A 68-year-old female patient presented three times to the Emergency Room with confusion, lethargy, dysarthria, nausea and vomiting. Each time the first evaluation disclosed normal vital signs where neurological examination always revealed a minimally responsive woman without apparent focal deficits but with asterixis and increased tendon reflexes. The crisis resolved after conservative therapy. Brain computed tomography (CT) showed chronic cerebral vascular disease, absence of any acute intracranial lesions and electroencephalography did not reveal any subclinical epileptiform sign. Chest X-ray did not reveal any insurgent abnormalities, except for outcome signs of fractures of the IV, V and VI ribs that patients reported two months before because of an accidental fall. Normal levels of hemoglobin, leukocytes, platelets, glucose, electrolytes and creatinine were found. In the last admission serum ammonia level was finally measured and found to be far above the normal value (nv < 75 mg/dL): > 400 mg/dL. Based on these findings, the patient was eventually transferred to our Internal Medicine department and started on lactulose, rifaximin, low-protein diet. The past medical history revealed: normal growth and pubertal development with no history of congenital malformation, left quadrantectomy plus lymphadenectomy followed by radiotherapy and chemotherapy with tamoxifen for breast carcinoma in 2003 (at that time patient was declared free of disease and was not taking any anti-cancer therapy); hypertension in treatment with beta-blockers and ace-inhibitors; mechanic aortic valve replacement due to severe aortic stenosis for which was taking oral anticoagulants and pacemaker implantation in 2007; depression and migraine self-treated. She had no history of alcohol abuse, hematologic disorder or liver disease. The biochemical assessment showed: normal thyroid function, euglycemia, normal levels of ACTH and cortisol, normal GH with low IGF-1 for age (37 ng/mL 116-353 ng/mL), INR in range (between 2.5 and 3.5 for mechanic valve), normal renal function and sodium/potassium levels. Slightly elevated transaminases (GOT 59 U/L, nv < 32 U/L; GPT 56 U/L , nv < 33 U/L) and direct/indirect bilirubin (1.3 mg/dL, nv < 1.2 mg/dL; 0.7 mg/dL, nv < 0.3 mg/dL) were also found. Viral Hepatitis markers (HAV/HBV/HCV/HEV) were negative as well as HIV1-2 serology, tumor markers (CEA/CA19.9/CA15.3/CA125/alpha-fetoprotein/NSE/CYFRA21-1) and humoral autoimmunity (ANA/ENA/pANCA/cANCA/AMA/ASMA/LKM1/SLA-LP) markers. There was not a history of hepatotoxic drugs assumption or alpha-1-antitrypsin deficiency. Hemochromatosis and Wilson’s disease were ruled out based upon normal circulating levels of iron, ferritin, transferrin, copper and ceruloplasmin. The persistent presence of variable floating degree of hyperammonaemia, unresponsive to conventional treatment, prompted us to take into account more rare causes sustaining high levels of circulating ammonia in the absence of liver disease. Gastroscopy and colonscopy did not identify any bleeding or macroscopic alteration (in particular absence of gastroesophageal varices) of the gastrointestinal tract and obstinate constipation did not affect our patient. She was not taking any drug potentially producing hyperammonemia and had not performed a high-protein diet. Coproculture was negative and excluded bacterial colonization, urine culture was positive for E. coli and the asymptomatic infection was easily treated. Lastly, plasmatic and urinary amino acids chromatography excluded the rare condition of urea cycle disorders. Abdominal ultrasound revealed normal liver volume and echogenicity without focal lesions and no ascites. EcoColor Doppler ruled out thrombosis of portal vein and its intrahepatic branches with hepatopetal flow. Main portal trunk diameter was within normal values (< 1.5 cm) with enlarged appearance of splenoportal confluence. Brain MRI examination was not performed because of the presence of mechanic aortic valve. Abdomen computed tomography angiography revealed the patency of portal vein trunk with an enlarged superior mesenteric vein and a giant portosystemic shunt. The shunt presented a maximum caliber of 20 mm and showed a large retroperitoneal loop emerging from the spleno-mesenteric confluence with discharge in the left hypogastric vein (Figure ). No collateral gastroesophageal pathways of portal circulation, normal spleen volume and no ascites were found. Then, hepatic venous pressure gradient (HVPG) measurement of portal vein pressure was performed. From right internal jugular vein the right hepatic vein was catheterized. HVPG of 1 mmHg (free flow 13 mmHg; wedge 14 mmHg) was recorded thrice with repeated occlusion balloon (Occluder, Boston Sc., United States) inflations and deflations. To test the hypothesis of a possible pre-hepatic portal hypertension, causing a misleading low HVPG, a 5 French (Fr) diagnostic catheter was negotiated through the inferior vena cava and the shunt directly in portal vein. Hepatopetal portal flow was opacified and direct portal pressure was 14 mmHg, confirming the HVPG value. Fibroscan examination revealed liver elastance of 10 KPA compatible with a low probability of clinically significant liver cirrhosis. Thus, it was not worth the risk to perform a liver biopsy taking into account that the patient was on anticoagulant therapy. Three days later transfemoral embolization of the shunt was performed. Thus, by right groin access the contralateral shunt outflow in left hypogastric vein was catheterized (Figure ). A 45 cm long 8 Fr introducer was engaged deeply inside the left hypogastric vein and a 5 Fr catheter (Glidecath, Terumo) was advanced over a 0.035”inch glide guidewire through the loop of the shunt up to the main trunk of portal vein. Then a high flow microcatheter (Progreat, Terumo) was inserted through a side-valve into the 5 Fr catheter. Thereafter multiple coils (Ruby Coils, Penumbra INC, United States), for a total length of 974 cm, were released. Due to apparent instability of the cast of coils, the 8 Fr introducer was advanced over the 5 Fr catheter, the last was eventually removed. More proximally two large nitinol plugs were deployed near the shunt confluence in the internal iliac vein (Figure ). Mechanical embolization was preferred to sclerosis with chemical agents to avoid the risk of reflux in the portal system with potential catastrophic complications. A control CT at 1 mo confirmed the complete shunt exclusion (Figure ). As a consequence, mesenteric venous blood restarted to fully hepatopetally flow into the liver. The venous blood containing a high level of ammonium reached once again the liver where metabolic ammonia detoxification increased and encephalopathy subsided. After that, we observed a complete remission of symptoms, normalization of transaminases (GOT 26 U/L, nv < 32 U/L; GPT 22 U/L, nv < 33 U/L), direct/indirect bilirubin (1.1 mg/dL, nv < 1.2 mg/dL; 0.2 mg/dL, nv < 0.3 mg/dL) and ammonia level 35 mg/dL (nv < 75 mg/dL), in the absence of any specific therapy, and stable conditions after a two years follow up.


A 70-year-old Asian man with a nonsignificant medical history visited a local clinic with a complaint of a palpable, painless, and nonmovable perineal mass for several months. The overlying skin was slightly erythematous. He denied any recent history of unintended weight loss, fever, nausea, melena, or hematochezia. In February 2016, an excisional biopsy was performed in the local clinic. The pathology results showed metastatic adenocarcinoma with positive margins. Further workup for carcinoma of unknown origin included a thorough physical examination, basic blood tests, esophagogastroduodenoscopy, colonoscopy, contrast-enhanced computed tomography (CT) of the chest, abdomen, and pelvis, and positron emission tomography-CT (PET-CT). On physical examination, painless palpable movable masses were detected in the bilateral inguinal area, which were presumed to be enlarged lymph nodes (LNs). The CT scan showed an enlarged necrotic LN in the left inguinal area with a maximum standardized uptake value of 11.7 on PET-CT, in addition to several small LNs in the bilateral inguinal area ().
The patient was transferred to Asan Medical Center and the surgical specimen was re-examined. Final histopathologic examination of the excised skin and subcutaneous tissue revealed a tumor with the typical appearance of invasive breast carcinoma of no special specific type without evidence of nonneoplastic breast tissue (). The invasive component measured 1.7 cm in the greatest dimension. The tumor involved the dermis, subcutis, and skeletal muscle, and lymphovascular and perineural invasion was also present. The overlying skin showed mild acanthosis. The tumor cells were strongly positive for estrogen receptor (1:100; NCL-L-ER-6F11; Novocastra, Newcastle, UK) and progesterone receptor (1:200; NCL-L-PGR-312; Novocastra), and negative for human epidermal growth factor receptor 2 (1:250; A0485; Dako Glostrub, Denmark). The tumor cells were focally positive for gross cystic disease fluid protein 15 (1:50; MS1170A, NeoMarkers, Fremont, USA), and negative for prostate-specific antigen (1:200; M0750; Dako) staining, which reduced the possibility of metastasis from the prostate. Immunohistochemical staining did not show p53 overexpression (1:1,500; M7001; Dako). The Ki-67 labeling index (1:200; M7240; Dako) was approximately 10%. Since the patient did not have primary breast cancer, we assumed that this tumor had arisen from ectopic breast tissue in the perineum, and the invasive carcinoma had replaced the entire nonneoplastic ectopic breast tissue.
The patient underwent local wide excision in the perineum and dissection of bilateral inguinal LNs at our hospital. Microscopic examination revealed multifocal residual invasive carcinomas measuring up to 0.4 cm in the greatest dimension, and two metastatic left inguinal LNs. Postoperative adjuvant chemotherapy and hormonal therapy were performed. After 8 months and at the time of writing, the patient had showed no evidence of recurrent disease. The study was approved by the Institutional Review Board of Asan Medical Center (IRB No. 2016-1251) and performed in accordance with the principles of the Declaration of Helsinki. The informed consent was waived.


