A 66-year-old man, affected by type II DM for 4 years and arterial hypertension for 10 years, developed severe non-proliferative diabetic retinopathy without macular edema just 2 years after DM diagnosis. The patient was treated with panretinal photocoagulation (PRP), because he had a poor glycometabolic control and was unable to adhere to a close follow-up. One year after PRP, he developed severe visual loss due to diffuse non tractional bilateral DME. Thus, the two eyes were treated with an IV triamcinolone injection 1 month apart (the right eye [RE] before the left eye [LE]) and laser grid photocoagulation was performed in both eyes 2 weeks after IV injection. After no visual recovery for over 2 months due to the persistence of macular edema in both eyes, an IV injection of bevacizumab was given in the RE, where a larger amount of intraretinal fluid and subfoveal neural detachment were observed. However, 1 week after the injection, anterior ischemic optic neuropathy (AION) with a residual visual acuity of hand motion was observed. During the entire period, the patient had poor glycometabolic control (glycated hemoglobin >9%, high hypertension, and hypercholesterolemia) and poor adherence to therapy. This fact could have influenced the patient’s poor response to therapy and the development of AION.
As a result of this unfortunate adverse event, the patient refused an IV injection of bevacizumab in his LE. After informing the patient about the risks and benefits of different treatment options such as observation, off-label IV injections of steroids, and subtenon injections of IFNα, the patient signed an informed consent for a cycle of subtenon injections of IFNα.
Before the treatment, the BCVA in the LE was 20/200. The patient received posterior subtenon injections of IFNα (1×106 IU/ml) three times (on Monday, Wednesday, and Friday) for a week in his LE, according to the following procedure: after the administration of topical 0.4% oxybuprocaine surface anesthesia, 1 ml of IFNα was slowly injected into the inferotemporal quadrant under the Tenon’s capsule, using a 27-gauge needle on a 2.5-ml syringe. The needle was moved toward the macular area, until the hub was firmly pressed against the conjunctival fornix. After the first injection, topical 0.3% netilmicin eye drops were prescribed three times a day for 7 days. During the period of IFN-α therapy, the patient had a good systemic condition and a good glycometabolic control (glycated hemoglobin = 6.9%).
A complete ophthalmic examination including BCVA, indirect ophthalmoscopy, and SD-OCT of the macular region was conducted several times throughout the period of IV injection and laser grid therapy and, specifically, preoperatively, as well as at 1 week, 1 month, 4 months, and 1 year after the last injection of INFα. SD-OCT images were obtained using Spectralis OCT spectral-domain (Heidelberg Engineering GmbH, Heidelberg, Germany) and the baseline macular scan was set as the reference (Figure
). Fifteen months after the treatment, the BCVA in the LE was 20/40, with reduced cystoid macular edema but the SD-OCT highlighted the persistence of the photoreceptor inner segment/outer segment (IS/OS) disruption already highlighted at the baseline (Figure
). The patient gives his written consent for the use of his data and any accompanying images.


A 56 year-old female presented to Nephrology in 1993 with nephrotic syndrome (15 g/day proteinuria, peripheral edema and serum albumin of 14 g/L) and normal renal function (Cr 70 umol/L). Percutaneous renal biopsy was performed which was consistent with stage II MN. There were no tubular or interstitial infiltrates. Her MN was felt to be idiopathic. She was treated with prednisone for a total of 12 weeks with decline in proteinuria to 1.1 g/d. Over time, she experienced a gradual and partial relapse with fluctuating urinary protein excretion (2.5-7.5 g/day) managed using RAS blockade. This was associated with gradual elevation of her serum creatinine to 105 umol/L by April 2009. In June 2009, prednisone (6 month treatment period) was initiated along with cyclosporine. Although her proteinuria did improve, her creatinine slowly increased and the cyclosporine was eventually discontinued in March 2010 when her creatinine reached 200 umol/L. During therapy, trough cyclosporine levels were less than 130 ug/L.
In April 2012, at her routine nephrology clinic visit, she was found to have a Cr of 319 umol/L. Her creatinine over the past year had ranged between 200–220 umol/L. (Figure ) She was clinically well and denied any recent illnesses, fevers, rashes, nausea, vomiting, diarrhea or reduced oral intake. She denied any flank pain, changes in her urination or shortness of breath. She had no symptoms of systemic vasculitis and had no constitutional symptoms. There were no recent changes in her prescription or over the counter medication, other than initiation of varenicline in Jan 2012 (Cr 225 umol/L). There was no non-steroidal anti-inflammatory (NSAID) medication or herbal remedy use.
Physical examination was unremarkable. She had no rashes, uveitis or dry eyes/mouth. Numerous granular casts were seen on urine microscopy. Other investigations including ANA, ENA antibodies (SSA/Ro, SSB/La, SM, RNP, Scl-70), RF, C3, C4, serum calcium, ANCA, HBV and HCV were all unremarkable. Peripheral blood eosinophil count was normal at 0.7 × 109/L. Urinary protein to creatinine ratio was 0.76 g/mmol as compared to previous in May 2011 of 0.51 g/mmol. Ultrasound revealed normal sized kidneys (10.0 and 10.7 cm) with mildly increased echogenicity bilaterally. There was no evidence of renal vein thrombosis or hydronephrosis.
It was questioned whether the varenicline was the culprit and this was discontinued in late April 2012 (exact date unknown). As renal function did not improve and urine microscopy remained active, the patient eventually consented to renal biopsy. This was performed in late June 2012 and revealed 18 glomeruli, 3 of which were globally sclerotic. The glomeruli were normocellular but had marked thickening of the basement membrane with complex chain-like abnormalities on silver stain. There was moderate interstitial fibrosis and tubular atrophy. There were also patchy moderately intense interstitial inflammatory infiltrates of lymphocytes and eosinophils along with a lymphocytic tubulitis and acute tubular epithelial injury. There were no granulomas. Immunofluorescence showed diffuse moderate to extensive staining for IgG, C3, kappa and lambda. Electron microscopy was not performed.
When the results became available in early July 2012, prednisone therapy was initiated at 40 mg for 4 weeks. The dosage of prednisone was then tapered to 30 mg, followed by a decrease of 10 mg following each subsequent week of treatment. Once at 10 mg/day it was decreased by 2.5 mg on a weekly basis. In total, the full course of prednisone treatment lasted 10 weeks.
Renal function improved throughout the course of prednisone treatment, with creatinine levels returning to baseline (228 umol/L) by Sept 2012 and absence of any activity on urinalysis. Two weeks after prednisone cessation creatinine began climbing again gradually reaching 300 umol/L by November 2012. The patient declined repeat biopsy, re-initiation of prednisone or the introduction of alternate immunosuppressant agents. Her most recent creatinine value was 315 umol/L (March 2013).
Of interest, she had also taken varenicline for three months during the time of kidney function deterioration (January –March 2010) which had been attributed to cyclosporine. She stopped the varenicline and resumed smoking the same month the cyclosporine was discontinued (exact dates unknown to patient). A renal biopsy was not performed and there is no record of urine microscopy. By July 2010, creatinine levels had dropped to 140–160 umol/L from 200 umol/L (March 2010). Over the subsequent year kidney function slowly deteriorated with creatinine values remaining stable throughout 2011 at 200–220 umol/L.
Her past medical history otherwise includes hypertension, steroid-induced diabetes that has since resolved and remote knee surgery. She has never been diagnosed with an auto-immune/connective tissue disorder or sarcoidosis. She has never been hypercalcemic. Chest X-ray was unremarkable. She has no family history of renal disease. Her medications in April 2012 included darbepoetin 20mcg subcutaneously every 21 days, ferrous gluconate 900 mg per oral (po) daily, ASA 81 mg po daily, atorvastatin 20 mg po daily, and enalapril 15 mg po daily. She was not consuming any non-steroidal anti-inflammatory drugs (NSAIDS) and she had no recorded allergies. The enalapril was subsequently discontinued and replace with Cardizem CD.


A 74 year-old Caucasian male was admitted elsewhere due to persistent fever. During his stay he sustained an acute coronary syndrome. A subsequent coronary angiogram revealed a 90% left anterior descending (LAD) stenosis and a 100% right coronary artery (RCA) occlusion. Both were addressed with drug eluted stents and the patient was discharged two days later.
Since the fever persisted, he was admitted 4 days later to the General ward of our Hospital. Blood cultures were positive to enterococcus faecalis and the appropriate antibiotic treatment was commenced. Whilst still on diagnostic workup the patient had a transthoracic echocardiogram (TTE) which revealed marginal normal dimensions of the left ventricle with a reported ejection fraction ranged between 30–35%. There was no evidence of left ventricular thrombus but there was significant mitral valve regurgitation with vegetations onto the posterior mitral leaflet (Figure ).
Nine days later despite the appropriate medical treatment, the patient’s condition deteriorated and he became unstable with need for inotropic support.
A second transthoracic echocardiogram revealed a surprising finding. The aforementioned significant mitral valve regurgitation with vegetations was in fact a large pseudo aneurysm of the left ventricle (6.5 × 6.0 cm) with remarkable movement of the involved ruptured apical wall mimicking the movements of the mitral leaflets, and concomitant moderate mitral valve regurgitation. A new transoesophageal echo (TOE) as well as a CT scan confirmed the diagnosis of a left ventricular pseudo aneurysm rather than mitral valve endocarditis.
Immediately after, a left ventriculoplasty (Dor procedure) was offered. After median sternotomy the pericardium was opened revealing abundant blood-tinged pericardial fluid and fresh, thick adhesions. After careful dissection of the pseudoaneurysm (Figure ), cardiopulmonary bypass was initiated through standard aortic and right atrial cannulation. After cardioplegic arrest and left ventricular venting through the right superior pulmonary vein, the pseudoaneurysm of the apex was opened. The aneurysmatic chamber’s diameter was estimated to be 6 cm (Figure ). Necrotic tissue was excised and the defect was closed with a synthetic Dacron patch sutured with interrupted pledgetted mattress sutures (Ti cron 2–0™) (Figure ). The aortic cross clamp time removed after 40 min and the heart took over the circulation fairly easily with modest inotropic support. Total bypass time was 72 min run.
His post-operative course was unremarkable except from transient renal insufficiency treated with sessions of haemofiltration and minor pulmonary atelectasis treated with the appropriate chest physiotherapy. Left ventricular diameter and movement was back to normal limits. Ejection fraction was back up to 55%. His temperature gradually declined and three consequent blood cultures were negative. He was discharged on an ambulatory setting on the 24th postoperative day and remains well in one year follow-up.


