A 23-year-old Japanese woman was referred to our hospital due to splenomegaly of unknown etiology and cytopenia. Before referral, she underwent intensive examinations, including bone marrow aspiration and spleen biopsy, at other medical care centers for the detection of hematologic, endocrine, and hereditary diseases in a differential diagnosis. However, a definitive diagnosis was not made. Upon admission, the patient presented with dyspnea upon exertion and abdominal distention. Chest CT scan revealed the presence of both centrilobular and randomly distributed small nodules in both lungs (Fig. a). In addition, abdominal CT scan revealed splenomegaly (Fig. b). Lung function test upon admission revealed restrictive ventilatory impairment with reduced pulmonary diffusion capacity. Liver biopsy was performed, and the biopsy specimen revealed a noncaseating granuloma, indicating sarcoidosis. Then, the patient underwent transbronchial lung biopsy and BAL to evaluate for lung involvement. Although an increased number of small lymphocytes in the BALF, which is a typical finding, was observed, the CD4/CD8 ratio decreased to 0.12 (Fig. c and Table ). Moreover, the biopsy specimen from the lung showed noncaseating epithelioid granuloma positively stained with CD68 in the lung interstitium (Fig. a, b). The tuberculin reaction test had a negative finding. On the basis of these results and the elevated level of angiotensin-converting enzyme (ACE) (45.1 U/L), lysozyme (36.4 μg/mL), and serum soluble interleukin-2 receptor (5290 U/mL), the diagnosis of sarcoidosis was confirmed (Table ). Because of progressive cytopenia and high risk of splenic rupture, splenectomy was chosen as the initial therapy. The pathological findings of the resected spleen revealed noncaseating granuloma, which was consistent with the splenic lesion in sarcoidosis (Figs. c, d). Along with the decrease in the level of serum ACE (24.1 U/L) and lysozyme (22.4 μg/mL), dyspnea and abdominal distention improved after splenectomy (Table ).
The patient complained of gradually exacerbating dyspnea and increase in purulent sputum 3 months after splenectomy. Chest CT scan revealed the presence of centrilobular nodules with bronchial wall thickening in both lung fields (Fig. a). The second BAL revealed a remarkable increase in neutrophil count (Fig. b, Table ). The bacterial culture from the BALF tested positive for Hemophilus influenzae. In addition, the cold agglutinin test had positive results, and paranasal sinus CT scan revealed inflammation of the maxillary sinus (Fig. c). Because these data showed the characteristics of DPB, we re-evaluated the lung biopsy specimen obtained during the first bronchoscopy. The foamy cells infiltrating the interstitial wall surrounding a respiratory bronchiole supported the diagnosis of DPB (Fig. e). Because DPB primarily caused the patient’s current symptoms and radiological deterioration, 600 mg of erythromycin was administered daily as the initial treatment. After administrating low-dose macrolide therapy for 3 months, the patient’s condition improved based on the clinical and radiological findings (Fig. ).


A six-month-old girl was admitted to the pediatric unit with fever, cough, stridor, vomiting, and respiratory distress for seven days. She had previously been hospitalized multiple times and had required intensive care during the two previous hospitalizations. Of note, when the patient was not ill, she was healthy and noted to be asymptomatic. Birth history was uneventful, but one sibling died due to the same condition. Second-degree consanguinity was documented between the parents.
On initial assessment, the patient was lethargic and dehydrated with marked Kussmaul breathing. Blood gas analysis revealed metabolic acidosis (pH 7.22, partial pressure of carbon dioxide (PCO2) 14.6 mmHg, bicarbonate (HCO3; 5.9 mmol/l) with a high anion gap (20.7 mmol/l) and an increased base excess (-19.1 mmol/l). Computed tomography (CT) chest showed bilateral ground-glass appearance (infective) (Figure ), complete blood count (CBC) showed a high total leukocyte count (TLC) of 37, mostly neutrophil (60%), and her C-reactive protein (CRP) was 1.56 mg/dl. The results of the biochemical analysis were within normal limits (ammonia 37 umol/l). Ketonuria was also present (ketone 3+ / 300 mg/dl).
The patient was suspected to have bronchopneumonia with concern for underlying metabolic disorder. The patient was initiated on antibiotic ceftriaxone 50 mg/kg/day. Repeat arterial blood gas (ABG) revealed continued metabolic acidosis and the patient was initiated on a continuous bicarbonate infusion. Subsequent ABGs after the infusion of bicarbonate showed an improvement in her metabolic acidosis. Plasma amino acids and urine for organic acids were sent to evaluate for underlying metabolic disorders. The patient was also initiated on oral sodium bicarbonate, biotin, vitamin B12, and protein-free milk, and mother's milk was stopped after consultation with the chemical pathologist. The patient improved with the treatment and was shifted to the ward. She was initially on nasogastric feed and gradually oral feed was established. Repeated CBC showed hemoglobin of 6 mg/dl and red cell concentrates (RCC) was transfused at 10ml/kg during the treatment.
Urine organic acid analysis by gas chromatography-mass spectrometry showed marked excretion of 3-hydroxybutyric acid along with moderate excretion of 3-hydroxy-isovaleric acid, the diagnosis of isovaleric acidemia was made and the patient was sent home with medication and a scheduled follow-up.


A 61-year-old male with a history of inotrope-dependent end-stage nonischemic cardiomyopathy with a severely depressed left ventricular ejection fraction and an implantable cardioverter defibrillator (ICD) presented with bilateral lower extremity edema and seven-pound weight gain. He had no history of atrial fibrillation or venous thrombosis. This was his second hospitalization for acute on chronic decompensated systolic heart failure in a matter of a few months. His examination was notable for a heart rate of 100 bpm, a BP of 85/66 mmHg, and an oxygen saturation of 100% breathing ambient air. He was in no apparent respiratory distress. He had jugular venous distention and a third heart sound, clear lung fields, and bilateral lower extremity pitting edema. He was continued on his inotrope, and treatment with intravenous diuretic therapy was initiated. A transthoracic echocardiogram (TTE) was performed mainly to assess his right ventricular (RV) function since he was being considered for a possible orthotopic heart transplant or left ventricular assist device implant. In addition to severely depressed RV systolic function, he was found to have a new large highly mobile echo density in the right atrium intermittently prolapsing through the tricuspid valve into the right ventricle (, ). The patient had no evidence of infectious signs or symptoms, serial blood cultures remained sterile, and the elongated serpiginous echocardiographic appearance was more suggestive of thrombus than a vegetation. Systemic anticoagulation with intravenous heparin was initiated, and a surgical consultation was obtained. Doppler ultrasound of the bilateral lower extremities did not demonstrate any deep venous thrombosis, and the TTE did not show that the thrombus was clearly attached to the lead. Testing for Factor V Leiden mutation was negative, as was testing for heparin-induced thrombocytopenia. Age-appropriate cancer screening (colon, prostate) was normal, and computed tomography of the head, chest, and abdomen/pelvis did not show any masses or pathologic lymph node enlargement. Additionally, a bone marrow biopsy was negative for any infiltrative or malignant process.
He was deemed to be too high risk for conventional surgical thrombectomy given his severe heart failure, and we were hesitant to administer systemic thrombolytic therapy given the high embolic and bleeding risk. Despite therapeutic systemic anticoagulation, a subsequent TTE 2 days later did not demonstrate reduction in the right atrial thrombus size. After discussions with the cardiothoracic surgery team and the interventional radiology team, the decision was made to attempt a minimally invasive percutaneous thrombectomy using the AngioVac Aspiration System (AngioDynamics, Latham, NY).
Transesophageal echocardiography (TEE) was performed intraoperatively to confirm persistence of the right atrial thrombus prior to proceeding. Under general anesthesia, venous access was obtained via bilateral femoral veins to perform aspiration and reinfuse the blood. An AngioVac device was advanced to the right atrium in proximity to the thrombus (, ). Subsequently, a 22 cm thrombus () was removed, and no residual thrombus was seen on intraoperative imaging. There were no apparent procedural complications, and the patient was continued on systemic intravenous anticoagulation afterwards. Subsequent cultures of the excised thrombus did not reveal any growth. The patient underwent successful cardiac transplantation three weeks later.


A boy aged 1 year and 7 months was seen at our clinic after referral for refractive errors with Plusoptix. Retinoscopy under cyclopegia revealed: –14.5 diopters with an oblique astigmatism of –2 diopters on the right eye and –9.50 diopters with an oblique astigmatism of –4 diopters on the left eye. Fundoscopy and intra-ocular pressure were normal. Glasses were prescribed. An A-scan showed symmetric axial lengths of the left and right eye. B-scan echography showed a thicker lens on the right eye, which could explain the anisomyopia. A few months later, glasses were adapted based on new values on retinoscopy: right eye –10.5(–8x180°), left eye –3(–6x140°). Due to high anisomyopia, patching of the left eye was initiated daily for 2 hours.
At the age of 2 years and 2 months, glasses were adapted to –7.00 (–9.00x40°) for the right eye and –5.00 (–7.00x140°) for the left eye. Patching was continued.
Due to the unexplained high refractive error and the difficult clinical examination of this child, a detailed examination under general anesthesia with a keratometry, automatic refractor and pachymetry, detailed examination of the anterior segment and dilated fundoscopy were performed. Results are shown in Table 1 . Upon retro-illumination, bilateral parallel oblique folds in Descemet’s membrane were found. Gonioscopy was not performed.
The general history of this boy reports a serious car accident of the mother during pregnancy at the gestational age of 27 weeks. All follow-up controls afterwards were reassuring. The patient was born on the postmenstrual age of 35 weeks and 3 days. Labor was induced because of preterm premature rupture of membranes (PPROM). An urgent caesarean section was performed because of fetal distress. There were no other neonatal problems. The patient was diagnosed with platelet dysfunction at the age of 1 year on the occasion of recurrent hematomas caused by minor trauma. An extended screening for underlying metabolic disease could not reveal any abnormalities. There were no other health problems. Familial history for any eye disorders was negative.


A 38-year-old female presented to the Department of Prosthodontics and Crown-Bridge for the prosthetic rehabilitation of postmaxillectomy defect resulting from squamous cell carcinoma of the left maxilla 12 months back. The patient complained of difficulty in mastication, nasal regurgitation of fluids, and nasal tone in her voice. She had worn surgical and interim obturator. Intraoral examination revealed well-healed surgical defect in the maxilla involving part of the hard palate, alveolar ridge, and maxillary tuberosity creating an oroantral communication. All teeth posterior to the first premolar were missing on the left quadrant of the maxilla (). Masticatory and phonetic functions of the patient were affected. After a thorough examination, the defect was classified as Aramany's Class II maxillary defect. The treatment plan was made to rehabilitate this patient with a definitive obturator with a cast metal framework.


