A 40-year-old male patient under evaluation for pulmonary complaints was referred to the radiology department for CT thorax study. In addition to infective pulmonary lesions, there were unrelated abnormalities in bones as well.
CT thorax study revealed diffuse osteosclerosis, anterior longitudinal ligament (ALL) ossification, syndesmophytes, vertebral facet joint arthropathy, and ankylosis, along with the variable extent of arthropathy and ankylosis at costovertebral and costotransverse joints. The patient also had hypertrophic arthropathy at the bilateral sternoclavicular joint. Smooth periosteal thickening was noted in some of the ribs. In view of these findings, a CT study of the abdominopelvic region/lumbosacral spine was done, which revealed a similar pattern in the lumbar spine. Additionally, involvement of the bilateral sacroiliac (SI) joints with ankylosis was also seen. Moreover, there were changes consistent with chronic arthropathy at the left hip joint with joint space reduction and subchondral cysts, and hyperostosis along the acetabular margins.
The patient was then questioned about any symptoms of muscle or limb or joint pains. He then volunteered about on going backache for many years. In addition, there was a history of a single, episode of acute left hip pain many years back for which he was managed conservatively. The patient in his current status had a mild limp along with dull backache. The patient also had bilateral plantar pustules. However, there was no palmar pustulosis and no acne.
Laboratory findings were nonspecific with raised ESR and WBC count. The Patient had an active pulmonary infection in the right lung and respiratory complaints, which were the primary reason for his medical consultation.
In view of the predominant sclerotic and hyperostosis pattern of bone and joint disease, and the presence of plantar pustulosis, a diagnosis of SAPHO was arrived at.


A 65-year-old male presented with persistent hiccups for 4 days and 26 kg weight loss in 4 months. His past medical history and family history were both unremarkable. Relevant negative symptoms included abdominal discomfort, fevers and night sweats. A diagnosis of haemochromatosis had been made in the community based on a raised ferritin and heterozygosity for the H63D gene. He was venesected twice prior to this presentation.
Physical examination revealed a tall gentleman with blood pressure of 104/75, heart rate of 90 bpm and weight of 89 kg. Bilateral flank masses were palpated but no lymphadenopathy was identified. Admission blood tests were indicative of impaired renal function, hyperkalaemia and hyponatraemia (). An adrenocorticotropic hormone (ACTH) stimulation test confirmed adrenal insufficiency.
Computed tomography (CT) chest, abdomen and pelvis revealed large bilateral suprarenal masses measuring 135 × 125 mm on the left 115 × 85 mm on the right ( and ) with several sub centimetre retroperitoneal lymph nodes. Associated visceral invasion was noted with displacement of the renal vasculature and inferior vena cava (IVC) on the left and encasement of the left renal hilum, left renal vasculature and proximal ureter on the right. Posteromedially, the left lesion was not separable from the diaphragmatic crus. No nodal disease was identified above the diaphragm. Positron emission tomography (PET) scan confirmed fludeoxyglucose (FDG) avidity of these masses with an additional FDG-avid right-sided para-aortic lymph node (). Lumbar puncture and MRI brain and whole spine excluded CNS involvement.
Following the exclusion of pheochromocytoma biochemically, to avoid insurgence of catecholamine and hypertensive crisis following biopsy, ultrasound biopsy of the right adrenal gland was undertaken. This demonstrated a diffuse large B-cell lymphoma of activated type (). Concurrent flow cytometry on the adrenal sample revealed a monoclonal B-cell population with CD5/CD10 negativity consistent with B-cell lymphoma. Immunohistochemical testing demonstrated positivity for B-cell lymphoma 2 (BCL-2), patchy positivity for BCL-6 and negative for C-MYK (). Ki-67 was estimated at 90% exhibiting a very high mitotic rate (). A bone marrow biopsy was not performed as haemophagocytic lymphohistiocytosis was felt unlikely given an unremarkable full blood count and a ferritin count of 1936 μg/L.
Initially, chlorpromazine was used for symptomatic control of hiccups with mild effect. Intravenous and subsequent oral hydrocortisone was commenced for primary adrenal insufficiency with clinical and biochemical improvement. Fludrocortisone was added at a later stage. Full resolution of hiccups was observed within 24 h of commencing corticosteroid therapy.
After three of six cycles of R-CHOP-21 chemotherapy, PET imaging has demonstrated significant interval regression of bilateral masses (70 × 76 × 50 mm on the right and 131 × 92 × 46 mm on the left). There has been no further recurrence of hiccups and renal function has since normalized.


An 85-year-old man was brought to emergency department following a fall at home while attempting to change his incontinence pad. He sustained no injuries from the fall; however, he reported a background of several days of worsening lethargy, a productive cough and decreased oral intake. His medical history was complex and included transfusion-dependent chronic myelomonocytic leukaemia type 1 (CMML-1) (see baseline results in ), type II diabetes mellitus, atrial fibrillation with a permanent pacemaker, chronic kidney disease stage III (baseline creatinine 100–120 μmol/L), hypertension and a permanent suprapubic catheter due to neurogenic bladder and benign prostatic hypertrophy.
His medical history also included restless leg syndrome, duodenal ulcers, hypercholesterolaemia, renal calculi, pyelonephritis, infective endocarditis of the aortic valve, non-ST elevation myocardial infarction, polymyalgic rheumatoid arthritis and chronic obstructive pulmonary disease.
His chronic treatment consisted of irbesartan, digoxin, pantoprazole, bisoprolol, apixaban, sulfasalazine, pramipexole, domperidone, atorvastatin, cholecalciferol, mixed insulin, paracetamol and lifelong dicloxacillin (1 g two times per day for the last 10 months) for suppression of infective endocarditis.
He had a low-grade fever (37.9°C), blood pressure of 131/47 mm Hg, heart rate of 70 bpm, respiratory rate of 21 and oxygen saturation of 93%. Clinical examination was notable only for some crackles at the lung bases bilaterally and massive splenomegaly (first noted 2 months earlier).


A 64-year-old male patient presented with hematuria and underwent three transurethral resection of bladder tumors (TURBTs) between November 4, 2014, and March 18, 2015. The pathological diagnosis was bladder adenocarcinoma (), with immunohistochemistry (IHC) showing CK7 (+), CK20 (+), CDX2 (+), GATA-3 (±), LI-cad (+), PSA (-), P63 (-), P53 (+), HER2 (3+), and the Ki-67 proliferation index was 80% (). The initial clinical stage was stage I (T1N0M0), and repeated TURBTs were carried out without any residual tumor identified. This patient received regular intravesical chemotherapy with gemcitabine after TURBTs.
In October 2015, two new lesions in the lungs were discovered by surveillance CT scans and were confirmed as metastatic adenocarcinoma on further biopsy. Four cycles of paclitaxel (135mg/m2 every 3 weeks) and oxaliplatin (130mg/m2 every 3 weeks) were administered beginning on November 17, 2015, and stable disease (SD) was achieved. Radiation therapy for the two lesions, with a total dose of 48 Gy (BED=86.4 Gy), was delivered concomitantly with chemotherapy (paclitaxel and oxaliplatin) from January 1, 2016, to April 16, 2016. This treatment was previously found to be well-tolerated, with mild adverse events, including grade 1 transaminitis and grade 1 thrombocytopenia. Then, the CT scan showed a PR, and capecitabine was given to the patient orally beginning on April 30, 2016. To determine potential treatment opportunities, NGS of the primary bladder tumor was conducted on March 23, 2016, and the results showed that the HER2 gene was amplified approximately 7 times, with an S310F mutant abundance of 90% ().
Lung lesion progression was identified by a CT scan on August 8, 2016, and apatinib was administered at a dose of 500 mg every day. The best response to apatinib was PR, and progression-free survival (PFS) reached as long as 20 months. The CT scan revealed progressed disease (PD), with new lesions identified on March 30, 2018 (). Then, fourth-line treatment with trastuzumab and albumin-bound paclitaxel was administered beginning on April 11, 2018. The regimen comprised trastuzumab (8 mg/kg first dose and then 6 mg/kg every 3 weeks) and albumin-bound paclitaxel (250 mg/m2 every 3 weeks). A PR was achieved after two cycles of treatment () according to the Response Evaluation Criteria in Solid Tumors (RECIST) (version 1.1) and confirmed two months later. Trastuzumab-based therapy was continued for 8 cycles until CT scans revealed enlargement of the lesions (), with a PFS period of 6 months. During the treatment, he periodically performed echocardiography (every 3‑6 months) to monitor the potential cardiotoxicity of the biological drug but no sign of cardiomyopathy was revealed and the ejection fraction maintained an excellent value (55–65%). No signs of adverse events were identified except for mild thrombocytopenia and decreased hemoglobin (both are grade 0). Then, a rebiopsy of a pulmonary metastatic lesion was carried out. Pathological and histopathological examination revealed metastasis of bladder adenocarcinoma (), with IHC showing HER2 (2+) (). NGS was conducted again on October 25, 2018. The results showed no amplification of HER2, and the S310F mutant abundance was reduced to 27.9% (). The gene correlation analysis is shown in .
This study was approved by the Medical Ethics Committee of Drum Tower Hospital. Written informed consent was also provided by the patient for publishing the case details and accompanying images in the case study.


A 37-year-old female patient started to have low back pain since 11 years ago. She denied any trauma history. The pain bothered her life every day and exacerbated in long time standing. She had ever received several times of physical therapies, facet joints injections, and acupunctures but the pain did not improve. There was no radiation, numbness, weakness or reflex abnormality. On physical examination, posterior to anterior compression over the L5-S1 interspinous area provoked deep back pain, and her pain became worse in extension. The magnetic resonance imaging (MRI) revealed a high-intensity zone (HIZ) at the posterior L5-S1 IVD which indicated a posterior annular tear (). We performed PRP (ACP double syringe system, Arthrex, Germany) injection (3 mL) to the L5-S1 intervertebral disc (IVD) under US guidance during which the concordant pain was produced (–, ). No procedure-related complications were reported. Three months later, the visual analogue scale (VAS) improved from 8 to 2 and she became more active in physical activities.


A 36-year-old female patient suffered from chronic low back pain for three years. She was a skateboarder and had ever fallen on the ground for numerous times during skateboarding. She had most obvious pain and stiffness of her low back when she got up in the morning or after a period of immobilization. The pain was centrally located without peripheral radiation. There was no numbness, weakness or reflex abnormality. On physical examination, posterior to anterior compression at the L4-5 and L5-S1 interspinous area provoked deep back pain. The MRI revealed an HIZ at the posterior L4/5 and L5-S1 IVD, respectively (). PRP (ACP double syringe system, Arthrex, Germany) injection (3 mL) under US guidance was performed to the IVDs which produced the concordant pain ( and , ). The patient had no procedure-related complications. One month later, she reported significant improvement without pain or stiffness when she got up in the morning (VAS improved from 7 to 1).