A 35 years-old male with left distal radius and ulna fracture was scheduled for closed reduction and percutaneous pinning. His Physical examination was normal. The patient did not have any past surgeries and didn’t take any medication. The patient was somewhat anxious about having surgery under general anesthesia and requested the regional anesthesia. Standard monitoring including non-invasive blood pressure recording, pulse oximetery and electrocardiography were applied and a single injection ultrasound guided infraclavicular block was implemented for the procedure. Midazolam 2 mg was given intravenously 10 minutes before the start of the block, and IV fentanyl (75 micg) before insertion of the needle was administered. We used a Honda HS – 2100 System and a 7.5-10 MHz linear ultrasound probe. The patient assumed a supine position and turned his head to the opposite side.
With the probe placed in a parasagital plane medial and inferior to the coracoids process, we obtained a clear short-axis image of the axillary artery. The entry point of the needle was anesthetized with lidocaine (3 mL, 1%). Then, using an in-plane approach, a 22 gauge, 100-mm short bevel insulated needle (pajunk, UniPlex NanoLine) was advanced and placed posterior to the axillary artery, next to the posterior cord. After reassuring that the needle tip is in correct location, and confirming a negative aspiration for blood, we slowly injected bupivacaine (30 mL, 0.25%) and dexamethasone (8 mg), and then withdrew the needle. During the injection no adverse effect was noticed. Five minutes after the injection muscle coordination in the extremity was lost and the pain diminished substantially. These were indicative of an impending successful block. After 15 minutes the surgery was allowed to start. The surgical procedure (closed reduction and percutaneous pinning) lasted 40 minutes. After the end of surgery, the patient was monitored for half an hour in the PACU and then transferred to ward.
The next morning, we were informed that the patient had not recovered completely from the motor and sensory blockade. Examination of the patient proved the same. The patient’s condition was reassessed once more 24 hours after the performance of the block. No significant change in the patient’s condition was found; complete motor and sensory blockade was persistent. Despite that the patient had no complaint about pain or paresthesia during injection of the local anesthetic and that we did not notice any unusual resistance to injection, a complete motor and sensory block in C6, C7, and C8 had remained, and the possibility of damage to nerves emerged. A nerve conduction study was requested and until then a trained nurse from acute pain service was put in charge of evaluating the patient every hour. It was approximately thirty hours after the original injection that the nurse reported the patient had felt occasional stabbing pain in his fingers. Expecting that recovery would gradually come about, the nerve conduction study was postponed to a later time in the next morning. Complete recovery of motor and sensory functions required 42 hours. Second examination of the brachial plexus 24 hours later did not reveal any residual motor or sensory blockade.


Our patient was 21 years old when he was first hospitalized. This single, Caucasian, right-handed man was living with his mother and had finished high school five years earlier. During this period, he did not pursue any goal-oriented projects; he did not work or try to go back to school, and spent most of his time withdrawn in his bedroom. He had no siblings and very little contact with his father. He had been drug free for the past year and his family history for neurological and psychotic disorders was negative.
He was admitted to the emergency ward of a psychiatric hospital about four years ago after a court order was issued to evaluate his mental state. Three days earlier, our patient had been arrested after his mother called the police to report being assaulted and threatened by him. The fight took place after she had scolded him for being lazy and aloof. His initial psychiatric evaluation revealed a very fragile mental state for at least the five previous years. After quitting school because of a lack of motivation, our patient started to spend most of his time reading about subjects related to spirituality. Previously, he had been a relatively good student, with no disruptive behavior and above average marks. He seemed obsessed with developing new concepts to reach a more ‘perfect’ existence and thought that studying anything but these subjects was simply futile. The initial mental examination, performed at the time of his admission, revealed coherent speech but a perplexed attitude and what sounded like loose associations. He seemed suspicious, but he had no suicidal or homicidal thoughts. He claimed feeling odd sensations in his mind and in his body that could be linked to some sort of ‘evolutionary’ process resulting from spiritual uplift. He also reported non-specific visual hallucinations (seeing odd shapes and colored lines) and what sounded like auditory hallucinations and telepathic powers. There was no hypergraphism and hypermoralism. His medical history was not contributory. The initial diagnosis was a possible schizophreniform disorder and our patient was admitted. His Global Assessment of Functioning (GAF) scale score was marked at 25, and a dose of risperidone 1mg at bedtime was initiated.
An electroencephalogram (EEG) was performed shortly after his admission and revealed a background symmetric alpha rhythm of about 10Hz, predominantly in the posterior and temporal regions of the brain, coupled with an important subcortical epileptic activity with 3 to 5Hz of slow, pointed waves outbursts. The results of a thorough neurological examination were perfectly normal. However, our patient reported having frequent ‘déjà vu’ sensations and depersonalization tantrums over the past few years to the consultant neurologist. The neurologist concluded our patient was a case of partial complex epilepsy with psychosensorial and psychotic symptoms and prescribed levetiracetam 500mg twice a day. Meanwhile, the risperidone was ceased since our patient showed worsening of certain symptoms. A neuropsychological evaluation showed important deficits in executive functions (especially working memory and concentration) and lack of motivation, probably secondary to intellectual understimulation for a long period of time. However, since our patient had no developmental delay up to the middle of high school, his intellectual potential was probably superior and was enhanced with proper management and stimulation. A control EEG performed one month later showed no improvement and carbamazepine continued release was progressively increased to 400mg twice a day (blood concentration stabilized at 40μmol/L). The results of a computed tomography (CT) scan and cerebral magnetic resonance imaging (MRI) study were both normal. Follow-up EEGs (performed six months after his first admission) continued to show the same epileptic activity and carbamazepine was stopped and replaced by valproic acid (up to 500mg twice a day with blood concentration stabilized at 452μmol/L). In spite of this, his EEG results remained practically unchanged.
Even though abnormal epileptic activity remained, our patient showed significant improvement during this period. Three months after admission, our patient left the hospital and started an integrated psychological therapy (IPT) group three times a week, a program that combines neurocognitive and social cognitive interventions with social skills approaches for patients who are schizophrenic. IPT has been shown to be an effective rehabilitation approach for patients experiencing psychotic disorders []. In addition, he was also integrated into a rehabilitation home in order to practice these skills in a protected and proactive setting adapted to his strengths and weaknesses and willing to follow his pace of learning. Monthly individual cognitive-behavioral therapy (CBT) sessions, adapted for psychotic patients [,], were also initiated by the treating psychiatrist to further potentiate the treatment. In a matter of months, our patient realized his deficits in social interactions. When confronted with them, he tried to isolate himself by going back to his old thoughts on spirituality and pursuit of a perfect world. However, these beliefs were found to be primitive psychological defense mechanisms and our patient himself knew this. The previous visual and tactile hallucinations became much more scattered and were more the fruits of odd interpretations of reality. Almost a year and a half after being hospitalized, he started a job reinsertion program and is looking forward to moving into his own apartment. The final psychiatric diagnosis was a psychotic disorder and personality change due to a general medical condition (epilepsy). However, despite active epilepsy, non-responsive to medication, he has managed to control a lot of symptoms deriving from the intertwining of his organic and psychiatric disorders. A control neuropsychological evaluation performed more than a year after the first one revealed steady scores for executive functions, but significant improvement in speed of information processing. His most recent GAF scale score was 65.


A 35-year-old male reported to the dental clinic with the chief complaint of painless swelling over the palate and anterior maxilla. The swelling was gradually increasing in size for the past 3 months with associated displacement of maxillary central incisors. There was no associated history of trauma.
On examination, a well defined firm nontender swelling was seen on the left side of anterior hard palate and crossing over the midline to the right side. The crown of left central incisor was displaced labially and mesially, overlapping the crown of right central incisor (). A provisional diagnosis of periapical cyst was given but the vitality tests of the central incisors were negative. The differential diagnosis was established with other conditions such as an nasopalatine duct cyst, enlarged nasopalatine duct, central giant cell granuloma, a root cyst associated to the upper central incisors, a supernumerary tooth follicular cyst, primordial cyst, and nasoalveolar cyst. Radiological investigations were subsequently advised.
Maxillary occlusal radiograph showed a well-defined round radiolucency approximately 2.5 cm in size with corticated margins in the midline and between the central incisors which was the typical radiographic feature of NPDC (). The lesion was causing displacement of the roots of the incisors. Superiorly it was extending till the floor of nasal fossa. Also there was deviation of the nasal septum to the right side.
CT scan also showed a well-defined radiolucency in anterior maxilla in the region of incisive canal, 2.6 cm × 2.8 cm in size. Mesiodistally the radiolucency was causing displacement of the roots of both maxillary central incisors and it was not extending laterally beyond the roots of incisors. Inferiorly it was extending till the crest of interdental bone. Loss of cortication was seen along the buccal and palatal aspects of the lesion in the sagittal sections. The lesion was causing mild elevation of the floor of nasal fossa in the anterior region with deviation of the nasal septum to the right side (Figures , , and ). Mucosal polyp was seen associated with the floor of left maxillary sinus. A radiographic diagnosis of NPDC was established and surgical enucleation was planned.
A surgical enucleation was done with intact removal of the cyst () and the specimen was sent for histopathological examination which showed cystic lining composed of stratified squamous epithelium about 2-3 cell layer thick. Lining was flattened and showed pseudostratification at places (). This was conclusive for NPDC.


