endotracheal tube based on 3D reconstruction right next to the middle finger into his trachea. Tube position was confirmed with capnography with endotidal CO2 of 120 mmHg. Once airway secured, patient had MDO procedure without any problem. He was sent back to SICU and successfully extubated there on postoperative day 5.


A 15-year-old boy was operated for traumatic endophthalmitis in the right eye in April 2015 with vitrectomy, lensectomy, and intravitreal antibiotics. After three months, he developed inferior retinal detachment for which a repeat vitrectomy, relaxing retinotomy, and SiO (1,000 centistoke viscosity) tamponade were performed. Both procedures were performed through 20 gauge sclerostomies which were sutured with 7-0 polyglactin sutures. The wounds were watertight at the end of the surgeries. He experienced persistent redness and foreign body sensation following the second procedure. He gradually developed outward deviation of the right eye and binocular horizontal diplopia for which he was referred to the strabismus clinic in October 2016. At this visit, his best corrected distance visual acuity in the right eye was 0.8 LogMAR (with +10.25 D contact lens) and near vision was N36 (with +3.0 D add). The conjunctiva was diffusely congested and boggy. Multiple shiny subconjunctival nodules of variable sizes were seen in all four quadrants. They were predominant in the superotemporal and inferotemporal quadrants (Figure 1a ). Other clinical findings were linear corneal scar superiorly, aphakia, attached retina at the posterior pole, and peripherally detached retina. The intraocular pressure was 10 mm Hg. Orthoptic evaluation revealed exotropia of 25 ΔD with unrestricted ocular movements. A diagnosis of sensory exotropia with sub-conjunctival cysts was made. Lateral rectus (LR) recession and medial rectus (MR) resection were planned which were delayed till May 2017 due to the patient’s unwillingness for further surgery.
During the surgery, LR muscle was approached under local anesthesia using a paralimbal conjunctival incision. The underlying sclera did not show any thinning or melt. Multiple clear thin-walled oil filled uni- and multi-loculated cysts, ranging from pin-head size to 5 mm, extending up to at least 14 mm posterior to limbus, were encountered below the conjunctiva. They were firmly adherent to the surrounding structures and ruptured on attempted dissection. Numerous cysts were found embedded within the tenon’s capsule, the muscle sheath, and under the LR muscle (Figure 2a–d ). This had rendered the tenon’s fascia thick and fibrosed (Figure 2e ). Dissection led to profuse bleeding making visualization and delineation of the muscle onerous and time consuming. The muscle sheath and portion of the tenon’s fascia surrounding the muscle had to be resected to remove the cysts and facilitate isolation of the muscle (Figure 2f ). The large cysts were removed by rupturing or resecting them. The SiO released from ruptured cysts was thoroughly washed from the ocular surface. After securing the LR with 6-0 polyglactin suture, it was recessed 6.5 mm using a sliding noose adjustable suture technique (Figure 2g ). Thereafter, medial rectus muscle resection (4.5 mm) was performed (Figure 2h ). The SiO cysts were sparse in the medial, superior and inferior quadrants.
In the immediate postoperative period, the patient was orthotropic and did not require adjustment. The conjunctival inflammation gradually reduced (Figure 1b–d ) eliminating the ocular discomfort. At the last follow-up thirteen months later, his BCVA was 0.8 LogMAR and central retina was attached under the silicone oil. He was diplopia free and was satisfied with the cosmesis.


A 47-year-old male who complained of upper abdominal pain and vomiting was referred to our hospital. He was a heavy drinker and had a past history of hospitalization for alcoholic chronic pancreatitis. Laboratory data revealed elevated levels of amylase (245 IU/L), CRP (14.99 mg/dl), and white blood cell count (14900/μL). Plain abdominal computed tomography (CT) showed a cystic lesion of 7 cm in size in the lumen near the second part of the duodenum. The cystic lesion showed high density inside. The pancreas was slightly enlarged, and the main pancreatic duct was dilated. Calcifications were seen in the uncus of the pancreas (Fig. a). Gastrointestinal endoscopy revealed that the lumen of the duodenum was deformed by a submucosal tumor-like mass and the endoscope could not pass through it (Fig. b). However, active bleeding was not seen in the lumen of the duodenum. A submucosal tumor or hematoma of the duodenum or a pancreatic pseudocyst associated with chronic pancreatitis was suspected. On the fourth day of hospitalization, his hemoglobin level had decreased from 14.0 to 11.1 g/dl. Contrast-enhanced CT demonstrated a high-density spot on the wall of the cystic lesion (Fig. c). A pancreatic pseudocyst complicated with intracystic hemorrhage was preliminary considered. Angiography was immediately performed, and a pseudoaneurysm was identified in the branch of the anterior superior pancreaticoduodenal artery (ASPDA) (Fig. d). The pseudoaneurysm was successfully treated with transcatheter arterial embolization (TAE). Anemia did not progress after that. Upper gastrointestinal series demonstrated a filling defect in the duodenum, while the inside of the cystic lesion was not contrasted (Fig. a). Magnetic resonance cholangiopancreatography (MRCP) was performed but did not show a communication between the cyst and the pancreatic and biliary ducts. Follow-up CT on the 27th day after TAE showed that the cyst had decreased in size to 2 cm and obstruction of the duodenum was gradually improved (Fig. b). Surgical treatment was considered for the pancreatic pseudocyst with intracystic hemorrhage. However, he refused an operation and was discharged on the 34th day after TAE.
Two years later, abdominal pain and vomiting recurred. The cyst was enlarged again, and CT showed that it contained high-density fluid. Recurrence of a pancreatic pseudocyst with intracystic hemorrhage was suspected because of anemia progression. TAE was performed in the branches of the ASPDA and posterior superior pancreaticoduodenal artery (PSPDA). After TAE, the size of the cyst decreased and symptoms were relieved. However, the same symptoms recurred 2 months later. We obtained informed consent for surgical treatment, and we performed subtotal stomach-preserving pancreatoduodenectomy (SSPPD). Intraoperatively, severe inflammatory adhesion was noted around the pancreas head and the border between the pancreas and the cystic lesion was unclear. Macroscopically, a cystic mass of 5 cm in size was adjacent to the second part of the duodenum on the pancreas side and was close to the ampulla (Fig. a). A pinhole-sized communication was identified between the cyst and the duodenum lumen. Microscopically, the cyst was filled with mucus and the wall of the cyst was composed of gastric mucosa and shared a common proper muscle layer with the duodenum. Chronic ulcers and erosions were seen in the cyst (Fig. b, c). Ectopic gastric mucosa was observed in non-ulcerative region (Fig. d). Based on these findings, a diagnosis of duodenal duplication cyst was made. The patient’s postoperative course was uneventful, and he was discharged on the 30th day after the operation.


