Patient A, a 12-year-old boy, presented a congenital myopathy phenotype from the first years with difficulty raising from the floor. At the last examination he reported a rapid and severe worsening, in a short time he went from walking a couple of km in the mountains to walking only 50–100 m on level ground with the help of the rail. His 43 year-old mother (patient B) showed a moderate and progressive evolution of the limb-girdle disease.
In the second family, the daughter (patient C) of 33 years old and her mother (patient D) of 59 years old showed atrophy of upper girdle muscles, especially in trunk, deltoid and triceps, difficulty in lifting arms over the head, arachnodactyly and scoliosis. The daughter’s muscle MRI shows advanced atrophy and fat/connective substitution in gluteus minimus and medius, of posterior thigh muscle semitendinosus and anterior thigh vastus lateralis, sartorius and tensor fasciae latae. There is atrophy of tibialis posterior, flexor hallucis longus and gastrocnemius. The mother is doing relatively well, although her muscle biopsy shows more advanced signs of muscle fiber splitting and segmentation. The muscle MRI shows atrophy of glutei and posterior thigh muscles. There is also atrophy of anterior thigh compartment particularly in vastus lateralis and rectus femoralis and advanced connective substitution of sartorius, tensor fasciae latae and both gastrocnemius muscles.
This group is composed by three non-consanguineous cases and one consanguineous case.


Patient E is a 22-year-old man with a severe and rapid evolution of the disease. His parents came from a small village in Moldavia. He had an early onset progressive weakness of lower limb and was not able to run or walk fast, has difficulty climbing stairs that he can do only with the help of handrail and is not able to perform Gowers’ maneuver. Muscle strength graded by MRC scale is 4/5 in biceps brachialis and quadriceps, and 3+/5 in iliopsoas. His biceps muscle is atrophic, as well as calf muscles, and has bilateral pes cavus. His CK is 7060 U (n.v. <308 U) and the EMG was myopathic.
He is in therapy with methylprednisone 16 mg/day and vitamin D with no benefit.
Muscle MRI shows marked atrophy with adipose involution of the posterior glutei and thigh muscular structures, and minor atrophy of the posterior components in the calf.
Patient F is a female of 50 years old. At the last visit, the patient has bilaterally scapular winging, slightly waddling walk, scoliosis and she complains of pain in the back leg. One sister and her daughter have elevated CK. In her muscle biopsy a myopathy was observed: Where was type 1 fiber predominance with atrophic fibers of both types.
Her muscle MRI shows marked fibro adipose substitution on gluteus and bilaterally of posterior thighs muscles. She was carrying a heterozygous missense mutation in the CAPN3 gene (1486 G > A in exon 11, with amino acid substitution p.G496R).
Patient G: This 59-year-old woman has been suffering from lower limb weakness since age 17. EMG showed myopathic sign and a muscle biopsy was done were inflammatory signs with macrophagic infiltration was observed, for which she was seen by Lord Walton at age 18, and she was found to suffer from a LGMD myopathy. She has used a wheelchair since age 45. Her CK was 1025 U (n.v. up to 50 U). She has suffered from hypertension since 50 years of age.
She carries two heterozygous mutation of the CAPN3 gene.
Patient H, a 49-year-old patient, was born from a consanguineous family affected by weakness and fatigability, for which, at 23 years old, a muscle biopsy was done.
The weakness was mostly in lower girdle; he had a wadding gait, winging scapulae and was diagnosed as affected by LGMD. He was treated with steroids and BCAA infusion. A mutation of CAPN3 gene was found in exon 21 homozygous. He has been WCB (wheelchair bound) since age 49.
This group is composed by three female patients that presented a mutation in γ (patient I), β (patient L) and α (patient M) sarcoglycan gene, respectively.


Patient I is a 21-year-old female of Tunisian origin. The onset of the disease was at about 12 years old when the patient began to notice easy fatigability; she was no longer able to sustain the running competitions with her peers. Subsequently, a waddling gait on tiptoes and proximal weakness of the lower limbs appeared. In 2009, she performed muscle biopsy that showed marked fibers variability with numerous hypertrophic and atrophic fibers, splitting and centralized nuclei. Recent MRC scores are present in .
Patient L, a 22-year-old female, diagnosed at three years of age with elevated CK value (22,206 U), showed a progressive muscular dystrophy and dystrophic muscle biopsy. At three years old she had scapular winging, Gowers’ sign and was unable to climb stairs. At six years of age, a muscle CT scan showed no significant atrophy. In this patient, a muscle MRI was not performed. She became wheelchair bound at age 12 years. During the following years, she developed cardiac involvement with reduction of ejection fraction, diffuse ventricular hypokinesia and life threatening arrhythmias for which she was in list for cardiac transplantation.
Patient M. This 65-year-old woman had lower-girdle weakness for which, at age 31. she was hospitalized and a biopsy was done. She had in her history a consanguineous family with a diagnosis of LGMD entertained. She had at α sarcoglycan immunohistochemistry a partial reduction of staining.
She was found to carry a homozygous change in α-sarcoglycan gene C > T at nucleotide 850. Muscle MRI was not performed.


A 44-year-old woman had presented hematuria and proteinuria from 7 years ago. She was admitted to perform renal biopsy in our hospital. Besides, she has showed PIP joints pain for several years. Both rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibody (anti-CCP) showed positive. Thus, rheumatoid arthritis (RA) was diagnosed by rheumatologist.
Blood examination showed Cr 0.43 mg/dl, eGFR 121.8 mL/min/1.73 m2, IgG 1429 mg/dl, IgA 653 mg/dl, C3 72 mg/dl, RF 734.4 IU/ml, anti-CCP-Ab 6.2 IU/ml. Antinuclear antibodies (ANA), myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA), proteinase3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and cryoglobulins were negative. Urinalysis showed urine protein–creatinine ratios (UPCR) 2.4 g/gCr, urinary red blood cells (U-RBC) 11–15/high-power field (HPF).
Light microscopic finding (LM) revealed mesangial cell proliferation and increase of mesangial matrix as well as endocapillary proliferation. Immunofluorescence analysis (IF) revealed mesangial deposition of IgA and C3. Mesangial and capillary deposition of IgG and IgM also noted. (Fig. ).
Based on the pathological findings, we diagnosed as IgAN. To exclude secondary IgAN caused by RA, immunostaining with KM55 mAb was performed. Finally, we diagnosed as primary IgAN due to positive staining of KM55 mAb colocalized with IgA deposit area (Fig. ).
According to the diagnosis of primary IgAN, tonsillectomy with steroid pulse (TSP) therapy was performed. Urinary abnormalities has been gradually improved and achieved clinical remission (CR) afterward (Fig. ). CR defined as three consecutive negative results over a 6-month period in urinary occult blood tests; urinary sediment red blood cell count of < 5/HPF; and urinary protein of < 0.3 g/day []. After TSP therapy, serum levels of IgA and Gd-IgA1 significantly decreased (Table ).
Clinical symptoms of RA initially getting better by steroid therapy. However, after tapering steroid dosage, PIP joints pain has gradually getting worse without relapse of urinary abnormalities. Thus, treatment with methotrexate (MTX) was started after finishing steroid therapy. Currently, the disease activity of RA has been under control with MTX treatment and urinary findings have also maintained remission.


A 42-year-old woman who was diagnosed as systemic lupus erythematosus (SLE) when she was 39 years old with the findings of polyarthritis, positive for ANA (1:640, homogeneous and speckled pattern) and anti-ds-DNA Ab, lymphocytopenia, and proteinuria (0.5–1.0 g/gCr). She had been followed by rheumatologist without any treatment. However, proteinuria and hematuria had been persisted. Thus, a renal biopsy was performed for definitive diagnosis.
Blood examination showed Cr 0.35 mg/dl, eGFR 177.8 mL/min/1.73m2, IgG 1214 mg/dl, IgA 282 mg/dl, C3 103 mg/dl. ANA, anti-ds-DNA. anti-SS-A, anti-SS-B, RF, and anti-CCP were positive. MPO-ANCA, PR3-ANCA, anti-GBM, and cryoglobulins were negative. Urinalysis showed UPCR 0.64 g/gCr and U-RBC > 50/HPF.
LM revealed mild mesangial cell proliferation and increase of mesangial matrix. IF showed mesangial deposition of IgA and C3, but negative for C1q that is atypical for lupus nephritis (Fig. ). Based on the pathological findings, we could not diagnose definitively IgAN or lupus nephritis. Then, immunostaining with KM55 mAb was performed and showed its positive staining colocalized with IgA (Fig. ). Finally, we diagnosed as primary IgAN. After TSP therapy, urinary abnormalities have been gradually improved and achieved CR (Fig. ). Moreover, level of Gd-IgA1 significantly decreased (Table ).
Disease activity of SLE had been relatively stable during steroid usage. However, after discontinuation of steroid treatment, polyarthritis exacerbated and serum level of anti-ds-DNA Ab gradually elevated. She has been followed by rheumatologist. Currently, induction of additional immunosuppression therapy is considered. Both proteinuria and hematuria have been able to maintain remission.


A 63-year-old man had presented with proteinuria (0.5–1.0 g/gCr) from 7 years ago. Proteinuria progressed to around 5.0 g/gCr. A renal biopsy was performed for definitive diagnosis. Besides, he had hepatitis C (HCV) without any anti-viral treatment.
Blood examination showed Cr 0.74 mg/dl, eGFR 114.3 mL/min/1.73m2, IgG 1905 mg/dl, IgA 338 mg/dl, C3 120 mg/dl. ANA, RF, MPO-ANCA, PR3-ANCA, anti-GBM and cryoglobulins were negative. HCV-RNA showed as 5.6 LogIU/mL. HCV subtyping showed type 1b. Urinalysis showed UPCR 3.6 g/gCr and U-RBC showed 1–4/HPF. Serum level of Gd-IgA1 was low, compared with cases 1, 2 and 4 (Table ).
LM revealed mesangial cell proliferation and increase of mesangial matrix as well as endocapillary proliferation. IF showed slightly positive for IgA and C3 in mesangial area (Fig. ). IgG also showed weakly positive in capillary area. For the presence of active inflammatory lesions, such as endocapillary proliferation, we diagnosed as active IgAN with massive proteinuria.
Initially, we considered to arrange steroid pulse therapy to treat acute lesions. However, for the complication of HCV, steroid therapy would be the risk of its activation. We decided to administer oral steroid therapy instead of steroid pulse therapy after discussing with gastroenterologist.
After informed consent, we started steroid therapy with close monitoring of liver function. However, improvement of proteinuria was limited and the liver function gradually getting worse. Thus, we stopped steroid therapy and performed immunostaining with KM55 mAb for definitive diagnosis. Finally, we diagnosed as HCV-related nephropathy (HCV-RN) due to negative staining of KM55 mAb in glomeruli (Fig. ).
After the diagnosis of HCV-RN, we consulted gastroenterologist for HCV treatment. Antiviral therapy with daclatasvir and asunaprevir was arranged. Proteinuria has been gradually improved by the antiviral treatment (Fig. ). HCV also achieved sustained virological response (SVR).