A 17-year-old female presented with painful nonhealing ulcer over a large scar on the left leg since 1 year. The ulcer did not improve on treatment with various topical and oral medications. The lesions started with formation of red blister, which doubled in size within 2 days and ruptured to form an ulcer within 1 week period. It took about a year to heal forming a large hypertrophic scar. After around 1 year, she developed another ulcer over the scar, which progressed to the present size over 1 month. There was no history of any prior trauma, abnormal bowel habit, joint pain, or frequent bleeding from the ulcer. On examination, there was a well-defined ulcer of size 11.5 cm × 6.7 cm over a hypertrophic scar (20 cm × 8 cm) over the left lower leg []. The base of ulcer had red granulation tissue. The margin was pigmented with undermining at some portions and punched out edge. The healed scar had resulted in flexion deformity at the ankle joint []. The examination of other parts of skin was normal. The site of Bacille Calmette Guerin injection had healed without hypertrophic scar. Provisional diagnosis of PG, Marjolin ulcer and posttraumatic eczema was made. Biopsy from edge of ulcer was done for histopathological examination. All routine investigations, such as complete blood count, peripheral smear comments, were within normal limits. Pus on Gram stain and culture sensitivity did not show any organism. Biopsy showed subcorneal neutrophilic pustules with dermis showing neutrophilic infiltration [Figures -]. The upper and mid dermis showed fibrillar collagen in orderly arrangement [Figures and ]. Based on history, clinical morphology and histopathology a final diagnosis of PG over a healed hypertrophic scar was made. The patient was treated with dexamethasone pulse (100 mg dexamethasone in 300 mL 5% dextrose infused over 2 hours) on 3 consecutive days with 30 mg oral prednisolone and 100 mg dapsone daily. There were signs of improvement in the form of decrease size and re-epithelization of the ulcer characterized by decreased depth of ulcer over 2 weeks with slopping of the edge. After seven doses of monthly dexamethasone pulse and oral prednisolone in tapering doses and dapsone 100 mg, the ulcer healed completely [Figure and ]. The patient is on regular follow-up.


A 27-year-old male labeled by an outside institution as a case of Ehlers-Danlos syndrome since the age of 10 years was referred to the thrombosis clinic at our tertiary care center. He presented with right lower extremity varicose veins, multiple lipomas and hemihypertrophy, recurrent unprovoked (ie, not associated with an environmental risk factor) pulmonary embolism and deep vein thrombosis. Upon further questioning, the mother stated that she started to notice asymmetry of the limbs at the age of 4.
On physical examination, the cardiac and respiratory exams were normal. The patient had a high arched palate, small fused tongue, and hyperelastic skin and joints all over. In addition, there was an anterior abdominal mass (CT abdomen finding was “right-sided hypertrophy of the subcutaneous fat”). During the musculoskeletal exam, hypertrophy of left-hand middle finger was noted. The lower limb showed length discrepancy with bilateral genu valgum deformity, right leg hemihypertrophy and prominent varicose veins with multiple lipomas ().
Laboratory examinations were negative for hereditary and acquired thrombophilia and autoimmune investigations (including Anticardiolipin screen IgG, IgM, IgA, B2 - Glycoprotein I IgG, IgM, IgA, Protein C and Protein S, Antithrombin III, Lupus anticoagulation - La1, Factor V Leiden (APCR), Prothrombin gene mutation G20210A). Doppler Ultrasound of right lower limb showed dilatation of the main great saphenous vein measuring 1.7 cm at leg level, the femoral and popliteal veins were aneurysmal but patent; there was detectable reflux in distal popliteal and tibial veins secondary to incompetent valves, and there was no evidence of arteriovenous malformation or deep vein thrombosis. In the skeletal survey, the patient was found to have a gentle levoscoliosis of the lumbar spine, and pelvis was tilted and rotated left. There was bowing of the femoral shaft with bilateral coxa valga deformity, and asymmetrical bowing of tibia and fibula bilaterally with limb length discrepancy. There was focal gigantism involved with the left-hand middle finger phalanges and left big toe. MRI brain showed small T2 abnormal signal in the right frontal white matter superolateral to the head of caudate nucleus suggested of cavernous venous malformation ( and ). There was no hemi-megalocephaly on MRI and there was no evidence of arteriovenous malformation (AVM).
During outpatient follow-up and therapeutic anticoagulant for unprovoked pulmonary embolism, the patient was complaining of per rectum bleeding. Colonoscopy showed a single solitary ulcer in the rectum with sigmoid and rectal polyps which were resected. Biopsy revealed hyperplastic polyps and mucosal prolapse syndrome with no dysplasia ().
A genetic investigation was performed in Mayo Clinic laboratories in Rochester Main Campus. The interpretation revealed several large regions of homozygosity encompassing at least 6% of the genome with no clinical significant copy number changes were identified.
The most likely diagnosis is Klippel-Trenaunay syndrome based on the presence of extensive lower limb varicosities, soft tissue hypertrophy with bone deformities, central nervous system arteriovenous malformation, and recurrent venous thromboembolism.


A 75-year-old female was referred to the outpatient dermatology clinic for management of angioedema. She presented with a diffusely swollen, firm, erythematous facial plaque that developed suddenly and persisted for 1 year. There were no other associated systemic symptoms or inciting event. There were mild fluctuations in the facial swelling, although it never fully resolved. She had undergone some initial investigations initiated by her family physician and allergologist, and was treated for angioedema without success. Multiple allergy tests were unremarkable. Chest X-ray had demonstrated normal heart size, unremarkable mediastinum, and no evidence of mediastinal mass or vascular dilation.
Past history included controlled type 2 diabetes and dyslipidemia. There was no previous history of malignancy or prior dermatology conditions. Medications included metformin, insulin, and atorvastatin. Family history included both parents with unspecified cancers. There were significant second-hand smoke exposures from both her parents and her partner.
Examination revealed a diffusely edematous facial plaque that was firm to palpation and associated with erythema that was continuous throughout including the nasolabial folds (). On further evaluation, she was found to have prominent and dilated veins on the ventral chest and neck (), as well as enlarged tortuous veins on her abdomen (). There was no extremity edema or erythema.
Based on history and physical examination, a diagnosis of angioedema was unlikely. The differential diagnosis included solid facial edema (Morbihan’s syndrome) and various infiltrates such as lupus, lymphoma, mucinosis, amyloidosis, myxedema, leprosy, and sarcoidosis. The prominent vascular findings taken in context with the facial features led to a vena cava syndrome being considered.
Biopsy of the facial skin reported benign solar elastosis, with no other significant pathologic abnormality, therefore helping to rule out Morbihan’s syndrome and infiltrative etiologies. Blood work including antinuclear antibody with serum and urine protein electrophoresis was normal. An urgently ordered computed tomography (CT) of the chest revealed complete obstruction of SVC due to thrombosis, with multiple collateral vessels throughout chest and mediastinum (). There was no evidence of intraluminal airway mass, pulmonary parenchymal abnormality, nor pleural thickening or effusions. In addition, no axillary, mammary, or cardiophrenic lymphadenopathies were identified.
The patient was admitted to hospital for anticoagulation therapy and workup to determine the thrombus etiology. Investigations did not uncover any malignancy or other cause for the thrombus. International normalized ratio (INR) was 1.0 and partial thromboplastin time of 24.9 (range 25–38) was within normal limits. The D-dimer level was elevated at 1.54 (normal range below 0.49). Within 1 month of being seen in the outpatient dermatology clinic, her condition had progressed to include a swollen left arm with pitting edema and the facial and neck swelling persisted. An ultrasound venous Doppler showed superficial thrombophlebitis of the basilic vein, but no evidence of deep vein thrombosis. Despite to date there being no known evidence of malignancy or intrathoracic mass, the former remains the most likely underlying cause. Radiology and thoracic surgery consultation agreed that an undiagnosed lung malignancy, including lymphoma, was the most likely etiology due to the patient’s age and obstruction location. Covert neck or breast malignancy remains possibilities. Mass lesions such as esophageal cancer, thyroid goiter, Castleman’s disease, lymphadenopathy secondary to cystic fibrosis, restrictive pericarditis, histoplasmosis, and syphilitic or tuberculotic aneurysm can rarely manifest as SVC syndrome. Repeat imaging and clinical exam helped exclude these mass lesions. The patient will be monitored longitudinally with further imaging and thoracic surgery follow-up.


A 23 years old female patient was consulted to neurology department with left hemifacial atrophy with the involvement of the zygomatic area extending to the left jaw, mouth, and ipsilateral temporo-parietal area (). The patient did not have any other significant medical or traumatic history in the past, and there were no history of dental procedures or infections either. The onset of the manifestations was at the age of eight with hyperpigmentation in the left zygomatic region and left hemifacial atrophy, and it resulted in severe bone, fat tissue and muscle atrophy of the aforementioned regions after a steady progression during the following 2 years. The disease first developed since the patient was at 8 years old. The diagnosis of PRS was made at the age of 8 by a skin biopsy. Until then, the patient had not received any medications related to this.
In addition to the left hemifacial atrophy, the patient ( and ) was characterized by atrophy of the ipsilateral upper limb with hypoplasia of the fifth finger of the left hand, left enophthalmos () as well as eyelid retraction without involvement of the eye movements. Additional cutaneous manifestations included linear scleroderma and bandlike alopecia (). Regarding dental manifestations, the patient presented with radicular resorption of dental organs within the left superior hemiarch without headache or arthralgia. The laboratory tests including complete autoimmune panel showed no abnormal results. Since 2006, the patient was performed seven surgeries for multiple muscle and fat grafting.
The patient was referred to the neuro-epilepsy clinic for her neurological manifestations, which included seizures that initiated at the age of eighteen. It was characterized by a blank stare, supraversion, and tonic posture with extension of the four limbs, followed by tonic tremor and dystonic posture of the hands, sialorrhea, tongue biting, for about 1–2 minutes, and occasionally postictal confusion. The frequency of seizures was three episodes each month, which did not respond to the first line of anticonvulsants (carbamazepine and valproic acid) for more than 3 months, while adequately controlled by levetiracetam and lacosamide afterwards. The brain magnetic resonance imaging (MRI) revealed focal cortical dysplasia in the left frontal and temporo-polar regions (). The video-electroencephalography (EEG) monitoring for more than 72 hours showed abnormal epileptiform activities in the left fronto-temporo-occpital areas. The cognitive function test was not performed, but the patient reported emotional changes without cognitive dysfunction. The patient eventually diagnosed as major depression.
The patient provided her consent to report this case including all of the images in this article.


