A 31-year-old Caucasian woman presented with 3 months of progressive right arm weakness, speech disturbance and seizures. Neurological examination showed dysarthria, dysphasia (reduced verbal fluency and word finding difficulties), right-sided hemiparesis and hemisensory loss. There were no signs of systemic disease, intercurrent illness, stigmata of neurofibromatosis or Down syndrome. She had no personal or family history of stroke. She was an active smoker of 10 cigarettes a day for 10 years. She was also a chronic cannabis and methamphetamine user, which escalated in the 9 months prior to admission to daily methamphetamine smoking.
Brain MRI revealed both bilateral embolic and watershed infarcts in frontal regions. CT angiography showed development of new lenticulostriate collateral vessels, suggestive of moyamoya pathology (). Digital subtraction angiography (DSA) demonstrated steno-occlusive disease of bilateral anterior circulations, confirming the diagnosis ().
Transthoracic echocardiography did not show a cardioembolic source of stroke. Lumbar puncture, vasculitis screen, autoimmune screen and thyroid function studies were unremarkable. Hepatitis and viral serology were negative. She was not hypertensive.
A repeat MRI demonstrated a new asymptomatic left frontal lobe infarct, which may have been related to the DSA procedure or embolism from her stenotic disease (). She was subsequently started on clopidogrel in addition to aspirin to prevent further intracranial artery-to-artery embolism. Nimodipine was prescribed to treat any underlying cerebral vasospasm and carbamazepine was started for seizure prophylaxis.
She underwent an 8-week period of inpatient rehabilitation. At outpatient review 4 months later, she had clinically improved and experienced no further stroke symptoms while on dual antiplatelet therapy and ceasing methamphetamine. Repeat MRI showed no new ischaemic changes but the appearance of the intracranial vessels was unchanged. She continues to be managed conservatively with consideration of neurosurgery in the event of further neurological deterioration.


A 13-year-old male presented with a midline neck swelling since birth with growth retardation and hypothyroidism, he required L-thyroxine (L-T4) replacement for 5 months. 99mTc-pertechnetate thyroid scintigraphy revealed features suggestive of dual ET tissue, one in the prehyoid region, and another small focus in the lingual region with the absence of orthotopic thyroid gland [].


A 17-year-old male presented with a midline suprathyroid neck swelling of 2 years duration with hypothyroidism, requiring thyroxine replacement. 99mTc-pertechnetate thyroid scintigraphy was done following the withdrawal of L-thyroxine for 3 weeks, which showed the presence of dual thyroid ectopia with an ovoid focus in the neck in the subhyoid region in the midline and another small focus in the lingual region []. The orthotopic thyroid gland was not seen.


An 8-year-old female child presented with midline neck swelling of 4 months duration. She had hypothyroidism and was on L-T4 replacement. Ultrasonography (USG) neck showed an oval mass in the pretracheal region with the absence of an orthotopic thyroid gland. Thyroid scintigraphy revealed a large ovoid focus seen in the suprahyoid region of the neck with an additional small focus of thyroid tissue in the lingual region, suggestive of dual thyroid ectopia []. Following scintigraphy, the patient was examined and a pea size swelling with a smooth surface was seen at the posterior third of the tongue.


A 7-year-old male child presented with midline neck swelling since birth with euthyroid status. USG neck revealed the absence of an orthotopic thyroid gland, but a 2.0 cm × 2.2 cm × 0.8 cm size, nodular swelling was seen in the subcutaneous plane, suprathyroid, midline neck region, having echotexture, and vascularity similar to thyroid tissue with suspicion of the thyroglossal cyst (TGC). 99mTc-pertechnetate thyroid scintigraphy showed features suggestive of dual ET tissue, larger one in midline neck suprathyroid region, and another small focus in the lingual region with the absence of orthotopic thyroid gland [].


A 25-year-old woman with no medical history visited our hospital with left eye ptosis and diplopia. She was working as a nurse in a pediatric clinic and had, therefore, been vaccinated for prevention of influenza. She developed symptoms 2 weeks after the influenza vaccination. She did not complain of ocular pain or other neurological symptoms. In addition, she had no clinical signs or symptoms of possible autoimmune disorders like lupus or Sjogren's syndrome. She was a social drinker and a nonsmoker. She had no family history of neurological diseases.
On general physical examination, her blood pressure was 132/74 mm Hg. Neurological examination revealed a dilated pupil in the left eye, severe ptosis, and diplopia with reduced adduction, elevation, and depression of the left eye, which suggested complete left oculomotor palsy (Fig. ). Other neurological examinations showed no abnormalities.
The blood tests, including the complete blood count, erythrocyte sedimentation rate, electrolytes, creatinine, total cholesterol, triglyceride, fasting glucose, and glycosylated hemoglobin levels, were normal. Autoantibodies for vasculitis, including anti-neutrophil cytoplasmic antibodies, anti-nuclear, cardiolipin, ds-DNA, phospholipid, and Ro/La antibodies were negative. Anti-ganglioside antibodies (IgG, IgM) including GM1, GD1b, and GQ1b were also negative. Cerebrospinal fluid (CSF) analysis showed the following results: red blood cells 0/UL, leukocytes 0/UL, protein 38 mg/dL, glucose (CSF) 65 mg/dL, and glucose (serum) 102 mg/L (ratio 0.64). The brain computed tomography (CT) and brain CT angiography were unremarkable. Orbital magnetic resonance imaging (MRI) revealed enhancement of the left oculomotor nerve (Fig. ).
The patient was treated with Solu-Medrol® (methylprednisolone sodium succinate) 1,000 mg/day i.v. for 5 days. The pupillary light reflex was restored after 3 days of high-dose steroid therapy. On the following day, ptosis and diplopia began to improve. After 4 weeks of persistent symptoms, gradual and complete recovery was achieved.


A 32-year-old nulliparous married woman presents with complaints of chronic lower abdomen pain, dyspareunia, and infertility. Her general physical examination was normal with body mass index of 22 kg/m2. On speculum examination, a longitudinal vaginal septum was seen in the proximal portion of the vagina and the cervix was partially seen beyond the septum, at a high up position []. On vaginal examination, a single cervix was felt high up, slightly deviated to the right, and a normal anteverted uterus. An ultrasound showed a subseptate uterus with midline thick muscular septum and normal adnexa. MRI revealed septate uterus with septa extending till the cervix [Figures and ]. No anomaly was found in any other organ.
To complete the diagnosis, consent was taken for vaginal septum removal, followed by diagnostic hysterolaparoscopy. The couple was given the option of septoplasty in the same sitting or later in case of pregnancy complications. They chose to have it later.
Under anesthesia, the vaginal septum was held in between two alles forceps and divided in between the two with cautery. After the septum was removed, two dimples were seen high up in the vagina. They were double cervix confirmed by magnification with the hysteroscope. Two separate sounds could be inserted through the two cervices, ruling out any cervical atresia []. The uterocervical length in both sides was 3.5 inches. Hysteroscope was introduced through both the cervices. The septum was found to be complete till the level of the cervix delineating two separate cervical canals and two cavities. Cavities on both sides of the septum were found to be similar in size. The two ostia were visualized on the respective sides of the uterus. On introducing the laparoscope, the fundus was found to be smooth with a 2 cm fundal midline fibroid and with normal tubes and ovaries []. Thus, a diagnosis of complete uterine septum with two cervices and longitudinal vaginal septum was made. On chromopertubation spill was seen on both sides.
After 6 weeks of the vaginal septum resection, she reported an improvement in the dyspareunia and the heaviness in the lower abdomen []. She is being kept under follow-up to look for pregnancy complications and manage accordingly.


A 70-year-old male patient reported suffering from lumbosacral pain and walking difficulty for several months.
From his personal medical history, he had arterial hypertension disturbance and he had undergone a bilateral total knee replacement and a total right hip replacement several years algo. He did not refer claudication and nor neurological damage was found during the clinical examination. The patient referred important impairment since he found it difficult to maintain an upright position, presenting a limitation to keep the horizontal field of vision, and needing a walking stick to stand still and to walk.
A standing full spine X-ray assessment showed rigid spine signs with anterior spinal osteophytes due to the calcification of the anterior longitudinal ligament (ALL), which is compatible with DISH. The sagittal vertical axis (SVA) C7-S1 was 22 cm, the lumbar lordosis (LL) was 10º, and the spinopelvic parameters showed a pelvic incidence (PI) of 46º, a sacral slope (SS) of 23º and a pelvic tilt (PT) of 23º (). In the pelvic and lower limbs X-Ray images, both pelvic and knee replacements were adequately positioned. The acetabular component inclination angle was 59º and anteversion cup angle of 24.6º. Measurements were performed following Lewinnek method using Surgimap (Nemaris) software. The magnetic resonance (MR) revealed a decrease of LL and signs of moderate lumbar spinal stenosis, mostly at the L3-L4 level. Supine decubitus with bolster in the apex of the deformity X-Ray image was done to check for deformity’s flexibility ().
Information about the different surgical and non-surgical options was provided to the patient. After trying medical treatment with no clinical response, a surgical procedure by a spinal osteotomy was agreed with the patient.
Positioning the patient in prone, using somatosensory evoked potentials (SSEP), motor evoked potential (MEP) and electromyography (EMG) monitoring, a posterior approach was performed, which began with the subperiosteal dissection of paravertebral muscles and bilateral T11-S1 pedicle (except from L3) and iliac screws were used. This was followed by an L2 partial inferior and an L4 partial superior laminectomy, with the facetectomy of both L2 inferior facet joints, were performed. Then, an L3 pedicle subtraction osteotomy was performed. At the L4-L5 levels, a circumferential arthrodesis via transforaminal interbody fusion (TLIF) with interbody Polyetheretherketone (PEEK) implants and autologous bone from the posterosuperior iliac spine (PSIS) was performed. Finally, a T11-S1 postero-lateral arthrodesis with autologous bone from the surgical field was executed to prevent mechanical failure.
In the immediate postoperative period, surgical wound drainage was observed, with the microbiological confirmation of an infection by Klebsiella oxytoca, Enterococcus faecalis, Corynebacterium amycolatum and Finegoldia magna, which was treated and resolved after debridement, irrigation and antibiotics, without any further complications.
Two years after the surgery, good clinical assessment was observed, not referring any lumbosacral pain or neurological damage and not needing the use of a walking stick. The X-ray images showed improvement presenting the following and in comparison to the previous 2 years as follows: a SVA of 64 mm (from a SVA of 22 cm), a LL of 30º (from 10º), PI of 46º, a SS of 18º (from 23º) and a PT of 28º (from 23º). Postoperative inclination angle of acetabular cup was 59º and anteversion of 27.7º. Complications related to THA such as impingement or luxation weren´t observed ().