A 35-year-old man presented with proteinuria and hematuria which were noted by regular health check. No abnormal urinalysis was noted until three years ago. A renal biopsy was performed for definitive diagnosis. He was diagnosed with Crohn’s disease (CD) when he was 29 years old. The disease activity of CD was stable under treatment with mesalazine and adalimumab.
Blood examination showed Cr 1.06 mg/dl, eGFR 76.6 mL/min/1.73 m2, IgG 1304 mg/dl, IgA 502 mg/dl, C3 93 mg/dl, CRP 0 mg/dl. ANA, RF, MPO-ANCA, PR3-ANCA, anti-GBM and cryoglobulins were negative. Urinalysis showed UPCR 0.56 g/gCr and U-RBC > 50/HPF.
LM revealed mesangial cell proliferation and increase of mesangial matrix. IF showed positive for IgA and C3 in mesangial area (Fig. ). To distinguish primary IgAN and secondary IgAN with CD, immunostaining with KM55 mAb was performed and showed its positive staining colocalized with IgA (Fig. ). Finally, we diagnosed as primary IgAN. After TSP therapy, urinary abnormalities have been gradually improved and achieved CR (Fig. ). Furthermore, serum levels of IgA and Gd-IgA1 significantly decreased (Table ).


A 32-year-old lady with smear-positive pulmonary tuberculosis (PTB) on treatment presented with recurrent episodes of shortness of breath (Modified Borg Dyspnoea Scale 6 at rest: severe) and orthopnoea. She was treated as presumed adult-onset asthma by a primary care doctor and was empirically commenced on inhalers but to no avail. Computed tomography (CT) demonstrated luminal narrowing of the upper one-third of trachea secondary to EBTB. Bronchoscopy confirmed the presence of a concentric membranous tracheal stenosis with an opening diameter of 2 mm and a length of 0.5 cm (Fig. ). As her tracheal stenosis was beyond the subglottic level, after discussion with colleagues from ear-nose-throat (ENT) team, she underwent rigid bronchoscopy followed by gradual balloon dilatation of stenotic segment (Fig. ). TMC was subsequently applied using soaked gauze balls at a dose of 0.4 mg/mL for 2 min each over upper, middle, and lower parts of the stenotic segment (Fig. ). Post procedure, she reported improvement in symptoms (Modified Borg Dyspnoea Scale 4 at rest: moderate) which were sustainable at four months during her last clinical encounter without any TMC-related adverse events.


A 57-year-old lady with PTB two years previously presented with dyspnoea and stridor for the past year (Modified Borg Dyspnoea Scale 7 at rest: very severe) due to TSTB. Prior to this, she underwent multiple endoscopic procedures (balloon dilatation and laser cauterization) at a private hospital. CT demonstrated luminal narrowing at mid-trachea (Fig. ). The stenotic segment length was at 1.5 cm, significantly longer than the first case. Flexible bronchoscopy confirmed tracheal stenosis with an opening diameter of 2 mm. A dense and thick fibrotic band was seen anterior to the luminal opening, presumably aggravated by scarring secondary to previous procedures (Fig. ). She underwent rigid bronchoscopy followed by balloon dilatation of stenotic segment. Following that, mechanical coring of stenotic segment was carefully performed by utilizing a rigid bronchial tube (outer diameter 10 mm, colour coded red, Series-2 Efer-Dumon Bronchoscope, France) (Fig. ). TMC was then applied using soaked gauze balls at a dose of 0.4 mg/mL for 2 min each over upper, middle, and lower parts of the stenotic segment. Post procedure, she initially reported symptomatic improvements (Modified Borg Dyspnoea Scale 5 at rest: moderate) which lasted for less than two weeks. She subsequently successfully underwent airway surgery for resection of stenotic segment.


A 38-year-old lady with PTB 10 years previously presented with a three-year history of progressively worsening dyspnoea (Modified Borg Dyspnoea Scale 3 at rest: moderate) due to right main bronchus stenosis. Flexible bronchoscopy confirmed a narrowed and malacic right main bronchus with a length of approximately 2 cm and an opening diameter of 3 mm (Fig. ). Balloon dilatation of the diseased segment was done via rigid bronchoscopy followed by application of TMC. Post procedure, she reported dramatic improvements in symptoms which lasted for just two weeks. Repeated bronchoscopy showed recurrence of bronchomalacia. Following that, a multidisciplinary discussion involving cardiothoracic surgeons, pulmonologists, thoracic radiologists together with patient herself and her family members was conducted to determine the next best management plans. Airway surgery was deemed risky due to long diseased segment making end-to-end anastomosis technically challenging. As the patient was keen to proceed with intervention, we did not pursue further with regards to conservative measures such as continuous positive airway pressure (CPAP). She was subsequently scheduled for silicone stent insertion at right main bronchus.


Back in December 2016, a 22-year-old gentleman presented with cough, weight loss, and persistent dyspnoea on exertion (Modified Borg Dyspnoea 4 at rest: somewhat severe) despite already on three months of medications for PTB. CT revealed multilevel stenosis involving the trachea and right main bronchus with concurrent right upper lobe collapse (Fig. ). Flexible bronchoscopy confirmed a stenosed right main bronchus without bronchomalacia or thick fibrotic band. Rigid bronchoscopy was performed along with gradual balloon dilatation of the stenotic segment before the application of TMC. Again, TMC was applied via soaked gauze balls for a total of eight times along the diseased airway. He reported significant symptomatic relief which lasted for more than a year before recurrence. As he rejected surgery, he subsequently underwent another two rounds of rigid bronchoscopy, balloon dilatation, and TMC (April 2018 and January 2020) without any adverse events. Latest surveillance scope by June 2020 confirmed a patent right main bronchus. He remained well and active in between procedures.


A 43-year-old lady with PTB 10 years ago presented with a two-year history of cough and worsening dyspnoea (Modified Borg Dyspnoea 5 at rest: severe). Work-up for recurrence of PTB was negative but CT showed tracheal narrowing with right main bronchus stenosis. Flexible bronchoscopy revealed a short-segment concentric membranous stenosis at right main bronchus with an opening diameter of 2 mm. Similar to other cases, she underwent rigid bronchoscopy for balloon dilatation of the right main bronchus before TMC application for a total of six times along the airway. Her procedure was complicated by a small airway tear (<0.5 cm) at the right main bronchus but she nevertheless remained stable without needing emergency airway surgery. She reported sustained improvements in dyspnoea post procedure (Modified Borg Dyspnoea 2: slight). Her latest surveillance scope done more than 15 months post TMC showed a narrowed but patent right main bronchus (Fig. ).


A 29-year-old lady with a history of PTB back in 2017 presented with worsening dyspnoea (Modified Borg Dyspnoea Scale 3 at rest: moderate) a year after the completion of anti-tuberculosis chemotherapy. Similar to the first case, she was initially empirically treated (unsuccessfully) as presumed adult-onset asthma. CT showed left main bronchus stenosis with segmental atelectasis of the left upper lobe (Fig. ) while bronchoscopy revealed bronchomalacia with fibrostenosis at the distal end of left main bronchus. Rigid bronchoscopy was arranged for balloon dilatation of stenotic segment followed by TMC using the same method and dosage as described in previous cases. She reported initial improvements of symptoms which lasted for approximately two months. Repeated bronchoscopy revealed restenosis of the left main bronchus. She was then offered for evaluation for potential surgical treatment in which she declined. Fortunately, she remained stable on outpatient clinic follow-ups despite physical limitations (Modified Borg Dyspnoea Scale 3 at rest: moderate; similar to initial presentation).


A 26-year-old lady had multilevel tracheobronchial stenosis (trachea and left and right main bronchus) secondary to EBTB. She had a background history of recurrent tuberculosis involving the gastrointestinal system in 2014 followed by PTB by 2019. While on treatment for PTB (after five months of medications), she presented with new-onset worsening dyspnoea and reduced effort tolerance. With the diagnosis in mind, she underwent balloon dilatation of trachea and left main bronchus followed by application of TMC. The right main bronchus was totally obstructed. Her procedure was complicated by tracheal tear during balloon dilatation which fortunately was successfully managed conservatively without the need of emergency airway surgery. Post procedure, she reported improvements in symptoms which lasted for five months during her last clinical encounter with us.


A 45-year old white Caucasian female, with American Society of Anesthesiology (ASA) physical status 1 (57 kg, 169 cm), presented for elective laparoscopic hysterectomy. After premedication with midazolam 1 mg intravenously, anesthesia was induced with fentanyl 100 μg, propofol 180 mg and rocuronium 60 mg. Tracheal intubation was easily performed (Cormack and Lehane grade 1). Anesthesia was maintained with sevoflurane in O2/Air (50%:50%) and intermittent doses of fentanyl 50 μg. Muscle relaxation was achieved with 10 mg of increments of rocuronium. Standard monitoring included electrocardiography, non-invasive blood pressure, oxygen saturation (SpO2) and end-tidal carbon dioxide (ETCO2). Multigas analysis was used throughout the operation.
The patient was hemodynamically stable throughout the operation. The abdomen was insufflated blindly with carbon dioxide at a pressure of 15 mmHg. SpO2 was 98–99% and ETCO2 was maintained at less than 40 mmHg with peak inspiratory pressure between 18 and 27 cm H2O. Tidal volume was set at 570 mL and 14 breaths per minute respectively. The surgical procedure was prolonged, lasting 240 minutes. The patient was extubated without difficulty.
The patient was not mobilized on the first postoperative day due to pain; pain was alleviated with paracetamol 1000 mg and diclofenac 75 mg and the patient was able to walk on the next morning. Three hours after mobilization, the patient became dyspnoic; dyspnoea was worse in the supine position and by the next 15 minutes, she could maintain an SpO2 of 93%, only by Venturi mask of 30% FiO2 at an oxygen flow rate of 12 litres per minute and only in the sitting position. The patient readily developed dysphonia, neck and face edema that were initially treated as anaphylactic reaction. No improvement was seen, and the patient was transferred to the Intensive Care Unit (ICU). Crepitus could be felt in the neck, thorax and lower limb area. Chest X-ray was not diagnostic. The patient rapidly developed cardiovascular and respiratory impairment and was transferred to the operation theatre under inotropic support for emergency laparotomy. Intubation was achieved with an endotracheal tube of 5.5 mm internal diameter. Mechanical ventilation was copious with the patient deteriorating; suspected tension pneumothorax was treated with immediate decompression of the pleural space with needle thoracocentesis. Faecal odour was noticed on thoracocentesis and surgical exploration revealed sigmoid perforation which had taken place during the laparoscopic hysterectomy. The ruptured sigmoid was closed and a diagnosis of septic shock with subcutaneous emphysema caused by colon perforation was retained. The patient was transferred to the ICU, where she developed high fever and mediastinitis, 7 hours after surgery. Aggressive intravenous antibiotic therapy was started with clinical improvement during the next 3 days. The patient was extubated on the second postoperative day and was dismissed from hospital 10 days later, after the resolution of mediastinitis and infectious pneumonia. The patient suffered recurrent mild pneumonias over the next period and was completely symptom-free from the respiratory tract, two months after surgery.