A 71-year-old female with a basal metabolic index (BMI) of 27.03 kg/m2 and a history of hypertension, hypercholesteremia, and the thyroid nodules presented to the surgeon's office upon a referral from her primary care with a progressively enlarging mass in the right side of her neck. She further stated that several years previously she had a hysterectomy, sinus surgery, left knee surgery, breast biopsy, and a neck lump biopsy. She refutes any drug allergy, tobacco use, and alcohol consumption history. Furthermore, she had normal thyroid function studies.
The patient had a USG of the neck showing multi-nodular goiter and a large exophytic 4.3 cm mass near the upper pole of the right thyroid lobe which was not present on a previous study performed a few years earlier (Figure ). The study revealed two nodules in the right lobe of the thyroid gland measuring 1.1 cm and 1.7 cm, respectively. These were given a Thyroid Imaging Reporting and Data Systems (TIRADS) score of 3. Also, in the left thyroid lobe, the USG of the neck detected two nodules measuring 1.3 cm and 1.8 cm, respectively. A CT scan of the neck was also performed suggesting that the mass in the right upper neck was separate from the thyroid gland and may be an enlarged lymph node (Figure ). She had undergone previous fine-needle aspiration (FNA) of the right neck mass for cytology and was also benign.
At the initial presentation, she was afebrile (Temperature = 98.5°F) with a blood pressure of 162/78 mmHg, a heart rate of 68 beats/minute, and oxygen saturation (SpO2) of 100%. On physical examination, there was a dominant mass in the right upper neck below the jugulodiagastric region in the anterior cervical triangle that was mobile, soft, rubbery, smooth, and non-tender. The mass did not appear to be attached to the thyroid gland on the clinical examination. The thyroid gland was nodular and minimally enlarged. There was no cervical lymphadenopathy noted in the left neck or beyond the apparent large upper right neck mass. The trachea was normal and midline and no apparent vascular abnormalities were noted.
Based on the physical examination and preoperative diagnostic workup, a decision was made to perform a total thyroidectomy with intraoperative neural monitoring and excision and biopsy of a right neck mass if cancer was to be identified, the next step was to be central and right modified neck dissection. In the operating room, a transverse neck incision was made just above the suprasternal notch.
A separate incision was made over the visible and palpable mass in the upper right neck. It was enucleated and submitted to the pathologist for touch prep and frozen section histology. The mass measured about 3.9 cm in length and 2 cm in diameter. The pathologist reported that no lymphoid tissue was seen in the mass, and it was purely benign thyroid tissue (Figure ).
A total thyroidectomy was completed for the multinodular thyroid goiter. Lymph nodes surrounding the thyroid were submitted for touch prep and frozen section to determine whether there was metastatic thyroid carcinoma. The pathologist did not recognize any malignancy, and it appeared to be an ETT. Post-operative pathological specimen assessment revealed no metastatic foci in any specimens. The specimen retrieved from the right lateral cervical area only contained benign multi-nodular thyroid parenchyma (Figure ).


A 45-year-old man presented with paroxysmal palpitation to the Shinshu University Hospital. Electrocardiogram revealed PAF (Figure A), and the patient chose hot balloon ablation because of drug-resistant palpitations. Echocardiography showed no valvular heart disease, a left atrial volume index (LAVI) of 26.9 mL/m2, and a 65% left ventricular ejection fraction based on the modified Simpson method.
At admission, persistent atrial tachycardia (AT) was observed (Figure B). During AT, the P waves were positive in lead V1, negative in lead aVL, and positive in leads II, III, and aVF with high amplitude, indicating that the AT originated from the left superior pulmonary vein (LSPV) or the left atrial appendage.
The procedure was performed during AT. Although the A-A intervals varied beat by beat from 242 to 297 ms, the atrial activation sequence was constant (Figure A). By the transseptal approach, an activation map of the left atrium was obtained using CARTO (Biosense Webster, Inc) and PentaRay catheter (Biosense Webster). The activation map showed that the focus of the early activation of AT was on the posterior wall of the antrum of the LSPV (Figure B). It seemed that the focus of the AT was exactly on the ablation site of the hot balloon at the LSPV antrum. We inflated the hot balloon with 10 mL of the contrast media that was diluted 1:2 by saline at the LSPV antrum. Venogram showed good occlusion of the LSPV by the balloon, and we started ablation. AT was terminated 5 seconds from the start of the ablation, and sinus rhythm was restored. We continued the ablation for 180 seconds at 70°C. During ablation, cold saline was injected into the esophagus, such that the temperature would not exceed 39°C. Then, we proceeded with the PVI by ablating other pulmonary veins with the hot balloon in an ordinal manner. We confirmed noninducibility of any AT or AF by burst atrial stimulation and finished the procedure. After discharge, there has been no recurrence of AT or AF.


A 45-year-old male patient presented with persistent pain and effusion at the lateral aspect of his right knee. He underwent an uneventful ACL reconstruction ten years before, with total functional recovery and no instability or pain symptoms after full rehabilitation postoperative program. For ACL graft fixation, a bioabsorbable crosspin was used at the femur and a bioabsorbable interference screw was used at the tibia. The patient reported that he did not suffer any trauma, increased sports activity level, or other remarkable events in the weeks or months prior to the moment when pain and effusion started. The patient was first treated at another centre where femoral crosspin loosening was noted. After a magnetic resonance image (MRI) showed an intact ACL graft and no other complications, the patient underwent surgery for the removal of a femoral crosspin.
One month after femoral crosspin removal surgery, effusion and pain persisted. Then, the patient was evaluated at our hospital for the first time. Physical exploration showed swelling at the lateral aspect of the lateral femoral condyle (). A rounded, cyst-like mass with well-defined borders was evident at palpation. No local signs of infection were found. Lachman, pivot-shift, and anterior and posterior drawer tests were negative, with a full range of motion and no valgus or varus instability at physical exploration. Plain anteroposterior and lateral radiographs of the knee showed adequate tunnel position and no tunnel widening.
Suspected diagnosis was postsurgical seroma. Treatment included percutaneous drainage, nonsteroidal anti-inflammatory drugs (NSAIDs), and RICE (rest, ice, compression, and elevation). Samples from the drained seroma were sent for microbiological analysis and culture, with no bacterial ingrowth. Prophylactic antibiotic treatment was prescribed until a negative result of synovial fluid cultures was obtained. Initial evolution was satisfactory, with complete symptom release for 6 months. After this period, recurrent swelling at the lateral aspect of the knee reappeared and remained despite percutaneous drainage. A cyst-like rounded mass was palpable at the same location of previous episodes. Physical exploration was similar to our first evaluation; no local or systemic infection symptoms were found.
MRI showed a cyst formation at the lateral aspect of the knee, with fluid signal in the graft femoral tunnel and around the crosspin tunnel entrance at the lateral cortex of the lateral femoral condyle ().
Surgical treatment in collaboration with plastic surgery was carried out. With the patient in supine position and under ischaemia of the leg, standard knee arthroscopy was carried out. Full knee arthroscopic exploration showed an intact ACL graft with no meniscal or articular cartilage lesions. No widening of the femoral or tibial ACL graft tunnels was noted, and neither was there any graft loosening. During arthroscopy, as a result of fluid inflow to the knee, the lateral cyst increased in size, making it more perceptible at the lateral aspect of the knee. This finding confirmed that a fistula was connecting the knee joint to the lateral condyle; as noted in the previous MRI, this fistula was the origin of the cyst. No signs of septic arthritis were found. After knee arthroscopy, the operation proceeded with open surgery through a lateral approach using the previous surgical scarf from the crosspin removal surgery. The lateral cortex of the lateral femoral condyle was exposed. The hole at the cortical bone of the lateral aspect of the lateral femoral condyle, caused by the drilling carried out to introduce the crosspin used for femoral ACL graft fixation, persisted () with rounded soft tissue burn. At the lateral aspect of the femoral condyle, a cyst-like cavity was found. Once opened, it was filled with synovial fluid and no capsule or synovial tissue surrounded the cyst; however, it has dissected a cavity between bone cortical and deep muscular fascia. No signs of local infection were present. At the lateral end of the femoral tunnel, where the tunnel from the removed crosspin persisted, a burned bone was also noted. The bone tunnel end was surrounded with devitalized bone with negative paprika sign and sclerotic edges; bone edges and surrounding soft tissues were darkened due to burning during drilling. Owing to a small tunnel width of 10 mm, arthroscopic exploration introducing an arthroscope through the tunnel was not possible. The cyst was excised, and burned bone was refreshed until a bleeding marrow bone bed was obtained.
The bone tunnel was filled with autologous marrow bone graft before covering the cortical bone defect with local fascial flap. Postoperative course was uneventful and total recovery was achieved.
Three years after the femoral tunnel revision surgery, the patient remained with full functional recovery and without any clinical symptoms, effusion, or pain recurrence.


A 14-year-old boy presented to the emergency department with a painful left wrist following a fall with his bike after a low-velocity collision with a car. The exact mechanism of trauma could not be described by the boy, but most of the impact was received on the left hand and wrist. On clinical examination, there was swelling but no gross deformity of the left wrist. The wrist was diffusely tender on palpation and range of motion (ROM) was limited by pain. There were no neurovascular deficits and the skin was intact. Anteroposterior and lateral X-rays of the left wrist showed a displaced Salter-Harris type IV fracture of the distal ulna (Figures and ). Because of this uncommon fracture type with displacement of the fragment, subsequent computed tomography (CT) was performed. CT showed a displaced fracture through the epiphysis and metaphysis on the volar side of the distal ulna with an intraarticular step of 3 mm (). The patient's wrist was immobilized in a below-the-elbow plaster at the emergency department. Reduction and stabilization were, for practical reasons, planned 5 days later.
The procedure was performed under general anaesthesia. First, an attempt at closed reduction was made, with pressure applied to the volar aspect of the wrist under radioscopic control. This resulted in a partial but insufficient reduction of the fracture, which led to conversion to an open reduction and fixation procedure. The fracture was approached through an ulnovolar incision. The fragment was reduced under radioscopic control using Kirschner pins to manipulate and lever the fracture fragments. Three Kirschner pins were placed parallel to the physeal plate. Two pins were placed in the coronal plane with one through the proximal fragment and one through the distal fragment. A third pin was drilled in the anteroposterior plane through the distal fragment (). The patient was immobilized in an above-the-elbow cast for two weeks.
Follow-up after two weeks showed a favourable clinical evolution with maintained position of the fracture fragments on X-ray (). To encourage mobilization of elbow and fingers, a wrist brace had to be worn for three more weeks. Rotations had to be avoided. One K wire was removed after six weeks and automobilization was started. Evaluation four weeks later showed a good range of motion in flexion-extension and rotations of 45°. To improve supination, physiotherapy was added. Three months later, the second pin was removed because of migration.
At follow-up 6 months after injury, there was a full range of motion of the wrist compared to the other side with a similar grip strength on both sides. X-ray of the wrist showed the fracture was fully healed with no evidence for premature fusion of the physis or growth arrest (). X-ray 1 year postoperatively showed complete closure of the physis with development of a negative ulnar variance (). Further follow-up with radiographic control will be provided until skeletal maturity.