In 2015, a 60 years old male patient presented to the hospital with complaints of pain and swelling in right hip region and inability to bear weight on right lower limb for 15 days. Patient had history of motor vehicle accident and sustained injury to the right hip region. He had not taken any treatment previously and presented to us for the 1st time. On clinical examination, right lower limb was adducted and internally rotated. Movements at right hip joint were painful and restricted. Swelling was present in right gluteal region and vascular sign of Narath was positive. Distal neurovascular structures were intact. Radiograph of right hip revealed posterior dislocation and femoral head fracture with a large intra-articular fragment. There was disruption of posterior acetabular wall with a displaced acetabular fragment posterosuperiorly. The ilioischial and iliopectineal lines were intact as was the obturator ring. The femoral neck was also intact ().
Pre-reduction non-contrast computed tomography scan was performed for further evaluation. Images were reconstructed in bone window and volume rendered images were also obtained. The study confirmed the findings of radiography and revealed right posterior hip dislocation with femoral head fracture confined to the part of head caudal to Fovea Centralis. There was a coexisting isolated acetabular posterior wall fracture. Hence, the lesion was classified as Pipkin type IV ().
Reduction of hip dislocation was attempted in emergency room but found to be unstable. Patient was kept on skeletal traction for 24–48 h and subsequently planned for open reduction and internal fixation. Kocher-Langenbeck approach was used which allowed direct visualization of the dorso-cranial part of acetabulum. Femoral head fracture was fixed using Herbert screw and recon plate and spring hook plates were used to fix acetabular fracture fragment and to stabilize the reduction ().
Post-operative period was uneventful. Hip joint was kept in abduction and slight external rotation for optimal healing while allowing controlled flexion and extension. Hip muscles strengthening exercises were started slowly and gradually as tolerated by the patient. Bony union was noted 12 weeks postoperatively () and after 14 weeks patient was able to sit and squat easily.
In follow-up, patient was evaluated clinically and radiologically. There were no signs of avascular necrosis and arthritis after 5 years of follow-up. Patient had rewarding outcomes in the form of adequate range of motion and painless ambulation ().


Post-mortem examination of a 15 + 2 deceased female foetus born to a 39-year-old healthy woman revealed isolated bilateral kidney agenesis. The father of the foetus was diagnosed with kidney hypoplasia and minimal change nephropathy at the age of 36 years due to persistent proteinuria. Subsequently, he was diagnosed with hypertension and hyperuricaemia, both requiring medical therapy. However, at the age of 53 years, kidney function was only mildly impaired. The father has a strong family history of kidney disease ().
DNA extracted from foetal tissue was analysed using array comparative genomic hybridization. No pathogenic genomic copy number variants were identified. Subsequently, a gene panel (including CAKUT genes BMP7, CDC5L, CHD1L, EYA1, FRAS1, FREM1, FREM2, GATA3, GREM1, GRIP1, HNF1B, ITGA8, PAX2, RET, ROBO2, SALL1, SIX2, SIX5 and TBX18) was targeted for sequencing to identify putative disease-associated variants using next-generation sequencing. Smaller indels, up to 50 bp, were called in the variant analysis pipeline, whereas exon deletion/duplications were analysed using a copy number variation tool. These analyses only identified a heterozygous PAX2 missense variant c.68T>C [p.(Leu23Pro)].
Subsequently, the PAX2 candidate variant was assessed for cosegregation with kidney phenotypes in the family under study. The variant was identified in all family members affected by kidney disease for which DNA was available (). The kidney phenotypes seen in the family were remarkably heterogeneous, including bilateral kidney agenesis, minimal change nephropathy, ureteropelvic junction obstruction, duplex kidney with hydronephrosis of upper pole system and bilateral kidney hypoplasia with end-stage renal failure in early adult life or with only moderate chronic kidney failure in late adult life.
To study whether the severe foetal phenotype was exacerbated by additional variants in modifier genes, an additional 138 genes previously associated with kidney disease were analysed, including GREB1L, FGF20 and LMX1B. No additional candidate variants were identified.
Retinal examination of Patient III-3 revealed unilateral optic nerve coloboma and retinal examination of Patient IV-3 revealed unilateral optic pit ().


A 66-year-old woman with no prior cardiac history was admitted for primary percutaneous intervention with intermittent atypical chest pain radiating to the jaw for 1 day, and ST elevation on the electrocardiogram (ECG). Her past medical history included surgical removal of superficial melanoma of the thorax and left shoulder in 2008 and 2016, without evidence of metastasis. She suffered from impaired memory since 5 years, and probable CAA was diagnosed 2 months prior to the myocardial infarction. There were no other cardiac risk factors present at admission. Physical examination was without abnormalities.
Sinus rhythm was observed on the admission ECG, with normal heart axis, narrow QRS (120 ms) with ST elevation in leads V2 through V6. No conduction disturbances were present ().
A significant rise-and-fall of serum troponin was measured with a peak of 131 ng/L (cTnl (Cardiac-specific Troponin I) n < 14 ng/L). Other laboratory results were unremarkable with normal liver and renal function.
Radial approach coronary angiography was performed and the culprit vessel was shown to be the left anterior descending artery of the left coronary artery with a 100% obstruction in the distal segment. Two 2.5–16 mm drug eluting stents were placed with restoration of thrombolysis in myocardial infarction 2 flow (). Other coronary vessels were non-obstructed. Echocardiography with microbubbles (Optison™) injection revealed an LV function of 35% with hypokinesia of the anterior wall with akinesia of apical segments and aneurysm formation, without LV thrombi present (, Video Appendix).
Patient received medical treatment with aspirin, clopidogrel, a statin, a betablocker, and an angiotensin-converting enzyme inhibitor initially. During outpatient follow-up, LV function remained stable at 35%. An LV mass was detected after 6 months ().
The neurologist was consulted regarding this complication and its therapeutic consequences. A conjoined decision was made by the cardiologist and neurologist to discontinue aspirin and clopidogrel and start apixaban twice daily 5 mg, based on its efficacy- and safety profile in CAA patients with atrial fibrillation (AF) as well as multidisciplinary consensus. Despite stable cardiac function, she suffered from an ischaemic stroke 1 year later, the LV thrombus considered being the cause.
With no new ICH or hemosiderosis present on repeat brain magnetic resonance imaging (MRI), apixaban was discontinued and phenprocoumon [vitamin K antagonist (VKA)] was started. During follow-up, no complications or adverse events were reported.


A 64-year-old woman complained of a growing mass in her right lateral malleolus with intermittent blunt pain.
The growing mass in the right lateral malleolus lasted for > 12 mo, with intermittent blunt pain during the daytime for 3 mo.
The patient had no history of ankle trauma or surgery.
The patient had no previous or family history of similar illnesses.
A soft movable mass was palpated at the posterior lower margin of the right lateral malleolus tip, without symptoms of redness, erythema and drainage (Figure ); however, deeper palpation of the mass triggered severe blunt pain.
The following laboratory tests were all normal: complete blood count, serum biochemical test (including renal and liver function, creatine kinase, lactate dehydrogenase, and electrolytes), hypersensitive C-reactive protein, erythrocyte sedimentation rate, antistreptolysin O, rheumatoid factor, anti-cyclic citrullinated peptide, common tumor markers included a-fetoprotein, carcinoembryonic antigen, cancer antigen (CA)-125, CA-15-3, and CA-19-9.
X-ray and magnetic resonance imaging (MRI) of the right ankle were taken. The X-ray presented an osteophyte formation at the posterior margin of the subtalar joint and irregular soft tissue calcification (Figure ), whereas MRI showed a mass of low-intensity on both T1- and T2-weighted imaging protruding from the posterior margin of the subtalar joint into the soft tissue, with mild cartilage erosion (Figure ).
Based on the medical history, physical signs, and imaging examinations, a suspected diagnosis of diffuse PVNS of the right subtalar joint was made.
The mass was resected using the Kocher approach (Figure ). During surgery, we found deep brown soft tissue mixed with a small amount of cartilaginous tissue. The pedicle of this brown tissue was traced to the margin of the subtalar joint and the tendon sheath of the flexor hallucis longus tendon. After removing the mass in surgical vision, a 3.0 Steinmann pin was drilled into the lateral process of the calcaneal and talus, respectively. A retractor was used to distract the Steinmann pin (Figure ) (Huatrau, Chinatrau Instrument Co. Ltd., Guangzhou, China)[] until sufficient vision of the subtalar joint was achieved. The mass surrounding the articular surface was removed completely; however, some of the cartilage was mildly degenerated.


A 78-year-old male patient who complained of low back pain was diagnosed with spinal stenosis.
He was admitted to receive a unilateral laminectomy with bilateral decompression.
No other past history was reported.
The patient had been diagnosed with hypertension four years earlier and asthma more than 70 years earlier and was prescribed fluticasone furoate and vilanterol inhalers for the treatment of asthma; However, these medications were not used consistently.
Physical examination of the patient was not significant.
The results of pulmonary function tests were forced vital capacity 2.53 L (61%), forced expiratory volume at 1 s 1.37 L (52%), and forced expiratory volume at 1 s/forced vital capacity 54.
Chest radiographs showed subsegmental atelectasis in the left lower lobe and mild cardiomegaly (Figure ). Transthoracic echocardiography performed prior to surgery showed concentric left ventricular hypertrophy, trivial mitral regurgitation, and a mild relaxation abnormality with other findings within normal limits. As premedication, hydrocortisone 100 mg was administered on the morning of surgery. Lung sounds confirmed after the patient entered the operating room were normal, and anesthesia was initiated after prophylactic administration of salbutamol inhalants.
General anesthesia was induced with thiopental 250 mg and fentanyl 100 mcg, and the patient was intubated without special difficulty after administration of rocuronium 50 mg. Anesthesia was maintained at 50% N2O and 5%-7% desflurane. The operation was performed with the patient in the prone position, and there were no special events during the operation. The arterial blood gas analysis performed during the operation showed FiO2 0.5 to pH 7.420, pO2 242.5, pCO2 36.7, and base excess-0.6, and hemoglobin level was 12.2 g/dL. At the end of the operation, sugammadex 200 mg was administered for reversal of muscle relaxation, the patient’s position was changed from prone to supine under deep anesthesia, and the bispectral index score at that time was in the 40s. Subsequently, several aspiration attempts were made to remove the secretions from the trachea and oral cavity in order to manage the tracheal tube, but the patient was clenching his teeth and did not open his mouth. At this time, airway obstruction occurred for several minutes, making ventilation difficult. Oxygen saturation decreased by 40% on pulse oximetry, even though 50% of oxygen was provided and the concentration of inhalation anesthetics were not reduced. An additional 200 mg sugammadex was administered owing to severe airway obstruction caused by the patient clenching his teeth. After a few minutes, the patient stopped clenching his teeth, but a large amount of bloody discharge from the endotracheal tube was observed.


