A 49-year-old woman with a past medical history of depressive disorder and bilateral breast reduction mammoplasty was admitted to anal sphincteroplasty for the treatment of fecal incontinence as a labor complication. During the post-operative period she presented a subacute onset of opsoclonus, mioclonus, ataxia, sleep disturbance and irritability. Neurologic examination revealed spontaneous, involuntary, arrhythmic and conjugate rapid eye movements; facial, axial and appendicular myoclonus and gait ataxia. Her motor strength and deep tendon reflexes were normal. Sensation was intact in all modalities.
For etiological clarification, a full workup was conducted with blood tests, including tumor markers and serology for human immunodeficiency virus, cytomegalovirus and Epstein Barr virus; brain magnetic resonance imaging (MRI), CT scan of the chest, abdomen and pelvis, thyroid and breast ultrasound and all revealed normal. Her cerebrospinal fluid (CSF) showed normal cell counts and protein values, and negative cytology and viral markers. For further investigation of the clinical hypothesis of idiopathic opsoclonus–myoclonus syndrome, the patient was transferred to Neurology Department. Electroencephalogram was negative for epileptiform activity. The CSF was tested for presence of onconeural antibodies and anti-Ri was positive. A positron emission tomography was performed and revealed a small solid nodular lesion in the left breast and homolateral axillary adenopathy. Breast screen with mammography and ultrasound, followed by MRI showed a nodular opacity under the left nipple and axillary adenopathy. MRI guided core biopsy of the breast lesion revealed an invasive ductal carcinoma (IDC) luminal A type. The left axillary node biopsy confirmed lymph node metastasis. Meanwhile, patient received immunotherapy with a combination of corticosteroids and rituximab. Symptomatic medication as clonazepam, levetiracetam, thiamine was prescribed. An intensive rehabilitation program was initiated in Neurology Department and comprised both physical and occupational therapy.
The case was then referred to Oncological Multidisciplinary Group. Patient underwent a breast-conserving surgery, lumpectomy with left axillary node dissection and Grisotti mammoplasty; vertical symmetrization mastopexy was performed in the contralateral breast. The surgical pathology revealed IDC pT1cN1aM0—Stage IIA. This was followed by chemotherapy, radiotherapy and hormone therapy with tamoxifen. The neurological deficit progressed after the first cycle of chemotherapy and improved with intravenous immunoglobulin administration. At the 6 months follow-up there was a partial improvement, anti-Ri antibody was subsequently reported as negative and there was no evidence of disease recurrence.


An 8-month-old boy, human immunodeficiency virus (HIV) exposed but HIV deoxyribonucleic acid polymerase chain reaction negative at 6 weeks, presented to Princess Mar ina Hospital (the main public referral hospital in Gaborone, Botswana) with a 3 weeks’ history of vomiting after feeds. Vomiting was projectile, nonbilious, and nonbloody. It was not associated with diarrhea, but there was a 1-month history of intermittent episodes of passing small amounts of very hard stools every three or more days. There was no history of fever, shortness of breath, or cough.
He was born at term by uncomplicated spontaneous vaginal delivery with a birth weight of 3355 g and Apgar scores 9, 10, and 10 at 1, 5, and 10 min, respectively. He was not dysmorphic. His mother's Venereal Disease Research Laboratory result was negative at antenatal booking, and he had never received antibiotics.
His mother was HIV infected, and hence, he received single-dose nevirapine after birth followed by zidovudine for 4 weeks. Postnatally, he was a well baby and was discharged from hospital on day 2 of life. He was exclusively formula fed. His child vaccine card showed suboptimal growth between −2 and −3 Z-scores on the World Health Organization weight-for-age growth chart. At 7 months, he had a bout of acute gastroenteritis followed by weight loss, and he then plotted below −3 Z-scores for weight-for-age. This weight decline coincided with developmental regression in language and gross motor milestones. At 6 months, he was reported to have been attempting single words and sitting without support, but from 7-month onward, he could no longer sit or speak any words. His immunizations were up-to-date.
On physical examination, he was emaciated and irritable. He had no pallor, jaundice, or lymphadenopathy. He had no dysmorphic features or any signs of respiratory distress. His vital signs were axillary temperature 36.5° C, respiratory rate 40/min, and pulse rate 148/min. His anthropometric measurements revealed weight 3.7 kg, length 59 cm, head circumference 39 cm, weight for age −3 Z-score, weight for length −3 Z-score, and length for age −3 Z-score.
He had a soft, grossly distended abdomen. There were no distended veins, scars, or hepatosplenomegaly. Bowel sounds were present but reduced. There was no palpable mass in the epigastrium. Repeated abdominal examination after feeding revealed a mass in the left upper quadrant that appeared to move to the right in a peristaltic fashion. It measured about 4 cm in diameter approximately. Examination of other systems was unremarkable.
Arterial blood gas showed a metabolic alkalosis with PH 7.49, pCO2 37 mmHg, pO2 89 mmHg, standard bicarbonate 28 mmol/L, and base excess +4.6. Serum urea and electrolytes were essentially normal with sodium 135 mmol/L, potassium 4.5 mmol/L, chloride 94.4 mmol/L, creatinine 20 mmol/L, and urea 4.6 mmol/L. The full blood count was normal with white blood cells 6.9 × 103/mL, hemoglobin 11.3 g/dL, mean cell volume 80 fL, and platelets 218 × 103/mL.
Erect and supine abdominal X-rays showed a distended gastric bubble with dilated loops of bowel, and a granular pattern suggestive of fecal impaction in the colon. Abdominal ultrasound scan showed a gaseous abdomen with feces in the colon. No other abdominal masses were appreciated.
A barium swallow and meal showed the unrestricted passage of contrast in the esophagus but delayed and restricted passage through the pylorus into the duodenum, suggestive of a partial but significant gastric outlet obstruction [Figures and ]. We thus entertained the diagnosis of infantile hypertrophic pyloric stenosis (IHPS).
The child underwent a laparotomy. There was a soft band of tissue crossing from the porta hepatis to the transverse colon which was constricting the pylorus. The pylorus was so severely stenosed that a size 8 feeding tube could not pass through the pyloric sphincter. The band of tissue was so tight that the gastric antrum was partly incorporated into the inferior surface of the left lobe of the liver [Figures and ]. The pylorus and gastric antrum were freed after ligating the constricting band of tissue.
Postoperatively, his recovery was uneventful. He was later successfully followed up in our nutritional rehabilitation unit until the malnutrition resolved.


A 19-year-old girl was born without any congenital deformity. Her parents are healthy and non-consanguineous, and her only elder sister is normal. When she was 18-month-old, she suffered from repeated fever, and her left lower leg ulcer appeared after then. The wound was cured by Chinese medicine patches when she was 3. According to her previous medical history, we can deduce her diagnosis at that time was acute hematogenic osteomyelitis of left lower leg. As she was growing up, the shortening and deformity of her left lower leg became obvious. She could walk without crutches, but it was very difficult for her to walk since she had to crouch down and straight up to exchange the supporting leg of length discrepancy for every step. She was very eager to walk upright by her own leg rather than amputating.
During the physical examination, upper limbs and spine were unaffected, as well as the right lower extremity. The left side of hip was stable and normal in terms of range of motion. The knee was rigid and the range of movement was extension 0° and flexion 30°. There was a bowing with convexity laterally and the lower leg was externally rotated relative to the femoral condyle. Leg length discrepancy (LLD) was 22 cm, which was mostly from the left lower leg. The left foot was in a 45° equinovarus deformity but the foot shape was normal. The movement range of ankle was 90° extension and 15°plantar flexion (Fig. ; Video 1).
A radiograph (Fig. A and B) showed the pelvis tilted to the left severely. The left femoral condyles with mildly hypoplastic could not match the distal tibial plateau to form a normal articulated joint. Only 4.5 cm of the proximal tibia was present. The tibial shaft was absent, while the distal tibia was dysplastic and its length was about 7 cm. The fibula was hypertrophied and curved, with proximal migration of fibular head. There was a proximal subluxation of the fibula at the knee. In the ankle, the tibiotalar joint was also hypoplastic but the matching relationship of joint appeared to be nearly normal. However, the lateral malleolus was thickened and the distal tibiofibular joint was abnormal.
Since the circle external fixator was not accessible to our hospital in 2009, we used the Hoffmann II External Fixation System to correct the deformity at that time. Three-stage treatment was designed in our initial plan. In the first stage, we intended to straighten the bowing lower leg as much as possible. Subsequently, the shortened lower leg would be prolonged to the same length of the contralateral side in the second stage. The third stage was the fibula centralization to replace the missing tibial shaft.[
The first stage treatment was also conducted separately in sequential steps. During the first operation, we found that the disturbance of blood supply in deformity leg would occur if the contracted medial soft tissue was released too much. Therefore, we used the combined external fixator to distract the contractures of medial soft tissue gradually (Fig. ). However, the patient still suffered from severe pain caused by the disturbance of blood supply during the process of distraction. One month later, the dislocated fibular head was pulled down and fused with the proximal tibia by 2 screws; the fibular osteotomy was also performed to correct the hypertrophied and curved fibula (Fig. ).
Due to the blood supply disturbance in the first operation, we told the patient that her lower leg may not be corrected to the same length as the contralateral lower leg, and the alternative approach was lengthening the thigh to make both lower limbs equal length. The treatment time could be shortened with this approach since the femur lengthening could be performed before the bone healing of fibular osteotomy. The patient accepted our suggestion, and the femur was distracted 2-month postoperative of fibular osteotomy in the second stage (Fig. ). After 6 months of the femur lengthening, the fibula was prolonged and the equinovarus deformity of foot was corrected at the same time (Fig. A and B). However, the patient suffered from severe pain again, due to the recurrence of blood supply disturbance during the process of lengthening. In this case, the continued lengthening of lower leg had to be ceased when the deformity leg was lengthened to 11 cm. But the left lower limb was still 2 cm shorter than the contralateral limb (Fig. A and B). Considering that the fibula would be transformed to inward and downward and be centralized as the tibial shaft, the shorten deformity would be remedied.
The proper rehabilitation exercises, such as movement of the knee and ankle joint, were performed during the procedure of lengthening. When the regenerated bone began to be mineralized, the patient can walk in part weight-bearing with the external fixator. After maturation of the regeneration, the fixator and the internals were removed. Since then, the patient recovered well from these operations and was reviewed every 6 months for 5 years. We had advised that she should undergo the operation of fibula centralization to correct the force line of lower limb with Taylor ring external fixator[ at every follow-up visit. But the patient was satisfied with the outcomes of previous operations and refused our advice.
The data about surgical result and the follow-up outcome are as follow. The patient underwent 5 operations in the whole treatment time of 3 years and 2 months. The bone healing time of fibular osteotomy was 8 months. The lengthening procedure of femur had continued for 102 days, and the external fixator was applied for 663 days. The total lengthening of femur achieved during the reconstruction was 7.8 cm. The external fixator index of femur was 85 days/cm. It took 156 days to lengthen the fibula for 11 cm, and the external fixator was removed after 492 days, at which time the limb lengthening and bone consolidation were completed. The external fixator index of fibula was 44.7 days/cm. The left lower limb was still 2 cm shorter than the contralateral limb (Fig. A and B). Knee valgus angle was 7.22°, femoral angle was 93.36°, tibia angle was 80.36°, and the mechanical axis deviation (MAD) of left lower limb was 34 mm. (Figs. and A) The knee function was extension 0° and flexion 40°. The active ROM of ankle was normal (Fig. A–D; Video 2).