A 63-year-old woman had undergone left nephrectomy for RCC at our hospital 13 years previously. After 5 consecutive years of follow-up, she underwent an annual medical examination. Abdominal ultrasonography (US) revealed an abnormal mass in the body of the pancreas. CT revealed two lesions: a low-density mass (15 mm in diameter) in the pancreatic body that displayed rim enhancement and a homogeneously enhanced mass (8 mm in diameter) in the head (Figure ). Magnetic resonance imaging (MRI) did not show enhancement in either lesion. FDG-PET did not show any abnormal metabolic activity in the pancreas. To allow a pathological diagnosis, endoscopic ultrasonography (EUS)-guided fine-needle aspiration biopsy was performed, but only necrotic tissue was obtained from the specimen. Although the radiographic features of the lesions were different, pancreatic metastases from RCC were strongly suspected because of the patient’s history of RCC. We noted the following from the laboratory findings: DUPAN-2, <25 U/ml (normal, <25 U/ml); Span-1, 2.3 U/ml (normal, <30 U/ml); carcinoembryonic antigen, 1.7 ng/ml (normal, <5.0 ng/ml); carbohydrate antigen 19-9, 2.6 U/ml (normal, <37 U/ml) and gastrin, 480 pg/ml (normal, <200 pg/ml). The patient underwent pylorus-preserving pancreaticoduodenectomy using the Imanaga method []. For the R0 resection, an extended pancreaticoduodenectomy was required rather than a classical resection. Intraoperative US revealed a low echoic mass with a bright halo and peripherally enriched blood flow in the body and a low echoic mass with homogeneously enriched blood flow in the head. Gross pathological examination revealed a 15 mm × 13 mm tumor occupying the body of the pancreas and another 8-mm tumor in the uncinate process of the pancreas. The head lesion was soft, whereas the body lesion was firm in consistency. The cut surface of the head lesion was yellow, whereas that of the lesion in the pancreatic body was grayish-white. Metastatic tumor cells homogeneously occupied the tumor in the head of the pancreas, and the firm lesion in the body of the pancreas showed a necrotic change in the center, which was surrounded by viable tumor cells and a fibrous capsule, identified as a low-density area on a CT scan (Figure ). Immunohistochemically, the tumors were positive for CD10 and negative for chromogranin A and synaptophysin (Figure ). Microscopic examination revealed large epithelial cells with clear cytoplasm and eosinophilic nuclei arranged in alveolar structures with abundant vascularity (Figure a). In addition, histological examination revealed another 1-mm occult micrometastatic lesion in the head of the pancreas (Figure b). The harvested lymph nodes and surgical margins were free of malignancy. Taken together, the pathological findings indicated that the lesions were metastases from RCC, and the thick enhanced rim of the body lesion was believed to be composed of viable RCC cells with high vascularity. The postoperative course was uneventful, and to date the patient has survived for 6 months without any evidence of recurrence or metastasis.
RCC accounts for approximately 2% of all adult malignances. Among kidney-limited diseases, RCC has a high overall survival rate (up to 95%) []. However, 20% to 30% of patients have metastases at presentation, and the 5-year survival rate is less than 10% once metastases spread []. In studies of resected specimens, RCC was the most common primary tumor leading to isolated pancreatic metastases [,]. RCC recurrence is classified as early or late recurrence. Late recurrence after nephrectomy is not common: recurrence is seen in 10% of patients after more than 10 years after surgery []. In most studies, the development of pancreatic metastasis was observed after a disease-free period after nephrectomy exceeding 10 years (maximum, 32.7 years) []. The relation between the metastatic location of the pancreas and the primary RCC is controversial [] and can be either hematogeneous or via lymphatics []. Hematogeneous spread may occur along the draining collateral vein of a hypervascular renal tumor, and the spread through lymphatics may occur by retrograde lymph flow secondary to tumor infiltration of the retroperitoneal lymph nodes [].
The symptoms of pancreatic metastasis are often nonspecific. Sellner et al. [] reported in a review of 236 cases that 35% of patients were asymptomatic, whereas other patients had abdominal pain (20%), gastrointestinal tract bleeding (20%), obstructive jaundice (9%), weight loss (9%), pancreatitis (3%) or diabetes mellitus (3%). The diameter of the metastatic lesion was reported to have some association with patient symptoms. Bassi et al. [] reported that the median tumor diameter in asymptomatic patients was 25 mm, compared to 45 mm in symptomatic patients. Conversely, Reddy et al. [] reported that 93% (42/45) of patients had symptoms such as abdominal pain, jaundice or weight loss.
In general, the preoperative diagnosis of pancreatic metastases begins with a suspicion based on the patient’s history []. Imaging modalities such as CT, MRI, FDG-PET and EUS support the diagnosis. Muranaka et al. [] reviewed the CT findings of pancreatic metastases from 28 metastatic carcinomas and classified these into 3 types according to their configuration: (1) a single localized metastasis (50% to 73%) [-]; (2) a diffuse enlargement with homogeneous attenuation of the pancreas (15% to 44%) [-] and (3) multifocal metastases (5% to 10%) [-]. Metastases from RCC are usually hypervascular and consequently display homogeneous contrast medium enhancement in the arterial phase of CT. Hyperenhancement of pancreatic metastases from RCC plays an important role in both the detection of tumor locations and the distinction of metastases from primary adenocarcinoma of the pancreas []. When hypervascular pancreatic tumors are identified on enhanced CT scans, differentiation from primary pancreatic endocrine tumors, intrapancreatic accessory spleens and vascular lesions is difficult. Palmowski et al. [] observed two types of enhancement on CT scans of pancreatic metastases, namely lesions with either a homogeneous enhancement or a highlighted rim and nonenhancing internal components, depending on the size. In lesions greater than 15 mm in size, rim enhancement with hypodense central areas of necrosis has been observed on CT scans []. This hypodense aspect is associated with colonic metastases, hyperdense attenuation with RCC, or breast cancer [,]. We reviewed the radiological features of pancreatic metastases from RCC shown by dynamic CT scans (Table ). Of 66 patients, 45 (68%) had homogeneous hypervascular enhancement features, whereas 21 (32%) had central hypovascularity with rim enhancement. Of these, 31 patients had a metastatic configuration, 5 patients had multifocal metastases to the pancreas and 26 patients had solitary tumors. No patients with pancreatic metastasis from RCC developed diffuse enlargement of the pancreas. In the present review, the size of pancreatic metastases from RCC has no particular relation with the presence of central hypodense areas because even tumors > 5 cm in size displayed hypervascular attenuation. The present case was classified as one with multifocal metastases in terms of the metastatic location. To our knowledge, this is the first case of concomitant multifocal metastases featuring two different enhancement characteristics, hypodense areas and homogenous hypervascularity.
FDG-PET has not been established for the diagnosis of metastatic RCC. Ramdave et al. [] reported that FDG-PET was useful for identifying distant metastases from RCC in all six of the patients in the study. Majhail et al. [] calculated the sensitivity and specificity of FDG-PET for identifying distant metastases from RCC. They revealed that the sensitivity of the procedure was linked to the size of the metastases (83% for lesions larger than 15 mm; 93% for those larger than 20 mm). In the present patient with 15- and 8-mm lesions, FDG did not indicate any abnormal metabolic activity in either lesion.
Surgical resection of the pancreas is associated with substantial morbidity after surgery, and the survival benefit of surgery for metastatic lesions of the pancreas remains questionable since randomized control trials have not been conducted. However, surgical resection of metastatic deposits of RCC remains the most effective treatment because chemotherapy, immunotherapy and radiotherapy have generally proved to be ineffective for metastatic RCC [,,,]. We reviewed studies published from 1998 to 2013 that focused on surgical resection of the pancreas for metastases from RCC. The survival rates and features are listed in Table . The median 5-year overall survival rate after metastasectomy was reported to be 75% to 88%, compared to 0% to 50% without metastasectomy [,,]. In a review, Masetti et al. [] analyzed data for 159 patients who underwent metastasectomy of the pancreas for RCC: the median survival and 5-year survival rate were 5.8 years and 63.5%, respectively. Tanis et al. [] reviewed 170 articles, and data for a total of 411 patients who underwent resection of pancreatic metastases were analyzed. They reported a pancreatic recurrence rate of 4.0% after a median of 42 months and an extrapancreatic recurrence rate of 17.1%. The 5-year survival rate was 72.6%. The surgical mortality rate after pancreaticoduodenectomy for pancreatic metastases was reported to be 2.6% []. Sellner et al. [] compared the 5-year overall survival rate of patients with a solitary metastasis with those with multiple metastases (solitary metastasis, 64%; multiple metastases, 78%). Combining the findings outlined in several reports [-], radical surgery for multifocal metastases from RCC in the pancreas appears to be as justified as that for a solitary metastasis. Radical surgery with the R0 resection is the only curative option for patients with pancreatic metastases. Therefore, surgical resection for pancreatic metastases should be considered under individualized conditions including the management of comorbidities.


A 46-year-old woman with a past medical history significant for hypertension presented to the ED with an eight-week history of increasing pain and swelling of the right neck. Over the past 3 weeks the patient had developed odynophagia to solid foods and had intermittent fevers and chills. She denied any recent neck trauma but admitted to a nearly 30 lb. weight loss over the past two months. She denied recent dental pain and dental work. She did not have any recent abdominal pain or diarrhea. There was no history of raw milk consumption or undercooked meat exposure.
The patient was born in Puerto Rico and immigrated to New York one month before her symptoms. She denied history of tuberculosis contacts or intravenous drug use and had never been tested for HIV. The patient had one kitten and several ducks as pets. On presentation the patient was tachycardic and febrile (39°C). Physical exam revealed a tender, erythematous, nonfluctuant 5 × 6 cm mass in the right neck extending from the angle of the mandible to the hyoid bone. No other lymphadenopathy was identified. Oral cavity exam revealed good dentition. Flexible fiberoptic nasolaryngoscopy was unremarkable. Initial laboratory studies revealed a white blood cell count of 12.5 × 109/dL, with 89% polymorphonuclear leukocytes, and serum glucose of 350 mg/100 mL. Chest X-ray demonstrated clear lung fields, with a right sided neck mass. A computed tomgraphy (CT) scan was performed demonstrating a 4.6 × 4.2 × 6 cm multiloculated mass in the right neck involving the sternocleidomastoid muscle ().
The patient was immediately taken to the operating room for incision and drainage of the neck mass. Intraoperatively 20 cc of purulent discharge was evacuated and a Penrose drain was placed. The patient was given 3 grams IV ampicillin-sulbactam every six hours and insulin was used for blood glucose control. A Mantoux purified protein derivative (ppd) was placed which was subsequently negative. The patient had an uneventful postoperative course with normalization of her white blood count on postoperative day number 2. The Gram stain of the abscess culture revealed intracellular Gram-negative bacilli (). The TSI slant result was alkaline/acid with production of hydrogen sulfide gas (). The patient was switched to oral ciprofloxacin 500 mg twice daily on postoperative day 3 when cultures grew back Salmonella enterica serotype Enteritidis sensitive to ceftriaxone, ampicillin, ciprofloxacin, trimethoprim-sulfamethoxazole, and ertapenem. No other pathogens were recovered. Ciprofloxacin susceptibility was verified via E-test. Blood cultures as well as stool cultures were negative. A right upper quadrant ultrasound revealed no evidence of cholecystitis. A human immunodeficiency virus (HIV) test was negative. Hemoglobin A1c was elevated at 12.6%. The patient was discharged home on postoperative day number five with resolution of symptoms to complete a fourteen-day course of ciprofloxacin with appropriate blood glucose control.


A 55-year-old man with no known past medical history presented to the hospital with a four-week history of swelling and pain in the right side of the neck. The patient experienced dysphagia to solids as well as subjective fever accompanied by chills. He denied any trauma to the neck but did have 15 lb. weight loss. He did not have any recent dental pain or dental work. He had not experienced abdominal pain or diarrhea over the preceding month but did admit to polydipsia and polyuria. There was no history of raw milk consumption or undercooked meat exposure. The patient was born in Mexico and had been living in New York for three years, without any other travel history. He denied history of tuberculosis exposure or intravenous drug use and had never been tested for HIV. He had no pets in New York or in Mexico. On presentation the patient was mildly tachycardic and febrile (38.5°C). Physical exam revealed a tender, erythematous, nonfluctuant 6 × 7 cm mass in the right neck extending from the mandible of the jaw. No other lymphadenopathy was identified. Oral cavity exam revealed good dentition. Initial laboratory studies were remarkable for a white blood cell count of 8.6 × 10/dL, with 79% polymorphonuclear leukocytes and serum glucose of 180 mg/100 mL. Chest X-ray revealed clear lung fields. A computed tomography (CT) scan of the neck demonstrated a 4 × 7 cm multiloculated mass in the right neck involving the parapharyngeal space with extension around the right carotid artery ().
The patient was started on 3 grams of ampicillin-sulbactam intravenously every six hours. A scant amount of pus expressed superficially was sent for culture. Two days later the patient was taken to the operating room for incision and drainage. Intraoperatively 30 cc of purulent discharge was evacuated and a Penrose drain was placed. Insulin was used for blood glucose control. Two sets of blood cultures were negative for infection. The Gram stain of the abscess culture revealed Gram-negative bacilli with the TSI slant alkaline/acid. The culture yielded Salmonella enterica serotype Typhimurium, sensitive to ceftriaxone, ampicillin, ciprofloxacin, trimethoprim-sulfamethoxazole, and ertapenem. No other pathogens were recovered. Ciprofloxacin susceptibility was confirmed via E-test. Stool cultures and an HIV test were negative. A right upper quadrant ultrasound revealed no evidence of cholecystitis. The patient's hemoglobin A1c was 9.6%. The patient was discharged on postoperative day number four with resolution of symptoms to complete a fourteen-day course of oral ciprofloxacin 500 mg twice daily along with metformin for glucose control.


