A 14-year-old male neutered domestic mediumhair cat was referred for investigation of
4 months of inappetence and 1.2 kg weight loss. Diabetes mellitus had been diagnosed
2 years earlier and was well managed with insulin glargine (Lantus; Sanofi) with
doses up to 4 IU subcutaneously (SC) q12h and a high-protein/low-carbohydrate diet
(Hill’s m/d; Hill’s Pet Nutrition). In the month prior to referral the insulin dose
had been reduced to 2 IU SC q12h based on blood glucose curves.
Physical examination revealed a low body condition score (3/9) and a grade II/VI left
systolic cardiac murmur. Pertinent abnormalities on haematology and biochemistry
included a mild microcytic regenerative anaemia (packed cell volume [PCV] 24%
[reference interval (RI) 30–45%], mean cell volume 30.8 fl [RI 40–45 fl], absolute
reticulocyte count 326.8 × 1012), mild neutrophilia (15.35 ×
109/l; RI 3.76–10.08 × 109/l) and increased alkaline
phosphatase activity (76 IU/l; RI <50 IU/l). Urinalysis was unremarkable.
Abnormalities identified on abdominal ultrasound included a 5.0 cm diameter, large,
heterogeneous and poorly vascularised mass within the right medial lobe of the
liver, which was fluid-filled centrally (), and an eccentrically located,
intramural (2.0 cm × 1.4 cm) hypoechoic mass within the distal portion of the
transverse colon with loss of normal wall layering (). Thoracic radiographs were
unremarkable.
Fine-needle aspirate cytology of the masses was non-diagnostic. Coagulation times
were within the RIs. The cardiac murmur was not investigated owing to financial
constraints. Colonoscopy revealed a smooth, raised mass in the transverse colon and
biopsies were collected (). An ultrasound-guided liver mass biopsy (Vet-Core biopsy needle 16 G 9
cm; Smiths Medical USA) was performed and an oesophageal feeding tube was
placed.
Histopathology of the colonic and hepatic masses revealed disorganised large pale
pleomorphic spindle cells with prominent nucleoli. The colonic mass was suspected to
be a primary spindle cell sarcoma with the liver mass representing a metastasis.
Pre- and post-contrast enhanced CT of the abdomen was performed for tumour staging
and surgical planning. A heterogeneous soft tissue-attenuating 4.3 cm mass was noted
within the right medial lobe of the liver (). This mass was heterogeneously
enhancing post-contrast administration with multiple, coalescing
non-contrast-enhancing areas suggestive of necrosis. Within the transverse colon,
there was a hypoattenuating mass (2.4 cm × 1.4 cm × 0.6 cm) with heterogeneous,
predominantly peripheral enhancement post-contrast administration ().
Laparotomy was performed under general anaesthesia. The hepatic mass was separated
from the gall bladder by blunt dissection and right medial and quadrate liver lobe
resection performed because of shared vasculature (). The rest of the liver had a
diffusely mottled appearance and a 6 mm punch biopsy was collected from the left
medial lobe. The colonic mass was identified at the hepatic flexure of the colon and
removed by colectomy and anastomosis. Postoperative analgesia was provided by a
fentanyl continuous rate infusion (5 μg/kg/h for 48 h) followed by buprenorphine
(0.015 mg/kg IV q8h for 24h) and then 0.02 mg/kg sublingually for another 48 h.
The feeding tube was removed after 2 weeks. Postoperative medications included:
amoxicillin-clavulanate (Augmentin; GlaxoSmithKline) 20 mg/kg intravenously (IV) q8h
for 48 h then orally (PO) for 7 days; metronidazole (Metronidazole Sandoz IV;
Sandoz) 10 mg/kg IV q12h for 48 h then PO for 7 days; enrofloxacin (Baytril; Bayer)
2.5 mg/kg SC q12h for 48 h then PO for 7 days; maropitant (Cerenia; Zoetis) 1 mg/kg
SC q24h for 48 h then PO for 5 days; and insulin glargine 1 IU SC q12h.
Histopathology of the colonic mass () revealed a poorly circumscribed, unencapsulated,
well-demarcated, densely cellular, infiltrative neoplasm composed of spindle cells
arranged in interlacing streams and bundles separated by a scant eosinophilic
fibrillary matrix that was transmurally infiltrating and effacing the mucosa, lamina
propria, submucosa, tunica muscularis and serosa, and extending into the colonic
lumen. Neoplastic cells had variably distinct cell borders, moderate-to-abundant
eosinophilic fibrillar cytoplasm, oval-to-elongated nuclei containing finely
stippled chromatin and generally one distinct nucleolus. The mitotic rate averaged
one per 10 high-powered fields. There was marked anisocytosis and anisokaryosis. In
addition, there was scattered necrosis and lymphoplasmacytic aggregates. The mass
appeared to have been completely excised. Similar neoplastic infiltrates admixed
with scattered single-cell necrosis infiltrated and replaced the architecture of the
right medial liver lobe ().
The cat was diagnosed with a colonic malignant spindle-cell neoplasm with hepatic
metastasis. Immuno-histochemistry revealed that both masses stained positive for
S-100 protein, vimentin and glial fibrillary acidic protein, very weakly positive
for c-kit and negative for muscle-specific actin and CD18, consistent with a
diagnosis of a MPNST (–).
Metronomic chemotherapy was commenced 2 weeks postoperatively with cyclophosphamide
15.5 mg/m2 PO q24h. Cyclophosphamide was well tolerated long term.
Intermittent vomiting occurred and was treated with maropitant 1.5 mg/kg PO q24h and
omeprazole (Losec; AstraZeneca) 1.5 mg/kg PO q24h as required. Haema-tology
performed at 1, 2, 3 and 5 months revealed microcytosis for 3 months before
resolution. Insulin glargine was increased to 3 IU SC q12h by 5 months
postoperatively, based on blood glucose curves performed every 1–2 months. The cat
gained 200 g following surgery and maintained a stable body weight.
Sixteen months postoperatively, the cat was re-presented to the referring
veterinarian for polyuria and polydipsia. Fructosamine indicated over-control of
diabetes mellitus (203 µmol/l; RI 221–340 μmol/l), hyperthyroidism was diagnosed
(total thyroxine 92 nmol/l; RI 10–60 nmol/l), and pertinent abnormalities on
haematology and biochemistry included a mild anaemia (haematocrit 24%; RI 30–52%)
and increased alanine aminotransferase activity (138 IU/l; RI 12–130 IU/l). The
insulin dose was reduced to 2 IU SC q12h and methimazole transdermal gel 5 mg q12h
was commenced.
Eighteen months postoperatively the cat re-presented following 3 days of progressive
lethargy and inappetence. Physical examination revealed 100 g of weight loss, marked
lethargy, pallor, a right-sided goitre and a grade II/VI left systolic cardiac
murmur. Haematology revealed a marked non- or pre-regenerative anaemia (PCV 10%).
Coagulation times were prolonged (prothrombin time 39 s [RI 15–22 s] and activated
partial thromboplastin time >300 s [RI 65–119 s]). Abdominal ultrasound
identified multiple renal and hepatic nodules ( in the ). Thoracic radiographs were unremarkable.
Retroviral testing was negative (Witness FIV-FeLV; Zoetis). The owner elected
euthanasia. Post-mortem examination, including renal immunohistochemistry, confirmed
the presence of an MPNST within the kidneys and liver ( in the ). Additional findings of note observed
incidentally on gross and microscopic post-mortem examination included thyroid
cystic follicular adenomas, cardiac fibrosis and islet amyloidosis.


An otherwise healthy 19-year-old woman presented with 10 days of severe sore throat, odynophagia, and fever to a local health facility in Martha’s Vineyard, Massachusetts. Despite treatments with oral steroids, clindamycin, and penicillin on 2 separate occasions, her symptomatology persisted. Otolaryngology service was consulted as she returned home to Ohio after 2 weeks. Upon further questioning, she did admit to interacting with a rabbit. Physical examination revealed bilateral 2-cm tender, nonfluctuant cervical adenopathy without skin changes, 2+ mild necrotic tonsils (), and diffuse aphthous ulcerations throughout the posterior pharyngeal wall, tongue base, and palate. There was no involvement of the hypopharynx or larynx on flexible laryngoscopy. A respiratory panel with the most common types of viral and bacterial pathogens was negative. Infectious disease service placed the patient on empiric intravenous antiviral, antifungal, antibacterial, and steroid medications. Her symptoms subsided, and she was subsequently discharged. However, she had multiple readmissions due to relapsing fevers and cervical adenopathy. A contrast-enhanced computed tomography (CT) demonstrated bilateral cervical adenopathy with focal necrosis (). Excisional biopsy revealed friable lymphoid tissue with copious amount of purulent exudate bilaterally. Pathology showed lymphoid material with pyogranulomatous and necrotizing granulomatous inflammation. Histological stains were negative for mycobacteria and fungal organisms. Viral cytopathic effect was not identified. Ultimately, universal polymerase chain reaction was positive for Francisella tularensis. The patient was then treated with intravenous gentamicin and ciprofloxacin and responded appropriately.


A 54-year-old male presented with sudden appearance of new hairs over the dorsal aspect of his left foot []. It was preceded by severe itching and burning pain. On close observation, there were straight brown easily pluckable coarse hairs, quite distinct from his other body hairs, with surrounding slight erythema and scales. On further probing, the patient gave a history of removing caterpillar from his shoes a day prior. His routine blood investigations were unremarkable. Under the light microscope, at ×40 magnification microscopy, these hairs appeared light brown with cealomic appearance, very unlike human hair []. Histology of the lesion was suggestive of acute spongiotic eczema. Entomological examination of the caterpillar, which patient brought from his home, revealed an oak processionary caterpillar i.e., Thaumetopoea processionea [] having same hairs/setae as observed over the patient's foot. The patient's symptoms improved with the removal of hairs with cellophane tape initially and later by forceps along with oral levocetirizine 10 mg at night and twice daily application of topical mometasone furoate 0.1% w/w cream.