A 46-year-old male patient presented to the emergency department with acute, dull chest pain in the middle of the chest radiated to the back, accompanied by cold sweating, lasting for >12 hours. He also presented with acute profuse nasal bleeding from the right nasal cavity on admission. Previously, patient had three episodes of recurrent nasal bleeding a few hours before the onset of chest pain and was prescribed 1 g of TXA twice daily. Patient had several risk factors for coronary artery disease (CAD) including longstanding uncontrolled hypertension since 10 years ago treated with amlodipine 10 mg, and smoking cigarettes one pack/day, though this had been stopped for 5 years until now. Physical examinations were normal, except for hypotension (blood pressure 90/60 mmHg) and active bleeding at right anterior nasal septum. ECG showed ST-segment elevation at leads II, III, aVF, V5, V6, and V7–V9 () followed by sufficient increase in cardiac markers (CK-MB =30.93 ng/mL; high-sensitivity troponin I =2,113.1 ng/mL). Hemoglobin was slightly decreased to the level of 12.7 g/dL (hematocrit 39.2%), with normal platelet count (296×103/µL) and hypokalemia (2.9 mmol/L). Prothrombin time and activated partial thromboplastin time (aPTT) were 10.4 and 23 seconds, respectively. Complete blood count (CBC) examination is shown in .
A loading dose of normal saline solution 500 cc, intravenous morphine injection 2.5 mg, isoborbide dinitrate 5 mg, and simvastatin 20 mg were given as part of STEMI management. For anterior epistaxis, an anterior nasal tampon with gentamycin was placed to stop nasal bleeding along with prophylaxis oral amoxicillin 500 thrice/day. Invasive reperfusion could not be performed, as patients refused to do percutaneous coronary intervention (PCI) due to financial issues. Considering comorbid acute bleeding disorder, late-onset STEMI (>12 hours), and hypotension, thrombolytic therapy was not given. Anticoagulant and antiplatelet were postponed until active bleeding subsided. For the treatment of hypokalemia, 25 mEq potassium chloride in 500 mL normal saline solution was delivered in 24 hours as intravenous infusion along with oral potassium phosphate thrice/day. After 24 hours of potassium treatment, potassium measurement showed normal value of 3.8 mmol/L, so that intravenous potassium administration was stopped, though blood potassium levels continued to be monitored every 24 hours. Multiple potassium measurements during hospital stay showed potassium levels still within normal limit.
On the following day, the patient’s blood pressure increased to 160/100 mmHg, but no profuse active bleeding was observed. Instead, chest pain was subsided. Amlodipine 5 mg, bisoprolol 2.5 mg, ramipril 5 mg once daily, laxative lubricant, and a loading dose of unfractionated heparin (UFH) 60 IU/kilograms of body weight (kgBW) continued with maintenance dose 12 IU/kgBW/hour were then added to the regimens. Target aPTT was set at 54–90 seconds (1.5–2.5 times of upper normal value). Reevaluation of aPTT at 3, 6, 12, and 24 hours showed aPTT values 68.2, 64.6, 58.2, and 48.3, respectively, so that UFH dose was adjusted by increasing 100 IU/hour. After dose adjustment, aPTT continued to be monitored every 6 hours, which was 71.4, 65.3, 55.7, and 51, respectively, and then UFH was stopped after 48 hours of administration. There was no increase in nasal bleeding during the period of UFH administration. Clopidogrel 75 mg once daily was started on the second day of UFH administration and added by aspirin 80 mg once daily on the next day. Reevaluation of peripheral CBC test was performed after UFH has been stopped to ascertain possible heparin-induced thrombocytopenia (HIT) complication and found platelet level of 304×103/µL. However, hemoglobin level decreased to 9.9 g/dL (). Additionally, some bleeding points were still observed on the right nasal cavity examination. Hence, a new nasal tampon was inserted and removed just prior to discharge.
During hospitalization, patient was stable with complete symptoms and ST-segment elevation resolutions as noted on ECG (). Patient was discharged at day 7 and arranged for further follow-up at outpatient setting management.


An 85-year-old man was presented by his general practitioner complaining of the presence of an erythematous round lesion that was protruding from the surgical scar of his previous sternotomy. He mentioned that the lesion has been growing slowly over time and that lately was also associated with pain in the area. The patient has undergone aortic valve and aortic root replacement with the use of a prosthetic tube graft 7 years ago. The general practitioner referred him to the cardiothoracic unit of our hospital; physical examination confirmed the presence of a non-pulsatile, erythematous round lesion in the cranial third of the previous sternotomy scar with a surrounding bruise (Fig.
and .
Contrast-enhanced computed tomography (CECT) scan was performed and revealed a 7-cm pseudoaneurysm arising from a 5-mm fenestration of the distal suture line of the Dacron graft with significant amount of thrombus. The pseudoaneurysm was pushing the sutured sternum forward causing diastasis of the sternotomy and was extending anteriorly to the sternum in the subcutaneous tissue (Fig.
and .
The patient was discussed in the aortic multidisciplinary meeting and was considered as of high risk for surgical revision; an endovascular approach was suggested as an alternative option and was referred to interventional radiology.
The procedure was performed under local anesthesia. Written informed consent was obtained explaining the risks and benefits of the procedure, including the risk of potential pseudoaneurysm rupture during manipulation and potential embolic material migration in the cerebral circulation. A retrograde left femoral artery access was obtained and a 5F sheath was inserted. Through a pigtail catheter, angiographic evaluation of the ascending aorta in anteroposterior and oblique views was followed in order to delineate the neck of the pseudoaneurysm. A second arterial access through the left axillary artery followed by another 5F sheath was inserted. Using this access, the neck of the pseudoaneurysm was catheterized and a 0.035 in. standard hydrophilic guidewire was advanced into the pseudosac. The wire was then changed to a 0.035 in. Amplatz extra stiff guidewire (Cook Medical, Bloomington, IN, USA) and a 9F delivery sheath was introduced in the sac. An Amplatzer muscular ventricular septal defect occluder (St. Jude Medical, St. Paul, MN, USA) plug measuring 15 × 7 × 10 mm was advanced in the pseudoaneurysm neck and was successfully deployed. The final ascending aortogram confirmed the device position with the blockage of the to-and-fro flow into the pseudoaneurysm sac and with contrast stasis within the pseudoaneurysm cavity. The next day, the subcutaneous swelling was significantly reduced in size and a month later the skin wound was corrected surgically (Fig. ). CECT was further performed at 72 h after the procedure, at 1 and 6 months, and at 1 year after the procedure, and documented complete exclusion and reduction of the size of the pseudoaneurysm sac (Fig. ).


A 60-year-old Caucasian male noted a red lesion on the right lower eyelid which was growing over 3 months. He revealed previously treated cancers of the colon, ureter, and skin, all under control without recurrence. He also noted previous eyelid surgery 2 years ago revealing a well-differentiated sebaceous carcinoma. Family history disclosed multiple members with skin and colon carcinomas. On examination of the patient, visual acuity was 20/20 OD and 20/30 OS. There was an elevated, lightly colored cutaneous lesion on the right lower eyelid margin, temporal to the site of the previous biopsy and measuring 6 × 4 × 3 mm.


A 66-year-old female presented in June 2014 with hypoplasia and onychodystrophy leading to trachyonychia of all fingernails. She also presented with diffuse erythema of the proximal and lateral folds and extreme pain. Her medical history included rheumatoid arthritis, osteoarthritis, osteoporosis, and anorexia nervosa. Her medications included misoprostol, naproxen, and multivitamins. She had a family history of unspecified arthritis. The patient was seen once prior in December 2009 regarding fragile fingernails and fingertip fissures and was prescribed hydrocortisone cream and fluocinonide ointment. She had also used petrolatum and moisturizing cream. Between June 2014 and October 2018, the patient had many rheumatology and dermatology consultations regarding her fingernail dystrophy (). Her final diagnosis was ACH but various differential diagnoses over her treatment course included various infections, chronic dermatitis involving the fingertips and nails, lichen planus, Raynaud’s disease, and Bazex syndrome which highlights the difficulty in confirming a diagnosis of ACH. A negative workup for aerodigestive malignancy indicated that it was unlikely Bazex disease. She had negative testing on two occasions for connective tissue disease. She also presented to many clinic visits with secondary infection with bacterial and candidal colonization which complicated her course.
Initial treatments between 2014 and 2017 included topical agents (calcipotriol-betamethasone, tazarotene, clobetasol propionate, and mupirocin), as well as systemic agents acitretin, methotrexate, and phototherapy, all with little benefit. Acitretin seemed to worsen her condition. Her course was complicated by several small ulcerations on the fingers (not typical of distal ulcerations of Raynaud’s), which were treated unsuccessfully with fusidic acid 2% ointment. A biopsy demonstrated candidiasis. Fluconazole orally and clotrimazole topically were given with great response as the ulcerations healed and there was nail growth. A subsequent flare-up in winter 2016 led to violaceous swollen fingers and nails that were separating from the nail beds and ulcerations that had become resistant to further treatment with fluconazole and clotrimazole. Subsequent cultures grew Klebsiella oxytoca and Staphylococcus aureus and were treated with sulfamethoxazole/trimethoprim. Of note, she never presented with the pustules or lakes of pus typical of this condition.
The patient began alitretinoin, in addition to mupirocin ointment, in April 2017. She alternated between doses of 10 and 30 mg daily due to side effects of dry eyes and conjunctival inflammation, which were treated with ocular lubricant and suitable eye hygiene. Her fissures and ulcers responded well to alitretinoin; however, her nails remained thin or nonexistent. In addition, the condition seemed to worsen again with winter 2017. A bacterial swab of a finger ulcer revealed Pseudomonas aeruginosa, which was treated with ciprofloxacin (alitretinoin on hold). Dilute acetic acid washes were recommended but not tolerated. Acetaminophen was used to manage pain. During this care period, the patient was hospitalized for cellulitis in the left arm and had open wounds on fingers and nail beds, which was treated with cephalexin.
From March 2018 to July 2018, the patient had violaceous swollen fingertips bilaterally, atrophic thin nails which were only partially re-growing, erythema, and eroded macerated skin on left and right hand distal digits. A bacterial swab revealed group A streptococcal infection which was treated. Alitretinoin 10/30 mg was continued, and cephalexin, prednisone, penicillin, acetaminophen, mupirocin, and clindamycin were used during this period. Adalimumab 80 mg was initiated as a loading dose to treat an acute flare, and a subsequent 40 mg dose every 2 weeks had a great effect with near normalization within 2–3 months and no side effects. Initial hypergranulation tissue response was treated with clobetasol propionate.
The patient was diagnosed with ACH, a variant of pustular psoriasis with an atypical course and repeated secondary bacterial and candidal infections. Currently, at 70 years of age, her treatment plan comprised adalimumab 40 mg biweekly, alitretinoin 10 mg daily, ocular lubricant, and clobetasol propionate, with great response (see ).