A 19-year-old woman was admitted to the emergency room of a general hospital with fatigue, lethargy, arthralgia, dyspnoea, and hypotension. The patient showed a positive shock index (blood pressure 82/55 mmHg, heart rate 132/b.p.m.) and strongly reduced overall condition. She had oral iron supplementation with iron(II)-glycine sulfate (ferro sanol duodenal 1 × 100 mg Fe/d) for iron deficiency anaemia and no other medication. Physical examination on admission showed reduced general condition, very pale skin, no pathologic murmurs, muffled breath sounds on both sides, diffuse abdominal pain with weakened peristalsis and without palpable resistance, no peripheral oedema, no focal neurological deficit, and full orientation. The electrocardiogram (ECG) revealed sinus tachycardia and low QRS voltage in all leads, but no signs of acute ischaemia, and no bundle branch block. A cardiomegaly was diagnosed by chest X-ray and subsequent transthoracic echocardiography showed pericardial effusion compressing right atrium and ventricle consistent with cardiac tamponade (). An immediate needle pericardiocentesis was performed, and a total of 800 mL serous pericardial effusion was relieved without leaving the pericardial catheter in the pericardium. Blood biochemistry and haematology showed elevated inflammatory parameters (leucocytes 14.4 G/L, C-reactive protein 78.7 mg/L, and procalcitonin 2.97 μg/L), manifest hypothyroidism (thyroid stimulating hormone 15.54 mlU/L and free triiodothyronine 1.01 ng/L), and hyponatraemia (123 mmol/L) (). Computed tomography revealed bilateral pleural effusions () and ascites, compatible with polyserositis.
The patient developed progressive shock with an increasing need for catecholamine support. Due to recurrent bradycardia and finally asystole, the patient had to be resuscitated for 15 min until we achieved return of spontaneous circulation. Since haemodynamic stabilization could not be achieved despite maximum non-invasive intensive care therapy, VA-ECMO) was used as salvage intervention to provide temporary circulatory support (). A portable VA-ECMO system was implanted by our mobile ECMO team directly on site and the patient was then transferred to the intensive care unit of our university hospital. However, the cause of circulatory failure initially remained unclear. After implantation of VA-ECMO, transthoracic echocardiography showed normal left ventricular function and normal left ventricular size. In contrast, right ventricle was dilated and showed reduced function. The cardiovascular magnetic resonance imaging did not detect any cardiomyopathy, in particular, we found no evidence suggesting myocarditis or an arrhythmogenic right ventricular cardiomyopathy (). Based on the suspected polyserositis, an extensive rheumatological/immunological diagnosis was performed. Diagnostic tests for antinuclear antibodies, anti-double-stranded DNA antibodies, rheumatic factor, cyclic citrulline, parvovirus B19 IgG and IgM, adeno-, cytomegalovirus and coxsackievirus IgG and IgM, and human immunodeficiency virus were negative as was the QuantiFERON test for tuberculosis (). Due to low cortisol level (0.4 μg/dL) adrenal insufficiency was proven. Adrenocorticotropic hormone levels were elevated (1021 pg/mL) which indicated PAI. In addition, an ACTH-stimulation test was performed and confirmed adrenal insufficiency as there was an insufficient increase in serum cortisol (). Serum cortisol levels <18.0 μg/dL (<500 nmol/L) at 30 or 60 min in an ACTH-stimulation test are sufficient to confirm adrenal insufficiency. Furthermore, 21-Hydroxylase antibodies as markers of an autoimmune Addison’s disease were positive and finally confirmed diagnosis. Computed tomography of the abdomen revealed no morphological alteration of kidneys and adrenal glands on both sides (). We immediately started an appropriate substitution therapy with hydrocortisone and fludrocortisone according to the substitution scheme illustrated in with initial intravenous bolus dose of 100 mg hydrocortisone followed by a subsequent continuous intravenous infusion of hydrocortisone 200 mg/24 h as long as the patient required catecholamines. This was followed by a gradual tapering of the hydrocortisone dosage within the next days according to clinical status of the patient. The patient’s condition improved, VA-ECMO could be removed and the patient was extubated. The right ventricular function recovered completely, and pericardial effusion was no longer detectable (). In line with this, the initial low-voltage diminished on ECG. Likewise, pleural effusions () and ascites almost completely regressed therefore no drainage throughout the hospital stay was necessary. The transthoracic echocardiography at discharge confirmed normal biventricular function with normal left and right ventricular size. Initially, short-term levothyroxine substitution was necessary due to manifest hypothyroidism. The thyroid function, however, normalized in the course of the disease and finally no further substitution was necessary. In the absence of clinical evidence of a polyglandular autoimmune syndrome (no diabetes, no autoimmune hepatitis, no permanent hypothyroidism, no mucocutaneous candidosis, and no hypogonadism) no further antibody diagnosis was necessary. The intravenous hydrocortisone therapy was gradually reduced and then given three times daily per os ().
After a total stay of 4 weeks in the hospital, the patient has completely recovered and could be discharged. Prior discharge, she received an extensive patient training in the handling of the disease, in particular, adjustments to the cortisone dose in situations with an increased need for cortisol (fever, infection, vomiting, diarrhoea, and surgical interventions) and the timely detection and treatment of an Addison crisis. She was given an emergency pass including personal data and emergency treatment instructions () as well as an emergency medical kit with hydrocortisone powder and a solution for preparing an intramuscular injection as well as cortisone suppositories (). In addition, the patient’s twin sisters were also contacted and testing via the endocrinological outpatient clinic was recommended. The patient is doing very well to this day and symptom-free.


A 79-year-old woman was admitted to the emergency department with complain of dyspnoea the day before the first anniversary of her husband’s death. She had been affected with persistent atrial fibrillation, taking bisoprolol and apixaban. An initial ECG showed no significant ST-T segment change. Her serum troponin T, creatine kinase (CK), and CK-MB were within normal range. However, transthoracic echocardiogram (TTE) revealed severe hypokinesis of mid- and apical-anterior segments with reduced LV ejection fraction (LVEF) of 45%. Chest X-ray showed bilateral pleural effusion. The next day after admission, a follow-up ECG showed terminal T-wave inversion with QT interval prolongation in leads V1 through V5 (Figure ). Coronary computed tomography angiography found no significant coronary stenosis. One week later, TTE demonstrated normal LV wall motion, and she was diagnosed with focal type TTS based on the specific findings of ECG and TTE.
Three months after the first hospital admission, she had sore throat and cough. Three days after the onset of the symptoms, she suddenly had orthopnoea. Her blood pressure was 154/104 mmHg with tachycardia of 122 beat per minute, and respiration was accelerated at 32 per minute. On physical examination, we recognized jugular vein dilatation, gallop rhythm, and peripheral coldness. Chest X-ray showed acute pulmonary oedema, and ECG showed ST-segment elevation in leads V2 through V3 and terminal T-wave inversion in leads V2 through V6. TTE revealed broad akinesis in mid- and apical-left ventricle with akinesis of right ventricle (Figure ; Video 1). LVEF was significantly reduced to 23%, and neither LV outflow tract obstruction nor mitral regurgitation were observed. Her serum troponin T was slightly elevated to 0.088 ng/mL, though CK and CK-MB were within normal range. Emergent coronary angiography (CAG) showed no occlusive coronary artery (Figure ). She was diagnosed with recurrent TTS, which complicated bi-ventricular dysfunction. Although we had started a vasodilator drug and respiratory support using non-invasive positive pressure ventilation, she haemodynamically collapsed, and required low dose of norepinephrine and dobutamine with support of intra-aortic balloon pumping. The following day after the admission, ECG showed marked symmetrical giant negative T-wave with QT interval prolongation in leads V2 through V6 (Figure ). We had temporarily prescribed enalapril after stabilization of circulatory collapse, but discontinued it due to an occurrence of hyperkalaemia. After 2 weeks, her bi-ventricular dysfunction was completely normalized (Figure ; Video 2). 99mTc-tetrofosmin and 123I-beta-methyl iodophenyl-pentadecanoic acid dual myocardial single-photon emission computed tomography (SPECT) demonstrated a preserved whole perfusion of left ventricle and defect of fat acid metabolism in mid-anterior and apical segments (Figure ). Computed tomography showed no findings of subarachnoid haemorrhage and pheochromocytoma. She was discharged on day 22.
One month after the second discharge, she suffered from herpes zoster on her back. Two days after the onset of zoster, she presented with dyspnoea, and chest X-ray showed bilateral pleural effusion. ECG showed terminal T wave inversion in leads V2 through V6 (Figure ). TTE revealed mild hypokinesis in mid- and apical-anteroseptal segments, leading to reduced LVEF of 48% (Figure ). Right ventricular function was preserved. CAG depicted normal coronary arteries, and myocardial biopsy showed no specific findings. She was treated with intravenous diuretics, and TTE showed complete recovery of the LV wall motion on day 10. She was diagnosed with focal type TTS. Metanephrine titration on 24 h urine was within normal range. 123I-meta-iodo benzyl guanidine (MIBG) SPECT and flouro deoxy glucose positron emission tomography detected no evidence of pheochromocytoma and paraganglioma. She was discharged with enalapril, spironolactone and carvedilol.
Four months after the third discharge, she suddenly complained of central chest pain. The triggered stress was not identified. On admission, ECG showed ST-segment elevation in leads I, III, and aVF (Figure ), and her serum troponin T was increased to 2.00 ng/mL. CAG showed no coronary artery stenosis, and left ventriculography revealed akinesis of mid-inferior and apical segments indicating apical ballooning (LVEF: 50%). The next day following the admission, ST-segment elevation was normalized. Normal LV wall motion was regained after 1 week. The diagnosis of quadruple TTS was established. No more recurrence was recognized after 6 months from the last recurrence.


A 45-year-old male, the fourth of seven living siblings, was apparently normal until the age of 18 years when he reported right side facial twitching followed by a generalized tonic-clonic seizure. During these episodes, he bit his tongue, rolled his eyes upward, and produced a frothy secretion from the angle of the mouth. Post-ictally, the patient was tired, sleepy, and confused for minutes to a couple of hours. He presented initially to a local secondary care hospital where magnetic resonance imaging (MRI) and electroencephalography (EEG) services were not available. He was apparently normal at birth following an uneventful gestation, and his neonatal history and subsequent development including school performance were also normal. His family history was unremarkable with no history of spinocerebellar ataxia, epilepsy, or genetic disorders. He does not drink alcohol, use illicit drugs, or take any medications (both over the counter and prescription). Initial computed tomography (CT) scan of the brain performed in the emergency department was normal. He was started on a phenytoin loading dose followed by an oral intake of 100 mg three times daily. Unfortunately, one month following the onset of phenytoin therapy, the patient developed dysarthria, severe ataxia, imbalance, action tremor, and difficulty walking. On neurological examination, the patient was unable to stand or walk with severe ataxia involving both the trunk and all extremities. He also had nystagmus, dysarthria, and symmetrical postural and action tremor. The plasma phenytoin level was 360 μmol/L (90 μg/mL) (recommend therapeutic range, 40–80 μmol/L [10 to 20 μg/mL]). The dose of phenytoin was rapidly reduced to 100 mg daily and subsequently stopped, and treatment with valproic acid 750 mg twice daily was commenced. A serial assessment of phenytoin concentrations was done, and phenytoin concentration bounced back to nadir after 9 days. Later on, levetiracetam was gradually introduced up to 1,000 mg twice daily. The patient has developed no more seizures since that time. He was re-evaluated by a tertiary care center where several CT scans and MRI of the brain showed diffuse pan-cerebellar symmetrical atrophy with no structural congenital, traumatic, or ischemic changes. Routine EEG was repeated twice, which showed no evidence of epileptiform discharges. The patient refused admission for EEG telemetry monitoring. There were no metabolic abnormalities including liver dysfunction that could explain the highly toxic plasma level. Measurement of different vitamin levels including vitamin E, folate, and vitamin B12 was normal. Genetic assessment of different cerebellar genetic disorders including spinocerebellar ataxia using whole-exome sequencing was unremarkable. On discharge from the hospital 2 weeks later, he still had severe pan-cerebellar symptoms and signs with ataxic gait and inability to walk without support. He was bedbound for a period of 4 months and started on a physiotherapy program. Since then, there has been slow but continuous gradual recovery with an improvement of all different cerebellar modalities including gait. Ten years after the intoxication, his condition improved remarkably, and the only abnormality was mild dysarthria and unsteadiness when running or turning rapidly. Despite the clinical improvement, a follow-up CT scan of the brain showed marked cerebellar atrophy and enlargement of the fourth ventricle and basal cisterns, which were similar to those images performed few months following initial presentation ().