A 65-year-old white male was referred to our Vitreo-Retinal Surgery Service for a retinal detachment (RD) in his right eye. On examination, his best-corrected visual acuity (BCVA) was 20/63 in the right eye (RE) and 20/20 in the left eye (LE), with a small refractive error. Intraocular pressure was 15 mmHg OU. Slitlamp examination revealed quiet anterior chambers and slight nuclear sclerosis in both eyes. Dilated fundus examination of the right eye showed an inferior bullous RD involving the macula with no evidence of retinal breaks, while a large RPE tear was detected in the temporal quadrant (Fig. , A,B). There was no sign of uveitis or vitreitis. Fundus examination of the LE was unremarkable, except for slight RPE distrophic alterations at the posterior pole with RPE mottling. Enhanced depth imaging optical coherence tomography (EDI-OCT) revealed a pachychoroid pattern in both eyes (Fig. ). Moreover, OCT scans of the right eye showed sub-retinal fluid (SRF) reaching the macular area from the inferior quadrants. No SRF was detected in the LE. Ocular ultrasound showed no mass lesion (Fig. , C,D). Upon questioning his medical history, the patient referred a history of central serous chorioretinopathy (CSC) in the RE and the recent use of intravenous corticosteroids for bronchitis. Fluorescein angiography (FA) and Indocyanine green angiography (ICGA) were performed, revealing hyperpermeable and dilated choroidal vessels and multifocal and diffuse leakage in the late angiograms of the RE (Fig. ). No disc leak or vasculitis were detected. Swept-Source OCT angiography (SS-OCT-A) confirmed the pachychoroid pattern and clearly demonstrated the mid-pheripheral RPE tear in the inferotemporal quadrant (Fig. ). Axial lengths were 23.63 mm (RE) and 23.35 mm (LE).
Based on the clinical features, a diagnosis of bullous variant of CSC with RPE tear was made. Oral therapy with eplerenone at a dose of 50 mg/day was initiated. However, no improvement was detected. In fact, two months later, the exudative RD increased with a worsening of the BCVA to 20/80 (Fig. ), despite the continuing therapy with eplerenone. To prevent irreversible photoreceptor damage, management with observation or eplerenone treatment continuation were excluded, and alternative therapeutic procedures were discussed. Laser therapy was deemed inappropriate because the leakage was multifocal and diffuse, with multiple “ink blot” focal areas of leakage at the superior margin of the detached retina and other poorly defined leakage areas in the attached retina. Photodynamic therapy (PDT) was not applicable because of the extension and elevation of the RD. Therefore, the opportunity for surgical treatment was considered.
Written informed consent was given by the patient, after a comprehensive explanation of the procedures. Under general anesthesia, the patient’s eye was prepared with povidone-iodine and draped. After 180° inferior conjunctival peritomy, bridle suture was passed under the inferior, medial and lateral rectus muscles with 3–0 ticron, and the sclera was exposed. Two 4 × 4 mm almost full scleral thickness sclerectomies were done in the inferior quadrants, taking care to avoid the areas close to exit sites of the vortex veins. Finally, the conjunctiva was approximated with 8–0 Vicryl suture. The treatment resulted in complete RD resolution as of the first day after surgery. Upon follow-up six months after surgery- complete SRF resolution was confirmed and BCVA was stable at 20/80 (Fig. ).


A 77-year-old female with a history of chronic alcohol abuse presented with dizziness and confusion one month after a right knee replacement for degenerative joint disease. She described her dizziness as “lightheadedness” that was not associated with any specific maneuver or changes in position. Prior to undergoing knee surgery, she had been healthy, independent, and abstinent from alcohol. With the onset of the confusion, her family had found her to be “jittery” with sleep disturbances and difficulty concentrating. On examination, she was found to have impaired orientation to time and slow thought processing. Her Mini-Mental State Exam (MMSE) was 21/30, consistent with mild cognitive impairment, and the remainder of her systemic exam was unremarkable. A noncontrast CT of the head was negative for an acute intracranial process, and a follow-up MRI of the brain without contrast was unrevealing for underlying abnormality (Figures -). Given her prior history of chronic alcohol intake, supplementation with thiamine and folic acid was initiated.
One week later, the patient started to complain of visual hallucinations. An EEG was obtained to rule out seizures and showed bifrontal discharges more visible over the right hemisphere with right frontal faster frequencies suggestive of an increased tendency toward seizures. Generalized background slowing was also observed, suggestive of moderate to severe encephalopathy (Figure ). A repeat brain MRI with contrast showed cortical and subcortical T2 FLAIR hyperintensities in bilateral posterior temporal lobes and the left occipital lobe without associated enhancement (Figure ). The MRI demonstrated features suggestive of PRES. Workup including metabolic, infectious, and vasculitic panels in serum and CSF were all unremarkable with the exception of elevated antinuclear antibodies (ANA) at 1:80. A trial of high-dose methylprednisolone (1 g for 5 days) was administered due to concern for central nervous system vasculitis or autoimmune-mediated process. The patient showed no clinical improvement after completing a course of steroid therapy.
Nineteen days after the admission, her condition significantly deteriorated with frequent episodes of agitation and jerky myoclonic movements involving her orofacial and proximal upper limbs. Continuous electroencephalogram (cEEG) revealed multifocal epileptiform and generalized discharges, forming multifocal periodic discharges (MfPDs), and generalized periodic discharges (GPDs) at 0.5-1 Hz suggestive of CJD (Figure ). A repeat brain MRI with contrast showed diffuse cortical and subcortical T2 FLAIR hyperintensities without enhancement. Additionally, new diffusion restrictions in frontoparietal, temporal, insular, and occipital cortex were noted consistent with cortical ribboning characteristic of CJD (Figure ). A repeat lumbar puncture was performed, and CSF was sent for 14-3-3 and t-tau protein analysis.
Twenty-five days after the admission, she became increasingly somnolent and less responsive and was subsequently intubated. She was then transferred to the neurointensive care unit (NICU) where she remained intubated and sedated for the duration of the hospital course. She continued to decompensate and the family requested to withdraw care. Previous CSF results had returned positive for 14-3-3 and an elevated t-tau protein consistent with the diagnosis of sCJD. Autopsy and brain biopsy were not performed.


A 31-year-old male was referred to our hospital for further assessment of a huge liver tumor. He had no medical or family history, and laboratory examinations demonstrated no abnormal findings. Computed tomography showed an 80 mm-thick lobulated tumor in the liver adjacent to the inferior vena cava and left and right hepatic veins, with a central low-density lesion and mild enhancement of the tumor rim (Fig. a). The tumor showed a high-intensity signal pattern with T2- and diffusion-weighted images (Fig. b) using magnetic resonance imaging. The central part of the tumor showed higher signals, which we suspected to be the mucus component. Fluorodeoxyglucose–positron emission tomography (FDG–PET) demonstrated a ring-like, high and intense FDG uptake with a high-level standardized uptake value (SUV) of 9.15 ± 1.09 (mean ± SD) at the rim of the tumor and no uptake at the center of the tumor (Fig. c). No other uptake was seen. Histological analysis was performed, and because of its inoperability, we performed carbon ion radiotherapy of 64.8 Gy, which significantly decreased the cell viability, represented by an SUV of 1.25 ± 0.19 (Fig. d), 4 months after the completion of the therapy.
Histological analysis performed on a specimen obtained from the tumor rim exhibited adenocarcinoma cells, partly forming malignant glands with fibrous tissue (HE staining, Fig. e). The tumor cells were positively stained with cytokeratin 7 (Fig. f) and hepatocyte nuclear factor-4α and were negative for cytokeratin 20. These findings indicated the tumor to be an intrahepatic cholangiocarcinoma (ICC), which is the second leading primary liver cancer.


A 44-year-old man came to the emergency room for two-week progressive dyspnea. His medical history included morbid obesity, active smoking and childhood severe trauma that required splenectomy.
The computed tomography (CT) pulmonary angiography performed to rule out a pulmonary embolism revealed several peritoneal nodules in the upper abdomen (Figure ).
The additional abdominal contrast-enhanced CT revealed multiple other nodules and homogeneous disseminated peritoneal masses (Figure ). Splenosis nodules were suspected given the history of splenic trauma and the lack of cancer history. A Tc-99m-tagged heat-damaged red blood cells scintigraphy (Tc99m-DRBC) confirmed the diagnosis of peritoneal splenosis (Figure ).


A 57-year-old Caucasian male patient presented to the gastroenterology clinic with progressive dysphagia to solids and liquids for the last few months. The patient had a history of type 1 myotonic muscular dystrophy diagnosed at the age of 34, gastroesophageal reflux disease (GERD), diaphragmatic paralysis, and obstructive sleep apnea. He complained of occasional heartburn, but denied weight loss, anorexia, chest pain, regurgitation, cough, early satiety, abdominal pain, nausea, vomiting, diarrhea, hematochezia, or melena. Last upper endoscopy 5 years ago revealed a small hiatal hernia and a normal esophagus, stomach, and duodenum. His only home medication was a daily 40 mg tablet of omeprazole. Past surgical history was significant for bilateral cataract surgery. Both his father and brother died in their 50s because of unclear respiratory problems, and there was no family history of malignancies. He was a former smoker and did not drink alcohol.
On physical examination, the patient was alert, oriented, and in no acute distress. Vital signs demonstrated a temperature of 36.8° C, blood pressure of 126/85 mmHg, heart rate of 90 beats per minute, respiratory rate of 16 breaths per minute, and O2 saturation of 97% on room air. Cardiovascular, lung, abdominal, and skin exams were unremarkable.
Neurological examination was significant for bilateral facial muscle weakness and mild ptosis. His other cranial nerves were intact. He had atrophy and weakness of the distal upper and lower extremities. Deep tendon reflexes were absent. Sensory, cerebellar, and gait exams were unremarkable.
Laboratory workup including complete blood count (CBC) and basic metabolic panel (BMP) were within normal limits. An upper endoscopy was performed and showed mild hyperemia of the stomach, but a normal esophagus and no hiatal hernia. Esophageal biopsies were obtained from the upper and lower esophagus which were normal and showed no evidence of eosinophilic esophagitis.
After two weeks of being off omeprazole, the patient underwent a 24-hour esophageal pH testing which showed no significant acid reflux. Finally, he underwent esophageal manometry which revealed elevated LES pressure, elevated UES pressure, and very week peristalsis of the esophageal body.


A 54-year-old male cadaver during routine dissection showed composite piriformis muscle with peculiar arrangements of the two heads varying from its usual appearance and early splitting of the left sciatic nerve with tibial and common peroneal components (Figures and ). The common peroneal component passes between the two heads of the piriformis muscle, whereas the tibial component had its usual course.