A costal lesion was incidentally detected during a regular checkup of a 52-year-old woman. She had no history of trauma and no complaints. Her medical history and family history were unremarkable. Chest computed tomography demonstrated an expansile osteolytic tumor in the midportion of the left third rib, extending toward the thoracic cavity (Supplementary Figure 1(a)). 18F-Fluorodeoxyglucose positron emission tomography revealed a maximum standardized uptake value (SUVmax) of 2.3 (Supplementary Figure 1(b)). Based on these findings, the differential diagnoses were a primary malignant bone tumor and other malignancies. The patient underwent en bloc excision of the tumor along with the adjacent soft tissues by axillary thoracotomy. Grossly, the lesion displayed a spongiform cut surface with hemorrhage and measured 5.0 × 3.5 × 2.5 cm (Supplementary Figure 1(c)). Microscopically, the tumor comprised numerous irregular, dilated, and thin-walled vessels lined with a single layer of flattened endothelial cells without cytologic atypia, consistent with the findings of a cavernous hemangioma (). The bony cortex was eroded by this tumor. Both lateral margins were free from the tumor in permanent sections. The patient’s recovery was uneventful, and she demonstrated no evidence of local recurrence 1 year after surgery. This study was approved by the institutional review board (CBNUH IRB, Cheongju, No. 2018-08-015), and the patient provided informed consent.


A 50-year-old man presented to the Emergency Department with sudden onset of left upper quadrant pain, dizziness, and collapse for 2 hours on June 19, 2016. On arrival to the Emergency Department, the patient was clammy, pale, and agitated. There were no associated symptoms and there was no history of a previous similar episode. His medical history included hypertension for 2 years and spontaneous cerebral hemorrhage for 1 month. The patient had hemodynamic instability. He had hypovolemic shock, and his blood pressure was 80/50 mmHg, pulse rate was 144 beats/minute, peripheral oxygen saturation was 96%, and body temperature was 37.8°C. On a physical examination, his left upper abdomen was tender and slightly spastic, but without rebound tenderness. There was an absence of bowl sounds. Ultrasound of the abdomen showed massive free fluid. Diagnostic abdominal puncture showed active bleeding, which suggested hemoperitoneum. The patient was resuscitated and stabilized, and then shifted to the radiology suite. A computed tomography angiogram showed a large amount of free intraperitoneal fluid (), an SAA in the hilum (), and several tumor masses in the splenic parenchyma ().
The patient was appropriately resuscitated with blood and plasma transfusions, and underwent emergency exploratory laparotomy. A midline laparotomy incision was performed. Upon entering the peritoneal cavity, a lot of blood and clots were encountered. A large hematoma was observed in the lesser sac bulging through the gastro-colic omentum. The gastrocolic ligament and the transverse mesocolon were incised to approach a 4-cm SAA, which had a 5-mm rupture.
The amount of evacuated blood was 3 L. The splenic artery was ligated proximally, followed by splenectomy with distal pancreatectomy to excise the aneurysm. A pathological examination showed carcinoma cells in the spleen (). Immunohistochemical analysis showed CD34(+), factor VIII(+), CD31(−), CD68(+), VIM(++), and cytokeratin(−) (, ). The patient had a 2-day stay in the Intensive Care Unit. He had an uneventful postoperative course and was discharged home on the 18th postoperative day. The patient attended a check-up every 3 months. To date, no recurrence has been reported in the 16 months following surgery. The People’s Hospital of Zhuji ethics committee approved the study protocol. Written informed consent was obtained from the patient for publication of this manuscript and accompanying images.


A 74-year-old gentleman complained of a right neck swelling that had been increasing gradually in size for the past 2 years. He had no pain, dysphagia, dyspnea or dysphonia. He also did not complain of any shortness of breath, foul-smelling breath, fever, weight loss or loss of appetite. He had no symptoms of hyperthyroidism or hypothyroidism. Interestingly, he complained of cough whenever the neck mass was touched. The patient was a diabetic with hyperlipidemia and hypertension. He also had a cerebral insult in the previous year with right hemiparesis which had resolved. He had no history of tuberculosis or any contact with such patients. The patient was a chronic smoker.
The patient was alert and conscious and oriented to time and place. All cranial nerves were intact. The oral cavity and oropharyngeal examination was unremarkable. The neck examination revealed a fullness on the right Level III measuring 4 cm x 3 cm (). There were no skin changes and the fullness was not tender and soft on palpation. However, during the palpation of the mass, the patient developed bouts of coughing. Otoscopy and nasoendoscopy showed normal findings. On examination of the larynx and hypopharynx, all structures were normal, and the bilateral vocal cords were mobile.
A computed tomographic (CT) scan demonstrated a well-defined, solitary mass in the right carotid space, posterior to the carotid artery and internal jugular vein (). The mass was hypodense to the skeletal muscles and showed mild enhancement post-contrast. There was no calcification or necrosis within it. The mass measured 2.3 cm × 2.4 cm × 7.6 cm and extended from the level of C2/C3 to C6/C7. It compressed the right internal jugular vein, abutted the common carotid artery and stretched the adjacent sternocleidomastoid muscle. However, the vein was still patent and was not separated from the common carotid artery. These features were suggestive of a right vagal schwannoma.
Microscopically, the smears of high cellularity were composed of clusters of uniform spindle cells in a bloody background. The spindle cells had oval nuclei, inconspicuous nuclei and a moderate amount of cytoplasm. Blood vessels were also seen intermingled with these clusters. No mitosis or bizarre cells were seen. No lymphoid or squamous elements were noted. Sections from the cell block showed sheets of spindle cells arranged in a hypercellular pattern. Some cells exhibited palisading and formed vague Verrocay bodies. The fine needle aspirate of the mass showed the presence of spindle cells, indicating that it was most probably a schwannoma.
An attempt to perform fine needle aspiration for cytology (FNAC) failed due to coughing induced by palpation of the mass. In view of risk to the adjacent vascular structures, an ultrasound-guided FNAC was performed with difficulty due to the patient's paroxysmal cough during the procedure. The ultrasound reported a homogenous hyperechoic solid lesion measuring 2.3 cm × 2.6 cm in the right neck lateral to the right internal jugular vein and internal carotid artery. No calcification or vascular activity was noted within it.
Based on the patient's age and co-morbidities, we discussed the treatment plan with the patient and his family. In consideration of the patient's multiple co-morbidities; possible complications, such as hoarseness and injury to the adjacent structures; and the patient's preferences, we agreed to conservative watchful management, as the tumor was benign and slow-growing. As of now, the patient has been under our observation for the past year, tumor growth has been static and no compression symptoms or airway compromise have been exhibited.


A 53-year-old previously healthy man visited a local clinic due to a chronic heartburn sensation and cough. Based on abnormal findings on chest computed tomography (CT) and esophagogastroduodenoscopy, he was referred to Samsung Medical Center for further management.
Approximately 15 years ago, the patient had been hospitalized due to a traffic accident. The diagnosis was traumatic diaphragmatic and splenic rupture. He was successfully managed by bilateral chest tube drainage after surgical repair of the diaphragm and splenectomy via laparotomy. Two years previously, an incidental posterior mediastinal mass was detected by CT screening. However, he did not undergo any further work-up. At first, he received medical treatment, and took a proton pump inhibitor (such as dexlansoprazole), but it had no significant effect.
At our hospital, chest CT revealed a strongly-enhancing tubular soft-tissue lesion on the left side of the esophagus, measuring 5 cm longitudinally on the CT images (). There was no other lesion in the abdomen. The radiologic diagnosis included a strongly-enhancing lymphatic lesion, as observed in Castleman disease (lymphoid hamartoma), or a submucosal tumor of the esophagus, as observed in leiomyoma or gastrointestinal stromal tumor (GIST). The endoscopic findings demonstrated an ovoid-shaped elevated lesion, consistent with a submucosal tumor (). The lesion was located 35 cm from the incisor, without any mucosal involvement. Endoscopic ultrasonography also showed a mass originating from the esophageal muscle layer, with a maximum cross-sectional area of 29.1 mm×15.6 mm (). Accordingly, the gastroenterologist consulted our department for possible surgical management.
We considered that the mass had contributed to his symptoms for the following reasons: (1) the mass was located just above the esophagogastric (EG) junction and might have been related to functional impairment of the EG junction; and (2) CT and endoscopy did not demonstrate any lesions other than the mass. In addition, the mass had a considerable size (8–9 cm), so its mass effect may have affected the movement of the esophagus. Moreover, the location of the mass near the proximal stomach made it likely to cause symptoms and signs such as dysphagia, esophageal motility abnormalities, oropharyngeal dysfunction (including aspiration), and neuromuscular disorders, which are also associated with gastroesophageal reflux disease. Consequently, we planned elective mediastinal mass excision.
Surgery was performed through video-assisted thoracoscopic surgery on the right side with a 3-dimensional scope system. Prior to surgery, the mass was considered to be a submucosal tumor. The patient was put in a comfortable position, and a surgical approach from the right side was used, following the operator’s general preference. Submucosal tumors can be adequately resected if they are approached from the right, even if the tumor is located primarily on the left. Although it was not easy to approach the patient from the right side, it was considered that this approach was preferable in light of the possibility of a future Ivor Lewis. During surgery, the mass was observed to originate from the pleural surface, rather than the esophagus. The size of the mass was 3 cm×5 cm×2 cm, and it adhered to the distal thoracic esophageal wall (). After almost complete resection of the main mass, the specimen was sent to a pathologist for frozen section analysis. The result of the analysis revealed a lymphocyte and histiocyte-dominant tissue. Based on the results of the frozen biopsy, we decided not to perform extended dissection and finished the procedure. A chest tube drain was inserted into the right pleural space.
After surgery, the patient underwent extubation in the operating room, and was transferred to the recovery room. The final pathological examination revealed thoracic splenosis. The histopathological features of the tissue were consistent with those of normal splenic tissue, characterized by the presence of red pulp and follicular structures with germinal centers (). He recovered without any complications, was discharged on postoperative day 5, and his symptoms resolved after surgery.
Prior to publication, we informed the patient that his clinical data and perioperative imaging studies were included for case report, and the patient consent was obtained.