A 28-year-old male patient reported with the chief complaint of dentinal hypersensitivity on the lingual side of the mandibular right lateral incisor to cold liquids. Medical history was noncontributory, and there was no history of any type of habits.
The periodontal evaluation revealed probing depths of 2 mm midlabially, distally, and mesially with mild plaque accumulation and minimal bleeding on probing. The patient presented with a narrow and deep GR of 5 mm midlingually with minimal attached gingiva and keratinized tissue width of 1 mm with respect to #42 []. No recession was seen on the labial aspect of the same tooth, and there was no tooth mobility. A midline diastema of 1 mm between central incisors was seen with mild labial inclination. Although it is not possible to classify lingual recession according to the popular Miller classification, the recession was similar to Miller's Class II on the facial with no interproximal bone loss and recession extending up to the mucogingival junction. On radiographic examination, periapical radiographs revealed mild periodontal ligament space widening with mild to no horizontal bone loss interdentally []. After discussing the clinical findings, treatment options, and risks associated, the patient's oral and written consent was obtained.
The treatment plan was divided into three phases: presurgical phase, which consisted of oral hygiene instructions, full-mouth debridement, and occlusal adjustment, followed by surgical phase which involved periodontal plastic surgery, and finally, maintenance phase, involving a strict supportive periodontal therapy.
A full-mouth debridement along with slight occlusal adjustment was performed on the labio-incisal surface. Necessary oral hygiene instructions were given, and after 3 weeks on re-evaluation, the surgical therapy was suggested.
The patient was asked to rinse with a 0.2% chlorhexidine digluconate solution before surgery. Local anesthesia of 2% lidocaine with 1/100,000 epinephrine was administered following which the exposed root surface was debrided with curettes. Intrasulcular incisions were made on the lingual surface of #42 extending to adjacent teeth on either side without splitting the papilla. A full-thickness mucoperiosteal pouch and tunnel was created using Orban's knife and ophthalmic crescent blade (CB01–2.5 mm, 20G) which extended beyond the mucogingival junction, and interdental papillae were elevated completely from the bone and also creating a tunnel laterally on the adjacent teeth. All the muscle fibers were separated and released to achieve a tension-free flap which could be easily mobilized coronally. The interdental papilla was gently undermined to get complete mobilization of flap, and special care of interdental papillary tissues was taken to avoid any perforation [].
After recipient preparation, a subepithelial CTG (SCTG) of a thickness of 1.5–2 mm was harvested through Hurzler's single incision technique from the palate.[] The lance tip blades (SP15, 15°) and CB (CB01–2.5 mm, 20G) were also used in order to give precise incision and to avoid any secondary incision []. Any epithelium that was harvested with the graft was stripped away with a scalpel. The donor site was closed by interrupted sutures. The CTG was positioned in the tunnel covering the denuded recession area #42 []. The cross-mattress along with sling sutures using 4.0 sutures was given to stabilize the graft with respect to #42 [].
The patient was prescribed 500 mg of amoxicillin thrice a day and 500 mg of paracetamol analgesic for 5 days following the surgery. The patient was recalled for evaluation after surgery at every 3 days interval for 14 days. Sutures were removed after 14 days, and the patient was advised to continue with chemical plaque control along with regular professional oral hygiene care for the next 15 days. The patient was kept on recall visits for every 2-week interval for the next 3 months and after that at every 1-month interval for the 6 months.


A 73-year-old man was evaluated at the neurosurgical outpatient clinic with an 18-month history of intermittent back pain and progressive gait disturbance due to diminished strength in both legs. Seven years before, the patient had similar neurogenic claudication as main symptom and a lumbar CT scan was done solely, which showed stenosis at the L4-L5 level as the key feature. He underwent lumbar decompressive laminectomy in another institution, with transient improvement at that time.
During the following months, the symptoms slowly progressed and the patient became unable to walk without assistance. He also developed urinary sphincter disturbance with incontinence episodes. The neurological examination showed a bilateral lower extremity motor weakness with a motor strength grade 3/5 bilateral in hip flexion and extension, and a Grade 4/5 bilateral in knee extension, ankle dorsiflexion, knee flexion, great toe extension, and ankle plantar flexion. The patient also had hypoesthesia from L4 dermatome downward bilaterally, abnormally increased patellar and ankle reflexes bilaterally, and extensor plantar reflexes.
A lumbar MRI was performed, demonstrating prominent vascular voids near conus medullaris but no significant high T2 signal involving the spinal cord [ and ]. Further evaluation was undertaken with CT angiogram and digital subtraction angiography (DSA). These examinations identified a FTAVF which was fed by the filum terminale artery that arose from the ASA and were supplied by the left D10 segmental artery [ and ]. The patient also had an electromyographic study done that reveals chronic neurogenic changes of L3, L4, L5, and S1 bilaterally.
Therapeutic options were discussed with the patient, who decided to undergo surgery. Reopening and extension of the previous laminectomy down to S1 was performed, to expose the fistula. After durotomy, the direct connection between the artery of the filum terminale and the arterialized vein was evident, forming the arteriovenous fistula (AVF) at the level of L5-S1 []. With the aid of intraoperative neurophysiological monitoring, we confirm the filum terminale due to the absence of sensory-motor potentials on it [ and ]. An intraoperative angiography with indocyanine green (ICG) was performed to identify the exact shunt location []. After confirmation of the AVF, a clip was placed interrupting the fistulous path, followed by the observation that the draining vein was no longer arterialized []. This interruption was confirmed with the ICG video angiography [].
Surgery and immediate postoperative period were uneventful. Over the following weeks, both sensory and motor deficits, progressively improved. Ten days after surgery, the patient recovered the voluntary control of the urinary sphincter and the urinary catheter was removed. He underwent physiotherapy and was able to walk unassisted 6 months after surgery.


A 73-year-old woman with a past medical history of end-stage renal disease on hemodialysis,
chronic anemia, diabetes mellitus, and hypertension presented to the emergency department
(ED) with a chief complaint of dark-tarry stool and abdominal cramping for 1 day. One day
prior to the ED visit, the patient reported multiple episodes of abdominal cramping and a
change in the color of her stools from light brown to dark and tarry. She also noted
new-onset mild shortness of breath on exertion and significant fatigue for the same
duration. The patient reported high compliance with all her medications and hemodialysis (3
days/week). She did not have associated abdominal pain, change in stool consistency, change
on bowel or bladder habits, painful defecation, recent diarrhea, or sick contacts. She
denied using over-the-counter pain medications. She denied recent fevers, chills, night
sweats, weight loss, cough, chest pain, or leg swellings. However, the patient did report
multiple hospital admissions in the past with the most recent hospitalization about 2 months
prior for similar complaints of dark tarry stools and severe anemia requiring blood
transfusion. She was a nonsmoker and did not use alcohol, marijuana, or other
substances.
On examination in the ED, the patient was found to have a temperature of 98 °F, respiratory
rate of 18 breaths/min with an oxygen saturation of 98%, heart rate of 100 beats/min, and
blood pressure 90/60 mm Hg. Overall, she looked pale with cold extremities and weak
peripheral pulsations in all limbs. An arteriovenous fistula with thrill was present on the
left upper extremity. No peripheral edema was noted. On examination of the chest, normal
breath sounds were heard with good air entry bilaterally, and a crescendo-decrescendo
systolic murmur with maximum intensity at the right sternal border and radiation to the
carotids was also noted. Abdominal examination revealed a soft, nontender, and nondistended
abdomen with normal bowel sounds. Laboratory investigations revealed a critically low
hemoglobin level of 5.2 g/dL (12.1-15.1 g/dL), hematocrit 15.9% (35.5% to 44.9%), white
blood cell count 10.1 × 109/L (4.5 to 11.0 × 109/L), platelet count
266 × 109/L (150 to 400 × 109/L), and a high reticulocyte count of 4.4% (0.5% to
1.5%). The peripheral smear revealed polychromasia and schistocytes. The blood urea nitrogen
was found to be 53 mg/dL, creatinine 6.3 mg/dL (baseline creatinine 3 mg/dL), and B-type
natriuretic peptide (BNP) was noted to be 1880 pg/mL. Serum electrolytes, prothrombin time,
activated partial prothrombin time, and international normalized ratio (INR) were within
normal limits. The serum troponin was found to be elevated at 0.089 ng/mL; however, the
electrocardiogram (EKG) was unremarkable. Chest X-ray demonstrated cardiomegaly and mild
congestion bilaterally in the chest.
Due to the patient’s critically low hemoglobin level, she received 2 units of packed red
blood cells in the ED that led to an elevation of her hemoglobin level to 8.9 g/dL. A
decision was made to admit the patient to an inpatient setting for further investigation
into the cause of bleeding. Additionally, serial troponin level measurements were performed
every 6 hours, which eventually down trended. Serial EKGs performed every 6 hours were also
found to be unremarkable. An inpatient echocardiogram was obtained, which revealed a left
ventricular ejection fraction of 50% to 55%, grade 1 diastolic dysfunction, and severe AS
with an aortic valve area of 0.8 cm2. Furthermore, gastroenterology was consulted
for source control of the GI bleed. An esophagogastroduodenoscopy and colonoscopy were
performed, both of which were negative for active bleeding; however, there was high
suspicion for angiodysplasia that may have led to the patient’s presentation. The
gastroenterologist consulted on the case had a detailed discussion with the patient about
capsule endoscopy as the next intervention to locate the source of the bleed. The patient
reported that a capsule endoscopy had been performed a year ago at an outlying facility, and
the results were available with her primary care provider (PCP). We reached out to the PCP
who informed us that the patient had angiodysplasias throughout her GI tract. With a history
of significant and recurrent GI bleeding secondary to the angiodysplasias and the presence
of AS, a platelet function assay (PFA) was ordered. It was found to be abnormal pointing
toward the presence of AVWS. Hence, a diagnosis of HS was established. During the hospital
stay, the patient showed significant improvement with complete resolution of her presenting
symptoms; therefore, the decision was made to discharge her home. The patient was scheduled
to follow-up with her cardiologist for evaluation for transcatheter aortic valve replacement
(TAVR) procedure for the AS. Additionally, she was also advised to follow-up with her PCP
within 2 weeks of discharge from the hospital. Eventually, the patient underwent TAVR
without complications, after which she did not report episodes of GI bleeding.