A 58-year-old Japanese man was suffering from discomfort with left lower abdominal pain and visited our hospital. The patient's BMI was calculated as 41.7 kg/m2 (height: 179.8 cm; weight: 133.8 kg).
A total colonoscopy revealed a lesion in the rectum below the peritoneal reflection (4 cm above the anal verge) (Fig. ). The laboratory findings at the time of admission were within normal limits including tumor marker levels.
The patient was subsequently scheduled for laparoscopic intersphincteric resection via the transanal approach for early rectal cancer. The pull-through procedure with delayed coloanal anastomosis was performed in two surgical stages. Preoperatively, the patient underwent nutrition management for 3 months, and his BMI decreased to 34.3 kg/m2 (height: 177.4 cm; weight: 108.0 kg) (Fig. ).
In the first stage of the abdominal phase, the patient was placed in the lithotomy position. High ligation of the inferior mesenteric artery and vein was performed. The left colon was fully mobilized and brought down and reached the anal verge without tension. In the perineal phase, a Lone Star self-retaining retractor (Lone Star Medical Products, Inc, Houston, TX, USA) was placed at the anus, a purse-string suture was placed approximately 1 cm from the anal verge, and circumferential dissection was performed 1 cm from the purse-string suture. The AirSeal System (SurgiQuest Inc, Milford, CT, USA) was used to perform the TaTME after the GelPOINT (Applied Medical Resources Corporation, Rancho Santa Margarita, CA, USA) access system was set up.
Before that surgery, a urethral catheter with a guide wire was inserted into the urethra under fluoroscopic guidance, after the infrared illumination system urethral kit (IRIS U kit) was used []. Near-infrared light was used to increase visibility when using the laparoscopic system (1588 AIMTM; Stryker) during the laparoscopic and TaTME stages of the procedure. The patient recovered uneventfully without urethral injury.
A colonic pull-through segment of about 10 cm was left outside the anal canal without any tension and fixed by sutures under indocyanine green fluorescence imaging (ICG FI) (Fig. ). The prostatic segment of the urethra was easily and quickly identified with the green fluorescence during TaTME. The first total operation time was 435 min, including a perineal phase of 122 min, and the intraoperative blood loss was 65 mL.
The second surgical stage was performed 10 days after the first operation under general anesthesia. The patient was placed in the lithotomy position and a Lone Star retractor was positioned. The adhesion was fully detached at the anal canal. Final coloanal anastomosis was performed using near-infrared light. It was sutured using 16 interrupted absorbable sutures without diverting the stoma under ICG FI (Fig. ).
The second total operative time was 111 min, and the intraoperative blood loss was 118 mL. The patient demonstrated a good postoperative course and he was discharged from our hospital in remission 15 days after the latest operation.
In this way, we managed to perform a pull-though procedure with a two-stage operation for a case of lower rectal cancer in a patient with a high BMI.


The patient was a 32-year-old Japanese man who was referred to us for HSCT because of severe anemia. He was born with severe hemolytic anemia and jaundice. He was diagnosed with PKD based on the PK activity in the RBCs. He had been receiving frequent RBC transfusions since his birth. He underwent splenectomy at the age of 5. However, his anemia did not subside. Thereafter, frequent RBC transfusion for maintaining the hemoglobin level at 7.0 g/dL with iron chelation was continued for several years. He also underwent cholecystectomy because of hemolysis-related gallstone complication at the age of 19 years. He was diagnosed with severe PKD at the age of 32 years with mutations of the PKLR gene (Exon5 c.434 C del: PK Beppu). He had severe anemia and had undergone frequent RBC transfusion, therefore, he was unable to obtain full-time employment and had an impaired quality of life. Therefore, he wanted to undergo BMT as curative therapy. Prior to BMT, his serum ferritin level was 740 ng/mL. Abdominal magnetic resonance imaging (MRI) T2 sequences showed liver iron burden (). Although the MRI did not reveal cardiac iron burden, we predicted potential organ damage as hepatic hemosiderosis. He also had broad, high-titer and non-specific anti-human leukocyte antigen (HLA) class I antibody. Although their antibody had the capability of exerting adverse effects on engraftment, we administered Rituximab 375 mg/m2 thrice weekly and plasma exchange before BMT.
He underwent BMT with the immunosuppressive conditioning regimen from his HLA identical sister at the age of 32 (). Conditioning regimens included 3.6 Gy total body irradiation (day -8), fludarabine 30 mg/m2 (×6 days, from day -7 to -4), melphalan 90 mg/m2 (×2 days, day - 3 and day -2), and rabbit antithymocyte globulin 1.25 mg/kg (×4 days, day -7 to -4). Graft-versus-host disease (GVHD) prophylaxis included tacrolimus and short methotrexate. The number of infused nucleated cells was 2.5 × 108 cells/kg and that of CD34+ cells was 1.7×106 cells/kg. The early post-transplantation period was uneventful. Stable neutrophil engraftment occurred on day 17. The last blood and platelet transfusion days were day 22 and day 23. The patient presented with fever on day 19. Fever associated with engraftment was successfully treated with methylprednisolone. On day 55, he presented with a rash on < 25% of the body surface area; however, he did not have diarrhea and icterus. He was diagnosed with grade 1 GVHD. At the time of discharge, he had mild chronic skin GVHD but was in a state of complete donor chimerism. Three years following the BMT, the patient’s hemoglobin level was 15 g/dL without any other complications, and he was able to work a regular job. His health-related Quality of life (QOL) was assessed using the Japanese version of the SF-36. His SF-36 scores of physical function (PF) increased from 75 points before BMT to 100 points after BMT. Role physical (RP) increased from 81 points to 100 points, general health (GH) increased from 47 points to 70 points, and vitality (VT) increased 75 points to 93.8 points, respectively. Now he can take part in a marathon for interest, despite he was barely able to walk a little way before BMT.


A 57-year-old African American male patient was first diagnosed in 2015 with multiple myeloma (60% clonal plasma cells on bone marrow biopsy) and AL amyloidosis (confirmed by fat pad biopsy; Figure
). He presented with signs and symptoms of New York Heart Association class III heart failure (elevated jugular venous pulse at 12-cm H20 and 2+ peripheral and scrotal oedema), macroglossia, and bilateral carpal tunnel syndrome. His past medical history included atrial fibrillation and stage 3 chronic kidney disease. Cyclophosphamide, bortezomib (switched to lenalidomide because of peripheral neuropathy), and dexamethasone were initiated at diagnosis as first-line therapy. Intravenous chemotherapy was replaced by an oral regimen to enhance medication compliance in 2016, which improved light chain response. In late 2018, this regimen was replaced by the CD38 monoclonal antibody daratumumab in response to rising serum light chains, progressive CA, and recurrent admissions for decompensated heart failure (elevated jugular venous pulse at 15 cm, 3+ peripheral and scrotal oedema, and dyspnoea at rest) in the setting of ongoing patient non-adherence.
Over time, serial N terminal pro BNP, troponin T, and serum creatinine levels fluctuated along with serum M proteins and light chains, coincident with progression of AL amyloidosis (Table
). Electrocardiograms revealed evolving conduction disease (Figure
).
Transthoracic echocardiogram demonstrated features consistent with CA: normal left ventricular size, preserved ejection fraction, biatrial dilation with atrial thickening, biventricular hypertrophy (1.2–1.6 cm), trivial pericardial effusion, and abnormal myocardial global longitudinal strain (−13.9%) with relative sparring of the apex and a relative regional strain ratio of 2.1 (Figure
).
Interestingly, transthoracic echocardiogram also had evidence of progressive valvular disease with stenosis of the tricuspid and mitral valves coincident with worsening AL amyloidosis (Figure
; Table
). This stenosis was further interrogated with transoesophageal echocardiogram (Figures
 and and Figure
). Transoesophageal echocardiogram demonstrated diffuse thickening of the tricuspid valve leaflets and sub-valvular apparatus causing restricted leaflet motion and moderately severe tricuspid stenosis (mean gradient 4 mm Hg and peak gradient 8 mm Hg) and moderate tricuspid regurgitation. Similarly, there was thickening of the mitral valve leaflets leading to restricted leaflet motion and moderately severe mitral stenosis (mean gradient 9 mm Hg and peak gradient 24 mm Hg at 67 bpm; mitral valve area 1.5 cm2 by 3-D-planimetry) with mild mitral regurgitation. The aortic and pulmonic valves also demonstrated prominent and diffuse thickening, albeit without haemodynamically significant valvular stenosis or regurgitation.


A 56-year-old male was admitted to the emergency room after falling from a roof while working. His past medical history included hypertension and a prostate cancer. He presented back pain. The neurologic exam was normal. He was coughing without evidence of dyspnea. His body temperature was 37.9 °C. He was tested positive for COVID-19 by polymerase chain reaction. A chest X-ray was carried out, showing no major opacity. An additional computed tomography (CT)-scan performed because of suspected spinal trauma evidenced COVID-19 typical thoracic signs of peripheral ground glass opacities (Figure ). The CT further evidenced a vertebral compression fracture of L1 without significant posterior wall involvement, thus classifying the fracture as stable and without risk for neurologic complications (Figure ). Since the patient presented a compensated respiratory function, he was confined at home. The L1 fracture was treated conservatively although a significant vertebral body collapse was present. A secondary operation might be carried out once the patient is free of symptoms and the OR and postoperative care unit availability have improved.


A 71-year-old female with diffuse idiopathic skeletal hyperostosis fell and was admitted to the emergency room. Her past medical history included hypertension and diabetes type 2. She was febrile (38.6 °C) and presented with dyspnea. The polymerase chain reaction was positive for COVID-19 and her chest X-ray showed moderate bilateral opacities. A CT-scan was performed, showing an unstable non-neurologic T7-T8 fracture (Figure ). Furthermore images of a COVID-19 typical thoracic CT-scan with bilateral ground glass opacities were present (Figure ). A first line treatment of the COVID-19 respiratory distress syndrome was considered. A secondary operation was indicated after 16 days once the respiratory function had improved (Sat02 95%), since ambulation was impossible because of the highly unstable fracture. A cement augmented percutaneous instrumentation T5-T10 was performed (Figure ) and the patient was transferred to a specific surgical COVID-19 unit for postoperative care. A postoperative X-ray control apart from intraoperative fluoroscopy was avoided in order to limit any possible contact of the patient with other patients and staff at the radiologic department. She was discharged and confined after 5 d at home, once she was able to walk and her respiratory symptoms had returned to normal. Ambulatory care by a nurse was continued at home.