A 26-year-old man was admitted to our hospital on September 1, 2017. He had a history of repeated fatigue, poor appetite, yellowing skin, and yellowish urine for more than three years, which aggravated one month ago.
At the age of 26 (one month ago admitted to our hospital), after drinking beer, he developed significant aggravation of yellowing skin and yellowish urine, with fatigue, poor appetite, aversion to oil, diarrhea, progressive emaciation, and weight loss of about 20 kg. Extremely abnormal liver function was confirmed [alanine aminotransferase (ALT) 126.8 IU/L, aspartate aminotransferase (AST) 109.2 IU/L, gamma-glutamyl transpeptidase (GGT) 340.7 IU/L, total bilirubin (TBIL) 322.5 μmol/L, and direct bilirubin (DBIL) 173.1 μmol/L].
He started suffering from repeated skin lesions at the age of 3 years. The previous skin biopsy suggested photosensitive dermatitis. He was advised to avoid light at ordinary times, which relieved his skin condition. At the age of 18, he was infected with hepatitis A, which was subsequently cured. In the past three years, he often came in contact with pesticides and more frequently suffered from dermatitis. At the age of 23, he suffered from mild jaundice, with exposed skin thickening and chapping, while he was diagnosed with abnormal liver function for the first time.
His father had suffered from “jaundice hepatitis” at the age of 12, and his mother, partner, and children were all healthy.
Physical examination upon admission showed severe yellowing of the skin and sclera, rough and irregular skin on the arm, face and perioral area, and lichenification in some areas (Figure ). Abdominal palpation revealed swelling of the liver and spleen, and suspicious shifting dullness.
Abdominal magnetic resonance imaging (MRI) suggested splenomegaly, and a small volume of ascites, and the characteristic description was fullness and enlargement of the liver with diffuse fatty infiltration of liver parenchyma (Figure ).
The monitoring showed abnormal liver function, high proteinuria, and abnormal blood lipid metabolism. Initial diagnosis at admission was abnormal liver function of unknown etiology. We systematically checked for common diseases that caused liver damage. According to the history, alcohol consumption was the predisposing factor, but the alcohol consumption did not meet the diagnostic criteria for alcoholic liver disease. Due to frequent exposure to pesticides in recent years, “drug/chemical liver injury” could not be completely ruled out, but could not explain the existence of skin lesions. After admission, hepatitis B virus markers, anti-hepatitis A virus (HAV)-immunoglobulin M (IgM), anti-hepatitis E virus (HEV)-IgM, Torch check IgM (rubella, toxoplasma), cytomegalovirus IgM, Epstein-Barr virus (EBV), and pre-transfusion ICT [human immunodeficiency virus (HIV), hepatitis C virus (HCV), and TP] were tested to exclude virus-related hepatitis. The autoimmune antibody spectrum (ANA, AMA-M2, LKM, and ANCA) was tested to exclude autoimmune liver disease; while autoantibody profiles (ds-DNA, ANA, etc.) were checked to exclude liver injury caused by systemic diseases [such as systemic lupus erythematosus (SLE)] and connective tissue diseases. Thyroid stimulating hormone (TSH), T3, T4, FT3, and FT4 were checked to exclude liver injury associated with hyperthyroidism, while schistosomiasis antibodies were tested to exclude schistosomiasis-related cirrhosis. The normal blood amylase and imaging findings of the abdomen excluded chemical liver injury caused by pancreatitis, and myocardial enzymes and brain natriuretic peptide (BNP) were normal. Electrocardiogram, echocardiography, and myocardial enzymogram were used to exclude liver injury caused by cardiogenic liver congestion, and abdominal vascular ultrasound was performed to exclude vascular liver diseases, such as Bucca syndrome. Given the juvenile onset of the disease, further screening for inherited metabolic liver disease was performed. No nervous system signs were found, and electromyography (EMG)/neutral endopeptidase (NEP) and amplitude integrated electroencephalography (AEEG) were normal. Ceruloplasmin, serum copper, and 24-h urine copper were also normal. No corneal K-F ring was observed, thereby excluding hepatolenticular degeneration. Given the normal saturation of ferritin and transferrin and the absence of homogeneous and diffuse iron deposition in liver imaging, hemochromatosis was not considered. No developmental disorder, “baby face”, repeated or severe hypoglycemic attacks, muscle strength and muscle tone decline, evidence of glycogen accumulation, or history of chronic obstructive pulmonary disease (COPD) was found, and normal levels of α1 antitrypsin ruled out α1 antitrypsin deficiency. Based on the history of photosensitive dermatitis in childhood and liver damage in adolescence, the initial clinical diagnosis was porphyria. Further examination of uroporphyrin was negative; red fluorescence was seen around red blood cells in his peripheral blood smear under a fluorescence microscope (Figure ). Fluorescence intensity (Ex 400 nm, Em 595 nm) was measured with a Thermo VARIOSKAN LUX fluorescence spectrophotometer. The fluorescence intensities of urine, whole blood, and plasma were 2.14, 17.49, and 17.17, respectively, which were significantly higher than the normal fluorescence intensities of urine (1.231), whole blood (0.059), and plasma (0.912). Bone mineral density test suggested bone loss and increased fracture risk. Skin (Figure and D) and liver (Figure and F) biopsy results were consistent with porphyria, and liver histopathology (G3S3) suggested superposed drug-induced/chemical liver injury.


A 48-year-old white woman was admitted to the neurological department of the Careggi
Hospital (Florence, Italy) for a 15-month history of paresthesias of the lower
limbs, in the absence of motor deterioration. Her medical history was only
significant for gastric ulcer (chronically treated with proton-pump inhibitors
[PPIs]) and smoking (about 20 cigarettes). A spinal cord magnetic resonance imaging
(MRI) showed signal abnormalities in cervical (C2-C6) and thoracic (D6) regions;
none had contrast enhancement (). A brain MRI revealed a 44-mm meningioma situated on the floor
of left middle cranial fossa, without signs of raised intracranial pressure. A
lumbar puncture was performed, demonstrating 2.05 g of total proteins, normal cell
count, link index of 0.66, and oligoclonal immunoglobulin G (IgG) with a mirror
pattern.
The patient received a diagnosis of inflammatory myelitis and then a 5-day course of
intravenous methylprednisolone was prescribed. On Day 3 of PST, she complained of
chest tightness, which worsened over the next 2 days. On Day 5, she also developed
dyspnea, weakness, and malaise. Her blood pressure was 170/90 mm Hg and her pulse
rate (PR) was ranging from 37 to 40 bpm. Pulse corticosteroid therapy was
immediately suspended. An increase in body weight (5 kg) was detected compared with
Day 1. A 12-lead electrocardiogram (ECG) revealed sinus bradycardia (37 bpm),
without signs of acute myocardial infarction, acute coronary syndromes,
atrio-ventricular block, or other forms of arrhythmias (). A prior ECG, done a month earlier,
showed normal sinus rhythm at 76 bpm. Cardiac biomarkers were normal and blood tests
showed mild anemia, slight elevation of creatinine (0.95 mg/dL), potassium level of
3.9 mEq/L, sodium level of 141 mEq/L, calcium level of 8.4 mg/dL, and a mild
decrease of total protein 5.6 g/dL. A normal left ventricular function was proved
with echocardiography. A cranial computed tomography (CT) scan was performed, which
did not show any significant modifications compared with prior brain MRI images. The
patient did not receive any treatment, whereas high blood pressure and bradycardia
spontaneously resolved in few hours and 4 days, respectively.


A 16-year-old boy, 85 kg in weight, was admitted to our hospital (The First Hospital of Jilin University) for thyroglossal cystectomy under general anesthesia. Preoperative laboratory results, chest X-ray, and electrocardiogram (ECG) were within the normal ranges. The patient fasted for 12 hours prior to the operation.
In the operating room, noninvasive blood pressure, ECG, and oxyhemoglobin saturation (SpO2) were monitored by the IntelliVue MP50 monitor (Philips Medizin Systeme, Graseby Medical Limited, Watford, U.K.) and Narcotrend index by the Narcotrend Index Monitor (Technik, Bad Bramstedt, Germany). Rapid induction of anesthesia was performed with fentanyl (3 μg/kg), cisatracurium (0.2 mg/kg), and propofol (1.5 mg/kg). One minute after the induction, the Narcotrend index decreased from 99 to 27. After intubation, continuous infusion of 2% propofol (6 mg/kg/h and remifentanil 0.5 mg/h) was provided using an intravenous syringe pump (CP700TCI, SLGO Medical Technology Co China). Six minutes after the intubation, the Narcotrend index increased from 27 to 84 (Fig. ). At the same time, the surgical staff observed that the patient was in tears. These observations indicated that the patient was under light anesthesia and might be at risk of developing awareness. The patient was twice provided with a bolus of propofol (10 mg/bolus) through the syringe pump. The infusion rate of propofol was increased to 8 mg/kg/h because we believed that the drug clearance rate in this patient was high. However, the Narcotrend index remained in the 73–85 range. The Narcotrend index did not change even after the patient was administered with additional bolus of propofol. During the trouble-shooting procedure, we noted that the syringe fixing clamp of the infusion pump was not in the proper position. Further examination showed that the spring has been eroded by colloid and became viscid, and in consequence could not push up on the clamp. In effect, the syringe pump was found to be in working condition, but the issue with the syringe fixing clamp resulted in no infusion of propofol (demonstrated in Fig. A). This was confirmed by a lack of any change in volume of the propofol in the syringe in spite of multiple efforts at infusion. In light of this finding, the patient was manually administered with a bolus of 50 mg of propofol and 2 mg of midazolam using a syringe, and supplemented with inhalation of 2% sevoflurane by inhalation. We noted that the Narcotrend index had returned to 23 (a state of deep anesthesia) following the manual administration of anesthetic medications. The syringe pump was replaced, and the surgery was allowed to proceed and lasted for 105 minutes. During the surgery, the Narcotrend Index was maintained within the 22–64 range. The patient had an uneventful recovery, and showed no evidence of awareness immediately after the recovery period or in the 3-day follow-up.


