A 54-year old female presented with complaints of painful swelling with restricted movements of right shoulder for past five years which had worsened over the last one year. She was a known case of rheumatoid arthritis diagnosed ten years ago. Five years back she had noticed painful fullness around her right shoulder. Size of the swelling had increased over the period of five years. She had felt her shoulder becoming stiffer with increase in the size of the swelling. On examination there was a well demarcated, firm with smooth surface, non-fluctuant and immobile swelling located at the right sub-deltoid space, with purplish discoloration of the overlying skin. Right shoulder movements were painfully restricted. Sub-acromial impingement signs were present.
Her ESR was 104, Rheumatoid factor 121.2 IU/ml and Uric acid 4.9 mg/dl. Plain radiograph of shoulder showed normal glenohumeral articulation with sub-deltoid homogenous soft tissue shadow without calcification (). Ultrasound suggested diffusely enechoic soft tissue mass surrounding the shoulder joint. MRI suggested marked distension of the sub-acromial/sub-deltoid bursa but more of sub-deltoid bursa, approximately 3cm deep all around. The bursal lumen was filled with multiple loose bodies ranging from 1 to 7mm in size. Loose bodies show iso-intense signal on T1 weighted images and hypo-intense on T2 weighted images. Rotator cuff and glenohumeral joint were normal. This MRI picture was suggestive of synovial origin lesions of arthritis ().
The patient was scheduled for arthroscopic loose body excision and sub-acromial bursectomy. She was positioned in beach chair position under general anaesthesia. Standard posterior, anterior and lateral portals were used for arthroscopy. Diagnostic arthroscopy revealed normal glenohumeral articulation without communication of sub-acromial sub-deltoid bursa with the joint. Multiple shiny white rice bodies of different sizes were observed occupying the sub-acromial space and extending into the anterior, lateral and posterior sub-deltoid space. Rotator cuff muscles were normal. Synovial fluid was submitted for fluid evaluation, Gram stain, Ziehl-Neelson stain, Tuberculosis Polymerase Chain Reaction (TB-PCR) and bacterial culture. Tissue sample from the bursa and rice bodies were sent for histopathological evaluation. All rice bodies were removed through 8mm arthroscopic cannula, placed in different portals sequentially, and attached to a suction device. Residual rice bodies were removed manually with an arthroscopic grasper. Sub-acromial bursa was excised and acromioplasty was performed.
She was started on range of motion exercises of shoulder from first post-operative day. TB-PCR of synovial fluid was negative for tuberculosis. Culture of synovial fluid did not grow any organism. On histopathological examination the bursal tissue showed hyperplastic synovial lining with marked lymphoplasmacytic inflammatory infiltrates, few neutrophils with vascular proliferation of stroma without granulomatous reaction. The rice bodies were composed of dense fibrin deposition with fibroblast and mononuclear cells (). She was started on disease-modifying antirheumatic drugs after suture removal at 12th post-operative day. The pre-operative painful swelling with restriction of movements markedly improved after surgery. At one year post-operative follow-up, there was no recurrence of shoulder swelling, with excellent range of motion.


Case 1. A man aged 34 years with a 17-year history of soft contact lens use was evaluated for left eye redness and blurry vision. He reported sleeping in his contact lenses 3–4 nights per week and swimming with contact lenses. He was treated for bacterial and fungal microbial keratitis for 2 months without improvement. He was evaluated at an academic medical center, where confocal microscopy, a technique that provides serial images of sections through the cornea, revealed findings suggestive of Acanthamoeba keratitis. He was treated with topical polyhexamethylene biguanide and chlorhexidine hourly that was tapered over 6 months. The infection resolved with final spectacle-corrected visual acuity of 20/40, requiring rigid contact lenses for correction to 20/20.


Case 2. A man aged 59 years wore his soft contact lenses overnight during a 2-day hunting trip and developed eye pain on the third day. He used over-the-counter eye drops with minimal response. On initial evaluation, he was diagnosed with a corneal abrasion and treated with a bandage contact lens to promote healing, along with tobramycin/dexamethasone drops prescribed four times daily. With worsening symptoms, his treatment was changed to ofloxacin drops every 2 hours. While in the shower, he wiped his eyes with a towel, then heard a popping sound and felt a painful sensation in his left eye. He was referred to ophthalmology where a large perforated corneal ulcer was diagnosed. An urgent corneal transplant was performed to reestablish the integrity of the eye, and he was treated with broad-spectrum topical antibiotics postoperatively. He recovered useful vision, which improved to 20/25 after cataract surgery 1 year later.