A 33-year-old, previously healthy, nonsmoking, Caucasian male presented with a mild, painless, bilateral lower extremity oedema and gradually worsening dyspnea on exertion of one-month duration. His electrocardiogram (ECG) demonstrated negative T-waves, chest radiography indicated right pleural effusion, whereas the transthoracic echocardiogram (TTE) revealed the presence of small pericardial effusion. High-sensitivity cardiac troponin, virologic, and immunologic testing were negative, whilst D-dimers level was high.
A computed tomography pulmonary angiography (CTPA; Figure ) excluded pulmonary embolism, yet confirmed the presence of both right pleural and pericardial effusion, together with mild ascites. A subsequent cardiac magnetic resonance imaging (CMR; Figure ) depicted a broad-based right atrial mass that was infiltrating the underlying myocardium and pericardium. Finally, an endomyocardial biopsy via the right femoral vein, guided by two-dimensional transesophageal echocardiography, established the diagnosis of a primary heart angiosarcoma, whereas staging with positron emission tomography (PET) revealed widespread metastases in right scapula, in right first rib, and lungs.
Systemic combination treatment with weekly paclitaxel (150 mg/m2) and oral propranolol (40 mg three times daily) was commenced, resulting in prolonged disease control. Indeed, three months upon treatment initiation TTE revealed absorption of the pericardial effusion, whereas a computed tomography (CT) scan revealed substantial shrinkage of the pulmonary nodules. Second-line treatment with epirubicin plus ifosfamide was initiated eight months later, upon disease relapse in the liver, as it was revealed with routine follow-up scanning. The patient eventually died of multiple-organ progressive disease 17 months after the initial diagnosis.


A 60-year-old man had left-sided chest pain during inspiration after being hospitalized with cerebral infarction. There was a slight fever of 37°C from the time of admission, and there was a slight increase in peripheral leukocytes and c-reactive protein. No respiratory symptoms other than chest pain during inspiration. There was no pleural friction rub or crackles. No weight loss was observed. Past history includes type 2 diabetes, hypertension, hyperuricemia, hypertrophic cardiomyopathy, and paroxysmal atrial fibrillation. The patient smoked 50 pack-year and was a moderate drinker. There was no dental disease.
The chest X-ray film taken on admission revealed a large mass in the left middle lung field. Chest CT showed a 4.0 cm mass-like lesion in the lingular segment of the left lung. The mass-like lesion caused segmental consolidation and contained low-attenuation areas with peripheral enhancement on enhanced CT (Figure ).
Biochemical studies showed a slight elevation of carcinoembryonic antigen (CEA: 6.1 ng/ml, normal range 4.0). It was initially suspected to be complicated with lung cancer and obstructive pneumonia. F-18-FDG PET revealed high metabolism (early maximum standardized uptake value {SUV} of 9.5 and delayed maximum SUV of 12.1) by the mass-like lesion and also in lymph nodes (maximum SUV of 5.7) (Figure ).
We first suspected lung cancer, performed a CT-guided biopsy, and bronchoscopy. The biopsy specimen showed basophilic and lobulated bacterial mass by HE staining, and inflammatory granulation tissue was collected around it. Grocott's staining was positive. Ziehl-Neelsen staining was performed but was negative. No findings suspected of being malignant are found. Actinomyces israelii identified in bronchial lavage fluid and sputum cultures.
Initially, we administered tazobactam/piperacillin intravenously for the first two weeks, and after it was found to be actinomycete, we treated it with oral penicillin. The administration was continued until five months after the improvement of shadow was observed by follow-up Imaging. Even after the administration was completed, the shadow keeps shrinking and became a cord-like shadow, and the patient improved without complications (Figure ). No surgical treatment is performed.


Genetic counseling was provided to a 29-year-old Chinese woman (gravida 5, para 1) during her gestational 23 weeks since she had abnormal fetal ultrasonography results. The woman delivered a healthy female baby at term during her first pregnancy (). Ultrasonography at the 12th week of this pregnancy indicated that the fetus had multiple malformations, such as a short face, abnormal nasal bone, ear and mandible, and a cleft palate. At the 16th week of pregnancy, the fetus showed profound thickening of the palm and plantar soft tissues according to ultrasonography. The clinical diagnosis was not determined based on ultrasonography. Subsequent genetic tests were performed to make a definite diagnosis for the fetus. Combined with the results of whole-exome sequencing (WES) and ultrasonography, the fetus was finally diagnosed as HI. shows a timeline of the case report.
The baby was induced through vaginal delivery at 23 gestational weeks. The autopsy reports () are as follows: weight, 1,990 g; body length, 42.5 cm; head circumference, 33 cm; chest circumference, 27 cm; abdomen circumference, 24 cm; no necropsy; medium nutrition; there was no obvious ectropion of eyelid and undeveloped eyeball; the distance between the two eyes was 3.5 cm on the inside and 7 cm on the outside; nose collapse, the nasal bone showed poor development; the mouth and lip were everted and edematous, fish-like, with protruding alveolar and short mandible; the development of bilateral auricles was poor and the auditory canal existed; the skin of the whole body was covered with light yellow horns (0.2 cm thick, armor-like); the hands and feet were poorly developed; the fingers and toes were all flexed and claw-like; the development of external genitalia was poor; the gender discrimination was unclear (male consideration), and there was an anus. The liver was 81.2 g, red soft, 10 × 6 × 3 cm in size, and it was located at 4 cm below the xiphoid process and 2.5 cm below the right costal margin; the heart was 12 g with a size of 4.5 × 3 × 2.5 cm with an unclosed foramen ovale, and the heart ventricle wall was 0.5 cm thick. The peripheral diameters of the tricuspid valve, pulmonary artery, and mitral valve were 4, 1.5, and 3 cm, respectively; and the sizes of the two kidneys were both 4 × 2 × 2 cm. The left lung with two lobes was 5.5 × 3 × 2 cm in size and 14.6 g in weight, and it was also clear and sink in water; the right lung with three lobes was 6 × 5 × 2.5 cm in size and 19.8 g in weight, and it was also clear and sink in water. Pathological examination of the skin indicated severe hyperkeratosis of the epidermis.
The amniotic fluid was collected for genetic analysis. Genomic DNA was extracted using the QIAmp DNA Blood Mini Kit (Qiagen, Hilden, Germany) with standard protocols. The CytoScan 450K array (Affymetrix, CA, United States) was used to detect copy number variations (CNV). No abnormalities were identified through CNV array tests and karyotype analysis, so the whole-exome sequencing (WES) was performed for all the family members (including the fetus’ sister, parents, and grandparents) to clarify the genetic risk for the next pregnancy. For WES, exome capture was performed using SureSelectXT Human All Exom V4 (Agilent Technologies, CA, United States). Sequencing was implemented on the Illumina HiSeq 2000 system (Illumina, CA, United States). Sequencing data analysis was performed following the GATK best practice. Detected variants were annotated and filtered using Annovar based on information of functional prediction (e.g., Polyphen2, SIFT, varianttaster, REVEL MuMetaSVM), disease association (e.g., ClinVar, OMIM, GWAS, HGMB, and SwissVar) and population allele frequency (e.g., dbSNP150, ExAC, 1000 genome phase3, EVS). A compound heterozygote for the variants c.2157delA (p.Q719QfsX21) and c.6858delT (p.F2286LfsX6) was identified. Both variants were frameshift deletions. The variants were verified using Sanger sequencing with a set of primers (ABCA12-17F: 5′-ATTATCAGGTTCTCTTTCTCTGTTG-3′, ABCA12-17R: 5′-CTATTTTTATTCTTGGGGAAAATT-3′; ABCA12-46F: 5′-GAGAGATACAAAAGCAATGTCTCA, ABCA12-46R: 5′-CTCATTTAAGTATGTTGTACTCGCT-3′). The results suggested that the sister, the mother, as well as the maternal grandmother carried the heterozygous c.2157delA, and the father and the paternal grandmother carried the heterozygous c.6858delT (, ).


A 78-year-old female patient visited our emergency department with left elbow and wrist pain after slipping and falling. Her wrist and elbow showed swelling and painful limited ranges of motion. The neurovascular examination revealed no peripheral neurovascular injuries. The preoperative radiographs and computed tomography showed an AO type C3 distal radius fracture, a Mason type III radial head fracture, and Regan-Morrey type I coronoid process fractures. The elbow joint was not dislocated (Fig. ). The distal radius fractures were treated with open reduction and internal fixation (ORIF) with a volar approach using a 2.4 mm Variable Angle Locking Compression Plate Distal Radius system (VA-LCP, Synthes, West Chester, PA). For the radial head fracture, radial head replacement arthroplasty (Tornier SA. Saint-Ismier, France) was performed because of the patient's age and severely comminuted fragments. The coronoid process fracture was fixed with a 3.5 mm cortical screw through the lateral approach (Fig. ). Postoperative immobilization was applied, and an above-elbow splint was used for 2 weeks, after which a short arm splint was applied until postoperative 4 weeks. Elbow motion was allowed after postoperative 2 weeks, and wrist motion was allowed after postoperative 4 weeks. The bony union of the distal radius was visualized on x-ray at postoperative 6 weeks. The patient was followed for 13 months, at which her wrist showed 45 degrees of flexion and extension, full supination, and pronation. Her elbow showed near full range of motion. Quick Disabilities of the Arm, Shoulder and Hand score was 18 at the final follow-up visit. There were no major complications such as infection, loss of reduction, or nonunion. The x-ray showed 5.5 mm radial shortening.


A 19-year-old female patient visited our emergency department with left elbow and wrist pain after slipping and falling. Her wrist and elbow showed swelling and painful limited range of motion, and neurovascular examination revealed no peripheral neurovascular injuries. The preoperative radiographs and computed tomography showed an AO type B2 undisplaced distal radius fracture and a Mason type III radial head fracture (Fig. ). The distal radius fracture was undisplaced and treated conservatively. An ORIF with 2.4 mm LCP plates was performed for the radial head fracture (Fig. ). Postoperative immobilization was performed with an above-elbow splint for 3 weeks, after which a wrist brace was applied until postoperative 4 weeks. Elbow motion was allowed after postoperative 3 weeks, and wrist motion was allowed after postoperative 4 weeks. The bony union of the distal radius was visualized on x-ray at postoperative 4 weeks. The patient was followed for 14 months, at which time her wrist and elbow showed full range of motion. Quick disabilities of the arm, shoulder and hand score was 16 at the final follow-up visit. There were no major complications such as infection, loss of reduction, or nonunion. The x-ray showed no change in ulnar variance.