A 47-year-old male cadaver during similar routine dissection showed early splitting of the right sciatic nerve where the common peroneal component descends posterolateral to the piriformis muscle, whereas the tibial component had its usual course (). The result corresponded with Beaton and Anson type-c (2.5%).


A similar unilateral variation in the formation of sciatic nerve and its unusual relation with the piriformis muscle was encountered in the left gluteal region of a 51-year-old male cadaver. Additionally, an accessory belly of the piriformis muscle at its insertion was observed in the right gluteal region beneath the gluteus maximus of the same cadaver. The relationship of the sciatic nerve and piriformis muscle in this case was classified under type-c (2.5%). There were no any gross anomalic appearance or any surgical intervention in these cadavers near the dissected area.


A previously healthy 12-year-old girl, with a 10-day history of nausea, polyuria, and polydipsia, was brought to the emergency department for abdominal pain and vomiting. The patient did not have any history of alcohol consumption or medical health problems besides this presenting complaint. She was admitted in the pediatric unit. On admission to the ICU, the physical exam showed diffuse abdominal pain. She had sunken eyes with a clear acetone-smelling breath.
Her blood pressure was at 80/40 mmHg, pulse at 140 beats/min, respiration rate at 35/min, pulse oxygen saturation (SpO2) under air room at 96%, and body temperature at 38°C. Laboratory samples showed serum glucose level at 24 mmol/l, pH at 7.19, bicarbonate at 10 mmol/l, anion gap at 26, lipase at 260 U/l, amylase at 244 U/l, cholesterol at 33 mmol/l, and serum triglyceride = 133 mmol/l. WBC count was at 14650/mm3 and glycated hemoglobin was at 11.0%. Aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase, and bilirubin values were normal.
The abdominal ultrasound exam was normal. An abdominal CT scan was performed showing an AP grade E with three fluid collections and the presence of retroperitoneal air. The diagnosis of AP induced by DKA with concurrent hypertriglyceridemia was obtained, and the patient was transferred to our ICU for an adequate management. Continuous insulin infusion and fluid administration were initiated. The patient's health state improved. Her triglyceride rate decreased gradually (). Continuous insulin was switched to subcutaneous insulin, and the patient was discharged after five days.


A 12-year-old female child with no medical history and no history of prior biliary colic or alcohol consumption was admitted in the pediatric unit for abdominal pain and fever. Questioning the parents' revealed that, for 4 weeks, she had been suffering from polyuria and polydipsia associated with a significant loss of weight. The patient's health status deteriorated gradually, with abdominal pain and fever. The physical exam showed diffuse abdominal tenderness with an important decrease of bowel sounds. Her blood pressure was at 60/30 mmHg, pulse at 160 beats/min, temperature at 39°C, and respiratory rate at 30/min. The laboratory exams showed serum glucose level at 21 mmol/l, hemoglobin A1c at 16.5%, pH = 7.24, bicarbonate at 5 mmol/l, anion gap at 36, serum sodium = 142 mmol/l, serum potassium = 2.7 mmol/l, lipase = 411 U/l, amylase = 361 U/l, cholesterol = 12.8 mmol/l, and serum triglyceride = 53 mmol/l with apparent lipema in the lab test tubes. The other biochemical values were at the normal range, particularly the aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase, and bilirubin values.
Computed tomography (CT) scan revealed gastric distension, changes in the interstitium of the pancreas, and intrahepatic bile duct dilatation. The imaging did not show any other anomaly causing acute pancreatitis, in particular, a gallstone disease or extrahepatic duct dilatation. The patient developed respiratory distress needing the use of mechanical ventilation, then she was transferred to the intensive care unit. In ICU, she underwent fluid infusion, catecholamine prescription, and insulin infusion. During her ICU stay, she developed septic shock, requiring large-spectrum antibiotics, and acute kidney injury (AKI) with refractory metabolic acidosis, requiring hemodialysis. Despite the intensive treatment, the patient developed a multiorgan failure. She died 12 days after ICU admission.


A 37-year-old male presenting with 2 weeks of polyuria, polydipsia, and epigastric pain and vomiting was admitted in an intensive care unit for consciousness alteration. Taking his medical history by interrogating his parents did not reveal any alcohol abuse or a prior history of biliary colic.
On admission, the initial assessment showed a Glasgow Coma Scale Score at 8/15, body temperature at 38.3°C, heart rate at 120 beats/min, blood pressure at 80/45 mmHg, respiratory rate at 30 breaths/min, and an oxygen saturation of 93% under room air. He needed intubation and mechanical ventilation.
The laboratory tests showed serum glucose at 32 mmol/l, pH = 7.31, bicarbonate = 10.7 mmol/l, serum sodium = 132 mmol/l, serum potassium = 3.9 mmol/l, lipase = 511 U/l, cholesterol = 12.8 mmol/l, serum triglyceride = 75 mmol/l, WBC count = 3900/mm3, platelet count = 101000/mm3, and CRP at 496 mg/l. Aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, and bilirubin values were at the normal range.
An abdominal CT scan with contrast was obtained showing an acute pancreatitis grade E with two fluid collections in the pancreas. There was no evidence for gallstones. He was aggressively hydrated and received IV insulin.
A repeat abdomen CT scan, performed 7 days later, showed a necrotic fluid collection which was infected. An endoscopic drainage was performed. The patient's neurological status deteriorated with no sign of waking up and multiple episodes of seizures. A brain CT scan was performed, showing cortical and subcortical hypodense lesion in the right frontal area.
During his ICU stay, the patient developed septic shock with multiorgan failure. He died 45 days after ICU admission.


A 42-year-old man with no significant medical history, presented after 10 days of polyuria and polydipsia, was admitted into the emergency department for vomiting and abdominal pain. No history of alcohol consumption was found.
The physical exam showed a diffuse abdominal tenderness. His blood pressure was at 100/70 mmHg, pulse at 130 beats/min, temperature at 37°C, and respiratory rate at 32 beats/min.
The laboratory tests showed serum glucose level at 23 mmol/l, hemoglobin A1c at 16.5%, pH = 7.26, bicarbonate = 7 mmol/l, anion gap = 36, serum sodium = 143 mmol/l, serum potassium = 2.9 mmol/l, lipase = 441 U/l, amylase = 361 U/l, cholesterol = 12.8 mmol/l, and triglyceridemia = 53 mmol/l with apparent lipema in the lab test tubes. WBC count was at 9200/mm3, platelet count at 153000/mm3, CRP at 330 mg/l, AST = 35 IU/l, ALT = 28 IU/l, bilirubin = 26 mmol/l, and the alkaline phosphatase=120 IU/l.
Computed tomography (CT) scan revealed an acute pancreatitis grade E with two fluid collections in the pancreas associated with peritoneal effusion. Fluid bolus and continuous insulin infusion were initiated. The patient's health state improved, and he was discharged after seven days.
All patient characteristics and laboratory results on admission are summarized in Tables and , respectively. shows the evolution of triglyceride and blood glucose levels under treatment in all studied patients.


A 26-year-old woman presented to the emergency department immediately after a bicycle accident in which she fell on the ground with an outstretched hand. She complained of pain at her right wrist and elbow, especially when she rotated the forearm. On physical examination, the forearm was held in supination with the elbow in 90° flexion and the wrist in the neutral position. A superficial abrasion was first noted on the palmar surface of the right wrist. Tenderness was triggered with direct palpation on the distal radio-ulnar joint (DRUJ), as well as the lateral and medial side of the elbow. No tenderness was detected with palpation on the middle portion of the forearm. The active range of motion of elbow joint was flexion 110°/extension 180°, and rotation of forearm was supination 80°/pronation 40°. She had a normal movement of the fingers and intact sensation in the right hand.
Radiographs of both forearms were taken for comparison, revealing volar dislocation of the radial head and dorsal dislocation of the distal radius without associated fracture on the injured side, which formed a criss-cross appearance on the lateral radiograph (Fig. ). Computed Tomography (CT) scan was also applied to rule out associated fractures. To further evaluate the soft tissue injuries, Magnetic Resonance Imaging (MRI) of the forearm was taken, revealing partial rupture of the proximal IOM from its dorsal attachment and no impairment of the middle portion of the IOM, as well as partial rupture of the medial collateral ligament (MCL) (Fig. ). Aside from ligamentous injuries, bone contusion was also detected on the capitellum of the humerus and the proximal ulna on MRI images.
The patient was first diagnosed as “Simultaneous dislocation of the radial head and distal radio-ulnar joint without fracture”. We inclined not to diagnose this injury as an Essex-Lopresti injury because the central part of IOM seemed intact. Conservative treatment was firstly tried with closed reduction in the emergency room without anesthesia. With the elbow in 90° flexion, the forearm was successively immobilized in either supination, pronation or neutral rotation with a long-arm cast. In each position, CT scan of the whole forearm was taken (Fig. ). However, the radiocapitellar joint and the DRUJ could not be simultaneously reduced in either position.
The patient was then introduced to an expert in elbow surgery and advised to undergo surgical exploration. Before the surgery started, the stability of the elbow and the DRUJ were respectively tested under general anesthesia. During the Valgus Elbow Stress Test, the laxity of the elbow was increased and the radial head seemed to shift forward under a valgus force, indicating that the medial collateral ligament of the elbow and the annular ligament might be injured. The Ballottement Test also showed a positive result, revealing an unstable DRUJ.
After the examination, the surgery was performed, aiming to explore the lateral side of the elbow and repair the potentially injured annular ligament. A lateral approach of the elbow was performed. After dividing the subcutaneous tissues, the lateral collateral ligament revealed itself to be intact. Then, a longitudinal incision was made on the lateral collateral ligament to expose the annular ligament and the humeroradial joint. Surprisingly, the annular ligament was found to be perfectly intact, while the radial head was in a slightly anteriorly dislocated position. Further inspection of the radial head revealed an abnormal, convex and bumpy articular surface (Fig. ). An intraoperative Valgus Elbow Stress Test showed that the radial head would dislocate anteriorly under a valgus force. To reduce the radial head, rotation of the forearm was tried first and the results were not satisfying. So we apply a manual force upon the anterior part of the radial head to achieve a well-aligned reduction while the forearm was in full supination and the elbow was in 90° flexion. Subsequently, a 2.0-mm percutaneous K-wire was used to stabilize the proximal radio-ulnar joint (PRUJ). Intraoperative radiographs showed that the radial head and the DRUJ were simultaneously reduced (Fig. ). After closing the wound, a long arm cast was used to keep the forearm in supination, 90° of elbow flexion, and the wrist in neutral deviation.
The long arm cast was kept for 4 weeks and replaced by an adjustable brace to allow a full range of flexion and extension. The K-wire was removed at 6 weeks postoperatively to allow a full range of forearm rotation. At 6 months postoperatively, the patient had regained a full range of flexion and extension, with normal supination and a slight limitation in pronation. Until 18 months postoperatively, the patient still had a slight limitation in pronation, with the Mayo elbow score being 85 and the Mayo wrist score being 90(Fig. ). Radiographic findings revealed that the radial head and the DRUJ were in better positions, but there was still a criss-cross appearance between radius and ulna on the lateral view (Fig. ).