JM is a 46-year-old African-American man with a self-reported history of bipolar disorder, chronic shoulder pain on prescribed cannabinoids, who presents to the emergency department with suicidal ideation. The patient states that he has been having progressed depressed mood over the past week and started to have suicidal ideation for a few days. He endorsed to a plan of drinking bleach or any toxic liquid he could purchase in the grocery store. These thoughts were associated with insomnia, decreased appetite, feelings of hopelessness and helplessness. He states that over the past 2 days, he has noticed increased anxiety associated with ‘shadows’ which have been occurring more frequently in the evening. He reports medication non-compliance in the past 3–4 weeks; quetiapine 400 mg at bedtime and bupropion SR 150 mg twice daily. He states that he got into a verbal altercation with his girlfriend after she found out about his ‘infidelities’ and was kicked out of their home in Delaware. He decided to seek shelter with his aunt in New Jersey. He reports a long psychiatric history with initial 3-week inpatient hospitalisation in his mid-late twenties for decreased need for sleep, impulsive financial spending and hypersexual behaviour. He has experienced elated mood which cycles with periods of depression every few months. He reports a history of two suicide attempts; first in his 20s for overdose of pills and second 2 years ago when he ingested bleach and was subsequently hospitalised. The patient’s medical history is significant for a gunshot wound to his right shoulder which required emergent surgical stabilisation. He subsequently underwent over a dozen reconstructive surgeries to his right shoulder. The patient reports an allergy to aspirin and non-steroidal anti-inflammatory drugs and chart review is notable for anaphylaxis during a previous hospitalisation after the administration of ketorolac. He was ultimately prescribed medicinal marijuana through the state of Delaware for his debilitating chronic shoulder pain. He denied any history of substance dependence with tobacco, alcohol, opiate, benzodiazepines, cocaine or hallucinogens. To note, the patient’s quetiapine and bupropion prescriptions were confirmed at his local pharmacy in Delaware and marijuana card validated through the state of Delaware. In addition, the patient’s aunt provided collateral information which corroborated the patient’s presenting history.
The patient appeared in no apparent distress, normal posture, wearing a hospital gown, thin with no obvious skin lesions. There is obvious right shoulder atrophy and asymmetry with at least six visible scars throughout the anterior shoulder extending to the posterior shoulder blades and upper back. The right shoulder is in fixed position with no range of motion. There is some hypersensitivity to the surgical scars. There are no signs of erythema, fluctuance, effusion or discharge. The patient has a steady gait. The rest of the musculoskeletal examination is with normal tone, no rigidity or spasticity. The patient is cooperative and engaged with the examination. The patient displays normal psychomotor activity, appropriate eye contact and normal speech. Reports depressed mood with appropriate and congruent sad affect. His thought content was with suicidal ideations with plans to overdose on toxic liquids. He denies any homicidal ideation or delusions. His thought process is linear, clear and coherent. The patient is alert and awake to person, place and time. He denies any active perceptual disturbances. His memory, concentration, abstraction, language and vocabulary are grossly normal. His intelligence is noted to be average. The patient demonstrates questionable impulse control, fair insight and fair judgement.


A 36-year-old Caucasian male individual awoke suddenly at about 23:00, 1 h after taking a 200-mg dose of amitriptyline. The patient’s wife, who was also in the bedroom at the time, was alarmed to see the patient suddenly rise from bed and disrobe for no apparent reason. When asked what he was doing, the patient replied that he needed to get ready for work. As the patient had just recently switched to working day shifts again, this did not make much sense but before his wife could question him, he dashed into the kitchen upon where he began pulling out pots and pans from a bottom cabinet. The patient’s wife closely followed, demanding what was going on. Upon entering the kitchen, the patient explained in quick but articulate speech that he had to repair the pots and pans before work started.
No sooner had his wife inquired as to what was wrong with the pots and pans that necessitated their repair so late at night, the patient dashed back into the bedroom where he reportedly began digging through dresser drawers in search of cereal and milk. At this point, the patient’s wife became concerned enough to call the police, but before their arrival, the patient was able to dress himself for work, have a conversation with the bedroom curtains as if they were his coworkers and finally, he attempted to crawl out the upper-story bedroom window in an attempt to get to his car so he could drive to work.
The patient stated he had no recollection of these events but did remember becoming quite upset and irritable with the police because he was under the impression that they had arrived extremely early in the morning for no apparent reason. The patient denied recollection of the actual conversation between the police and himself but does remember they took him to jail shortly after their arrival. The patient had received a warrant for his arrest owing to an unpaid ticket for not wearing a seatbelt. He was able to remember that he became extremely tired upon arrival to jail and subsequently slept until morning; upon where he awoke his usual self.
The patient denied ever having an episode like this in the past. He had presented to his primary care nurse practitioner 2 months earlier for trouble sleeping secondary to racing thoughts. The patient had a history of chronic unspecified depression and anxiety, which were not being treated at the time. He was placed on brexpiprazole (Rexulti® is co-manufactured by Otsuka Pharmaceutical Co., Ltd., Chiyoda, Tokyo, Japan and H. Lundbeck A/S, Copenhagen, Denmark) 2 mg daily, along with amitriptyline 100–200 mg at night for depression and sleep, as well as metoprolol 25 mg twice daily for anxiety. He had no other medical history and took no other medications. The patient did admit to occasional marijuana use but advised he had not used marijuana for 2 days before the incident.
The patient states he was told to start with 100 mg of amitriptyline at night and to take a second 100-mg tablet if it did not seem to be working. He reports that he began taking 200 mg of amitriptyline every night about a week after being on the 100-mg dose. He asserted it was effective as he was able to get about 6 h of sleep per night with the 200-mg dose compared with the 100-mg dose where he was averaging only 3–4 h of sleep. The patient continued his medication regime for 7 weeks; at which point he was switched from day to night shifts at work. He discontinued his evening dose of amitriptyline and reported having tried to take the amitriptyline in the morning instead but found it to be too sedating as he seemed to be sleeping most of the day. The patient subsequently stopped taking his amitriptyline over the next 7 days as he seemed to be getting adequate sleep during the day without it. After the following weekend and upon resuming his normal work schedule that Monday, the patient took his previous 200-mg amitriptyline dose at night before going to bed. Approximately an hour later, the events described above transpired.


A 73-year-old woman was admitted to our hospital with symptomatic hypocalcaemia on June 2016. Her symptoms was muscle cramps, numbness, tingling sensation in the perioral area and impaired intellectual capacity. Physical examination revealed positive Chvostek and Trousseau signs. On the cardiac examination, bradycardia was present along with a QT prolongation on the ECG.
In 2005, she was diagnosed with breast cancer and she was submitted to total mastectomy. Chemotherapy (four cycles of epirubicin and paclitaxel and three cycles of CMF: cyclophosphamide, methotraxate and 5FU), radiation therapy and hormonal therapy with tamoxifen were given after mastectomy. In 2007, she was submitted to total thyroidectomy due to toxic multinodular goitre and a permanent postoperative hypoparathyroidism was developed as a postoperative complication. The patient was receiving replacement therapy with levothyroxin 100 µg/day, calcium carbonate 2 gr/day, cholecalciferol 1600 IU/day, alfacalcidol 2 µg/day, po.
The patient remained stable for several years until December 2015 (7 months prior to her admission), when liver and bone metastases were detected. Chemotherapy with paclitaxel and carboplatin was started and on March 2016 (3 months prior to her admission), zoledronic acid (ZA, 4 mg intravenously) was added to standard therapy in order to reduce the development of bone metastases and to improve disease outcome.


A 10-year-old female patient reported with the chief complaint of painless swelling in lower left side of her jaw for 15–20 days. The patient's parents gave previous history of dental consultation and symptomatic drug therapy 2 years back. The swelling was diffuse, measuring 3 cm × 3 cm on left mandibular angle region. It was firm and tender on palpation. Intraoral examination showed grossly carious 75. Orthopantomograph revealed a large unilocular radiolucency on periapical aspect of 75, extending to the lower border of mandible, displacing tooth bud of 35 [Figures and ]. Thinning of buccal cortical plate was evident on occlusal radiograph. The lesion was provisionally diagnosed as radicular cyst. Cystic fluid aspirate was obtained and sent for the histopathological evaluation. Surgical enucleation, followed by placement of autologous PRF as a surgical adjuvant, was planned under general anesthesia. For preparation of PRF, 5 ml of whole venous blood was collected in two sterile Vacutainer tubes without adding anticoagulant. The Vacutainer tubes were then centrifuged for 10 min at the speed of 3000 rpm. Extraction of 75 was done. Cystic site was exposed through intraoral approach and cystic lining was enucleated. Displaced tooth bud of 35 was preserved. PRF was placed in the defect. Hemostasis was achieved and primary closure was done using sutures. The excised cystic mass was submitted for histopathological examination. Histopathological features were consistent with the clinical diagnosis of radicular cyst. Sutures were removed after a week. A removable functional space maintainer was given till the eruption of mandibular second premolar []. The patient was kept under regular clinical and radiographic follow-up. After 3 months of follow-up, oral and radiological examination revealed healing wound and reduction in size of radiolucency. There was recall of 24 months which showed uneventful eruption of mandibular second premolar and complete bone healing [Figures and ].


A 24-year-old man presented at the emergency department with chest pain, left arm numbness, and minimal dry cough. He had no dyspnea or other respiratory symptoms. There was no finding on physical examination. Blood tests, cardiac enzymes, and electrocardiogram (EKG) were normal. He had a past medical history of obesity, lower extremity deep vein thrombosis, and intermittent headaches for the last several years. An anterior mediastinal mass was noted on a chest x-ray and a chest CT scan was ordered, revealing a heterogeneous anterior mediastinal mass (Fig. A, white arrowheads) and mediastinal lymphadenopathy. Biopsy was consistent with a primary mediastinal nonseminomatous germ cell tumor (NSGCT), yolk sac histology, with areas of somatic transformation to malignant nerve sheath tumor (Fig. B, hematoxylin and eosin stain, magnification 200×). He underwent 4 cycles of chemotherapy with Cisplatin, Ifosfamide with Mesna and Paclitaxel, followed by resection with clear surgical margins.[ He was then followed-up with imaging for recurrence and at 1 year after resection he was disease free as shown by a positron emission tomography (PET)/ computed tomography (CT) scan performed (Fig. ); follow-up body CT and MRI scans were also negative for disease recurrence. A CT scan performed approximately 2 years after initial surgery, demonstrated 2 new arterially enhancing hepatic lesions appeared on an abdominal CT scan (Fig. A, white arrows). Subsequent MRI showed the same lesions with prominent peri-lesional arterial phase hyperenhancement (Fig. B, arterial phase subtraction images, white arrows) and moderate diffusion restriction (Fig. C, diffusion weighted image, white arrows). A PET/CT scan was performed showing 2 areas (Fig. D, black and white arrows) of mildly increased FDG uptake in the central liver (lesion in hepatic segment 4 SUV max: 4.52, lesion in hepatic segment 8 SUV max: 3.43; liver background SUV mean 1.8). These lesions correspond to the ones seen on contrast-enhanced CT and MRI. No sites of extrahepatic disease were identified. A CT-guided biopsy of one of the liver lesions was performed and demonstrated (Fig. E, hematoxylin and eosin stain, magnification 200×) a hepatic metastatic transformation to angiosarcoma of the primitive NSGCT. Palliative chemotherapy was initiated with the scope of controlling the disease. On a follow-up abdomen CT scan performed 2 months later for diffuse abdominal pain an epiploic appendagitis was noted, and the liver lesions were confirmed to be stable in size.


A 64-year-old female patient presented to the clinical with a history of pain abdomen, localized in the flank, and loss of appetite for 1 month. She had documented a loss of weight of 3 kg. On examination, a right renal lump was palpable. Computed tomography (CT) scan of the abdomen was done before presentation, which showed a large 8.9 cm × 8.6 cm mixed density mass in the right kidney. The patient presented to us with a diagnosis of RCC, for further evaluation and management. To confirm the diagnosis, we did ultrasound guided FNAC from the right renal mass that showed large round cleaved nuclei, coarse chromatin, and scanty dense blue cytoplasm. This was suggestive of NHL. Positron emission tomography–magnetic resonance imaging (PET-MRI) was done to see evidence of metastasis, which showed fluorodeoxyglucose avid thyroid lesion, right renal mass [] with metabolically active subcentimeter nodes in the cervical, mediastinal, and enlarged discrete and confluent retroperitoneal and mesenteric nodes. An excision biopsy from a cervical node was done to classify the disease. Histopathology confirmed DLBCL, which was CD20 positive and Mib-1 labeling index of 80% []. The patient was started on chemotherapy with R-CHOP, which she tolerated well for three cycles. Repeat PET scan was done which showed regression of primary renal lesion and absence of any other lymph node.