A 30-year-old woman (79 kg, more than 38 weeks’ pregnant) presented at our institution with generalized pruritus and an elevated serum bile acid level. She was in good health, except for symptoms of an upper respiratory infection. She took a 5-day course of azithromycin (500 mg loading dose and then 250 mg daily). On the fourth day of azithromycin treatment, she developed severe pruritus. Blood test results revealed an abnormal total bile acid level (more than 10-fold of the normal level), while the alanine aminotransferase (ALT), aspartate aminotransferase (AST), and gamma-glutamyl transferase levels were normal. She denied taking other medications. She also denied alcohol use, herbal medication use, substance abuse, and other chemical exposure. She had no family history of liver disease. The ultrasonogram of the liver showed a good liver structure with no other positive symptoms. The laboratory tests are shown in Table . Before azithromycin administration, she showed no symptoms, and the liver enzyme and total bile acid levels were normal. After 4 days of treatment with azithromycin, she developed severe pruritus, and the serum total bile acid level increased significantly with normal hepatic aminotransferase levels. On the second day after cesarean section, hepatic aminotransferase levels increased slightly and reached its peak level on day 4 after cesarean section. The total bile acid level became normal after fetal delivery. The liver enzyme levels returned to normal within 4 weeks without any symptoms.
Management: (1) A diagnosis of ICP was made, and the patient was instructed to avoid using the antibiotic. (2) Cesarean section was performed immediately to lower the risk of fetal demise. The 1-minute, 5-minute, and 10-minute Apgar scores of the newborn baby were all 10. (3) The mother was re-evaluated when the liver enzyme level became elevated after cesarean section, and azithromycin-induced intrahepatic cholestasis was considered. Silymarin capsules and bifendate were prescribed to lower the liver enzyme levels and protect the liver from further injury.
Two years later, the same patient delivered her second baby without ICP recurrence or any hepatic injury (Table ).


A 67-year-old woman presented to our hospital with a 2-day history of pain and a feeling of coldness in her right hand. In her past medical history she had not received any treatment for dyslipidemia. There is no other medical history or smoking history. Upon physical examination she had no heart murmur or leg edema; her blood pressure was 154/96 mm Hg (left brachial), and oxygen saturation of peripheral artery was 96% at room air. Her right hand was pale and the right radial artery was pulseless. An electrocardiogram (ECG) showed sinus rhythm at 79 bpm. The serum D-dimer level was slightly increased (2.18 µg/mL). Computed tomography (CT) scan confirmed thromboembolism in the distal part of the right brachial artery, left pulmonary artery and right kidney infraction (). Cerebral infraction and stenosis of the main cerebral artery were not detected by the head magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA).
Doppler sonography of right upper limb revealed the disappearance of blood flow at the distal part of the brachial artery, and collateral circulation to the radial artery from the brachial artery. In this case, multiple thromboemboli occurred in both the systemic and pulmonary circulation. Thus, we suspected paradoxical thromboembolism. Transesophageal echocardiogram and venous ultrasonography findings showed the presence of a PFO and DVT (). Thus, we diagnosed paradoxical embolism due to PFO. Photoplethysmogram (PTG) of her right hand was very slow on admission (). The condition of her right upper limb had been relieved from 2 days previously due to the development of collateral circulation. Therefore, we thought that neither emergency operation nor catheter intervention for thrombectomy was necessary in this patient. We started treatment with urokinase (UK) 240,000 U/day intravenous injection (IV) and unfractionated heparin (UFH) continuous IV (target activated partial thromboplastin time (APTT) 60 - 80 s). As her condition and the serum D-dimer level were improving we started catheterization at day 7 after admission (); however the thrombus still remained in the brachial artery. Although her pain was relieved, her right hand was still cold and PTG of the right hand at day 7 was slow. For her outpatient care, based on the viewpoint of providing rapid anticoagulant therapy within the therapeutic range, having longest periods of initial intensive therapy we chose treatment using ribaroxaban (15 mg, two tablets twice daily) (). Three weeks after discharge from the hospital, her right hand had become warm and the serum D-dimer level and PTG were normalized (). From that time, we changed ribaroxaban to the maintenance dose (15mg, one tablet once daily). Three months after discharge, Doppler sonography and MRA of her right arm confirmed the achievement of reperfusion of her brachial artery ().


The chief complaints of a 16-year-old female with EDS were double upper limb weakness for 7 years and double lower limb walking instability for 2 years.
The patient developed limb weakness without obvious inducement for 7 years, and did not receive enough attention. She did not receive regular treatment else. And she gradually became unable to walk independently before one month.
She denied any history of trauma or surgery and did not have hypertension, diabetes, or cardiovascular and cerebrovascular diseases.
Her father and brother both had a blue tint to the sclera, joint hypermobility, abnormal scarring, and planovalgus feet.
This patient was noted to have a blue tint to the sclera (Figure ), joint hypermobility (Figure ), abnormal scarring (Figure ), and planovalgus feet (Figure ). The power of main muscles in four extremities was approximately level III-IV.
Routine blood tests, routine urine tests and urinary sediment examination, routine fecal tests and occult blood test, blood biochemistry, immune indexes, and infection indexes were all normal.
Imaging results (Figure ) demonstrated that kyphosis at the C3 level was 30° and the cervical spinal cord was compressed from C2 to C4.


A 35-year-old man was referred to the Department of Conservative Dentistry and Endodontics, Darshan Dental College and Hospital (DDCH), Udaipur, Rajasthan, India, with an endodontic mishap in the form of instrument separation. Radiographic examination showed that instrument fragment was separated in mesiobuccal canal of the mandibular molar []. A straight line access was prepared by Kerr Corporation Orange (California, USA) using anticurvature technique. Placing a Hedstrom file (H-file) [] down into the canal alongside, engaged with the separated file and activated by ultrasonic vibrations followed by simultaneous removal of both the H-file and separated instrument [] after proper irrigation and canal preparation, obturation was carried out [].


A 37-year-old man was referred to the Department of Conservative Dentistry and Endodontics with acute pain in the left upper back region for the past 3 days. Radiographic examination showed radiolucency involving pulp and according to treatment planning, root canal opening was performed. During canal preparation, file was separated at the apical region in mesiobuccal canal []. The file was then bypassed with help of ethylenediaminetetraacetic acid and K-file. Then H-file [] was inserted alongside the separated instrument and ultrasonic vibrations were applied along the file and separated file was shifted in the middle of the canal [], followed by retrieval of separated file [] by braiding technique. After that, root canal treatment was completed by following the recommended protocol [].


A 28-year-old man was referred to the Department of Conservative Dentistry and Endodontics with pain in the right lower back region for the past 4 weeks. Radiographic examination revealed inadequate obturation with missed canal in 46 [], and there were no periapical changes. After removal of obturating material, radiographic examination revealed that the instrument had separated at the junction of coronal and middle third of the canal []. Bypass the fragment and retrieval was done by braiding technique. In this technique, three Hedstrom no. 10 files were inserted down along the separated file in mesial, distal, and labial side and then twisted around one another to entangle the file and withdrawn in one stroke []. Confirmatory radiograph was taken to check the complete removal of file. After 7 days, the patient was asymptomatic and the canal was obturated with laterally condensed gutta-percha using Acroseal sealer (Septodont), and the root canal opening was sealed using composite material (Synergy D6; Coltene, Headquarters: Altstätten, Switzerland) [].


A 55-year-old man reported for endodontic retreatment of 24 in the Department of Conservative Dentistry and Endodontics, DDCH Udaipur. Radiographic examination showed a separated instrument in middle third of the mesiobuccal canalfigure [Figures and ]. It was decided to use the braiding technique for removal of separated instrument. Three H-files were inserted along the separated instrument as apically as possible from mesial, distal, and labial side, then twisted around one another to engage the file, and withdrawn simultaneously []. Subsequent to the removal of separated instrument, the canals were prepared and disinfected as per protocol. Patient was recalled after 7 days and the canals were obturated with lateral compaction method [].


A 60-year-old, non-diabetic, non-hypertensive female presented to us a day after undergoing pneumatic air descemetopexy for postoperative planar inferior Descemet membrane (DM) detachment [] in the left eye (LE). DM reposition was performed using air under peribulbar anesthesia. The intraocular pressure (IOP) measured in the LE at 12 h was 18 mmHg, with a blood pressure of 110/70 measured preoperatively.
About 24 h post-descemetopexy, the vision dropped to light perception (PL). Anterior segment was unremarkable with residual air bubble. Fundus examination showed a tongue-shaped area of patchy retinal whitening involving peripapillary and macular territory []. The fundus fluorescein angiography (FFA) showed a normal arm-to-retina time with areas of segmental hyperfluorescence having fuzzy margins, corresponding to the areas of retinal whitening [].
The optical coherence tomography (OCT) revealed an increased reflectivity of the outer retinal layers [the retinal pigment epithelium (RPE) and the photoreceptor layer] along with a thickened outer plexiform layer []. A hyperreflective line separated the inner layers from the outer layers, suggestive of photoreceptor swelling and ischemic injury to outer-retinal layers. The inner-retinal layers were well segmented and unremarkable.
Based on the clinical picture, FFA, and OCT findings a diagnosis of LE posterior ciliary artery occlusion (PCAO) was made. FFA repeated at 1 week showed transmitted hyperfluorescence. OCT showed outer-retinal thinning with loss of photoreceptor layer, accompanied with RPE hyperreflectivity [].


A 14-year-old boy, known patient of juvenile idiopathic arthritis (JIA) since 2008, was under follow-up with us since the past 2.5 years for the management of uveitis. He was investigated for other possible causes of uveitis elsewhere initially which included a negative Mantoux test and normal chest X-ray. He was initially started on oral methotrexate 25 mg/week and subsequently switched over to subcutaneous injection of adalimumab biosimilar (Exemptia). He received 40 mg subcutaneous injection of adalimumab every 2 weeks for 6 months, and then every 3 weeks for 6 months while on low-dose oral methotrexate (10 mg/week). He responded well to the systemic treatment and was maintaining quiet eyes for the past 1 year. Recently, he was diagnosed to have pulmonary tuberculosis and started on antitubercular treatment by the chest physician. His Mantoux test was positive (25 mm), and high-resolution computed tomography of the chest was suggestive of pulmonary tuberculosis. His best-corrected visual acuity in the right eye was 6/5 and left eye was 6/9. Slit-lamp examination revealed occasional cells in the anterior chamber of the right eye, posterior synechiae with minimal posterior subcapsular cataract in the left eye, and cells in anterior vitreous of the right eye. Fundus examination of the right eye revealed a solitary, choroidal nodule less than 1-disc diameter in size slightly temporal to the fovea []. Fundus examination of the left eye was normal. Swept-source optical coherence tomography of the right eye revealed a large homogeneous, hyporeflective area in the choroid, and fundus fluorescein angiogram of the right eye revealed early hypofluorescence in early phase of the angiogram followed by hyperfluorescence [Fig. and ]. Indocyanine green angiography detected multiple small hypocyanescent spots in addition to the clinically evident choroidal nodule [Fig. and ]. The patient was advised oral corticosteroid (40 mg/day) on a tapering schedule and to continue ATT. He was reexamined after a month. Fundus examination of the right eye revealed resolution of choroidal nodule. He was advised to continue the same treatment []. Fundus examination after 3 months of follow-up revealed healed choroidal nodule [] and he was advised to stop oral steroid and continue ATT as per the advice of the chest physician.