63-year-old woman who had suffered from the right medial knee pain for 5 years and was not responsive to conservative treatment was admitted to our clinics. 30° varus-valgus stress X-ray indicated that the patient had an intact MCL and LCL. After the detailed physical examination and reviewing of X-ray images, it was decided that UKR would be the most suitable option for the patient with anteromedial knee osteoarthritis. After spinal anesthesia application and sedation, the UKR surgery was performed with a standard minimal invasive midline vertical incision and a medial parapatellar approach; the patella was removed laterally but not dislocated or everted. The patient received a medial partial knee implant with a mobile-bearing insert (medium size with 4 mm thickness; Oxford®, Zimmer Biomet Inc., Warsaw, IN, USA). Following the UKR surgery (), weight bearing was allowed as tolerated by the patient and a standard postoperative physiotherapy was started on the first postoperative day. The patient was discharged at postop 2nd day when she met the following criteria: independent ability to get dressed, to get in and out of the bed, and to sit and rise from a chair/toilet; independence in personal care; and mobilization with crutches. After discharge, a home-based exercise program was given to the patient. At postoperative follow-up, our patient acquired a full knee RoM in the postop 1st month and returned to independent daily activities without any external support in the postop 3rd month.
At postoperative 1st year after first UKR application, the patient fell down while getting on a public bus; this caused that the right knee of the patient was exposed to the valgus force vector. After that moment, the patient heard a pop sound and felt an incredible pain that prohibited the flexion and/or extension of the medial side of the right knee. And then she was admitted to our emergency department. The first evaluation was performed, and the patient was diagnosed with a grade 3 MCL rupture and the UKR insert dislocation (). Having completed the preoperative preparations, the patient was operated on the same day. After anesthetic administration, a surgery with a standard minimal invasive midline vertical incision and a medial parapatellar approach (to a previous incision site) was performed to change the mobile-bearing insert with the same size (medium-sized mobile-bearing insert with 4 mm thickness; Oxford®, Zimmer Biomet Inc., Warsaw, IN, USA). After having changed the mobile-bearing insert, the MCL structures were repaired and anchored to its femoral origin with a 5 mm titanium anchor. Following the surgery, weight bearing and full RoM with a hinged knee brace were allowed as tolerated by patient and a standard postoperative physiotherapy was started on the first postoperative day. Crutches were recommended for 2 to 3 weeks to enable the patient to regain a normal gait. The brace was used continuously for 4 weeks and thereafter during the day for 2 weeks. After the physiotherapy program administration, the patient was discharged at postop 1st day.
The patients were evaluated regarding pain intensity (Numeric Pain Rating Scale (NPRS)), active range of motion (RoM), and quality of life (Short-Form 12 Health Survey (SF-12 Health Survey)). Functional capacity was evaluated using the Iowa Level of Assistance Scale (ILAS), Iowa Ambulation Velocity Scale (IAVS), Hospital for Special Surgery (HSS) knee score, and Timed Up and Go (TUG) test. Rehabilitation program and outcome evaluation were conducted by one clinical physiotherapist at preoperative period (before the first UKR application), at discharge (postop 2nd day after the first UKR surgery), and at postop 2nd year (after 2 years from the MCL repair and the insert change). The evaluation results are shown in .


A three-year-old male presented to the emergency department (ED) with the chief complaint of one episode of hemoptysis that occurred just prior to arrival. His adopted mother stated that he had cold-like symptoms for the past few days prior to arrival, and on the day of arrival, he began to cough up blood. She brought the blood-tinged rag with her to the ED. He had vomited the night prior, as well as on the morning prior to arrival, and had diarrhea during that same time frame. His adopted mother said that the diarrhea and vomit were not blood-tinged. There had been no change to his urine output. She stated that he had a fever the night prior, as well as on the morning of presentation, with a maximum temperature of 101º F. His adopted mother said that he attends daycare, and that multiple children in his daycare had recently come down with respiratory syncytial virus (RSV).
His adopted mother stated that he was born full term with no complications. She also said that he had multiple bronchitis infections since his adoption, which she stated was at a few months of age. According to her, he is up to date on all vaccinations. Due to his adoption status, his family history was unknown.
His temperature on arrival to the ED was 98.7º F. He had a pulse rate of 131 beats per minute, a respiratory rate of 22 breaths per minute, and a blood pressure of 89/60. Physical exam revealed the presence of clear rhinorrhea as well as diffuse crackles and expiratory wheezing in all lung quadrants.
A complete blood count and comprehensive metabolic panel were all within normal limits. Chest radiographs in two views were performed, and it was determined that no acute lung abnormalities or pulmonary infiltrates were present (Figures -). A hemoccult test was done on the blood-tinged rag which confirmed that the substance was blood. It was estimated that the total amount of blood on the rag was significantly less than 5 mL. This placed him in the category of scant hemoptysis.
Throughout his stay in the ED, he did not have any additional episodes of hemoptysis. An albuterol breathing treatment was performed, which he tolerated well. On re-auscultation of his lungs, the crackles and expiratory wheezing had resolved. Given his afebrile status and prior exposure to RSV, it was determined that he likely contracted RSV from his classmates. He remained stable throughout his entire stay and was discharged home with instructions to follow up with his pediatrician. It was also recommended that they follow up with a pediatric pulmonologist for evaluation of his extensive history of bronchial infections.