A 22-year-old female patient reported to the department of conservative dentistry with a chief complaint of pain and discoloration in relation to an upper front tooth. On examination, it was seen that tooth #11 presented with severe discoloration and considerable tooth structure loss on the palatal aspect []. The patient gave a history of prior attempted endodontic treatment a year back, and she could not follow up the treatment with the dentist as she had relocated. The onset of pain prompted her to seek immediate dental treatment.
Clinical examination revealed significant tooth structure loss in a failed attempt to gain access to the root canal resulting in thinning of dentin on the labial and palatal aspect of the crown and subsequent discoloration due to incomplete root canal treatment []. The tooth was mildly tender to percussion, absence of any swelling, and exhibited physiologic mobility. The radiograph revealed the presence of periapical periodontal widening along with dentin loss extending from middle third of the crown till the junction of the cervical and middle third of the root around the root canal [].
After examining the case, the patient was informed regarding the present condition and the treatment plan was explained, with which she complied. The procedure comprised of root canal treatment, followed by restoration of iatrogenic tooth structure loss with a dentin substitute and intracoronal bleaching.
At the first appointment, the dentinal floor was explored to locate the root canal orifice, which had been obscured due to lodgment of debris since the access opening was exposed to the oral environment for a considerable time. After the canal orifice was located, a no. #15 K-file was used to scout through the canal to reach the apex. The working length was established at 21 mm using no. 30-K file and confirmed using an apex locator and radiograph. The cleaning and shaping was initiated, and thorough irrigation was achieved using sodium hypochlorite 5.25%. The canal was enlarged to size #50 using K-files only. After irrigation and drying of the canal, calcium hydroxide (UltraCal XS, Ultradent Products, Inc.) intracanal medicament was placed and secured with a cotton pellet and temporary restoration.
The subsequent appointment was a week later. There was no tenderness to percussion and patient-reported absence of pain. After removal of the temporary restoration, the canal was further irrigated with sodium hypochlorite 5.25%, normal saline, and final irrigation with chlorhexidine 2%. The canal was dried and prepared for obturation. AH plus sealer and gutta-percha were used for obturation using a lateral condensation method.
At the same appointment, the tooth was prepared for restoration of the lost tooth structure using Biodentine. The access cavity was flushed with saline and dried to remove any sealer and particles of gutta-percha. Biodentine was mixed in a triturator as per instructions and placed in the access cavity. Following this is a crucial step, which was carried out using an anterior Schilder plugger size 11 (1 mm diameter). The plugger was inserted into Biodentine projecting exactly above the coronal end of gutta-percha. It was moved in a circumferential motion to create a short groove of approximately 4-mm length uncovering the coronal portion of gutta-percha. The plugger was continuously moved making a circle of 2-mm diameter along with lateral flaring to form a triangular shape to simulate the pulp chamber. This was done to sustain the artificially sculpted orifice until the Biodentine had set (setting time: 12 mins) eventually replacing the lost dentinal structure as well as maintaining the natural canal anatomy [] []. Subsequently, the pulp chamber was sealed with temporary restoration until next visit.
After a week, the patient was scheduled for intracoronal bleaching procedure. The patient was asymptomatic, and the radiograph revealed periapical healing. The procedure included an initial formation of an intraorifice barrier. Glass-ionomer cement (GIC) was mixed and placed in the artificially created orifice to form a 2-mm thick plug over the gutta-percha with a bobsleigh configuration []. Hydrogen peroxide-based powder and liquid bleaching system (Pola Office) were used for bleaching purpose. The powder and liquid were mixed to the consistency of wet sand and packed into the pulp chamber using a plastic instrument []. A cotton pellet was pressed over the bleaching agent, and a 2-mm thick plug of GIC was used to secure these contents into the pulp chamber [].
The next visit was scheduled after 2 weeks, at which the tooth bleaching was assessed. The patient was satisfied with the esthetic result of bleaching []. Hence, the bleaching agent was carefully evacuated from the pulp chamber and copiously flushed with water. The final restoration was done with fiber-reinforced composite (EverX Posterior) and final increment of conventional composite (3M ESPE Filtek Z350) [].
The patient was asymptomatic when called for a follow-up visit at 2 weeks, 3 months, 6 months and 1 year [].


A 63-year-old African-American female with past medical history of discoid lupus and hypertension presented with worsening right-sided eye swelling for the past 3 weeks. This was associated with a throbbing frontal headache, blurring of vision, nasal congestion, malaise, and weight loss. Patient has allergy to sulfamethoxazole. The medication list included hydrochlorothiazide. No previous surgeries. Social history was significant for smoking. Family history was pertinent for breast cancer of the daughter. Physical examination included diffuse right eyelid edema and right frontal tenderness (). The rest of the examination was unremarkable. Differential diagnosis on admission included giant cell arteritis, conjunctivitis, preseptal cellulitis, glaucoma, acute sinusitis, or cavernous sinus thrombosis. Extensive ophthalmologic evaluation did not show any intraocular abnormality. However, a magnetic resonance imaging (MRI) of the brain showed hyperintense foci in the right frontal calvarium leading to the eye swelling ().
Further evaluation revealed pancytopenia, elevated protein levels, inverse albumin-globulin ratio, elevated β2 microglobulin, elevated erythrocyte sedimentation rate, and a normal C-reactive protein suggestive of a plasma cell dyscrasia which differed from the patient’s lab work 2 years ago. Calcium and serum creatinine levels were within normal limits. A skeletal survey revealed multiple osteolytic lesions. Serum and urine protein electrophoresis revealed elevated immunoglobulin G (IgG) Kappa monoclonal gammopathy. Bone marrow biopsy demonstrated a hypercellular marrow comprised at least 70% mature appearing plasma cells staining positive for CD138 ( and ). Flow cytometry also showed a small population of kappa clonal plasma cells expressing CD38, CD138, and CD56 comprising 2% of the total cellularity, which was negative for CD19 and CD117. Further analysis of lymphocytes shows a predominant T-cell population (78% of lymphocytes) expressing pan T-cell markers and normal CD4/CD8 ratio (1.0). B cells (10% of lymphocytes) are polyclonal. Natural killer (NK) cells are within normal limits. There is no evidence of increased blasts (). Fluorescence in situ hybridization (FISH) test was positive for IGH@ gene rearrangement involving 14q32.3, CCND1/IGH@ gene for translocation (11;14)(q13.3;q32.3), and deletion 13q (RB1) ().
Four consecutive day CyBorD (cyclophosphamide, bortezomib, and dexamethasone) chemotherapy with 500-mg cyclophosphamide (Cytoxan) by mouth, 1.5-mg/m2 bortezomib (Velcade) subcutaneous, and 40-mg dexamethasone by mouth were initiated. Varicella zoster virus prophylaxis with valacyclovir and tumor lysis syndrome prophylaxis with allopurinol was started. Treatment was then changed on a weekly basis. Because of neutropenia, doses of Velcade and Cytoxan were reduced to 1.3 mg/m2 and 300 mg, respectively, during the second cycle of chemotherapy. Because of nausea, valacyclovir was then shifted to acyclovir. She reports pain from the osteolytic lesions, which is relieved with MS Contin and Oxycodone. She declined any orthopedic interventions; thus, she was scheduled to begin palliative radiation therapy for the right humerus, femur, and tibia. After 2 months of chemotherapy, M-spike significantly decreased from 7.2 to 2.5 g/dL, IgG decreased from 8350 to 3689 g/dL, Kappa light chains decreased from 1523 to 316 g/dL, and Kappa/Lambda ratio decreased from 179.1 to 34. She was referred for autologous stem cell transplantation after 14 weeks of treatment. Overall, she tolerated the chemotherapy moderately well and capable of all self-care but unable to carry out any work activities (Eastern Cooperative Oncology Group (ECOG) performance scale: 2) due to moderate fatigue and unsteady gait.


A 55-year-old female with one-sided nasal obstruction and rhinorrhea in her nose for more than five months referred to our center in the ENT department of Khalili Hospital (Shiraz, Iran) in 2014. She was a known case of papillary thyroid carcinoma with metastases to the pelvic and lung. She had a history of severe bone pain and haemoptysis (more than a year) and was a candidate for total laryngectomy and thyroidectomy due to her advanced thyroid cancer. However, due to the dissatisfaction of her visitors, she only underwent total thyroidectomy. Examination of the patient revealed an ulcer mass covered with crust in the anterior and inferior part of the right-sided nasal cavity.
Patient’s PNS CT with contrast revealed an irregular border mild enhancing tumoral solid mass measuring 30×30 mm at the anteroinferior of the right nasal cavity without calcification or lytic osseous lesion at adjacent bone and PNS were clear (). Based on the roll out of vascular lesion, a biopsy was done. The biopsy result indicated high suspicious to carcinoma. Thus, the patient was scheduled for mass excision with functional endoscopic sinus surgery (FESS). After a general and local anesthesia, bilateral endoscopic sinus was done. The left nasal cavity was normal for mucosal lesion, but a mass measuring approximately 3×3 cm was seen in the anteroinferior part of the right nasal cavity that involved uncinate posteriorly and inferior turbinate inferiorly. The mass was removed with inferior turbinate. Right uncinectomy, right antrostomy and right anterior ethmoidectomy were done. Right maxillary sinus was viewed with endoscope and showed no mass involvement. Septum showed neither mass nor involvement. The mass was sent for pathology and the report revealed metastatic papillary cell carcinoma (figures -). The patient was symptoms free at 6-month and 1-year follow-up after the surgery.
Written informed consent was obtained from the patient for the publication of this case report and any accompanying images according to ethical consideration of Shiraz University of Medical Sciences.