A 36-year-old woman presented with a two-month history of increasing fatigue and abdominal fullness with accompanying loss of appetite. There was no history of fever, jaundice, or bleeding manifestations. There was no history of any antecedent haematological disorder. On examination, the patient had pallor and splenic enlargement, measuring 22.9 cm in ultrasonography. Physical examination was otherwise unremarkable. Complete haemogram findings included haemoglobin of 7.5 g%, red blood cell (RBC) count of 3560 × 109/L, total leucocyte count of 27.9 × 109/L, and a platelet count of 364 × 109/L.
The peripheral smear showed microcytic hypochromic erythrocytes with many dacrocytes, polychromatophilic RBCs, and normoblasts. The leucocyte differential count was blasts 26%, myelocytes 7%, metamyelocytes 6%, basophils 4%, eosinophils 2%, lymphocytes 10%, and neutrophils and band forms 45%. The blasts showed scant basophilic cytoplasm, round nuclei with fine chromatin, and 2–4 nucleoli. Some of the blasts showed cytoplasmic blebs or projections (). The neutrophils showed dyspoietic features such as hypo- and hypersegmentation, Pelger–Huet anomaly, and hypogranulation. Many giant platelets and platelet aggregates were seen. Individual platelets showed marked variation in size ().
Bone marrow aspiration and biopsy showed increased abnormal megakaryocytes and bone marrow fibrosis (). Gomori’s trichrome and reticulin stains revealed a Grade II fibrosis. Monolobated and multinucleated megakaryocytes with hyperchromatic and pleomorphic nuclei were seen () and showed strong positive staining for CD 61 ().
A qualitative assay for breakpoint cluster region–Abelson (BCR-ABL) translocation was done on peripheral blood leucocytes using reverse transcriptase polymerase chain reaction and gel electrophoresis. The hybrid transcript for BCR-ABL was detected, and the genomic breakpoint was observed at e14a2 that corresponds to p210.
The patient was started on induction therapy with etoposide, cytarabine, and Adriamycin along with Imatinib Mesylate. She has received two cycles of chemotherapy with the above drugs and was maintained on Imatinib. Her peripheral blood blast count at the end of second cycle was 16%. The patient received chemotherapy again, because of the increased blast cell count. No stem cell transplantation was given due to patient’s non-compliance. No further cytogenetic studies were done to detect additional abnormalities or to assess molecular remission. The patient expired in March 2013 due to blast crisis.


A male patient aged 4 years and 3 months presented to the ER (emergency room) of our hospital with vomiting, diarrhea, and seizures. On day 1 of the illness, he had developed gastrointestinal symptoms, including vomiting and watery diarrhea that occurred 4-5 times per day. Four days later, bilateral generalized tonic-clonic seizures began, for which he was transported to our hospital. The seizures lasted up to 1 minute and subsided spontaneously. The patient was in a restless state of consciousness (level E3V4M5 according to the Glasgow Coma Scale and level II-10 according to the Japan Coma Scale). He stared, averted his gaze, continued to cry, and threw objects. This disturbance of consciousness persisted for 7 hours. The patient was sedated with midazolam, and MRI was performed. On awakening after 2 hours, the patient had regained a normal state of consciousness, after which there was no recurrence of disturbance of consciousness or seizures.
On admission, the patient's vital signs were as follows: temperature, 37.7°C; heart rate, 130 beats/minute; blood pressure, 100/60 mmHg; and SpO2, 98% (room air). No central nervous system abnormality, meningeal irritation, paralysis, or abnormalities in the thoracoabdominal region were observed. MRI revealed a hyperintense signal in the splenium of the corpus callosum on DWI and a hypointense signal in the ADC-map (). The patient's peripheral circulation was good.
The patient's medical history was unremarkable. He was born by spontaneous cephalic delivery after 39 weeks and 3 days of gestation (birth weight, 2664 g). No remarkable observations were noted during the pregnancy or perinatal period. The patient's growth and development were normal. The family medical history revealed that the father had febrile seizures.
The results of the patient's biochemical blood tests were as follows: WBCs, 3900/μL; Hb, 13.4 g/dL; Plt, 26.1 × 104/μL; BUN, 16 mg/dL; Cre, 0.3 mg/dL; Na, 132 mEq/L; K, 4.5 mEq/L; Cl, 101 mEq/L; AST, 41 IU/L; ALT, 21 IU/L; CRP, 2.59 mg/dL; Glu, 84 mg/dL; lactic acid, 7.8 mg/dL; pyruvic acid, 0.32 mg/dL; NH3, 49 μg/dL; pH, 7.424; PCO2, 39.6 mmHg; HCO3, 25.5 mmol/L; and BE, 1.6 mmol/L. No abnormality was detected on blood amino acid analysis or tandem mass screening. Cerebrospinal fluid (CSF) examination revealed a cell count of 4 cells (all mononuclear), a glucose concentration of 95 mg/dL, and a total protein concentration of 16 mg/dL.
On day 2 of admission, an electroencephalogram was obtained during sedated sleep. The spindle wave was appropriate for the patient's age. No bursts were observed. The patient's stool was positive for RV antigen. RT-PCR did not reveal RV RNA in the blood or CSF. RT-PCR analysis of RNA extracted from the stool revealed that the RV genotype was G5P[6].
On day 5 of admission, no recurrences were observed, and the patient was discharged. Eight days after admission, all abnormal signals had disappeared (). The clinical course was typical of MERS.


A 25-year-old woman was admitted to our otorhinolaryngology clinic at Quaem Hospital at Mashhad University of Medical Sciences with a complaint of a non-tender, rapidly growing mass first observed five weeks earlier on her right auricle. There were no other accompanying symptoms.
On physical examination, a 2 × cm mass lesion was observed on the right auricle attached to the concha. The mass was fragile and easily became bloody by manipulation. It was not possible to examine the external auditory canal (EAC) and tympanic membrane, but the lesion appeared to be discrete from the EAC. No cervical lymphadenopathies were detected. A thorough whole-body skin examination revealed no other lesions. With a pre-diagnosis of a pyogenic granuloma, and also to rule out the malignant lesions such as sarcoma, a punch biopsy was taken. At subsequent 1-week follow up, the lesion had grown to approximately 3 × 4.5 cm ().
Microscopic findings showed a proliferation of plump, mitotically active spindle-shaped cells with oval, pale-staining nuclei and short irregular bundles in a loosely mucoid matrix with extravasated red blood cells and multinucleated giant cells. An immunohisto- chemical (IHC) panel for cytokeratin (CK), S-100, epithelial membrane antigen (EMA), smooth muscle actin (SMA), and vimentin (Vim) was performed. On IHC study, CK, EMA, and S-100 were negative, and SMA and Vim were expressed, which confirms the diagnosis of NF ().
According to the pathologic diagnosis of nodular fasciitis, the patient underwent an excision of the lesion under general anesthesia. The basal skin of the lesion was completely excised and the surrounding skin was primarily repaired. There was no cartilage invasion. The permanent pathologic result was also compatible with nodular fasciitis. There was no recurrence after a year.


In June 2009, a 69 year old female patient was diagnosed with PTCL not otherwise specified (PTCL-NOS) after complaining of hoarseness. Staging procedures revealed a single manifestation with involvement of the epipharynx with no evidence of B symptoms (Ann Arbor stage IE A). The international prognostic index (IPI) and the prognostic index for T-cell lymphoma (PIT) were low (age above 60 years as the only risk factor). The patient was otherwise healthy with no relevant concomitant diseases. Following two cycles of chemotherapy with CHOP-21 (cyclophosphamide 750 mg/m2 day 1, vincristine 1.4 mg/m2 day 1, doxorubicin 50 mg/m2 day 1, and prednisolone 100 mg absolute day 1–5), she achieved a partial remission, but showed progressive disease after completing six cycles CHOP-21 with new enlarged cervical lymph nodes on both sides of the neck. The patient was scheduled for myeloablative chemotherapy with autologous stem cell transplant using a DHAP protocol (cisplatin 100 mg/m2 day 1, cytarabine 2.000 mg/m2 bid day 2, and dexamethasone 40 mg absolute days 1–4) for tumor reduction and stem cell collection. Following two courses of DHAP, the patient again had progressive disease showing further increase of the cervical lymph nodes and new supraclavicular lymph nodes. A third line chemotherapy regimen with DexaBEAM (dexamethasone 8 mg absolute TID day 1–10, carmustine 60 mg/m2 day 2, melphalan 20 mg/m2 day 3, etoposide 75 mg/m2 day 4–7, and cytarabine 100 mg/m2 BID day 4–7) did not result in lymphoma remission; due to chemo-refractory disease, the myeloablative concept was abandoned (Figure
).
The patient was enrolled in January 2010 in the multicenter, open-label BELIEF-trial (Phase II study PXD101-CLN-19). Belinostat was started at a dose of 1000 mg/m2 for days 1–5 in a 21 day cycle. In April 2010 following two cycles of belinostat, the patient achieved a complete response (CR, Figure
). After 1 year of belinostat therapy (~ 12 cycles), the interval between cycles was extended from 21 days to 42 days due to the long lasting remission, and to avoid potential toxicities. The patient has received a total of 28 cycles with the last cycle completed in December 2012, and maintains a CR at her last follow-up in July 2013.
In general, belinostat therapy was tolerated and administered for 28 cycles. Hematologic side effects were mild without any toxicity more than CTC grade 2. However, after cycle 2 the patient developed > 2 grade non-hematologic toxicity: CTC grade 3 relapsing bipulmonary alveolitis which was successfully treated with steroids: CTC grade 3 repeated episodes of Clostridium difficile toxin-associated diarrhoea during the first 4 months of treatment, that responded to metronidazole: CTC grade 3 antibiotic resistant atypical pneumonia while on steroid medication, that responded to treatment with voriconacole, suggesting pulmonary fungal infection. All of the non-hematologic adverse events except for the alveolitis were not considered related to belinostat treatment.