A 42-year-old Hispanic man with a history of tattoos covering 80% of his body surface area and regularly shaven legs presented for evaluation of an asymptomatic solitary leg lesion within a tattoo. Further history revealed that his leg tattoos were done in his early twenties with a touch-up three years prior. Subsequently, he developed a papule that was progressively increasing in size within the area that received additional tattoo pigment during the touch-up.
Cutaneous examination revealed a 5 x 5 mm dermal papule within the green tattoo pigmented area on the left pretibial leg (Figure ). There was a depression in the center of the papule. An excisional punch biopsy was performed.
Microscopic examination showed bone (with Haversian canals) in the upper dermis that was perforating through the overlying epidermis. The site of perforation demonstrated a keratin-plugged crater and extension of the adjacent hyperplastic epidermis into the dermis. A proliferation of small endothelial-lined vessels and a predominantly lymphocytic inflammatory infiltrate was located in the dermis beneath the site of perforation and surrounding the bone. Also present in the dermis, adjacent to the bone, was pigment from his green tattoo which appears as small black particles (Figure ).
He had no recurrence at six-month follow-up.


In 2013, a 72-year-old Caucasian male patient with extensive history of sun exposure
presented with right eye pain and associated forehead dysesthesias. He was noted on
examination to have a palpable 3 mm dermal nodule within the right lateral eyebrow.
Biopsy revealed keratin-positive SCSCC with PNI. Staging computed tomography scans
revealed no evidence of metastasis. Mohs surgery performed in February 2014
confirmed a stage 1 lesion without extension to the epidermis and negative surgical
margins.
In August 2014, he developed double vision and right upper facial pain. He was found
to have a right cranial nerve (CN) VI palsy and partial CN III palsy. The etiology
of the right facial pain was not clear at the time. Magnetic resonance imaging (MRI)
of brain and computed tomography imaging in September 2014 were negative; however,
his symptoms progressively worsened. Repeat MRI of brain in February of 2015
revealed a new 0.6 × 0.5 cm right Meckel’s cave lesion. Due to the location and the
size of his central nervous system (CNS) lesion, it was not deemed safe for biopsy
by the neurosurgical team. Given the anatomical distribution and symptoms reported
by the patient, it was assumed that the SCSCC previously resected from the right
eyebrow had tracked along the VI branch of CN V through the cavernous sinus to the
right Meckel’s cave resulting in additional cranial neuropathies of CN III and CN
VI. The workup for other malignancies was negative. The patient received external
beam radiation to the area of the original SCSCC and brain. The radiation resulted
in significant improvement in the right upper facial pain. In February 2016, he
developed left arm weakness and underwent another surveillance MRI of brain that
showed a new extensive T2/FLAIR hyperintensity centered in the right brainstem with
a 1.2 cm enhancing lesion in the right pons. He underwent gamma knife therapy that
was completed in March 2016 with no recurrence of disease through June 2016.
However, in September 2016, he developed recurrent left upper and new lower sided
weakness and gait instability. Physical and occupational therapy evaluations at the
time showed profound left-sided leg weakness and foot drop requiring bracing and a
cane for ambulation. A repeat MRI revealed changes assumed to be
radiation-associated necrosis, and he was treated with pulse dose steroids. In
January 2017, he was admitted for profound weakness, despite MRI showing stable
disease. In May 2017, he presented with vertigo and left eye abduction deficits and
worsening left-sided weakness. An MRI showed interval increase in the enhancement of
the V3 portion of the right trigeminal nerve extending into the foramen ovale and
destruction of the clivus on the right side with involvement of the right sixth CN
().
At this point, the patient was no longer a candidate for any further radiation
treatments given extensive prior treatment. He was considered for the SWOG S1609
DART trial (Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors), but the risks
of acquiring a biopsy for study enrollment from the brainstem lesion were felt to be
too great. The tumor specimen originally resected from the right eyebrow in 2013 was
sent for further profiling and found to have retained expression of PMS-2, MLH-1,
MSH-6, and MSH-2 PDL1, therefore was unlikely to be microsatellite instability high.
However, the PDL1 score was found to be between 1% and 5%. In July 2017, the patient
was started on pembrolizumab 200 mg every 3 weeks after his steroid dosing was
steadily lowered to prednisone 10 mg. Given the convenience of every 21-day dosing,
pembrolizumab was chosen over nivolumab. Within 6 weeks of starting PD1-inhibitors,
the patient experienced dramatic neurological improvement in his arm weakness and
gait. He regained the ability to walk without any assistance and has continued to
experience progressive reduction in his residual deficits of right face numbness and
paresthesia. During treatment with pembrolizumab, he did experience a mild rash,
which was evaluated by dermatology and felt to be more consistent with his known
history of rosacea than an immunotherapy-related rash. Repeat MRI as of November
2018 has demonstrated continued response with near complete resolution in
enhancement along the pontomedullary junction in the region of CN VI, with stable
disease at the right clivus, and with no new areas of enhancement ().


A 42-year-old woman was admitted to our hospital with a 1-month history of melena and mild abdominal pain. She denied nausea, vomiting, hematemesis, jaundice, cough, hemoptysis, fever, and/or menstrual abnormalities. She had no significant medical history or family history of pulmonary TB. She also denied tobacco or alcohol abuse. Upon admission, her general examination showed pallor, and abdominal examination revealed mild upper abdominal tenderness. Laboratory findings were as follows: hemoglobin 76 g/L, hematocrit 25.6%, mean corpuscular hemoglobin 25.3 pg/cell, erythrocyte sedimentation rate 36 mm/hour, and a C-reactive protein level of 24 mg/L. A T-SPOT.TB assay was performed and showed a positive result. Hepatic enzymes such as alkaline phosphatase, as well as bilirubin, albumin, prothrombin time, and partial prothrombin time showed no abnormalities. Additionally, the human immunodeficiency virus, a hepatitis panel, and tumor markers were negative. After treatment with pharmacological agents, such as proton-pump inhibitors and octreotide, active gastric variceal bleeding stopped.
Gastroduodenoscopic examination showed isolated gastric fundal varices with red color signs (Fig. a). Colonoscopic examination did not reveal any abnormality. Abdominal contrast-enhanced computed tomography (CECT) revealed non-enhanced masses of soft-tissue density in the lesser omentum and behind the head, body, and the tail of the pancreas, as well as multiple para-aortic lymph nodes and multiple small hypodense splenic lesions (Fig. b). CECT findings were suggestive of an infectious or metastatic disease or lymphatic cysts. Whole body [F-18]2-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET)-CT was performed to evaluate for lymphoma and metastatic disease. This test showed hypermetabolic lymph nodes in the right supraclavicular fossa, right tracheoesophageal groove, the lesser omental and retropancreatic areas, the mesentery, the porta hepatis, the splenic hilum, and the retroperitoneum with maximum standardized uptake values (SUVmax) ranging from 5.3–7.5 (Fig. c). Additionally, the bone marrow showed hypermetabolic FDG activity with a SUVmax of 5.2. The PET/CT findings were suggestive of lymphoma.
Bone marrow biopsy revealed no abnormality. Subsequently, endoscopic ultrasonography (EUS) was performed and revealed a hypoechoic mass at the splenic hilum, compressing the splenic vein (Fig. d). EUS-guided fine-needle aspiration was performed to obtain tissues for histopathological examination. Histopathological examination showed sheets of inflammatory cells, primarily lymphocytes and macrophages along with some glandular tissue. CT-guided biopsy of the retroperitoneal lymph nodes was performed to establish a definitive diagnosis, and the histopathological examination of these biopsy specimens showed necrotizing granulomatous inflammation and microorganisms that stained positive with acid-fast stains (Fig. a-c).
Based on all the aforementioned findings, she was diagnosed with abdominal TB. Anti-TB treatment comprising rifampicin, isoniazid, and ethambutol was initiated following histopathological confirmation. After leaving our hospital, she went to a TB hospital for further treatment, where her doctor added pyrazinamide to the treatment. She received a 6-month regimen of rifampicin: 2HRZE/4HR. During the course of treatment, the patient experienced mild elevation of transaminase. Abdominal CECT showed a decrease in the size of the lesser omental and retropancreatic masses, as well as the para-aortic lymph nodes after 4-month anti-TB therapy (Fig. d). The patient was asymptomatic at her 12-month follow-up.