A 34-year-old male presented with sudden-onset pain and diminution of vision in the right eye after sustaining trauma at work. The visual acuity at presentation was perception of light. On slit lamp examination, a full thickness corneal tear, para central in the inferotemporal quadrant with pupillary sphincteric tear, and a traumatic cataract with breach in the anterior capsule with a tract extending up to the posterior capsule was noted.
X-ray orbit showed a radiodense object more radio-opaque than bone in the posterior segment, suggestive of a metallic foreign body. Ultrasound examination of the right orbit revealed vitreous hemorrhage. Hyperechoic signals with acoustic shadowing near the optic disc suggested the presence of an IOFB. Computerized tomography confirmed the ultrasound findings [].
Primary closure of the corneal wound with pars plana lensectomy and vitrectomy with the removal of the IOFB was planned. Corneal wound was sealed with two interrupted 10-0 nylon sutures. Twenty-five gauge pars plana lensectomy followed by core vitrectomy was performed. Intraoperatively, after clearing the vitreous hemorrhage, a wedge-shaped metallic foreign body was seen lodged in the superficial layers of the retina inferotemporal to the disc []. Posterior vitreous detachment was created using high vacuum. White discoloration of the retina was noted inferotemporal to the foreign body extending along the inferior arcade and suggestive of a BRAO []. The foreign body was gradually dislodged with the help of the cutter. A pars plana sclerotomy was made using a microvitreoretinal blade in the superotemporal quadrant. The IOFB was removed through the incision using an intraocular magnet. Subsequent to IOFB removal, the sclerotomy was sutured with interrupted 8-0 vicryl sutures. Residual vitreous and hemorrhage was cleared at this stage. Fluid air exchange was carried out and a thorough screening of the peripheral fundus was done with the help of external indentation.
At 1-month follow-up, visual acuity had improved to 6/36 with aphakic correction. Fundus evaluation showed a chorioretinal scar at the impact site and the retina within the inferior arcade showed whitish discoloration []. A deep retinal tear inferior to disc and retinal striae extending across the papillomacular bundle from the optic disc toward the fovea were seen. Spectral-domain optical coherence tomography (SD-OCT) revealed corrugation of the inner retinal layers with loss of differentiation of the retinal layers inferiorly [].
A secondary intraocular lens implantation was planned 3 months postoperatively. On the 7th day after lens implantation, the best-corrected visual acuity improved to an unaided 6/24. Fundus examination revealed decreased pallor of the previously whitish retina [] and better differentiation of the retinal layers on SD-OCT []. Fluorescein angiography was performed which showed normal foveal avascular zone with dipping of the vessels into the chorioretinal scar with mild delayed filling of the inferior retinal artery [].


A 65-year-old male presented to our department with a 3-month history of slowly growing prominence of the right eye. On physical examination, the right eye showed painless proptosis without diplopia. Ocular motility of the right eye was mildly limited both in lateral and downward directions. Hertel exophthalmometer values of the right eye and left eye were 23 mm and 13 mm, respectively. Visual acuity measured with Snellen chart was 0.4 for the right eye and 0.8 for the left eye. Anterior segment examination revealed a clear cornea with grade 1 nuclear sclerosis in both eyes. Fundus examination showed choroidal folds in the right eye.
Orbital magnetic resonance imaging revealed a well-defined mass measuring 32 mm × 31 mm × 25 mm in intraconal region of the right eye, which was isointense relative to the adjacent soft tissue in T1 signal and hyperintense in T2 signal [Fig. and ]. There was no sign of bone erosion or infiltration to adjacent soft tissues. Lateral orbitotomy through upper lid skin crease incision was performed to gain access to the posterior orbit. The dissection extended through orbicularis muscle and deep fascia to the periosteum of the orbital rim. Periosteum along the lateral orbital rim was cut and elevated from the lateral orbital wall. The bone was cut with an oscillating saw, angling the cut slightly inferiorly and parallel to the orbital roof. The cut was made into the thin bone along the sphenozygomatic suture line. A second cut was made through the orbital rim just above the zygomatic arch. Small holes were drilled on either side of each cut near the rim to facilitate later replacement of the bone. The bony rim fractured outward. The periorbita was then opened. A well-circumscribed, encapsulated, and highly vascular lesion was seen. To improve exposure, inferior subciliary approach for inferior orbitotomy was performed following canthotomy and cantholysis. Then, the tumor was freed up entirely with blunt dissection and removed in total []. Macroscopically, the tumor was ovoid, highly vascular, white-gray with smooth borders []. Microscopic examination revealed richly vascular, well-circumscribed, patternless spindle-shaped cells arranged in sheets. Floret-like multinucleated giant cells often lined the pseudovascular spaces []. Immunohistochemistry was performed; spindle cells as well as the giant cells showed reactivity for Vimentin, CD34, and bcl-2 []. Neural crest marker S100, desmin, KI67 and ALK-1 were all negative. The morphology and immunohistochemical profile was consistent with the diagnosis of GCA. Immediate postoperative period was uneventful [Fig. and ]. Proptosis regressed completely with recovery of full extraocular motility. No recurrences were detected over 2-year follow-up with good cosmetic and functional results.


An 80-year-old man, diagnosed with myeloproliferative disease (polycythaemia vera), was admitted after ultrasonography and computed tomography (CT) scan detection () of bilateral parapyelic solid renal lesions that can simulate renal carcinoma. The right mass (6.5 cm sized) infiltrated the pelvicalyceal system, causing extrinsic mass effect and continued in the perirenal spaces. The left solid lesion was 2.3 cm in size. Investigations showed a haemoglobin level of 121 g/L (12.1 g/dL); white blood cell (WBC) 20.1 × 109/L (20.1 × 103/μL), platelet count (PLT) 82 × 109/L (82 × 103/μL); plasma creatinine 141.4 μmol/L (1.6 mg/dL) and estimated glomerular filtration rate (eGFR) 0.70 mL/s (42 mL/min). As some doubts persisted about the moderate contrast enhancement, a CT-guided needle biopsy was performed without complications. The histological examination was compatible with the final diagnosis of EMH, containing cells of three distinct lineages including myeloid and erythroid cells and rare megakaryocytes. The immunohistochemical staining was positive for myeloperoxidases and glycophorin (), while CD34 staining was negative. No other signs of EMH were detected in the abdominal parenchymas. The conclusion of a subsequent bone marrow biopsy indicate myelofibrosis post-polycythaemia and he was treated with hydroxyurea and allopurinol. The patient is still alive 28 months after hospital admission.


A 79-year-old man with a previous history of ischaemic cardiopathy was admitted to another department with persistent fever and complaints of fatigue and weakness. Examination revealed splenomegaly. Haemoglobin was 113 g/L (11.3 g/dL), WBC 17 × 109/L (17 × 103/μL), PLT 676 × 109/L (676 × 103/μL); plasma creatinine 101.6 μmol/L (1.15 mg/dL); eGFR 1.01 mL/s (61 mL/min) and lactate dehydrogenase 1394U/L. Abdominal ultrasonography and magnetic resonance imaging (MRI) showed a perirenal infiltrating tissue associated with hepatosplenomegaly. Given the suspicion of lymphoma, the patient performed an osteomedullary biopsy that showed idiopathic myelofibrosis. A CT () confirmed bilateral perirenal tissue with modest contrastographic impregnation and showed a solid mass in the lower pole of the right kidney with intense contrast enhancement. The patient was referred to us for a CT-guided needle biopsy that revealed the co-presence of two different lesions to the right a clear cell renal carcinoma, while the bilateral perirenal tissue was haematopoietic tissue, confirmed by immunohistochemical cell phenotype. The patient underwent a polar right nephrectomy and he is still alive 24 months after diagnosis with a minor renal dysfunction, plasma creatinine 114.9 μmol/L (1.3 mg/dL) and eGFR 0.88 mL/s (53 mL/min).


A 28-year-old male patient was admitted to the emergency room with impaired consciousness after ingesting antifreeze for suicide purpose about 5 h earlier. At the time of admission, his guardians brought the container of the antifreeze that he had ingested, which was then checked accordingly. The patient had an elevated blood pressure of 130/79 mmHg, pulse rate of 98 beats/min, and respiration rate of 30 breaths/min at the time of admission; his body temperature was 35.4℃. He showed signs of acute illness and presented with lethargic consciousness and restlessness. No other specific findings were found. The venous blood gas analysis results showed pH 6.90, PaCO2 34 mmHg, PaO2 25 mmHg, bicarbonate 6.7 mmol/L, base excess −26.1 mmol/L, and oxygen saturation 15%. Serum biochemical analysis showed sodium 136.4 mEq/L, potassium 7.80 mEq/L, chloride 110 mEq/L, calcium 9.9 mg/dL, phosphorus 3.2 mg/dL, glucose 125 mg/dL, osmolality 375 mOsmol/kg, blood urea nitrogen (BUN) 11 mg/dL, and creatinine 1.67 mg/dL. The patient also showed signs of high anion gap metabolic acidosis with an anion gap of 19.7 mmol/L and osmolar gap of 91.34 mOsm/kg H20. Urinalysis showed proteinuria and microscopic hematuria. Electrocardiogram findings showed sinus tachycardia with a heart rate of 134 beats/min, whereas chest radiography showed mild pulmonary edema. Fluid replacement, sodium bicarbonate, and thiamine were administered as the initial treatment, and fomepizole was loaded at 15 mg/kg intravenously and administered four times, each at 10 mg/kg in 12-h intervals. One hour after admission, the patient's consciousness deteriorated from lethargy to stupor, necessitating tracheal intubation. Two hours after admission, overall condition deteriorated and acidosis was not corrected. Consequently, he was transferred to the intensive care unit, where continuous renal replacement therapy was initiated. Thirty hours after dialysis, metabolic acidosis was controlled, as indicated by pH 7.46, PaCO2 36 mmHg, PaO2 106 mmHg, HCO3 25.6 mEq/L, and anion gap 5.9 mmol/L (). His vital signs stabilized on the 6th day of admission, and consequently, he was extubated. However, the patient's levels of BUN and creatinine remained elevated at 39 and 4.28 mg/dL, respectively, and his hourly urine output was only 10–30 mL, and calcium oxalate crystals were detected on urine microscopy (). The patient's acute renal failure persisted, and therefore the treatment was switched to routine hemodialysis, maintained at three rounds every other day. Starting from the 12th day of admission and the start of dialysis, daily urine output increased to >1,000 mL; thus, dialysis was discontinued. On the 23rd day of admission, the serum BUN/creatinine level decreased to 18.4/1.73 mg/dL, and the daily urine output was being maintained at 3000–4000 mL. The patient was discharged and monitored through Follow-ups. During the outpatient follow-up visit at 3 months after discharge, the levels of BUN and creatinine were 17 and 0.95 mg/dL, respectively, with no proteinuria or hematuria ().