A 14-month-old male castrated domestic shorthair presented with a recent history of four self-limiting generalised tonic–clonic seizure-like episodes over a period of 1 month. The seizures were characterised by loss of consciousness, autonomic dysfunction (urination, defaecation, ptyalism) and tonic–clonic involuntary movement of all four limbs. These episodes lasted 2–3 mins and were accompanied by a postictal phase 30–120 mins in length, characterised by ataxia and a depressed mentation. In between the seizures, the cat was reported to act normally.
Two months prior to the episode history the cat was involved in a road traffic accident, causing irreparable left hindlimb trauma resulting in an amputation. General physical examination was otherwise unremarkable. No abnormalities were noted on neurological examination.
Based on the presence of generalised tonic–clonic seizures the cat was suspected to have a disease process affecting the forebrain.
Results of haematological examination, including smear evaluation, identified mild microcytosis (mean cell volume 41.2 fl; reference interval [RI] 41.3–52.6 fl), while white blood cell morphology was otherwise unremarkable. Complete serum biochemistry analysis was unremarkable. Preprandial bile acid was unremarkable while blood ammonia was mildly elevated (89.9 μmol/l [RI 0–50 μmol/l]). Cerebrospinal fluid analysis, including total protein, total nucleated cell count and cytology, was unremarkable. Serum cobalamin level was elevated (1294 pmol/l [RI 231–617 pmol/l]).
An MRI study of the brain using a 1.5 T magnet (Philips Ingenia CX) was performed with the cat under general anaesthesia. The following sequences with a slice thickness of 2 mm were acquired: sagittal T2-weighted (T2W) turbo spin echo (TSE) and transverse T2W TSE, fluid-attenuated inversion recovery (FLAIR), T1-weighted (T1W) spin echo (SE), diffusion-weighted imaging with selection of three b-values (b 0, b 500, b 1000) and susceptibility-weighted imaging (VEN_BOLD). A three-dimensional (3D) T1W turbo field echo series with a slice thickness of 700 µm was also acquired. Following intravenous administration of gadolinium-based contrast medium (Prohance; Bracco Diagnostics) at a dose of 0.1 mmol/kg the transverse T1W SE and 3D TW1 TFS series were repeated.
There was marked T2W hyperintensity of the grey matter throughout the cerebellum and part of the mesencephalon ( and ), with no suppression on FLAIR and no associated contrast enhancement. This T2W hyperintensity was bilaterally symmetrical affecting the caudal cerebellar peduncles, the deep cerebellar nuclei (dentate, interposital and fastigial) () and the rostral and caudal colliculi ().
Given the MRI findings and the cat’s clinical presentation the presumptive diagnosis was an inherited neurometabolic disorder. Urine was submitted for mucopolysaccharide, amino acid and organic acid screening. A marked elevation of 2-hydroxyglutaric acid was identified. Repeat sampling 35 days later confirmed the same marked elevation of urinary 2-hydroxyglutaric acid.
Following MRI, the cat was started on 2 mg/kg oral solution of phenobarbitone q12h that was subsequently increased to 3 mg/kg q12h following a recrudescence of seizure activity. Following a telephone update at 12 months after diagnosis, the cat remains alive and has seizure episodes every 2–3 months.


A 62-year-old female with a medical history of controlled hypertension on an angiotensin-converting enzyme inhibitor presented to the cardiovascular center with abrupt, typical angina associated with pre-syncope and palpitations. Her vital signs and physical examination were unremarkable. A 12-lead electrocardiogram revealed sinus tachycardia with a right bundle branch block. Pertinent diagnostic laboratory investigations included an elevated erythrocyte sedimentation rate 48 mm/hr (normal 0–30 mm/hr), d-dimer 2757 ng/dL (normal≤500 ng/mL), pro-brain natriuretic peptide 858 pg/mL (normal≤300 pg/mL), cardiac biomarkers, CK-MB 25U/L (normal 0–20 U/L), troponin I 1.36 ng/mL (normal 0.0–0.15 ng/dL). The patient’s complete blood count, comprehensive metabolic panel, fasting lipid panel and HbA1c were within normal limits, and she was subsequently initiated on non-ST-segment myocardial infarction (NSTEMI) pharmacotherapies including aspirin, clopidogrel, enoxaparin, and high-intensity rosuvastatin.
Initial transthoracic and subsequent transesophageal echocardiography demonstrated a large 42 mmx40 mm mass arising from the left atrium and adherent to the inter-atrial septum (see and ). There was preserved left ventricular systolic function with an estimated ejection fraction of 65% and no regional wall motion abnormalities. The left atrial dimensions were normal, however, there was prolapse of the myxoma, obstructing both the mitral valve orifice during atrial systole and the left ventricular outflow tract (see and ). Diagnostic coronary angiography demonstrated mild, non-obstructive coronary artery disease with thrombolysis in myocardial infarction (TIMI) 3 antegrade flow (see and ). Urgent open-heart surgery was performed the following day, and gross pathology of the specimen excised revealed a large polypoidal mass. The mass suspected to be a myxoma was excised by resecting the pedicle implanted on the interatrial septum (). Histopathological examination of the specimen confirmed the diagnosis of myxoma with papillary fragments of tumor composed of fibro-myxoid hyalinized cores with elongated stellate shaped myxoma cells with abundant eosinophilic cytoplasm. The patient’s postoperative course was uneventful, and she was safely discharged on optimal medical therapy with routine follow-up appointments.


A 23-year-old woman reported dissatisfaction with the visual appearance of her smile due to the presence of yellowish teeth, generalized anterior diastema, and a gummy smile (). Based on a clinical and radiographic examination, the following treatment plan was proposed: periodontal plastic surgery, tooth whitening, and cementation of ceramic veneers on the upper and lower arches. The patient signed an informed consent form, and routine preoperative tests (blood count, coagulation, and fasting glucose) were requested. No abnormalities were found.
A 1325-nm Swept-Source OCT device (Thorlabs, Newton, NJ, USA) was used to image the front teeth. For the OCT readings, the patient remained seated with her head stabilized and perpendicular to the laser light source. The positions of the chin and glabella were standardized for all imaging sessions. Sagittal images of the cervical (), middle (), and incisal thirds () of the right maxillary central incisor were obtained through OCT before the treatment. It was possible to observe the enamel, dentin, dentin-enamel junction, gingiva, gingival sulcus, and incisal border.
An intraoral mock-up with A1 bis-acryl resin (Protemp 4; 3M, Maplewood, MN, USA) was constructed (). Periodontal plastic surgery was performed with the aid of a surgical guide. The bands of the keratinized soft tissue around the teeth were removed to obtain a parabolic shape. A flap was raised, exposing the local bone tissue. Osteotomy and osteoplasty were performed, aiming to restore the biological width. The flap was repositioned and sutured.
Gingival health was evaluated clinically and through OCT after 15 days and 2 months of tissue healing. The final sagittal OCT image of the cervical third of the right maxillary central incisor, when compared with the initial image and the image taken at 15 days, demonstrated gingival recovery, as shown by the formation of a new gingival sulcus around the teeth (). Clinically, gingival health was observed. In addition, close contact of the gingiva with the tooth, as present before surgery, was shown in the OCT images. The gingiva was in the process of healing and reestablishment of its position around the tooth 15 days after the periodontal surgical procedure. The periodontal tissue showed normal patterns of healing given the formation of junctional epithelium (15 days) and maturation of connective tissue (45–60 days after periodontal surgery).
The patient was given the necessary material for at-home vital bleaching and was instructed to use a 16% carbamide peroxide whitening agent in a tray for 4 hours once a day for 2 weeks.
After the bleaching treatment was completed (), minimal tooth preparations for ceramic veneers were carried out with diamond burs (KG Sorensen, Cotia, São Paulo, Brazil) and Sof-lex™ polishing discs (3M, Maplewood, MN, USA). Gingival retraction was performed to allow access for the impression material to record the finishing line of both arches. The impression material used was addition silicone (Express; 3M, Maplewood, MN, USA). Face bow and bite records were also taken.
The impressions were cast in type IV plaster and the physical models were mounted on a semi-adjustable articulator (A7 Plus; Bio-Art Soluções Inteligentes, São Carlos, SP, Brazil) and IPS e.max (Ivoclar Vivadent AG, Schaan, Liechtenstein) ceramic veneers were made.
The fit and the gingival adaptation of the veneers in the mouth were checked and the color shade of the cement was selected using Variolink Veneer Try-in pastes (Ivoclar Vivadent AG, Schaan, Liechtenstein).
The ceramic veneers were etched with 5% hydrofluoric acid (IPS Ceramic Etching Gel, Ivoclar Vivadent AG, Schaan, Liechtenstein) for 20 seconds, washed, and dried. The restorations were silanized (Monobond Plus, Vivadent AG, Schaan, Liechtenstein) for 1 minute. The adhesive (ExciTE F, Vivadent AG, Schaan, Liechtenstein) was applied to the internal face of each veneer and polymerized for 15 seconds (Radii-call LED curing light, SDI, Australia).
The teeth were etched with 37% phosphoric acid (Total Etch, Vivadent AG, Schaan, Liechtenstein) for 15 seconds, washed and dried. The Adhesive (ExciTE F, Vivadent AG, Schaan, Liechtenstein) was applied to the dental enamel and polymerized for 15 seconds (Radii-call LED curing light; SDI, Bayswater, Australia).
The ceramic veneers were cemented with photopolymerizable resin cement (Variolink Veneer; Vivadent AG, Schaan, Liechtenstein) one by one, starting with the central teeth (). The veneers were placed on the teeth under slight pressure followed by the removal of excess resin cement and polymerization for 30 seconds (Radii-call LED curing light, SDI, Bayswater, Australia). For the removal of remaining excess cement, scalpel blades, finishing and polishing discs, and abrasive polyester strips were used.
The sagittal OCT images after veneer cementation showed gingival health () and micrometer-sized bubbles in the cementation line (). Given that the treatment was considered satisfactory, the patient was instructed about 6-month recall dental appointments to assess the health of the hard and soft tissues and the integrity of the veneers.