A 30-year-old male with a past medical history of asthma and gastroesophageal reflux disease presented with intermittent sharp left shoulder pain and radiated to the neck and arms for the last two weeks, in addition to progressive dyspnea on exertion for the past couple of months. He was previously able to walk and run long distances without symptoms but showed profound shortness of breath with minimal exertion associated with palpitations at the time of presentation. He denied lifting heavy objects or recent trauma, syncopal events, lightheadedness or dizziness. Family history was pertinent to premature coronary artery disease. He denied consumption of tobacco, alcohol, and illicit drug use. Physical examination revealed bilateral lung base crackles and trace pedal edema. Chest X-ray was grossly normal (Figure ), and had a normal sinus rhythm with inadequate R-wave progression at the electrocardiogram (Figure ).
Trans-esophageal echocardiogram (TEE) and CT angiography (CTA) scan were ordered owing to findings at 2D Trans-thoracic Echocardiogram (TTE), which revealed left ventricle ejection fraction at 50%, grade 1 diastolic dysfunction, moderate to severe aortic regurgitation and dilated aorta. TEE proved a bicuspid aortic valve with normal cusp separation with moderate eccentric regurgitation, and confirmed the presence of a dilated aortic root measuring 4.8 cm and dilated ascending aorta measuring 4.2 cm (Videos , ).
Chest CTA reported no evidence of aortic dissection, rupture, or intramural hematoma, with aneurysm of sinuses of Valsalva (Figures -).
Left and right heart catheterization were performed, proving normal hemodynamic parameters and unremarkable coronary anatomy (Figures -).
Cardiothoracic surgery service was consulted, and it was decided to address the management with a Bentall surgical correction. After explaining the methods, benefits, and risks involved, the patient and family agreed with surgical intervention. A St. Jude valve conduit was placed without acute complications during the procedure, and close monitoring took placed in the surgical intensive care unit for the next five days. The patient was discharged after an appropriate recovery time on temporary anticoagulation with a vitamin K antagonist. The patient exhibited significant improvement in symptoms and functional capacity as an outpatient follow up. A follow-up TTE was performed eight months after discharge, showing a mechanical aortic valve functioning normally, without stenosis or regurgitation and normal cusp separation (Video ).


A ten-year-old male patient suffering from CF was brought to the Emergency Department of our hospital because of low-grade fever (axillary temperature [AT] 38.0 °C) associated with left hemithorax pain and slight polypnea. In his medical history, several previous recurrences of respiratory bacterial infections were reported. The last episode had occurred approximately 3 months previously, was due to a methicillin-sensitive S. aureus and was treated with a combination of amoxicillin/clavulanic acid for 15 days. During the following period, no antibiotic treatment was administered.
At admission, his physical and neurodevelopmental growth was within the normal range. However, he was febrile (AT 39.2 °C), his respiratory rate was 40 breaths/min, his heart rate was 111 beats/min, his oxygen saturation in room air was 91%, and his blood pressure was 114/68 mmHg. Thoracic auscultation revealed cracking rattles in the left basal area and forced expiratory volume in 1 s (FEV1) was 46%. Initial laboratory tests showed leucocytosis with neutrophilia but without eosinophilia (white blood cell [WBC] count 17,130/mm3; neutrophils [N] 12,916/mm3, 75.4%; eosinophils [E] 462/ mm3, 2.7%), an increased C-reactive protein [CRP] serum level (11.19 mg/dL), a high cold agglutinin serum concentration (> 1:16) and IgM and IgG positive for M. pneumoniae (specific IgM, 1:300; specific IgG, 1:800). The sputum culture test was negative for respiratory bacteria and fungi. The chest X-ray showed an average left basal thickening, right paracardial thickening and widespread interstitial thickening. Suspecting a bacterial respiratory exacerbation, intravenous therapy with cefazolin (150 mg/kg/day in 3 doses) and tobramycin (10 mg/kg/day in single dose) was started. Moreover, oral clarithromycin (15 mg/kg/day in 2 doses) was added.
Treatment was apparently effective, as clinical conditions rapidly improved and fever disappeared within two days. However, after six days, despite antibiotic therapy being continuously administered, a new episode of thoracic pain associated with the reappearance of fever (AT 38.5 °C) occurred. A second chest X-ray showed a significant increase in the previously identified lung thickening associated with pleural effusion in the left basal site; new blood tests showed an increase of leucocytosis with neutrophilia (WBC 19,870/mm3, N 76%), in this case with eosinophilia (E 2384/mm3, 12%), and a further increase in the CRP serum concentration (16.80 mg/dL). Suspecting a lung infection resistant to first-line antibiotic therapy, the ongoing drugs, except for the macrolide, were withdrawn, and a new therapeutic course with vancomycin (45 mg/kg/day in 3 doses) and meropenem (60 mg/kg/die in 3 doses) was started.
Contrary to what was observed a few days before, a new sputum culture test was positive for A. fumigatus and remained negative for aerobic bacteria. Unfortunately, the new drug regimen was ineffective, as no modifications in clinical conditions and chest X-ray findings were demonstrated after one week of treatment. Moreover, blood tests revealed an increase in the number of WBCs (21,220/mm3), N (15,193/mm3), and E (1782/mm3). CRP was only slightly reduced (9.71 mg/dL).
Suspecting an infection due to macrolide-resistant atypical bacteria, clarithromycin was withdrawn, and ciprofloxacin (1 g/day in 2 doses) was started. Three days later, a chest computed tomography (CT) scan confirmed the chest X-ray findings but revealed bilateral bronchiectasis/bronchiolectasis with material in the context, suggesting high-attenuation mucus plugs (HAM) (). The blood test showed a reduction in WBCs (17,080/ mm3) and N (10,128/mm3, 59.3%), but E were found to be significantly increased (3296/mm3, 19%). CRP was reduced (4.26 mg/dL).
Considering all these findings, ABPA associated with macrolide-resistant atypical bacterial infection was considered. A bronchoalveolar lavage (BAL) and several tests for the diagnosis of sensitization to A. fumigatus were planned. BAL showed spores and fungal hyphae of A. fumigatus and was positive for galactomannan. Total IgE, previously undetermined, were found significantly increased (4096 KU/L, n.v. < 100 U/mL). RAST for food and inhalants showed multi-sensitization. Finally, high serum level of anti-Aspergillus IgE (26.20 KU/L, n.v. < 0.1 KU/L) was evidenced. However, the galactomannan antigen was negative. A diagnosis of ABPA was made, and endovenous prednisone (1 mg/kg/day) associated with itraconazole (5 mg/kg/day) in 2 doses was started.
Subsequent blood tests showed a progressive reduction in the flogosis indexes, finally reaching negative CRP and a progressive reduction in eosinophils, while the chest X-ray examination revealed a reduction in lung thickening and the disappearance of pleural effusion. summarizes laboratory exams at admission and during follow-up. The follow-up at one month after discharge showed also normalization of radiographic findings (), further supporting the diagnosis of ABPA.
The management of this patient was approved by the Ethics Committee of Umbria Region (PED- 2019-9), and both parents provided written informed consent for the evaluation of the child. The Ethics Committee of Umbria Region approved the publication of this case and both parents provided written informed consent for the publication of this manuscript.


The patient was a 75-year-old female who received four implants in the mandible for prosthetic rehabilitation by overdenture. She was in therapy with warfarin for chronic atrial fibrillation. Three months after the implant insertion, a clinical examination revealed the poor quality and quantity of keratinized gingiva around the fixtures. More precisely, it revealed the proximity of caruncula sublingualis and the absence of an inadequate deepening of both buccal and lingual vestibula of the mandible limited the prosthetic space and causing instability of the peri-implant gingiva (Figures -). The patient was treated by diode laser surgery (wavelength 980 ± 10 nm; continuous wave, output energy 1.5 W) without warfarin discontinuation; healing caps were removed and the implant was re-covered with gingiva using stitches, and vestibuloplasty was performed (by using a diode laser) on both aspects of the mandible with contextual lingual frenulotomy (Figure ). No bleeding was observed during surgery and stitches were unnecessary; no post-surgical complications occurred. After 15 days, a complete resolution of all wounds was observable with an evident increase of the available prosthetic space (Figure ).


A 68-year-old woman with type-II diabetes, arterial hypertension (treated with a calcium antagonist), and chronic atrial fibrillation, was treated with amiodarone and direct oral anticoagulant (DOAC) therapy factor II inhibitor (dabigatran) from September 2016 to October 2018. At this time the patient showed bleeding (hematuria) and hematomas in the lower limbs and gluteus, so drug administration was stopped.
We screened for coagulation factors:
Vitamin K-dependent coagulative factors (II, VII, IX, X) to evaluate vitamin deficiency or liver disease.
No vitamin K-dependent coagulative factors VIII and V to exclude vitamin-K deficiency or acquired hemophilia A.
We dosed markers of viral liver infection to exclude liver disease as alternative diagnosis of consumptive coagulopathy. Lupus anticoagulant, anticardiolipin autoantibodies, anti β2-microglobulin were dosed to diagnose an autoimmune disease.
We found a very low level of FV (0.1% vs. normal value 60–140%)—no other coagulative factors were altered (
). Autoantibody research did not prove positive. We applied the Mixing test by measuring coagulation time, i.e., international normalized ratio (INR), activated partial thromboplastin time (aPTT) ratio at time 0 and 2 hours after incubation at 37°C. Correction in coagulation time after the Mixing test was not found. Based on these findings, we postulated that the low FV level ensued from AFVI (measured as 1.94 BU on the Bethesda units scale).