The patient, a 52-year-old healthy woman without a significant past medical history, presented to a community care center complaining of 5 days of productive cough, diffuse myalgia, and subjective fever with chills. She was prescribed a course of azithromycin for presumed bronchitis. The following day, she presented to her local emergency department with acute onset of severe shortness of breath and associated chest pain. She was found to be hypotensive (90/60 mmHg), tachycardic (115 beats/min), and was admitted to the intensive care unit (ICU) with a presumptive diagnosis of septic shock. Norepinephrine and empiric IV vancomycin and piperacillin/tazobactam were started. Initial labs revealed: white blood cell count (WBC) 12.7/μL with 77.9% neutrophils, hemoglobin 12.7 g/dl, hematocrit 39.8%, platelets 141, lactate 8.2 mmol/L, sodium (Na) 149 mmol/L, potassium (K) 4.3 mmol/L, bicarbonate 16 mmol/L, chloride (Cl) 115, blood urea nitrogen (BUN) 28, creatinine (Cr) 1.3 mg/dl, blood glucose 214 mg/dl, aspartate aminotransferase (AST) 128 IU/L, alanine aminotransferase (ALT) 200, alkaline phosphatase (AP) 104 IU/L, serum troponin I 12.6 ng/ml, and creatine phosphokinase (CPK) 339 U/L. Her electrocardiogram revealed an accelerated junctional rhythm with a rate of 115 beats/min, poor R wave progression and low voltage. An emergent transthoracic echocardiogram (TTE) showed severely impaired left ventricular (LV) function with an estimated ejection fraction (EF) of 10%. Within 24 hours of presentation her condition deteriorated and she required intubation and mechanical ventilation. The patient then was transferred to our center for further management.
Upon arrival the patient was intubated, sedated, and afebrile. Her blood pressure was 70/40 mmHg and she had a heart rate of 110 beats/min. Examination revealed bilateral crepitations in the lungs, normal heart sounds and a benign abdomen. Her skin was mottled; and her extremities were cold and clammy. There was no pedal edema. Chest radiograph revealed diffuse bilateral patchy infiltrates and pulmonary vascular congestion. A repeat TTE demonstrated a left ventricular ejection fraction of 10%, normal LV and RV dimensions, moderate mitral regurgitation, left ventricle wall thickness of 12 mm, and global hypokinesis of LV with minor regional variation. There was no evidence of a pericardial effusion. Notable labs were a WBC 14.8/μL with 82% neutrophils, hematocrit 36.5%, platelets 113, CPK 365 U/L, troponin 7.6 ng/ml and Cr 0.76 mg/dl. The patient remained hypotensive despite maximum doses of norepinephrine, dobutamine and vasopressin. She was continued on empiric antibiotics and empiric oseltamivir (75 mg twice daily) was added. Immediate left and right heart catheterization demonstrated normal coronary anatomy, with a cardiac index of 1.16 L/min/m2. Emergent venoarterial ECMO (flow = 4 L/min) was placed via left common femoral vein and artery.
On day 3 of hospitalization, pulmonary congestion significantly improved but another repeat TTE showed persistent severe LV systolic dysfunction with an EF of 15%. Endotracheal aspiration at admission returned positive for influenza B by polymerase chain reaction (PCR). Right ventricular endomyocardial biopsy was performed on hospital day 3 (total 4 specimens). Histopathology was negative for inflammation. Immunohistochemical stain for parvovirus B19, HHV6, HSV and PCR for enteroviruses, EBV and CMV were all negative. Bronchoscopy revealed mild mucosal erythema with clear secretions. The bronchoalveolar lavage (BAL) fluid was negative for enteroviruses, adenoviruse, CMV, EBV and HSV by PCR. Blood, BAL and urine cultures were negative for bacterial growth and empiric antimicrobials were stopped on day 5 and oseltamivir was continued for a total of 7 days.
The patient’s clinical status gradually improved, inotropic support was stopped and a repeat TTE on day 7 showed significant recovery of left ventricular function with an estimated EF of 50%, and ECMO support was able to be withdrawn.
The patient was extubated on hospital day 8; low doses of a β -blocker and an angiotensin converting enzyme inhibitor were started. The patient’s course was complicated by a pseudoaneurysm of the left common femoral artery secondary to the ECMO therapy, which was repaired successfully. She was discharged on hospital day 15. Transthoracic echocardiography one month after discharge demonstrated normal LV systolic function with an EF of 60%, normal LV wall thickness, trace mitral regurgitation and no ventricular dilation.
During the 1918–1919 influenza pandemic an association between myocarditis and influenza viruses was noted during an autopsy study []. While there have been reports of fulminant myocarditis or cardiomyopathy associated with influenza B in children, it has very rarely been reported in adults [,-]. In 1958, a group of four patients with pericarditis, subacute myocarditis, and fatal chronic myocarditis associated with influenza B were documented []. Ray CG et al. described a 34-year-old healthy woman who presented with acute onset of dilated cardiomyopathy and cardiogenic shock following a few days of flu like syndrome. She had a very complicated course and died 6 weeks after the onset of symptoms. Influenza B virus was isolated from the nasopharynx and the diagnosis of acute myocarditis was confirmed by cardiac autopsy [].
McCarthy et al. described fulminant myocarditis as a distinct clinical presentation of abrupt onset of severe heart failure and cardiogenic shock preceded by a viral syndrome similar to our patient []. The typical echocardiographic findings in fulminant myocarditis are described as near normal ventricular dimensions, severely depressed LV systolic function, and increased left ventricle wall thickness []. Histological diagnosis of fulminant myocarditis can be difficult especially in the early phase of disease. The limited sensitivity of cardiac biopsy for detection of myocarditis is well recognized and negative cardiac biopsies results cannot exclude the diagnosis of myocarditis []. Myocarditis usually presents as a focal or patchy infiltration predominantly in the lateral free wall of the left ventricle and often not involving the right ventricle, therefore, biopsies taken from the right ventricle could be falsely negative [].
This patient’s clinical presentation of refractory cardiogenic shock 6 days after the onset of influenza and severely impaired left ventricular systolic function, and subsequent normalization of ventricular function after recovery of her viral illness; taken together with her typical echocardiogram findings in the setting of normal coronaries supports the clinical diagnosis of viral fulminant myocarditis; regardless of non diagnostic right ventricle biopsies. During influenza infection, severe myocardial dysfunction can be caused not only by direct injury to cardiac myocytes but also by overexpression of cytokines and severe inflammatory response to viral infection resulting cytokine and humoral mediated forms of myocarditis with no cellular infiltrate.
The reason for the unexpected severity of influenza B infection in our patient was not clear. Clinical data about risk factors and complications of influenza B are limited. Her only past medical history was remote myasthenia gravis and thymectomy over 20 years prior. The predominant circulating B strain was B/Wisconsin/1/2010- LIKE when the patient presented to the hospital, which was matched with the B component of the 2012–2013 vaccine []. We do not know if the patient had received an influenza vaccine this season. The possible lack of immunologic memory for this infection might have contributed to the severity of influenza B virus infection in our patient. Increasing awareness among patients and physicians about the importance of vaccination as the most important strategy for minimizing the severe complications of influenza infection could help to prevent similar cases in the future.
The treatment of influenza associated fulminant myocarditis still remains supportive. Studies have shown the early use of mechanical circulatory support for patients with fulminant myocarditis and cardiogenic shock who fail aggressive pharmacologic treatment would increase survival rate [,]. Our patient received oseltamivir, standard heart failure treatment but more important early initiation of ECMO support before irreversible organ failure developed.


The patient was a 38-year-old female (gravida 1,
para 0) with a 20-year history of infertility. In 2009
she underwent a hysteroscopy with resection of a
broad, short septum. She became pregnant during
the fourth intra-cytoplasmic sperm injection (ICSI)
treatment at Royan Institute. The patient presented
with a significantly elevated beta human choronic
gonadotropin (beta hCG) level of >100 mIU/mL
two weeks after embryo transfer (ET). Four weeks
later, the beta hCG level was 1630 IU/mL. The first
transvaginal ultrasound at 6.5 weeks gestational age
showed an embryo, 5 mm in length with cardiac activity
that was implanted in the cervical canal. No
intrauterine pregnancy was seen. Since the pregnancy
was so important for the mother, she was followed
with repeated ultrasound studies. One week
later, ultrasound confirmed a seven-week gestational
age embryo with cardiac activity in the cervical
canal (Figs , ). She was referred to the hospital
for medical treatment. Initially, 1cc of 10% KCl was
injected in the gestational sac, followed by injection
of 5cc methotrexate (50 mg) in the gestational
sac and its surroundings. After two intramuscular
methotrexate injection the β-human chorionic gonadotropin
(β HCG) decreased. The complete disappearance
of the gestational sac was confirmed by
a follow-up ultrasound and the level of beta hCG
immediately reduced during the weekly follow-ups
(<5 mIU/mL).


A 59-year-old Japanese man presented for treatment of an intra-cardiac thrombus. At 52 years of age, he was diagnosed with mid-ventricular obstructive hypertrophic cardiomyopathy (HCM) associated with paroxysmal atrial fibrillation. Electrocardiography revealed poor R-wave progression at the V4 and V5 leads, and echocardiographic images demonstrated asymmetrical septal hypertrophy and mid-ventricular obstruction associated with paradoxical diastolic flow (Figure ). Although the global left ventricular ejection fraction was not markedly impaired, aneurysm formation was observed at the left ventricular apex (Figure ), and technetium (99mTc) tetrofosmin single-photon emission computed tomography demonstrated severe hypoperfusion in this area (Figure ). Coronary angiography was concurrently performed, but revealed no atherosclerotic lesions in the coronary arteries. At 55 years of age, the patient developed sustained ventricular tachycardia associated with hemodynamic collapse, for which an implantable cardioverter-defibrillator (ICD) was inserted. Although the administration of bisoprolol (5mg/day) and amiodarone (200mg/day) were continued for the suppression of ventricular tachycardia after ICD implantation, anti-coagulation therapy was not initiated because no thrombus was detected on two echocardiographic examinations of the patient at 52 and 55 years of age. During the most recent echocardiographic examination, however, an asymptomatic thrombus (15.0mm×17.0mm) was detected in the apical aneurysm (Figure ). Therefore, hospital admission was recommended for anti-coagulation therapy with continuous heparin infusion and warfarin administration. However, because the patient obstinately declined hospital admission and requested the continuation of medical treatment at an ambulatory clinic, dabigatran administration (150mg b.i.d.) was initiated. Coagulation markers were measured before the commencement of anti-coagulation therapy, and all were within the normal range (Figure ). Following dabigatran administration, an approximately two-fold increase in activated partial thromboplastin time (measured four hours after dabigatran administration with meals) was observed and the prothrombin time internationalized ratio (PT-INR) was increased (Figure ). However, the levels of D-dimer, thrombin-anti-thrombin III complex (TAT) and fibrinogen were not significantly altered (Figure ). Moreover, weekly echocardiographic examinations demonstrated a gradual decrease in thrombus size (Figure ). Echocardiographic examinations performed one week after initial dabigatran administration showed that thrombus size had decreased to 10.0mm×9.0mm. Thrombus size had further decreased to 6.8mm×6.0mm after two weeks, and no thrombus was detected after three and four weeks. During the four-week treatment period, no systemic thromboembolic events occurred. Thus, the thrombus appears to have been resolved after dabigatran administration. However, dabigatran administration was continued even after thrombus resolution.