A 70-year-old hypertensive and diabetic male patient was admitted to our ICU with respiratory distress following COVID-19 infection. Chest x-ray showed bilateral diffuse infiltrates. ABG on 15 L of oxygen by non-rebreathing mask showed severe hypoxia (Pao2/Fio2 < 100), and respiratory acidosis with pH 7.52, PO2 56.3, PCO2 31.5, SpO2 <90. We gave non-invasive ventilation (CPAP) support with Fio2 80%, pressure support 14, and PEEP 7 (cm H2O). On the third day of CPAP therapy, the patient was unable to maintain oxygen saturation (SpO2 < 92%) despite FiO2 100% and became more tachypnic (RR > 40). There was increased respiratory distress and SpO2 decreased to <90% despite FiO2 100%. We gave supplemental oxygen by nasal cannula with O2 flow 10 L by another flowmeter (). The patient became more comfortable, and SpO2 increased to 95%. ABG showed PO2 90 mm Hg, PCO2 35, and SpO2 97%. The patient recovered successfully from respiratory distress.


A 57-year-old man presented with a long history of a persistent, firm raised nodule in the right lumbar area with increasing size. Clinical impression was a sebaceous cyst. The patient was otherwise well, with no constitutional symptoms and no significant past medical history.
The specimen was composed of a nodule measuring 1.7 × 0.9 × 0.9 cm embedded in subcutaneous tissue with an overlying ellipse of unremarkable skin. Multiple sections of the excisional biopsy demonstrated nearly circumscribed growth of the basaloid lesion in dermis and subcutaneous fat tissue with a surrounding pseudocapsule (Fig. ). There was zonation between basaloid cells at the periphery with numerous “shadow” cells in a fibrocollagenous matrix with an inflammatory background (Fig. ). Numerous foci of keratin were evident, with an associated multinucleated giant cell reaction. The tumor exhibited focal atypical features, including very focal infiltrative pattern at the periphery (Fig. ). Irregular foci of central necrosis (comedonecrosis) were present in several lobules (Fig. ). Also, the tumor was composed predominantly of basaloid cells with a variable degree of atypia (Fig. ) and an increased mitotic rate, up to five mitotic events/high-power field (Fig. ). No definite vascular or lymphatic invasion was identified. The tumor was close to the surgical margins but appeared to have been completely excised. These features are consistent with noncalcified pilomatrixoma with atypical features.


A 47-year-old man with a history of painful testicular masses visited a local clinic. The serum tumor markers AFP, hCG, and serum lactate dehydrogenase were elevated at 8680 ng/mL, 2.9 mIU/mL, and 338 U/L, respectively. Computed tomography revealed masses in left scrotum and metastatic lesions in the left lung (Fig. ). A high inguinal orchiectomy was performed. The histopathological diagnosis was germ cell tumors of several histological types consisting of yolk sac tumor, embryonal carcinoma, and teratoma mixed type. The clinical diagnosis was NSGCTs with lung metastases pT2N0M1aS2 Stage IIIB, and the patient was given an intermediate prognosis according to International Germ Cell Consensus Classification. After surgery, the patient declined the adjuvant chemotherapy. Ten months later, he returned with complaints of dyspnea and significant left pleural effusion. His serum AFP level had increased to 52852.6 ng/mL. Four cycles of a bleomycin, etoposide, and cisplatin regimen were completed. After first-line chemotherapy, serum AFP and hCG levels increased again. Therefore, we selected nedaplatin 100 mg/m2 and irinotecan hydrochloride 200 mg/m2 regimen as second-line chemotherapy because of his renal dysfunction and anticancer activity. The serum tumor markers returned to normal after second-line chemotherapy. However, the huge pleural and pulmonary tumors remained (Fig. ). Preoperative ventilation-perfusion scintigraphy indicated that the pulmonary metastasis of the NSGCTs caused left middle and lower lobe atelectasis. The preoperative magnetic resonance imaging showed the metastatic NSGCTs in fat tissue near the pericardium. To achieve complete removal of the tumor, EP was conducted as a salvage surgery after predicting a postoperative DLCO2 of 42% by the single-breath method. The resected tumor was 23 × 17 × 6 cm in size and 1476 g in weight (Fig. ). The pulmonary metastatic tumor had been compressing the normal lung tissue (Fig. ). Histopathologically, the resected tissue was immature teratoma (Fig. ). The resected margin was negative. Postoperative imaging 6 months after surgery showed that the pleural effusion in the left thorax (Fig. ). The patient has shown no evidence of recurrence for 5 years since the EP and has led a daily life without the need for an oxygen inhaler.


A 58-year-old woman was newly diagnosed with advance (T3N1M0) moderately differentiated squamous cell carcinoma of distal esophagus involving gastroesophageal junction. She received her first cycle of neoadjuvant chemotherapy Carboplatin and Paclitaxel. The next day, she presented to the emergency room with two episodes of hematemesis containing fresh blood. In addition to that she had progressive dysphagia for solids. She remained hemodynamically stable. The Rockall et al. score was 2, owing to the disseminated esophageal malignancy. Her blood reports showed a hemoglobin drop of 2.6 g from 13.1 to 10.5 mg/dL. The platelet count and coagulation profile were within the normal range along with the liver and renal functions also being unremarkable. After initial resuscitation, patient was made nil by mouth and started on proton pump inhibitor (PPI) infusion. After taking informed written consent, an upper GI endoscopy was done. The findings revealed a stenosing non-traversable tumor, starting at 33 cm from the incisors, containing old clotted blood as well as mild oozing, as shown in . Initially, an attempt was made to maintain hemostasis using conventional methods including adrenaline injection and hemospray, but this was unsuccessful for this purpose. In view of the stenosing tumor and ongoing bleed despite using the aforementioned measures, it was decided there and then to place a temporary 20 mm × 130 mm FCSEMS (nitinol). This would not only help to achieve luminal patency but would also aid in bleeding control due to the tamponade effect. Using a catheter, a guidewire was passed through the stricture and its position confirmed fluoroscopically, as shown in . An FCSEMS was deployed over the guidewire under both direct vision and fluoroscopic guidance, which is shown in and , respectively. No further episodes of hematemesis or melena were observed along with no further hemoglobin drop. Patient stayed for 72 h in the hospital and was later discharged with a follow-up plan for stent removal at 2 weeks. Patient, however, was lost to follow-up with the GI team but continued to have chemotherapy and radiation therapy. She was sent back for esophageal stent removal after 5 months. Esophago-gastro-duodenoscopy (OGD) revealed no bleed from tumor, stent was removed, and scope was passed freely across the tumor, as shown in . Patient followed up notes of radiation oncology clinics confirmed no episodes of upper GI bleed between the time of stent insertion and removal. No stent-related early and late complications were seen.


A 79-year-old woman with HF with preserved ejection fraction (HFpEF) presented to the emergency department reporting a sudden increase of breathlessness and chest discomfort over the last few hours. Arterial blood pressure was 160/95 mmHg, the pulse was regular with a heart rate of 115 BPM. The peripheral oxygen saturation (SpO2) was 88% by breathing ambient air (94% with 4 l/min of oxygen) and the respiratory rate 32/min. The physical examination was remarkable for pitting oedema on both legs, distended jugular veins and bilateral pulmonary crackles. Extremities were warm and appear well perfused. The ECG showed sinus rhythm; no repolarisation abnormalities were present. Levels for high-sensitivity troponin were mildly elevated without relevant changes after 1 hour. The patient was treated with loop diuretics for AHF without relevant improvement of symptoms. The junior physician on night shift duty suspected the presence of concomitant PE and called his senior to discuss how to further manage this patient.


A 49-year-old man presented at our emergency room (ER), complaining of pain localized to the posterior left thigh, started about 2 hours after an accidental side split (hip flexed and knee fully extended) by falling down the stairs. His past medical history was significant for an episode of ophthalmic artery thrombosis for which the patient was taking aspirin daily. At physical examination, while the left anterior thigh was nontender at palpation, the posterior thigh presented as swollen, tense and painful to light touch from the gluteal fold down to 2 cm proximal to the posterior aspect of the knee. Muscle strength of the inferior limb was recorded: knee extension was 5/5 while knee flexion could not be assessed due to pain; ankle plantar flexion, ankle eversion and great toe flexion were all registered to be 5/5. Ankle dorsiflexion and great toe extension were 5/5. No sensory deficit were recorded. Dorsalis pedis and posterior tibial pulses were present as well. About thirty minutes after the ER admission ankle dorsiflexion and great toe extension were reported to be 0/5. At the following physical examination numbness was reported on the dorsal and plantar aspects of the left foot. A CT angiography was promptly carried out () and reported positive bleeding of the femoralis profundis artery giving indications to readily perform an angiography that resulted in no vessel to embolize. On the basis of the abovementioned clinical workup and the persistent clinical presentation, we made the diagnosis of compartment syndrome of the posterior tight; A CT angiography was promptly carried out () and reported positive for a jet of the femoralis profundis artery giving indications to readily perform an angiography that resulted in no vessel to embolize. On the basis of the abovementioned clinical workup and the persistent clinical presentation, we made the diagnosis of compartment syndrome of the posterior tight; approximately 8 hours after the initial injury, our patient was taken to the operating room to operate an emergency fasciotomy. The patient was placed in prone position to have better access to his left thigh; a posterior incision was performed starting at the gluteal fold and prolonged distally down to approximately 8 cm proximal to the popliteal fossa. A complete fasciotomy and release of the posterior compartment was performed by cutting longitudinally the overlying fascia (). Approximately 500 mL of hematoma were forcefully ejected out of the surgical excision at the release of the posterior intracompartmental pressure. The exposed muscles appeared dark red but presumably viable while the long head of biceps femoris, semitendinous and semimembranous were completely avulsed from the ischial tuberosity. The sciatic nerve was identified along with the posterior femoral cutaneous nerve of the thigh () and both were isolated and preserved. Any active vessel hemorrhage was rapidly blocked. Then we proceeded to suture back in place the above-mentioned tendons on the ischial tuberosity using 2 bone anchors, specifically, Corkscrew® FT suture Anchor 5,5mm x 16,3mm with two #2 Fiber Wire Arthrex® (Naples, Florida, USA)(). The surgical excision was copiously irrigated with saline solution and sutured while leaving the fascial layer open. Postoperative care was aimed at performing fresh blood and plasma transfusions; at clinical inspection the patient reported an immediate pain relief. On the 3rd postoperative day (POD), sensation on the plantar aspect of the foot had returned while the sensation on the dorsum of the foot had improved markedly, with the patient reporting just paresthesia over the toes. Motor strength was assessed as 5/5 in ankle plantar flexion, ankle eversion and great toe flexion while 4/5 for ankle dorsiflexion and great toe extension. Postoperative anticoagulation therapy was administered daily, and for the following 30 days, by subcutaneous injections of 4.000 UI enoxaparin. The patient was discharged on the 5th POD with the following indications: wheel-chair use, avoid weight-bearing for 4 weeks and vitamin B12 complex for a month. At six-week follow-up the patient could successfully stand up with the aid of crutches. Full weight-bearing was achieved 8 weeks postoperatively. At five-month follow-up our patient showed full ROM of the hip and the knee while sensation was reportedly complete: the Perth Hamstring Assessment Tool score [6] was 65 when assessed at this time.