A 63-year-old man presented with exertional dyspnea in the background of normal cardiac evaluation, and computer tomography (CT) findings of a vascular malformation in the posterior mediastinum (). The malformation was supplied by 4 arteries (right bronchial artery and three intercostals) arising from the thoracic aorta with a large vascular mass in the posterior mediastinum and single drainage vessel into the right upper lobe pulmonary artery (). The appearance was consistent with a BP-AVM.
In view of the size and the risk of hemoptysis, the patient was referred for embolization. Through a left common femoral vein access, the right upper lobe pulmonary was accessed. The drainage vessel arising the superior segmental artery of the right upper lobe was first cannulated. However, due to the angulation of the artery with sheath instability, a tri-axial system was needed to allow deep catheterization required for vascular plug embolization (). This consisted of an 8.5F Agilis NxT (St Jude Medical, Minnetonka, MN, USA) steerable sheath positioned in the ostium of the segmental artery, followed by an 8F Mach 1 guiding catheter (Boston Scientific, Natick, MA, USA) and a 5F 125 cm long Ultimate 1 diagnostic catheter (Merit Medical Systems, Inc., South Jordan, UT, USA) inserted co-axially. In a telescoping fashion, the guiding catheter was advanced over the diagnostic catheter and guidewire to reach a stable position within the segmental pulmonary artery, and a 16 mm Amplatzer AVP II vascular plug (St Jude Medical) was deployed, occluding the solitary drainage pulmonary artery ().
Through a right common femoral artery access, aortography demonstrated the 4 feeding arteries as seen on CT (). The plan was for staged coil/plug embolization (without particulate embolics) of the feeding arteries (2 arteries in a single sitting) due to the difficulty in resolving any potential spinal supply. If needed, we would return for an interval embolization of the remaining two vessels. The largest supplying artery (intercostal artery) was first cannulated and a 6F Brite-tip sheath (Cordis, Miami, FL, USA) was used to deliver a 12 mm AVP II plug successfully deployed (). The hypertrophied right bronchial artery was then accessed using a Progreat microcatheter and embolized using a total of 8 Interlock coils ranging from 5 to 12 mm in size (Boston Scientific) (). Completion angiography showed complete embolization of the two vessels with significant slow flow into the malformation through the remaining two untreated arteries ().
The immediate post-embolization CT at day 1 showed significant thrombosis of the BP-AVM () and the patient was discharged well. At 6 months follow-up CT, complete thrombosis with a significant reduction in size of the BP-AVM was demonstrated (). The patient reported improvement in effort tolerance from previous modified Medical Research Council score (mMRC) of 3 to 1 over the same period and remained so at 1 year.


A 47-year-old woman was referred for a dysfunctional brachial–basilic arm fistula. Assessment of her left radial artery revealed a 2.1 mm caliber and a type A waveform on the modified Barbeau test. Our institution’s protocol is to use transradial access for tandem lesions in both the juxta-anastomotic and drainage vein so that all the lesions could be treated in a single retrograde fashion. Another advantage of transradial access is ease of hemostasis by manual compression over the puncture site. A 6F radial sheath (Glidesheath Slender, Terumo, Tokyo, Japan) was inserted into the artery at the distal radius. A short focal stenosis in the proximal basilic vein that was resistant to angioplasty via a Mustang high pressure balloon (Boston Scientific, Marlborough, MA, USA) was encountered (), and a 6×20 mm cutting balloon (Boston Scientific, Natick, MA, USA) was then inflated to nominal pressure, successfully effacing the stenosis (). The cutting balloon was completely deflated and retrieved.
After removal of the cutting balloon, blood was found to be spurting from around the vascular sheath (peri-sheath bleeding). Suspecting a lacerated sheath, a new 6F sheath was exchanged over the indwelling guidewire. Despite the new sheath in place, peri-sheath blood spurting persisted. A blood pressure cuff was then inflated (30 mmHg above the systolic pressure) over the proximal forearm for temporary hemostasis and a radial pressure bandage (STEPTY™P, NICHIBAN Co., Ltd., Tokyo, Japan) was applied over the sheath entry site, which halted the hemorrhage. Inspection of the removed sheath revealed a full-length laceration of the sheath by the atherotome of the balloon. A 5F sheath was inserted into the basilic fistula as a second access and antegrade arteriography of the radial artery was obtained. Laceration with perforation of the radial artery was performed beyond the tip of the sheath (). The radial artery was crossed using a V18 guidewire (Boston Scientific, Marlborough, MA, USA), the radial sheath was removed, and a 3×10 mm Sterling balloon (Boston Scientific) was inflated over the distal radial artery including at the entry site for 10 min (). Simultaneous external pressure with the radial pressure bandage was re-applied. At the end of the balloon inflation, angiography (without releasing the radial pressure bandage) showed resolution of the previously demonstrated laceration (). The radial pressure bandage was then left for the next three hours for complete hemostasis.
After three hours, on removal of the radial bandage, arterial spurting occurred again at the site of sheath entry. Once again, application of the forearm blood pressure cuff and reapplication of the radial pressure bandage was used to secure the hemostasis. At this time, the idea that the laceration of the radial artery was more extensive and severe than initially believed and likely beyond conventional endovascular salvage like stent grafting was thought. A vascular surgical consult was made for surgical repair. Due to the distal radial location, radial artery ligation was the surgery of choice. As such, embolization of the radial artery was thought to be a reasonable and conceptually similar alternative. Through the basilic vein access, a 5F Ber catheter was used to deposit a total of three 4 mm×7 cm Macro-Nester coils (Cook, Bloomington, IN, USA), extending across the puncture site from the distal to mid radial artery (). Completion angiography confirmed successful embolization of the distal radial artery and good ulno-palmar collateral flow (). Cessation of the puncture site hemorrhage was noted on removal of the radial bandage. The patient was discharged the next day and remained asymptomatic with a functioning fistula at a 6-month follow-up.


A 70-year-old male patient with a previous history of multiple sclerosis, previously successfully resected colon cancer 10 years earlier and late-onset asthma (age of 62 years) presented with retrosternal chest pain, small troponin rise, and without ECG changes. Physical examination revealed no abnormalities of the cardiovascular and respiratory system. Echocardiography showed normal left ventricular function with no regional wall motion abnormality and no valvular disease. A diagnosis of non-ST elevation myocardial infarction was made, and diagnostic coronary angiography showed a smooth stenosis in the right coronary artery (RCA) and unobstructed left coronary artery (, Videos S1 and S2). He was listed for angioplasty to the RCA but upon reimaging, the RCA looked smooth and unobstructed. However, the mid-circumflex artery now had a smooth stenosis which resolved with administration of intracoronary isosorbide dinitrate (, Videos S3 and S4). A diagnosis of coronary artery spasm was made, and the patient was treated with aspirin and he was started on oral isosorbide mononitrate. A month later he was admitted with anterior ST-segment elevation which completely resolved with administration of sublingual glyceryl trinitrate in the emergency room. The chest pains continued intermittently at rest and diltiazem and nicorandil were added in stepwise fashion to try and settle ongoing symptoms. Cardiac magnetic resonance (CMR) imaging revealed small areas of myocardial infarction in both the RCA and the LAD territories. Due to concern over possible ongoing infarction due to spasm, clopidogrel 75 mg o.d. was added and continued long-term. Over the next 18 months, the patient remained stable, with minimal symptoms and no changes on serial CMR scans at 6 monthly intervals. He was continued on maximal antianginal therapy, dual antiplatelet therapy, and advised to avoid beta-blockers and phosphodiesterase 5 inhibitors.
He subsequently represented with further episodes of atypical chest pain at rest, unresponsive to glyceryl trinitrate (GTN) and associated marked troponin rise (troponin I > 400 ng/L [normal range <16 ng/L]), that fluctuated over a couple of months with no correlation with symptoms or any ECG changes. Full blood count was normal apart from raised eosinophils (4.3 × 109/L [normal range 0.02–0.5 × 109/L]) with normal renal and liver function tests. Erythrocyte sedimentation rate (ESR) was normal (<5 mm/h) and C-reactive protein (CRP) 5.7 mg/L. Over the next few weeks, CRP rose to 28 and troponin fluctuated, albeit markedly elevated, with no correlation with symptoms. Coronary anatomy was revisited with a CT coronary angiography which showed a calcium score of 202 with no obstructive lesions.
Repeat CMR (Figure) revealed normal right and left ventricular volumes and function. In the late gadolinium phase, there were two separate regions of infarction. There was infarction in the inferior/inferoseptal wall from the basal to apical level. This was predominantly transmural (>50%) in the inferior and inferoseptal wall at the basal to mid-level. There was some new extension into the basal inferolateral wall not seen on the previous scan that was also transmural. Separately, there was localized infarction (>50% transmurality) in the basal anterior wall.
Given the presence of eosinophilia, recurrent episodic myocardial infarction and the lack of correlation between the extent of epicardial coronary disease and CMR findings, combined with the suspicious history of late-onset asthma, a diagnosis of Churg–Strauss syndrome was postulated. The patient was treated with prednisolone 30 mg o.d. and azathioprine 50 mg t.d.s. Following immunosuppressive therapy, the chest pains resolved with corresponding normalization of eosinophil and troponin levels. antineutrophil cytoplasmic antibody (ANCA) levels checked in the non-acute phase was normal with myeloperoxidase (MPO) <0.3 (normal range < 3.5 IU/mL) and PR3 < 0.7 (normal range < 2.0 IU/mL). Nine months after treatment began, his medication has been gradually weaned to prednisolone 5 mg o.d. and azathioprine 50 mg o.d. with a plan for slow ongoing tapering of both.


We describe the case of a 50-year-old man who had been diagnosed with Behçet’s disease (BD) 14 years previously and met the international criteria. He had first presented with recurrent oral aphthae at the age of 15 and presented 5 years later with simultaneous and recurrent genital ulcers with a clear positive pathergy test. The disease progressed with panniculitic lesions on the inferior limbs, bilateral auricular chondritis (MAGIC syndrome) and asymmetrical polyarthritis. Following the diagnosis of BD, he was initially treated for 3 years with corticosteroids and colchicine, but with only partial improvement. More exuberant oral and genital ulcers justified the initiation of thalidomide, initially at 50 mg per day but increasing to 100 mg per day 2 months later. Less than a month after beginning therapy, the patient showed complete remission of the genital ulcers and marked improvement of the oral aphthae, polyarthritis and auricular chondritis.
However, 8 months later, the patient developed paraesthesia of the lower limbs. As these symptoms did not improve when thalidomide was reduced to 50 mg, an electromyogram was performed and revealed sensitive peripheral neuropathy. Thalidomide was suspended, and the patient was medicated with azathioprine together with corticosteroids and colchicine, but showed only a partial response. Later, due to worsening of BD symptoms and the development of abdominal pain and bloody diarrhoea (with colonoscopic evidence of colonic involvement), the patient was treated with adalimumab with an excellent response. However, 4 months later, he presented hilar and mediastinal adenopathy with pulmonary interstitial involvement; biopsy confirmed pulmonary sarcoidosis probably induced by adalimumab. Total reversal of the pulmonary lesions was observed when adalimumab was suspended. However, the BD worsened with erythema nodosum requiring high doses of corticosteroids, with the consequent development of iatrogenic diabetes and lumbar osteoporosis. The oral and genital ulcers persisted, causing a decrease in quality of life.
Lenalidomide was proposed and approved and the patient was started on 5 mg per day. There was immediate improvement, and the corticosteroid dose was reduced with no signs of peripheral neuropathy or haematological toxicity. Twenty months later there had been no new episodes of oral or genital ulcers and there was total remission of the bilateral auricular chondritis. The most recent electromyogram shows absence of motor or sensitive polyneuropathy of the lower limbs.