The proband was a 37-year-old man who was referred to our hospital with a 7-month history of progressive weight loss, slurred speech, limb stiffness, and blunt response. Seven months ago, the patient suffered from rapid emaciation with 20-kg weight loss in 2 months. Then, the patient gradually developed dysarthria and occasionally choked when drinking water. Three months later, the patient had upper limb tremors and clumsy hands. Four months later, blunt response, memory loss, and irritability were observed by his family. In the course of the disease, the patient had constipation, accompanied by sweating and sebaceous gland hypersecretion. He denied fever, headache, loss of consciousness, convulsions, muscular atrophy or fibrillation. The medical history information was collected in Feb 2018 when he was admitted to the inpatient department of our hospital. His parents, one elder brother, two elder sisters, and his son are all physically healthy.
On examination, he (high school degree) had a significant global cognitive decline with a mini-mental state examination (MMSE) score of 18/30 and a Montreal cognitive assessment scale (MoCA) score of 15/30. He had obvious dysarthria with bilateral reduced palatal movements, indicating pseudobulbar palsy. Tone was increased in the neck and right limbs. Hyperreflexes of the legs with ankle clonus were noticed, but bilateral pathological signs were negative. His gait had a slow and shuffling characteristic, and he had difficulty turning around. Both upper limbs showed slight postural tremor and clumsy rotation. Finger-nose coordination was slow with mild intention tremor, and poor heel–knee coordination was found on the left limbs.
Blood laboratory tests were normal/negative, including the serum erythrocyte sedimentation rate, C-reactive protein, vitamins (B1\B2\B6\B9\B12), thyroid function, adrenocorticotropic\sex hormone, renin-angiotensin-aldosterone, and antinuclear antibodies. Cerebrospinal fluid analysis of paraneoplastic antibodies, antibodies specific for demyelinating diseases of the central nervous system, autoimmune encephalitis and oligoclonal IgG bands were normal/negative. Syphilis serology and HIV tests were negative.
Brain T2 fluid-attenuated inversion recovery (Flair) and diffusion-weighted imaging (DWI) MRI showed multiple, patchy, symmetrical, and hyperintense lesions in the periventricular areas, corpus callosum, and deep white matter regions of the frontal and parietal lobes. The splenium of the corpus callosum showed a homogeneous hyperintense signal in both Flair and DWI. With disease progression, patchy white matter lesions, which were initially small dots, became more widespread and confluent in this patient. DWI images showed a sustained intense signal over time, which is very unlike DWI high signal in stroke disease. An MRI scan also showed enlarged lateral ventricles (-–).
Histopathological examination of brain biopsy showed white matter lesions with myelin and axon damage and neuroaxonal swelling (spheroid formation), accompanied by prominent gliosis in the frontal lobe (-,). The activated microglia and phagocytic cells appeared to be segregated (-–). Electron microscopy confirmed the loss of myelin and axons. The axons were swollen and spherical (-). Spheroids had a thin and discontinuous myelin sheath or no myelin sheath. Large numbers of neurofilaments and organelles were observed in the axoplasm (-).


The patient was a 48-year-old woman who had undergone a cesarean section at the age of 35 years. She underwent plain computed tomography (CT) as a screening before surgery for pectus excavatum, and was incidentally found to have a pelvic mass. She was examined by a local gynecologist but showed no remarkable findings and was then referred to our hospital for further investigation of the pelvic tumor. Her height was 169.0 cm; body weight, 52.7 kg; and BMI, 18.5 kg/m2. The white blood cell count was 3600/μL; hemoglobin level, 13.5 g/dL; and CRP level, 0.02 mg/dL. A blood test for tumor markers including carcinoembryonic antigen, CA19-9, and CA125 showed negative results. Abdominal plain radiography showed no remarkable findings. Abdominal enhanced CT (Fig. ) showed a 40-mm mass containing linear high-density areas. The capsule of the mass had a slight enhancement effect. It was not clear whether the mass was continuous with the intestinal tract. However, the mass contained air, thus raising the possibility that the mass was continuous with the intestinal tract. We, therefore, decided to perform transanal double-balloon enteroscopy for further investigation. The enteroscopic examination showed a small fistula that was likely caused by penetration of the ileum dozens of centimeters from the ileocecal valve (Fig. a). A yellow–brown, movable, and fibrous body was found in the fistula (Fig. b). Contrast enhancement via the fistula showed a defect in the enclosed cavity (Fig. ). When the body was grasped with forceps, the defect inside was found to be movable. The body was held, and part of it was extracted with forceps (Fig. c) and submitted for pathological examination (Fig. ). The examination of the extracted fibrous body suggested that it was gauze. On the basis these findings, the condition of the patient was diagnosed as gossypiboma penetrating the ileum. Presumably, the gauze had been left during the cesarean section because the patient had not undergone any other abdominal surgery. The diagnosis was explained to the patient and her family. The patient desired to undergo surgery for gossypiboma at the hospital where she had undergone the cesarean section, and was referred to that hospital.