An 11-month-old female infant presented to pediatric surgery outpatient department (OPD) with complaints of recurrent seropurulent discharge from umbilicus for 3 months. There were no other confounding complaints. She had two episodes of similar complaints in the last 3 months and was treated on a conservative basis with antibiotics, dressing, and proper nutrition. The baby had a history of VP shunting done 6 months ago for congenital hydrocephalus. On clinical examination, her vitals were stable with a heart rate of 110 beats/min and normal capillary refill time. Systemic examination revealed. The abdomen was soft and non-tender with no lump palpable. Umbilicus was inverted with no swelling or abnormal skin changes. There was occasional spontaneous seropurulent discharge from the umbilicus which was thick in consistency, scanty, non-foul-smelling, and was not expressible. Neurosurgical reference was taken which revealed a functioning and patent VP shunt.
Hematological investigations were completely normal. The seropurulent discharge was sent for Gram stain, culture, and sensitivity, which revealed no growth of microorganisms. The baby was started on antibiotics, adequate nutrition, and regular dressings. The discharge was reduced after the management but did not stop completely.
A contrast-enhanced computed tomography (CECT) of the abdomen and pelvis was done which revealed free fluid in the pelvis with small peripherally enhancing collection on the right side of the urinary bladder adjacent to the distal end of the VP shunt [] and minimal hypodense collection in the periumbilical area near the loop of VP shunt [].
She was planned for exploratory laparotomy. Intraoperatively the tip of the VP shunt was found near the fundus of the urinary bladder with a fistulous tract [] covering the distal end of the VP shunt which was attached to the fundus of the urinary bladder and proximally to the umbilicus. The fistulous tract was excised and the VP shunt visualized [] with purulent discharge noted at the distal end. Neurosurgery intervention was called for intraoperatively and due to the purulent nature of discharge the VP shunt was removed and the fistulous tract was excised.
Postoperatively the baby recovered with the cessation of umbilical discharge. A CT brain was done which revealed hydrocephalus. At present, she is being planned for endoscopic third ventriculostomy.


A 70-year-old male presented with 18 months of indolent right shoulder pain attributed to a slow growing mass. Medical history was notable for prostate cancer treated with prostatectomy and 50-pack-years of smoking. On exam, there was a prominent mass anteriorly over the right shoulder that was immobile, indurated, and nontender. There were no overlying skin changes. Shoulder range of motion was limited to 110o of forward flexion, 90o of abduction, 60o of external rotation, and internal rotation to L5 compared to the contralateral side (180o, 170o, 60o, and T12, respectively). Motor and sensory function was intact. Radiographs demonstrated an ossified mass arising from the surface of the proximal humeral meta-diaphysis with cortical scalloping (, ). MRI revealed a lobulated soft tissue mass with T1 and T2 heterogeneous signal enhancement around the proximal humerus, cortical scalloping, and areas of linear dense calcification (-).
Workup proceeded with a percutaneous core needle biopsy of the right proximal humerus. Fourteen samples were obtained and all exhibited a cartilaginous appearance. Histopathology diagnosed intermediate-grade chondrosarcoma of periosteal origin (). Tumor staging was carried out with a chest CT and whole-body bone scan, which revealed no evidence of metastasis (, ). The patient consented for wide resection of the right proximal humerus and reverse total shoulder endoprosthetic reconstruction.
A standard extended deltopectoral approach was used (). The previous core needle biopsy site was ellipsed. Vessels feeding the tumor were ligated. The distal musculotendinous junction of the biceps muscle was resected leaving tendon with the tumor. All segments of the deltoid muscle were spared. The rotator cuff muscles were resected leaving a 1 cm margin of healthy tendon on the resected humeral head. Neurolysis of the axillary and musculocutaneous nerves was performed. The pectoralis major, latissimus dorsi, and teres major muscles were resected from the medial aspect of the humerus leaving a 1 cm margin of healthy tendon on the resected bone. Dissection was carried out distally and the humerus was resected 12 cm from the glenohumeral joint line. Neurolysis of the radial nerve was performed. The resected proximal humerus measured 12x2.5x2.5 cm and peri-humeral mass 8x7.5x6.9 cm. (). Tissues were sent for frozen sectioning and confirmed tumor-free margins.
Reverse total shoulder endoprosthetic reconstruction was performed(Comprehensive Reverse Shoulder System, Zimmer-Biomet). The glenoid was predrilled and the base plate was affixed with 1 center screw and 4 peripheral screws slightly inferior on the glenoid to avoid scapular notching and shoulder impingement. The glenosphere was sized and engaged with the baseplate by Morse taper. The humeral shaft was reamed and press-fit components were secured (). The rotator cuff tendons were sutured to the prosthetic neck and the deltoid tendon was myodesed. The pectoralis major, latissimus dorsi, and teres major tendons were sutured through the prosthetic portals (, ). A standard multilayered closure was performed. Radiographs demonstrated satisfactory prosthetic positioning (-).
Postoperatively, the patient remained in a shoulder immobilizer for 6 weeks before starting physical therapy. Tumor surveillance was planned with shoulder and chest X-rays every 3 months until 2 years postoperatively, biannually for the next 3 years until 5 years postoperatively, and annually thereafter. At 1 year postoperatively, the patient was doing well with acceptable shoulder range of motion: 70o of forward flexion, 80o of abduction, 30o of external rotation, and internal rotation to S1. At 2 years postoperatively, he sustained a fall with the arm hyperflexed resulting in glenosphere dissociation requiring revision arthroplasty (, ). Intraoperatively, the glenosphere was completely disengaged (), there was minimal fretting wear and no corrosion of the Morse taper (), the polyethylene was inferiorly worn (), there was no scapular notching, and the humeral component and baseplate were intact (). Decision was made to increase the prosthetic constraint to account for soft-tissue attenuation by upsizing both the glenosphere and humeral tray (, -).
The patient is currently 3 years postoperatively from the index surgery and 1 year postoperatively from revision arthroplasty. There has been no clinical or radiographic evidence of tumor burden or hardware complication. Shoulder range of motion is limited to 70o of forward flexion, 60o of abduction, 30o of external rotation, and internal rotation to S1.


A fit and well 23-year old man presented to a dental hospital after sustaining dental trauma falling onto the back support of a dining room chair. The patient presented to an accident and emergency (A+E) department where he was assessed by oral and maxillofacial (OMFS) clinicians. An orthopantomogram (OPG) was requested to assess facial fractures was ordered by our OMFS colleagues at the time of presentation to the accident and emergency department. He was discharged without treatment and advised to seek further management by his general dental practitioner. He presented to a dental hospital 36 hours following injury. His medical history was unremarkable.


A 54-year-old man presented to the neurologic clinic of The Fourth Affiliated Hospital Zhejiang University School of Medicine, after episodes of seeing round, colored flickering lights, and reporting that he “can't see things in his right visual field” 7 d before admission.
One week prior to admission, the patient saw paroxysmal green and red flickering lights in his right visual field. He then experienced blurred vision in his right visual field. He visited a local ophthalmic clinic several days before coming to our hospital, and a visual field examination showed a right homonymous hemianopia (Figure ). His visual field defect improved partially without treatment. The paroxysmal flickering lights lasted for about 3 d before remission, but there was no further improvement in his hemianopia.
The patient was otherwise healthy. He denied a history of hypertension, diabetes mellitus (DM), prior cerebrovascular disease or other neurological complications.
The patient was a journalist with a height of 178 cm and weight of 76 kg. He had no history of drug use, drinking, smoking, or bad sexual life. One of his four siblings had DM, and there was no family history of stroke, epilepsy, or other neurologic diseases.
His general examination was unremarkable. Neurologic examination revealed right homonymous hemianopia with normal pupillary reactivity. No other positive neurologic signs were found.
His blood glucose was 645 mg/dL (35.83 mmol/L) without ketoacidosis, although his urine ketone test was weakly positive. Serum osmolarity was 297.09 mOsmol/L (range: 280-310 mOsmol/L), with serum sodium 126.42 mmol/L (range: 137-142 mmol/L), and serum potassium 4.21 mmol/L (range: 3.50–5.30 mmol/L). HbA1c was 14.4% (range: 3.6%-6.0%). Other laboratory tests were unremarkable.
Cranial magnetic resonance imaging (MRI) showed abnormalities in the left occipital lobe with decreased T2 and fluid-attenuated inversion recovery (FLAIR) signals in the white matter, restricted diffusion, and corresponding low signal intensity in the apparent diffusion coefficient map (Figure ). Magnetic resonance angiography imaging of the brain showed a mild stenosis of the right posterior cerebral artery, with arterial spin labeling showing decreased perfusion of the lesion (Figure ). The patient’s electroencephalogram (EEG) was mildly abnormal, showing slowing activities without spikes and sharp waves.


A 52-year-old Chinese woman was hospitalized with dry mouth and eyes, which reduced her quality of life. She attended the ophthalmology clinic because of her symptoms and was diagnosed with keratoconjunctivitis sicca. She had not experienced or had a history of vaginal dryness. The patient was not experiencing joint or muscle pains. Physical examinations revealed dryness and cracking in her lips, and multiple dental cavities. outlines her laboratory data upon admission. The patient had several notable analytical parameters, including antinuclear antibodies (ANA)+, anti-histone antibodies (AHA)+, Sjogren’s Syndrome A antibody (SSA+), Sjogren’s Syndrome B antibody (SSB+), and Glutamic Acid Decarboxylase Antibody (GAD65-Ab+). Her labial gland biopsy was performed, and diagnosed with SS and HT ().
The presence of GAD65-Ab represents a sign of autoimmunity directed towards the pancreatic islet β-cells (chemiluminescence assay: Linear range: 5IU/mL-250IU/mL; Minimum detection limit: no little than 0.2 IU/mL; maximum detection limit: no more than 2000IU/mL; Accuracy: the relative deviation was within ± 10.0%; Repeatability: the coefficient of variation (CV) of repeatability test was not more than 10.0%; Inter batch difference: the coefficient of variation (CV) of three batches of reagents was not more than 15.0%). Cognizant of this, hemoglobin A1c (HbA1c), oral glucose tolerance test (OGTT), C-peptide, and insulin release tests were performed upon admission (). The HbA1c and the initial OGTT (OGTT0) values were “technically normal” based on the ADA criteria. Follow-up laboratory examinations after discharge were recommended because the fasting C-peptide (0.2 nmol/L) and insulin (17.66 pmol/L) levels were borderline low during the OGTT0 (normal: fasting C-peptide, 0.27–1.28 nmol/L; fasting insulin, 35–145 pmol/L). The fasting plasma glucose levels were within the normal range during the follow-up examinations in the OGTT1.5y and OGTT3y despite the 180 min glucose levels (2.82 mmol/L) in the OGTT1.5y being below the normal range (3.36 mmol/L). The patient had no hypoglycemia-related symptoms. The patient’s response to glucose stimulation of C-peptide levels during the three years was stable in the normal range (peak time: 30min-1h, response: 5-6-fold to fasting level) despite the fasting C-peptide and insulin levels being relatively low. These findings strongly suggested a relatively preserved pancreatic function. The HOMA-IR value in the patient was within the normal range, suggesting that insulin resistance and insulin sensitivity at the OGTT1.5y and OGTT3y were similar to that of the OGTT0. However, the HOMA-β values were higher at the OGTT1.5y than the OGTT0, but identical to those of the OGTT3y. Nevertheless, the relatively low HOMA-β values were not attributed to insulin secretion dysfunction because the patient’s HOMA-IS index showed intact insulin sensitivity. The small amounts of insulin were able to maintain normal blood glucose levels ().