A 77-year-old male presented with painless obstructive jaundice. He had a history of right nephrectomy for a T2N0M0 renal clear cell carcinoma 3 years ago. Ultrasound and abdominal computed tomography (CT) scanning depicted the common bile duct dilated up to its distal end. Endoscopic retrograde cholangiopancreatography (ERCP) revealed an ampullary tumor (Figure ). Consequently, ampullectomy with endoscopic sphincterotomy and placement of a plastic 10 Fr biliary stent were performed (Figure A).
Histology showed a clear cell carcinoma, consistent with renal origin. Immunochemistry confirmed the diagnosis [vimentin(+), CD10(+), CK8(+), RCCa antigen(+)]. Further evaluation with chest CT and radionuclide bone scanning revealed the presence of lung and bone metastases. The presence of multiple metastatic foci excluded the need for pancreatoduodenectomy and the patient was treated with oral sunitinib. For better palliation, repeat ERCP was performed a month later and additional excision of remnant tissue was performed in combination with argon plasma coagulation (APC) and placement of a partially covered metallic biliary stent (Wallstent; Boston Scientific, Natick, MA) (Figure B). Six months later the stent was removed and multiple biopsies showed no evidence of residual tumor (Figure ). The patient was re-evaluated with endoscopy every 6 months. The patient succumbed to metastatic disease 1.5 years later without jaundice or abnormal liver function tests.


We present the case of a 24-year-old newly married woman, who was admitted to our hospital from the Emergency Department complaining of acute and constant left flank pain for 3 days with macroscopic haematuria.
This pain was nonradiating, was not related to meals, did not respond to over-the-counter analgesics, and was not associated with anorexia, nausea, vomiting, or fever. This pain was preceded by macroscopic haematuria that appeared for 2 months before.
Personal and family history was noncontributory; especially she denied any history of surgeries, chronic illness, recent immobilisation, recent prolonged travel, or trauma or oral contraceptives. She was not pregnant. No thrombogenic factors were identified.
She reported no change in her bowel habits.
The patient's vitals were stable and exam revealed isolated left costovertebral angle tenderness. Physical examination revealed also a height of 172 cm and weight of 51 kg with a lower BMI at 17.2.
Her initial labs revealed only haemoglobin at 9.0 mg/dL.
Computed tomographic angiography (CTA) was performed demonstrating left renal vein thrombosis (), regardless of renal tumor or hydronephrosis. The CTA also showed a compression of the left renal vein (LRV) between the aorta and the superior mesenteric artery (SMA), SMA angle (the angle between aorta and SMA) was approximately 10°, the posteroanterior diameter of the hilar portion and that of the aorticomesenteric stenotic portion of the LRV were 9.9 mm and 1.0 mm, respectively (), and the diagnosis of anterior nutcracker syndrome leading to left renal vein thrombosis was confirmed.
When we asked her again, she reported a long history of various symptoms including intermittent macroscopic haematuria and chronic moderate left lumbar pain aggravated by physical activity, associated with systemic signs dominated by chronic fatigue and headache, which lasted for his childhood, despite a long investigational history combined with several imaging examinations and laboratory tests. Urinalysis revealed 4+ proteinuria, and 24 hour urine collection analysis showed 2 g of proteinuria.
Based on clinical presentation, urinary and systemic symptoms, laboratory reports, and computed tomographic angiography finding, a diagnosis of anterior nutcracker syndrome leading to left renal vein thrombosis was confirmed.
Anticoagulation was started by a low molecular weight heparin bridge to warfarin. Three weeks later, CTA showed complete disappearance of the renal vein thrombosis ().
The patient was informed of her diagnosis and there was a discussion about possible treatment options. We proposed transposition of left renal vein that the patient consented to.
Since she was newly married, it was decided to initially bridge her to warfarin with plans for 6 months of anticoagulation followed by repeated imaging. Need for additional management would be decided at that time.


The patient was an 89-year-old woman, American Society of Anesthesiologists Physical Status 3, with hypertension, hypothyroidism, and a 58-pack-year history of smoking who five years prior had undergone a left lung lower lobectomy for cancer. She was scheduled to undergo a right total shoulder replacement for worsening degenerative disease and pain. Pulmonary function testing performed 17 months prior to surgery revealed a FEV1/FVC ratio of 0.68, indicating mild obstructive disease, and a diffusion capacity (DLCO) of 9.5 mL/mm Hg/min, indicating a moderate gas transfer defect. Physical examination revealed clear lung fields bilaterally and a short hyomental distance on airway exam. Preoperative pulse oximetry on room air revealed an oxygen saturation of 100%. The patient and her family wished to proceed with surgery only under regional anesthesia after consulting with her primary care physician. After discussion of the risks and benefits of regional anesthesia, including the possibility of impaired lung function, pneumothorax on the operative side postoperatively, and mechanical ventilation postoperatively, the patient agreed to perform surgery under a continuous interscalene nerve block (CISB) with light sedation.
The block was performed using continuous ultrasound guidance (GE Logic E, Wauwatosa, WI) and nerve stimulation (B. Braun, Bethlehem, PA). An in-plane, posterior approach technique was utilized for needle insertion and visualization based on the preference of the anesthesiologist performing the procedure (). A total of 30 mL of ropivacaine 0.5% was injected incrementally after negative aspiration to the area adjacent to the C5 and C6 nerve roots and a 20 g multiorifice peripheral nerve catheter (B. Braun Medical Inc., Bethlehem, PA) was inserted 5 cm beyond the needle tip. Sensory block was confirmed with decreased pinprick sensation in the C5 and C6 dermatomes. The patient was given a propofol infusion of 15–25 mcg/kg/min for sedation with oxygen via nasal cannula and surgery proceeded uneventfully.
In the postoperative anesthesia care unit (PACU) the patient had excellent analgesia. Intravenous fentanyl and morphine were available but she requested no rescue opioids or other medications. Close postoperative monitoring was continued on a surgical ward with frequent pulse oximetry measurements. Analgesia consisted of a multimodal regimen including CISB with ropivacaine 0.2% running at 8 mL/h without a demand function and ketorolac, aspirin, and pregabalin, with a morphine PCA for breakthrough pain. She consumed the equivalent of morphine 17.5 mg IV for breakthrough pain during the first 24 h postoperatively. She reported no side effects and denied significant dyspnea while the catheter was in place, receiving no more than 2 L/min of oxygen via nasal cannula as a precautionary measure during her admission. She used her incentive spirometer multiple times per day. She was discharged home on postoperative day no. 2 after the continuous interscalene catheter had been removed.


A 40-year-old woman originally from Ecuador was referred to our institution for evaluation of progressive dyspnea on exertion. Two months prior to her presentation, she was treated at an outside hospital for presumed community acquired pneumonia with symptoms of dyspnea on exertion, fevers, chills, and productive cough. After not seeing measurable improvement, the patient then presented to the emergency department at another institution where a transthoracic echocardiogram (TTE) revealed that the patient had a large pericardial effusion with tamponade physiology and a normal left ventricular ejection fraction (LVEF) of 60%. Pericardiocentesis was performed with 700 cc of fluid removed, which grew Mycobacterium tuberculosis (TB). The patient's purified protein derivative (PPD) skin test was positive. The patient was started on anti-TB therapy and discharged.
She continued to complain of persistent dyspnea on exertion and thus was referred to our institution for a repeat of TTE at the outpatient cardiac diagnostic center. TTE revealed the rare presystolic flow through pulmonic valve, excessive variation in the mitral valve and aortic valve spectral Doppler velocities, and markedly thickened pericardium (Figures –), consistent with constriction []. The study also demonstrated septal bounce, small pericardial effusion, and pleural effusion. The patient was admitted for further evaluation. On admission, the patient's vital signs showed a temperature of 37 degrees Celsius, a blood pressure of 108/70, a heart rate of 117, and respiratory rate of 20 with an oxygen saturation of 99% in room air. Her exam revealed a young woman in mild distress with 11 cm internal jugular vein distension, decreased breath sounds at lung bases bilaterally and scant bibasilar crackles, regular tachycardia, and bilateral lower extremity edema. Her BNP was 124, CRP was 5.1, and WBC count was 5.9 with a normal differential. Her complete metabolic panel was unremarkable. The patient underwent a left and right heart catheterization due to the suspicion of constrictive pericarditis. A coronary angiogram demonstrated no obstructive epicardial coronary artery disease. The right heart catheterization revealed elevated right atrial pressure, right ventricular end diastolic pressure, and an elevated pulmonary capillary wedge pressure. Simultaneous right and left ventricular pressure tracings demonstrated discordant respiratory variation consistent with constrictive pericarditis. A computed tomography (CT) scan of her chest with contrast confirmed marked thickening of both the parietal and pleural pericardial surfaces, measuring up to 13.4 mm in thickness, and a large right-sided pleural effusion (Figures and ). Cardiac magnetic resonance imaging (MRI) with and without contrast showed diffuse pericardial thickening (), which showed intense delayed gadolinium contrast enhancement () that did not involve the myocardium. Additionally, there was a greater interventricular septal bounce during diastole and inspiration, consistent with chronic inflammatory constrictive pericarditis.
Due to her worsening symptoms and diagnosis of constrictive pericarditis, she was referred to cardiothoracic surgery at our institution for pericardiectomy. The surgery was without incident and the surgical samples were sent for pathology. While being negative for AFB by concentrated smear and culture, the stripped pericardium showed noncaseating granulomas, extensive fibrosis, and severe lymphohistiocytic infiltrate.
In the immediate postoperative period, the patient began to develop signs and symptoms of central volume overload, heart failure, and hypotension. An electrocardiogram (ECG) at this time showed sinus tachycardia with nonspecific ST-T abnormalities with poor R wave progression, new from prior ECGs. A subsequent TTE on postoperative day one showed significant changes from the prior study, with severely decreased biventricular dysfunction. The LVEF was severely depressed at 26%, with early evidence of elevated filling pressure within the left ventricle, indicated by the time difference between pulmonary vein (Ar) duration and mitral A-wave duration (Ar-A) of positive 46 ms. For the right ventricle, fractional area change (FAC) was abnormally low at 29%, with reduced tricuspid annular plane systolic excursion (TAPSE) distance of 0.96 cm. There was also moderate tricuspid regurgitation, enlarged right atrium (area 27 cm2), and moderately elevated pulmonary artery systolic pressure of 41 mmHg. There was akinesis of the entire apical and midventricular segments of both the right and left ventricles (Figures –) with hypercontractile base. Findings were consistent with stress cardiomyopathy.
While in critical condition postoperatively, the patient had episodes of atrial fibrillation and atrial tachycardia, for which she was chemically cardioverted with intravenous amiodarone, with subsequent conversion to sinus rhythm. Initially, the patient required multiple vasopressors and inotropes (epinephrine, norepinephrine, milrinone, and dopamine drip) to maintain her blood pressure and cardiac output, while gentle diuresis was achieved carefully for the symptoms of heart failure. Once the patient was hemodynamically stable, she was placed on appropriate oral medical therapy, including carvedilol and furosemide, followed by gradual clinical improvement and was able to be discharged on postoperative day 10. Due to decreased urine output and hypotension, an angiotensin-converting-enzyme inhibitor or angiotensin receptor blocker was not initiated at the time. A posthospital TTE 7 weeks after discharge showed normalized function of both the right and left ventricles (Figures –).