A 74-year-old female patient attended the vascular surgery outpatient clinic and was referred to the hospital for revascularization of the distal arteries. She had necrotic ulcers in both legs, worse in the right. She reported pain, signs of local infection and myiasis on the lateral side of the ankle, tendon exposure, edema, and dry skin, but no signs of acute ischemia. Her underlying diseases were difficult to control: systemic arterial hypertension for 20 years; type II diabetes mellitus (DM) for 13 years; hypothyroidism; a stroke 6 years ago, chronic renal failure class IV; peripheral arterial occlusive disease, and postmenopausal osteoporosis. The patient referred to previous angioplasty performed 1 year earlier on the lower right leg due to peripheral arterial occlusive disease. Upon hospital admission, several sites of infection other than skin and soft tissue were discarded. Laboratory tests showed a normal leukocyte count and reactive C protein of 3.98 mg/dL (reference value: < 0.30 mg/dL). Empiric treatment with piperacillin-tazobactam (4.5 g IV 6/6 h) was initiated, which was prescribed for 5 days.
Two days after admission, surgical debridement was performed. Limb amputation was discussed, but rejected by the patient and family members. During the surgery, a fragment of the ulcer tissue was collected and sent to the hospital’s microbiology laboratory. In the staining procedure, a few gram-positive cocci and gram-negative bacilli were observed. The specimen was submitted for enrichment in the brain-heart infusion broth for 24 h/37 °C and later seeded in 5% sheep blood agar and MacConkey agar, incubated for 37 °C, and presented growth after 24 h. VITEK 2 system (bioMérieux, Marcy l’Etoile, France) identified Enterococcus faecalis, Stenotrophomonas maltophilia, and B. trematum. The isolate was subsequently identified as B. trematum, using VITEK MS (bioMérieux, Marcy l’Etoile, France) and confirmed by 16S rRNA gene sequencing with Illumina MiSeq (Illumina, San Diego, CA, USA). The oxidase test was negative. MICs were determined by Sensititre gram-negative MIC plate (Thermo Scientific, Waltham, MA, USA) (Table ).
After surgery, the intubated patient was transferred to the ICU, using vasoactive drugs through a central venous catheter. Three days later, she presented a worsening clinical condition. Oxacillin-resistant Staphylococcus hominis was isolated from a blood culture drawn through a peripheral vein. Piperacillin-tazoctam was replaced by meropenem (500 mg IV 24/24 h) and vancomycin (1 g IV 24/24 h), prescribed for 14 days. Four days later, levofloxacin (750 mg IV 24/24 h) was added for 24 days aiming at S. maltophilia isolated from the ulcer tissue.
The necrotic ulcers evolved without further complication and the patient’s clinical condition improved, leading to temporary withdrawal of the vasoactive drugs and extubation. However, the patient’s general condition and kidney function worsened, probably due to the severity of her underlying diseases, and she died from sepsis of cutaneous origin 58 days after hospital admission. Autopsy was not performed. Figure shows a timeline of the events.


An 18-year-old male patient with Hunter syndrome visited at the Department of Surgery at Tottori University Hospital for evaluation of a palpable mass in the right groin area. Computed tomography revealed a right indirect inguinal hernia containing the small intestine (Fig. ). He had a history of repeated admission to our hospital and pediatric treatments for pneumonia, heart failure, and convulsions after birth. He was small in stature (height, 133.7 cm; weight, 36.6 kg). We observed no apparent hernia on the left side; however, a wide hernia orifice was present. Therefore, we selected a laparoscopic approach instead of an anterior approach to check for an occult hernia on the left side.
We performed TAPP repair for the right-side inguinal hernia after confirming that no hernia was present on the other side under laparoscopy. After inserting a 12-mm trocar in the umbilicus and identifying the indirect inguinal hernia on the right side and the absence of a hernia on the left side, we inserted 5-mm trocars in the bilateral flank regions. Insertion of the trocars was very difficult because of the softness of the abdominal wall (Fig. a), which may have been due to Hunter syndrome. We cut the peritoneum outside of the hernia orifice (Fig. b), identified the spermatic cord and testicular artery, and dissected the preperitoneal space with gauze. We found a network of veins around the spermatic cord and testicular artery (Fig. c), and special care was required to avoid hemorrhage. We inserted a prosthetic mesh (16.0 × 10.8 cm) into the preperitoneal space and tacked it onto either side of the inferior epigastric artery, transverse abdominal muscle, abdominal rectus muscle, and Cooper’s ligament. The hernia orifice was completely covered, and the peritoneum was closed by suturing. Although the operation involved minimal bleeding, it took 1 h 53 min to complete because the softness of the abdominal wall and the patient’s shorter height made the operative procedure difficult. Although a mild convulsion and subsequent aspiration pneumonia after the operation prolonged his hospital stay, he recovered with appropriate treatments and was discharged on postoperative day 9.