A 51-year-old Chinese Han woman visited Shanghai Pudong Hospital because of unknown anemia, aleukemia, and infections. She had not experienced or had a history of vaginal dryness. She also had no joint or muscle pain. Physical examinations revealed no distinct abnormal signs and were thus subjected to relevant laboratory examinations upon admission. Her laboratory data is outlined in . Positivity for SSA and SSB indicates the existence of SS. This patient only had ANA+ and HKL+ autoimmune antibodies. Her RF, ESR, and AHA were normal. Only the islet autoantibody (IAA) was positive. The patient was thus diagnosed with SS. She received the same treatment for the SS as the 1st case. Measurements of her thyroid function revealed the presence of HT with normal TRAb levels and high thyroperoxidase antibody levels ().
The sole presence of insulin auto-antibody (IAA-Ab) suggested potential immune injury to the pancreatic islet β-cells. Cognizant of this, blood examinations on hemoglobin A1c (HbA1c), oral glucose tolerance test (OGTT), C-peptide, and insulin release tests were performed (). The initial OGTT (OGTT0) showed no impaired glucose tolerance because the glucose levels were below the threshold. The patient’s response to glucose was generally normal despite the fasting C-peptide level being relatively lower than the normal level. The OGTT was repetitively checked at 1.5 years (OGTT1.5y) and 3 years (OGTT3y). The patient’s BMI at 1.5 years was high with high postprandial glucose at 120 min. Fasting and response to glucose challenge of C-peptide levels were generally stable at the OGTT1.5y and OGTT3y than OGTT0. However, the higher peak of C-peptide response at OGTT1.5y appeared after 120 min. The patient had a slight increase in HOMA IR and decreased HOMA-β at OGTT1.5y (– and and ). The OGTT3y returned to the normal range after controlling the patient’s body weight for 3 years ().


A 48-year-old female patient presented to our outpatient clinic two years ago with complaints of severe muscle weakness. She stated that muscle weakness had been troubling her for more than 10 years and she had presented to a hospital with muscle weakness, fatigue, and respiratory distress at the time. The epicrisis drafted for the patient back then showed the proximal muscle strength in both upper and lower extremities to be between ⅖ and ⅗ as identified in physical examination. No family history was indicated in the given report. According to the laboratory analyses, the patient had a creatine kinase (CK) level of 4,267 (29-200 U/L) and tested negative for antinuclear antibodies and extractable nuclear antigens. Electromyography findings were suggestive of inflammatory myopathy in the proximal muscles of both lower and upper extremities. Furthermore, muscle biopsy performed back then led to the observation of necrosis in the deltoid muscle fibers, which was considered to be suggestive of PM. Neither thoracic computed tomography nor electrocardiographic examination indicated pathologies. At the time, the patient was put on a 1,000 mg/day of methylprednisolone treatment for three days, and the treatment was continued with 1 mg/kg/day of methylprednisolone and 15 mg/week of methotrexate, and the dose of methylprednisolone was gradually reduced to 4 mg/day. The patient benefited from the treatment to a certain extent; however, she had to come back to the clinic within eight months because of recurrence of the previous complaints, upon which hydroxychloroquine was added to her treatment regimen. Nine years ago, the clinic shifted to 1 mg/kg/day of prednisolone and 3 mg/kg/day of azathioprine because of insufficient improvement in the patient’s condition. Seven years ago, subcutaneous administration of 15 mg/week of methotrexate was added to her treatment regimen, upon which the patient suffered further deterioration and muscle weakness and received a course of intravenous immunoglobulin, followed by 40 mg/day of leflunomide in the next years. She also received 1,000 mg of intravenous ritixumab twice with a two-week interval nearly a year ago. No positive effects were observed after these treatments, and the CK levels of the patient was identified to be above 1,000 U/L all the time, ranging between 1,000 and 4,000 U/L. A written informed consent was obtained from the patient.
The patient received a total hip replacement because of aseptic necrosis in the right femoral head that developed due to the use of steroids. Our physical examination indicated the muscle strength to be ⅕ in the proximal side of the bilateral upper extremities and ⅖ in the proximal side of the lower extremities. There were no marked findings of atrophy in the muscles. We found the body mass index to be 30 kg/m2. With a level of CK at 3,025 U/L, the patient was put on a monotherapy regimen of 125 mg/week of abatacept. After two months of treatment, muscle strength was found to be ⅗ in the upper extremities and ⅘ in the lower extremities, and the CK level dropped to 1,950 U/L (Figure 1). Muscle strength was found to be ⅗ in the upper extremities and ⅘ in the lower extremities in the fourth month of the treatment, with CK level coming down to 1,200 U/L. Her examinations at month eight showed a muscle strength of ⅘ in both upper and lower extremities and a CK level of 1,003 U/L. The patient was put on resistance exercises. The whole cycle of a 12-month treatment was completed in November 2018 with full muscle strength achieved and a CK level of 484 U/L. The patient, who is being regularly followed-up, is still receiving a monotherapy of 125 mg/week of abatacept, and her condition is stable.


An 87-year-old female with a medical history of hypertension and dyslipidemia presented to our emergency department with severe dysarthria, slurred speech, and right hemiplegia for 1.5 hours. A National Institutes of Health Stroke Scale (NIHSS) score of 18 was reported. Computed tomography angiography (CTA) revealed the middle cerebral artery (MCA) occlusion at the M1 segment. The patient was transferred to a neuroangiography suite for endovascular treatment.
Excessive tortuosity of the aorta was reviewed using CTA before the procedure was performed (). Reaching through the aorta through the transfemoral approach was considered difficult because of a low-lying, tortuous left CCA and the fact that the aorta was type III; therefore, PBWT was chosen. After successful placement of a GS into the distal left CCA (, ), a 6-Fr reperfusion catheter (ACE 60; Penumbra, Alameda, CA, USA) was then inserted into the GS. Suction thrombectomy was performed with successful thrombolysis in cerebral infarction (TICI) 3 recanalization in 1 pass (, ). The procedural time was 20 minutes from femoral puncture to guiding catheter placement inside CCA, 8 minutes for carotid puncture, and the total time taken to complete recanalization was 37 minutes from femoral puncture. After recanalization, the femoral puncture site was closed with a vascular closure device (VCD) (Angioseal VIP, 8 Fr; Terumo, St. Jude Medical, St. Paul, MN, USA), and the cervical puncture was manually compressed for 10 minutes. No immediate complications were noted. The patient was uneventful during an 11-month follow-up period, with a functional outcome of a modified Rankin Scale (mRS) score of 1.


An 80-year-old male with a medical history of hypertension and hyperlipidemia had previously had a left ethmoidal dural arteriovenous fistula, which was causing intracerebral hemorrhage. He had received craniectomy and stereotactic radiosurgery 10 years before the present case. During a follow-up magnetic resonance study, severe left M1 stenosis was found (). The premorbid functional outcome was an mRS score of 1. In the present case, he presented to the emergency department with severe dysarthria, aphasia, and right hemianopia for 70 minutes. The NIHSS score was 10 on arrival. CTA showed a left M1 acute occlusion (). Extremely tortuous aorta and CCA were also noted (). Endovascular revascularization was conducted immediately following discussion.
However, 40 minutes following femoral puncture, the GS could not be successfully inserted into a stable position in the CCA. The PBWT was applied after disinfection and preparation of the neck (). The GS was successfully inserted into the distal CCA. The reperfusion catheter could not pass the carotid siphon. A microcatheter (Prowler Select Plus; Codman, Raynham, MA, USA) was navigated through the occluded MCA segment, and a salvaged stent (Enterprise; Cordis, Miami Lakes, FL, USA) was implanted (). TICI 2b reperfusion was achieved despite residual stenosis. The procedure time from femoral puncture to recanalization was 96 minutes. The procedural time was 54 minutes from femoral puncture to guiding catheter placement inside CCA, 10 minutes for carotid puncture, and the total time taken to complete recanalization was 96 minutes. Tirofiban infusion was initiated after intracranial stent implantation. The cervical puncture site was managed with manual compression without incident. The femoral puncture site was closed with an 8-Fr VCD.
Two weeks after discharge, the patient’s symptoms were ameliorated and his NIHSS score was 6 with shuffling gait and aphasia. He received dual antiplatelet therapy. However, in-stent occlusion was noted 3 months later after an episode of acute stroke. No further endovascular treatment was performed. His mRS score was 3 at 3 months after recurrent stroke.


A 19-year-old man was admitted to our department with repeated haemoptysis. He had been followed up for neutrophil migration failure and bronchiectasis at the paediatrics department in our hospital since birth. Five months before the admission, the patient was referred to our department with haemoptysis for 2 weeks. Although airway bleeding associated with bronchiectasis was suspected, his symptom was improved with carbazochrome sodium sulfonate hydrate and tranexamic acid. Three months before the admission, he visited again because of recurrence of haemoptysis. Plain CT showed lobular central granular shadows in the right middle lobe, left lingular segment and bilateral lower lobes. He was admitted for evaluation of the source of bleeding. On admission, his height was 168.5 cm, and his weight was 53.5 kg. His vital signs were blood pressure, 103/49 mm Hg; pulse rate, 81 beats/min; pulse oximetry, 99% in room air; and body temperature, 36.7℃. Physical examination showed coarse crackles in both lung fields. Laboratory examinations showed no abnormality. Chest X-ray showed bronchial wall thickening in the right middle and left lower lung field and the surrounding ground-glass opacity and consolidation (). Plain chest CT also showed bronchial wall thickening and dilation in bilateral lung fields, partial mucoid impaction and multiple centrilobular granular shadows in the right middle lobe, lingular segment and bilateral lower lobe (). Contrast-enhanced CT showed hyperplasia and tortuosity of the bilateral bronchial artery; however, it was difficult to obtain a detailed evaluation of the origin and running by the axial views (). The three-dimensional (3D) image showed that the right bronchial artery branched from the left subclavian artery and was running on the dorsal side of the aortic arch ( and ). On the other hand, the left bronchial artery branched from the aortic arch and perfused to the left lingular segment. In 4D CT, the pulmonary artery was imaged again after the bronchial artery was imaged, indicating a bronchial artery–pulmonary artery shunt on both sides ( and ). Bronchial angiography confirmed the branching of the right bronchial artery from the left subclavian artery and shunting to the pulmonary artery ( and ). Additionally, the left bronchial artery branched from the aortic arch and perfused to the left lingular segment consistent with the CT findings.