A 26-year-old, male semiprofessional soccer player had a prominence of tibial tuberosity for 10 years and reported anterior knee pain during sports activity and while climbing stairs. He claims pain commencement about 15 years ago and since then he mentions occasional pain episodes. He underwent conservative treatment for the last two years with anti-inflammatory drugs and physiotherapy and he had three injections of corticosteroids at different times. On physical examination, there was a permanent tibial tubercle with pain during palpation (Figure ). Radiographic examination showed an ununited ossicle beneath the patellar tendon (Figure ). Magnetic resonance imaging sections showed tendinitis on the patellar tendon and an ununited ossicle anterior to the tibial tubercle (Figure ). Dimensions of the ossicle were 17 mm x 8 mm.
Diagnostic arthroscopy was performed using a low anterolateral portal close to patellar tendon (Figure ). The intra-articular arthroscopic examination of the knee was unremarkable and no concomitant abnormality was noticed. An anteromedial portal close to the patellar tendon was used and the infrapatellar fat pad beneath the patellar tendon was debrided with a motorized shaver. Localization of the ununited ossicle was performed by use of an 18-gauge needle that was advanced through the insertion site of the patellar tendon on the tibial tubercle under fluoroscopic control. The needle and the adjacent ossicle were visualized arthroscopically (Figure ). In order for a better and adequate arthroscopic visualization to be achieved, a multidirectional arthroscope (ENDOCAMELEON® ARTHRO HOPKINS® Telescope) was used at 70°. The knee was extended in order to maximize the working space by relaxing the patellar tendon. The ossicle was identified, embedded in the patellar tendon and the dissection of the ossicle began with debridement of its proximal aspect with a radiofrequency device. Then the removal of the ossicle performed gradually with the use of a motorized shaver (Figure ) and a motorized semi-hooded barrel burr (Figure ). In order to achieve an even better approach to the ossicle, a second low anterolateral assisting portal was used. This was created below the first anterolateral portal, just lateral to the patellar tendon and to the ossicle (Figure ). Fluoroscopic control was performed after removal of the ossicle to ensure no residue of bone (Figure ).
Postoperatively, instructions for partial weight bearing and isometric flexion extension 0°-90° exercises for two weeks were given. After two weeks walking without crutches was allowed while no squatting for six weeks was suggested. After two months, the patient returned to sport activities without any restrictions and complaints. Postoperative X-ray showed minimal ossicle residual, and clinically there was no prominence (Figures , ).


A 37-year-old African American female with a past medical history of controlled asthma (no recent exacerbation or corticosteroid use) and multiple episodes of recurrent pancreatitis that is not related to diabetes (last episode was one year prior to admission) presented to the emergency department with abdominal pain, nausea and vomiting over two days. The patient described the lower abdominal pain as sharp and intermittent in nature, rated as 10/10 in intensity, radiating to the back, with no worsening or relieving factors and associated with multiple bouts of nausea and vomiting. The patient reported starving for two days prior to admission, as she had not been able to take in foods and worried that food would worsen the abdominal pain. Otherwise, the patient denied fever, chills, rigors, changes in bowel movement and urinary symptoms. The patient also denied vaginal discharge, and reported no history of sexually transmitted diseases or oral contraceptives and was not sexually active. The patient’s last menstrual period was a few days prior to admission. She also denied any recent sick contact or travel. The patient was not on any medications and supplements such as acetaminophen, salicylates, isoniazid or iron. The patient also denied the use of tobacco, alcohol and illicit drugs.
In the emergency room, the patient started to complain of shortness of breath, tachypnea and acute respiratory distress. Initial vitals showed a blood pressure of 152/104 mmHg, a heart rate of 142 bpm, a respiratory rate of 24 bpm and a saturation rate of 98% on 2 L/min of oxygen. Abdominal examination showed soft, non-distended, diffuse tenderness over the lower abdomen, no hepatosplenomegaly and normal bowel sounds. Lung and cardiovascular examinations were otherwise normal. Complete metabolic profile showed sodium 132 mmoL/L, potassium 6.4 mmoL/L, bicarb 5 mmoL/L, chloride of 100 mmoL/L, corrected anion gap 29 mmoL/L, lipase was 97 U/L and HbA1c 4.4%. Arterial blood gas showed pH 6.88, pCO2 12 mmHg, pO2 162 mmHg and HCO3 2.7 mmHg. Delta (delta) gap was 0.9, and the patient was experiencing mixed anion gap metabolic acidosis. Urine anion gap was 31.7 mmol/L, with urine pH of 5.5, 2+ protein, no glycosuria and no urine casts observed on urine microscopy. Serum glucose was normal (128 mg/dL) and 3+ ketonuria were present. Lactic acid was 1.7 mmol/L with no uremia (blood urea nitrogen was 6 mg/dL). Lipase was 73 U/L (within normal range). Salicylate acid, acetaminophen and ethanol level were normal. The patient’s beta-hydroxybutyrate level was more than 4.5 and 3+ ketonuria, suggestive of ketoacidosis as the cause of metabolic acidosis. The workup for methanol, ethylene glycol, isopropanol and paraldehyde was negative; however, the patient had an elevated osmolar gap, with a gap of >20 which was thought to be from the ketoacidosis. Chest X-ray was unremarkable. EKG showed sinus tachycardia (with heart rate of 130 bpm), normal axis and no ST-T wave abnormalities. CTs of the abdomen and pelvis did not show any signs of acute pancreatitis, pancreatic atrophy or peripancreatic fluids. There was a large uterine fibroid noted at the endometrial cavity. A transvaginal ultrasound showed two discrete fibroids measuring 2.3 x 1.5 x 2 cm and 4.7 x 2.6 x 3.4 cm on the subserosa (Figure ) and intramural (Figure ) of the endometrium, respectively. The lower abdominal pain may have been related to the uterine fibroids, as she had negative pregnancy test and no previous gynecological problems.
We diagnosed the patient with starvation ketoacidosis due to vomiting, two days of starvation and the absence of other causes of high anion gap metabolic acidosis. Patient was given six ampoules of sodium bicarbonate at the emergency department and was then sent to intensive care unit (ICU). The patient received supplemental oxygen, dextrose and a bicarbonate drip in the ICU. The patient developed refeeding symptoms with hypokalemia, hypomagnesemia and hypophosphatemia. Electrolytes were replenished, and patient’s anion gap was improved and resolved with dextrose and fluid infusion.


A 52-year-old African American male presented to the emergency department from his primary care physician's office due to persistent fever. The patient had been discharged from the hospital one week prior after being hospitalized for diarrhea due to Clostridium difficile colitis, which was treated with oral vancomycin. During that hospitalization, he was started on allopurinol for an acute gout flare after which he developed a generalized purpuric, morbilliform rash, which started on his chest and spread to his extremities. He was discharged on a prednisone taper for a suspected drug rash, which he completed prior to returning to the hospital.
His past medical history includes hypertension, chronic kidney disease stage 5, osteoarthritis, GERD, and gout. On initial examination, his temperature was 38.3°C with vitals within normal limits. Skin excoriations were noted, but no rash or lymphadenopathy. His cardiopulmonary and abdominal exams were unremarkable. Laboratory data demonstrated a white blood cell count within normal limits, hemoglobin 7.9 gm/dL, platelet count 71K/mcl, creatinine 6.4 mg/dL (baseline 5-6 mg/dL), BUN 60 mg/dL, and elevated LFTs with AST 878 units/L, ALT 1,117 units/L, alkaline phosphatase 169 units/L, and GGT 209 units/L. Of note, the patient had known anemia of chronic disease; however, his thrombocytopenia was a new occurrence. Lactic acid was normal, and urinalysis showed no signs of active urinary tract infection. Chest X-ray demonstrated no acute processes.
During hospitalization, his LFTs continued to increase and thrombocytopenia worsened. The gastroenterology service was consulted on the second day of hospitalization. His temperature of 38.7°C persisted. His LFTs remained elevated with AST 676 units/L, ALT 1,262 units/L, alkaline phosphatase 173 units/L, and total bilirubin 0.6 mg/dl with a normal prothrombin time.
Despite a negative acetaminophen level, N-acetylcysteine (NAC) was administered via three consecutive doses given over 21 hours with subsequent decrease in LFTs. Viral serologies (hepatitis A/B/C, EBV, CMV, HSV, and VZV), autoimmune studies (IgG, ANA, smooth muscle antibody, and mitochondrial antibody), and ceruloplasmin were normal as well as serum electrolytes, thyroid function tests, ammonia level, and stool studies. Abdominal ultrasound with doppler demonstrated hepatomegaly without evidence of vascular occlusion or biliary abnormalities.
On the third day, he developed peripheral eosinophilia (13%). Given that he had taken allopurinol two weeks before presentation, subsequently presented with fevers, rash, elevated LFTs, and peripheral eosinophilia, with an extensive workup negative for alternative processes, DRESS syndrome was suspected. LFTs continued to improve significantly. The remainder of testing was negative including infectious workup, alternative autoimmune and genetic etiologies, and toxicology screening leading to discharge on day seven.


A 46-year-old man presented for evaluation of a nodular lesion on his fifth right fingernail that firstly appeared in February 2018. He did not smoke and had a history of ulcerative colitis, treated with Infliximab since March 2019. Vaccination with nonavalent Gardasil® for HPV was administered one year before this clinical manifestation. Dermatologic examination revealed an exophytic, erythematous mass on the perionychium with focal superficial erosions. Biopsy of the nodular mass was performed, and the histopathological examination showed features consistent with a diagnosis of squamous cell carcinoma in situ (). In this case, surgical treatment is the first treatment option; however, in order to reduce the field of cancerization and tumor size, and to minimize damages to the surrounding structures, according to the willingness of the patient to preserve the cosmetic and functional integrity of the finger, we prescribed a course of treatment with imiquimod 5% cream daily for eight weeks. (). In order to evaluate the therapeutic effects of the topical treatment, the patient was monitored by both dermoscopic and RCM examinations before and during therapy, and four weeks and 12 weeks after the treatment. The latest examination of the affected area showed a complete absence of residual tumor.


A 45-year-old male with a past medical history of human immunodeficiency virus (HIV) on highly active anti-retroviral therapy (HAART) presented to the emergency department with complaints of retrosternal chest pain and dark stool for one week. On admission, his vitals were blood pressure 136/74 mmHg, heart rate 89 beats/min, respiratory rate 16/min, oxygen saturation 96% on room air, and temperature 37.7 °C. Physical examination was positive for dark stool in the rectal vault which tested positive on bedside fecal occult blood test (hemoccult).
Labs were remarkable for troponin <0.03 ng/m, serum creatinine 1.55 mg/dl, potassium 5 meq/l, bicarbonate 32 meq/l, hemoglobin 15.5 g/l and (international normalized ratio (INR) 1.1, and D-dimer of 2800 (normal < 500 ng/L). An electrocardiogram (EKG) showed normal sinus rhythm with right bundle branch block. Computed tomography (CT) of the chest with contrast was performed to rule out pulmonary embolism which in turn showed aberrant origin of the right coronary artery from the left sinus of Valsalva, demonstrating a malignant course between the aorta and the pulmonary artery. Additionally, there was aneurysmal dilatation of the left sinus of Valsalva measuring 3 x 2 cm (Figures -). Cardiology, cardiothoracic surgery, and gastroenterology was consulted. He was admitted for cardiac monitoring and an hour after being transferred to the medical ward was found unresponsive. Cardiac monitor showed asystole and cardiopulmonary resuscitation was initiated per advanced cardiac life support (ACLS) protocol. Return of spontaneous circulation was achieved in 30 minutes and the patient was transferred to the intensive care unit (ICU). A second episode of asystole occurred on arrival to the ICU from which the patient could not be revived and led to his demise.