Case 3. A woman aged 34 years was evaluated for 3 days of sharp right eye pain. She routinely slept in her soft contact lenses and used lenses for longer than the recommended monthly replacement schedule. She reported not seeing an eye care professional in years and refilling her contact lens prescription through an online contact lens retailer for at least 5 years. Examination of the right eye revealed a paracentral 1.5 mm infiltrate with surrounding edema and trace anterior chamber cells. Symptoms and signs were improved the day after treatment with topical moxifloxacin. She was instructed to continue moxifloxacin but failed to return for a 1-week follow-up appointment as instructed.


Case 4. A man aged 57 years was evaluated in the emergency department with bilateral reduced vision and eye pain. He reported wearing the same soft contact lenses continuously for approximately 2 weeks. He did not disinfect his lenses daily, slept in them on a regular basis, and did not replace them regularly. On examination, uncorrected visual acuity was light perception in the right eye and hand motion in the left eye. The right eye revealed a central corneal infiltrate and perforation of the cornea. The left eye revealed a central infiltrate with two infiltrates paracentrally and a hypopyon (leukocytes in the anterior chamber of the eye). He received a diagnosis of bilateral bacterial keratitis. Hourly fortified tobramycin and vancomycin drops were required for treatment. A corneal transplant was required to save the right eye. The left eye responded to topical therapy with visual acuity of 20/40 and a central stromal scar.


Case 5. An adolescent female aged 17 years who slept in a soft contact lens purchased without a prescription at a chain store developed a right corneal ulcer; a culture grew Pseudomonas aeruginosa. She was started on fortified tobramycin and vancomycin eye drops. Her vision was light perception in the right eye, and the cornea showed a central white dense ulcer, stromal infiltrates, and 0.5 mm hypopyon. On follow-up, her vision had improved to 20/100, pinhole to 20/60. She had a stromal scar with thinning.


Case 6. A man aged 18 years went to the emergency department with a 3-day history of pain, redness, light sensitivity, and tearing in his left eye. He had a 1-year history of wearing decorative soft contact lenses obtained at a local store without a prescription. He also reported sleeping in his lenses. He was given fluoroquinolone eye drops in the emergency department and subsequently was seen at a local eye clinic, at which time bacterial keratitis was suspected. His vision was 20/25 in the right eye and 20/50 in the left. His left eye showed moderate injection with a central ulcer, edema, and moderate inflammatory reaction. Cultures were obtained, and hourly fortified cephalosporin and aminoglycoside drops were prescribed. Follow-up cultures of the patient’s eye, his lenses, and lens case each yielded heavy growth of Klebsiella pneumoniae and Pseudomonas aeruginosa. Ten days later his symptoms were better; vision in the left eye had improved to 20/25, but a stromal scar remained.


A 65-year old male patient who was diagnosed with gastrointestinal stromal tumor at bulbus of duodenum by gastric endoscopy was referred for a 68Ga-DOTANOC PET/CT for investigation of metastasis. PET/CT demonstrated increased tracer uptake in the right ischium, right iliac, and pubic bones (Figure ). He was asymptomatic without any complaint. Subsequent conventional radiography and CT images confirmed the presence of Paget disease in the right hemi-pelvis (Figure ).


A 74-year-old female was referred to the emergency department with persistent abdominal pain. Five months earlier, she had experienced acute small bowel intestinal obstruction necessitating emergency laparotomy. This occlusion was caused by anterior parietal adherences related to recurrent previous abdominal surgery. Contrast-enhanced MDCT excluded now significant dilatation of the gut but demonstrated an unusual accumulation of abdominal fat incarcerating mesenteric vessels and bowel segments in the midline of the anterior mesogastrium (Figure , white arrows). Careful multiplanar analysis (Figure shows the antero-posterior coronal MPR views, and Figure shows the axial oblique MPR view) revealed the omental nature of the encircling fatty structure through the clear delineation of the encircling omental vessels (white arrows). This 360-degree whirl of the greater omentum was incarcerating the right ileocecocolic mesenteric vessels (white star); the two incarcerated and compressed bowel segments were identified as the terminal ileum and the transverse colon (black arrows). Selective volume-rendering reconstruction confirmed the whirl (Figure ). Despite the fact that there was no critical dilatation nor suffering signs of the gut at the time of MDCT, the risk of occlusion was nevertheless considered important, and it seemed unlikely that this complete omental whirl would resolve spontaneously. This caution justified laparotomy.