A 3.5-year-old Syrian female was admitted to our hospital with complaints of abdominal pain. She had had several episodes of non-bilious, non-bloody vomiting for 36 hours, and her last normal bowel movement was 2 days prior. Although the patient had a history of sickle cell anemia confirmed by electrophoresis from her first year of life, she had not experienced any sickle cell crises and had not needed blood transfusion or hospitalization. She had no history of diarrhea, either. Family history for celiac disease was positive in a second-degree relative. On admission, her vital signs were normal. Blood analysis indicated low hemoglobin (Hgb) concentration of 9.3 g/dl, but other initial laboratory results were normal. Abdominal examination showed tender distended abdomen. Abdominal ultrasonography suggested the presence of intussuscepted bowel in the right iliac fossa with free fluid between the bowels. Hydrostatic reduction (HR) was not successful, so she underwent surgical reduction. Although no lead points were found, there were multiple enlarged mesenteric lymph nodes and edematous ileocecal junction. On the third postoperation day, the patient again developed similar symptoms, and this time ultrasonography revealed IS in the left side of the abdomen. There was no improvement in symptoms for 24 hours, and ultrasonography revealed the same results after an HR attempt. The patient was operated on a second time, and manual ileoileal IS () reduction and prophylactic appendectomy were performed. The postoperative course was uneventful, and the patient was discharged 3 days later. Three months later, the patient developed abdominal pain, and the diagnosis of IS was confirmed via ultrasonography. This time, IS reduced over 24 hours of observation. As no lead point was found through the later laparotomy, further investigations were conducted. Upper gastrointestinal endoscopy was performed and showed a mosaic pattern of the duodenum mucosa suggestive of CD. On microscopic evaluation, sections reveal advanced atrophy of duodenal villi with occasional intraepithelial lymphocytes (>30/100 IEL). Crypts show moderate hyperplasia; the lamina propria is infiltrated by diffuse mononuclear inflammatory cell infiltrate. Histopathologically, it is compatible with Grade B2 on the new grading system for CD classification (–). The child was then administered with a gluten-free diet. Eight months later, she visited the hospital as outpatient, and examination revealed she was free of any symptoms.


A 78-year-old wheel chair bound male developed a severe spastic quadriparesis with sphincter disturbance over a 2-year period. His modified Japanese Orthopedic Association (mJOA) score was 8. Cervical X-rays, MR, and CT studies demonstrated OO instability with subaxial C4-C6 cord compression; there was an accompanying 87° fixed kyphosis [-].
The patient underwent a C2 to C7 laminectomy with C1 lateral mass screw placements and insertion of bilateral pedicle screws from C2 to C7 bilaterally []. After assembling a rod on one side from C2 to C7, multilevel Smith-Peterson osteotomies (SPOs) were performed on the contralateral side and vice versa [ and ]. An expandable cage was placed within the corpectomy site; when it became loose intraoperatively, the patient had to undergo anterior cage repositioning. Notably, all procedures were performed utilizing intraoperative neuromonitoring that demonstrated no changes.
The intraoperative cervical cross-table X-ray ultimately confirmed adequate C1 to C7 instrumentation with a 100° correction of the kyphosis []. Three months later, the patient was able to eat and button his shirt without difficulty and ambulated with a walker (mJOA score: 11) []. At 1 postoperative year, he demonstrated no further recovery, and the cervical X-ray showed no further changes in sagittal alignment [].


A 21-year-old male patient visited the Department of Oral Medicine and Radiology, RajaRajeswari Dental College and Hospital, with a chief complaint of painless swelling in his left cheek region since 3 months.
Patient presents with the history of extraction in his upper front tooth region 2 months back, followed by swelling, which was initially small in size, which gradually increased to its present size. There was no history of pain, pus discharge, bleeding, and trauma. Medical history and family history were noncontributory. On general physical examination, patient was moderately built and nourished.
On extraoral examination, solitary diffuse swelling was present on left middle third of the face measuring approximately 4 × 3 cm in dimension, extending superioinferiorly from infraorbital margin to corner of the mouth and mediolaterally from ala of nose to line drawn from outer canthus of the eye. Surface over the swelling appeared smooth and slightly glossy, surrounding skin was not stretched and no visible pulsation seen []. On palpation, swelling was soft to firm in consistency and nontender. Submandibular and submental lymphnodes are nonpalpable.
Intraorally, a well-defined swelling was present on upper left back tooth region measuring about 2 × 3 cm, extending antero-posteriorly from gingival margin of 24, 25, 26 to buccal vestibule of 24, 25, 26, and mediolaterally from distal aspect of 23 to mesial aspect of 28. Mucosa overlying the swelling appeared normal []. On palpation, swelling was firm to hard in consistency, nontender with smooth, well-defined margin. Other findings include periodontal pocket present irt 24, 25, 26. Hard tissue examination revealed grade 1 mobility irt 24, 25 with tenderness on percussion, supragingival calculus, and generalized white intrinsic stains.
Based on history and clinical examination, a provisional diagnosis of Adenomatoid odontogenic tumor irt 24, 25, enamel hypoplasia, and chronic generalized gingivitis was considered. Differential diagnosis of Pindborg's tumor and keratocystic odontogenic tumor was considered.
Radiographical investigations included orthopantomograph, intraoral periapical radiograph (IOPAR), occlusal radiograph, and CBCT of maxilla.
Orthopantomograph revealed ill-defined radiolucency involving left maxilla extending mediolaterally from distal aspect of 23 to mesial aspect of 28 and anteroposteriorly from alveolar crest into the maxillary sinus []. Intraoral periapical radiograph of 24, 25, 26 revealed mixed radiolucency and radio-opacity with displacement of roots of 24 mesially and 25 distally [], while maxillary occlusal radiograph revealed buccal cortical plate expansion [].
CBCT of maxilla revealed, a well-defined osteolytic lesion seen irt left maxilla, which was roughly circular in shape, extending from 23 to 28 region mesiodistally, from superior wall of left maxillary sinus to crestal bone of inter premolar region superioinferiorly and from medial wall of the maxillary sinus to buccal cortical plate mediolaterally. Borders are thin, corticated, scalloped, and internal structure is mixed radiolucent-radiopaque. Internal structure shows thin, wispy septae and incomplete loculations with multilocular appearance seen irt 23 and 24 region. Roots of 24, 25, and 26 show mild resorption. Two-third of the maxillary sinus was involved with antral cloudiness and thinning of the antral walls. Roots of 24 and 25 appeared diverged. Lateral wall of the left nasal fossa is thinned out in 24 region. Palatal cortical plate is thinned out irt 24 and 25 region yet is intact [Figures –]. So, the radiographical differential diagnosis of ameloblastoma and OM was considered.
Based on the clinical diagnosis of AOT, biopsy was performed. The microscopic examination of hematoxylin and eosin-stained section showed lesional tissue fragments composed of fibrocollagenous stroma with hypocellular myxoid areas and scattered band appearing spindle shaped cells []. No frank pleomorphism or mitosis or necrosis was noted and the section was negative for granulomas or malignancy. Subsequently, the lesion was diagnosed as OM and surgical resection followed by prosthetic reconstruction was proposed.


A 51-year-old Vietnamese female, who migrated to the United States at age 25 years,
was seen at an outside clinic in East Texas in 2015. She had a 1-year history of
lesions consistent with areas of erythema and infiltration of dermis of the cheeks,
chin, nose, and chest. She reported numbness, weakness, and edema of her hands and
feet for 4 months. Skin biopsy of the left ear showed chronic inflammatory
infiltrates replacing 75% of dermis but separated from the basal layer of the
epidermis by a clear zone. The infiltrates were composed of foamy histiocytes and
lymphocytes involving cutaneous nerves. Fite stain revealed numerous acid-fast
bacilli within histiocytes and cutaneous nerves. She was diagnosed with lepromatous
leprosy. The treatment consisted of rifampin 600 mg daily (eventually monthly),
clarithromycin 500 mg daily, and levofloxacin 500 mg daily for 2 years. Dapsone and
minocycline were also prescribed but discontinued due to adverse effects (hemolytic
anemia and hyperpigmentation, respectively). She did not receive clofazimine due to
prior hyperpigmentation. Her extremity edema was attributed to T2R; however, edema
is also an identified feature of lepromatous leprosy. Treatment with prednisone and
eventually methotrexate was started but she was lost to follow-up for 19 months
before reestablishing care at our institution.
She presented with new indurated tender skin lesions and worsening pain and edema on
her extremities, since last seen. She also reported subjective fever and weight loss
(30 lbs in 4 months). On physical examination, she had extensive erythematous
subcutaneous papules and nodules with a background of gray hyperpigmentation on her
arms and legs (),
and few scattered tender superficial erythematous nodules on the face and abdomen.
Madarosis and hypotrichosis of arms and legs were seen. Decreased light touch
(monofilament 0.2 gr) in the left arm and loss of protective pain sensation (10 gr
monofilament) in both feet with glove-stocking pattern were noted. The remainder of
her examination was unremarkable. The white blood cells count was 10.3
cells/cm3 (neutrophil predominance) and hemoglobin was 10.8 mg/dL.
Routine chemistries were normal. A 4-mm punch biopsy taken from the right forearm
revealed foamy macrophages in the dermis with an intense neutrophilic infiltrate
extending into the subcutaneous fat (). A lower magnification view (x4)
showed intense inflammatory cell infiltrate that outlined the dermal vasculature and
extended into the subcutaneous fat. There was a perivascular mixed inflammatory
infiltrate and fibrinoid necrosis of small vessels. Fite stain showed beaded and
granular acid-fast bacilli within the histiocytes and focally within cutaneous nerve
twigs ().
Polymerase chain reaction for M leprae DNA was positive. Tissue
culture was negative for bacteria and fungi. The biopsy was reviewed by the US
National Hansen’s Disease Program in Carville, Louisiana. Compared with a biopsy
from 2015, the inflammatory infiltrates were diminished and the bacilli had a
degenerating appearance. These findings were consistent with appropriate response to
antimycobacterial therapy. Treatment with thalidomide 100 mg daily was initiated
with improvement in the appearance of the skin lesions (). A repeat biopsy performed 6 months
later showed lack of neutrophilic infiltrates and decreased number of acid-fast
bacilli. Monthly skin scrapings from affected sites showed progressively decreased
bacterial load.


A 1-day-old term male presented to hospital with multiple necrotic skin lesions. His
mother was healthy; however, the antenatal course was complicated by presumed viral
gastroenteritis in the first trimester, three viral upper respiratory infections in
the second trimester, and a two-day history of diarrhea and nausea 2 weeks prior to
delivery. The pregnancy was otherwise unremarkable with protective serologies,
negative group B streptococcus screening, and normal ultrasounds. Family history was
unremarkable and there was no history of travel. The family had dogs, chickens,
turkey, and sheep. Labor was precipitous, with the patient born via spontaneous
vaginal delivery to paramedics at term. Apgars were 10 and 10, and the placenta was
delivered intact. A midwife arrived shortly after delivery and recommended urgent
evaluation for his skin lesions.
Examination revealed approximately 20 cutaneous lesions ranging from brown macules to
purple-blue-black necrotic and hemorrhagic-crusted papulonodules (). The two largest
lesions were each 0.5 cm in diameter, located on the midline back and shoulder
(). In addition
to full skin examination, a thorough full physical examination verified by multiple
pediatricians and pediatric specialists was normal.
A punch biopsy was performed on a subtly violaceous crusted macule on the right
forearm (). Pathology
demonstrated an isolated focus of dense perifollicular histiocytic and dendritic
cellular infiltrate sparing the epidermis (). Eosinophils were not identified.
Immunohistochemistry for CD1a (), CD163, S100, and Cyclin-D1 were strongly positive in the
lesional cells and confirmed them to be Langerhans cells. A repeat biopsy () of a fully
developed crusted necrotic blue-black papule was pursued given the poor
clinicopathologic correlation. This second sample revealed extensive epidermal
ulceration and dense infiltrates of eosinophils and CD1a-positive Langerhans cells
with vesicular nuclei with prominent grooves and ample eosinophilic cytoplasm in the
dermis and subcutaneous tissue (). BRAF mutation testing was negative.
Laboratory and imaging investigations following the recommendations of the Histiocyte
Society confirmed the diagnosis (for details, see LCH in ). Infectious serologies, including
a TORCH screen, were negative. Complete blood count, liver transaminases,
electrolytes, bilirubin, urea, and creatinine were all within normal limits.
Skeletal survey, chest radiograph and abdominal and head ultrasounds of our patient
revealed no evidence of systemic involvement. Our patient was reassessed by both
pediatric dermatology and pediatric oncology at 2 weeks of age, and then regularly
every 3 months until the age of 2. All LCH lesions had resolved by 18 months of age.
He continues to be followed every 6 months and has no evidence of internal organ
involvement on repeat bloodwork or imaging.