A 48-year-old Caucasian male was diagnosed with HIV-AIDS in 2009 after presenting with oral candidiasis. His absolute CD4 count was 38/mm3 with a viral load of 176,000 copies/mL. He began highly active antiretroviral therapy (HAART) with efavirenz/emtricitabine/tenofovir (Atripla, Bristol-Myers Squibb). Also present at the time of presentation was a melanoma on the right cheek, which was subsequently excised. Due to neuropathy, the patient was unable to tolerate lymphoscintigraphy, preventing sentinel node localization.
Two years later, he experienced progressive, left-sided facial, upper extremity, and lower extremity numbness and weakness. MRI of the head and spine revealed a hemorrhagic mass involving the right precentral gyrus measuring 2.7 × 3.5 × 3.1 cm with surrounding vasogenic edema (). He was admitted to Johns Hopkins Hospital and underwent a craniotomy for tumor resection. Pathologic evaluation confirmed metastatic melanoma.
Three weeks after surgery, he began treatment with ipilimumab, administered at the standard dose of 3 mg/kg of body weight every three weeks for four doses. One week following the first dose of ipilimumab, he received an SRS boost to the tumor bed of 2100 centigray (cGy) in 3 fractions prescribed to the 73% isodose line using Cyberknife. At the time of the planning MRI, two more brain metastases were noted: one in the right occipital lobe and another in the left frontal lobe, each measuring 7 mm in greatest diameter. Both were treated with SRS 2000 cGy in a single fraction to the 73% isodose line. A restaging FDG-PET/CT scan demonstrated multiple metastases in the right and left hepatic lobes, left iliac external lymph nodes, left deep inguinal nodes, left distal thigh, left popliteal fossa, left anterior tibialis muscle, and right femur.
Eleven days following SRS, he presented to the Emergency Department with weakness in his left upper extremity. An MRI revealed an interval increase in all brain lesions. The right occipital lobe lesion measured 2.4 cm while the left frontal lobe lesion measured 1 cm in greatest diameter. High dose steroids were prescribed as the interval increase in all brain lesions was likely a result of post-SRS inflammation and not true progression of underlying disease.
Interval MRI scans over the next 6 months showed stabilization of brain metastases while interval FDG-PET/CT imaging showed stabilization or complete resolution of extracranial lesions. Seven months after completion of SRS, a single new brain metastasis was seen in the left supramarginal gyrus and was treated using SRS 2000 cGy to the 73% isodose line in a single fraction. Representative pretreatment and 8-month posttreatment MR imaging are shown in .
At the time of paper preparation the patient is nine months post-surgery after resection of his first brain metastasis. He has remained on efavirenz/emtricitabine/tenofovir, though his adherence to the HAART therapy has been poor. His absolute CD4 counts have ranged from 126 to 449/mm3 with an undetectable viral load. He is able to function at his baseline. He experienced only minor gastrointestinal discomfort throughout his treatment with ipilimumab and SRS. His extracranial metastases have remained stable or resolved.


A 32-year-old woman was transferred to our hospital for the management of severe acute thrombotic microangiopathy resulting in multiorgan failure.
The patient was in her usual state of health until she experienced heavy menstrual bleeding. Two days later, she developed fatigue and dyspnea on exertion while at work. She returned home and went to bed early to rest. Overnight, she developed cough, shortness of breath, chest pain, nausea, and vomiting. She was transported to a local hospital for evaluation. Her past medical history was notable for several spontaneous miscarriages and recent hair loss. She was a native of Haiti and worked in a local restaurant in Miami. She denied use of alcohol, tobacco, and drugs. On physical examination, the patient was a well-developed young woman in respiratory distress. The temperature was 37.0°C, the blood pressure 121/66 mm Hg, the pulse 106 beats/minute, the respiratory rate 42 breaths/minute, and the oxygen saturation 79% while breathing ambient air. Auscultation of the lungs demonstrated diffuse crackles. Chest radiograph revealed extensive bilateral pulmonary infiltrates. Supplemental oxygen was administered. However, the patient developed hemoptysis and was immediately intubated.
Laboratory data are summarized under “Day 1” in . There were severe hemolytic anemia, profound thrombocytopenia, and severe renal disease leading to azotemia, hyperkalemia, anion-gap metabolic acidosis, proteinuria, and hematuria. Blood films revealed numerous schistocytes. In addition, there were leukocytosis, neutrophilia, monocytosis, and hypoalbuminemia. Coagulation studies demonstrated markedly elevated D-Dimer. Erythrocyte sedimentation rate was elevated. Serologic tests disclosed antibodies against nuclear antigens including double-stranded DNA, chromatin, Smith antigen, ribonucleoproteins, SSA/Ro, and SSB/La. Complement C3 level was reduced and C4 was low-normal. Antibodies against glomerular basement membrane were not detected. Indirect immunofluorescence staining for antineutrophil cytoplasmic antibodies demonstrated a weak cytoplasmic pattern (c-ANCA). Plasma ADAMTS-13 activity (a disintegrin and metalloprotease, with thrombospondin-1—like domains) was normal. Serologic tests for hepatitis B and C and HIV were negative. Transthoracic echocardiography revealed reduced ejection fraction (45–50%) without regional wall motion abnormality as well as moderate to severe mitral and tricuspid regurgitation.
The patient was diagnosed with severe acute thrombotic microangiopathy, DAH, renal failure, cardiomyopathy, and valvular heart disease in the setting of new-onset SLE. Over the ensuing two weeks, she was treated with high-dose glucocorticoids including intravenous methylprednisolone 1 g/day for three days, plasma exchange (a total of eight daily treatments), intravenous cyclophosphamide 1 g, and multiple blood transfusions (). Hemodialysis was initiated for worsening renal function. Since no tangible recovery could be appreciated, intravenous rituximab (0.5 g) was administered. She was transferred to our hospital for further management after developing fevers and bleeding from gums and vagina.
Upon arrival at our hospital, the patient was sedated and intubated on mechanical ventilation. Bloody secretions were noted in the endotracheal tube. There were alopecia and hyperpigmented areas on the scalp. There were punctuate subconjunctival hemorrhage bilaterally and scattered petechiae on the lower extremities. Pitting edema was present over shins. Chest radiography revealed extensive bilateral pulmonary infiltrates. Renal ultrasonography revealed enlarged kidneys (13.2 cm in length) with increased echogenicity. There was no hydronephrosis, masses, or stones. Laboratory data are summarized under “Day 17” in . There were neutrophilia, lymphopenia, hemolytic anemia, profound thrombocytopenia, hypoalbuminemia, and renal insufficiency. Blood films revealed numerous schistocytes. Urinalysis revealed proteinuria and hematuria. Urinary protein excretion was estimated at 14.6 g/day based on a random urine protein to creatinine ratio. Urine sediment revealed numerous white blood cells and dysmorphic red blood cells. Several red blood cell casts and muddy brown casts were also observed. Serologic tests confirmed antinuclear antibodies of the same specificities previously reported. Complement C3 level remained low and C4 was normal. C-reactive protein was elevated. Plasma activity of ADAMTS-13 was normal. Antibodies against platelet factor-4, myeloperoxidase, proteinase-3, and glomerular basement membrane were not detected. Coagulation studies demonstrated markedly elevated D-Dimer. Elevated dilute Russell viper venom time (DRVVT) ratio and positive hexagonal phospholipid (Staclot LA) test indicated the presence of lupus anticoagulant.
The patient was diagnosed with catastrophic antiphospholipid syndrome with concurrent hemorrhagic manifestations in the setting of new-onset SLE. Plasma exchange therapy was continued with one plasma volume exchanged with fresh frozen plasma every day for three consecutive days. Intermittent hemodialysis was continued. Medications included prednisone 60 mg/day and hydroxychloroquine 400 mg/day. Platelets were infused as needed. On the 5th hospital day, the patient was extubated. In the meantime, urine output increased. On the 6th hospital day, the last hemodialysis treatment was given. On the 8th hospital day, intravenous rituximab 1 g was administered for persistent thrombocytopenia. A 24 hour urine collection demonstrated protein excretion of 12.7 g/day. On the 15th hospital day, the patient was ambulating requiring no supplemental oxygen. Laboratory tests revealed serum creatinine concentration of 0.8 mg/dL, hemoglobin concentration of 8.5 g/dL (no blood transfusion for 16 days), and platelet count 33,000/mm3 (no platelet transfusion for 7 days). She was discharged home on prednisone 60 mg/day and hydroxychloroquine 400 mg/day, dapsone for PCP prophylaxis, esomeprazole, and calcium/vitamin D supplements. She missed her follow-up appointments after discharge.


The patient was a 27-year-old man (weight: 66 kg; height; 166 cm) with no underlying disease. This patient became aware of coxalgia 1 year earlier. The coxalgia was associated with continuous pain that became more severe, and at the time of presentation he could not walk without an axillary crutch. X-ray images showed joint narrowing and multiple bone erosions of the left hip joint (). Computed tomography (CT) clearly showed femoral neck and acetabular bone erosions as well as ONFH (Figures and ). In contrast, magnetic resonance (MR) imaging showed ONFH and mass lesions around the femoral neck. T2-weighted fat-suppressed MR imaging also revealed an irregular tumoral lesion (). The patient had type C ONFH according to the Nishii et al. classification [] and stage 4 ONFH according to the Association Research Circulation Osseous classification [].
We performed an arthroscopic biopsy before radical treatment. Histological analysis of the synovial tissue showed diffuse PVNS. The patient subsequently underwent total hip arthroplasty (THA) (KYOCERA Medical Corporation, Osaka, Japan) with complete synovectomy and tumor resection using an argon laser (). Severe invasive PVNS was seen in the inferior space of the hip joint, particularly around the femoral neck.
Pathological findings from an analysis of permanent preparations of the synovium and femoral head were consistent with PVNS and avascular bone necrosis, respectively. Proliferative histiocyte-like mononuclear cells were observed with multinucleated giant cells in the fibrotic stroma. Hemosiderin-laden macrophages and foamy cells were also occasionally observed. The subchondral zone of the femoral head showed avascular bone necrosis. Both empty lacunae and fat necrosis were found in the resected femoral head. Fibrosis and proliferation of vessels were observed around the necrotic trabeculae, and new bone apposition was present.
After the procedure, the Harris hip score improved from 79 to 97. No operative complications occurred, and no recurrence of PVNS was detected for 3 years after the procedure.