An 11-year-old boy (case 1) consulted on 9 September 2019 due to a pruritic maculopapular skin rash of one day’s duration (). He had traveled with his family through Thailand from 16 August to 6 September 2019, visiting Bangkok, Chiang Mai, and Koh Tao. During the flight back to Barcelona (Catalonia, Spain), he experienced headache, elbow arthralgia, and a low fever that lasted 3 days. Subsequently, a maculopapular rash appeared, and then gradually faded over the next week. To investigate the suspected flavivirus infection, diagnostic tests were performed on 9 September 2019. A real-time reverse transcription–polymerase chain reaction (rRT-PCR) assay for ZIKV (RealStar Zika Virus RT-PCR Kit 1.0; Altona Diagnostics, Germany) yielded positive results in the serum and urine, whereas initial immunoglobulin (Ig) G and IgM tests (anti Zika virus ELISA, IgG and IgM, Euroimmun, Germany) were negative. Serologies and rRT-PCR for dengue virus (DENV) and chikungunya virus (CHKV) were negative. Subsequent serological ZIKV IgG tests showed seroconversion, with negative rRT-PCR results in the serum and urine. Curiously, DENV IgG tested positive in one of the follow-up analyses, but later tests were negative. Hence, the DENV result was considered a false-positive due to the known antibody cross-reactivity when analyzing these viruses (). None of the other family members travelling at the same time showed signs or symptoms of flavivirus infection, or positive test results.
A 33-year-old pregnant woman (case 2) travelled with her 2-year-old daughter (case 3) to Thailand from 17 December 2019 to 14 January 2020. They visited some of the islands of Thailand and the city of Bangkok. The mother reported having been bitten by mosquitoes in a Bangkok hotel on 12 January. Within 2 days, the mother and daughter had both developed a generalized skin rash that lasted 48 h. Neither of them had fever, headache, or conjunctivitis.
After returning from Thailand, the mother sought health care advice on 19 January (4 days after the symptoms onset). Based on the high clinical suspicion, serological and molecular tests for arboviruses in blood (ZIKV, DENV, and CHKV) were performed. ZIKV rRT-PCR and ZIKV IgM tested positive, but CHKV and DENV viruses were negative. Intensive antenatal follow-up was then carried out. Diagnostic testing was done monthly, with successive negative ZIKV rT-PCR results in the urine and serum. ZIKV IgG was first detected 23 days after symptoms onset, and a ZIKV plaque reduction neutralization test (PRNT) was positive. ZIKV IgM remained positive for 2 months ().
Frequent ultrasound examinations performed during pregnancy showed no fetal abnormalities. At birth, the central nervous system (CNS) ultrasound, automated auditory brainstem response (AABR) tests, and ocular fundus examination in the newborn (case 4) were normal. All ZIKV IgM and rRT-PCR (blood, urine, and saliva) tests were negative in the immediate postnatal period. ZIKV IgG was initially positive, with decreasing titers until seroreversion at 4 months of life. Serologies and rRT-PCR for other flaviviruses were negative ().
The 2-year-old daughter was seen 17 days after the onset of symptoms. ZIKV IgM, IgG, and PRNT tested positive, but ZIKV rRT-PCR was negative. Serologies and rRT-PCR for CHKV and DENV tested negative (). She presented no other clinical symptoms apart from a generalized maculopapular rash.


A 55-year-old male presented to the clinic with a suspicious growing lesion involving the nasal septum and columella. The lesion was firm and there was associated swelling of the distal aspect of the nasal septum. The nasal septum was severely deviated due to past untreated nasal fractures. A punch biopsy of the lesion was performed in the clinic under local anesthesia, which demonstrated fragments of necrotic cartilage and no malignancy. The lesion continued to grow in size, prompting excision under general anesthesia in the operating room. An inverted gull-wing incision was made and iris scissors were used to lift off the skin over the dorsum of the nose. The incision was then extended into a hemitransfixion incision into the nasal septum. Iris scissors were used to dissect around the lesion which encompassed bilateral lower cartilages. The excised lesion measured 2.5 x 1.5 x 1.1 cm and was sent for pathology.
A ragged polypoid firm-tan white lesion resembling a cartilaginous tumour was observed upon gross examination. Histopathological examination revealed a well-circumscribed tumour composed of lobulated mature hyaline cartilage with slightly increased cellularity. The chondroid matrix was evenly distributed in a well-defined lobular pattern invested by fibrous septae (Figure ). The majority of chondrocytes were arranged in individual lacunae and appeared bland with single, uniform, small nuclei surrounded by clear to eosinophilic cytoplasm (Figure ). Binucleated chondrocytes were also identified (Figures -). No signs of ischemic change, significant pleomorphism, mitoses, or necrosis were present. This was consistent with features of a benign chondroid neoplasm rather than a low-grade chondrosarcoma.


History
The patient is a 32-year old Caucasian male with diabetes mellitus type 1, intermittent alcohol consumption, and drug abuse. He had lived in Thailand for six years and returned to Sweden two years ago. He had been involved in three mild motorcycle accidents in Thailand but no neuroradiological investigations had been done. Furthermore, he had previously tested negative for both HIV and hepatitis.
Presenting symptoms
He complained of recurrent episodes of frontoparietal headache for the last year that was relieved by over-the-counter analgesics. His symptoms worsened during the last month before first admission with increased sensitivity to light, decreased ability to concentrate, and irritability. He sought medical advice at the primary health care centre and was referred to get a computerized tomography (CT) of the brain. This showed a large epidural mass at the right frontoparietal area that crossed the midline and compressed the sagittal sinus (Figure , B and D). The adjacent neurocranium showed scalloping of the inner table (Figure ). Initial radiological diagnosis was an organised chronic epidural hematoma and accordingly, he was referred to the emergency department at Sahlgrenska University Hospital.
At initial examination, he was fully oriented and had intact higher cognitive functions. The detailed neuro-ophthalmological and neurological examination were unremarkable. Laboratory analysis showed C-reactive protein (CRP) 9 mg/L, leucocyte count 5.7 x 109, and normal haemoglobin and creatinine levels. Magnetic resonance imaging (MRI) with and without contrast was performed, which showed high signal intensity on the T1-sequence (Figure , B and C) and low signal intensity on the T2 sequence (Figure ), and signs of heterogeneous diffusion restriction. Initial MRI report was also interpreted as an old, organized epidural hematoma. There was a peripheral contrast enhancement in the mass. At initial examination, he was fully oriented and had intact higher cognitive functions. The detailed neuro-ophthalmological and neurological examination were unremarkable. Laboratory analysis showed a CRP of 9 mg/L, leucocyte count 5.7 x 109, and normal haemoglobin and creatinine levels. Magnetic resonance imaging (MRI) with and without contrast was performed, which showed high signal intensity on the T1-sequence (Figure -C) and low signal intensity on the T2 sequence (Figure ), and signs of heterogeneous diffusion restriction. Initial MRI report was also interpreted as an old, organized epidural hematoma. There was a peripheral contrast enhancement in the mass.
Course of care
The patient was transferred to the neurosurgical department and underwent evacuation of the epidural collection. A craniotomy centralized over the mass was performed with a size of 57 x 70 mm. This turned out to be a white foul-smelling tissue of medium thickness mixed with pus. Furthermore, a well-organized white solid epidural mass was found. It was excised and sent for histopathology and bacterial culture. The dura was left intact and the bone flap was replaced using titanium plates. No perioperative complications were reported and postoperatively he was started on prophylactic cefuroxime. At the second postoperative day, he developed fever at 38.60C, general malaise, and tachycardia. Laboratory analysis showed CRP 74 mg/L, leucocyte count 7.0 x 109, and negative bacterial cultures in both blood and urine. Thereafter, his antibiotic regimen was changed to the carbapenem meropenem in meningitis dosage (2 g every eight hours). He started to improve clinically at day 4-5 after surgery and CRP gradually decreased. Bacterial culture from the epidural collection showed growth of Burkholderia pseudomallei, MIC values (mg/L) was 1 for meropenem, 2 for ceftazidime, 1.5 for amoxicillin/clavulanic acid, 6 for trimethoprim-sulfamethoxazole (TMP-SMX) and 1 for doxycycline. He was admitted to the Department of Infectious Diseases, Sahlgrenska University Hospital, for further treatment. Seven days postoperatively, he developed signs of wound infection and CT of the brain showed a highly suspected intracranial abscess in the previously operated area. He underwent surgical evacuation of the abscess and removal of the bone flap and continued with meropenem with the addition of TMP-SMX for eight weeks. After this period of time, the previously healed wound spontaneously opened and pus emptied out. The patient was clinically stable with no other symptoms or signs. CT scan showed an epidural fluid collection. New samples for bacteriology was taken and no surgical intervention was performed. The samples showed no growth of Burkholderia pseudomallei, but confirmed presence of DNA by PCR. The patient continued the antibiotic regimen stated previously for six months, with some interruptions of TMP-SMX because of adverse reactions such as fever and nausea.
One month after cessation of antibiotics he underwent a new CT scan with and without contrast with no apparent signs of infection, and he was scheduled for cranioplasty with acrylic plate after three months. However, the immediate preoperative CT scan with contrast showed a contrast-enhancing fluid collection epidurally. Surgical evacuation was performed without any cranioplasty, and the samples for bacteriology again showed growth of bacteria Burkholderia pseudomallei, MIC values (mg/L) was 0.5 for meropenem, 1 for ceftazidime, 2/2 for amoxicillin/clavulanic acid, 1/19 for TMP-SMX. He was treated using intravenous meropenem for two months with concomitant and adjuvant oral treatment with TMP-SMX for a total of 8 months. This time total length of treatment was eight months and the patient tolerated TMP-SMX much better. Three weeks after the antibiotic regimen was finished a new CT scan was performed, which showed total regression of the abscess and no contract-enhancement. This was followed three months later by a cranioplasty with acrylic plate. The preoperative CT scan with contrast did not show any signs of infection, nor was there any suspicion regarding infection during the surgery.