A 66-year-old man was transferred to our hospital for multiple organ failure caused by severe pneumonia. He had pre-existing chronic heart failure (New York Heart Association class II), with 40% left ventricular ejection fraction (LVEF) caused by atrial fibrillation.
On examination, his vital signs were as follows: Glasgow Coma Scale score, 15 (E4V5M6); blood pressure, 139/86 mmHg with 0.15 μg/kg/min norepinephrine infusion; heart rate, 192 b.p.m.; respiratory rate, 24 breaths/min; body temperature, 38°C; and oxygen saturation, 92% with 10 L/min oxygen. Arterial blood gas analysis revealed hypoxia and metabolic acidosis with hyperlactatemia. Blood examinations revealed elevated inflammatory markers and impaired renal function (Table ). A transthoracic echocardiogram (TTE) showed diffuse left ventricular hypokinesis with 30% LVEF and no findings suggesting infective endocarditis. Computed tomography revealed consolidation of the lower left lung lobe. He was diagnosed with pneumonia, septic shock, acute kidney injury, and acute exacerbation of chronic heart failure.
After admission, antibiotics (meropenem, linezolid, clindamycin, and minomycin), hydrocortisone, vasopressors (norepinephrine and vasopressin), and landiolol were initiated. Continuous renal replacement therapy and mechanical ventilation therapy were also initiated. Before introducing ECMO, the ventilator was set to the airway pressure release mode (high airway pressure, 16 cm H2O; fraction of inspired oxygen, 0.6). Blood gas analysis showed pCO2 46.1 mmHg and pO2 116 mmHg. However, hyperlactatemia and tachycardia persisted, and purpura appeared over the limbs and trunk. The TTE findings on the second hospitalization day revealed refractory cardiac failure with 10% LVEF regardless of the improving oxygenation; thus, septic cardiomyopathy was suspected. His hemodynamics remained unstable; metabolic acidosis progressed despite intra-aortic balloon pump (IABP) placement on day 2. Streptococcus pneumoniae was found in the blood and sputum cultures. Because of his intractable shock, V-A ECMO was implemented as a bridging therapy on day 3. The pre-ECMO implantation Sequential Organ Failure Assessment (SOFA) score was 15 points, and Acute Physiology and Chronic Health Evaluation II score was 26 points.
Once V-A ECMO was initiated, the patient gradually recovered from the infection and circulatory failure and showed decreased serum lactate levels and cardiac index and LVEF recovery. He was successfully weaned from V-A ECMO on day 12 and IABP on day 13 (Fig. ). Abscessed left lower pulmonary lobe resection was carried out on day 16. He was taken off mechanical ventilation and continuous renal replacement therapy on day 19 and discharged home in an ambulatory condition on day 107. The TTE at discharge showed that his cardiac function had returned to baseline.


A 26-month-old, previously healthy boy was admitted with a 4-week history of recurrent head drops. Prior to this illness, he was well and achieving age-appropriate milestones. Three episodes of generalized seizures heralded the onset of illness, 3 months back, following which he had progressive decline in developmental milestones. He had a history of measles-like illness at the age of 1 year, despite receiving measles vaccine at 9 months of age. On examination, he was in a minimally conscious state. There were repetitive periodic myoclonic jerks at a regular frequency of 6-8 s. Motor examination revealed generalized central hypotonia. On investigation, electroencephalography (EEG) revealed periodic generalized complexes []. Magnetic resonance imaging (MRI) showed periventricular white matter changes []. The diagnosis of SSPE was confirmed with elevated (1:625) titers of cerebrospinal fluid (CSF) and serum anti-measles antibody (immunoglobulin G). The child was initiated on Isoprinosine and antiepileptic medications. At 2-months of follow-up, the child was in vegetative state.


A 27-month-old unimmunized girl presented with developmental regression and recurrent head drops for the past 1 month. She had language predominant global delay with a current developmental age of 15–18 months. Her perinatal period was uneventful. There was no past history of exanthematous illness. The child had repetitive myoclonic jerks every 9 s. Neurological examination revealed a normal fundus with central hypotonia and bipyramidal signs. The EEG and MRI were suggestive of SSPE which was confirmed by elevated anti-measles antibody titers in CSF. At 4-week follow-up, the child was in vegetative state.
SSPE is frequently misdiagnosed or not suspected because of the variable presentation. Given the heterogeneous expression, accurate initial diagnosis of SSPE was made in only 21% cases presenting to a tertiary care center in South India.[] The first child in our report is exceptional for the early onset of SSPE and also for the short latent period of 14 months. The two cases highlight the fulminant nature of such an early presentation typified by a vegetative state within 3 months of onset.
The earliest description of SSPE in infancy and toddlerhood dates back to the late 1960s by Dayan et al. with histological and immunologically confirmed cases of 5-month and 15-month-old children at necropsy.[] Review of English literature identified 13 comparable cases in the past 30 years highlighting the rarity of this atypical presentation. Perinatally acquired measles were reported in 5 of the 13 cases []. Several important observations emerge on review of these children. First, the clinical course is atypical and does not follow the classic four stages of the Jabbour Classification. Second, the typical clinical picture of subacute mental deterioration with stereotyped generalized myoclonus is often preceded by a premorbid developmental delay with or without seizures in all five children as in our second child. On the other hand, in the primary measles group of 8 children, fulminant course of the disease is characterized by progression to a vegetative state within 3 months or fatal outcome within 6 months was the norm. The median latency in this group was 12.5 months with the shortest latency of 2 months.[] The exact reason behind this short latency and fulminant course is yet to be ascertained. Genetically determined immune dysfunction in the first 2 years of life preventing a successful cell-mediated immune clearance of measles virus has been implicated in the susceptibility to SSPE.[] Genetic polymorphisms of programmed cell death-1, Toll-like receptor 3, MxA, interleukin-4, and interferon-1 genes have also been hypothesized but not proven. Besides, the role of phylogenetic spectrum of the wild-type measles virus in the etiopathogenesis has to be considered.[] The wild-type measles D3 and D6 are the most prevalent genotypes identified in SSPE on nucleotide sequence analysis. However, D7 genotype with hypermutation in the M gene has been isolated in an adult with fulminant SSPE from autopsy in South India and its association is still speculative.
The other early presentations of SSPE include visual complaints, Balint's syndrome, dystonia, and ataxia. The diagnosis is usually established by Dyken's criteria with periodic electroencephalographic complexes and raised CSF measles antibody titers (100% sensitivity and positive predictive value). SSPE is essentially an incurable disease with limited treatment options inclusive of Isoprinosine, interferon, ribavirin, and flupirtine.[] Given the poor eventual outcome of the therapeutic strategies, prevention through vaccination is the only way to eradicate the disease. Hence, there is a need for intensification of the primary immunization and measles eradication program in India.
Meanwhile, atypical presentation of SSPE should be considered in the differential diagnosis of any unexplained neurological illness irrespective of age, especially in endemic countries. A knowledge of the measles-like illness (also in the mother during the antenatal period), focused visual examination, and predominant white matter changes in MRI supplemented by an elevated CSF antibody titers are essential for an early diagnosis of this devastating condition.
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Nil.
There are no conflicts of interest.


A 76-year-old male patient was referred to our outpatient clinic for further
evaluation of an elevated systolic pulmonary arterial pressure (sPAP) in the
transthoracic Doppler echocardiogram. He was suffering from progressive dyspnea
(World Health Organization functional class [WHO FC] III) without chest pain on
exertion. He had no recent episodes of cough or fever. The physical examination
revealed no pathological findings except a loud p2 heart sound and some minor
inspiratory rales over the posterolateral basal parts of the lung. His medical
history included arterial hypertension, type 2 diabetes mellitus, obesity, and
thrombendarterectomy of the left femoral artery. He had been a cigarette smoker (30
pack-years) and quit 40 years ago. His medical treatment included lercanidipine,
enalapril, bisoprolol, hydrochlorothiazide, simvastatin, aspirin, allopurinol, and
metformin. The laboratory results showed normal C-reactive protein (CRP) and
leucocyte count, mild renal impairment (creatinine = 1.36 mg/dL, glomerular
filtration rate [GFR] = 50 mL/min), elevated NT-proBNP (1323 pg/mL, normal range
<150 pg/mL), and mild IgG4 elevation (2.50 g/L, normal range = 0.03–2.01 g/L).
Spirometry and bodyplethysmography revealed normal values for FEV1/FVC with 77%, and
% predicted values of FVC, FEV1, PEF, and TLC of 101, 104, 95, and 97, respectively,
and mild decreases in MEF 50 and MEF 25 with % predicted values of 81 and 68,
respectively, while DLCO was significantly impaired (DLCOcSB = 2.89 mmol/min/kPa,
40% predicted). The flow-volume loop is depicted in . The arterial blood gas analysis
revealed severe hypoxemia despite compensatory hyperventilation
(pO2 = 47.5 mmHg, pCO2 = 26.4 mmHg, pH = 7.42,
BE = –6.6 mmol/L) on room air. A high-resolution computed tomography (CT) scan of
the chest showed mild emphysema of the right upper lobe with a small nodule, mild
infracarinal lymphadenopathy, and no signs of interstitial lung disease (–). There were also no signs of pulmonary
veno-occlusive disease. V/Q scintigraphy revealed no signs of acute or chronic
pulmonary embolism.
Echocardiography showed concentric left ventricular (LV) hypertrophy and E/e’
suggested mild diastolic LV dysfunction with preserved ejection fraction, and no
pericardial effusion. The right atrium and right ventricle were significantly
enlarged and a paradoxical interventricular septal motion was observed. There was a
mild tricuspid valve insufficiency where a sPAP of 70 + 5 mmHg could be estimated.
TAPSE was normal (21 mm). The 6-min walking distance was 327 m (BORG Dyspnoea Index
4) and significant desaturation (oximetry SpO2 = 60%) was recorded at the
end of the walk. Right heart catheterization (RHC) revealed a moderately increased
mean pulmonary arterial pressure (mPAP) of 36 mmHg, increased pulmonary vascular
resistance (PVR; 5.8 WU), a normal right atrial pressure (RAP; 4 mmHg), and
pulmonary arterial wedge pressure (PAWP; 8 mmHg). Cardiac index (CI) was in the
lower normal range (2.5 L/min/m2) and a normal mixed venous oxygen
saturation (venSO2 = 70.2%) on 2.5 L/min of oxygen was observed.
Autoantibody screening was negative.
In agreement with current recommendations, the diagnosis of idiopathic
pulmonary arterial hypertension (iPAH) was made and the patient was started on an
endothelin receptor antagonist. His clinical condition improved in the first month,
but then deteriorated within two weeks including syncope and progressive leg edema.
He was admitted to a general internal medicine ward where diuretic therapy was
intensified; he was discharged home symptomatically improved after one week. Two
months later he developed a pleural effusion and progressive signs of cardiac
decompensation. He was admitted to our ward and increasing doses of diuretics were
administered. The high-resolution chest CT control showed very mild reticulation in
the basal areas of the lung (–) but no
signs of right ventricular (RV) decompensation. Based on these findings, the ERA was
stopped and a short-term therapy with oral prednisolone was started. This led to a
significant improvement in oxygenation; after three weeks, the patient was
discharged home on 12.5 mg prednisolone o.d. Two weeks later, we were informed that
the patient had decompensated at home with circulatory shock, had been admitted to
the ICU, and died within 24 h.
Postmortem examination showed that the heart was enlarged (500 g), with dilatation of
the right atrium and ventricle, slight increase of the RV wall thickness (6 mm) and
significantly increased thickness of the LV wall (22 mm), and middle to high-grade
atherosclerosis with significant stenosis of all major coronary arteries.
Histological analysis of the lung revealed architectural distortion of the lung
parenchyma in all samples taken from all lung lobes. Unevenly distributed, diffuse
interstitial fibrosis with some lymphocytic infiltration was present in the
subpleural and central lung areas. This was slightly more pronounced around the
blood vessels, with cicatricial organizing pneumonia (–). Centroacinar emphysema was also evident.
Most arteries showed intimal and medial hypertrophy, with congestion and
intra-alveolar edema. In the intra-alveolar space, there was an increased number of
macrophages and siderophages. Anthracotic pigments as well as focal pulmonary
ossifications were also present. Histological findings were consistent with advanced
interstitial lung fibrosis with organizing pneumonia pattern at different stages of
organization, smoking-induced changes (emphysema and anthracosis), and severe
pulmonary vasculopathy, complicated by acute on chronic lung edema. A timeline is
shown in .
This study was approved by the Ethical Committee of the Medical University Graz
(23-408 ex 10/11).