A 28-year-old Togolese woman was admitted to the Gynecology Department of Kara University Hospital with a 1-week history of bleeding and pelvic pain. She reported her age at first menstrual period as 14 years old and her age at first sexual intercourse as 15 years old. She did not smoke, drink alcohol, or take contraceptive pills. She did not report any history of sexually transmitted infections. The patient had no medical, obstetric, social, environmental, or special family history. She had never received an intervention, and she had a good psychosocial state.
Clinical examination revealed an axillary temperature of 38 °C, body weight of 54 kg, and height of 1.68 m. The patient’s blood pressure was 100/60 mmHg, and her pulse was good. Her general condition was good. Upon inspection, her conjunctivas were moderately hyperemic, and her abdomen palpated normally but was painful on palpation of the pelvic region. There was palpable lymphadenopathy in the region of the inguinal lymph nodes. Her gynecological speculum examination showed a macroscopically healthy uterine cervix. The result of examination of her external genitalia was normal. Her neurological examination and other investigations were unremarkable. The result of biological explorations, namely hemoglobin, was normal (13.5 g/dl). Her renal biology was normal (urea 0.22 g/L, creatinine 9 mg/L, blood glucose 0.9 g/L). The result of her hepatic evaluation was also normal (transaminases 19 IU/L, phosphatases 104 IU/L, γ-glutamyltransferase 21 IU/L). The result of her human immunodeficiency virus serology test was negative.
Abdominal ultrasound showed regular thickening of the endometrium, measuring 19 mm thick, and no substantial masses (Fig. ). Her ovaries were normal in size. Biopsy with curettage of the endometrium was performed.
Anatomopathological examination with hematoxylin and eosin (H&E) staining showed neoplastic cells of an epidermoid nature with bridges of unions arranged in lobules, often centered, with dyskeratotic maturation. Cellular atypia of epidermoid cells, particularly anisokaryosis, and mitotic figures were noted (Fig. ). Immunohistochemistry (IHC) investigations showed positivity for anti-epithelial membrane antigen markers and anti-pancytokeratin 1 (KL1+) markers of tumor cells (Figs. and ). The cells were negative for chromogranin A, actin, S100, estrogen receptor (ER), and progesterone receptor (PR). Positive staining for Ki-67 (antigen of cell multiplication) was observed in 50% of the tumor cells. In situ hybridization demonstrated HPV genotype 16/18. A diagnosis of PSCC of the endometrium associated with HPV genotype 16/18 was made.
Total hysterectomy with bilateral adnexectomy and bilateral inguinal dissection were performed. The inguinal dissection revealed a total of five ganglia (three lymph nodes on the right and two lymph nodes on the left). The cervix, fallopian tubes, and ovaries were macroscopically normal. Histologically, the tumor consisted of well-differentiated epidermoid cells with union bridges and dyskeratotic maturation invading the middle third of the myometrium. The diagnosis of a well-differentiated, invasive SCC of the endometrium was confirmed. There was no differentiation of adenocarcinoma, and no squamous metaplasia or dysplasia was observed. No tumor cells were seen in the cervix, the fallopian tubes, the ovaries, the omentum, or the lymph nodes.
The histoprognostic grade was classified as FIGO (International Federation of Gynecology and Obstetrics) stage IB, corresponding to pT1N0M0. The patient received three cycles of docetaxel (75 mg/m2, day 1, pump in) combined with carboplatin (200 mg/m2, day 1, drop in) chemotherapy.
After 3 and 6 months of follow-up, the patient was well, without recurrence.


This was a 76-year-old man who was admitted to the hospital due to abnormal liver function tests and septic shock associated with systemic bacterial infection. Further workup during hospital stay revealed multifocal lymphadenopathy including cervical and inguinal regions. Ultrasound guided biopsy was performed from an inguinal lymph node. The patient was discharged after treatment of infection, improving cardiovascular symptoms and liver function. The patient electively refused treatment for lymphoma.
The biopsy showed diffuse large lymphoid cell infiltration with necrosis without recognizable follicular architecture. The lymphocytes showed moderate amount of cytoplasm and round nuclei with prominent nucleoli.
Immunohistochemical stains (IHC) with the following antibodies were performed following standard IHC protocol on Leica Bond Max stainer. The following antibodies from DAKO were used for staining: CD20, PAX5, CD3, CD5, CD10, CD23, CCND1, BCl-2, BCL-6, MUM1, SOX11 and Ki67.
IHC stains showed that the tumor cells were positive for CD20, cyclin D1, BCL6 and MUM-1. There was no CD5 or CD10 expression. SOX11 was negative (Fig. ). EBER was negative. Proliferation index by ki67 was 80%.
Interphase fluorescence in situ hybridization (FISH) was performed in the cytogenetic laboratory at Department of Pathology, Duke University Health System. Briefly, 4um sections were cut and de-paraffinized, FISH for CCDN1/IgH was performed using dual color, dual fusion probe from Abbott Molecular. This probe targets the CCND1 locus at 11q13 and the IgH locus at 14q23 for detection of the fusion gen associated with the translocation of 11;14. FISH for BCL-2, BCL-6 and myc were also performed using the probes from Abbott molecular.
Abnormal hybridization patterns with at least 2 fusion signals were overserved in 23/100 (23%) of the nuclei examined. The predominant abnormal signal pattern, presented in 13 cells, was consistent with two CCND1, one IgH and two fusion signals. Additional abnormal signal patterns with a single fusion signal were also observed. Gain and loss of signals, likely related to the truncation of nuclei in paraffin sections, was also observed.
39–52% of interphase nuclei examined showed an atypical pattern for the BCL6 locus consistent with either atypical rearrangement or partial deletion of the locus, and 3 signals for the IGH locus, consistent with CCND1/IGH fusion. No evidence of MYC (8q24.2) rearrangement or IGH/BCL2 fusion was observed. Taken together all the results from histology and molecular studies, this case was diagnosed to be diffuse large cell lymphoma (DLBCL) with BCL-1 expression (Fig. ).


A 73-year-old man with unresectable stage IIIB MCC was referred to the National Institutes of Health for treatment with the monoclonal anti-programmed cell death ligand 1 (PD-L1) antibody avelumab. On physical examination, there were multiple pink to deep red smooth tumors with prominent vasculature on the central scalp (Fig. a) and left cervical lymphadenopathy was palpable. Biopsy of a scalp tumor revealed neuroendocrine carcinoma with positive staining for cytokeratin 20 (CK20) and synaptophysin, confirming the diagnosis of MCC. Positron emission tomography/computerized tomography (PET/CT) scanning showed metabolically active cutaneous and subcutaneous nodules on the vertex of the scalp, and multiple metabolically active enlarged cervical and supraclavicular lymph nodes.
The patient was started on avelumab at a dose of 10 mg/kg infused every two weeks. He was pre-medicated with acetaminophen, diphenhydramine and ranitidine. Two weeks after his first infusion his scalp lesions were inflamed and enlarged, consistent with pseudo-progression (Fig. b). The scalp tumors and lesions on CT scans subsequently regressed (Fig. c).
Between his second and third infusions, the patient developed a pruritic erythematous eruption on the chest, upper back, upper arms and right lower extremity. Examination revealed numerous thin, pink-brown scaly plaques ranging in size from 1.0 cm to 1.5 cm and involving sites of pre-existing seborrheic keratoses (SK) and solar lentigines (Fig. d, f & g). A shave biopsy of an affected lesion on the right posterior shoulder was performed and histology demonstrated papillomatous epidermal hyperplasia with hyperkeratosis and focal parakeratosis. The epidermis contained scattered exocytosed lymphocytes associated with mild spongiosis, intermittent hypergranulosis, and copious dyskeratotic keratinocytes. The dermal-epidermal junction was obscured by a lichenoid infiltrate primarily composed of T-lymphocytes. These clinical and histological finding are consistent with lichen planus-like keratosis (Fig. a-e). Treatment with topical triamcinolone 0.1% ointment twice daily provided symptomatic relief. Inflammation of affected lesions diminished over the following two weeks (Fig. e & h), however, the patient experienced intermittent inflammation in scattered keratoses and lentigines during continued therapy with avelumab. Treatment with cryotherapy was effective at ablating individual symptomatic lesions and resolving the local inflammation.