A 43-year-old man was admitted to the emergency department due to intermittent fever, dry
cough and progressive dyspnea started a week ago. He had a 24-year history of hypertension
and chronic renal disease due to non-specific glomerulonephritis, receiving a kidney
transplant from a cadaveric donor 6 months ago and surgical parathyroidectomy due to
tertiary hyperparathyroidism. His medications were nifedipine 30 mg twice daily, metoprolol
100 mg twice daily, mycophenolic acid 500 mg twice daily, tacrolimus 1 mg twice daily,
prednisone 25 mg/day, calcium carbonate 4 g/day and calcitriol 0.25 mcg/day. Physical
examination revealed tachycardia, shortness of breath and a peripheral capillary oxygen
saturation level of 83% (room air). Relevant laboratory findings were leucopenia
(1980 mm3, normal range [NR] 4000–10 000 mm3), lymphopenia
(0.200 mm3, NR 0.600–3400 mm3) and lactate dehydrogenase 981 U/L (NR
240–480 U/L); Serum electrolytes were normal: sodium 136 mmol/L (NR 136–145 mmol/L),
potassium 4.1 mmol/L (NR 3.5–5.1 mmol/L), magnesium 1.8 mg/dl (NR 1.6–2.5 mg/dl), calcium
8.8 mg/dl (8.6–10.2 mg/dl), phosphorus 3.91 mg/dl (NR 2.7–4.5 mg/dl). Electrocardiogram
(ECG) showed sinus tachycardia. Chest radiograph showed bilateral ground glass image. The
SARS-CoV-2 polymerase chain reaction with nasopharyngeal swab was positive.
The patient was classified as COVID-19 severe disease according to World Health
Organization classification [] and was transferred
to the department of Internal Medicine where he was evaluated to select the management as
follows: the QTc of the baseline ECG was calculated manually using the Bazett formula, being
410 ms (NR 350–480 ms), and a validated risk score to predict QT interval prolongation in
hospitalized patients was performed, by which he was classified as ‘low risk’ (6 points)
[]. Therefore, a standard regimen with
chloroquine plus azithromycin was started (chloroquine 500 mg twice on day 1, then 500 mg
daily on Days 2–5 plus azithromycin 500 mg daily on Day 1, then 250 mg daily on Days 2–5).
An informed consent for the treatment was obtained according to the institutional standards.
However, on Day 4 of therapy, the patient suddenly developed a fast-WCT at 180 beats/min,
managed as ventricular tachycardia (VT) with amiodarone 300 mg IV. Unfortunately, it was not
possible to record the complete 12 lead ECG, only a DII rhythm strip. A close inspection
showed possible atrio-ventricular dissociation (,
red dots) that suggests the diagnosis of VT. The rhythm following cardioversion 5 min after
starting amiodarone infusion was visualized with a DII rhythm strip, which showed an
irregular rhythm, QRS 110 ms at 100 beats/min on average with biphasic T waves with
ST-segment depression, without organized atrial activity, suggesting atrial fibrillation.
There was no evidence of prolonged QT (). A
measurement of troponin I was obtained during the arrhythmia episode, which was 0.2 ng/dl
(NR 0.0–0.6 ng/dl). Laboratory tests obtained on the same day (prior to the arrhythmia
episode) were not relevant to explain the event (leucocytes 2050 mm3, neutrophils
1790 mm3, lymphocytes 0.110 mm3, sodium 139 mmol/L, potassium
4.26 mmol/L, magnesium 1.7 mg/dl, calcium 9.1 mg/dl, phosphorus 4.1 mg/dl). A Cardiology
Service consultation was requested; unfortunately, the patient presented a cardiac arrest
(50 min after pharmacological cardioversion) without response to cardiopulmonary
resuscitation and died.


A previously healthy 26-year-old man without a significant past medical history was admitted to the emergency department with pleuritic, substernal chest pain that had steadily increased in frequency and intensity for 2 weeks. The pain was sharp, localized to the left precordium and was exacerbated by deep respirations or changes in position. He also reported a dry cough and dyspnoea. In 2016, a workup for similar chest pain that included an electrocardiogram, transthoracic echocardiogram, and myocardial perfusion imaging study were reportedly unremarkable.
On physical examination, he appeared to be in moderate distress due to chest pain. He was tachycardic (pulse 100–110 b.p.m.), afebrile, and normotensive. A coarse systolic murmur was audible at the parasternal border and radiated throughout the precordium. S1 and S2 were distinct and there were no additional heart sounds. Electrocardiography disclosed diffuse ST-segment elevation and PR segment depression (). The patient was admitted to the hospital with an initial plan for medical therapy of presumptive acute pericarditis using non-steroidal anti-inflammatory drugs and colchicine.
Subsequently, plain chest films revealed bilateral nodular opacities and a widened mediastinum. Transthoracic echocardiography (TTE) identified an anterior mediastinal mass (). The mediastinal mass and surrounding oedema compressed the main pulmonary artery to a minimum diameter of 7 mm and generated a 48-mmHg gradient across the RVOT. Correspondingly, the right ventricle was moderately dilated (57 mm) with reduced systolic function. Left ventricular systolic function was preserved (ejection fraction 65–69%). There was no evidence of direct pericardial involvement or invasion by the mass. Colour Doppler also detected a small, transient right to left shunt through a restrictive ventricular septal defect (VSD) proximal to the site of maximal RVOT compression (Video 1). Computed tomographic angiography identified multiple enhancing pulmonary nodules and demarcated the complete extent of the mass (70 mm × 76 mm × 92 mm) in the anterior mediastinum (). Initial laboratory values were unremarkable. The red blood cell count was 4.78 M/µL (reference values: 4.60–6.20 M/µL) and the white blood cell count was 13 K/µL (reference values: 4.5–12 K/µL). Serum troponin, AFP, and β-hCG were undetectable. Transbronchial biopsy and subsequent pathologic analysis confirmed that the mediastinal mass was a metastatic stage III mediastinal non-seminomatous germ cell tumour. Over the next four months, the patient received VIP chemotherapy using cisplatin, etoposide, and ifosfamide with an initially favourable response. After multiple rounds of chemotherapy, the patient underwent a median sternotomy with successful resection of the anterior mediastinal mass. Treatment resulted in decompression of the RVOT, restoration of RV systolic function, and resolution of the patient’s chest pain (). However, at 8 months, imaging detected new disease progression with enlarging pulmonary parenchymal lesions and probable bony metastases. This occurred in concurrence with an up-trending hCG serum level after the initial rounds of chemotherapy (2 mIU/mL) until detection of the lesions and metastases (4630 mIU/mL). hCG serum levels increased one month afterward to 9570 mIU/mL. The patient underwent additional rounds of salvage chemotherapy.


Case 2- After normal delivery of a 4,000-g, 39-week-old male infant, he was diagnosed with partial thoracoabdominal EC in addition to cleft palate/lip and Pentalogy of Cantrell. There was a ventral supraumbilical defect in which the heart was partially located (Figure ). Echocardiography revealed DORV with a large VSD, PS, and persistent superior vena cava. A cardiac MRI was performed for defining anatomical relationships more clearly (Figure ). A diagnostic angiography showed transposed DORV and subpulmonic significant PS. The RV had a large diverticulum placed toward the apex protruding from the defect. Cleft palate/lip repair was performed at 14 months of age. The patient underwent a left modified Blalock-Taussig (mBT) shunt operation at the age of four years for cyanosis. The patient is currently six years old, and the defect has decreased in size relative to the body with normal growth and development and is covered with epithelium (Figure ). A written informed consent was obtained from each parent for all diagnostic and therapeutic procedures.


Case 4- A seven-day old girl infant was referred from another province with the diagnosis of EC, ASD, and PDA. Initial examination revealed a term, healthy infant with a midsternal defect from which the heart was readily observable (Figure ). Pericardium partially covered the exposed heart. The patient was operated urgently upon arrival. At the operation, the sternum was found to be completely bifid. There was a persistent left superior vena cava and the PDA was ligated. Aorta, superior, and inferior vena cava were cannulated, and the 7-mm ASD was closed with primary suture under cardioplegic arrest. For closure of the thoracic defect, both sides of the sternum were mobilized by detaching the sternoclavicular joints. Pectoralis major muscles were also mobilized. These maneuvers facilitated partial approximation of the sternum with steel wire sutures and closure of muscle, fascia, and skin layers (Figure ). The patient had an uneventful recovery with a normal development at her latest follow-up at the age of five years (Figure ). A written informed consent was obtained from each parent for all diagnostic and therapeutic procedures.


A 59-year-old man with a previous history of multifocal bilateral posterior circulation cerebral infarctions from paroxysmal atrial fibrillation was referred for neuro-ophthalmologic evaluation for persistent visual field deficits 3 years after his strokes. He had no past ocular history. The patient had presented with sudden vision loss to the right side in both eyes' field of vision. He also reported binocular loss of color vision and difficulty reading. Brain magnetic resonance imaging at the time revealed acute infarctions of the left posterior cerebral artery territory involving the left occipital and temporal lobes, left thalamus, and left hippocampus, as well as punctate acute infarctions involving the right medial occipital lobe. On neurological examination upon discharge, he had a right homonymous superior quadrantanopia to confrontation, alexia without agraphia, and prosopagnosia.
On evaluation 3 years later, the patient reported subjective improvement in his reading ability. Visual acuities were 20/20 in the right eye and 20/15-2 in the left eye, and color vision with Ishihara plates was 12 of 14 correct in the right eye and 13 of 14 correct in the left eye. Pupils were normal. Dilated fundus examination revealed cup-to-disc ratios of 0.3 in both eyes and normal optic nerves without visible pallor []. Automated perimetry demonstrated a right homonymous superior quadrantanopia and left homonymous superior paracentral scotomatous defects []. OCT of the peripapillary RNFL (Cirrus HD-OCT 5000, Zeiss, Oberkochen, Germany) was within normal limits except for mild inferonasal thinning in the right eye and mild inferotemporal thinning in the left eye as seen on the temporal-superior-nasal-inferior-temporal (TSNIT) graph []. Macular OCT showed inferior homonymous GCC thinning to the left and right of the foveae, worse to the left in both eyes, corresponding precisely with his visual field defects [].