A 24-year-old woman, G5P0, had experienced 4 spontaneous abortions followed by curettage each time. An ultrasonography (US) imaging at 12 weeks of gestation revealed multiple cyst area beneath the chorionic plate. Repetitive US demonstrated a thin placenta covering the most part of uterine wall, completely covering the internal cervical ostium, with multiple cyst area beneath the placenta. Complete placenta previa and PM were suspected and the patient was scheduled for US evaluation of placenta and fetal growth in third trimester. At gestational age of 29 weeks, this patient underwent Magnetic Resonance Imaging (MRI) test which confirmed placenta previa but excluded placenta increta. Fortunately, this patient had no vaginal bleeding ever throughout the pregnancy duration. She was hospitalized after gestational age of 37 weeks and meticulous plans were formulated, including the provision of dexamethasone for fetal lung maturity. Two days later, the patient underwent elective cesarean section and delivered a live-born baby, weighing 2810 g with Apgar scores of 10 and 10. Placental adherence was found during the operation and the placenta was successfully dissected by placental curettage. The placental disk measured 30 × 20 × 1.5 cm and weighed 710 g (Fig. ). No hemorrhage and infection was observed after operation. Microscopically, most of the chorionic layers of the membranes were absent, chorioamnionitis, placental invasion of the myometrium and placenta increta were observed. The patient was discharged 4 days after the operation in good condition.


A 29-year-old woman, G5P0, had histories of two artificial abortions and two spontaneous abortions. She received regular prenatal care and routine US test. At gestational age of 24 weeks and 5 days, US image demonstrated a thin placenta covering the most part of uterine wall, partially covering the internal cervical os, with multiple cyst area beneath the placenta, and fetus small for gestation age. Three weeks later, repeated US test was performed and the situation got worse. The fetus was only equivalent to gestational age of 22 weeks and 6 days, with abdominal circumference (AC) < 10th percentile, as a result, fetal growth restriction (FGR) was diagnosed.[ The thickness of placenta varied from 1 to 4.7 cm accompanied by extensive anechoic area and low-lying near the internal os. After seeking prenatal counseling on possibility of poor postnatal outcome, the mother required termination of pregnancy at gestational age of 28 weeks and one day. The placenta and dead fetus were vaginally delivered after induction of labor by rivanol. The weight of fetus was 677 g, and the weight for gestational age (WGA) was below the 10th percentile.[ Owing to intrapartum retention of the placenta, uterine curettage was performed. The placenta measured 12 × 10 × 1.5 cm, weighed 182 g (Fig. ). Microscopic examination of placenta showed chorioamnionitis, placental calcification and focal infarction. The patient was discharged in good condition 2 days later.


A 20-year-old primigravida, had a history of painless, intermittent, vaginal bleeding during the first and second trimester. At 15 weeks of gestation, US imaging revealed anechoic area beneath the placenta, and the following US 2 weeks later revealed oligohydramnios and enlarged anechoic area. Prenatal US at 20 weeks of gestation revealed placenta covering two thirds of the uterine wall, lack of the echo of placental parenchyma, and PM was highly suspected. In addition, the fetus was only equivalent to 16 weeks and 5 days of gestation, and observed absence of end diastolic velocity (AEDV) of the umbilical artery, the peak systolic velocity (PSV) of the middle cerebral artery was 43.8 cm/s (between 1.29MoM and 1.50MoM).[ At 25 weeks and 6 days of gestation, with the initial complaint of abdominal pain and vaginal discharge, the fluid was identified as amniotic fluid by pH test, and ultrasonogram revealed intrauterine fetal demise. After admission, the patient was induced labor with mifepristone and delivered a stillborn male fetus. The placental disk was measured 12 × 10 × 2 cm and weighed 307 g, with blood clots on the maternal surface (Fig. ). Histologically, the chorionic layers of the membranes were absent, amnionitis and infarction with hemorrhage were found on the placenta (Fig. ). The patient was discharged on the second day of postpartum in satisfactory condition (Table ).


A 46- year old Sudanese male has few months’ history of gastric symptoms. He was planned for upper GIT endoscopy. There was no significant past medical history and he is not on immunosuppressive therapy. One day before endoscopy, he started to feel right lower costal pain which worsened next morning. His symptoms were initially explained by anxiety from the planned procedure. In few days time, he developed severe localized pain on the right lower costal area that radiates from the spine forward to the mid abdomen. He noticed mild change in the color of the skin in this area with redness, small vesicular rash, and soreness. These gradually improved with symptomatic treatment for herpes zoster. By the end of the second week, he started to feel heaviness of the right leg and numbness on the left. There were no urinary symptoms or fever. The clinical examination 3 weeks from the onset of neurological symptoms showed an average weight and height patient with abnormal gait. The abnormalities were confined to the central nervous system in the form of flaccid right lower limb weakness (Grade III + MRC), brisk knee and ankle jerks on the right. The right planter reflex was unobtainable. The examination of the sensory part showed normal pin prick vibration, position, and temperature sensations on the right lower limb but pain and temperature were impaired on the left lower limb which had normal power. A diagnosis of herpes zoster induced autoimmune myelitis was made and a dorsal MRI was requested. The clinical features of myelitis were in the form of Brown Sequard Syndrome. Mean while the patient was started on IV methyl prednisolone injections 1 g daily for 3 days followed by a 4 week's course of oral steroids starting with 45 mg/d. The non contrast dorsal MRI was normal and LP procedure was declined by the patient. Fortunately, the motor weakness improved and at 3 months assessment, there were minimum remaining sensory abnormalities on the left lower limb with no motor weakness on the right lower limb. The final clinical diagnosis was possible D 6 herpes zoster induced myelitis presenting clinically as Brown Sequard Syndrome. The patient was reassured and discharged from the clinic without further neurological complains for 2 years now.


A 37-year-old Sudanese housewife (non-pregnant) was seen in the clinic because of new onset severe headache. Her problem started 4 days before when she was diagnosed as chicken pox. She had a wide spread classical rash for which she was using symptomatic topical and oral treatment in the form of analgesics. Two weeks earlier her child had chicken pox. Her headache was generalized, persistent, becoming severe with some visual blurring, dizziness, and nausea. It made her feel unusually sick. There was no significant past medical history and she was not on immunosuppressive therapy. She was admitted in the general medicine unit where her non contrast brain CT was normal. The patient then received IV fluids, NSAIDs, antiemetics, and IV antibiotics with poor response in regard to the symptoms. Seen in the neurology clinic, her clinical examination, showed a multi stage erythematous eruption (chicken pox) in an ill patient. The higher mental functions were normal. There was no evidence of cranial neuropathy, papilloedema, weakness, or sensory abnormalities. Though there were no positive neurological signs but a brain MRI was requested because the patient headache was severe. Mean while the patient was started on IV aciclovir for 2 weeks and IV methyl prednisolone injections 1 g daily for 3 days followed by a 4 week's course of low dose oral steroids starting with 30 mg/d. Her symptoms improved over 48 hours dramatically. The initial brain MRI is shown below []. The signals in the scan with the head pain concluded the diagnosis of varicella zoster meningio-encephalitis.
In a course of one month, she was almost a symptomatic. A follow-up Brain MRI is shown below and was reported as normal []. The patient was seen one year later without new neurological symptoms.