A 71-year-old Caucasian female with end-stage renal disease due to p-antineutrophil cytoplasmic antibodies-positive vasculitis underwent cadaveric kidney transplantation in April 2012. It is also important to emphasize that the patient had a previous history of moderate aortic stenosis with aortic valve area 1.5 cm2 but she rejected further diagnostics and treatment.
In May 2016, she presented to the emergency department with dyspnea, anasarca, and precordial oppressions. Signs of global heart failure were present and confirmed by the diagnostic imaging techniques. No bleeding tendency was present in patient's laboratory values– they included a red blood cell count 3.34 × 1012/L, Hg 91 g/L, platelet count 196 × 109/L, prothrombin time 0.98, international normalized ratio 1.02, and activated partial thromboplastin time 29.3 s. Cardiac enzymes were negative (creatine kinase –40 U/L, lactate dehydrogenase – low-density lipoprotein [LDH] 126 U/L, troponin 9 ng/L) and worsening of allograft function (creatinine 200 μmol/L, previously 153 μmol/L) was present. Thus, a double lumen CVC was placed in the right subclavian vein and intensified hemodialysis was commenced, the total of nine sessions. During the CVC insertion, the patient was restless and disorientated. The chest radiography revealed catheter tip in the right atrium and no signs of pneumothorax. However, due to further worsening of the symptoms-patient became hypotensive, dyspnoic, tachycardic, and agitated, she was immediately transferred to the Intensive Care Unit. There was no laboratory sign of bleeding (red blood cell count 3.42 × 1012/L, Hg 97 g/L, platelet count 186 × 109/L, LDH 110 U/L). Pulmonary angiography outruled pulmonary embolism or signs of pneumonia. However, in comparison to previous scans from June 2013, it revealed pericardial effusion 2 cm thick []. During the next 3 days, patients were stabilized with intravenous fluids without vasopressors, echocardiography was performed and showed concentric hypertrophy of the left ventricle, with ejection fraction 70%, and without regional hypocontractility. There was also pericardial effusion 2 cm thick and without repercussions on the blood flow, predominantly reclining the free surface of right atrium, with fibrin scar tissue covering the epicardium (differential diagnosis – spot of previous cardiac wall perforation, spontaneously recovered) []. With careful fluid administration and antibiotic treatment, the patient became hemodynamicaly stabile with good diuresis and stabile allograft function (creatinine after the discharge 147 μmol/L) without the need for dialysis treatment. Before the discharge, echocardiography was performed once more. The pericardial effusion was now in regression; it was 5–6 mm thick and without repercussions on the blood flow []. CVC was hence extirpated without further complications during the last outpatient clinic visit in June 2016.


An 8-year-old male patient weighing 15 kg with congenital heart disease was referred to our institute with provisional diagnosis of infective endocarditis following two episodes of seizure and episodes of fever for the past 20 days. His general physical examination revealed cyanosis, clubbing, and features of developmental delay. Auscultation of chest revealed Grade IV pansystolic murmur over precordium. Neurological examination was unremarkable. Laboratory investigation showed Hb 17.3 g%, normal total leukocyte count, platelet count, and sterile blood culture. A transthoracic echocardiogram demonstrated situs solitus, levocardia, atrioventricular concordance, ventriculoarterial discordance, a large VSD (size 27 mm) with severe pulmonary stenosis (peak gradient of 80 mmHg), and a large (18 mm × 14 mm) mobile mass attached to the pulmonary valve. Computerized tomography pulmonary angiogram showed normal-sized pulmonary vessels with no evidence of pulmonary thromboembolism. The diagnosis of d-TGA with pulmonary valve stenosis and pulmonary valve endocarditis was made. He was planned for excision of vegetation.
His preoperative systemic oxygen saturation on room air was 85%. A transesophageal echocardiography examination after induction of anesthesia in midesophageal five-chamber view confirmed the presence of a large VSD and a mass attached to the undersurface of the pulmonary valve. In addition, a thick cord-like structure originating from the endocardial surface of the left ventricle was found attached to the mass [ and ]. Color Doppler examination showed turbulent flow across the pulmonary valve []. Continuous-wave Doppler confirmed the gradient across the obstruction. In midesophageal four-chamber view, chordae from the tricuspid valve were seen attached to the tip of interventricular septum (IVS) [ and ]. Midesophageal aortic long-axis view showed a mobile mass attached to the undersurface of the pulmonary valve, a large VSD, and normal-appearing pulmonary and aortic valves. A careful look showed the mass moving with the movement of the pulmonary valve and a chord-like structure attached to the mass after originating from both right and left ventricles [ and ]. The surgeon was informed about tricuspid valve straddling and LVOT obstruction due to tricuspid valve tissue. However, in view of the clinical presentation of fever, we decided to proceed with surgery.
The intraoperative surgical finding confirmed straddling of the tricuspid valve, and the mass attached to the pulmonary valve appeared as normal tricuspid subvalvular tissue. The pulmonary valve leaflets appeared normal. Excision of mass was abandoned, and a small piece of tissue from it was excised for histopathological confirmation. Postoperatively, the patient was discharged from hospital after a week of uneventful course with long-term plan for single-ventricle pathway. The histopathology confirmed the excised tissue as normal myocardium.


A 14-year-old male became acutely tetraplegic 12 h following treatment for tracheal stenosis. Three months earlier, after treatment for a metatarsal fracture, he sustained a respiratory arrest for 30 min; this was attributed to a massive pulmonary embolism. He was hospitalized in the intensive care unit (ICU) for the next 2 months. After discharge, he suffered from tracheal stenosis requiring tracheal resection/reconstruction. His neurological status before surgery was normal except for 4/5 motor power in his left distal lower extremity. Within 12 h of surgery, he became acutely tetraplegic with a C4 sensory level. His neurological examination revealed dissociated sensory loss with preservation of deep sensations (position and vibration). Anal tone was absent and plantar responses were mute.
The T2-weighted cervical magnetic resonance imaging revealed a diffuse hyperintense signal, with accompanying diffuse cord swelling from C2-C7 []. The sagittal cervical computed tomography (CT) additionally demonstrated cervical kyphosis and stenosis []. Although the diagnosis of an anterior spinal artery syndrome (ASAS) was established, resulting in the administration of high-dose methylprednisolone (30 mg/kg loading dose, 5.4 mg/kg maintenance dose) for 5 days, his deficit remained unchanged.


A 65-year-old male was admitted following a road traffic accident with Glasgow Coma Score of 15/15, normal vitals and temperature, acute paraplegia of 0/5, decreased sensations below D8 level, and American Spinal Injury Association score of A. Imaging revealed D10 fracture – dislocation with cord compression []. He underwent dorsal decompression – stabilization under nonneuroleptic general anesthesia. The patient was on prophylactic antibiotics, deep venous thrombosis prophylaxis, and other supportive measures till 9th day when there was a temperature spike (oral) of 38.9°C that further rose to 40.6°C on the following day []. Antipyretics, tepid sponging, cold gastric lavage, and axillary/inguinal ice packs were unsuccessful. Physical examination and pan-cultures (cerebrospinal fluid [CSF], blood, urine, and sputum done on the day of first spike of fever and repeated 48 h later) were unremarkable. Examination of surgical site showed no collection or discharge and the wound was healthy. His C-reactive protein (CRP), thyroid function, and cortisol levels were within the normal limits, with leukocytosis of 18,000 (neutrophils – 80%). Drug-induced hyperthermia was excluded.
The patient was shifted from a ward with temperatures around 30°C–35°C to Intensive Care Unit with temperature set at 18°C, followed by immediate and sustained temperature normalization (37°C) []. This was followed by gradual weaning back to the ambient temperature over 5 days and an uneventful course in hospital thereafter.


A 19-month-old female patient weighing 6.7 kg was admitted for atrial septal defect (ASD) surgery. She had undergone omphalocele repair during the neonatal period. She had been admitted for pneumonia and desaturation at the age of 5 months. Left main bronchus focal narrowing developed at the age of 5 months, based on chest computed tomography (CT). However, the respiratory symptoms and pneumonic infiltration improved on follow-up chest X-rays, so we did not consider the left main bronchus to be a major issue. Subsequently, she was admitted 4 times for a recurring lung issue in the left lung field. We rechecked the chest CT and found an enlarged main pulmonary artery (PA) with a right aortic arch, accompanied by a 10-mm ASD compressed in the left main bronchus between the right-sided descending thoracic aorta and enlarged right PA (RPA) (). The presence of a 10-mm ASD with a significant left-to-right shunt and a tricuspid valve regurgitation velocity of 2 m/sec was confirmed on echocardiography.
We considered several surgical options: (1) ASD closure only, (2) ASD closure and aortopexy via right thoracotomy as a staged operation, (3) ASD closure and aortopexy via sternotomy, (4) ASD closure and RPA anterior translocation, and (5) ASD closure and slide tracheoplasty. Among these surgical possibilities, we considered options 1, 3, and 4 to be the best procedures to undertake as the first surgical procedure.
Under general anesthesia, the patient underwent a median sternotomy. We pulled the ascending aorta upward with forceps, avoiding the dissection of other tissue, and we checked whether the left main bronchus showed improvement under bronchoscopic guidance. However, we did not observe bronchial improvement. Thus, we decided to perform ASD closure with RPA anterior translocation. We conducted a routine cannulation and extensive PA mobilization to the second PA branch under cardiopulmonary bypass support. Aortic cross-clamping (ACC) was performed. The ASD was closed with a glutaraldehyde-fixed autologous pericardium after a right atrial incision was made. The RPA was excised from the posterolateral wall of the main PA under spontaneous beating after ACC was removed.
The length of the RPA was adequate to make a direct anastomosis, so a direct anastomosis of the anteriorly translocated RPA to the main PA was performed (). The cardiopulmonary bypass weaning was smooth. The cardiopulmonary bypass time and ACC time were 132 and 25 minutes, respectively.
The intubation tube was removed on the first postoperative day (POD). However, reintubation was conducted on the third POD due to CO2 retention. We conducted successful re-extubation on the patient’s fifth POD after extensive lung care. She was discharged on the 14th POD. We conducted postoperative CT angiography 2 months later. We found improvement in the narrowing of the left main bronchus ( 5 mm) and no narrowing site in the RPA () . Improved left lung aeration was shown on a chest X-ray. The patient was doing well as of the latest follow-up.