A 44-year-old woman experienced sudden onset of difficulty using her left hand and walking during her menstrual phase. She had a history of adenomyosis and infertility treatment for 18 years, and heavy menstrual bleeding. She complained of headache, abdominal pain, nausea, and had a fever (37.7 °C) at presentation. She is not obese (BMI of 21.5 kg/m2), had no history of taking steroids or contraceptives. Neurologic examination revealed left spatial neglect, left facial hypoalgesia, mild paresis in her left arm, and right pyramidal signs. Brain magnetic resonance imaging (MRI) revealed bilaterally multiple infarctions in the cerebrum and cerebellum, including cortical and subcortical lesions (Fig. ). MR angiography presented severe stenosis in the M2, M3, >and M4 portions of right middle cerebral artery (Fig. ). Contrast computed tomography revealed a splenic infarction (Fig. ). Blood examination revealed normocytic anemia (hemoglobin 10.3 g/dl, mean corpuscular volume 90.5 μm3), thrombocytopenia (112,000 /μl), and low-grade elevation of C-reactive protein (2.9 mg/dl). The serum levels of D-dimer (17.0 μg/ml, normal < 0.5 μg/ml), CA125 (2115 U/ml, normal < 35.0 U/ml), and carbohydrate antigen 19–9 (CA19–9) (1824 U/ml, normal < 37.0 U/ml) were increased. Results of a hypercoagulable panel, including protein C and S, antithrombin Ш level, lupus anticoagulant, and anticardiolipin antibody titers, were within normal limits. Pelvic MRI revealed giant adenomyosis (Fig. ), but no malignancy was detected. Fluorine-18–2-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography revealed FDG accumulation in the adenomyosis, but no malignancy was detected by cervical cytology. The result of continuous electrocardiography monitoring, transesophageal echocardiography with agitated saline injection, carotid ultrasonography, upper gastrointestinal endoscopy, and colonoscopy were normal.
She was treated with edaravone (60 mg/day) and anticoagulated with heparin for 2 weeks. Subsequently, she was treated with rivaroxaban (15 mg/day). Her serum levels of D-dimer, CA125, and CA19–9 improved (2.7 μg/ml, 911 U/ml, and 501 U/ml, respectively), and the treatment was continued.
At day 31, a day after her menstrual phase started, she complained of numbness in her left lower limb. Brain MRI revealed a new CI in the right cerebrum (Fig. ). The serum levels of D-dimer, CA125, CA19–9 were 2.4 μg/ml, 561 U/ml, 417 U/ml, respectively. We discontinued the rivaroxaban treatment, and started anticoagulant therapy with warfarin. Afterwards, her menstrual bleeding increased, the anemia progressed, and the serum level of D-dimer increased (14.1 μg/ml). She started to receive pseudomenopause treatment with a GnRH agonist for the adenomyosis. Ten days after initiating the GnRH agonist treatment, her serum D-dimer level improved (2.30 μg/ml). She continued the warfarin and GnRH agonist once a month for 6 months, and showed no recurrence of CIs during that time. The serum levels of CA125, CA19–9 improved after 3 months of initiating the therapy (117 U/ml, 224 U/ml, respectively).
She presented with transient weakness of her right lower limb and visited our clinic 5 months after her last GnRH agonist therapy, when her irregular menstrual bleeding had continued for a month. Brain MRI revealed new multiple cortical and subcortical infarctions in the left occipital lobe and right parietal lobe (Fig. ). Her serum D-dimer, FDP, CA125, and CA19–9 levels were elevated (22.0 μg/ml, 56.5 μg/ml, 1291.6 U/ml, and 803.2 U/ml, respectively). Anticoagulant therapy with warfarin was well controlled (PT-INR 2.5), and her electrocardiographic findings were normal. We concluded that total hysterectomy would be the most effective therapy for preventing CI recurrence. She underwent total hysterectomy and bilateral salpingo-oophorectomy, which was effective in not only preventing CI recurrence, but also for normalizing the serum D-dimer, FDP, CA125, and CA19–9 levels for the last 2 years.