A 27-year-old woman, who was a foreign domestic worker from Indonesia, came to our outpatient department of Family Medicine in December 2015 with a chief complaint of pain in the right side of the neck for 2 weeks. The pain was intermittent, aggravated with neck rotation, and unrelieved by analgesia, and its severity had gradually increased. She had a sore throat, dysphagia, odynophagia, intermittent fever, body weight loss for 1 month, and a gradually enlarging mass over the right anterior chest wall for 4 months. There was no history of cough, dyspnea, paresthesia, or weakness to the upper and lower limbs, or head and neck trauma. Her only remarkable past history was left ureteral stricture with hydronephrosis, and she underwent laparoscopic pyeloplasty in August 2015. She did not take any drugs, had no family history of systemic diseases, and had no travel or contact history. On physical examination, her body temperature was 37.2°C; heart rate, 110 beats/min; and blood pressure, 115/71 mm Hg. The patient looked ill, with swelling and redness without overlying skin ulceration in the right side of the neck. Palpation of the right side of the neck revealed softness, tenderness, and heat. Examination of the oral cavity showed anterior displacement of the posterior pharyngeal wall. The mass over the right anterior chest wall measured approximately 5 × 4 cm in size. The lungs were clear to auscultation, and neurological examination revealed normal findings.
She was referred to the ear, nose, and throat (ENT) outpatient clinic. Flexible fiberoptic endoscopy revealed a bulging mass originating from the retropharyngeal space. Laboratory tests revealed a white blood cell count of 10.57 × 103/μL (neutrophils, 78.9%) and C-reactive protein level, 5.7 mg/dL. The antibody test for human immunodeficiency virus (HIV) yielded negative results. All other laboratory tests were normal.
Cervical spine radiography on anteroposterior and lateral view revealed a prevertebral soft tissue mass anterior to the C1 to C7 level, displacing and indenting the adjacent airway (white arrow); focal destruction of the C3 vertebra (black arrow) and straightening of the cervical spine; and ground glass opacity over the right upper lung field, located in the extrapulmonary space (Fig. A and B).
Chest radiography revealed an extrapulmonary mass over the right upper lung field, which was a similar finding on cervical spine radiography (Fig. ) and was compatible with the patient's right anterior chest wall mass.
A contrast-enhanced computed tomography (CT) scan of the neck showed multiloculated retropharyngeal fluid collections with enhancing walls spanning from the level of the nasopharynx down to the level of the supraclavicular fossa (Figs. A–C, A, and B, double-ended black arrow), extending to the prevertebral space and epidural space of the spinal canal at the levels of C1-C3 (Figs. B and B, black arrow), with destruction of the C3 vertebral body. These lesions caused significant narrowing of the oropharyngeal and hypopharyngeal airways. Destruction of the T9 vertebra from a left paraspinal mass measuring 3.2 × 2.9 × 1.9 cm was also found (Figs. C and , white arrow). Moreover, a large fluid collection with thick irregular walls over the right anterior chest wall measuring approximately 6 × 5.7 × 5.3 cm in size with the destruction of the anterior part of the right second rib was noted, and it extended to the extrapulmonary intrathoracic cavity (Fig. ). These findings were suggestive of retropharyngeal abscess extending to the cervical spine and chest wall abscess.
The patient was admitted to the ENT ward for further operative management, and a thoracic surgeon at our hospital was consulted for the management of the chest wall abscess. Preoperatively, she received intravenous amoxicillin/clavulanic acid (1200 mg) every 8 hours and gentamycin (80 mg) every 8 hours. Surgery was performed on the second day of hospitalization, and drainage of the right retropharyngeal abscess and chest wall abscess with debridement of chest wall was performed simultaneously. Pus cultures yielded negative results for common bacteria. Acid-fast stain (AFS) yielded positive results. Polymerase chain reaction (PCR) yielded positive findings for Mycobacterium tuberculosis complex. Histopathology showed chronic caseating granulomatous inflammation and Langhans multinucleated giant cells. Pus cultures revealed M tuberculosis complex after 3 weeks of inoculation. Three sputum samples all tested negative for acid-fast bacilli, and cultures were negative for M tuberculosis. Based on these findings, a diagnosis of tuberculous retropharyngeal abscess with Pott disease and chest wall abscess was established.
The patient received isoniazid (300 mg), rifampicin (600 mg), pyrazinamide (1500 mg), and ethambutol (800 mg) daily. Antituberculous drug sensitivity test showed that the M tuberculosis complex was susceptible to these medications. Laboratory examination revealed a white blood cell count of 9.51 × 103/μL (neutrophils, 88%) and C-reactive protein level, 4.74 mg/dL. All other laboratory test results were normal on postoperative day 1. Postoperative chest radiography revealed marked regression of the chest wall abscess as compared with the preoperative image. The patient made an uneventful postoperative recovery with considerable improvement and resolution of both abscesses. She was discharged on postoperative day 13 and returned home to Indonesia.
The patient returned to her home country after discharge, and so we were unable to obtain her informed consent. The need of obtaining informed consent was waived off as per institutional review board of Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation. This case report was approved by the institutional review board (approval number: 07-CR-099) of Taipei Tzu Chi Hospital.


The patient was a healthy 4-year-old Japanese girl, who was the second child of
healthy non-consanguineous parents. She was referred to our hospital because of
elevated serum liver enzyme levels of unknown cause, which were discovered by
chance. Her blood urine nitrogen level was 18.4 mg/dL, and her serum creatinine
level was 0.3 mg/dL. Urinary sediments contained 50 to 99 erythrocytes per
high-power field. An abdominal X-ray image showed a staghorn calculus in the left
kidney. Intravenous pyelography detected left duplicated renal pelvis. Urinary
concentrations of cystine, ornithine, lysine, and arginine were 1078.2, 961.0,
3646.2, and 1728.7 µmol/day, respectively. Renal ultrasonography showed hyperechoic
lesions with sonic shadows in the left renal pelvis, as shown in , and hexagonal
crystals were detected in her urine sediment, as shown in . These findings led to the
diagnosis of cystinuria. Elevated liver enzyme levels were unlikely related to
cystinuria because they improved without any treatment. Treatment with tiopronin and
citrate was initiated for cystinuria, and she was regularly followed-up at our
hospital. Although X-ray findings of urolithiasis did not improve as shown in , she did not suffer
from flank pain.
At 9 years of age, we performed sequence analysis of SLC3A1 and
SLC7A9 in the patient and her parents, from whom informed
consent was obtained under ethical approval (Showa University, Human Genome, Gene
Analysis Research Ethics Committee, No. 197). As shown in , the analysis revealed that she had
a novel heterozygous mutation (c.1113C>A, Y371X). Her mother had no mutation. Her
father had heterogeneous mutations in SLC3A1 (c.1113C>A, Y371X)
and polymorphism of SLC7A9 (c.667>A, L233M) without cystinuria.
His urinary concentrations of cystine, ornithine, lysine, and arginine were 82.1,
8.9, 247.6, and 11.7 µmol/g·Cr, respectively.


A female patient aged 60 years (with skin type III according to Fitzpatrick), with moderate chronic plaque psoriasis (Psoriasis Area and Severity Index [PASI] =7.7), came to the dermatological clinic to receive phototherapy. The study was approved by the Ethics Committee at the University of Gothenburg and the National Data Inspection Board (approval number DNR:089–12, T285-16). The patient´s written informed consent was obtained for case details and publishing accompanying images. The patient was treated with NBUVB therapy (311 nm, according to standard protocol, 28 sessions, total dose 3.0 J/cm2 (increasing from 0.3 to 3.0 J/cm2) and psoriasis significantly improved (PASI =0.6). Four 4 mm punch biopsies were taken, one from intralesional psoriatic skin and one from perilesional healthy skin (about 2 cm from the psoriasis plaque) before and 24 hours after the last NBUVB session (). The biopsies were wrapped in aluminum foil and frozen at −70°C. Each sample was sectioned into 10 μm thin slices and placed on conductive glass slides. The whole process is illustrated in . The sections on the slides were used as they were for NLM and ToF-SIMS measurements; no treatment was done before the measurements. For histological assessment, the frozen sectioned slides were stained with H&E ().


A-37-year old male presented with right side abdominal pain for 2 months.
The pain was dull in nature, with no aggravating and relieving factors, there were no associated symptoms. The patient had non relevant past medical and surgical history.
During examination; the patient had normal vital signs and no findings detected on general examination. Abdominal examination showed a visible large bulging mass in the right side of the abdomen extending from the right subcostal region to the right iliac fossa, which was firm in consistency, non-tender, and smooth surface, Fig. .
The biochemical tests were normal including the serum bilirubin level and liver enzymes. CT-scan of the abdomen showed two non-enhancing cystic lesions in the right lobe of the liver 11 cm and 10 cm containing undulating membrane (water-lily sign), and a third large cyst extending to the pelvic cavity 40 cm in length (features of multiple hydatid cyst of the liver), Fig. .
During surgery through a midline laparotomy, isolation of the cyst done with packs soaked with chlorhexidine solution to prevent contamination of the wound and the abdominal cavity with the cyst fluid, aspiration of six liters of bile stained fluid done at the first instance, Fig. .
The cyst mobilized outside the abdominal cavity, and further four liters of the fluid aspirated, Fig. .
The cyst found to be connected by a narrow pedicle to the edge of the right lobe of the liver, Fig. .
Excision of the cyst done with extraction of the other two cysts from the cyst in the liver parenchyma, two small bile canals detected connected to the cyst cavity which then sutured with 3/0 slowly absorbable monofilament suture material, Fig. .
Tube drain put inside the cyst cavity with omentoplasty and the patient discharged 6 days after the surgery. There was bile leak to the drain which stopped gradually over one month. The patient received anthelminthic medication for 3 months.


A 31-year-old Thai woman came to Siriraj Diabetes Center, Siriraj Hospital, Bangkok, Thailand for her diabetes management.
The patient has been following up every 3 months at the Siriraj Diabetes Center, Siriraj Hospital, Bangkok, Thailand. Currently, she was 44 years old and treated with glicazide 20 mg/d. She has excellence glycemic control without diabetic compli-cations. Laboratory assessment included fasting plasma glucose (FPG) 78 mg/dL, hemoglobin A1c (HbA1c) 6.7%, serum creatinine (0.56 mg/dL), total cholesterol (TC) 173 mg/dL, high-density lipoprotein (HDL) 99 mg/dL, low-density lipoprotein (LDL) 62.6 mg/dL, and triglycerides (TG) 57 mg/dL.
The patient was first seen at Siriraj Diabetes Center when she was 31 years old and diabetes was diagnosed at age 14.
Her mother and brother were diagnosed with diabetes at age 17 and 13, respectively. There was no history of diabetic ketoacidosis, and glycemic control could be achieved without insulin treatment for more than 5 years after diabetes diagnosis in all 3 patients.
The patient’s body mass index (BMI), waist-to-hip ratio, and blood pressure was 19.43 kg/m2, 0.83, and 120/70 mmHg, respectively (Table ).
Laboratory assessments at her first visit to Siriraj Diabetes Center included FPG 126 mg/dL, HbA1c 9.5%, serum creatinine (0.6 mg/dL), TC 156 mg/dL, HDL 71 mg/dL, LDL 90 mg/dL, and total TG 55 mg/dL.
Sanger sequencing of her DNA revealed heterozygous mutation of HNF1A at codon 203 in exon 3 that caused substitution of cysteine for arginine (R203C) (Figure ). This mutation was also identified in all diabetic family members, but not in non-diabetic family members whose DNA were available for sequencing (Figure ). The patient’s glycemic control profile (with and without SU) is shown in Figure . The results of our analysis revealed that excellent glycemic control could only be achieved when our patient was taking SU. Interestingly – when SU treatment was withdrawn, severe hyperglycemia eventually developed, even when insulin was given. The optimal dose of glicazide in this case was 20 mg per day. This patient continues to do well with no observed diabetic complications.