A 50-year-old woman presented to our primary care clinic in Japan with a chief complaint of AKP. She had undergone arthroscopic anterior cruciate ligament (ACL) reconstruction of her right knee 10 months previously (Figure ) and was undergoing postoperative rehabilitation, including manual therapy (percutaneous traction on scars) described by Alvira-Lechuz et al., range of motion exercises, and strength exercises []. However, the AKP persisted long after the surgery and the pain made it difficult for her to descend the stairs.
Magnetic resonance imaging confirmed that there was no re-tearing of the reconstructed ACL but showed a scar from the skin incision from the previous arthroscopic surgery (Figure ). Lachman’s test was negative, and there was no instability of the knee. The range of motion of the knee showed an active extension of -5˚ and an active flexion of 140˚ (contralateral, 0˚ to 150˚), as well as a loss of patellar mobility in supra-inferior directions. Palpation on the scar was painful, suggesting limited mobility of formed fibrotic tissue. The Kujala scale was 62 points and the visual analogue scale (VAS) during descending the stairs was 72mm []. The Knee Injury and Osteoarthritis Outcome Score (KOOS) showed KOOS pain of 69.4, KOOS symptoms of 75.0, and KOOS ADL of 94.1 []. Ultrasound imaging showed the IPFP scarring and sliding defects (Figure ). She was diagnosed with patellofemoral pain syndrome and treated as follows.
The patient was lying on the treatment bed in a supine position, with the knee joint at 30˚ of flexion. The inferolateral side of the patella was palpated to identify the scarring after arthroscopy at the IPFP area. Ultrasound was performed using a 4-18MHz B-mode linear array scanner (SONIMAGE HS2, Konica Minolta, Tokyo, Japan). The probe was applied vertically to identify the adhesion area of interest. Ultrasound-guided hydrorelease was performed using 7.0mL saline injected with a needle (22G, 60mm) directed at the site within 10 mm depth of hypoechoic changes in the IPFP (Figure ). The images pre-and post-injection were compared to identify the sliding of the IPFP. The ultrasound-guided hydrorelease was performed only once, and the treatment time was about 10 minutes, including ultrasound image examination. All procedures were performed by one orthopedic surgeon with more than 10 years of experience.
After ultrasound-guided hydrorelease, the patient was given rehabilitation including manual therapy (percutaneous traction on scars), mobilization of the patella, and strength exercises for the quadriceps femoris muscle for four weeks.
After four weeks, the Kujala scale was 82 points, and the VAS score during descending the stairs was 28mm. KOOS improved only slightly, with KOOS pain of 75.0, KOOS symptoms of 75.0, and KOOS ADL of 98.5. However, the items related to stair descent improved (P6, “going up or downstairs” and A1, “descending stairs”). The range of motion of the knee showed an active extension of 0˚, and the mobility of the patellar was no longer limited. The normalization of the IPFP sliding was confirmed by ultrasound image.
The patient had moderate needle prick pain (seven of 10 on an 11-point numerical rating pain scale) while undergoing ultrasound-guided hydrorelease. She felt a heaviness in her right knee for two days after the procedure, which improved spontaneously without specific treatment. There was no recurrence of heaviness thereafter.


A 22-year-old female with no significant past medical history presented to clinic for evaluation of vague abdominal pain. She reported a history of progressively worsening pain in the epigastric region along with nausea and bloating that had been ongoing for the past two months. She had been followed by her primary care physician and reported minimal response to over-the-counter analgesics including acetaminophen, nonsteroidal anti-inflammatory drugs, and antacids. She also noted no improvement with diet changes. Initial bloodwork revealed elevated liver function tests on workup and she was referred for imaging evaluation.
An ultrasound was performed which demonstrated cholelithiasis without cholecystitis and a cystic structure between the left kidney and the spleen with atypical features. The patient was sent for a subsequent CT of the abdomen and pelvis with and without contrast which revealed a large left upper quadrant cystic mass with septations and calcifications. The mass compressed multiple structures and displaced the pancreas anteriorly and the spleen laterally. An MRI was performed and demonstrated a 10.0 cm x 12.2 cm x 13.3 cm [anterioposterior (AP), transverse, and craniocaudal] retroperitoneal mass with predominate fat signal. Displacement of the left kidney inferiorly, spleen laterally, and stomach superiorly led to a presumed suprarenal origin (Figures -).
The patient underwent laparotomy and surgical excision of the mass. Histopathological examination after open surgical excision demonstrated a left suprarenal GN negative for malignancy. The patient had an uneventful postoperative course. Her postoperative pain was well managed with patient controlled analgesia (PCA) utilizing low dose hydromorphone. She had no complications and made a full recovery.


Nodule-type skin lesion was found in a ten-year-old male dog, German Shepherd crossbreed (35 kg), from Białowieża. The dog was adopted from a local shelter and has not left Białowieża since 2015. The nodule was located in the subcutaneous tissue of the cheek near the external angle of the left eye (Fig. ). A fast-growing lesion reached a diameter of 2 × 2 cm within 2 weeks from the moment the first skin changes have been observed (Fig. ). The nodule was removed surgically in August 2019. A cluster of several nematodes (3 specimens) was found in the cross-section (Fig. ). Monthly administration of the medicine containing imidacloprid and moxidectin was prescribed for 6 months.


R. is a 23-year-old, male, with a family psychiatric history of psychotic disorder and whose symptoms started at 13 with social isolation and poor hygiene. At 15, he became aggressive and suspicious, and risperidone 3 mg was prescribed daily; then, auditory hallucinations developed, so the antipsychotic was increased to 4 mg daily. However, he subsequently presented on numerous occasions to the emergency room (ER) with aggressive behavior and persecutory delusions. When he was 18, disorganized behavior and delusions of prejudice were added; for this reason, sulpiride 400 mg and olanzapine 10 mg daily were prescribed but without good response. Then, he presented with nihilistic delusions concerning his existence “I am dead, I died in 2012,” nihilistic delusions related to his body “I do not have a heart,” hypochondriacal delusions “my organs are not working,” delusions of guilt “I am taking the blame,” and suicidal ideation. For these reasons, he was again taken for his dad to the ER. In the mental examination, he was agitated, asking people to kill him. The PANSS upon admission of the patient was 125. During the hospitalization, he continued with the suicidal ideation, and the initiation of electroconvulsive therapy was recommended. The patient received 25 sessions of electroconvulsive therapy with partial improvement in the psychotic symptoms. His previous medication was changed to quetiapine 900 mg daily, without improvement. He was given a diagnosis of treatment-resistant schizophrenia, and clozapine was started. During hospitalization, the patient started refusing food, and a nasogastric tube was inserted. On one occasion, he became unwell, developing aspiration pneumonia () and spent 15 days in intensive care. As his medical condition subsequently improved, he said “I don't have parents, the people who raised me are impostors, they look like my parents, they will be angels, my parents buried me and I never knew about them.” In the neurological physical exam, the patient was alert, oriented to person and time. He mentioned he was in purgatory. The speech was normal. The muscle strength was 5/5 bilaterally, with no motor deficits. The sensation was intact bilaterally. Reflexes are 2+ bilaterally. Brain computerized tomography scan showed no intra- or extra-axial fluid, hemorrhage, or mass; the ventricular size was normal, and the grey-white differentiation was preserved (). The complete blood count, liver function test, creatinine, and glucose levels were within normal values. He later transferred back to the mental health hospital. The clozapine dose was gradually increased to 650 mg daily with an improvement of the auditory hallucinations, the suicidal ideation, and the symptoms of Cotard and Capgras syndrome. PANSS at the discharge was 51. A weekly normal absolute neutrophil count was obtained. The last WBC was 6500 cells/mcL and the ANC 4290 cells/mcL.


A 37-year-old male with unremarkable medical history presented with bilateral diminution of vision. The symptoms began three days prior to presentation when he was sleeping on a floor of a rural house (cemented floor, brick walls, and galvanized zinc roof). He recounted being woken briefly by a sudden appearance of a bright flash from a nearby lightning (approximately six meters away) during the rainy season of July 2018, following which he had loss of consciousness lasting for approximately 45 minutes. After regaining consciousness, he noticed blurring of vision in both eyes (BE), which improved initially but worsened afterward for which he sought ophthalmic consultation. He also noticed decreased hearing in the left side. On initial evaluation, his best-corrected visual acuity (BCVA) was 6/18 in the right eye (RE) and 6/60 in the left eye (LE). His ocular movement was preserved. The eyelids, ocular adnexa, and pupils were symmetrical, but there was relative afferent pupillary defect in LE. The rest of his anterior segment evaluation was within normal limits including absence of lenticular opacification but there was presence of 1+ anterior chamber (AC) cells in LE. Dilated fundus evaluation revealed a bilateral dull foveal reflex simulating a macular hole- (MH-) like appearance in BE (). However, the Watzke-Allen test result was negative. The intraocular pressure (IOP) was 14 mmHg in RE and 16 mmHg in LE. Spectral-domain optical coherence tomography (SD-OCT) (Topcon 3D OCT, 2000 series, Japan) demonstrated evidence of intraretinal cystoid abnormalities prominent in RE, subfoveal outer retinal hyporeflective space suggestive of foveal cyst in LE, and increased retinal pigment epithelium (RPE) hypereflectivity in BE (). He was treated with topical steroid and cycloplegic agent in BE.
He then presented four weeks later with slight improvement in BCVA of 6/12 in RE, but the vision in LE was static. The SD-OCT showed intraretinal cystoid changes in the middle retinal layers which was exaggerated compared to previous scan in RE (), and persistent foveal cyst appeared as hyporeflective lucency in the outer retinal layer just anterior to retinal pigment epithelium in LE (). He was then continued on topical steroid and cycloplegic agent for four more weeks.
After two months of injury, his BCVA improved to 6/9 in RE only and 6/60 in LE. Fundus examination revealed an interval development of central macular thickening, and the SD-OCT confirmed intraretinal cystoid changes with disruption of the subfoveal ellipsoid zone in RE. Similarly, the foveal cyst was continual in LE (). He was then prescribed topical nonsteroidal anti-inflammatory drugs in BE. He pursued his profession as a cook overseas and committed to seek medical advice in case of need.
He followed up with us at an interval of one and half-year following the injury upon his arrival. The documented BCVA was 6/9 RE and 6/60 LE. A mild cortical and posterior subcapsular cataract was evident on slit-lamp examination. Fundus examination showed dull foveal reflex (). The Watzke-Allen test was negative, but he still complained of mild distortion in the Amsler grid test of LE. OCT affirmed the restoration of foveal contour in RE (). Nonetheless, there was still presence of few intraretinal cystoid abnormalities in LE. The previous hyporeflective foveal cyst was absent, and further loss of the outer retinal layers was evident in the macula ().
His continual follow-up with us at the passage of two years postlightning injury (July 2020) revealed a BCVA of 6/9 RE and 6/60 LE. Early anterior and posterior subcapsular lenticular opacification was present during slit-lamp examination () and a dull foveal reflex in dilated fundus examination (). Automated Humphrey 24–2 Swedish Interactive Threshold Algorithm (SITA) standard visual field analysis revealed mild deficit extending from the blind spot in the left eye. OCT revealed normal restoration of foveal anatomy in RE (), whereas the intraretinal cystoid abnormality was almost absent but the nasal retina was grossly atrophic in LE ().