An 82-year-old woman was referred to the Department of Gastroenterology at Keio University Hospital for unresolved diarrhea. Five months earlier, she had been diagnosed with left-sided mild active UC and treated with oral 5-aminosalicylic acid (5-ASA), which resolved the diarrhea. During the treatment of UC, she was administered 2 weeks of antibiotics for fever and suspected bacterial infection. Two months after the antibiotic treatment, she presented at another hospital with watery diarrhea, loss of appetite and was admitted for further evaluation. Stool examination by enzyme immunoassay (EIA) revealed toxins A and B. She was administered 2 courses of oral vancomycin (500 mg 4 times per day), which resolved her diarrhea and loss of appetite. However, CDI recurred a few days after each treatment. Therefore, she was referred to our hospital to undergo FMT, with her 48-year-old daughter as the donor. After signing the informed consent, she participated in a clinical trial of FMT approved by the ethics committees of Keio University on February 24, 2014.
Donor's stool was screened for transmissible diseases and pathogens, including C. difficile. Donor's blood was tested for HAV, HBV, HCV, human immunodeficiency, and human T-cell lymphotropic type 1 viruses, and Treponema pallidum. All these test results were negative. The donor had no history of IBS, IBD, diabetes mellitus, chronic diarrhea, chronic constipation, atopic dermatitis, morbid obesity, or cancer. On the morning of transplantation, fresh donor feces were collected. One hundred grams of the donated fecal matter were mixed with 200 mL of normal saline and homogenized. The fecal suspension was filtered with a sterile coarse metallic mesh, fine metallic mesh, and cotton gauze to remove large masses of feces and smoothen the texture. Following bowel lavage with 2 L of polyethylene glycol, the recipient underwent FMT via colonoscopy. The colonoscopy revealed diffuse erythematous mucosa with edema throughout the entire colon ().
On post-FMT day 4, the symptoms of diarrhea, fecal incontinence, and abdominal distension resolved without any complications. EIA test was negative for fecal toxins, although stool culture yielded toxigenic C. difficile . On post-FMT day 11, her stool culture was negative for C. difficile ().
C. difficile isolates from the first and third CDI episodes were available for analysis. Both isolates were positive for toxins A and B, but negative for binary toxin (actin specific ADP-ribosyltransferase). PCR-ribotype analysis showed that both isolates were ribotype 014 (). The gut microbiota of the stool specimens obtained from the patient before FMT and 1, 2, 4, 8, and 12 weeks after FMT were analyzed by pyrosequencing of the bacterial 16S rRNA gene (). Operational taxonomic unit (OTU) analysis of C. difficile showed that the stool sample before FMT was positive for C. difficile OTU, but the stool samples after FMT were negative. Furthermore, the total number of OTUs before FMT was under 100, whereas the total number of OTUs after FMT was 150 to 200. Shannon index improved from 2.8 to about 3.5 after FMT (). These results indicated that FMT could restore the diversity of the microbiota in the intestines and induce symbiosis.
No recurrence of CDI was observed for 1 year after FMT. UC remained in remission, as observed in clinical symptoms and endoscopic findings, before and until 5 months after FMT. Five months after FMT, the patient presented with stools containing mucous and blood. Testing for toxins A/B by EIA was negative. We diagnosed the patient with UC relapse without CDI recurrence and her partial Mayo (pMayo) score was 4. Additional suppository treatment with 5-ASA was needed. Five weeks of treatment with topical 5-ASA resulted in remission and reduced the pMayo score to 1.


A 6-year-old boy with a background of asthma presented to a tertiary paediatric unit in May 2016 with a 3-day history of right eye pain, proptosis and erythema under the care of the ENT team. A provisional diagnosis of a periorbital cellulitis was made secondary to ethmoid sinusitis and management with intravenous antibiotics (ceftriaxone), intranasal steroids, saline douches and topical oxyxlometazoline was initiated. Ophthalmological assessment demonstrated normal colour vision, acuity and light reflexes bilaterally but also proptosis-related ophthalmoplegia. Computed tomography (CT) imaging of the orbits and paranasal sinuses demonstrated complete opacification of the right-sided paranasal sinuses and compromise of the right frontal and ostiomeatal unit. In addition there was an expansile abnormality centred on the ethmoidal labyrinth with bony remodelling of the lamina papyracea and a subperiosteal collection adjacent to the medial orbital wall (Figs and ). The most likely diagnosis was felt to be an infective process with mucopyocele formation complicated by a subperisoteal post-septal collection. An MRI scan with gadolinium of the orbits and sinuses confirmed the unilateral pattern of sinus opacification within the right frontal, ethmoids and maxillary sinus. The lesion demonstrated multiple fluid–fluid levels and peripheral enhancement (Figs and ). The patient was treated by endoscopic drainage of the lesion, which revealed only blood. He initially had some improvement of his proptosis but recurred within a few days and so further, more extensive endoscopic debridement was performed.
At this point the MRI was reviewed and the possibility of an ABC was raised. This was due to the presence of fluid–fluid levels, cyst within a cyst appearance and lack of restricted diffusion within the sinonasal contents and material drained at the time of the first endoscopic procedure. He was discharged home on oral co-amoxiclav, and topical betamethasone nose drops.
Unfortunately, in June 2016 his symptoms recurred again, with evidence of soft tissue swelling medial to the medial canthus. Further imaging (CT and MRI) was requested and he was referred to both a quaternary centre for ENT surgery and a bone tumour multidisciplinary team who agreed that the lesion was most likely to represent a primary ABC. In the interim he remained under close evaluation by the ophthalmologists.
He underwent a third surgical procedure in July 2016, this time a joint external and open approach. The histopathology confirmed an ABC and his immediate post-operative was uneventful. His proptosis and ocular motility improved slowly.
Regrettably, the ABC recurred again by October 2016 and the imaging demonstrated substantial erosion of the anterior cranial fossa floor that required a bicoronal and transcranial approach with reconstruction of the medial orbital wall.
Currently (November 2017), he remains well, with near total resolution of the proptosis. His visual acuity is normal and the ophthalmoplegia has resolved. Post-operative imaging has demonstrated good clearance and removal of the cyst (Figs and ).


Here, we present a case of 52-year-old female who was referred to our thyroid clinic 1 year after she underwent total thyroidectomy (TT) for solitary thyroid nodule which was Bethesda category VI on fine-needle aspiration cytology (FNAC). Later histopathology showed it to be papillary microcarcinoma of size 8 mm. Six months following her surgery, she started complaining of anorexia, fatigue, and right hypochondriac pain. An ultrasound abdomen revealed multiple hypoechoic liver lesions, which were suspicious for metastases. In the next 6 months, she also underwent a contrast-enhanced computed tomography (CECT) and fluorodeoxyglucose-positron emission tomography-computed tomography (FDG-PET-CT) for the evaluation of the liver lesion. CECT revealed large hypodense enhancing lesion involving the right lobe of the liver and FDG-PET-CT showed them to be hypermetabolic, suggesting to be of a metastatic disease [Figure –]. She then underwent FNAC from the liver lesion which was reported as metastatic carcinoma. In view of a known thyroid malignancy, the primary clinician attributed the metastasis to be of thyroid origin and referred the patient to our center for radioiodine therapy. As per our institute protocol, 4 weeks off thyroxine, 100 mCi of empirical 131-I therapy was planned considering the possibility of non-thyroglobulin (Tg) secreting metastasis as serum Tg was <2 ng/ml. However, the posttherapy I-131 whole body scan did not show any abnormal concentration in the liver [ and ; anterior and posterior images, respectively]. In view of normal Tg, nonradioiodine concentrating liver lesion and history of micropapillary carcinoma, which is rarely metastatic, possibility of second malignancy was considered. To confirm the diagnosis, a repeat liver biopsy was planned at our institute. The histopathology revealed neuroendocrine tumor (NET) cells [ and ] which were immunopositive for chromogranin and synaptophysin [ and , respectively]. MIB-1 index could not be assessed due to tiny nature of the biopsy. Later, her serum chromogranin level was found to be >650 ng/l. Subsequently, a 68-Ga-DOTANOC PET-CT was planned which showed somatostatin receptor expressing tumor in both lobes of the liver with extensive skeletal metastasis [–; transaxial CT, PET-CT, and maximum intensity projection images, respectively]. Here, DOTANOC PET-CT detected skeletal metastasis which was not detected previously. In spite of both FDG and DOTANOC PET-CT, primary tumor remained undetected. In view of extensive metastatic disease, she was put on long-acting octreotide and planned for 177-Lutetium DOTATATE therapy.


A 75-year-old male presented to clinic with a decline in mental status following 6 weeks of progressive weakness. His past medical history included hypertension, coronary artery disease, stable chronic lymphoid leukemia (CLL) and newly diagnosed congestive heart failure (CHF) with moderate aortic valve insufficiency seen on outpatient echocardiogram 3 weeks earlier. Vital signs were within normal limits, and physical exam revealed a non-focal neurologic examination, diastolic heart murmur and diffuse mild abdominal pain. Admission laboratories were notable for WBC of 18 500/mm3 (slightly increased above CLL baseline), with neutrophil predominance. Brain MRI (Fig. ) and trans-esophageal echocardiography revealed multiple bilateral areas of restricted diffusion consistent with ischemic stroke, as well as a 2.2 cm aortic valve vegetation with severe aortic regurgitation, but no intra-cardiac abscess. Blood cultures confirmed Enterococcus faecalis endocarditis. Abdominal CT scan (Fig. ) revealed an 8.1 cm splenic lesion consistent with splenic abscess.
Though hemodynamically stable, his neurologic status deteriorated despite antibiotic therapy, with signs and symptoms of worsening CHF. A multi-disciplinary team determined emergent aortic VR (AVR) was necessary given his cardiac decompensation and ongoing neurologic decline. Simultaneous splenectomy and AVR were not considered a viable option. Instead, the splenic abscess was percutaneously drained and the patient underwent successful AVR with a 25 mm Magna bioprosthetic valve and two-vessel coronary artery bypass grafting. His post-operative course was largely unremarkable—however, severe deconditioning and malnutrition prevented interval splenectomy. His drain was removed and he discharged to a transitional care unit (TCU) with IV antibiotics.
After 12 weeks of intravenous antibiotic treatment, repeat CT revealed a persistent 7-cm splenic fluid collection. Definitive splenectomy was recommended to prevent re-infection of the aortic valve, and he underwent an uncomplicated robotic-assisted (da Vinci) splenectomy. His post-operative course was uneventful, and he discharged to TCU in stable condition. At 2-year follow-up, patient is doing well living independently at home.


A 40-month-old male patient, who was a product of spontaneous vaginal delivery after full-term uneventful pregnancy to a healthy 32-year-old woman and her 34-year-old first cousin. Birth weight was 3.28 kg, length at 50 cm, and head circumference at 34.5 cm, all were around the median, with APGAR scores of 9 and 9 at 5 and 10 minutes, respectively. The patient underwent newborn screening tests as part of the routine care for all newborns, which showed elevated methionine at 105 (11–27) µmol/L. A repeated sample confirmed the elevation at 85 µmol/L. Clinically the patient was stable with unremarkable complete systemic examination.
Other metabolic investigations were requested including homocysteine, and urine for organic acids and methylmalonic acid but all were unremarkable. Renal and liver functions were unremarkable.
At the age of 2 months, the patient developed irritability and skin eruption consisting of multiple scaly and erosive hyperkeratotic plaques all over the body but most pronounced on lower extremities. In addition to leukotrichia and positive hair pull test (), his zinc level was very low at 3.4 (9.18–18.36) µmol/L. All skin eruptions started to resolve after oral zinc supplementation over 2 weeks ().
At the age of 6 months, the final diagnosis was reached by the genetic study of MAT1A (NM_000429.2), which revealed a homozygous variant in c.1081G>T (p.Val361Phe). In silico study predictions were as follows: Mutation taster predicted a disease-causing variant, PolyPhen2: possibly damaging (score: 0.889) and SIFT: damaging variant. Further genetic testing to rule out acrodermatitis enteropathica (OMIM # 201100) caused by SLC39A4 (NM_130849) mutation was negative. All the genetic tests requested for the patient were single gene sequencing done at commercial accredited labs.
In addition, the patient was found to have iron deficiency, which was discovered at 9 months of age; hemoglobin level was 90 (110–145) g/L, mean corpuscular volume (MCV) 62.4 (75–89) fL, mean corpuscular hemoglobin (MCH) 17 (25–30) pg, serum iron 4.8 (9–21.5) µmol/L, and ferritin 7 (21.8–274.6) µg/L. Consequently, ferrous sulfate was prescribed.
Of note, the patient’s nutritional history was unremarkable during the time he developed the symptoms. He had combined breast and formula feeding initially then he was receiving regular diet. His feeding was well and he had acceptable feeding habits. The family history was negative for MAT, zinc, or iron deficiency.
Currently, the patient is 40 months old with normal developmental milestones except for mild speech delay in the form of dysarthria. All his growth parameters are around the median. He is still on zinc and iron supplementation. All the skin and hair symptoms improved. The patient’s zinc level is still low at 6.79 (9.18–18.36) µmol/L. The serum fer-ritin is still low at 13.7 (21.8–274.6) µg/L, hemoglobin was normalized at 128 g/L, while MCV and MCH are slightly low at 71.1 (75–89) fL and 24.2 (25–30) pg, respectively. Latest lab investigations showed methionine at 34 µmol/L and homocysteine at 6.1 (≤15) µmol/L. The highest methionine reading reported for our patient was 110 µmol/L and the average was at 59.2 µmol/L.