A 20 year old Sri Lankan male who was employed as a helper in a grocery, admitted to our unit with weakness of both hands of 1 month’s duration. He was treated for serologically confirmed (Dengue NS1 antigen positive) dengue fever approximately 5 weeks ago at the local hospital and had made an uneventful recovery. He has been given 5 days of inward treatment and the records from the local hospital revealed that he had simple dengue fever with no evidence of fluid leakage.
Five days after discharge from the hospital he has first noticed the weakness of his right hand when he dropped a glass of water due to poor grip. Weakness was more in the right hand which was his dominant hand and it was slowly progressive over 1 month. At the time of presentation to us he could not write or button on his shirt due the weakness of the hands. Weakness of the left hand was milder than that of the right. The weakness was confined to hands and did not involve forearms or arms. He denied any accompanying numbness, parasthesia or pain.
On inquiry he admitted that there was slight weakness of both feet which did not significantly interfere with walking. There was no associated neck/back pain or bladder/bowel incontinence. He did not complain of difficulty in breathing, diplopia, dysphagia, nasal regurgitation, dysarthria or fatigability. He did not give a recent history of trauma to the spine/neck or any preceding diarrheal illness or skin rash.
He had no previously diagnosed long term medical ailments and has not undergone any surgical procedures in the past. He was not on any long term medications and he denied smoking, use of alcohol or illicit drugs. He did not give a family history of any progressive neurological conditions.
On general examination he had an average built with no pallor, lymphadenopathy or any signs of malnutrition. No skin rashes or hypopigmented patches were noted. There was minimal small muscle wasting of bilateral hands and feet. No muscle fasciculations were noted. Distal upper limb (hand) power was diminished asymmetrically, right hand demonstrating a power of 3 out of 5 and left hand demonstrating a power of 4 out of 5. All fine finger movements including flexion, extension, abduction and adduction were affected with some degree of weakness in wrist extension as well. Bilateral supinator and biceps reflexes were diminished.
Distal lower limb (feet) power was also diminished but was less pronounced (power grade 4) when compared to the degree of hand weakness. Bilateral foot dorsiflexion was weak. Ankle jerks were elicited with reinforcement whereas the knee jerks were elicited without reinforcement. There was no objective sensory impairment of touch, pain, temperature, vibration and joint position sensations in both upper and lower limbs. Bilateral plantar responses were down going. No palpable nerve thickening identified. No cerebellar signs were demonstrated and his gait showed a minor degree of high stepping due to weak dorsiflexion. Examination of higher functions and cranial nerves including the fundal examination revealed no abnormality.
Examination of the cardiovascular, respiratory systems and the abdomen was essentially normal.
Full blood count revealed white blood cell count: 8.5 × 109/L, platelet count: 274 × 109/L, hemoglobin 12 g/dl with normal red cell indices. Blood picture showed normochromic normocytic cells with some reactive lymphocytes suggestive of a recent viral infection. Serum creatinine 80 μmol/l (60 - 110 μmol/l), serum sodium 138 mmol/l (135 - 145 mmol/l), serum potassium 3.8 mmol/l (3.5 - 5 mmol/l), serum magnesium 0.9 mmol/l (0.8–1.1 mmol/l), serum ionized calcium 1.2 mmol/l (1.05–1.30 mmol/l). Liver profile: AST 21u/l (10 - 40u/l), ALT 13u/l (7–56 u/l), ALP 67u/l (100–360 u/l), serum total bilirubin 0.7 mg/dl (0.1–1.2 mg/dl), serum albumin 36 g/l (35 - 50 g/l), serum globulin 32 g/l (20 - 35 g/l). CPK levels were normal. Inflammatory markers: ESR 25 mm/hour and CRP < 6 mg/dl.
Nerve conduction study revealed findings in keeping with multifocal motor neuropathy with conduction blocks involving the distal upper and lower limb peripheral nerves without any conduction abnormalities in the sensory nerves (Fig. ).
CSF analysis did not show any increase in proteins or cells and the values were within the normal limits. Anti-GM1 IgM antibody test was not carried out due to the high cost of the test and the patient’s unstable financial background. A sural nerve biopsy (a sensory nerve) was carried out and revealed histologically unremarkable nerve fibres and blood vessels with no evidence of inflammation, atrophy or granulomata formation. Recent dengue infection was confirmed with positive dengue IgM and IgG antibodies with enzyme-linked immunosorbent assay (ELISA).
As the patient fulfilled criteria, the diagnosis of multifocal motor neuropathy with conduction blocks was confirmed. He was then referred to the neurologist and was started on intravenous immunoglobulin (IVIg) therapy (2 g/kg/day) which was given for 5 days. He showed a mild improvement of his neurological weakness with the treatment and outpatient physiotherapy was arranged. The next immunoglobulin dose was planned to be given after 2 weeks.