A 20-month-old boy was admitted to another hospital because of fever (body temperature up to 39.1°C) continuing for seven days. Eight days before admission, the boy had an enlarged right axillary lymph node spreading to the chest wall with pain. Treatment with ampicillin and clindamycin was ineffective for the fever and enlarged axillary lymph node. Two days later, he developed erythema, which was like rubella, on the face, the trunk, and the upper and lower extremities. The rash soon disappeared after two days. The patient had no history of allergy, travel, or exposure to sick persons. His family did not breed any animals. Because of his persistent fever, the boy was referred to our hospital, and laboratory studies were performed.
On examination at admission, a firm, tender right anterior axillary lymph node, 3.5 cm in diameter, was palpable. The cervical lymph nodes were not palpable, and there was no overlying erythema or warmth. Hepatomegaly and splenomegaly were palpable. The blood counts were as follows: hemoglobin 10.2 g/dL, leukocytes 2,400/µL (neutrophils 40.2%, lymphocytes 50.8%, and monocytes 2.0%), and platelets 13.0×104/µL. However, leukocytes had increased to 13,510/µL in the previous 14 days. The erythrocyte sedimentation rate was 32 mm/hour. The level of aspartate aminotransferase was 87 IU/L (normal range: 11-33 IU/L), alanine aminotransferase was 55 IU/L (normal range: 6-43 IU/L), lactate dehydrogenase was 571 IU/L (normal range: 120-245 IU/L), alkaline phosphatase was 843 IU/L (normal range: 430-1,200 IU/L, see the details below), and C-reactive protein was 0.11 mg/dL (normal range: less than 0.15 mg/dL). Serology titers for Epstein-Barr virus, cytomegalovirus, Bartonella henselae, herpes simplex virus, Toxoplasma, adenovirus, and HIV were negative. A Mantoux test was negative after 48 hours. Anti-nuclear antibody was negative. Bone marrow examination revealed normocellular marrow.
A computed tomography (CT) scan of the lungs showed swelling of the mediastinal lymph nodes and interstitial pneumonia, accompanied by scattered smaller lymph nodes in both lung fields (Figure ).
A CT scan of the thorax disclosed right axillary lymph nodes and swelling of the many thoracic wall lymph nodes (Figure ). A CT scan of the abdomen and pelvis showed swelling of the mesenteric lymph nodes and inguinal lymph nodes. The results of cardiac ultrasonography were normal, with no aneurysms.
Blood, sputum, throat swab, and stool cultures were negative. The examination of a section of the axillary lymph node obtained by biopsy was compatible with KFD (Figure ). His parents gave written informed consent for the biopsy. Recovery was observed after the excision of the lymph node, and no further specific treatment was required. The fever continued for 30 days in his clinical course overall.


A 54-year-old man received a diagnosis of squamous cell carcinoma of the floor of the mouth. The tumor extended to the symphyseal portion of the mandible and fungated through the skin in the submental area. After bilateral selective neck dissections and composite floor of the mouth, partial tongue, and mandible resections, the residual defect measured 168 cm2.


A 46-year-old man (160 cm, 79 kg, body mass index: 30.9 kg/m2) was emergently admitted to the neurological department of our institution with tracheal intubation because of sudden respiratory failure at night. He had no congenital malformations or developmental, cardiovascular, respiratory, or endocrine disorders. At age 38 years, a high-pitched snore was noticed by his partner. When he was ~44 years old, he began complaining of neck and trunk rigidity, bradykinesia, postural tremor, and dysuria. At age 45 years, he was diagnosed with MSA with predominant parkinsonism.
As pulmonary inflammatory diseases were excluded by serologic and radiological examinations and swallowing function was substantially normal, we considered the possibility of MSA-related central respiratory disorder or obstructive respiratory disorder due to MSA and/or obesity. Tracheostomy was performed to maintain the airway after extubation, although continuous positive airway pressure may have also been helpful. At that time, physical examination revealed his temperature to be 36.5 degree Celsius, heart rate 77 bpm, respiratory rate 24, BP 108/81, and O2 saturation 95% on room air. Blood gas analysis revealed a pH of 7.40, carbon dioxide partial pressure of 33.8 mmHg, and oxygen partial pressure of 74.7 mmHg. Polysomnography showed obstructive SA (OSA), but not central SA. Awake fiberoptic laryngoscopy displayed no upper-airway obstruction but slight vocal-cord abduction impairment during inspiration, although the patient could endure the laryngoscope for <1 min because of discomfort. Sleep laryngoscopy was considered for upper-airway evaluation, although it cannot simultaneously evaluate different sites, but the patient refused because of discomfort. To assess the spatial and temporal conditions of the upper respiratory tract, including the nasal passages, pharynx, larynx, and portion of the trachea, we performed 4D-CT during sleep. The effective dose was 1.84 mSv (CT dose index: 19.5 mGy, dose–length product: 311 mGy.cm).
We placed the patient in the supine position, sedated him with intravenous administration of 5 mg diazepam, and closed the tracheostoma with adhesive tape. Reconstructed 4D-CT images of respiration during sleep (; , ) revealed abnormal upper-airway findings compared with the images acquired during awake respiration: glottis closure at the terminal stage of inspiration (normally, the glottis opens during inspiration) (), subsequent velopharyngeal closure (normally, the velopharyngeal space is open during nasal respiration), and succeeding glottis opening and expansion of pharyngeal pressure, but no other upper airway-obstruction findings, such as glossoptosis, pharyngeal-cavity narrowing, and retrodisplaced epiglottis ().
Thus, MSA-related sleep-induced airway obstruction was considered the cause of the respiratory failure. Consequently, retaining the tracheostoma was determined to be indispensable, although central apnea after tracheostomy may cause sudden central origin-related death; 14 months postoperatively, the patient had experienced no further airway-related complications.


An 81-year-old Hispanic male was found to have a 1.2-cm mucosal nodule along the incisura of the stomach (Figure ).
Pathology of the biopsy specimen revealed high-grade dysplasia in the setting of intestinal metaplasia, and he subsequently underwent endoscopic ultrasound (EUS) examination. EUS confirmed the presence of an isolated mucosal nodule with no deep invasion into the submucosa or muscularis propria (Figure ).
The decision was made to resect the lesion en bloc using ESD (Figure ).
A standard gastroscope was fitted with a distal attachment (cap) and introduced to identify the area of interest, along the lesser curvature of the stomach. An ERBE I-type Hybridknife was used to demarcate the peripheral margins of the lesion in a circular fashion (Figure ).
The ERBE I-type Hybridknife was then used to inject a mixture of saline and methylene blue into the submucosal plane (Figure ).
The lesion lifted symmetrically, and an en bloc ESD was successfully performed (Figure ).
On completion of the ESD, the residual mucosal defect was approximately 3 cm in diameter. The mucosal defect was closed with a running endoscopic suture (Figure ).
Pathology of the specimen showed multifocal high-grade dysplasia arising in the background of extensive intestinal metaplasia (Figure ). The resection margins were focally involved by intestinal metaplasia with low-grade dysplasia, and the deep resection margin was unremarkable.
In light of the persistent residual intestinal metaplasia with low-grade dysplasia, the patient returned for HAPC of the residual metaplastic mucosa (Figure ).
The scar site of the ESD was identified at the incisura. The gastric mucosa was lifted with a solution of saline and methylene blue and ablated in a circumferential manner using HAPC at a power setting of 50 W (Figure ).
HAPC was applied in a circumferential manner for comprehensive mucosal coagulation extending from the ESD scar to a 2-cm circumferential margin (Figure ).


A 20-year-old male trout farm worker presented to his local hospital with a 3-day history of malaise, fevers, diarrhoea, vomiting and jaundice. He developed haemoptysis, severe headaches, neck stiffness and photophobia, leading to emergency admission. There were no rashes, swellings, nor melaena. He had no significant past medical history and was taking no prescription medications. He was an occasional smoker and alcohol drinker. He remained hypoxic despite supplemental oxygen and after a short admission to the high-dependency unit he was intubated and ventilated in the intensive care unit (ICU). A post-intubation chest x-ray showed four-quadrant airspace ‘ground glass’ opacification (Fig. ). His admission bloods demonstrated haemoglobin (Hb) 99 g/l, platelets 25,000/μl, white blood cells (WBC) 10,400/μl, C-reactive protein (CRP) 195 mg/dl, creatinine 392 μmol/l, urea 17 mmol/l and total bilirubin 117 μmol/l. HIV, autoimmune, vasculitis and atypical pneumonia blood and/or urine screens were negative.
Empirical ceftriaxone was initiated following sequential escalating regimens of amoxicillin, gentamicin, metronidazole, then piperacillin-tazobactam and levofloxacin, following local microbiology advice. Intravenous doses of 1 g methylprednisolone daily for two days were initiated to treat the possibility of vasculitis associated pulmonary haemorrhage, pending results. He received early renal replacement therapy for acute kidney injury (AKI). A high-resolution CT scan of the chest after intubation demonstrated pneumomediastinum, pneumoperitoneum and extensive dependent consolidation. Due to the risk of worsening pneumothoraces and ventilator-induced lung injury (VILI) on optimised conventional ventilatory management, and concerns about the risk of prone positioning in this context, he was accepted for retrieval on the national severe acute respiratory failure (SARF) pathway. At the time of referral, he had SpO2 94%, pO2 11.4 kPa, pCO2 6.5 kPa and pH 7.29 on pressure-controlled ventilation with FiO2 1.0, positive end-expiratory pressure (PEEP) 15 cmH2O, total inspiratory pressure (Pinsp.) 30 cmH2O, tidal volume (VT) 450 ml, compliance 30 ml/cmH2O, PaO2/FiO2 ratio 86mmHg and a Murray score of 3.75. He was commenced on VV-ECMO (using a standard bi-femoral configuration: 25-French access cannula and a 23-French return cannula) at 2930 revolutions per minute (RPM), generating a blood flow rate of 4.7 L/min with a sweep gas flow of 5.0 L/min of 100% oxygen to facilitate lung protective ventilation.
At this ECMO centre, a further high-resolution CT scan was performed demonstrating persistent consolidation with pronounced branching nodularity and extensive mediastinal emphysema (Fig. a) with a normal CT head, abdomen and pelvis. The pneumomediastinum prevented a full echocardiographic examination but subcostal windows demonstrated preserved left ventricular function, non-dilated ventricles and no significant mitral or tricuspid regurgitation. On day 1 of his time on ECMO, positive serum Leptospira Panbio IgM ELISA and dual-target real-time 16SrRNA/Lipl32 polymerase chain reaction (PCR) results became available, confirmed by the reference laboratory, to which Public Health England was alerted. Further tests including blood β-d-glucan and galactomannan, as well as cultures, mycoplasma, viral PCR screen and acid-fast bacilli (AFB) from broncho-alveolar lavage (BAL) were all negative.
*Top images at the level of the carina; bottom images at the level of the superior pulmonary veins
The patient was kept on lung-protective ventilation using bi-level positive airway pressure (BiPAP) with PEEP 5 cmH2O, Pinsp. 12 cmH2O and VT 250–300 ml, rate 10/min. He was sedated and paralysed with a cis-atracurium infusion for the first 24 h. He received low-dose noradrenaline to keep his mean arterial pressure (MAP) above 65 mmHg but no inotropic support. Haemodiafiltration with regional citrate-based anticoagulation was required until day seven of the admission. Methylprednisolone was continued at a maintenance dose of 60 mg bd for 4 days until the full vasculitic screen had come back negative, with fluconazole added prophylactically in view of the steroid burden. Ceftazidime and gentamicin were started empirically for rising inflammatory markers and fever on day 6 of ECMO and continued for 7 days. The patient received 8 days of ECMO support. His coagulation studies were normal throughout the admission, but his platelets were 56,000/μl on arrival, with a prior nadir of 25,000/μl. Systemic heparinisation was therefore initiated cautiously with a target anti-Xa level of 0.2–0.3 IU/ml according to local policy.
Serial bronchoscopic examination by an experienced bronchoscopist initially showed old clots in the lower lobes bilaterally but on day 3 of the ECMO run, fresh blood was seen with mucosal bleeding points in the apical segments of the right and left lower lobes; these were treated with 2 ml of 1:100,000 adrenaline to achieve haemostasis. There was no central airway source of bleeding on careful inspection. His anti-Xa target was reduced to 0.2 IU/ml and tranexamic acid 1 g IV bd was commenced. A liver ultrasound was normal, hepatitis A and E serology was negative and a review from hepatology confirmed that the enzyme picture fitted leptospirosis and treatment should be supportive.
On day 9 at the Royal Brompton Hospital, the patient was decannulated from ECMO whilst still receiving lung protective ventilation. The patient was extubated three days post-ECMO decannulation. A post-decannulation vascular ultrasound revealed no deep vein thromboses and he was treated with 5000 units of unfractionated heparin subcutaneously twice daily, in view of his abnormal renal function and restored platelet counts. He was extubated 3 days after decannulation from ECMO and repatriated to his local hospital where he made a full recovery.
At 9 months follow-up, he was clinically better, with resolution of the CT scan findings (Fig. b), a normal 6-min walk test distance > 400 m, and near normal lung function, albeit with low normal gas transfer (FEV1 5.68 L, > 100%, VC 6.7 L, > 100%, TLCO 79.8%, KCO 74.2%).