A 47-year-old woman presented with a complaint of severe headache of sudden onset. On neurological examination, she had signs of meningeal irritation. Brain computed tomography (CT) revealed Fisher grade 3 SAH. Three-dimensional brain CT angiography showed an anterior communicating artery aneurysm. Emergent craniotomy and neck clipping of the aneurysm were performed.
In the early postoperative period, she complained of pain mainly on the right side of the sacrum, buttock, and upper part of the posterior aspect of the right thigh. She complained of worsening pain when sitting down, walking, or coughing. She had a previous history of lumbago 3 years ago and it was relieved with conservative treatment. CT of the lumbosacral spine performed at that time revealed a bulging disc at the L4-L5 level and a PC at the S2 level (Fig. A). Thereafter, she did not have lumbago and there was no history of recent trauma. The nature and location of her pain were slightly different from the nature and location of pain she had experienced 3 years ago. During this episode, sacral and buttock pain was more severe than lumbago. She complained of severe tingling sensation and right-side predominant pain. The pain did not subside fully when lying down. She suffered from the pain despite using analgesics for 4 weeks.
Magnetic resonance imaging (MRI) of the lumbosacral spine performed at 1 month postoperatively showed an 18×13×25 mm-sized PC with high signal intensity (SI) at the S2 level affecting the right S2 root and a so-called dark’ and bulging disc at multiple levels of the lumbosacral spine on the T2-weighted sagittal image (Fig. B). On the nonenhance T1-weighted sagittal image, mixed high and low SI with a fluid–fluid level within the cyst, suggestive of intracystic hemorrhage, was revealed (Fig. C). There is a perineural cyst that is located to the right aspect at S2 level (Fig. D). The size of the cyst measured on MRI showed no apparent enlargement of the cyst compared with the size on the CT performed 3 years ago.
It seemed that the size of the cyst had not increased as compared with the size of the cyst on the CT performed 3 years ago. An electrodiagnostic study was performed 6 weeks after the onset of the pain, and it showed subacute right S2 radiculopathy. There were abnormal findings such as delayed onset latency of the right bulbocavernosus response, spontaneous denervation activity, and fibrillations in the right side of the anal sphincter muscle, soleus muscle and foot intrinsic muscles (abductor digiti minimi, abductor halluces). The fibrillation potentials had increased amplitude of 300uV, which suggested that 6 weeks to 3 months had elapsed after denervation.[
With conservative treatment, her pain gradually diminished. Follow-up MRI performed 3 months later showed no change in the size of the PC. However, mixed SI and a fluid–fluid level within the PC disappeared on the enhanced T1-weighted sagittal image (Fig. E). As this is a case report, ethical approval was not required. Informed patient consent was obtained for the publication of this report.


An 80-year-old man, who had been treated in another hospital for the recurrent
accumulation of pleural fluid in the right thoracic cavity for 2 years, was referred to
our department because a cytological examination of the fluid suggested lymphoma cells. On
examination, breath sounds were decreased in the right hemithorax. There was no surface
lymphadenopathy or hepatosplenomegaly. Oxygen saturation was 96% in room air. A chest
radiograph showed prominent pleural effusion (). His hemoglobin level was 12.5 g/dL, white blood cell count was 6.95 ×
103/μL, and platelet count was 289 × 103/μL. Total serum protein
was 6.7 g/dL, albumin 3.2 g/dL, globulin 3.5 g/dL, lactate dehydrogenase 307 IU/L, higher
than the normal range of 124 to 222 IU/L, creatinine 0.6 mg/dL, and C-reactive protein
2.25 mg/dL. Serum protein electrophoresis revealed an M-component migrating in the γ
globulin area and immunofixation confirmed the IgM/λ M protein. No urinary Bence Jones
protein was detected. The level of IgG was 723 mg/dL, IgA was 83 mg/dL, and IgM was 1,526
mg/dL (normal range, 33 to 183 mg/dL). Serum free light-chain κ (FLC-κ) was 16.1 mg/L,
FLC-λ was 20.5 mg/L, and the κ/λ ratio was 0.79. Soluble interleukin 2 receptor was 3,051
U/mL.
Computed tomography (CT) of the chest revealed the accumulation of pleural fluid in the
right thoracic cavity in association with atelectasis of the middle and lower lobes, where
the marked accumulation of the tracer was demonstrated by
18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) combined with CT
(), suggesting that the atelectatic lesion
contained an infiltrative condition.
Thoracentesis yielded exudative fluid containing abundant neoplastic cells with the
morphological features of plasma cells at a concentration of 6.13 × 103 cells
per microliter (). These cells expressed
IgM/λ monoclonal immunoglobulins both on the cell surface and in the cytoplasm, and showed
a wide range of expression levels of CD20, CD22, CD23, CD38, and CD138. CD19 and CD45RA
were positive, and CD5, CD10, CD21, CD24, CD56, and surface IgD were negative (). The DNA index was 1.06 from normal
diploid cells. Bone marrow showed hypercellularity containing CD20-positive plasma cells,
and flow cytometry revealed a lymphoplasmacytic population with an identical
immunophenotype to that of pleural fluid cells. Tests for the MYD88 L265P
somatic mutation were negative (Supplementary Figure S1).
The G-banding of metaphase spreads obtained from a short-term culture of pleural fluid
cells demonstrated numerical and structural abnormalities, including a 14q+ chromosome and
uncharacterized small marker chromosome; the karyotype according to the ISCN 2016 was
48,XY,+3,add(9)(p13),+12,add(14)(q32),del(16)(q22),−18,+mar (). Fluorescence in situ hybridization (FISH) of
interphase nuclei with the MYC, BCL2, and
BCL6 dual-color, break-apart probes detected no rearrangement of these
3 genes, while nuclei carried 3 hybridization signals of BCL2 and
BCL6 (Supplementary Figure S2). The hybridization of metaphase cells
with the BCL2-IGH dual-color dual-fusion probe showed
that the add(14) chromosome was marked by the IGH and
BCL2 probes (); however,
these probes did not generate a fusion signal (Supplementary Figure S2). We then performed
hybridization with the MALT1 dual-color, break-apart probe and found that
add(14) was marked by the green-labeled telomeric probe, representing 3′
MALT1, while the red-labeled centromeric MALT1 signal
was missing, and, accordingly, interphase nuclei showed the one green and two yellow
signal pattern (). The uncharacterized
marker chromosome was marked by unrearranged BCL2 and
MALT1 probes, but not of IGH. These cytogenetic
studies indicated that the add(14) chromosome represented der(14)t(14;18)(q32;q21), at
which the MALT1-IGH fusion gene was localized, while the
reciprocal der(18)t(14;18)(q32;q21) was deleted.
Since the t(14;18)(q32;q21)/MALT1-IGH fusion gene was
suggested, DNA extracted from pleural fluid cells was subjected to a long-distance
polymerase chain reaction (LD-PCR) in order to amplify the junction encompassing the two
genes. PCR primers were designed for MALT1 exon 1 and for the Eμ enhancer
as well as the Cμ, Cγ, and Cα constant genes of IGH; the
MALT1 probe and IGH probes were oriented in the
opposite direction (, Supplementary
Table S1). As shown in , we obtained LD-PCR
products by the MALT1-Eμ and MALT1-Cγ primer
combination, indicating Cμ to Cγ class switching. Nucleotide sequencing of the
MALT1-Eμ product revealed that the junction occurred at the J6 segment
of IGH and the nucleotide position −5,399 of adenine of the ATG start
codon of MALT1 at the position +1, and a fragment of 9 base pairs (bp) in
length was inserted at the junction (). As a result of translocation, the MALT1 and
IGH Eμ-Cγ segments were aligned in the divergent transcriptional
orientation. Reverse-transcriptase PCR confirmed the expression of MALT1
in pleural fluid cells, even though the level was lower than those of in
vitro cultured lymphoma cell lines (Supplementary Figure S3).
We then applied LD-PCR using the MALT1-Eμ primer combination to another
case with t(14;18) reported previously by us. The sequences encompassing the
MALT1-IGH junction were composed of the J4 segment, a
46-bp insertion fragment, and the upstream sequences of MALT1 at the
position −1,738 of the start codon ().
The patient was treated with 6 cycles of the DRP (dexamethasone, rituximab, and
cyclophosphamide) regimen every 21 days.
This led to the resolution of pleural effusion, the disappearance of lymphoma cell
invasion in the bone marrow, and accumulation of the tracer within the lung lesion by
FDG-PET/CT. However, the serum IgM/λ M protein remained detectable by immunofixation and
the level of serum IgM remained higher than the normal range.


An 8-month old female child with motor delay presented with a history of left-sided weakness following a fall from the walker at the ground level. There were no other signs of traumatic brain injury such as loss of consciousness, vomiting, convulsions etc. She was first born to a non consanguineously married couple with normal birth history. However, she had attained neck control at 5 months of age and was still not sitting with support and cognitive as well as language milestones were normal. At admission, she was conscious, oriented to mother and surroundings, had normal vitals and anthropometry with no external injury. She had a paucity of movements of left upper and lower limbs with variable tone and brisk deep tendon reflexes on the same side. She had intermittent dystonic posturing of the left lower limb. There was no cranial nerve palsy. There were no neurocutaneous markers and fundus was normal. A workup of pediatric stroke was conducted. Her complete hemogram, bleeding time, PT, Aptt, INR, serum calcium, serum homocysteine, protein C, protein S, antithrombin III, HB electrophoresis, paratharmone, lipid profile, ABG, lactate, ammonia, plasma tandem mass spectroscopy and urine gas chromatography and mass spectroscopy echocardiogram were normal and HIV, TORCH titres, lupus anticoagulant were negative. MRI of the brain [] showed calcification of right basal ganglia and no evidence of infarct and MR angiogram of the brain was normal. CT of the brain [] showed multiple linear calcifications in bilateral basal ganglia predominantly involving caudate nuclei. This was a rare case of mineralizing angiopathy with developmental delay and basal ganglia stroke. The child showed improvement in her movements during her hospital stay. She was discharged with physiotherapy and is being followed up in our out patient department. Presently, she is using both upper limbs equally, but the paucity of movement in the left lower limb is still present.


A 42-year-old male patient was admitted to the emergency unit for 1 h because of a left thumb defect caused by machine gear injury []. Physical examination revealed that the left thumb was completely disconnected at the base of the first metacarpal bone in the distal end. The proximal middle portion of the severed thumb was intact, but the first metacarpal bone and the proximal end of the thumb were lost. The bone defect measured approximately 8 cm, and the skin defect covered approximately 8 cm × 6 cm. This defect was accompanied by extensor tendon and blood vessel defects. Surgery was scheduled to transplant the composite tissue flap of the second toe joint and to bridge and repair the severed fingers. After surgery, the transplanted tissue and replanted finger survived [Figure and ]. Internal fixation was removed 8 weeks after surgery and functional excision was initiated. Postoperative follow-up visits lasted for 5 months, and satisfactory bone healing was achieved []. Within 23 months of the last follow-up visit, the appearance of the replanted fingers and transplanted tissues improved, with the recovery of finger-to-palm and finger-to-finger functions. The sense of touch in the transplanted and replanted fingers recovered to S3+. The patient returned to his original job, with good walking function and satisfactory thumb-index web []. No scars and contractures were observed in the transplanted skin at the foot donor site []. The patient was satisfied.