A 60-year-old, para VI, Ethiopian woman presented with a progressively increasing vulvar swelling of 25 years’ duration. She developed a dull aching pain 3 months previously with difficulty of micturition and dysuria. There was no discharge or bleeding. She had undergone genital cutting and sewing during her childhood. Her medical and surgical histories were unremarkable. She is unemployed. There was no similar history of illness in her family. She neither smokes tobacco nor drinks alcohol. She had not visited any other health care facility for the complaint and had not been on any medication prior to diagnosis.
On physical examination, her vital signs were: pulse, 82; respiratory rate, 18; and temperature, 36 °C.
She had pink conjunctiva and non-icteric sclera; her chest was clear and resonant. Her heart sounds were all normal with no murmur or gallop. Her abdomen was soft and moved with respiration. There was no tenderness, guarding rigidity, palpable mass, or organomegaly. In her genitourinary system, there was a 18 cm by 12 cm sized, fluctuant, multi-lobulated, mobile, non-tender, right labia majora mass involving the mons pubis and stretching the ventral skin of the urethra (Fig. ). The overlying skin was normal. The two labial edges were fused at their cranial part. There were no abnormal findings in her vagina, cervix, and uterus on speculum and digital examinations. The inguinal lymph nodes were not enlarged.
Musculoskeletal and neurological examinations were unremarkable.
Laboratory tests results were: hemoglobin, 13 gm/dl; white blood cell count, 6500/mm3; platelet count, 250,000; and blood group/Rh B+. Urine analysis was non-revealing; a pelvic ultrasound scan showed atrophied uterus with no pelvic mass.
She was counseled for surgical removal (excision) and informed consent was obtained. Under spinal anesthesia, a urinary Foley catheter was inserted; the vulvar mass was excised successfully without any significant bleeding or injury to the adjacent structures. Her postoperative course was smooth with healed wound site on postoperative day 7 checkup visit. She had her second postoperative visit 1 month after the surgery and she had no genitourinary complaints with healed vulvar wound site. A histopathologic examination report was consistent with epidermoid cyst of the vulva.


The patient was a 75-year-old woman, who had developed left shoulder pain five years earlier without any known precipitating factor. She presented to our department because of gradual difficulty in raising her left arm and worsening pain. Physical examination on presentation did not reveal any swelling or feeling of heat in the left shoulder. The range of motion of the left shoulder showed extremely severe restriction; namely, flexion 80°, abduction 60°, and external rotation 0°, and prominent impingement symptoms were found. On plain radiographs and computed tomography (CT), prominent shoulder arthropathic changes and numerous calcified lesions around the joint were found. On plain magnetic resonance imaging (MRI), around the shoulder an irregular hypointense region was identified in the center on T1-enhanced images and on T2-enhanced images (). Routine blood examinations did not reveal any obvious abnormalities. This patient was diagnosed with synovial osteochondromatosis associated with a massive tear of the rotator cuff and shoulder arthropathic changes, for which the treatment RSA was chosen.
Intraoperative findings: A delto-pectoral approach was used. Full-thickness tears of the supraspinatus and infraspinatus tendons were found. When the joint capsule was incised, synovial proliferation and a whitish mass-like lesion, seemingly adherent to the synovium, were found. The intraarticular mass was resected to the extent possible, but the cartilage in the humeral head was severely damaged, while that in the glenoid cavity had almost disappeared. After loose body resection, RSA was performed.
The postoperative course was uneventful, with the left shoulder pain disappearing from early in the postoperative period. At one year postoperatively, there was no recurrence of pain and the left shoulder range of motion showed improvement to flexion 140°, abduction 130°, and external rotation 30°. Moreover, no complications such as recurrence of osteochondromatosis, implant loosening, or infection were seen.
The loose body was found to consist of a cartilage component and bone tissue with hyalinization (). No findings indicative of malignancy were evident, and since nodular cartilage arrangement was found, primary osteochondroma was diagnosed. Synovial osteochondromatosis could be diagnosed.