A 40-year-old woman with a clinical history of persistent epigastric pain was diagnosed by gastroscopy of a gastric submucosal tumor in the esophagogastric junction. Endoscopic ultrasound showed a 20 mm submucosal tumor with benign features. The biopsy performed guided by the endoscopic ultrasound revealed a leiomyoma. The locations of the lesion lead us to perform a laparoscopic intragastric surgery in order to avoid a transgastric approach through a large opening over the anterior wall of the stomach or a large resection of the upper third of the stomach. This intragastric approach was performed by using a single port device placed at the right upper quadrant of the patient.
A conventional laparoscopy was performed using three 5 mm trocars, one supraumbilical trocar, for a 5 mm 30° optic, and two ports as working channels, being inserted in the left and the right upper quadrants. Once peritoneal cavity was preliminarily examined, a gastroscopy was performed to confirm the location of the lesion at the esophagogastric junction. A 1.5 cm gastrotomy was performed at the anterior wall of the stomach, far from the lesion, using the Harmonic scalpel (Ethicon Endo Surgery, Cincinnati, OH, USA). By enlarging to 1.5 cm the previous 5 mm incision of the right upper quadrant port, the introducer of a single port multichannel, TriPort Plus (Advanced Surgical Concepts, Ireland) was introduced under laparoscopic vision through the abdominal wall into the gastrotomy. The self-expanding shaft of the TriPort Plus helps the device to firmly grip both abdominal and stomach walls allowing CO2 insufflation of the stomach ().
The 5 mm 30° optic, a roticulator atraumatic grasper (Covidien, Mansfield, MA, USA), and standard laparoscopic instruments were introduced through the single port device in order to work inside the stomach. Tumor location at esophagogastric junction was confirmed. Tumor was resected using the Harmonic scalpel, respecting the muscle layer of the esophagogastric junction. Simultaneously endoscopic movements of the scope provide us a good traction of the cardias. The mucosal defect left behind was repaired with intracorporeal interrupted absorbable sutures (). A good passage through esophagogastric junction was checked by removing and introducing the gastroscope once defect was repaired. An endobag was introduced through the single port device in order to introduce the specimen, being removed by the endoscopist inside the bag through the mouth of the patient. The TriPort Plus was removed from the stomach and placed just in the abdominal wall and the gastrotomy was closed using four absorbable sutures. The stomach was insufflated by the endoscopist and no leaks were observed at the anterior gastric wall ().
Patient was discharged home on day one postoperatively. Three months after surgery the patient remained asymptomatic with no side effect after this approach. Histological examination of the surgical specimen confirmed the presence of a leiomyoma.


At the time of testing, IG was a 44 year old woman who suffered from an ischemic stroke related to acute vasospastic angiopathy in the posterior cerebral arteries established with an angiogram. Magnetic resonance imaging revealed a hyperintense signal on T2 sequencing that was fairly symmetrically, located in the posterior parietal and upper and lateral occipital cortico-subcortical regions (). Reconstruction of the lesion [] indicated that it involved mainly Brodmann’s areas 19, 18, 7, a limited part of area 39. This therefore included the parieto-occipital sulcus and the caudal part of the intraparietal sulcus. IG was given a set of standard clinical tests involving visual field topography (Goldman perimetry), sensory stimulation tests (visual and tactile extinction), neurological evaluation of reflexes and muscle tone and joint movements. Visual fields showed a partial right inferior homonymous quadrantanopia (visual scotoma). The patient did not exhibit any purely motor or somatosensory deficit, any sign of sensory extinction or any sign of neglect during conventional testing (on standard line bisection, star cancellation and drawing tasks) but she demonstrated bilateral optic ataxia [,]. OA patients typically display in impaired online peripheral guidance of a limb [,] with improved accuracy when given more processing time as in delayed reaching [], along with preserved low-level visual and motor function [-]. Reaching and grasping inaccuracy predominated for her right hand in her right peripheral hemifield. However, visually elicited hand movements were generally accurate when performed in foveal vision. Note that IG initially showed simultanagnosia, which prevented her to see the target and her hand at the same time, which could lead to a contradictory result []. In the present experiment, however, IG was tested when the bilateral optic ataxia persisted without associated clinical simultanagnosia.


About 6 months before at 22 weeks of gestation, a cystic lesion was identified during a routine obstetric ultrasound examination of the pelvic area in a 23-year-old female patient without any previous discomfort. Due to the compression of the cyst on the cervix, the delivery was achieved by caesarean section. Postpartum ultrasonographic examination showed that there was a 7.5 × 8.5 cm sized thin-walled cystic lesion in the midline of the pelvic region compressing the rectum, uterus and bladder (). The origin of the lesion could not be determined during the ultrasonographic examination. The T2-weighted magnetic resonance imaging (MRI) showed a thin-walled hyperintensity in the uterus, and the rectum posterior while T1-weighted images revealed a hypointense cystic lesion. The cyst was not associated with the ovaries. The fourth and fifth sacral bones were not seen. In the posterior, the cyst was communicating with the spinal subarachnoid space (). Based on these findings, the patient was diagnosed with sacral agenesis and anterior sacral meningocele. In addition, MRI showed that the conus medullaris ended at the 4th lumbar spine level and a distinct arcuate uterus was observed. The patient's neurological examination was normal. Complete blood count and serum biochemical tests were within normal limits.
Because she planned another pregnancy, gynecological surgeons and neurosurgeons suggested to the patient a surgical management. But she denied.


A 34-year-old male patient presented with BAT with history of fall from 10-meter height. On presentation, the patient was hypotensive (blood pressure of BP 81/43 mmHg) with heart rate of 130 beat per min. He had depressed fracture of left frontoparietal skull bone, contusion with abrasion of left side of face, mild chest wall hematoma, and abdominal guarding. Patient was intubated immediately with rapid sequence induction. Focused abdominal sonography for trauma (FAST) was positive; chest X-ray showed left subcutaneous emphysema with fractures of the left 6th, 7th, and 10th ribs. Chest tube was inserted and massive transfusion protocol was activated. Initial laboratory investigations showed an INR of 1.2, low pH 7.16, base excess −9.4, low hematocrit (17%), high myoglobin (2549 ng/mL), creatinine 139 umol/L, low hemoglobin (10.1 g/dL), high white blood cell count (25.9 × 103 u/L), normal platelet (246 × 103 u/L), and high sensitive troponin T (90 ng/L). Patient was shifted to operation theatre immediately.
An exploratory laparotomy was performed which revealed shattered spleen requiring splenectomy; otherwise, formal exploration showed no other findings.
Postoperative laboratory investigation showed hemoglobin of 11.5 g/dL, WBC 13000 u/L, platelet 192000 u/L, creatinine 64 umol/L, myoglobin 1324 ng/L, and high sensitive troponin T 189 ng/L. Follow-up laboratory findings after 6 hours revealed low hemoglobin (8.5 g/dL), normal WBC (7300 u/L), and platelet count (130000 u/L).
A CT scanning of the head showed comminuted depressed fracture of left frontoparietal bone with minimal subdural hematoma, subarachnoid hemorrhage, tiny hemorrhagic contusions of left temporal area, and fracture of left zygomatic arch. Neck and chest CT scanning revealed evidence of C6 facet fracture and fractures of left 5–8 ribs anteriorly and left 6-7, 9–12 ribs posteriorly with left hemo- and pneumothorax and bilateral lung contusions. With a smooth course in trauma intensive care unit (TICU), the patient was started enteral feeding through nasojejunal feeding tube after 24 hours and then he was extubated 2 days later. After removal of nasojejunal tube, the patient developed abdominal distention; for that nasogastric tube was inserted and 1100 mL fluid was removed initially. After a couple of days in the surgical ward, the patient showed a wax and wane course of abdominal distention. All serum electrolytes were within normal range.
Further CT scan of the abdomen showed dilated small bowel with no organic obstruction (). Finally, he started to improve with partial distention and the nasogastric tube was removed. A clear fluid was commenced and gradually advanced to full diet owing to the patient tolerance. Two days later, he experienced severe abdominal distention and vomiting, and therefore he was returned back to TICU and another abdominal CT scan with trial of oral contrast was performed and showed characteristic intussusception at the distal ileum (). Patient declined operative intervention and so he was kept on nasogastric decompressing tube and antibiotics. Later on, the patient agreed on exploratory laparotomy that revealed severe adhesions of stomach and small bowel to anterior abdominal wall with dilated small bowel loops up to 9 cm with collapse 1 meter from the ileocecal junction. There was an intussusception of 1.5 cm length at 1 meter from the ileocecal junction with perforation of small bowel proximal to it (). The affected area was isolated and almost 3 L of fecal material was evacuated. This area was resected and side-to-side anastomosis was fashioned using gastrointestinal anastomosis (GIA) linear cutting staplers (sized 100 mm). Oversewing of the anastomotic staple line with 3-0 Vicryl sutures was done. Omental defect was closed with 3-0 Vicryl sutures. Peritoneal cavity was irrigated with 5 L of warm saline and then the abdomen was closed. Histopathological study of small bowel specimen (9 × 3 cm) showed foci of transmural necrosis and inflammation with ulceration. The postoperative course of the patient was unremarkable and he was discharged home in a stable condition.


Case 1. A 42-year-old female with hypertension and hyperlipidemia was admitted to our facility for atypical chest pain. There was no reported history of syncope. Serial cardiac biomarkers were normal and the patient underwent coronary CT angiogram for assessment of coronary anatomy. Cardiac CT revealed no significant coronary artery disease but did show a left main coronary artery with an anomalous origin off of the right sinus of Valsalva with subsequent intraseptal course (). Invasive coronary angiography () was completed with normal fractional flow reserve measurements in the proximal left anterior descending with adenosine infusion. The patient is currently doing well without further symptoms on optimal medical therapy alone.


Case 2. A 72-year-old female with hypertension and hyperlipidemia was admitted to the chest pain unit for evaluation of atypical chest pain. There was no reported history of lightheadedness, dizziness, or syncope. The patient underwent a stress echocardiography with no ischemic changes on electrocardiogram and no angina with evidence of mid anteroseptal wall hypokinesis at peak exercise. The patient underwent invasive coronary angiography () showing no significant coronary artery disease and a left main coronary artery with an anomalous takeoff from the right coronary cusp. The myocardial course of the left main was unable to be definitively determined by invasive coronary angiography and cardiac CT was completed. Cardiac CT showed the left main to have an anomalous takeoff from the right coronary cusp with a course between the great vessels (Figures and ). The patient is currently asymptomatic on maximal medical therapy.