Our patient was an otherwise healthy 43-year-old male who had intermittently taken prescription methadone, fentanyl, and oxycodone over a 14-year period of time for chronic low back pain. In the past 2 to 3 years, he used heroin after coming home from working his job during the night shift; he would inject heroin intravenously to relax and fall asleep, and later would join his spouse and daughter for dinner before going back to work. Aware of the severity and progression of his problem, the patient had reached out to several local addiction treatment programs. Upfront costs, required travel to a treatment center, and the inability to be away from work prevented him from participating in any treatment program.
On one occasion, the heroin he obtained was more potent than expected. He injected himself and fell asleep. When he did not answer his spouse’s calls, she came home from work and found him unresponsive and apneic; this was just moments before their pre-teen daughter would have come home from school. His spouse, a layperson, was unable to locate naloxone in their home and performed cardiopulmonary resuscitation until first responders arrived, even though she does not work in health care herself. First responders administered 2 intranasal doses of naloxone as the patient was transported to the emergency department (ED) of the critical access hospital in the same town. There, he was medically stabilized and monitored overnight. Coincidentally, his family medicine physician, who cared for his entire family and was familiar with the patient, was working in the ED that night. He was aware of Suboxone therapy for OUD being offered by his colleagues in the outpatient practice and made an urgent referral to the MAT provider. The following morning, the patient was seen in the family medicine clinic. He was actively in withdrawal with a COWS score of 16, indicating moderate withdrawal. Suboxone therapy was initiated according to the MAT protocol, 2 mg initially and 2 mg every hour thereafter for a total of 4 doses. He was stabilized over several days of follow-up at a dose of 8 mg of Suboxone twice daily. Follow-up consisted of frequent weekly visits for the initial 4 weeks, monthly visits for 6 months, and then continued office visits every 3 months thereafter.
At a follow-up visit after 6 months of MAT, the patient was motivated to share his positive experience with others and referred 2 people for MAT in our practice. One year after beginning MAT, he was still taking Suboxone at 8 mg twice daily and felt that he was ready to begin weaning to a lower dose. He was working, had received a promotion, was actively participating in family activities, and made it a point to attend all of his daughter’s school events. He and his family remain in our family medicine practice and are otherwise physically and emotionally well.


A 76-year-old male presented to the emergency department with recurrent painless bleeding from his ileal conduit site. This was his fifth visit to the hospital with similar complaints. He had a history of muscle invasive bladder cancer with peri-vesical tissue involvement, for which he underwent radical cystoprostatectomy with ileal conduit urinary diversion 7 years prior to presentation, after which he was lost to follow up. He did not take chemotherapy or radiation. He also had a history of Stage III chronic kidney disease, alcohol use of approximately 2–3 cans of beer daily for the last 40 years, and a history of medication non-compliance and non-compliance with follow-up appointments. He denied any history of nausea, vomiting, abdominal pain, back pain, blood in stools, hematemesis, or any other site of bleeding. He gave a history of fatigue, dizziness, and on and off painless bleeding from his ileal conduit site for over a year. At presentation, his heart rate was 103 beats/min, blood pressure was 126/60 mmHg, and respiratory rate was 18/min, saturating at 91% on room air. His examination was unremarkable except for gross bleed in his urostomy bag with blood clots. [His investigations revealed a hemoglobin of 3.8 g/dL (13.5–17.5), white blood cell count of 6.1 K/μL (4–11 K/μL), platelet count 114 K/μL (140–400), International Normalised ratio (INR) 1.38 (0.85–1.12), prothrombin time 17.6 s (12.1–15.0), total bilirubin 0.7 mg/dL (0–1.3), aspartate aminotransferase 40 U/L (14–59), alanine aminotransferase 36 U/L (11–66), and alkaline phosphatase 69 (38–126 U/L).] The patient's creatinine was elevated at presentation at 2.78 mg/dL (0.50–1.30), from his baseline creatinine of 1.32 mg/dL (estimated glomerular filtration rate = 56 ml/min/1.73 m2). After volume resuscitation, his creatinine improved to 1.4 mg/dL. He was transfused with packed red blood cells (PRBC's). His noncontrast computed tomography (CT) of the abdomen and pelvis showed subtle nodularity of liver contour and mild splenomegaly (14.1 cm in length). Flexible cystourethroscopy through ileal conduit showed a small area of hemorrhage in the anterior portion of the conduit just deep to the abdominal wall fascia. The hemorrhagic site was fulgurated without evidence of other bleeding sites. On further examination, purple discoloration of the ileal stomal site was noticed []. Given his recurrent episodes of bleeding from the stomal site, purplish discoloration of the skin around the stoma, and liver nodularity, there was a high degree of suspicion of underlying liver cirrhosis with portal hypertension and extrahepatic peristomal varices. He underwent CT abdomen and pelvis with contrast, which showed heterogeneous density and enhancement of the liver, nodularity of the liver edge, and parastomal varices with mild ascites []. He was treated conservatively with octreotide for variceal bleeding. During the evaluation of his liver cirrhosis, his hepatitis C antibody was reactive, and his hepatitis C RNA load was 121012 IU/mL. Evaluation for hepatitis B was negative; abdominal Doppler did not show any portal venous thrombosis. His model for end-stage liver disease score at the time of TIPS procedure was 16 points, 6% estimated 3-month mortality. He successfully underwent TIPS with portosystemic pressure gradient reduction from 10 to 4 mmHg in addition to the coil embolization of ileal conduit varices by an interventional radiologist. The patient was advised to follow up with hepatology and gastroenterology for further evaluation for cirrhosis and treatment of his hepatitis C infection, respectively, and with endoscopy for evaluation of esophageal varices. He was seen in the hepatology clinic at 5 months; at which time he did not have any new episode of bleeding He was lost to follow-up after that appointment.


A 41-year-old woman with a history of hypertension, presented with fever, productive cough and general fatigue. She denied any recent travel but had a history of contact with sick people. In the emergency department she was tachypnoeic (respiratory rate of 22 breaths/min), tachycardiac (130 bpm), febrile (39°C) and hypoxic (oxygen saturation (SpO2) of 88% on room air). Physical examination of the lungs revealed bilateral crackles and wheeze. The white cell count was normal and the absolute lymphocyte count was low (0.61–103/μl), while the erythrocyte sedimentation rate (77; range, 0–20 mm/h) and C-reactive protein levels (110; range, 0–5 mg/l) were increased. D-dimer was 1657 ng/ml and procalcitonin was normal. Assays for influenza viruses and a respiratory syncytial virus were all negative. A nasopharyngeal swab was positive for SARS-CoV-2 on RT-PCR assay. Computed tomography (CT) at the time of admission revealed bilateral ground-glass opacities and consolidation (). The diagnosis of COVID-19 pneumonia was made and treatment with oseltamivir, hydroxychloroquine and low-molecular-weight heparin was initiated. The patient received supplemental oxygen through a nasal cannula at a rate of 4 l/min. Blood and urine cultures were negative.
After 3 days of hospitalization the patient’s clinical condition deteriorated (respiratory rate of 26 breaths/min and SpO2 decreased to 78%) and she required intensive care unit (ICU) admission. A chest x-ray at the time of ICU admission revealed hazy bilateral lobe opacity (). The patient was intubated and diagnosed with CRS. She was given a single intravenous (IV) dose of 400 mg TCZ and IV methylprednisone 60 mg daily for 3 days. Ventilatory support requirements reduced day-by-day. C-reactive protein, ferritin and D-dimer levels dropped from 310 mg/l, 3500 ng/dl and 2123 ng/ml on the day of ICU admission to 13.2 mg/l, 300 ng/dl and 320 ng/ml after 3 days, respectively. A chest x-ray showed that the lesions significantly improved within 3 days of TCZ administration (). The patient was successfully extubated 5 days after treatment with TCZ. No adverse events were described. On day 10, a clear improvement in the patient’s general condition was observed, with an SpO2 of 97% without any need for supplemental oxygen ().


A 49-year-old woman presented with a 3-day history of fever, generalized malaise, and worsening of severe, diffuse, acute-onset abdominal pain that was greatest felt in the left upper quadrant.
She had a history of three hospitalizations and one short observation stay for acute pancreatitis over a 4-month period. Her symptoms were classic for acute pancreatitis: abdominal pain with radiation to the back, elevated lipase level, and radiologic findings of acute pancreatitis involving the tail of the pancreas. She was treated conservatively following these events and recovered uneventfully, except for the persistence of intermittent left upper quadrant abdominal pain. The diagnosis was considered idiopathic, as she reported no significant alcohol consumption, trauma, new culprit medications, laboratory test result abnormalities, or imaging findings to suggest gallstone pancreatitis, hypercalcemia, or hypertriglyceridemia. On her second admission (approximately 8 weeks since the first admission), imaging revealed new peripancreatic (tail) fluid collection abutting the spleen and gastric fundus (). On the third admission (approximately 6 weeks since the second admission), imaging revealed new acute splenic vein thrombosis, gastric varices, and splenomegaly in addition to the known peripancreatic fluid collection (). The patient was started on anticoagulation with low-molecular-weight heparin bridged to a direct-acting oral anticoagulant (DOAC) after hematologist consultation. The international normalized ratio on DOACs ranged from 2.1 to 3.4. Diagnostic endoscopic ultrasound (3 weeks since the third admission and 3 days before the current admission) showed a 3-cm heterogeneous fluid collection in the pancreatic tail with an irregular ill-defined margin extending to the splenic hilum consistent with the known finding (). No mass lesion was identified. The pancreatic parenchyma in the head and body of the pancreas was within normal limits. The common bile duct and pancreatic duct were non-dilated with no filling defects. Several benign-appearing 1-cm lymph nodes were noted.
On arrival to the emergency department, the patient was hypotensive at a blood pressure of 75/45 mm Hg and tachycardic at a heart rate of 120 beats/min. The laboratory test results were remarkable for the lipase level (147 IU/L), white blood cell count (15×103 /μL), Hb level (10 g/dL), creatinine level (1.6 mg/dL), and beta-hemolytic non-group A or B streptococcus bacteremia presence. Abdominal and pelvic CT showed acute-onset chronic pancreatitis with fluid collection along the pancreatic tail (not organized) that extended into the postero-inferior aspect of the spleen and known thrombosis of the splenic vein with portosystemic collaterals, gastric varices, and mild splenomegaly. She was initially treated with fluid resuscitation and vasopressor and antibiotic administration with initial improvement in clinical status. However, she developed acute shortness of breath, worsening abdominal pain (progressed to diffuse, severe, and constant pain), and distension the following day. Repeat CT showed splenic rupture with moderate hemoperitoneum and perisplenic hematoma (). Emergency laparotomy with splenectomy was performed successfully with an uncomplicated recovery. Pathology revealed multiple lacerated spleen fragments.