A 71-year-old man with previously known keratoconus presented with bilateral cataract (Fig. ). In the left eye, PKP had been performed when the patient was 25 years old because of keratoconus (Fig. ). No surgery had been done in the right eye. Because of discomfort with contact lenses, the patient wore glasses both for near and far distance. The patient had a medical history of a transient ischemic attack and medicated with acetylsalicylic acid. The right eye presented with advanced keratoconus including Vogt striae (Fig. ) in the cornea and moderate senile nuclear cataract but no other pathology. The left eye presented with a clear corneal graft and moderate senile nuclear cataract but no other pathology. First surgery was only planned in the left eye. After more than 1.5 years surgery was also performed in the right eye. Written informed consent was acquired from the patient.
Preoperatively, best corrected visual acuity (BCVA) was 20/40, with − 0.25 sph − 5.0 cyl 50°. The cornea exhibited regular astigmatism (K1 44.5 D, K2 48.5 D, astigmatism 3.9 D) (Fig. ) based on corneal tomography performed with Scheimpflug imaging (Pentacam, Oculus, Germany). The toric IOL AcrySof IQ Toric SN6AT8 (Alcon, USA), 22 D was implanted with target refraction − 2.26 D. The target refraction was chosen to match the more myopic right eye. Biometry was performed with the IOLMaster (Carl Zeiss Meditec, Germany) and Haigis formula was used. Preoperative marking of the toric IOL axis was performed with the patient in upright position to avoid misalignment due to cyclotorsion, using the RoboMarker (Surgilum, USA). Phacoemulsification and lens implantation were performed through a 2.2 mm limbal incision. One day postoperatively, BCVA was 20/40 with − 2.0 cyl 90°. Five weeks postoperatively BCVA was 20/30 with + 0.5 sph − 2.75 cyl 71°. Nine months postoperatively BCVA had improved to 20/25 with − 3.25 cyl 90° and the astigmatism was still regular (Fig. ) based on corneal tomography performed with Scheimpflug imaging. Two years postoperatively BCVA was still 20/25 with − 0.5 sph − 3.25 cyl 80°. The spherical equivalent 2 years postoperatively only differed − 0.135 D from the intended target refraction. During all postoperatively controls the toric IOL only misaligned 1° (from 139° to 140°) from the implanted axis and the corneal graft remained clear. The patient was very satisfied with the visual result from day one postoperatively.
Preoperatively, BCVA was 20/150, with − 5.75 sph − 9.75 cyl 72° and the cornea had irregular astigmatism (K1 53 D, K2 57.7 D, astigmatism 4.7 D) (Fig. ) based on corneal tomography performed with Scheimpflug imaging. In the right eye, the astigmatism was judged as being too irregular for toric IOL implantation and cataract surgery was performed with the spherical IOL Acrysof Multipiece MN60MA (Alcon, USA), 5 D. Conventional biometry (IOLMaster) and Haigis formula was used to calculate the power of the spherical IOL. Two months postoperatively, BCVA was 20/80 with + 1.25 sph − 3 cyl 65°, spherical equivalent − 0.25. Target refraction prior to surgery was − 2.33 D, however the patient was pleased with the obtained result. He continues to wear glasses for far distance but does not require glasses for near distance.