A 72-year-old man was known for a chronic HCV infection, treated with success in the past with actual undetectable viremy. On routine ultrasound screening, a hepatic nodular lesion was found in segment 8 (S8). Alpha-fetoprotein was low (4.7 ng/ml) and liver function was normal. A 4-phase CT-scan confirmed a 5.5 × 4.7 × 5.2 cm3 lesion typical for HCC, and showed a second 2 cm hypovascular lesion in segment 2 (S2) (Fig. ). The staging was negative for distant metastases, but a 4.6 × 3.7 × 4 cm3 lymph node was observed in the coeliac region, retrospectively present on a CT-scan made 3 years before. The MRI confirmed the CT findings. A percutaneous US-guided biopsy of the S8 lesion confirmed a classical HCC, with a trabecular growth pattern, while a biopsy of the coeliac node, by US-endoscopy, showed carcinoma cells. After initial proposition of a palliative treatment with Sorafenib, the patient’s case was re-assessed, basing on the possibility of two different lesions: a classical hypervascular HCC in S8, and a hypovascular and slow growing second one in S2 with a positive lymph node. An F18-FDG PET scan showed a clear hypermetabolism in the S2 nodule and in the coeliac lymph node, while no capitation was found in S8 lesion.
A multimodal minimally invasive approach was decided at multidisciplinary board.
The S8 HCC lesion was successfully treated with TACE (lipiodol-epirubicin injection), while the second lesion was resected laparoscopically with a lateral left sectionectomy and a complete lymphadenectomy of the hepatic pedicle and coeliac trunk, including a large lymph node of the common hepatic artery (Figs and ).
The postoperative course was uneventful and the patient was discharged home 5 days after the operation.
Surprisingly the final pathologic analysis revealed a medullary-like hepatocarcinoma, pT1, pN1 (coeliac node), R0. To date, after 12 months follow-up, the patient is alive and disease-free.


A 51-year-old female with a 25 pack year smoking history and newly diagnosed right breast cancer underwent right skin sparing mastectomy with sentinel lymph node biopsy, left prophylactic skin sparing mastectomy and immediate tissue expander reconstruction at our institution. The patient had right breast stage IA multifocal invasive ductal carcinoma and multifocal ductal carcinoma in situ (DCIS). Margins were negative with invasive carcinoma closest at 0.7 cm and DCIS closest at 0.5 cm. Sentinel lymph node biopsy was negative for metastatic carcinoma. The patient denied history of coronary artery disease, myocardial infarction, congestive heart failure, diabetes mellitus, kidney or lung disease, venous thromboembolism, or any bleeding disorders. She likewise denied drug use or daily alcohol consumption. Having stopped smoking for several months prior to the surgery, the patient did not consider herself a smoker and reported herself as a “non-smoker”. On physical examination, she had 40 DD cup breasts with grade 3 ptosis. The patient was deemed an American Society of Anesthesiologists Physical Status of 2, indicating mild systemic disease. She was a good candidate for skin sparing mastectomy because her breasts were not pendulous. 700 mL fill tissue expanders were placed sub-pectorally with Flex HD support for the inferolateral breast pocket. Each expander was filled with 500 mL intraoperatively. At conclusion of the mastectomy, her flap appeared viable with good capillary refill. At completion of expander placement, her flaps continued to appear very viable. Her postoperative course was complicated by extensive bilateral mastectomy skin flap necrosis (). This was managed with tissue expander removal, debridement and primary closure two weeks after her initial surgery given the need to start chemotherapy. It was only after post-hoc questioning that the patient revealed that she was actively engaging in e-cigarette use. Indeed, she noted switching to e-cigarettes approximately 3 months prior to her surgery due to the belief they were safer than traditional cigarettes and maintained a usage similar to her previous 1.5 pack per day amount.


A 43-year-old man with paraplegia for 17 years presented with dark purple discoloration of left second and third toes beginning 3 days earlier. He complained of having a cold sensation in both feet for 1 year. He was diagnosed with hypertension 4 years earlier and was taking hydrochlorothiazide-valsartan. He had also been taking kallidinogenase, ranitidine, levosulpiride, phloroglucinol, and loxoprofen. Physical examination showed that both of the patient’s feet were cold. However, the pulse was palpable on ankles and heels on both sides. A portable Doppler device (Minidop ES-100VX, Hadeco, Japan) showed that the pulse signal intensity of the left and right dorsalis pedis artery was 4–6 and 10 cm/s each, and that of the posterior tibial artery was 6–8 and 10 cm/s each. In addition, the capillary refilling time for both toes was > 10 s. Even though a vascular function test with air-calibrated plethysmography showed a normal segmental arterial pressure ratio in thigh to ankle, normal ankle/brachial systolic index (ABI) in both ankles (> 0.9), and normal triphasic pulse wave pattern in thigh to ankle (Fig. ), the skin over the left toes showed dark discoloration with skin necrosis. His antihypertensive drug was changed to a calcium channel blocker (amlodipine besylate 2.5 mg per day) under suspicion of Raynaud’s phenomenon aggravated by hydrochlorothiazide, and the cold sensation in both toes and forefeet decreased and the capillary refilling time normalized within 3 days. The wound bed became well vascularized and was filled with pink granulation tissue (Fig. ). The skin defect was too large for spontaneous healing, and a medium split-thickness skin graft was performed with the foot sole as a donor site (Fig. ). The wound healed without complications.


A 51-year-old man with paraplegia for 10 years presented with severe dark purple discoloration of right first and second toes, and left first, fourth, and fifth toes in October 2015. Those wounds were recurrent unstable wounds presenting every year from early October to late January, beginning 5 years earlier. He was diagnosed with hypertension 12 years earlier and was taking a mixture containing olmesartan medoxmil 20 mg and hydrochlorothiazide 6.25 mg. Physical examination showed that both of the patient’s feet were cold. However, a weak pulse was palpable on ankles and heels on both sides. A portable Doppler device (Minidop ES-100VX, Hadeco, Japan) showed that the pulse signal intensity of the left and right dorsalis pedis artery was 4 and 10 cm/s, respectively. In addition, the capillary refilling time for both toes was longer than 7 s. Subsequently, his antihypertensive drug was changed to olmesartan medoxmil 20 mg and amlodipine besylate 2.5 mg per day under suspicion of Raynaud’s phenomenon aggravated by hydrochlorothiazide. The cold sensation in both toes and forefeet did not decrease; however, the capillary refilling time was normalized to < 2 s within 7 days. He has not had any unstable wounds so far for 30 months.


We present the case of a previously healthy, now 67-year-old female patient developing vertigo, nystagmus, wide-based gait, and memory loss throughout July and August 1999 (49 years of age at that time). A Computed Tomography (CT) scan of the brain (August 1999) revealed a 35-mm macrolobulated, extraaxial, brightly contrast enhancing mass in the left posterior fossa with extension along the petrous temporal bone, tentorium cerebelli and sigmoid sinus, with cerebellar edema, compression of the fourth ventricle, supratentorial hydrocephalus as well as erosion of the petrous temporal bone. The tumor was radiologically assessed as a meningioma. Subtotal resection (STR) and ventricular drain placement were performed shortly thereafter (Sept 1999). Audiograms prior and after this surgical intervention are currently not available for review. The patient's condition improved after surgery (pre-operative KPS = 70, post-operative KPS = 90). The initial microscopic evaluation proved complex; after major scrutiny, the pathological report described the tumor as a fibroblastic meningioma.
The patient was then lost to follow-up until readmitted six years later (2005) for unilateral progressive hearing loss and pressure like symptoms in her left ear. A clinical examination revealed abnormal bulging of the left tympanic membrane and conductive hearing loss assessed as Gardner-Robertson grade I. A new CT scan and a corresponding MRI (May 2005) showed heterogeneous contrast enhancement suspicious of a local recurrence. The patient underwent a second surgery (August 2005) again assessed as STR. The histopathological examination revealed this time an endolymphatic sac tumor (ELST). Available medical data showed no evidence of von Hippel Lindau (VHL) disease screening prior to both surgeries; to our knowledge, the patient has no known criteria for VHL disease.
Follow-up imaging between 2006 and 2008 showed gradual, but very slow increase in size of a suspected residual/recurrent tumor. A follow-up MRI in February 2009 confirmed a local recurrence in the left sigmoid sinus region and the patient underwent a third surgery (STR) in February 2009. The corresponding pre-operative audiogram is currently not available. The histopathology confirmed an ELST [; Figure , and ]. The microscopic re-evaluation of all samples collected at first surgery (1999) proved consistent with ELST []. A post-operative audiogram in August 2009 showed further hearing deterioration (Garden-Robertson II) [].
MRI examinations from April 2009 to August 2011 demonstrated no evidence of focal recurrence. Follow-up MRI scans from 2012 to 2013 are currently not available for review. Follow-up MRI in October 2014 showed a local, aggressive recurrence within the lateral limits of the surgical region []; clinical evaluation demonstrated further (partial) hearing loss on the left side without any further cranial nerve dysfunction; unfortunately, the corresponding audiogram is currently not available. A fourth microsurgical intervention was assessed not indicated at this point. Gamma Knife radiosurgery (GKRS) was assessed as the best surgical option. The patient underwent single session GKRS-treatment (Perfexion™ Gamma Knife model) in February 2015 (72 months after the third and last microsurgery). Fixation was achieved using the Leksell® Coordinate Frame G (Elekta AB, Stockholm). Leksell GammaPlan® (LGP)-gross tumor volume (GTV) assessment proved a substantial increase in tumor volume of approximately 15% between the pre-GKRS MRI (4.43 cc - October 2014) and the stereotactic-MRI (s-MRI) at treatment (5.15cc - February 2015). The tumor was best delineated by the fat-saturated T1 contrast-enhanced s-MRI sequence; the peripheral dose was set at 18Gy on the 50% isodose line. GTV margins were equal to clinical target volume (CTV) []; planning target volume (PTV) margins were not required. The overall procedure was well tolerated by the patient.