A 39-year-old female patient (weight 116 kg) was scheduled to undergo laparoscopic cholecystectomy. She had history of hypertension, chronic obstructive pulmonary disease (COPD) successfully treated with bronchodilatators and obesity (BMI 44.9 kg/m2). Before anaesthesia induction, 11.6 mg dexamethasone and 1 gm paracetamol were given intravenously (IV). Anaesthesia was induced with IV 0.04 mg/kg midazolam, 1 mg/kg lignocaine hydrochloride, 1.5 mg/kg propofol, 0.5 mg/kg ketamine, and 0.6 mg/kg rocuronium bromide. After tracheal intubation general anaesthesia was maintained with sevoflurane (MAC 0.7-1) and continuous intravenous infusion of magnesium 25 mg/kg/h and lignocaine 2 mg/kg/h. At the end of the surgery 2.5 gm metamizole was given intravenously. VAS score 1 and 4 h after surgery was 5/10 at rest and 6/10 on coughing, and 100 mg ketoprofen was given. After surgery 8, 12 and 24 h pain was 2/10 at rest and on coughing.


A 56-year-old male patient (weight 83 kg) was scheduled to undergo laparoscopic cholecystectomy. Previously, he underwent two open thoracic operations but postoperatively he could not awake easily from anaesthesia, had severe pain, was sedated and suffered from delirium. Six months after these surgeries he still had concentration difficulties, memory deficits, and pain. Previous anaesthetic charts showed high amount of fentanyl used during surgery, without using thoracic epidural anaesthesia. The same regime for OFA was performed with continuous infusion with 0.2 mg/kg/h ketamine, 2 mg/kg/h lignocaine, and 40 mg/kg/h magnesium. After removal of gallbladder, intraperitoneal instillation of 20 ml 0.5% bupivacaine for postoperative analgesia was performed and 1 gm metamizole was given. Patient awoke smoothly from anaesthesia and VAS score of pain was 0 at rest and 1 on coughing. Two and eight hours after surgery VAS pain scores were 2/10 at rest and on coughing. Eleven hours after surgery patient reported pain at rest 4 and 5 on coughing and 1.5 gm metamizole was given.


A 74-year-old woman was scheduled for laparoscopic cholecystectomy and previous history of hypertension, COPD, chronic cardiomyopathy, supraventricular extrasystole, and insulin dependent diabetes mellitus. Spirometry tests reveale dpredicted value of FVC was 35%, FEV1 was 29%, results from capillary blood sample were pO2 62mmHg, and pCO246 mmHg. OFA was used with continuous infusion of magnesium 35 mg/kg/h and lignocaine 2 mg/kg/h. At the end of the surgery, 1.5 gm metamizole was administered. She complained of having pain 1 and 24 h after surgery and VAS pain score was 3/10 and 4/10 at rest and 5/10 and 5/10 on coughing respectively, without any respiratory and cardiovascular complications in the postoperative period.


A 68-year-old woman (weight 68 kg) and 57-year-old man (weight 86 kg) were scheduled for open colectomy to remove colon cancer. A thoracic epidural anaesthesia (at level Th9-Th10) together with OFA was used in both patients (with continuous infusion with lignocaine 2 mg/kg/h and magnesium 25 mg/kg/h). Total amount of bupivacaine given into epidural catheter during surgery was 25 mg and 27.5 mg of bupivacaine in two patients, respectively. They remained haemodynamcially stable during surgery. At the end of the surgery 2.5 mg metamizole was given. Female patient reported pain score 0/10 at rest and on coughing, 2 h after surgery and was transferred to the department. During 72 h after surgery she reported higher pain scores only 6 h after surgery. Pain was 7/10 at rest and 8/10 on coughing and 2 mg morphine was given via epidural catheter with 7 ml of saline. After 24 and 36 h pain was 5/10 at rest and 6/10 on coughing (1 gm paracetamol was given). After 12 h, 48 h, and 72 h no pain was reported. Male patient reported pain 5/10 at rest and 6/10 on coughing, 2h after surgery (1 gm paracetamol was given). During 72 h after surgery he reported higher pain scores 6 h after surgery, pain 8/10 at rest, and 9/10 on coughing and was treated with 2 mg morphine through epidural catheter. Twelve hours and 36 h after surgery pain scores were 5/10 at rest and 6/10 on coughing and paracetamol 1 gm was given. No analgesic was given 24 h, 48h, and 72 h after surgery—pain at rest was 1-2/10 and 2-3/10 on coughing.


A 68-year-old male patient was scheduled for laparoscopic colectomy (removal of colon cancer). He refused having thoracic epidural anaesthesia, but accepted to have transversus abdominis plane block (TAPB). Continuous infusion contained lignocaine 2 mg/kg/h and magnesium sulphate 35 mg/kg/h. Ultrasound-guided bilateral TAP block was performed with 20 ml 0.25% bupivacaine on each side. During surgery they changed from laparoscopic to open and lasted for 3:15 h. At the end, 2.5 gm metamizole was given and he was transferred to PACU with no pain. After 2 h he reported pain 3/10 at rest and on coughing. He reported higher pain scores 8 h after surgery, 7/10 at rest and on coughing and 100 mg tramadol was given. Thirty-six hours and 60 h after surgery pain was 4/10 at rest and on coughing and 1 gm paracetamol was given. After 12 h, 24 h, 48 h, and 72 h, he didnot complain of having pain (1/10 at rest and on coughing).


A 28-year-old male patient was scheduled for laparoscopic herniorraphy and from previous history he used to consume narcotics 4 years ago. Considering the fact for narcotic abuse relapse in the postoperative period, he received OFA with bilateral TAP block. Protocol for OFA was the same, but i.v. infusion contains lignocaine 2 mg/kg/h, magnesium sulphate 40 mg/kg/h, and ketamine 0.2 mg/kg/h. At the end of the surgery bilateral TAP block was performed with 20 ml 0.25% bupivacaine on each side and 2.5 gm metamizole was given. After extubation patient was taken to the PACU and stayed there for 2 h. Two hours after surgery he reported having pain 5/10 at rest and 6/10 on coughing and 100 mg ketoprofen (NSAID) was given. He received 1 gm paracetamol and 100 mg ketoprofen given every 4 h alternately. Tramadol 100 mg was given 28 h after surgery because of persistence of severe pain. He was discharged home 40 h after surgery with prescribed oral analgesics paracetamol.


A 50-year-old woman presented with a 6-week history of pain and swelling on her right
first toe. The patient reported no preceding history of significant trauma or
inflammation and no signs of arthritis of the right foot. Her medical history
included diagnosis of an undifferentiated nasopharyngeal neoplasm 6 years
previously. In 2011 she had undergone radiotherapy and chemotherapy, which is the
standard treatment for nasopharyngeal cancer., The initial response was
complete, and the patient was followed up by clinical examination every 6 months.
Pulmonary metastases and coughing were noted 1 year before the current presentation,
and palliative care was instituted. The cough was relieved and the patient
discontinued further treatment.
Physical examination revealed a tender, firm, red swelling on the right big toe
(). The pain
increased when pressure was applied on the toe. The patient exhibited decreased
active interphalangeal flexion. No loss of sensation or passive range of motion was
found. The neurovascular status of the digit was normal. Blood tests revealed an
inflammatory syndrome (C-reactive protein concentration of 35.25 mg/L). The
patient’s symptoms were partially eased by anti-inflammatory drugs. Radiographs
showed an osteolytic process involving the distal phalanx of the right big toe with
no periosteal reaction (). Magnetic resonance imaging (Siemens Essenza 1.5T scanner; Siemens
Healthineers, Erlangen, Germany) showed a mass in the distal phalanx of the right
big toe. The dimensions of the tumor were 2.3 × 2.0 × 2.1 cm. The mass showed equal
signal intensity on T1-weighted images and high signal intensity on T2-weighted
images. The tumor showed significant enhancement after administration of contrast
agent ().
Clinical evaluation indicated a malignant tumor. Options of amputation versus wide
resection and toe salvage were discussed with the patient, and the decision was made
to perform amputation through the first metatarsophalangeal joint. The surgical
procedure was performed under general anesthesia. A tourniquet was used to avoid
hemorrhage. After toe amputation, a piece of skin was used to cover the wound.
Macroscopically, the lesion contained necrotic tissue with bony invasion. The lesion
was referred for histologic analysis because of these atypical intraoperative
findings.
Grossly, the tumor tissue appeared as a soft gray mass measuring 2.6 × 1.7 cm.
Microscopically, invasive tumor growth was evident as shown by clusters of malignant
cells featuring central necrosis. Immunohistochemical staining demonstrated positive
expression of p63, CK5/6, and EBER and negative expression of CD56, CgA, and Syn
(). These
findings were similar to the primary nasopharyngeal neoplasm diagnosed 6 years
previously and confirmed the diagnosis of nasopharyngeal metastasis with negative
surgical margins.
The patient underwent antibiotic therapy for 1 week after the surgery. The wound
healed well, and no infection occurred. Two weeks after the surgery, the patient was
able to walk and her pain had resolved. Her postoperative recovery was uneventful,
and she was referred to an oncologist. The patient underwent a clinical follow-up
examination every month. Six months after surgery, she had experienced no symptom
recurrence.
This case report followed the CARE guidelines. The study protocol was approved by the
Medical Ethics Committee of the First Affiliated Hospital, College of Medicine,
Zhejiang University. Written informed consent was obtained from the patient.