An HIV-positive 4-year-old boy presented to clinic for evaluation of worsening skin lesions after starting ART. His absolute (percentage) CD4 count at the time of ART initiation was 32 cells/μL (3%) (Fig. ), and he was started on ART with zidovudine-lamivudine-nevirapine. Approximately 3 months after ART initiation, he developed > 30 violaceous papules and plaques, a necrotic edematous circumferential plaque on the distal shaft and glans of the penis, and scattered small subcutaneous nodules in his submandibular and cervical regions (Fig. , panel a). He was clinically diagnosed with Kaposi sarcoma secondary to the immune reconstitution inflammatory syndrome due to the classic appearance of his cutaneous lesions. His ART was shifted to abacavir-lamivudine-lopinavir/ritonavir due to anemia (hemoglobin 7.2 g/dL) and potential activity of protease inhibitors against human herpes virus-8 [, ]. He was started on a chemotherapy regimen of bleomcyin (15 U/m2) and vincristine (1.4 mg/m2) every 2 weeks. Biopsy was not obtained initially due to logistical and resource constraints.
After five cycles of this chemotherapeutic regimen, doxorubicin (25–35 mg/m2) was added based on failure to achieve complete clinical remission. The patient completed an additional 10 cycles of bleomycin-vincristine-doxorubicin every 3 weeks without complete remission of the violaceous lesions or penile nodule. During the course of chemotherapy, he achieved full virologic suppression and his CD4 count had increased to 265 cells/μL (13%) after 143 days of antiretroviral therapy (Fig. ). However, his KS lesions continued to persist, and chemotherapy was shifted to monthly paclitaxel. He eventually completed 12 cycles of paclitaxel monotherapy with dexamethasone as a premedication and showed improvement and full resolution of the violaceous skin and penile lesion. However, during this time he developed numerous additional subcutaneous nodules on his trunk, tongue, and extremities that rapidly and progressively increased in size in number (Fig. , panel b).
A biopsy was taken for further investigation. Histopathology of the nodule revealed a cystic structure with a central larval scolex and serrated spiral canal consistent with T. solium, which confirmed the diagnosis of DC (Fig. ). Additional history taking with the caregiver revealed that the patient and his family consumed pork frequently as a regular part of their diet.
Chemotherapy was stopped and he was treated for DC with albendazole 7.5 mg/kg twice daily and praziquantel 25 mg/kg twice daily for 10 days. Prednisone was administered as an adjunctive treatment to limit the possibility of cerebral edema as NCC could not be ruled out (computed tomography scans of the head were unavailable). He had almost complete resolution of all subcutaneous nodules during his 10-day treatment course (Fig. , panel c). All treatment was well-tolerated with minor toxicity (anemia). A small sublingual nodule persisted acutely following treatment, fully resolving several months later. He has not had recurrence of KS or DC after 42 months of follow-up.


A 28-year-old female was referred to us for the management of a persistent lesion on the floor of the mouth. Intraoral examination showed a red-brown firm, painless, and round lesion of the ventral tongue that had been present for six months (Figure ). Diode laser-assisted excision was suggested, and the patient agreed to the procedure. With minimal local infiltration of anesthesia, the lesion was surgically removed using a diode laser with a wavelength of 800 ± 10 nm, in continuous modality, and with an output energy of 1.5 W (Figure ); no intraoperative bleeding was observed and stitches were unnecessary. Histological examination revealed a cystic lesion containing mucin with a surrounding epithelial lining, consistent with a diagnosis of BNM (Figure ). The surgical wound healed completely in 12 days.


The patient was a 10-year-old boy (23kg) who had epidermolysis bullosa (EB) disorder referred from a local clinic from Nehbandan City in 2018, Northeastern of Iran. He suffered from periodic painful episodes in urinary system for the past 9 months. Symptoms were intermittent, and urine contained brown sediments similar to bladder stones (). There were no signs of gross hematuria and fever during the mentioned time. Complete medical examinations including urine analysis, urine culture, urine and blood biochemistry, hematology, Immunoassays for autoimmune diseases, thyroid function tests and renal ultrasound were performed.
Ultrasound was performed after observation of the excreted brown sediment from urethra for finding kidney stones. Kidney and bladder appeared normal in ultrasound. Urinalysis revealed no abnormality except for occult blood. Urinary culture was negative. Blood cell count and creatinine levels showed the normal ranges. Moreover, urine biochemistry was normal. Erythrocyte sedimentation rate (ESR) was 21 mm/1h.
The boy’s mother found three larvae in his urine more than two weeks after symptoms start. Morphologically, the larvae had six short legs close together near the head. The head has a pair of short hooks and was creamy white in color. The larvae had 26mm length and 0.5 mm width. They had three pairs of feet on the belly near the head and with each foot had 4 sections and nodes hocks curved claws (). Based on the characteristics, the larva was diagnosed as T. molitor.
Debris, hyperemia and inflammation were identified in bladder and in the urethra cystoscopy (). However, no larva was seen in bladder and urethra. A single dose of 3mg of ivermectin was orally administrated. The patient recovered after oral Ivermectin therapy. Following treatment, high volume of insect shells was repulsed (). In addition, the abdominal pain completely resolved within a few months following treatment. Histopathology of excreted particles from urethral duct showed keratinizing squamous metaplasia after treatment.
Informed consent was taken from the patient’s parents. This study was reviewed and approved by the Ethics Committees of Mashhad University of Medical Sciences, Iran.


A young 24-year-old male presented at our institution for intense epigastric pain, radiating to the right hypochondrium and to the lumbar region, not related to meals nor during nighttime. Informed consent was obtained from the patient studied. All procedures performed were in accordance with the ethical standards of our institutional research committee (IRCCS Giovanni Paolo II, Bari). Previously, for several months, the patient had experienced upper abdominal pain and gained partial benefit from the administration of high dose proton pump inhibitors (PPI). An upper gastrointestinal endoscopy (esophagogastroduodenoscopy, EGD) did not reveal specific erosions or inflammatory lesions of the gastric mucosa, and Helicobacter pylori infection was absent. Further workup with abdominal computed tomography (CT) revealed an ovoid lesion measuring 1.3 cm, slightly hypervascularized and exophytic, adjacent to the lateral margin of the head of the pancreas, in the space between the duodenal bulb and the inferior vena cava (Fig. ). Magnetic resonance imaging (MRI) revealed a single peripancreatic lymph node, suspected to be a metastatic lymph node. No evident injury to the pancreas, biliary tract, stomach, and duodenum was observed. All laboratory test results were within normal limits, except for a significantly elevated fasting gastrin level at 245 pg/ml (normal < 40 pg/ml), chromogranin A level > 2000 ng/ml (normal 10-110 ng/ml), with gastric pH < 2 (off PPI). Therefore, it was not judged necessary to perform the secretin stimulation test, also owing to the gastroscopic exclusion of G-cell hyperplasia. Further laboratory evaluation ruled out a multiple endocrine neoplasia type 1 syndrome (MEN1). Finally, the patient underwent laparotomy, and gave consent to a possible pancreatic-duodenal resection.
Meticulous surgical exploration of the operative field (upper abdomen, including manual duodenal exploration, and sonography of the liver and pancreas) demonstrated the lesion in the pancreatic head, with a diameter of about 2 cm. The nodule, approximately 2 cm in diameter with a smooth, regular surface, was excised and a frozen section submitted to pathology for extemporaneous intraoperative examination. Intraoperative frozen sections analysis disclosed a single lymph node measuring 1.5 cm in maximum diameter, almost completely replaced by a tumor whose appearance was consistent with a low-grade neuroendocrine tumor (NET-G1). After careful exploration of the gastrinoma triangle, no lesion other than the one identified preoperatively was found at surgical exploration and intraoperative ultrasound (IOUS); therefore, further procedures were suspended and the abdomen was closed. The definitive histology and immunohistochemistry analysis on paraffin-embedded sections confirmed a lymph nodule of a well-differentiated neuroendocrine tumor (WD - NET), with less than 3% of cells staining positive for Ki67 (MIB-1) and a number of mitoses < 2 for 10HPF, thereby classified in accordance with 2017 World Health Organization (WHO) classification [] as a NET G1 (Fig ). On immunohistochemistry, the lesions showed strongly positive staining for chromogranin and synaptophysin and weakly positive for gastrin (Fig. ). The diagnosis of a well-differentiated, low-grade, neuroendocrine tumor (NET-G1) was made. The patient’s post-operative course was uneventful and he was discharged home 1 week later. After a post-operative follow-up of 4 months, the patient was asymptomatic, showed significantly improved general conditions and his fasting gastrin and chromogranin A levels were within normal limits.