A 55-year-old man presented to the emergency department (ED) with acute onset abdominal pain started insidiously approximately 1-hour prior to presentation. He was discharged only a week before from the same hospital when he was admitted with acute abdominal pain when he underwent a CT-angiogram and a digital subtraction angiography (DSA) of the abdomen, which showed incidental finding of Arc of Buhler (). There was discussion regarding management of this incidental finding and decision was taken not to embolize it by intervention radiology as it was incidental finding and pain was not attributed to that. He was subsequently discharged after observation in the hospital for a couple of days when his labs remained steady. During this admission the patient was alert and oriented in time and place. Abdomen was soft to palpation and tender on deep palpation without peritoneal signs on clinical exam. The abdominal radiography showed partial small bowel obstruction. The CT-scan of the abdomen showed big retroperitoneal hematoma and free fluid around the liver (). Review of systems was grossly nonspecific on arrival to the ED except sharp abdominal pain. His past medical history was essentially negative for any chronic diseases; his surgical history consisted of inguinal hernia repair. After 40-min the patient had sudden cardiac arrest and cardiopulmonary resuscitation as per ACLS protocol was initiated. The patient was emergently intubated. A right subclavian central line was placed. Volume resuscitation was initiated, patient was started on pharmacologic presser agents and massive transfusion protocol was set up. We were working on provisional diagnosis of ruptured Arc of Buhler. Seven units of packed red blood cells (PRBC), 6 units of fresh frozen plasma (FFP) and 2 units of platelets were transfused. His abdomen was massively distended. His vitals were recorded as BP 90/52; HR 120; oxygen saturation at 90%. His labs showed Hb/Hct of 4.8/14.8; and platelet of 90. His serum chemistry was normal at this time with Na 135; K 3.6; Cl 93; CO2 21; BUN 17; Cr 0.1. The patient was taken to the operating room (OR) emergently. A midline trauma incision was made extending from the xiphisternum to the symphysis pubis. Two cell saving suctions were used. About 2L of hemoperitoneum and clotted blood were suctioned out. Massive transfusion was continued intraoperatively. Four quadrant packing was used to temporize the bleeding. A window through the gastrohepatic ligament was used to clamp the supra celiac aorta. There was large bulging retroperitoneal hematoma with gross distortion of anatomy. The spleen was ruptured. There was significant generalized diffuse oozing suggestive of disseminated intravascular coagulation (DIC). We used packs and hemostatic gauze were placed to tamponade the bleeding. The abdomen was left open with an Abthera and Vaccum assisted closure (VAC) with a plan to return to the OR when the acidosis, hypothermia and coagulopathy is corrected. The patient was transferred to the PACU in critical condition. Early next day morning the patient had a sudden cardiorespiratory arrest. ACLS protocol along with CPR was initiated. However, the attempt was futile and the patient was pronounced dead after all resuscitative efforts.


An 85-year-old woman with severe aortic valve stenosis (AS) was admitted to undergo transcatheter aortic valve implantation. She had a history of cerebral infraction, with no remarkable family history. Recently, she experienced chest pain, clammy sweat, and anorexia; she visited a local doctor for AS treatment.
She complained of chest and back pain and developed fever after undergoing preoperative transesophageal echocardiography (TEE). The next day, the symptoms did not improve and computed tomography (CT) revealed prominent mediastinal emphysema and pleural effusion. Upper gastrointestinal endoscopy confirmed esophageal perforation located 30 cm from the incisors (A), and gastrografin contrast revealed mediastinum leakage ().
She was diagnosed with thoracic esophageal perforation. Radical thoracotomy surgery (primary repair or resection) was difficult because she was elderly and had severe AS. Therefore, two-stage surgery and indirect approach, comprising cervical esophagostomy to avoid contamination, gastrostomy for decompression, and jejunostomy for nutrition, was adopted. Reconstruction was planned after the mediastinitis and perforation were healed.
An emergency operation was performed 32 h after TEE under general anesthesia; a 12-mm trocar for the laparoscope was placed through the umbilicus, and four 5-mm ports were placed in the left upper, right upper, left middle, and right middle quadrants. We washed the contaminated mediastinum with saline through the esophageal hiatus from the abdominal cavity side and placed the drainage tube in the mediastinum. We then performed gastrostomy and jejunostomy laparoscopically, followed by cervical esophagostomy using a tube. Esophageal dissection was performed by an autosuture device (operation time: 2 h 14 min; blood loss: minimal).
Postoperatively, clinical course was good. At 11 days postoperatively, CT revealed almost complete resolution of the mediastinal air and cavity and the mediastinal drain was removed.
At 22 days postoperatively, endoscopic retrograde observation via gastrostomy revealed a healed perforation (B), and the cervical esophageal stump that was separated during surgery was connected; it became patent spontaneously (A). Endoscopic findings confirmed that the recanalized segment’s lumen was strong, and we assessed the recanalized segment’s continuity by CT. Anastomotic surgery was unnecessary; however, because of esophageal stenosis, endoscopic balloon dilation was necessary. After four sessions of dilation (B), she could orally consume food without additional surgery. Currently, she can walk with assistance and has a good oral intake ().