A 10-month-old male infant was referred to the paediatric emergency room for a suspected FB in the oesophagus. The patient was in good general health, eupneic, presenting a peripheral capillary oxygen saturation (SpO2) of 99% on room air and normal pulmonary auscultation. The mother reported a history of a choking episode, followed by paroxysm of coughing and gagging while the patient was playing, with resolution within a few minutes. She also reported a lowering of the child’s voice tone after the episode; the crying was characterized by an unusual rattling, described as biphonic by the examiner. In the emergency room, the patient underwent neck, chest, and abdominal radiographs that did not detect any FBs in the digestive tract or in the tracheobronchial tree. On the suspicion of an FB in the airway, an ultra-low-dose MDCT was performed. Thin slices of 1.00 mm thickness were reconstructed that showed the presence of a fine linear image of 8 mm with amorphous density in the glottic region, vertically arranged between the vocal cords (Fig. A, B).
The patient was immediately submitted to direct laryngoscopy in emergency; the removed FB was found to be a piece of conventional snack wrapping (Fig. C).


A 10-month-old infant was admitted to the paediatric emergency department with chronic tracheobronchitis resistant to therapy for further investigation.
The patient presented with symptoms of respiratory system inflammation, cough, and hoarseness; at clinical examination, he was in good general conditions and eupneic. Chest and abdomen radiographs were negative. In consideration of the persistent symptomatology and lack of response to therapy, a low-dose MDCT scan with thin slices of 1.00 mm reconstruction, followed by virtual bronchoscopy (VB), was performed (Fig. A–C).
This technique allowed the detection of a fine linear image with amorphous density in the glottic region, vertically oriented between the vocal folds.
The patient underwent direct laryngoscopy in emergency that showed a piece of a toothbrush cover casing wedged in the glottis, which was promptly removed (Fig. D).


A 65-year-old woman was admitted to our hospital because of cough, sputum, and fever. No particular personal and family medical history was reported, and she never smoked. Physical examination showed moist rales on both sides, and no other abnormal vital signs.
Blood examination showed white blood cell (WBC) 7.95 × 109/L, procalcitonin (PCT) 0.073 ng/mL, C-reactive protein (CRP) 31.2 mg/L, IgG 23.3 g/L, IgG 942 IU/mL, and normal levels of IgA, IgM, G test, and GM test.
Chest computed tomography (CT) revealed multifocal areas of consolidations, nodules, and ground glass opacities in both lungs, with cervical and axillary lymph node swelling and a little pleural effusion (Fig. ). After 7 days treatment of moxifloxacin, she still had fever. Bronchial bronchoscopy and percutaneous lung biopsy were performed. All the bronchia were unobstructed, and a lot of purulent secretions and punctate hemorrhages were found. No neoplasm or caseous lesions were seen (Fig. ).The pathological findings from the biopsy showed infiltration of lymphocytes and plasma cells (Fig. ). Persistent fever did not improve. Chest CT was performed again, and indicated progression of the lesions (Fig. ).
The patient and her family went to Peking Union Medical College Hospital with her percutaneous lung biopsy specimens. Immunohistochemistry showed more than 40% of the total plasma cells were IgG4-positive, and >50 IgG4-positive plasma cells/high-power field (HPF). Serum levels of IgG4 were elevated to 10,200 mg/L.
We diagnosed the case as IgG4-RLD. The patient was started on oral prednisolone 30 mg/d for 28 days, and then was gradually tapered. After 1 week of treatment, the temperature was stable, and CT showed significant improvement in the areas of consolidations and nodules (Fig. ). As of the time of writing, the patient has remained well without disease progression.
Informed consent was signed by the patient.


A 26-year-old female patient presented for routine ophthalmic evaluation. Visual acuity was 20/20 in each eye. Fundus examination of the right eye revealed a fairly well-defined ellipsoid area of variable pigmentation just above the horizontal raphe and temporal to the fovea []; left eye fundus was within normal limits. Fundus autofluorescence better defined the torpedo-like lesion. However, the autofluorescence pattern was variegated []. SSOCT showed a dome-shaped cavity temporal to the fovea due to an anterior elevation of the ellipsoid zone from an intact retinal pigment epithelium-Bruch's membrane complex; the external limiting membrane and layers anterior to it appeared intact []. OCTA did not reveal any abnormality within the superficial or deeper retinal plexus. On segmenting below the RPE, changes were visible in the choriocapillaris layer. A convoluted pattern of fine vessels with some empty spaces between them was visible beneath the area of the separation of neurosensory retina on SSOCT []. This was more marked on the nasal and middle portion (red outlined arrow) of the lesion and appeared to slowly merge with the normal pattern on the choriocapillaris on the temporal half (blue outlined arrow). The signal strength by an OCTA depends on erythrocyte movement (flow rate and flow volume) indicating the vessels could be of a larger caliber than the normal choriocapillaris. On segmenting further below the level of choriocapillaris, the larger choroidal vessels with slower flow were seen as dark channels (red arrows). Some of these were masked by a projection artifact of the overlying anomalous vessels (area within yellow arrows) [].


A 55-year old female presented with altered mental status, lethargy, incontinence, and an unsteady gait. Past medical history was significant for Hashimoto's thyroiditis, lung carcinoma treated with chemotherapy and radiation therapy followed by lobectomy, and rheumatoid arthritis. Vital signs revealed a blood pressure of 116/76 mmHg, heart rate 45 bpm, respiratory rate of 10/min, temperature of 35.3°C, and SaO2 98%. She was alert and oriented to person but slow to respond and would not follow commands. Neurologic examination demonstrated a right facial droop, aphasia, right-sided neglect, and a right-sided pronator drift. Laboratory findings were significant for an elevated TSH (151 mcunit/mL) and a decreased free T4 (0.6 ng/dL). Brain computed tomography (CT) revealed a 4.0 × 3.1 × 3.3 cm AP/TR/CC left frontal temporal mass, with significant surrounding vasogenic edema, associated with mass effect/near-complete effacement of the left lateral ventricle and rightward midline shift of approximately 1 cm []. Magnetic resonance imaging (MRI) of the brain revealed a meningioma in the left frontotemporal convexity with surrounding edema, a 1.3 cm midline shift to the right, and a compression of the lateral ventricle with transependymal resorption of cerebrospinal fluid (CSF). A left uncal herniation was noted.
Shortly thereafter, the patient became obtunded with worsening bradycardia (HR 40), bradypnea, and became comatose. A multidisciplinary team involving anesthesia, intensive care, endocrinology, and neurosurgery was called to evaluate the patient. The decision was made for aggressive treatment of the severe hypothyroidism with administration of intravenous T3 and T4 while using osmodiuretics and steroids as temporizing agents. Shortly thereafter, the patient returned to her baseline mental status. Neurosurgery was on standby with close neurological observation and preparedness for surgical intervention should deterioration have occurred while conservative medical management was continued. After medical optimization and resolution of altered mental status, the patient underwent frontotemporal craniotomy and tumor resection. Endotracheal intubation was uneventful, and general anesthesia was maintained with a balanced concentration of intravenous remifentanil and propofol with sevoflourane titrated to <0.5 MAC. The tumor was isolated in the Sylvian fissure, and given the location along the dominant frontal lobe and temporal lobe, decision was made to perform a subcapsular resection. Using a microsurgical technique, the tumor was aggressively resected and the integrity of all the pial surfaces and the arachnoid/capsule was maintained. The surgical procedure and general anesthesia were well tolerated and no complications were noted. The patient was awakened uneventfully after extubation in the surgical intensive care unit 4 hours postoperatively.


A 15-year-old male patient reported to the Department of Periodontics, JCD Dental College, Sirsa, Haryana, complaining of intense pain, burning sensation and difficulty in chewing, swelling of gums, and bleeding from gums since 4 months. Dental and medical history of patient’s family was nonsignificant. His medical history was unremarkable except for a vague abdominal pain, for which a physician’s expert advice was sought and abdominal ultrasonography was noncontributory.


A 41-year-old man was admitted to our institution with acute exacerbation of schizophrenia, which was accompanied by disorganized behavior and psychomotor excitation. Shortly after admission, he developed catatonic stupor: his extremities were severely rigid, and he was verbally unresponsive. He had a history of two catatonic episodes that had occurred 1 and 3 years prior to admission, respectively. On both occasions, diazepam (40–60 mg/d) was ineffective, although he had attained remission after four sessions of BL-BP ECT using a Thymatron System IV (Somatics LLC, Lake Bluff, IL, USA). Therefore, we chose to start an ECT course shortly after completing a pre-ECT workup. Informed consent for treatment was obtained from his mother. He had taken quetiapine (600 mg/d) for 3 years, and the medication was continued orally or through a tube during admission. ECT was administered twice weekly using the same ECT device, with bitemporal electrode placement.
In the first session, anesthesia was induced using 125 mg of thiamylal (2 mg/kg), just as in the previous successful course of ECT, although we were unable to induce a seizure with stimulus settings of 55% (277.2 mC) and 90% (453.6 mC) using bitemporal electrode placement and the preset Low 0.5 program (0.5 ms pulse width). In the following session, we reduced the dose of thiamylal to 50 mg (0.8 mg/kg) and added 100 μg (1.6 μg/kg) of remifentanil. However, stimulus levels of 90%–100% (504 mC) were unable to elicit therapeutic generalized seizures. Although such stimulus levels produced mid-to-high-amplitude slow waves and apparent postictal suppression in the ictal electroencephalogram (EEG), neither motor seizures nor cardiovascular responses were observed (). Following the first session, the patient developed aspiration pneumonia because of persistent catatonic dysphagia. The patient was switched from quetiapine to olanzapine (gradually increased from 5 to 20 mg) after the fifth session and received intravenous diazepam (60–80 mg/d) for 4 days after the seventh session, both of which failed to alleviate his catatonic symptoms. In the eighth session, flumazenil (0.5 mg) was administered at the induction of anesthesia to antagonize the anticonvulsant effect of diazepam. However, the seizure responses remained inadequate, and he exhibited no clinical improvement.
In the eleventh session, we switched to RUL-UBP ECT using the Low 0.25 program (0.25 ms pulse width) and titrated the stimulus dose from 5% (in increments of 5%) to determine the ST. A stimulus level of 15% enabled the induction of seizure activity, which involved tonic-clonic motor responses and was associated with low-to-mid-amplitude slow waves on EEG (). In the subsequent sessions, the stimulus was increased to 100% (~6.7 times the ST), which produced adequate therapeutic seizures associated with high-amplitude slow waves and postictal suppression on the ictal EEGs (). The patient exhibited clinical improvement: after the 14th session, he regained the ability to speak (albeit haltingly), which revealed the existence of hallucinations and delusions, and after the 15th session, he recovered the ability to eat. He eventually achieved complete remission, and the ECT course was terminated at the twenty-first session. He experienced muscle weakness in the lower extremities and went to a rehabilitation clinic following discharge. He remained in remission with 10 mg of olanzapine. Written informed consent was obtained from the patient for publication of this case report and any accompanying images.