A 63-year-old woman with decompensated liver cirrhosis secondary to hepatitis B virus (HBV) infection was referred as a candidate for LDLT. She had been diagnosed with hepatitis B 20 years before, but it had not been actively treated. She had received best supportive care, but she and her family chose to proceed with LDLT. Laboratory findings before LDLT were as follows: serum total bilirubin, 8.4 mg/dL; serum albumin, 2.5 g/dL; prothrombin time, 40%; platelet count, 84,000/μL; and Model for End-stage Liver Disease score, 17. A large amount of ascites, liver atrophy, and collaterals were observed on computed tomography scan. At the time of admission, her urine volume was decreased to 50 mL/day, and continuous hemodiafiltration treatment was started for renal failure. The predictive risk score [] was 0.80, which was lower than the score of 1.3 which predicts a poor prognosis, and the risk of postoperative mortality was therefore expected to be high. After obtaining full informed consent from both the donor and the recipient and approval from the Liver Transplantation Committee of Kyushu University, the patient was prepared for LDLT using a right posterior section graft.
The donor was the patient’s husband, who was 63 years old and had an identical blood type B. The surgical techniques were carried out as described previously []. The graft weight was 581 g, which was equivalent to 56.8% of the recipient’s standard liver volume (graft–recipient weight ratio, 1.12%). The hepatic arterial flow in the RHA was 87 mL/min, and the portal venous flow was 510 mL/min after reperfusion. The portal system pressure was 18 mmHg at the end of surgery, and splenectomy was not performed. The anhepatic time, and cold and warm ischemic times were 158 min, 92 min, and 49 min, respectively. The surgical time was 10 h, and the estimated blood loss was 4440 g. Ten units of red blood cells, 16 units of frozen plasma, and 40 units of platelets were transfused during surgery.
The post-transplant course is shown in Fig. . The patient received post-transplant immunosuppression with tacrolimus, steroid tapering, and mycophenolate mofetil. On postoperative day (POD) 7, the patient developed SFSS with serum total bilirubin 20.0 mg/dL and abdominal ascites 2000 mL/day [], indicating prolonged cholestasis and intractable ascites []. Tests for HBV DNA and cytomegalovirus (CMV) were negative, and repeated blood cultures were also negative until POD 24. There were no signs of abnormal hepatic flow or surgical complications detected by daily Doppler echo until POD 14 and by routine computed tomography scan on POD 7. There was also no notable elevation of hepatobiliary enzymes, thus ruling out the possibility of graft rejection.
The patient developed a fever above 39 °C on POD 9, and laboratory findings showed a white blood cell (WBC) count of 1120/μL and a platelet count of 40,000/μL, which fell to 300/μL (neutrophils, 30/μL) and 25,000/μL, respectively, on POD 11. She was treated with a combination of G-CSF and intravenous immunoglobulin, but her leukocytopenia/neutropenia failed to improve. Bone marrow aspiration was performed on POD 12, and histology revealed many macrophages and phagocytosis of hematopoietic cells (Fig. ). The patient was therefore diagnosed with HPS following LDLT. Steroid pulse therapy with 1000 mg/day methylprednisolone was initiated on the same day and continued for 3 days. Her WBC count increased to 4290/μL on POD 15, suggesting an improvement in her peripheral blood leukocytes. However, the patient developed sepsis with a fever above 39 °C on POD 24, and blood cultures were positive for Enterococcus faecium and Escherichia coli infections. She recovered rapidly with empiric antibiotic therapy including carbapenem and vancomycin, but her graft dysfunction was prolonged with a consistent serum bilirubin value of around 30 mg/dL, and her renal failure never improved. She also had several episodes of bacterial pneumonia. Splenic artery embolism was performed on POD 84 but failed to improve her graft dysfunction. Regarding immunosuppressive treatment to control infections, mycophenolate mofetil was stopped on POD 8 and not restarted, and the minimum dose of tacrolimus was administered to maintain a trough blood concentration of 3–5 ng/mL. The patient underwent a tracheotomy, and her liver function was expected to improve with long-term management; however, she developed bacterial sepsis and died of liver failure on POD 146.