A 42-year-old male with no ocular and medical history was struck by lightning (June 2019) while on his way back home resulting in a loss of consciousness. Upon arousal after 20 minutes, he complained of pain and swelling of the right side of his face. He was then brought to the emergency department of a general hospital where the primary management of his condition was done, and the neuroimaging revealed no abnormality. He was then referred to us for the complaint of BE blurring of vision associated with pain and photophobia after two days. Upon evaluation, the BCVA was 6/24 in RE and 6/18 in LE. The ocular motility was full in all cardinal gazes, and no pupillary abnormality was noted bilaterally. Anterior segment examination showed mild conjunctival congestion in BE with presence of 2+ AC cells in RE and 1+ AC cells in LE. Dilated fundus examination revealed bilateral dull foveal reflex and bilateral central yellow-white lesion surrounded by orange-brown pigmentation in the macular region (). SD-OCT did not divulge any significant abnormality except for slight blunting of inner retinal layers more prominent in right (Figures and ). He was treated with topical steroid and cycloplegic agent for traumatic iritis.
Three weeks after the injury, the BCVA was similar to the vision at presentation, but the patient complained of marked distortion. Upon examination, the iritis has quietened. The Watzke-Allen test was negative, but the distortion of the lines in Amsler grid was more pronounced. OCT showed an increase in central macular thickness with loss of foveal contour, intraretinal cystoid abnormalities in the middle retinal layers, and disruption of ellipsoid zone. The changes were more marked in RE compared to LE (Figures and ). The patient was continued on topical steroid. On follow-up evaluation at five weeks postinjury, the BCVA had declined to 6/60 RE and 6/24 LE. Fundus examination showed interval development of macular edema BE, similarly OCT confirmed the increment in cystoid abnormalities more in the right (Figures and ).
At the end of two months, the BCVA was restored as of baseline to 6/24 in RE, whereas it improved to 6/12 in LE. The OCT of RE was analogous to the previous finding, but increment in central macular thickness was noted. There was slight restoration of foveal anatomy but still presence of subfoveal cystic changes in LE (Figures and ). He was advised to for topical nonsteroidal anti-inflammatory drugs BE. Serial OCT scans were obtained during his follow-up over the next two months. By the end of four months, the BCVA in RE was 6/24 and 6/12 in LE. Development of early posterior subcapsular cataract was evident during slit lamp examination. OCT demonstrated decreased intraretinal cystic changes in BE (Figures and ).
Five months after the event, the BCVA RE improved to 6/18 RE and was static to 6/12 LE. Dilated fundus examination revealed no other findings except for dull foveal reflex. Repeated OCT scan established interval restoration of foveal anatomy in RE. The outer layers of the retina could be distinguished, and there was resolution of intraretinal cystic abnormalities, but the foveal contour was not restored completely (). Similarly, in LE, there was persistent cystic space with restoration of outer layers of the retina (). The patient lost for follow-up afterward.


A 66-year-old Japanese male patient had received regorafenib (160 mg/day) as an anticancer treatment for metastatic colorectal carcinoma. His medical history included hypertension, diabetes mellitus, dyslipidemia, chronic heart failure, deep vein thrombosis (DVT), and permanent pacemaker implantation due to complete atrioventricular block. His DVT had been managed with low-dose apixaban (5 mg/day; body weight: 75 kg, creatinine: 1.0 mg/dL) to avoid bleeding complications.
Two months ago, he had suffered from non-ST-elevation myocardial infarction with the high-lateral (HL) branch as the culprit lesion, accompanied by chest discomfort; the HL branch occlusion was treated with balloon dilation only to avoid the need of a long-term triple antithrombotic therapy. At the same time, coronary angiography (CAG) revealed severe stenosis in the proximal left anterior descending (LAD) artery. A fractional flow reserve value of 0.72 confirmed a physiologically significant lesion. After PCI for the HL branch, regorafenib was withdrawn temporarily, and a triple antithrombotic therapy (aspirin: 100 mg/day, prasugrel: 3.75 mg/day, and apixaban: 5 mg/day) was successfully continued for two months without bleeding complications. Subsequently, the patient underwent PCI for severe stenosis in the LAD lesion two months after PCI for HL, as he demonstrated good tolerance to the triple antithrombotic therapy. Other medications administered included bisoprolol (1.25 mg/day), enalapril (2.5 mg/day), furosemide (20 mg/day), esomeprazole (20 mg/day), amlodipine (5 mg/day), sitagliptin (50 mg/day), mitiglinide (30 mg/day), and insulin degludec (9 units). In addition, routine blood testing revealed an elevated level of B-type natriuretic peptide (BNP), normal kidney function, high value of HbA1c, and mild anemia (BNP: 191.6 pg/mL, creatinine: 0.68 mg/dL, estimated glomerular filtration rate: 89.3 mL/minute/1.73 m2, HbA1c: 8.4%, hemoglobin: 9.9 g/dL, and platelet count: 318 × 103/µL). He was classified as having a high thrombotic and bleeding risk at this time, according to the CREDO-Kyoto thrombotic and bleeding risk scores. A sirolimus-eluting stent, which is a drug-eluting stent (DES) (Ultimaster Tansei 3.5/38 mm; Terumo Corporation, Tokyo, Japan), was implanted in the LAD lesion without malapposition or significant under-expansion, as confirmed by intravascular ultrasound imaging (IVUS) analysis (Fig. ). After PCI for the LAD, regorafenib was resumed because it exhibited a clinical effect on the patient's cancer. Nevertheless, the patient was admitted to our hospital for congestive heart failure with dyspnea one month later (BNP: 1,512.5 pg/mL). Echocardiography demonstrated new severe hypokinesis of the anteroseptal wall and apex. Despite continued triple antithrombotic therapy, CAG revealed focal and eccentric contrastive defects in the previous LAD stent, and OFDI confirmed ST (Fig. a–e). A 3.5/20 mm perfusion balloon (Ryusei, Kaneka Medical, Tokyo, Japan) was inflated for 3 min using an embolic protection device (Fig. f). CAG and OFDI confirmed optimal initial gain in the ST site and thrombolysis in myocardial infarction flow grade 3, with no complications including distal emboli (Fig. g–k). Antithrombotic therapy was further enhanced temporarily to prevent the recurrence of ST; it included aspirin (100 mg/day), ticagrelor (120 mg/day), and apixaban (10 mg/day). The administration of regorafenib was discontinued after PCI for ST in the LAD, and aspirin and ticagrelor were discontinued 1 and 2 months later, respectively. No further thromboembolic events (including definite/probable ST) were observed, and heart failure was managed well. However, he died due to the progression of metastatic colorectal carcinoma about 3 months after ST onset.


An asymptomatic 60-year-old woman was incidentally diagnosed with a twin oesophageal mass, which was suspected due to mediastinal widening on a routine chest radiograph. A CT of the thorax discovered the lesions with eccentric oesophageal wall thickening in the distal third of the oesophagus, causing narrowing of the lumen (Fig. ). Gastroscopy revealed an oesophageal submucosal protrusion with a smooth surface located 32 to 39 cm from the incisors, close to the Z line. The lesions were imaged as separate, 4 × 3 cm and 4 × 2 cm, inhomogeneous masses with some calcareous shadows arising from the muscularis propria on endoscopic ultrasound and were suspected to be leiomyomas because of their smooth surfaces and normal colours (Fig. ). According to the NCCN (National Comprehensive Cancer Network—NCCN) Task Force Report, a biopsy may not be necessary if the tumour is easily resectable and preoperative therapy is not required []. Thus FNA (fine needle aspiration—FNA) of the lesions was not performed. The results of her physical examination were normal.
Before the scheduled operation, the patient had a history of a soft diet and received a prophylactic dose of LMWH (low-molecular-weight heparin—LMWH) a day before surgery. Third-generation cephalosporin and metronidazol were administered one hour before incision; then laparoscopic-transhiatal surgery was performed. The procedure was done under general anaesthesia with the patient in a semilithotomy, 25° reverse Trendelenburg position. Three ports were inserted in the abdominal cavity along the left costal arch, with one port placed in the epigastrium on the right side and one port, 10–12 mm in diameter, inserted directly above the umbilicus (Fig. ). Slightly lower, intraabdominal pressure was set to 12 Hgmm to prevent pneumomediastinal side-effects. Laparoscopic exploration was done using a 30° camera. After retracting the left lobe of the liver, the peritoneum was divided up to the level of the median arcuate ligament with a Ligasure device (Valleylab, Boulder, CO, USA, Fig. ). A nasogastric tube (size 16 Fr.) was placed into the oesophagus to identify its anatomical patterns and to avoid oesophageal side injuries. After incision of the hiatus (Pinotti’s manoeuvre), the lower third of the oesophagus was mobilised anteriorly; then transhiatal exploration revealed that the masses located right under each other (oral and aboral tumours) and orally had spread high to the middle third of the oesophagus. From the cardia side, approximately 3 cm above it, the muscularis of the oesophagus was cut in the expected line of myotomy to expose the capsule of the aborally located tumour. A well-defined solid tumour was revealed with an intact capsule and rich blood supply. The lower pole of the aboral tumour along the capsule was gradually isolated and removed using fine dissecting instruments and a high-energy cutting device. The incision was extended until it reached the upper edge of the orally located tumour, allowing for exploration of the oesophageal mucosa and gradual isolation of the tumour outside the mucosa until complete tumour resection was achieved. Technically, we opened the oesophageal muscle layer approximately 7–8 cm long, started 3 cm from the cardia and finished intramediastinally. The anterior vagal branch was under close monitoring and retracted during the entire operation. The integrity of the mucosal membrane of the oesophagus was checked with intraoperative endoscopy; the leak test during air insufflation was negative (Fig. ). The oesophageal muscular wall was repaired by absorbable 3/0 monofilament sutures. Fundoplication was not added because the localisation of the duplex tumour was at least 3 cm above the cardia so the natural antireflux mechanisms were not compromised during surgery. On the other hand, the wrapped segment would have formed more distally from the sutured oesophageal wall. The specimens were removed in an endobag. The operation took approximately 120 min to complete (Fig. ). The tumours were approximately 38 × 29 × 27 mm and 37 × 20 × 30 mm in diameter and tough in quality, with a well-defined round shape (Fig. ). After the operation, the patient underwent water fasting, gastrointestinal decompression and acid suppression. Contrast radiography of the oesophagus on the fifth postoperative day showed intact smooth oesophageal mucosa with no obvious leakage, so the nasogastric tube was removed and the patient began to consume liquid food. She was discharged on the 10th postoperative day. She has been followed up for six months with a normal swallowing test, adequate oesophageal function and no complaints observed. A pathological examination confirmed the diagnosis of leiomyoma in both lesions. Immunohistochemistry resulted negative for DOG1 (discovered on GIST1—DOG1) and C-kit (CD117) and positive for SMA (smooth muscle actin—SMA) and desmin. The mitotic rate (Ki-67 index) was low. The specimen assessment showed complete removal of the two lesions, with no damage to the capsulae seen.