A 1-year-and-2-month-old male baby, first child of a non-consanguineously married couple with uneventful antenatal, natal (no history of difficult delivery, birth trauma), and postnatal a history with normal developmental milestones was brought with history of tilting of the neck to the left side noticed by parents since the age of 4 months. There was no history of head injury, no history of feeding difficulties, and no family history of any ocular disorders. Torticollis secondary to contracture of the sternocleidomastoid muscle was diagnosed elsewhere and physiotherapy was instituted with no improvement.
On examination, head tilt to the left side with chin rotated to the right side was present with full range of neck movement assessed by active head turning during visual tracking. On inspection of the neck, there was no asymmetry of skin folds. All superficial cervical muscles were palpated with no evidence of mass or contracture of the sternocleidomastoid muscle. No facial asymmetry or plagiocephaly noted. Musculoskeletal examination was unremarkable (no CTEV, congenital dislocation of hip, scoliosis). Orthopedic opinion was taken. X-ray of the cervical spine was normal.
On ophthalmological evaluation, right eye hypertropia was present. In forced primary position (when his head was held straight), there was right eye hypertropia. In right gaze, there was no change in the hypertropia, but in left gaze, increase in hypertropia was noted suggestive of right superior oblique muscle palsy. This is a positive Bielschowsky head tilt test and is indicative of paralysis of the superior oblique muscle. Diagnosis of ocular torticollis secondary to right superior oblique muscle palsy was made and the patient was referred to an ophthalmologist for further management.


A 28-year-old male was admitted to the emergency department (ED) for acute left testicular pain that had been gradually increasing for 3 days. Physical examination revealed a mildly swollen left testis that was painful on palpation, with no nodules being apparent. Biochemical tests showed no leukocytosis, and testicular markers were normal. Scrotal color Doppler ultrasound (SCDU) showed a hypoechoic and hyperemic area with faded outlines of 2.5 cm, located anteriorly, surrounded by poorly vascularized testicular parenchyma. At the posterior side of this area, there were two hypoechoic pseudonodular images of 3 and 13 mm [].
Since testicular torsion with a suspicious testicular neoplasm could not be excluded, an explorative inguinotomy was performed showing no torsion of the spermatic cord. The tunica albuginea was incised, and the hypoechoic nodule was removed []. The pathological examination showed diffuse hemorrhagic infarction of the testis tissue, surrounded by normal parenchyma without neoplastic findings. No surgical complication was reported; the patient was discharged after 2 days and then lost to follow-up.


A 43-year-old male presented to our ED with severe left scrotal pain with fever. A scrotal examination revealed a painful and swollen lower pole of the left testicle. Blood analysis showed leukocytosis while testicular markers were normal. SCDU evaluation revealed that the lower two-thirds of the left testicle were constituted of a neoformation with a dishomogeneous structure. Because of the SCDU findings and refractory pain, an explorative inguinotomy was performed. no torsion was found during surgery, but the left testicle had a bluish-white appearance; numerous subalbugineal clots were evacuated allowing for the visualization of poor, necrotic parenchyma. Radical orchiectomy was performed after the intraoperative frozen section analysis showed fresh hemorrhage with no appreciable tumor. One day after surgery, his pain was under control, and he was discharged from our hospital. To exclude systemic causes of testicular infarction, a hematological screening was performed revealing moderately elevated levels of homocysteine (18.9 μmol/L).


A 36-year-old male presented with a 1-day history of acute right-sided testicular pain. His medical history was marked by an episode of paroxysmal atrial fibrillation regressed with flecainide 3 years earlier, hemorrhoidectomy 7 years earlier for several episodes of hemorrhoidal thrombosis, and a recent finding of patent foramen ovale. He had undergone screening for thrombophilia with mild positivity for anti-beta-2 glycoprotein 1 immunoglobulin G (IgG) (21 U/mL). The testis was tender on palpation, and all hematological markers including inflammatory and tumor markers were negative except for lactate dehydrogenase (300 U/l). SCDU performed on day 1 revealed multiple heterogeneous nodular formations of solid appearance, the largest measuring 13 mm, with irregular vascularization. The left testis was normal. A provisional diagnosis of testicular neoplasm was suggested, and a surgical testicular exploration was scheduled. A total-body contrast-enhanced computed tomography was performed without significant findings. Seven days after the onset of symptoms in the right testis, the patient experienced a sudden and severe pain in the left testis. His laboratory tests were positive for leukocytosis. His examination revealed that the right testis was still slightly increasing in volume and consistency, whereas the left testicle had now markedly increased in volume and acquired an irregular surface that was very painful and tender on palpation. The patient underwent a second SCDU that showed an appearance similar to that originally seen on the right didymus. A left explorative inguinotomy was performed showing multiple testicular hematomas; several clots were evacuated preserving residual testicular trophic parenchyma. An intraoperative frozen section analysis of the testicular tissue showed hemorrhage with no observable tumor. Considering the clinical history and the histological findings, a testis-sparing approach was performed without contralateral exploration. The patient was discharged 5 days after surgery. The subsequent 9 months were without event.


The patient, a 26-year-old male, with a history of acid injury in the right eye 3 months prior had been variously treated with symblepharon release, hAM transplantation, and BCL insertion in the acute phase followed by lid reconstruction, symblepharon release, and an Ahmed glaucoma valve (AGV) for uncontrolled intraocular pressure (IOP). At presentation, he had a total LSCD with total conjunctivalization of the cornea []. There were symblephara of the upper and lower lids [Fig. and ]. Vision was perception of light with inaccurate projection of rays. Digital tension was higher than normal. The left, unaffected eye was within normal limits with a healthy limbus. B-scan ultrasound of the affected eye revealed an attached retina. The technique of SLET used was as described by Sangwan et al.[] – blunt dissection of the pannus from the corneal surface, release of all symblephara, gluing of an hAM (non-cryopreserved, fresh), and fixation of limbal explants from the contralateral eye with fibrin glue (Tisseel Kit from Baxter AG, Vienna, Austria). At the end of the procedure, a BCL was placed. Tissue harvested from the healthy eye was approximately 2 mm wide and was divided into 11 bits which were placed in two concentric circles on the right eye []. shows the slitlamp photograph of the operated eye on post-operative day 2.
The patient was followed up daily for the first 6 days and subsequently on days 8, 10, and 14. At each visit, besides a full clinical evaluation, slit lamp photographs were taken and anterior segment OCT was done on a Cirrus™ HD-OCT (Carl Zeiss Meditec AG, Goeschwitzer Strasse 51–52, 07745 Jena, Germany), spectral-domain OCT with an axial resolution of 5 μm and a transverse resolution of 15 μm.
For the purpose of the study, three explants were identified on a clinical photograph [] and labelled as 1, 2, and 3. Serial OCT was done along a radial axis for each explant reaching up to the limbus, as shown in by a single investigator (MS) at each follow up day on each of the three explants. Quality of all OCT images achieved was satisfactory. The best images were obtained for explant 2 and are reproduced here.
Postoperative regime followed was prednisolone acetate drops 6 times a day, moxifloxacin drops 6 times a day, and carboxy-methyl cellulose drops 2 hourly. The BCL was retained in the operated eye for the entire duration of the study.
There was rapid resolution of conjunctival congestion and good mobility of the globe with release of the symblephara. Lid closure was good. Digital IOP was normal. Anterior chamber details could be made out hazily through the translucent cornea, with the pannus removed. Vision remained perception of light with inaccurate projection of rays. The donor fellow eye had rapid closure of the epithelial defect at the donor site.
The hAM remained of even density (OCT reflectivity) and thickness throughout the study duration []. Sub-hAM space increased from day 3 and resolved completely by day 10. There was thinning of the explants from day 3 which corresponded to the sub-hAM space expansion, which may have been compressing the explants against the BCL. Fibrin around the explants started decreasing from day 2 onwards and disappeared by day 4. The hAm, even though glued to the corneal surface with fibrin glue, was not absolutely stable in the early days after SLET. The fibrin glue beneath the hAM appeared to swell, displacing the hAM anteriorly. However, this settled by day 10, and thereafter the hAM was adherent to the cornea. There were no changes in the consistency (reflectivity) and thickness of the hAM. The fibrin around the explants developed optically hollow spaces immediately adjacent the explants and started decreasing from day 2 and disappeared by day 4. Comment regarding the thinning rate of the explants is difficult as it is impossible to ensure that the OCT section is being taken at exactly the same site of the explants on each occasion. Further, the thinning of the explants appear to correlate with the initial bulging of the sub-hAM space and continued even as the hAM settled down and adhered to the cornea. There was no evidence of epithelialization up to day 8. By day 14, there was complete epithelialization on the limbal side, in contrast centripetally directed epithelialization was poor extending only about 0.24 mm on day 14. The nature of the epithelium was boggy and not the crisp reflective layer that is seen in OCT of normal corneas. There was little change in the corneal stromal thickness and lucidity on OCT.


The patient was a 33-year-old G4, L2, and D1. Her blood type was A RhD negative, and the blood group of her husband was known to be A, Rh (D) positive. Her first pregnancy produced a healthy female infant, delivered at the 38th week of gestation by natural delivery. Her second pregnancy produced a healthy male infant, delivered at the 38th week by natural delivery. Her third pregnancy had been terminated at the 35th week of pregnancy by cesarean section due to severe preeclampsia. The newborn died a few minutes afterbirth because of hydrops fetalis. There was no history of blood transfusion or blood product exposure. She had never received anti-D immunoglobulin and she was unaware of the blood groups of her two first children.
In her fourth pregnancy, the Nuchal Translucency ultrasound and the anomaly scan were both normal. Due to bad memories of her unfortunate previous pregnancy, she was referred to perinatologist for further management of her pregnancy. The indirect coombs test was positive. D alloimmunization was detected in this pregnancy, with a corresponding Rh (D) titer of 1:128 at 17 weeks of gestation.
Fetal ultrasound examinations and Doppler study of middle cerebral artery (MCA) was normal at 17 weeks of gestation, so therapeutic plasma exchange (TPE) was initiated and then repeated weekly. Plasma exchange (PE) was performed by HAEMONETICS PCS2, Model NO, 6002 (400 Wood Road, Braintree, Massachusetts 02184, USA) by peripheral venous access. During PE, in each procedure 500 cc plasma (250 cc twice) was removed and was replaced by 0/9% normal saline. The anticoagulant used was 4% sodium citrate. Fetal heart rate was monitored before and after the procedure. The patient was placed in lateral position, especially in the third trimester. She was controlled with Doppler sonography every 3 weeks. All ultrasound examinations were normal and PEs were tolerated without any complications. At 35 weeks of gestation, ultrasound examinations reported an increase in MCA peak systolic velocity (PSV MCA: 72 cm/s) that was suggestive of mild anemia.
She was admitted at 37 weeks of gestation due to the initiation of labor. She inclined to have vaginal delivery after her previous cesarean section (VBAC), but due to fetal distress and prolonged deceleration, cesarean section was performed. A healthy male infant weighing 2,520 g, with APGAR scores of 7 at 1 min, and 8 at 5 mins was born. The blood group of the infant was O Rh positive. Direct antiglobulin testing was negative. The infant’s hemoglobin was 13.8 g/dL and stable. Both mother and infant were discharged on day 3.
The patient provided written informed consent for the publication of the case details. The ethical committee of Mashhad University of Medical Sciences approved the publication of this case report.