A 35-year-old Hispanic male presented to the emergency department for sudden onset worsening headaches over the past four days. Headaches were diffusely felt, not localized to a specific head region, and not relieved by over the counter pain medication. There was no associated trauma, fever, night sweats, loss of consciousness, photophobia, neck stiffness, or visual disturbances. His past medical history was significant for migraine headaches, type 2 diabetes mellitus, and benign essential hypertension. Physical exam revealed Glasgow Coma Scale score of 3T on 15L of oxygen, pupils 2-2.5 mm bilaterally, inability to arouse by voice or painful stimulation, and paralysis of upper and lower extremities bilaterally. Vitals include (heart rate = 89/min, blood pressure = 159/78 mmHg, temperature = 37°C, respiratory rate = 22/min, and oxygen saturation = 99% on 15L of oxygen). Labs were significant for respiratory alkalosis (blood pH 7.54, pCO2 27.5 mmHg), normal oxygenation (159.3 mmHg), and negative urinary toxicology screen.
A head CT scan revealed a 1.5 cm hematoma in the left thalamus with expansion into the lateral ventricle (Figure ). Cerebral angiogram demonstrated complete stenosis of the left internal carotid artery (ICA) and partial stenosis of the right ICA (Figures -). Diagnostic studies coupled with clinical presentation were compatible with a diagnosis of MMD. Patient treatment was initialized with emergent bedside right ventriculostomy (with opening pressure of 11 cmH2O) for intracranial pressure monitoring and for diversion of cerebrospinal fluid and hematoma. This patient ultimately underwent surgical revascularization. The procedure included a left superficial temporal artery-left middle cerebral artery bypass without any complications.


A 15-year-old female with no significant past medical history presented after being struck in the face by a ball while playing water polo. The patient felt pain in her jaw, which was the chief complaint when she presented to the emergency department. Upon neurological assessment, the patient complained of midline tenderness from the skull base to midline cervical spine over C3; denied headaches, changes in vision, speech or swallowing, extremity weakness or paresthesias. A maxillofacial computed tomography (CT) scan did not show evidence of an acute facial fracture. However, the CT scan did reveal a radiolucent, ovoid-shaped lytic lesion arising in the left lateral mass of C1, between the anterior tubercle and the transverse process. Magnetic resonance imaging (MRI) studies further confirmed an enhancing osseous lesion at the left lateral mass of C1, with cortical breach and extension into the left lateral atlantodental joint space (). Of note, three years prior, patient had a CT cervical spine which, upon retrospective review, demonstrated a similar but much smaller lesion.
Differential diagnoses underlying this vertebral cortical erosion included those of infectious etiology, as well as oncologic lesions, such as giant cell tumor of bone, aneurysmal bone cyst, osteoblastoma, osteosarcoma or even Langerhans histiocytosis (LCH). Oncology recommended that the cervical spine lesion be biopsied for tissue diagnosis. Due to the unusual location of the lesion and risk of locally aggressive pathology, or possible tumor seeding along the biopsy track, interventional radiology was unable to perform a CT guided needle biopsy. It was therefore decided that the patient would require open neurosurgical biopsy for diagnosis.
Due to the anterior and lateral location of the vertebral lesion, an anterior transoral approach to the C1 lesion was performed, in order to obtain a sufficient amount of the contrast enhancing component of the mass for pathologic diagnosis. The transoral approach was performed in a multidisciplinary fashion, during which the otolaryngology team used direct visualization, as well as stereotactic navigation, to expose the C1 anterior tubercle on the left side. Once exposure was completed, neurosurgery team utilized a matchstick burr to then drill the anterior outer cortex of C1. Multiple specimens from the fibrous tumor were taken, with curettes and pituitary forceps.
The sampled tissue did not show features of osteoblastoma or osteosarcoma, nor were there features of LCH or signs of infection. In the sampled region, the lesion consisted of a proliferation of nondescript stromal cells with intermixed multinucleated giant cells, and occasional clusters of foamy histiocytes (). Special testing for giant cell tumor of bone (G34W staining) was negative, as was fluorescence in situ hybridization (FISH) testing for Ubiquitin Specific Peptidase 6 (USP6), making a primary form of aneurysmal bone cyst unlikely. However, due to the aggressive nature of the patient’s osteolytic lesion and the significant risk for atlantoaxial instability associated with its location, it was decided to start the patient on Denosumab. Denosumab is an osteoclast inhibiting pharmaceutical agent, which was administered to the patient in order to stabilize and consolidate the lesion. Samples of the patient’s lesion were also sent out to a nationally recognized expert bone pathologist, whose findings were most consistent with benign giant cell rich lesion with histiocytes.
The patient was re-assessed three months postoperatively and MRI studies revealed that there was no interval decrease in the size of the tumor. In fact, there was a slight progression of the lesion anteriorly, despite treatment with Denosumab. After presenting the patient’s case at our institution’s multidisciplinary tumor board, it was decided to offer the patient a gross total resection of the offending lesion. This would inherently lead to significant atlantoaxial instability, therefore a posterior occiput to cervical three instrumented fusion was also warranted.
The transoral approach was performed in a multidisciplinary fashion, during which the otolaryngology team used direct visualization as well as stereotactic navigation, to expose the cervical vertebrae through the posterior pharynx. Fibrous tumor was identified and dissected until superior, inferior, and lateral margins of tumor resection were confirmed grossly, with fluoroscopy, and neuronavigation. Additional C1 anterior tubercle eccentric towards the right side was also taken, to include a normal bony margin. A small rim of tumor adherent to the vertebral artery was left behind. After the otolaryngology team closed the posterior pharynx, the patient was carefully turned prone, maintaining spinal precautions. Base of the occiput to cervical three was then exposed. C2 pedicle screws were placed. C3 lateral mass screws were placed. An occipital plate was sized. Screws into the occiput were placed. Fluoroscopy confirmed excellent position and spinal alignment. There were no post-operative complications and the patient was discharged home in good condition. Pathologic examination of the resected material at this time showed complete disappearance of the giant cells, due to Denosumab therapy, with the remaining lesional tissue resembling benign fibrous histiocytoma (). Post-operative imaging studies revealed a stable posterior cervical spine construct, along with minimal rim-enhancement along the vertebral artery, as expected (). At a three-week follow up visit in clinic, the patient’s incisions were healing well, she was neurologically intact, tolerating regular diet, and was eager to return to school.