A 30-year-old Caucasian male active duty service member in the United States Army with a history of recurrent sinusitis and multiple childhood pneumonias presented to the pulmonary sub-specialty clinic with a chief complaint of chronic cough since childhood. The patient reported the cough to be productive of yellow-green sputum and worse in the morning. He denied shortness of breath, chest pain, gastrointestinal symptoms or post-nasal drip. His only medication was albuterol which he utilised on average once every 48 h. The patient denied any functional limitations and he noted he was able to perform and pass his biennial military mandated physical fitness assessment, including a two-mile run, without difficulty. The patient had never smoked and denied recreational drug use. There were no significant occupational exposures in his history. He has no family history of cystic fibrosis (CF) or other pulmonary disease. He has a sister who is alive and well with one non-biologic child. The patient was previously married and divorced and he did not have children of his own. Vital signs were normal and his body mass index was 25.8 kg/m2. His clinical exam was unremarkable with no evidence of sinus disease and clear lung sounds amidst frequent coughing.
Over a period of decades, the patient had been evaluated by multiple providers and had been empirically treated for conditions including seasonal allergies, upper airway cough syndrome, reactive airway disease and gastroesophageal reflux without any significant improvement of his cough. He described occasionally disjointed care that often resulted in trials of similar medications with limited effect. The patient noted frustration with his medical care to date and described periods of time where he was lost to follow-up related to his frustration.
Additionally, he had been diagnosed and treated for bacterial pneumonia four times in the past. Of the four episodes, the most severe occurred when he was a teenager. During this illness, he underwent bronchoscopy with bronchoalveolar lavage. Bronchoalveolar lavage culture grew Staphylococcus aureus. During a subsequent case of pneumonia seven years prior to the current presentation, he had reported haemoptysis. This presentation prompted a chest CT which demonstrated bilateral varicose bronchiectasis (). The patient completed a course of antibiotics for community-acquired pneumonia at that time with overall improvement and resolution of his haemoptysis. However, no additional evaluation for his chronic cough and bronchiectasis was pursued.


A 48-year-old man was referred to our department due to recurrent urethral stricture. His history revealed an initial episode of difficulty in urinating 3 years prior to the presentation. He was diagnosed with bulbar urethral stricture, at another hospital, and was treated by four failed attempts of balloon dilation over this time period. He had been performing self-catheterization since the last balloon dilation to prevent recurrence. He had experienced urethral trauma in his 30s from a bamboo pick in an accident, which was suggestive of a traumatic stricture etiology.
We proceeded with the placement of a suprapubic catheter to eliminate the need for intermittent self-catheterization, with the aim of urethral rest for 3 months. Following this urethral rest period, we proceeded with urethroscopy and retrograde urethrography, which revealed a complicated 20-mm long stricture in the bulbar urethra, which had a particularly narrow segment (Fig. a,b). Initially, we planned to use a dorsal onlay procedure to treat this stricture, as per previous recommendations. This surgery was performed via a midline perineal incision. The bulbar urethra was strongly adherent to the adjacent tissue due to prior procedures. After the mobilization of the bulbar urethra, the stricture site was opened along its dorsal surface, which revealed complete obliteration of the urethral lumen over a distance of >30 mm (Fig. a,b). We altered our surgical plan at that point, focusing on the excision of the obliterated segment and reconstruction with augmented anastomotic urethroplasty (Fig. ). We completed a ventral anastomosis between the healthy mucosa of the proximal and distal urethra using 4-0 PDS interrupted sutures (Fig. c). An oral mucosa graft was harvested from the left inner cheek and spread and fixed to the corpora cavernosa (Fig. d), with the edge sutured to the opened urethral mucosa.
The postoperative course was uneventful. Retrograde urethrography, performed 3 weeks after the surgery, revealed minor leakage, and the urethral catheter was removed (Fig. a). Urethroscopy performed 4 months after surgery confirmed easy passage through the augmented lesion, with no recurrence of stricture (Fig. b).


The case we present was a 71-year-old Japanese man. When he was being treated for pneumonia at another hospital, CT image incidentally revealed a BT and mild right-sided hydronephrosis (Fig. ) without metastasis. After his pneumonia had been cured, the patient was referred to the SIMC to receive treatment for his BT (SIMC-Uro# 11116, a unique non-sequential patient control number issued at the Department of Uro-Oncology, SIMC). At his first visit, the patient's physical (height: 1.56 m, weight: 48.9 kg) and routine laboratory data were within the normal ranges. He had been diagnosed with diabetes mellitus, which required regular insulin injections. MRI showed many BTs (Figs –). The largest tumor detected on MRI is indicated by the blue arrowhead in Figure a. The tumor indicated by the yellow arrowhead was observed as it progressed between the right ureteral wall (white arrowhead in Figure a) and bladder wall (red arrowhead). Moreover, a tumor was found within the right orifice of the ureter (green arrowhead), which had presumably caused the mild right-sided hydroureter (“u” in Figure a, see also Figures –). Thus, the patient was diagnosed with bladder carcinoma, which was suspected to have infiltrated into the ureter (green arrowhead). No apparent muscle invasion, lymph node metastasis, or distant metastasis was observed (clinical classification: T1, N0, M0, according to the classification of the Union for International Cancer Control).
The patient underwent oral-PDD-TUR-BT (20 mg/kg of 5-ALA was administered 2 hours before surgery) using the D-LIGHT system (Table ). As suggested by MRI (blue arrowhead in Figure a), the main papillary tumor was located on the right ureteral orifice (blue arrowhead in Figure b), and it clearly emitted fluorescence under blue light (Fig. c). It had invaded the bladder neck, the prostatic urethra, and the right posterior wall of the bladder (Video ). In addition, three daughter tumors were found on the posterior wall of the bladder (Video ). Random bladder biopsy samples were obtained from the surrounding normal tissue (the posterior, left, and right walls) and the tumor-infiltrated tissue (the bladder neck and prostatic urethra). Subsequently, we deeply resected the largest tumor (Fig. b,c). The right ureteral orifice was exposed, and it was found to have been blocked by papillary tumors (the tumors in the ureter and the ureteral wall are indicated by green and white arrowheads, respectively; Fig. d,e). We deeply resected the tumors in the ureteral orifice; however, after the resection procedure residual tumors were identified within the ureteral orifice (green arrowhead in Figure f), which emitted potent fluorescence under blue light (Fig. g, see also Video ). We decided to attempt to remove the remaining tumors. After the second and third attempts to resect the ureteral tumors (deep resections, Video ), no tumors were visible under white light in the right ureter (Fig. h); however, marked fluorescence was observed from the ureteral mucosa (Fig. i), which was indicative of residual cancer. If the patient had undergone TUR without PDD, we would have finished the surgery at this point. Instead, we performed a biopsy of the fluorescence-emitting tissue. We then attempted to remove the suspected residual carcinoma. After the fourth resection of the ureteral orifice, the hydroureter-affected lumen was exposed, the mucosa of which was smooth and did not emit fluorescence (Fig. j,k). Subsequently, we performed a biopsy of the ureteral mucosa (Video ).
The pathological diagnosis of the BT was low-grade urothelial carcinoma, which had not invaded the muscular layer (pTa). No cancer cells were detected in random biopsy specimens. As expected from the PDD, low-grade cancer cells were detected in the ureteral mucosa after the third resection of the ureteral orifice, in which an irregular and fluorescent mucosa was identified (Fig. h,i). On the other hand, no cancer cells were found in the smooth and non-fluorescent mucosa after the fourth resection procedure. To prevent tumor recurrence, the patient is currently being treated with intravesically administered BCG via a right ureteral stent, which exposes both the ureter and the bladder to BCG. We concluded that the ureteral tumor was a BT that had infiltrated into the ureter because (i) preoperative MRI (Fig. a) showed that the main tumor was located in the bladder, (ii) the hydronephrosis was mild (CT findings, Figure ; the hydronephrosis would have been severe if the ureteral tumor had originated from the ureteral orifice), and (iii) the pathological analyses did not show muscular invasion. Thus, we succeeded in resecting the tumor in the ureteral orifice, which was accomplished via PDD using orally administered 5-ALA.


A 45-year-old female presented with bilateral eye pain over the past few days, reporting inappropriate handling of contact lenses and risky behavior. On first examination, visual acuity was 20/100 in the right eye and 20/80 in the left eye. Biomicroscopy showed conjunctival hyperemia, purulent conjunctivitis, corneal abscess in both eyes and hypopyon in the right eye. The presumptive diagnosis was bilateral infectious keratitis. Multiple samples were taken from the lesions.
Treatment was initiated with cyclopentolate vancomycin 50 mg/ml, ceftazidime 50 mg/ml, amphotericin B 5 mg/ml and oral fluconazole 150 mg. At the 7-day follow-up examination, size of lesions remains unchanged (); therefore, topical voriconazole 0.5 mg/ml was initiated.
Although the maximum antifungal treatment was initiated, corneal lesions began to increase in size and depth. Conjunctival autograft was performed but with unsuccessful results.
With the patient’s consent, CXL was performed in both eyes using Dresden Protocol with riboflavin instillation (vitamin B2, 402.7 Osm/L) for 30 minutes, followed by UVA irradiation (3 mW/cm2, 5.4Joules/cm2, and 370 nm) for additional 30 minutes while riboflavin instillation continued.
After 48 hours of CXL treatment, corneal abscesses were more defined, with a minor stromal inflammatory reaction and with no pain.
After 17 days, the result of the microbiological culture showed the presence of F. solani in both eyes.
The abscesses developed into inactive corneal scars and 6 months after treatment, corneal scar lesions remained, with a consequent best corrected visual acuity of 20/40 in the right eye and 20/30 in the left eye.


A 19-year-old male with no medical history was referred to our service with approximately 2 weeks of headache and 2 episodes of vomiting. Detailed history from his parents revealed a change in behavior, including apathy and uncharacteristically poor university grades, over the prior 12 months. With the exception of a right pronator drift, his neurological examination was normal. CT brain revealed a large extra-axial heterogeneous mass involving the left frontal and temporal lobes with well-defined lobulated margins and avid enhancement of the high-density areas. The lesion eroded the inner table of the left frontal bone with the outer table almost completely eroded []. MRI brain with contrast demonstrated a large extra-axial ovoid heterogeneously enhancing left frontal convexity mass measuring 6.2 cm ×7.1 cm ×7 cm []. There was considerable susceptibility artifact, suggesting calcification and/or hemorrhage. There was also evidence of destruction and erosion of the calvarium, and a small extracranial component of the tumor was noted. The diagnosis of hemangiopericytoma was entertained and surgery planned.
Intraoperatively, the tumor was found to have breached the dura and as suggested by preoperative imaging, had invaded bone []. The involved bone (including the lateral sphenoid wing) and a generous cuff of dura were resected. The tumor was soft and moderately vascular. The tissue plane between tumor and brain was easily developed using standard microsurgical technique. A gross total resection was achieved. The dural defect was repaired with pericranial graft, and titanium mesh used to repair the bony defect.
The patient’s postoperative recovery was uneventful and he was discharged home day 5 after surgery. Postoperative MRI revealed no evidence of residual tumor. PET scanning did not reveal any other lesions. The patient was referred to a local specialist sarcoma unit for adjuvant chemotherapy with VIDE (vincristine, ifosfamide, doxorubicin, and etoposide), to be followed by focal irradiation and second round of chemotherapy with VAI (vincristine, dactinomycin, and ifosfamide).
The patient is now 1-year postsurgery with no clinical or radiological evidence of recurrence or metastatic disease.
Under light microscopy, the specimen showed a durally based, highly cellular tumor composed of relatively monotonous small blue cells with slightly irregular nuclei and thin rims of cytoplasm [] displaying PAS- positive material []. The mitotic rate was high at 34 mitoses per 10 high-power fields. There were numerous vessels, some displaying thrombosis and there were areas of hemorrhage and hemosiderin deposition. There was no necrosis. The tumor cells were positive for FLI-1, MIC-2, and vimentin []. The cells were negative for STAT6, CD 34, DUX-4, WT1, desmin, myogenin, ERG, synaptophysin, chromogranin A, pan-CK, EMA, P40, CK8/18, LCA, SOX10, CD 20, CD3, and TDT. Ki67 was positive in 30% of the cells, visually estimated. The specimen was sent for FISH and tested positive for EWSR1 (22q12) rearrangement.