In September 2017, a 72-year-old woman was referred to the cardiology unit of Casa di Cura Villa dei Fiori, Mugnano, Italy because of a 2-year history of recurrent paroxysmal atrial fibrillation associated with occasional palpitations and shortness of breath. She was on warfarin anticoagulation medication and no significant medical problems and structural heart disease were reported. Despite treatment with 100 g atenolol orally per day for 1 year, she experienced several pharmacological and electrical cardioversions and took the opportunity for a potentially curative approach with transcatheter radiofrequency ablation. The procedure was performed via the femoral route; 7-Fr catheters were used to map the left atrium and pulmonary veins. Energy applied was limited to a power of 20 W achieving a maximum temperature of 45 °C. Radiofrequency applications lasted an average of 3–4 min per vein resulting in elimination of pulmonary vein action potentials. No phrenic nerve stimulation was performed during ablation of the superior right pulmonary vein. During and immediately after the procedure, the patient presented with no symptoms.
Three days after the procedure, the patient developed shortness of breath and also progressive dyspnoea. Electrocardiograms and echocardiograms were well within normal limits, while the chest X-ray revealed an elevated right hemidiaphragm ().
Subsequently, the patient was referred to the I Division of Neurology and Neurophysiopathology, University of Campania Luigi Vanvitelli, Naples, Italy for neurological and neurophysiological assessments in February 2018. The neurological examination was of no particular relevance. The patient had no hiccups or cough. A fluoroscopic sniff test (i.e. diaphragm fluoroscopy), was performed during chest fluoroscopy, which demonstrated a lack of right hemidiaphragm movements during voluntary inspiration (). These findings suggested right phrenic nerve palsy. In addition, motor nerve conduction was examined and showed the absence of a right phrenic nerve compound motor action potential (CMAP) compared with the normal left side (), confirming the diagnosis of right phrenic nerve palsy.
The patient did not require noninvasive mechanical ventilation. She experienced a spontaneous complete recovery within 2 months. Asymptomatic paralysis persisted at an 8-month follow-up visit.
Consent to publish this case report was received from the Ethics Committee of the University of Campania Luigi Vanvitelli, Naples, Italy. Verbal informed consent was obtained from the patient.


A purplish spot located on the right labia majora for 2 months.
A two-month-old girl presented to our department with her parents. She was born at full-term (38 wk) and weighed pproximately 3300 g. Three weeks after birth, she was noted to have a light purplish spot located on the right labia majora. The vascular lesion promptly grew in size over a period of 5 wk. No ulcers or hemorrhagic areas were noted externally. No prior treatment had been administered.
Unremarkable.
There were no similar patients in the family.
Physical examination showed that the right labia majora was covered with a thick hemangioma (Figure ).
There were no suitable laboratory examinations.
There were no suitable imaging examinations.


A 14-year-old boy presented with gradually worsening spastic quadriparesis with nonambulatory power and cervical kyphotic deformity as a consequence of laminectomy for tumor debulking and chemoradiotherapy done 2 years prior for cervical PNET.
Problems to be addressed particularly to the case were a rigid kyphosis of 102.7°, poor soft-tissue coverage, internal gibbus compressing cord, perched facets, and superficial location of cord. Figures and show the preoperative X-ray and magnetic resonance imaging of the patient with rigid kyphosis.
The staged plan of management included:
Stage 1: Anterior release and decompression. Anterior release involving removal of C3, C4 vertebral body, and decompression resulted in partial correction of kyphosis to 70°. The patient was advised strict immobilization, and Gardner-Wells tongs was applied and cervical traction was given with gradual increase in weights from 3 to 9 kg with resultant gradual reduction in the degree of kyphosis [Figures –] Stage 2: Posterior instrumentation, reduction, and fusion using C1 lateral mass screw, C2 pedicle screw, C5, C6, and C7 lateral mass screw, and posterior iliac crest bone grafting [] Stage 3: Anterior reconstruction using anterior iliac crest strut bone grafting [].
With meticulous-staged surgeries, we obtained correction of cervical kyphosis from 102.7° to 25.72° amounting to a total correction of 77°. Review of the current literature found that the largest correction of cervical kyphosis averaged 48°. The patient is ambulatory with Grade 5 power in all four limbs. Computed tomography scan at 18-month follow-up showed good bony consolidation.


A 46-year-old woman was admitted to our hospital with left side chest pain for 5 days. The pain was dull and was relieved after resting. There were no cough or sputum, no hemoptysis, no shortness of breath, no fever or night sweating, and no limb numbness or fatigue. She denied smoking, drinking, nor a history of human immunodeficiency virus (HIV) or exposure to hepatitis B. There was no occupational or recreational exposure to toxins either. The physical examination found no other symptom or physical abnormalities.
Laboratory examinations were all normal, including blood routine test, coagulation function test, liver and kidney function tests, urinalysis, test for tumor markers.
Chest computerized tomogram (CT) showed the lesion with a clear boundary located under the left dorsal pleura with dimension 43 mm × 36 mm × 39 mm. The mass appeared to be heterogeneous, with a CT value about 17 HU. After contrast, the mass showed a mild heterogeneous enhancement with no obvious necrosis, and the CT value was about 32 HU (Fig. ).
As there was no evidence of local or distant metastasis, a resection operation was carried out. During the operation, the mass was found to be located at the left dorsal pleura, about 4 cm in length, hypervascular, and with a firm texture. The tumor did not invade adjacent ribs and was completely resected. The intraoperative pathological examination showed that the small cells were in chrysanthemum shape encompassing a neuropil. Immunohistochemical investigations revealed that the tumor cells expressing CD56, S-100, Syn. Based on its morphologic and immunohistochemical features, the tumor was finally diagnosed as neuroblastoma (Fig. ). Unfortunately, patient refused to have further genetic testings, and we were unable to properly assess her staging based on the International Neuroblastoma Risk Group (INRG) Task Force.[ According to the International Neuroblastoma Staging System (INSS) criteria, the patient had stage 1 neuroblastoma.[
According to the INSS criteria, the treatment for patients with stage 1 neuroblastoma is surgical resection alone.[ The patient had not received any chemotherapy or radiotherapy after surgery. Prognosis was promising so far, as there was no signs of recurrence or metastasis 3 years postoperatively.


An 18-year-old previously healthy male presented to cardiology clinic after experiencing syncope while running laps. He experienced loss of consciousness for a few minutes but was successfully resuscitated with cardiopulmonary resuscitation. Initial workup including electrolytes and toxicology screen was normal. However, his ECG showed the typical coved-type ST-segment changes in V1 and V2 consistent with BrS []. Since the syncope was triggered by exercise, he underwent a treadmill stress test, during which he became near syncopal and his rhythm degenerated to a wide complex tachycardia consistent with ventricular tachycardia []. His symptoms self-resolved shortly after exercise was stopped. A cardiac magnetic resonance imaging did not demonstrate any pathologic findings.
Genetic testing was done and the patient was found to have a pathogenic disease-causing mutation in the SCN5A gene consistent with BrS. The c. 4140_4142delCAA variant that was identified is located in coding exon 22 of the SCN5A gene and has been associated with varying degrees of cardiac conduction disorders, as well as a family history of sudden death in several studies.[] Additional testing including ECGs and genetic testing for the same mutation in his brother and parents came back negative. The patient underwent an electrophysiology study, which showed easily inducible, hemodynamically unstable polymorphic ventricular tachycardia at 260 bpm, requiring DC cardioversion for conversion to sinus rhythm. The patient was started on nadolol 40 mg daily and a transvenous dual-chamber implantable cardiac defibrillator was implanted. The patient underwent a subsequent exercise test that showed no ventricular tachycardia on beta-blockers.


A 46-year-old female presented to the emergency department seeking acute psychiatric services subsequent to significant changes in mood, including suicidality, irritability, sleep disturbance, and an inability to utilize coping skills to feel safe. The patient had an extensive past psychiatry history notable for bipolar I disorder, borderline personality disorder, posttraumatic stress disorder, and nicotine use disorder. Her past medical history was significant for a number of chronic illnesses, including hypertension, thyroid disease, and chronic obstructive pulmonary disease—none of which were exacerbated in the previous weeks.
The mental status exam found the patient to be calm with appropriate speech, language, and attention. No abnormal movements were found although some restlessness was noted. Suicidal ideations were present although hallucinations and delusions were absent. Basic laboratories and the physical exam were unremarkable.
Medications prior to admission included cyclobenzaprine, gabapentin, lamotrigine, quetiapine, venlafaxine, trazodone, and vortioxetine. The only change noted in these in the previous 5 months leading to admission was a decrease in the dose of venlafaxine. One new medication, varenicline, was added to her regimen 3 weeks prior to presenting. This was prescribed by the patient's pulmonologist in regards to her continued smoking despite her chronic obstructive pulmonary disease. The doses were titrated per approved labeling up to 1 mg by mouth twice daily. Due to the patient endorsing concerns that varenicline may have induced her current symptoms, the attending psychiatrist discontinued this agent and admitted the patient to the inpatient psychiatric unit.
On the second day of admission (3 days since the last varenicline dose), the patient reported intense anxiety and restlessness, which she described as similar in nature to a previous episode she developed after initiation of an antipsychotic. The patient was diagnosed with akathisia because of this description and the psychiatrist's assessment. No changes had been made to her antipsychotics, and no medication known to cause akathisia was added. The clinical pharmacist on the team was asked to investigate varenicline as a possible cause of this extrapyramidal reaction and recommend treatment. No specific literature describing this was found, and the patient's akathisia was subsequently treated per usual. Propranolol 10 mg by mouth 3 times daily provided relief within 24 hours.