A 23-year-old male suffered an industrial machine-press injury to the right little finger. One hour later, the patient was admitted to the emergency unit. The finger was amputated at the proximal interphalangeal joint level, with 0.8 cm of skin tissue attached. The distal amputated finger lacked a blood supply and had a joint defect. The length of the phalanx defect was about 3 cm, while the area of the skin defect was 4 cm × 2 cm [Figure and ]. A composite tissue flap with the proximal interphalangeal joint taken from the second toe was used to repair the injured finger []. The donor toe was amputated. The replanted finger and transplanted joint both survived after the operation []. The patient was followed up for 2 years. The appearance of the repaired finger was good, and it could achieve digital opposition with the thumb. The range of flexion motion for the proximal interphalangeal joint was up to 70° [Figure and ].


A 40-year-old male patient presented with back pain and focal sensory and motor deficits. Magnetic resonance imaging (MRI) spine revealed a well-circumscribed intramedullary space-occupying lesion in C6-D2 region with contrast enhancement []. Complete excision of the tumor was done.
Intraoperative squash cytology was performed. The smears were cellular and composed of cohesive clusters of round to oval cells with bland nuclei and salt and pepper type of chromatin. At places, the tumor formed large papillary structures with fibrovascular core having cells attached to blood vessel in multiple layers. No necrosis or mitosis was detected. Atypia was absent. In some places, thick bottle brush appearance was noted [Figure –].
Based on these findings and MRI, a diagnosis of ependymoma was suggested.
The histopathological sections showed well-formed papillae and finger-like projections lined by single or multiple layers of tumor cells. Several perivascular pseudorosettes and ependymal canals were noted. The tumor cells were monopolar and cuboidal or columnar in shape with a moderate amount of cytoplasm and bland nuclei having speckled chromatin. A smooth surface was formed by the apical surfaces of the tumor cells. The tumor lacked nuclear hobnailing. There was no evidence of necrosis, microvascular proliferation, endothelial proliferation, or mitosis. The tumor stained strongly for glial fibrillary acidic protein (GFAP) which was highlighted in the processes of perivascular pseudorosettes [Figure –]. It also showed positive staining for S100 and vimentin. Epithelial membrane antigen (EMA) showed apical membrane positivity []. The tumor was negative for CK and synaptophysin. Ki-67 labeling index was 5%. Based on the histological findings and IHC pattern, a diagnosis of papillary ependymoma was made.


A 13-year-old female patient reported to the hospital with a complaint of forwardly placed teeth. The patient's medical, dental and family histories were noncontributory. On clinical examination, 12 and 13 were found to be missing and 53 was over-retained.
On routine radiographic examination, a well-defined mixed radiolucent-radiopaque lesion in relation to 12 measuring 1 cm × 1 cm with radiolucent rim and an impacted 13 were incidentally noted. The radiopaque material had a density similar to enamel and dentin suggesting tooth-like mass []. A provisional diagnosis of compound odontoma with impacted 13 was made.
Excisional biopsy was performed in relation to the anterior labial region of 12 and 13 under local anesthesia, and follicle-like tissue encapsulating pieces of small calcified masses were obtained from the surgical procedure. Pathological gross findings revealed multiple soft- and hard-tissue bits. The soft-tissue bits were creamish-white in color, irregular in shape and measured 2 cm × 1.8 cm × 0.3 cm together. Hard tissue bits were multiple, irregularly shaped with tooth-like appearance and measured together 1.8 cm × 1.4 cm × 0.6 cm.
Microscopic examination of hematoxylin and eosin (H&E) stained soft-tissue sections revealed cystic epithelial lining with basal layer of ameloblast-like cells and suprabasal stellate reticulum-like cells. Focal areas of ghost cell keratinization associated with epithelial lining were noted. Few ghost cells showed calcification. Melanin pigmentation was noted focally within few ghost cells and few lining cells. The connective tissue capsule revealed fine collagen bundles, scattered inflammatory cells, spherules of calcifications, odontogenic cell rests and focal areas of melanin pigmentation []. Microscopic examination of decalcified, H&E stained hard-tissue sections revealed calcifications resembling dentin and cementum, with in a loose fibrous tissue. The ghost cells were positive for Van Gieson special stain []. Fontana–Masson special stain was carried out to confirm the presence of melanin pigmentation [].
A final diagnosis of pigmented CCOT associated with compound odontoma was arrived at due to the presence of well-developed denticles within the small cystic structure lined by typical odontogenic epithelium based on the criteria for the diagnosis of CCOT by the World
Health Organization (WHO). The postoperative course was uneventful. The patient is currently under regular follow-up.


A 68-year-old man was referred to the cancer institute of Tehran University of medical sciences with a history of painless swelling in the right side of the mandible [] and a medical history of thyroidectomy 3 years ago because of PTC. After that, he received suppression therapy with levothyroxine. One year later, follow-up in the whole body scan with I-131 showed no evidence of residual thyroid tissue in thyroid bed and negative for local and distant metastasis. On the second year after thyroidectomy, he complained of swelling in the mandible then admitted. Laboratory tests were normal except elevated thyroid-stimulating hormone level that showed subclinical hypothyroid. Abdominal-computed tomography (CT) scan with and without contrast was normal although lung CT scan shows sparse hypodense nodules in bilateral hemithorax that suspect metastasis. Neck sonography revealed disruption of the mandibular cortex and a hypoechoic lesion in the mandibular bone that posed to bony mass with a pathological fracture. Furthermore, hyperechoic of related soft tissue and multiple lymph nodes was observed. A hypoechoic region closed to the cortex probability proposed hematoma. For the second time, the whole body scan with TC-99 shows hyperactive hotspots in the right side of the mandible and degenerative changes in the body joints []. No other markable abnormality in other skeletal bones were reported. Panoramic radiography shows an invasive ill-defined radiolucent lesion in the body, angle and ascending ramus of the right side of the mandible. The destruction of the lower cortex and pathological fracture of medial border of ramus was also observed. The cortical border of the inferior alveolar canal was not detected []. CT scan without contrast from maxillofacial region shows an expansile lesion that measured about 50 mm × 32 mm which laterally extends and destructs the mandibular body and causes thinning buccal plate, medially extension to the mylohyoid muscle is seen. The lesion also involved the mandibular canal and mental foramen [Figure –]. Perforation of the lingual plate and inferior border of the mandible were also observed []. Hematologic malignancies or minor salivary gland tumors with same features can be suggested. A thick cortex 15 mm × 13 mm level I LAPD is observed on the right side.
Then, the incisional biopsy was performed. Microscopic examination shows bone trabeculae infiltrated by a malignant epithelial neoplasm composed of papillary structures which are characterized by distinctive nuclear features. The papillae are formed by fibrovascular cores, which are covered by cuboidal to columnar neoplastic epithelial cells. The crowded cells show round-to-ovoid nuclei with frequent indentations and typical clefted or grooved appearance. Furthermore, some nuclei have an empty or clear appearance. The cell cytoplasm is typically smooth and eosinophilic. The fibrous stroma shows lymphoplasmacytic infiltration. Perineural and lymphovascular invasion are identified [Figure –]. Due to the history of malignancy of thyroid and microscopic appearance, the diagnosis was metastatic carcinoma. The mentioned diagnosis was confirmed immunohistochemically by thyroid transcription factor 1 and thyroglobulin markers [Figure and ].
The patient undergoes hemimandibulectomy and the right neck dissection level I-V. The mandible was reconstructed with a costochondral rib graft []. The pathology showed one reactive lymph node in level I. It was reported metastatic papillary thyroid carcinoma.


A 55-year-old female patient presented to the otorhinolaryngology department with complaints of painless swelling in the right submandibular region and the floor of the mouth for the last 2 months. It was soft, mobile, nontender and multinodular. The provisional diagnosis of salivary gland lesion was made, and the patient was referred to fine-needle aspiration cytology (FNAC) clinic for further evaluation.
Cytomorphology of the lesion revealed cellular smears showing large elongated to round cells with abundant eosinophilic granular cytoplasm, small nuclei and occasionally prominent nucleoli. Few cells showed cytoplasmic vacuolations and were positive for periodic acid–Schiff (PAS) staining. Immunostaining for myogenin was also present in the cells. Cytological diagnosis of rhabdomyoma was rendered followed by excision of the lesion and histopathological examination [].
Grossly, four well-circumscribed, unencapsulated, nodular soft-tissue pieces were received ranging in size from 5 cm to 1 cm in maximum diameter []. Microscopically, the lesion composed of large, oval to polygonal cells with the abundant amount of deeply eosinophilic and granular cytoplasm, with small peripherally placed nuclei with few cells showing prominent nucleoli; occasional giant cells were seen [Figure –]. Partial fibrous capsule and normal muscle was also seen. Prominent cytoplasmic vacuolations were present in fair number of cells which were found to be PAS positive []. Immunohistochemically, the cells showed strong reactivity for myogenin (clone F5D, Dako Cytomation) and desmin (clone D33, Biocare Medical) [Figure and ].
Based on the above findings, the final histological diagnosis of adult rhabdomyoma was rendered.