A 2-year-old male toddler weighing 15 kg presented with a history of fever which is high grade continuous type associated with chills and rigors. The patient had cough (wet cough more in amount) whitish colour sputum not foul smelling. Swelling over face was present which initially started around peri-orbital (which is more during morning) and gradually progressed to face which decreases by evening. The toddler had decreased urine output (oligouria). The baby was delivered by C-section and weighed 2.75 kg after birth. On examination pitting type of oedema was present over lower limbs and swelling over face was present. Based on these clinical presentations, nephrotic syndrome was suspected and specific laboratory testing was performed to establish diagnosis.
The urine dipstick indicated for proteinuria, no signs of haematuria. Blood testing showed a significantly depressed C3 level of 0.638 g/L (reference interval 0.9-1.8 g/L) and hypoalbuminaemia of 2.0 g/dL (reference interval 3.5-5.5 g/dL) indicating nephrotic syndrome (NS). The urine creatinine level was – 620 mg/L (reference interval 400-3000mg/L) and APTT was prolonged- 47.7 Sec (reference interval 24-30 Sec). Serologic testing for active infections: anti-streptolysin-O titer was positive. The lipid levels were markedly increased as outlined in the . LDL was measured and calculated by enzymatic selective protection (Direct). The urine protein/creatinine ratio was found to be high (7.3). Mantoux test was done before administration of steroids which was negative.
After establishing diagnosis, optimal supportive treatment including Enalapril p.o., Prednisolone p.o., intravenous albumin, furosemide, low salt intake, high caloric and protein diet were given along with Ceftriaxone and Ascoril-LS. The urine output and blood pressure was monitored.
Successful control of peripheral oedema with the administration of albumin and diuresis with furosemide was seen. The peri-orbital oedema and leg swelling reduced, and there was a concomitant increase in serum protein levels. The lipid levels also gradually decreased in due course of time without any medication.


A 79-year-old man was first admitted to the Urology Clinic due to clinically verified tumor of the urinary bladder, when computed tomography (CT) urography revealed superficial urinary bladder tumor, near the right ureteral orifice. Transurethral resection of the tumor was performed, and the specimen was sent for histopathological analysis, which confirmed the diagnosis of papillary urothelial high-grade pT1 carcinoma. After that, intravesical BCG immunotherapy was initiated, according to protocol currently used in our institution, consisting of 6 weekly instillations in induction course of the therapy, followed by one-month break, and a further three instillations at monthly intervals. Upon completion of therapy with BCG, we re-examined the patient and, using ultrasound, found a change in the right kidney, resembling moth bites not seen on CT scan before TURBT ().
The patient was again admitted to our Clinic, for diagnostic evaluation and treatment of a newly discovered change in the right kidney (). Separate urine samples from each ureter for cytological analysis were obtained for three consecutive days. The presence of malignant cells was not detected in any of the specimens; however, signs of macrohematuria and inflammation were described in the cytological report. At the same time, we obtained a urine sample for mycobacterial culture on Löwenstein-Jensen medium, and additionally performed direct microscopy test with Ziehl-Neelsen stain, and they were both negative. The patient was afebrile, without any clinical signs of systemic infection, although he reported flu-like symptoms which were appearing only on therapy initiation and subsiding on symptomatic therapy. Additionally, CT-guided core-needle biopsy of the affected kidney was performed, and the specimen was sent for histopathological analysis, which revealed chronic necrotizing granulomatous inflammation. According to the histopathological findings, the diagnosis of renal tuberculosis was established () with signs of caseous necrosis ().
Antituberculotic therapy was initiated for 6 months. The initial empiric treatment was based on a 4-drug regimen: isoniazid, rifampin, pyrazinamide, and ethambutol. Upon completion of 2-month therapy, pyrazinamide and ethambutol were discontinued, and isoniazid 300 mg plus rifampin 600 mg were continued as a daily therapy for 4 more months, in addition to L-ornithine L-aspartate 150 mg, three times a day for liver protection, and ranitidine 150 mg, two times a day for the protection of gastric mucosa. Upon completion of antituberculotic therapy, control CT-scan () was performed at follow-up, indicating regression of changes on the right kidney.


A 62-year-old woman visited our hospital with a 1-day history of lower abdominal pain and bulging from a right groin lesion. Her physical examination showed a right inguinal bulge and pain. Her abdomen was not distended and was without rebound tenderness and muscular defense. However, the bulge in the right groin lesion could not be reduced manually. Contrast-enhanced computed tomography showed a hypodense rounded lesion medial to the right femoral vessels () with fluid collection (), with no intestinal obstruction or ischemia. Based on these physical and radiological findings, the patient was diagnosed as having a right incarcerated femoral hernia containing fat tissue. As there were no clear findings of intestinal obstruction, elective TEP repair was performed 1 day after admission following preoperative evaluation of her general condition. An infra-umbilical incision was made under general anesthesia. The preperitoneal space was created with blunt dissection with the aid of CO2 gas insufflation to a pressure of 12 mmHg. Two 5-mm trocars were inserted into the left mid-abdomen. The hernia sac had herniated into the right femoral canal (). Therefore, we diagnosed a right incarcerated femoral hernia containing greater omentum. However, there were no findings of abscess formation or small bowel necrosis. We were able to release the hernia sac using laparoscopic forceps (). After reduction of the hernia sac, 10.3 × 15.7 cm polypropylene mesh was placed in the preperitoneal space and fixed to Cooper’s ligament with a fixation device (ProTack™, COVIDIEN) (). Intraperitoneal observation was not performed because we considered it unnecessary to confirm the presence of intestinal damage. The patient’s postoperative course was uneventful, and she was discharged from our hospital 3 days after surgery. Presently, at 1 month after surgery, the patient is doing well without recurrence or mesh infection.


A 31-year-old pregnant female at 27 weeks gestational age visited our hospital with symptoms of acute epigastralgia, which had moved down to the right lower intestinal quadrant, and vomiting. She was gravida 8 and para 7, with no history of abdominal surgery. The blood exam showed an elevated white blood cell count (17 200/μl) and a high C-reactive protein level (2.01 mg/dl). The appendix could be detected with abdominal ultrasonography; it was inflamed and swollen to a length of 12 mm. A computed tomography (CT) scan was performed to confirm the degree of appendix inflammation and its location, showing the dirty fat sign and a swollen appendix located underneath the liver, close to the gallbladder fundus (). The patient was clinically diagnosed with acute appendicitis. An emergency laparoscopic appendectomy was performed with the RPLA approach.
A lap protector with an EZ-access device and two 5-mm trocars were attached to the 20-mm umbilicus incision with the Hasson technique. CO2 insufflation was maintained at 10 mmHg. An additional 5-mm trocar was inserted in the right lower quadrant (). To gain a good field of view, the operation table was tilted to the left (). The inflamed appendix was covered with omental adhesion in the space between the liver and the extended uterus (). The appendix was dissected with laparoscopic coagulating shears and the pus was drained and collected (). A tocolytic agent was administered by the obstetrician to prevent preterm delivery.
The patient was allowed to drink clear fluids on the first postoperative day (POD), and solid food intake was started on POD3. Antibiotics were administered until POD4. The patient was discharged on POD8 without any postoperative complications (). A vaginal delivery was achieved at term.


The patient was a 54-year-old housewife (gravida 3, para 2, 1 living child, 1 deceased child) who lived in a village. She had been using an Intrauterine Device (IUD) for 12 years after her second delivery to prevent pregnancy and later, withdrawal method until her menopause. The onset of menopause was at the age of 47 and 1 year later, the patient experienced hot flashes, for which she has not sought any treatment. She never had a pap smear or mammogram. She reported no history of trauma or the use of hormonal medicines. At the age of 52 (05/06/2016), she lost her 24-year-old son (her second child) in a car accident and was subsequently diagnosed with hypertension and diabetes. She reported a history of using fluoxetine capsules (20 mg per day), methyldopa (3 times per day or TDS), metformin 500 mg (2 times per day or BID), and 5 units of insulin a day after the accident and before her pregnancy. She had encountered no problems in her two previous pregnancies. Unaware of her pregnancy (at 20 weeks pregnant), the patient referred to a general physician in her village with the complaint of vaginal bleeding associated with no pain.
Despite the physician's advice, she did not permit abdominal and vaginal examination. Therefore, he administered hormonal medications. For the first time in 14/3/2018, she referred herself to a health center in Arak, Iran, with the complaint of gradual abdominal enlargement and her pregnancy was diagnosed after requesting a transabdominal ultrasound at 28 weeks. Later, prenatal care was initiated. The patient did not continuously follow-up with her prenatal care. She was hospitalized due to vertigo and epistaxis caused by hypertension and hyperglycemia (350 mg/dL) at 34 weeks of gestation in another city. On 28/04/2018, she gave birth to a girl (weight: 1780 g; height: 47 cm; circumference of head: 33 cm; and Apgar score: 7.8) through the cesarean section with her gynecologist's opinion. The newborn was hospitalized in the Neonatal Intensive Care Unit (NICU) due to prematurity and icterus and was fed through a Nasogastric (NG) tube during this period. Her newborn was discharged from the hospital in good general condition 20 days after birth and formula feeding was continued.
After delivery, metformin 500 mg (BID) and amlodipine 5 mg were administered to the mother once a day instead of methyldopa. This information was obtained from the patient's medical file. On the third visit (45 days after delivery), the mother's blood pressure was 130/80 and the infant's weight was 2300 g. It should be noted that unfortunately the patient and her husband, especially her husband, did not effectively communicate with health providers. They were reluctant to answer questions and seemed to be very anxious and worried.
The authors certify that they have obtained a consent form from the patient. In the form, the patient has given her consent for her clinical information to be reported in the journal. The patient understood that her name and initials would not be published and due efforts would be made to conceal her identity.