A 70-year-old male with a limited past medical history that was positive for stage I large B cell non-Hodgkin lymphoma diagnosed in 2012 but currently in remission after three cycles of chemotherapy presented to Sanford Medical Center Fargo for further evaluation and management of large left-sided temporal mass with evidence of secondary infection.
For over a year, he began to notice an ulcerating mass in the left temple, which slowly grew in size. He sought care from a naturopathic provider and maggot therapy was attempted. While on this treatment course, he had a mechanical fall and sought care in the emergency department. His vital signs were stable and was never febrile. He was noted to have a very large ulcerative and infiltrative soft tissue lesion in the left temple with evidence of secondary bacterial infection with associated maggot infestation (Figure ).
He was subsequently admitted for further management. White blood cell count was normal at 8.7 K/ul (reference range, 4-11) but inflammatory markers erythrocyte sedimentation rate and C-reactive protein were significantly elevated at 78 mm/hr (reference range, 0-15) and 107 mg/L (reference range, 0-8), respectively. Renal function was normal. He lives with his wife. He is retired and used to work on a variety of jobs including grounds keeping of a local school. He is a prior smoker and only drinks occasionally.
Otorhinolaryngology was consulted and performed tissue biopsy which revealed invasive squamous cell carcinoma. During his hospital course, he was noted to have a secondary bacterial infection of his left temple wound. Local cultures of the wound grew Staphylococcus aureus and Proteus mirabilis. Blood cultures grew W. chitiniclastica susceptible to quinolones, trimethoprim/sulfamethoxazole, cefepime, and piperacillin/tazobactam. He was able to be discharged after a week of hospitalization on oral levofloxacin. He eventually underwent extensive surgery including left total parotidectomy, facial nerve dissection, excision of the left cheek, scalp, including total auriculectomy, neck dissection, and left latissimus free tissue transfer with microvascular anastomosis followed by adjuvant radiation therapy which resulted in complete healing of the wound.


A 50-year-old woman with a history of HBV infection over 20 years presented with abdominal pain, hematemesis, hematochezia, and even syncope on December 29, 2018. The patient had no experience using antivirals for HBV or hepatotoxic drugs. She was diagnosed with GIST by gastroscopy and abdominal-pelvic computed tomography (CT) scan. At the time of diagnosis, the patient had a normal liver function. Serologic tests for HBV showed that the patient was positive for HBsAg, hepatitis B e-antibody (HBeAb), hepatitis B core antigen (HBcAg) but negative for hepatitis B e-antigen (HBeAg) and hepatitis B surface antibody (HBsAb). The HBV DNA level was 3.10 × 104 IU/mL (normal, <10 IU/mL). The patient underwent resection of two tumors with partial resection of the stomach on January 11, 2019. Postoperative pathology confirmed the diagnosis of two gastric GISTs [size, 10.5 cm × 7 cm × 6 cm and 4 cm × 2.5 cm × 2 cm; mitosis, both>5/50 high-power field (HPF)]. Immunohistochemistry indicated that the tumors were positive for CD34, CD117, Dog1, and Ki-67 (3%). Mutational analysis demonstrated a mutation in KIT exon 11. The patient was classified as high-risk under the modified National Institutes of Health (NIH) classification system (2008) (). To reduce the risk of tumor recurrence, administration of imatinib 400 mg daily was started postoperatively in January 2019.
On June 25, 2019, the patient presented with nausea, vomiting, and jaundice. Liver function tests showed elevated aspartate transaminase (AST) at 434.0 U/L (normal, 13.0–35.0 U/L), alanine transaminase (ALT) at 407.0 U/L (normal, 7.0–40.0 U/L), total bilirubin at 24.5 umol/L (normal, 1.7–17.1 umol/L), and direct bilirubin at 12.4 umol/L (normal, 0.0–6.8 umol/L). No change in serum HBV serology was indicated, and the HBV DNA level increased to 3.57 × 106 IU/mL. With the exclusion of other causes of liver injury such as infection with hepatitis A, C, or E virus, cytomegalovirus, Epstein-Barr virus, and autoimmune hepatitis, a diagnosis of imatinib-related chronic HBV reactivation was established. Thus, the patient immediately received entecavir (0.5 mg/d), discontinued imatinib, and took artificial extracorporeal liver support 3 times for severe liver damage. After 1 month, the symptoms and liver function abnormalities resolved. The HBV DNA level decreased to 6.59 × 102 IU/mL. After discharge, the patient continued to take entecavir (0.5 mg/d) (up to present). In January 2020, the patient resumed taking low-dose imatinib (100 mg/d), with doses increased to 200 mg/d in February 2020 and 300 mg/d from March up to present. Patient recovery was uneventful. In April 2020, her liver function subsequently remained within normal ranges, No HBV DNA was detected, and no evidence of GIST recurrence was found in the abdominal–pelvic CT scan.


A 59-year-old man without any symptoms was diagnosed with GIST by physical examination including gastroscopy in November 2018. He was diagnosed with HBV infection 20 years ago without undergoing antiviral therapy. After admission to the local hospital, he tested positive for HBsAg, HBeAb, and HBcAg but negative for HBsAb, HBeAg, hepatitis C virus, and normal liver function. He underwent laparoscopic complete stomach resection of the tumor with an uncomplicated postoperative course. The mass was diagnosed as a gastric GIST and categorized as high-risk in accordance with the modified NIH classification system (2008) (). (size, 7 cm × 5 cm × 4 cm mitosis, >5/50 HPF). Immunohistochemistry showed the following: CD117(+), CD34(+), Dog-1(+), and Ki-67 (<8%). Adjuvant treatment with imatinib (400 mg/d) was started on December 23, 2018.
In January 2019, the patient manifested abdominal pain. Laboratory and imaging tests showed normal results. The abdominal pain resolved after symptomatic treatment. In February 2019, the patient presented with edema of eyelids, hands, and ankles as side effects of imatinib. On May 15, 2019, the patient was admitted to a local hospital because of increasing systemic edema. Owing to serious side effects, imatinib administration was stopped. From November 2018 to May 2019, the patient received imatinib (400 mg/d), and his liver function was normal. No other hepatotoxic medication, except for imatinib, was taken.
However, after discontinuing imatinib, the patient reported dizziness, body weakness, and weight loss in June 2019. The patient was immediately admitted to Xiangya hospital. Laboratory findings showed the following measurements: ALT, 977.1 U/L (normal, 7.0–40.0 U/L); AST, 1382.0 U/L (normal, 13.0–35.0 U/L); total bilirubin, 20.5 umol/L (normal, 1.7–17.1 umol/L); and direct bilirubin, 11.3 umol/L (normal, 0.0–6.8 umol/L). Serology results indicated no change in serum HBV; moreover, HBV DNA was 6.11 × 107 IU/mL (normal < 10 IU/mL), and the tumor marker CA125 was 82.73U/mL (normal, 0–35.00 U/mL). However, positron emission tomography–computed tomography did not indicate recurrence or metastasis of GIST and other tumors. Thus, HBVr was identified as the potential cause of liver damage, and the patient was immediately administered entecavir (0.5 mg/d). However, the clinical condition of the patient progressively deteriorated because of severe hepatitis caused by HBVr. The patient ultimately developed severe liver failure leading to death.


A 51-year-old man was found to have multiple hepatic masses and a gastric mass after a physical examination in September 2019. The patient subsequently underwent radical surgical resection for the gastric mass and palliative resection for the hepatic masses in a local hospital. The patient had a history of HBV infection of over 10 years but received no antiviral therapy for HBV. During hospitalization, the patient showed normal liver function, tested positive for HBsAg, HBeAb, and HBcAb, and tested negative for HBsAb, HBcAb, and hepatitis C virus. Pathological examination revealed that the gastric mass was a high-risk gastric GIST (size, 4.5 cm × 3.5 cm × 3 cm; mitotic index>10/50 HPF), which stained positive for CD117, CD34, DOG1, and ki-67 (20%). The liver mass was a metastatic GIST (the maximum tumor diameter was 1.5 cm), which stained positive for DOG1; one lymph node was metastatic (1/1). Additional molecular analysis confirmed the mutation in KIT exon 11. The liver metastases were only partly removed, requiring lifetime treatment with imatinib (400 mg/d), which the patient started to receive in January 2020.
Regular laboratory examination after treatment with imatinib for 3 months showed that the patient had mildly elevated ALT, 80.0 U/L (normal, 9.0–50.0 U/L), but no symptoms and signs. Laboratory examination after treatment with imatinib for 6 months showed further increases in ALT to 282.6 U/L (normal, 9.0–50.0 U/L), AST to 154.0 U/L (normal, 15.0–40.0 U/L), total bilirubin to 19.9 umol/L (normal, 1.7–17.1 umol/L), and direct bilirubin to 10.5 umol/L (normal, 0.0–6.8 umol/L). HBV DNA was 4.99 × 108 IU/ml (normal, < 10 IU/ml). Serology testing results showed that HBsAg and HBcAb were positive, whereas HBsAb, HBeAb, and HBeAg were negative. After excluding other causes of hepatitis, we considered that hepatitis could be attributable to HBVr. Thus, entecavir (0.5 mg/d) was administered, and imatinib was discontinued immediately. The patient now was followed up under close observation.


A previously well 6-month-old black female infant weighing 6.5kg, with a history of fever in the past, and with no known underlying conditions were referred to Agogo Presbyterian Hospital (APH) in the Asante Akim North District of the Ashanti region after having presented to a peripheral facility with 3 days of fever, diarrhea, and cough as reported by her mother. The diarrhea was watery and mucoid but non-bloody and was associated with episodes of non-projectile cough and vomiting. The infant was treated with homemade oral herbal preparations without improvement.
Upon presentation at the first point of call health facility, the child was managed with parenteral antibiotics, including intravenous cefuroxime, gentamicin and oral dispersible zinc tablets in addition to IV fluids according to the referral note. The child was subsequently referred to APH on account of her deteriorating health condition. Per the referral note, we were unable to ascertain if any laboratory tests or antimalarial treatment was initiated.
On examination, the child appeared ill, but was not febrile, with an axillary temperature of 36°C, pale, not jaundiced and adequately hydrated. Further examination focusing on the central nervous system found the child to be lethargic, prostrated (loss of neck control since the onset of illness) and with a Blantyre Coma Score (BCS) of 4/5 (Eye-opening E score 1/1; Motor score 1/2, and Vocal score 2/2). The patient was tachycardic with a heart rate of 146 beats per minute (bpm). The child was in severe respiratory distress with tachypnoea at rest (respiratory rate: 46 cycles per minute), grunting, nasal flaring and severe intercostal recession. Chest auscultation revealed adequate air entry with bilateral bronchial breath sounds. A rapid diagnostic test for malaria (Care Start Malaria, ACCESS BIO, Somerset, USA) was positive. The full blood count revealed moderate anemia with a hemoglobin concentration of 8.3 g/dL, leukocytosis (24.500/μL; neutrophils: 73.2%) and thrombocytosis (485,000/μL). Malaria microscopy was positive for P. falciparum with a parasitemia of 224 parasites/µL. Based on these findings the admission diagnosis was severe malaria with prostration and severe bronchopneumonia.
The patient was started on parenteral cefotaxime at 300 mg four times daily and 30 mg of gentamicin. Later on, the same day of admission, IV clindamycin at 40 mg four times daily for 4 days was administered. IV Artesunate was also started at 20 mg daily at time 0, at 12 h and 24 h per standard recommended treatment in addition to supportive care, being paracetamol when needed for pyrexia and supplemental oxygen for the respiratory distress. The patient was judged to be at a high risk of aspiration and was kept nil-by-mouth and maintained on IV fluids.
By day 2 of admission, the BCS had dropped to 2/5 (M-1, V-1, E-0). Her chest findings had not changed significantly. The same day, a diagnosis of cerebral malaria was included in her records. Treatment was continued and urine output monitored. The patient was started on a minimal enteral feed of 5 mL every 2 h. After 12 h the clinical state improved, with a decreased respiratory rate and Heart rate of 38 cpm and 126 bpm respectively, no signs of respiratory distress and an increasing BCS of 3/5 (E-1, M-1, V-1), which improved further three days into her admission to 5/5 (E-2, M-2, V-1). Her urine output was found to be less than 100 mL over the 24 h but improved following furosemide challenge.