An 85-year-old elderly female patient reported to our outpatient department with a chief complaint of swelling in her right lower back tooth region, accompanied with mild pain for 3 months. Her previous dental history revealed a tooth extraction in the same region 3 months earlier, following which she noticed a growth from the unhealed extraction socket. There was no relief of symptoms on medication. Medical history revealed that she was hypertensive and suffered from hyperlipidemia and cardiac problems for 6 years for which she was under medication. Upon extraoral examination, a diffuse swelling on the right side of the mandible was observed that extended anteroposteriorly from the symphysis to the posterior lower border of the mandible and superoinferiorly 1 cm below the tragus line to 1 cm beyond the lower border of the mandible. On palpation, the swelling was firm, nontender and noncompressible, with the overlying skin being normal. The right and left submandibular lymph nodes were palpable, firm in consistency, tender and fixed. The left middle cervical lymph node was palpable, round, soft in consistency, tender and freely movable [Figure and ].
Intraoral examination revealed a localized, well-defined swelling of size 4 cm × 5 cm in its greatest dimension obliterating the buccal, labial and lingual vestibules in the region of the nonhealed extraction sockets of 43, 44 and 45. The lesional mass was extending anteroposteriorly from the distal aspect of 42 to the distal aspect of 46, obliterating the buccal vestibule. Lingually, the swelling extended from the mesial aspect of 41 to the mesial aspect of 46 [Figure and ]. On palpation, the swelling was firm in consistency, tender and with smooth and shiny surface with cortical expansion on the lingual aspect. The superficial surface of the swelling exhibited ulceration covered by pseudomembranous slough. Based on the clinical findings, carcinoma of alveolus was considered as a provisional diagnosis. Radiographic examination revealed an ill-defined radiolucency of 2 cm × 1 cm dimension on an orthopantomogram, extending anteroposteriorly from 42 to 46. Based on the radiographic findings, a radiological diagnosis of alveolar carcinoma in relation to 44 and 45 regions was made []. An occlusal radiograph also revealed the presence of cortical defect in the area extending between 42 and 46 [], following which an incisional biopsy from 44 and 45 regions was advised.
Upon histopathological examination, hematoxylin and eosin-stained section revealed the proliferation of small, uniform, round, hyperchromatic, neoplastic lymphocytes with prominent nuclei and scanty cytoplasm, arranged diffusely. Some areas exhibited multilobulated cells, resembling Reed–Sternberg cells [Figures , and , ]. The histopathological features were suggestive of malignant lymphoproliferative lesion, following which an immunohistochemical (IHC) analysis was done using antibodies against CD20 and BCL-2, which showed a positive expression [Figure and ]. The IHC profile confirmed the clinical and histopathological diagnoses of NHL (B-cell lymphoma), following which the patient was referred to a regional cancer institute for further management. Whole-body contrast-enhanced computed tomography and positron-emission tomography were performed which revealed intensely hypermetabolic lesions involving the suboccipital region with intracranial extension, the mandible, bilateral cervical lymph nodes, left axillary nodes and pelvis, which were likely sites of disease involvement in the current clinical context [Figures – and ].


A 35-year-old male patient reported with the chief complaint of a large swelling in the right side of the upper lip. The swelling was noticed by his parents at the age of 10 years, which was the size of a peanut. The patient underwent multiple treatments including intralesional injections and surgical excision for the same in the past at other centers with failures.
When the patient reported to the department, the lesion measured 6.5 cm measuring from the infraorbital region to the lower border of mandible on the right side of the face and 7.5 cm from the right ear lobule to the contralateral commissure of the left side of the face. The thickness of the lesion was variable with maximum thickness of 5 cm in the region of right side of the upper lip. The overlying skin appeared nodular over the swelling, and multiple intraoral mucosal telangiectasias were visible. On palpation, the swelling was soft, fluctuant and compressible. Pulsations were felt, and on auscultation, bruit was also heard. Stone-hard nodules were also palpated on the superficial aspect of the lesion, suggestive of phleboliths [].
Computed tomography (CT) with angiography was done. CT angiogram of the neck and circle of Willis showed serpiginous hyperdense vascular channels causing significant soft-tissue thickening of the upper lip, right cheek region and philtrum []. Postcontrast, there is an avid enhancement. Feeding arteries appear to arise from maxillary and facial branches of the right external carotid artery. Draining vein is seen draining into the right internal jugular vein. There is early opacification of the right internal jugular vein. There is also a feeding artery from maxillary branch of the left external carotid artery. Hence, the diagnosis of diffuse subcutaneous facial AVM involving the right cheek and philtrum was given.
The patient underwent surgery as the principle modality of treatment. The surgery was planned in two phases, in the first phase ligation of right facial artery, external jugular vein; and the left facial artery was performed. This resulted in decrease in the blood volume and shrinkage of the lesion by 2 cm in overall dimensions. One unit of blood was transfused before the surgery and one unit intraoperatively. In the second phase, upper labial incision was placed along the vermillion border, bleeders were identified and circumferential ligation was done using 4’0 vicryl. Around 100 circumferential ligating sutures placed. Orbicularis oris muscle reduction was done minimally; debulking of excess tissue was done. The entire lesion was excised. In the postoperative 2nd month, secondary cosmetic correction or lip reconstruction was done.
Histopathological findings include stratified squamous epithelium and fibrous connective tissue infiltrated by neutrophils, lymphocytes and mast cells. Deeper down connective tissue showed large vascular channels of varying sizes, thick and thin walled, some empty and interspaced with areas of hemorrhage []. Based on clinical, radiographic and histopathological findings, the lesion was diagnosed as AV malformation. The patient was reviewed after 3 years; there was no recurrence of the lesion [].


A 59-year-old male with a BMI of 14.36 kg/m2 (40/1.672) was admitted to the hospital for a lung transplant in March 2014. He was diagnosed with interstitial lung disease, pneumoconiosis, and severe pulmonary hypertension five prior to admission. One year prior, he started home oxygen (nasal cannula) and his six-minute walk distance was less than 100 m. On admission, his muscle strength was found to be very weak. His upper limbs could not defend against resistance by the examiner, and his lower extremities could move, but not against gravity. Echocardiography showed mild to moderate systolic tricuspid valve regurgitation, with systolic pulmonary artery pressure (sPAP) of 93 mm Hg. Before surgery, he was placed on veno-arterial extracorporeal membrane oxygenation (VA-ECMO) with settings of 2531 rpm/min, blood flow 1.89 L/min, and oxygen flow 2 L/min to assist with the bilateral lung transplant. Three hours after surgery, the patient returned for a second thoracotomy surgery to stop the bleeding.
On the first postop day, sPAP was 26 mm Hg and hemoglobin was 84 g/L. ECMO was weaned 34 hours later. However, the attempt to switch ventilator mode from pressure-assist control (P-AC) to pressure support ventilation (PSV) failed. Electrical activity of the diaphragm showed weak neuromuscular drive. A chest computed tomography (CT) scan found bilateral collusive pneumothorax (). Percutaneous tracheotomy was performed at the bedside on postop day five.
The patient’s muscle strength decreased after surgery (muscle strength grade 2) due to immobility for a period of time (). Passive limb functional exercise was initiated, followed by combined passive and active limb exercises four times a day. Daily therapeutic bronchoscopy was performed to help clear the copious amount of secretions. Ventilator settings were carefully titrated based on vital signs and subjective complaint. Eight days later, he was placed on Optiflow via a T-piece with FiO2 0.40–0.60 and flow 30–50 L/min. However, his PaCO2 gradually increased from 35 mm Hg to 66 mm Hg in the following three days. He also felt short of breath and was diaphoretic and was then placed back on the ventilator with P-A/C. A chest CT showed some improvement in the right-side pneumothorax, but worsening consolidation and a persistent left-side pneumothorax (). Five days later, the T-piece with Optiflow was again attempted intermittently. However, on day three the patient became drowsy, with diminished and coarse breath sounds in the left lung. Arterial blood gas (ABG) showed hypercapnia (pH 7.279; PaCO2 85.7 mmHg; PaO2 206.9 mmHg). He then was placed back on mechanical ventilation with PSV: PS 16 cm H2O, positive end-expiratory pressure (PEEP) 6 cmH2O, FiO2 30%.
Nutritional support was reevaluated and adjusted, and a percutaneous gastrointestinal ostomy was performed at bedside on week six. Slight suction pressure was connected with a left chest tube to help drain gas and his paradoxical breathing gradually improved. His cough ability was still weak, but the need for bronchoscopy decreased. We initiated as aggressive bronchial-hygiene therapy as he was able to tolerate, such as postural drainage and chest percussion three to four times a day. He was mobilized to the chair at bedside and given active resistance muscle exercises. Ventilator settings were gradually reduced, and he was intermittently switched from the ventilator to the T-piece with Optiflow, and his ABG became stable.
The patient was able to sit on a chair without assistance and stand up at bedside on week eight (limb muscle strength grade 4). At week 10, he was able to perform assisted walking with ventilator support. Ventilator settings were adjusted based on his vital signs and subjective complaint. A chest CT on week 11 displayed no presence of pneumothorax, and consolidation in the right lower lobe had improved. However, a bronchoscopy showed copious secretions in the right main bronchus. Chest wall oscillation was used to clear secretions. During this time, the patient started exhibiting insomnia and anxiousness. An antidepressant and sleep medication were prescribed by a consulting psychologist. The patient was weaned intermittently from the ventilator to the T-piece (FIO2 24%–28%) on week 13. Finally, a cuffless tracheostomy tube was placed on week 14 and capped 5 days later. He was decannulated with 2 L/min nasal cannula on week 15, and discharged from the intensive care unit with a BMI of 16.06 kg/m2.


A 62-year-old female with a medical history of diabetes, hypertension, atrial fibrillation, and hyperlipidemia came to our hospital because of fever and pain on the left hip joint. She underwent cemented THA on the left hip joint with transtrochanteric approach at 61 years old ( and ) and the symptoms appeared suddenly 13 months after the operation. On admission, her body temperature was 38.4°C, and the left thigh was swollen and tender, but sinus tract is not seen on her skin. Blood examination showed her white blood cell count was 9490/µL (reference range in our hospital is 4000—/µL) and C-reactive protein (CRP) was 24.7 mg/dL (reference range is <0.5mg/dL). Loosening of the implants was not seen in the X-ray photograph (); however, magnetic resonance imaging showed high-intensity area around her left hip joint and gluteus on short-TI inversion recovery ( and ). We tried arthrocentesis, but very poor amount of synovial fluid appeared. Hence, we performed synovial fluid culture, but cell count was not available. She was started intravenous ceftriaxone (CTRX) 1 g/day from the day of admission after collecting of samples of blood culture, but both blood and synovial fluid culture turned out to be negative. To identify the causative bacterium, we remove the wire in her greater trochanter and debride granulation and synovia on the 26th day. From multiple pieces of the trochanteric wires, granulation, and synovia, YE was detected, and it was susceptible to CTRX. We suggested revision arthroplasty to her, but she never agreed because the symptom subsided, so we continued treatment by antibiotics. Her symptoms subsided, and CRP kept negative so we changed the antibiotics to oral ciprofloxacin which was susceptible to the causative bacterium on the 61st day and we continued the treatment to the 74th day. She discharged on the 76th day. No recurrence has been seen in 24 months after the discharge ().


A 14-year-old female presented with a history of pain in the right hip for 4 years. 1 year before presentation she sustained a fracture of the right hip following trivial trauma and was treated operatively. The patient was kept non-weight bearing for 3 months, following which she achieved full weight bearing over the next 3 months. Her gait was always painful. On examination, the patient walked with antalgic short limb gait. Muscles around right hip appeared wasted. There was fixed flexion deformity of 10° and fixed adduction deformity of 30°. Range of motion (ROM) was as follows: Flexion till 130°, adduction till 50°, external and internal rotations of 30° and 20°, respectively. There was an apparent shortening of 6 cm and true shortening of 3 cm. Radiographs revealed ununited subcapital fracture with sliding hip screw in situ. Femoral metaphysis showed central expansile lytic lesion with ground glass matrix suggestive of underlying fibrous dysplasia.