A 20-year-old female presented to our outpatient department complaining of intractable “clicking” tinnitus for the last two years. She had been in her usual state of health two years back when she had experienced an upper respiratory tract infection (URTI) for which she had taken antibiotics for one week, which had led to the resolution of the infection. One week after the resolution of her symptoms and discontinuation of antibiotics, she had developed clicking in both ears. She reported her symptoms to be persistent and bilateral (left > right). She denied any history of ongoing headache, blurring of vision, dysphagia, dysarthria, fever, weight loss, or any other neurological or auditory symptoms.
An otologic examination revealed normal tympanic membranes and hearing was normal on audiological testing (pure tone and impedance audiometry). There were continuous, involuntary rhythmic contractions of the soft palate (Video ), and the physician could appreciate the clicking sounds, which were asynchronous with the pulse. The lips, tongue, larynx, and diaphragm were not involved. The rest of the examination was unremarkable.
She had no significant past medical or family history. The contractions persisted during phonation and sleep. Her tinnitus was refractory to treatment with various antidepressants, antiepileptics, skeletal muscle relaxants, and physiotherapy. She underwent an MRI of the brain, which revealed no notable lesions. She has been advised to undergo a trial of botulinum toxin therapy and is currently awaiting the procedure.


A 10-year-old boy was referred to our center with a painful right elbow. The patient and his family had no considerable medical history. The patient had no history of elbow trauma and surgery, as well. In clinical examination, an asymmetric enlargement was noticed on the right elbow. In physical examination, the elbow range of motion was restricted so that a flexion-extension motion arc of 30–90° and 15° of pronation to 20° of supination was recorded.
The findings on the plain radiograph (Fig. a) and computed tomography (CT) scan (Fig. b) revealed an irregular mass with focal ossification at the epiphyseal section of the proximal radius. Magnetic resonance imaging (MRI) demonstrated an epiphyseal osteocartilaginous lesion originating in the radial head epiphysis (Fig. c). The annular ligament could not be seen clearly in preoperative MRI. With suspecting a diagnosis of DEH, the decision was made for resecting the lesion surgically. Under general anesthesia, in a supine position, and through the anterior-lateral approach to the radial head, the lesion was resected and sent to the pathology department for further evaluation. The remnant of the annular ligament was seen intraoperatively and resected.
The histologic evaluation of the excised lesion revealed clusters of chondrocyte arranged in a fibrillary matrix, a thick cartilaginous cap, and ossification centers, which were consistent with the diagnosis of DEH (Fig. ).
The patient’s arm was supported in a swathe and sling for 2 weeks. The full range of motion exercises and physical therapy was administered two times a week. The first visit to the patient was 2 weeks after the surgery. The patient had no pain, and the elbow range of motion was near to normal (flexion-extension: 0–130°, pronation: 50°, supination: 50°). At the six-month follow-up, the patient was still pain-free and had a full range of elbow movements. No sign of recurrence was noticed in the follow-up radiographs. Secondary ossification or growth arrest of the forearm bones was not seen either (Fig. ).


A 61-year-old woman with a history of dyslipidemia presented in the emergency room with recurrent vomiting. Her blood pressure was 62/37 mm Hg on arrival, and she was admitted to our critical care center. She had only mild abdominal pain despite abdominal distension. Her face was pale, and there was weak palpation of the radial artery. The patient's hemoglobin level at admission was 12.4 g/dL. A venous sheath was inserted in the left femoral vein, and rapid infusion by crystalloid fluid of approximately 1000 mL was initiated in the resuscitation room. Enhanced computed tomography (CT) scan was performed after confirming increased blood pressure; this revealed a ruptured PDA aneurysm and celiac axis stenosis that was compressed by the median arcuate ligament (Figure ). We decided to perform transarterial embolization (TAE) as an endovascular treatment. The patient was anesthetized and underwent oral intubation followed by transfer to the emergency angiography room.
The patient's blood pressure suddenly dropped, and she developed hemodynamic shock. Her hemoglobin level dropped to 7.3 g/dL. It was difficult to secure the right femoral artery because the patient was obese. The pulse was weak, and the artery had collapsed as a result of the hemorrhagic shock. After several times to puncture the artery, she developed a subcutaneous hematoma and local arterial dissection was suspected. We attempted to access the left femoral artery; however, this too was difficult to secure. The blood pressure declined gradually, and prompt management to control the hemodynamics became necessary. We decided that the treatment was difficult with interventional radiology and it was necessary to switch to surgical treatment. We also believed that performing REBOA promptly would be more important to maintain the hemodynamics until a surgical intervention can be performed. Surgical exposure of the left femoral artery was performed to insert an aortic balloon occlusion catheter.
Celiac artery arteriography was performed to confirm the point of rupture before inserting the balloon catheter (Figure ). The position of the aortic balloon was confirmed using fluoroscopy, and inflation of the balloon resulted in increased blood pressure. Hemodynamics were stabilized, and the patient was moved to an operating room.
Laparotomy revealed a massive retroperitoneal hematoma surrounding the duodenum. Intraoperative bleeding was controlled by REBOA; therefore, the bleeding did not hinder the surgery. The point of bleeding was determined to be the point of rupture that was revealed by collapsing the balloon. The rupture point was confirmed, and both ends of the aneurysm were secured and ligated.
Although there were other visceral aneurysms visualized on the CT scan, we decided against performing any additional interventions since saving life was the priority.
Hemoglobin levels decreased from 12.4 g/dL to 5.5 g/dL. She required 8400 mL of red cell concentrate, 7920 mL of fresh frozen plasma, and 600 mL of platelet concentrate. After intensive care was performed, the patient was extubated on day 8.
A second surgery for incision of the median arcuate ligament and removal of the remaining aneurysm was performed as an elective surgery on day 12.
Finally, the patient was discharged on day 22. She was followed up at the hospital for 6 months. No complications were observed, and no repeat interventions were required.


A 20-year-old German Caucasian female was admitted to the ED with complaints of dyspnea at rest and anterior thoracic pain. These symptoms started during a commercial flight, initially with enough severity to justify assistance in the airport after landing and emergent transportation to our hospital. She had no previous medical history except for an episode of bronchiolitis during childhood. No smoking, alcoholic or drug abuse habits were reported. On admission, the patient was already asymptomatic. Blood pressure, cardiac frequency and peripheral oxygen saturation were normal without supplemental therapy. On physical examination, a thoracic asymmetry was visible. Breath sounds were abolished in the left hemithorax and hyperresonance was noted at thoracic percussion. Laboratory evaluation including complete blood count, C-reactive protein, D-dimers, creatine kinase, lactate dehydrogenase, troponin and arterial blood gas test (ABG) were unremarkable. Chest X-ray showed an hyperlucent left lung which was initially suspected to be an hypertensive pneumothorax (Figure ).
Complete remission of symptoms, absence of hemodynamic instability, and ABG findings were, however, not suggestive of that hypothesis. A thoracic computed tomography (CT) was performed and described an area of hyperinflation occupying the upper two-thirds of the left lung (23 cm in the longest axis) with scarce vasculature, causing contralateral mediastinal shift and compressive atelectasis of the remaining pulmonary parenchyma, suggestive of congenital lobar emphysema (Figures , ).
Based on clinical presentation and radiologic findings, a diagnosis of CLE was established and the patient was admitted to the medical ward for clinical surveillance. Afterwards, in the absence of symptoms that would justify immediate surgical management, a conservative approach was chosen.


Fifty-three-year-old obese male who had a single-port laparoscopic gastric band placement performed 10 years prior. The band eroded through the skin and was surgically removed but closure was complicated by secondary dehiscence. At the time of intervention, the patient had a non-healing abdominal wound for 11 months, a more recent revision surgery (5 months prior) had also failed with a dehisced surgical site. Previous management of the defect was carried out via alginate dressings, cadexmer iodine, and saline gauze. The defect was causing moderate pain, but was clean with no evidence of infection and hypertrophic granulation tissue was present (). Surgical incision was made at the defect margins, through subcutaneous adipose tissue and down to the fascia (). An ECM graft (Myriad™, “Thick”) was trimmed to fit the defect and placed onto the fascia (). Due to the depth of the defect and potential for dead space, second and third layers of ECM graft were prepared and placed in the defect in a layered arrangement. The defect was then closed by flap advancement with subcutaneous running suture with JP drain placed and incisional NPWT initiated. After seven days, cutaneous tissues demonstrated good apposition and tissues exhibited no inflammation (), the NPWT and drain were removed and patient was discharged with instruction to wear abdominal binder and 6 weeks heavy lifting/strenuous activity restriction. At 6 months, the site remained closed with no dehiscence or other complications.


Seventy-three-year-old female who was non-ambulatory secondary to Parkinson's disease, Lupus, and rheumatoid arthritis. Sacral pressure injury had been present for 7 years and had been previously managed with medical honey and enzymatic debridement. The patient had undergone a reconstructive procedure 1 month prior, using excision of the defect and flap coverage. The flap had subsequently dehisced and while there were no signs of infection, the defect was chronically inflamed (). Defect was excised with side margins down to the coccyx, with a partial ostectomy (). An ECM graft (Myriad™, “Thick”) was trimmed to fit the defect and into the base of the defect and covering the bone protrusion of the coccyx (). The excision was closed via a flap advancement with suture used to the close the primary incision line (). JP drains were placed and incisional NPWT initiated. A dehiscence of the site developed, which subsequently went onto heal via secondary intention at 3 months. At 6 months the defect remained healed.


Twenty-six-year-old female with a 13-month-old non-healing full thickness defect following open reduction and internal fixation surgery of the left ankle (). Orthopedic hardware remained in place, and the wound was culture negative. The defect had previously been managed through use of alginate dressings. Surgical excision of proximal chronic tissues was carried out (). Trimmed ECM graft (Myriad™ “Thin”) was placed contacting the tissues of the defect bed and allowed to rehydrate in situ via absorption of blood components (). The defect was closed by flap advancement () and underwent incisional NPWT for seven days. The defect exhibited signs of infection and secondary dehiscence 1-week post-surgery (). Additional ECM graft material was packed in the dehiscence cavity () and covered with a gentian violet/methylene blue foam secondary dressing. Over the following weeks, the defect demonstrated marked improvement with healthy granulation tissue development (). Therefore, application of additional ECM graft material, hydrolyzed collagen dressings covered with a non-adherent dressing layer and continued use of gentian violet/methylene blue secondary dressings were used to heal the defect via secondary intention. Successful closure of the defect by secondary intention was achieved 8 weeks after the initial flap surgery ().