HPS, also referred to as hemophagocytic lymphohistiocytosis, is a rare and often fatal disease despite treatment [], characterized by a variety of symptoms including fever, lymphadenopathy, hepatosplenomegaly, jaundice, and skin rash []. We report a rare case of a 63-year-old woman who developed HPS 2 weeks after LDLT with a right posterior section graft, who was treated with steroid pulse therapy following an early diagnosis via biopsy. This case may help to shed light on the relationship between HPS and graft dysfunction, including prolonged SFSS.
HPS is defined as a proliferation of phagocytic macrophages in the bone marrow, spleen, or lymph nodes, with clinical findings including fever for ≥ 7 days with peaks ≥ 38.5 °C, cytopenia (at least two of three lineages), and splenomegaly []. However, this definition is based on infant HPS and may be difficult to apply in adults and patients with various basic diseases such as liver cirrhosis or after LDLT. It is therefore necessary to perform bone marrow aspiration immediately to reach an early diagnosis of HPS in these patients []. The laboratory findings in the current patient showed WBC counts of 1120/μL on POD 9 and 300/μL on POD 11. Apart from HPS, other differential diagnoses such as medicine-induced disease, infections such as sepsis, hypersplenism, leukemia, and lack of vitamin B12 were unlikely and bone marrow aspiration on POD 12 showed typical HPS characteristics of phagocytic hematopoietic cells. This rapid diagnosis allowed early treatment with steroid pulse therapy, which contributed to an improvement in the patient’s peripheral blood leukocytes.
HPS is a rare complication after liver transplantation with a prognosis for patient survival of 50% [, ]. Fifteen patients, including the present case, have been reported to date (Table ) [, , –], only four of whom survived. About a third of secondary HPS cases are associated with virus infection, half with lymphoma, and the remainder with bacterial and fungal infections. Given that HBV DNA and CMV tests on POD 7 were negative and blood cultures were also negative until POD 24, it was considered highly unlikely that the secondary HPS in the current patient was associated with a viral or bacterial infection. The combination of anemia and thrombopenia also added the possibly of thrombotic microangiopathy as a differential diagnosis, but no typical hemolytic anemia was detected in the bone marrow biopsy and there was no sign of encephalopathy. Thrombotic microangiopathy was thus discounted as a possible cause of HPS, and leukopenia was therefore considered the most likely cause. However, liver biopsy after LDLT and measurement of ADAMTS-13 were not performed in the present patient.
In general, patients may develop secondary HPS via hypercytokinemia [] due to systemic inflammatory response syndrome associated with a viral or bacterial infection. The current LDLT recipient had SFSS, which might have been associated with the donor’s older age (63 years) []. SFSS can also induce oxidative stress and hypercytokinemia [].
Based on this hypercytokinemia theory, 10 of the past 15 cases (66.7%) were treated with steroids, 10 (66.7%) with intravenous immunoglobulin, and four (26.7%) with plasma exchange. The present patient started combination therapy with intravenous immunoglobulin, G-CSF, calcineurin inhibitor conversion, and steroid pulse therapy on POD 12, with subsequent improvement in her peripheral blood leukemia. However, her course was complicated by bacterial sepsis on POD 24. The causal relationship between steroid pulse therapy and sepsis could not be clarified, but the possibility of a septic attack is always present.
In addition, the relationship between SFSS and HPS was not unknown in the present case, and the possible causative effect of SPSS remains speculative. To reduce the risk of HPS, it is necessary to avoid developing viral and bacterial infections and hypercytokinemia, which induce secondary HPS, while the use of elderly donors might also increase the risk of SFSS. However, it may not be possible to avoid these factors in the setting of LDLT. Further studies on the mechanism of hypercytokinemia-induced HPS and more basic studies are needed to confirm the optimal treatment for HPS.


A 47-year-old man presented with a 10-day history of right scrotal pain, swelling and erythema. He had malodorous drainage from right scrotum for two days. He had no diabetes mellitus (DM), hypertension or any other co-morbid diseases; also there were no any risk factors including drug-use, immunodeficiency, genito-urinary or anorectal trauma and infection in his medical history. Massive edema in both of two hemiscrotum and black necrotizing area with malodorous pus drainage in the bottom of the right hemiscrotum was detected in his physical examination (). His anorectal examination was normal. Laboratory analysis revealed as a serum creatinin 0.9 mg/dl, hemoglobin 14.9 g/dl, glucose 486 mg/dL, CRP 156 mg/L, WBC14.5x106 cells/mL, sodium 132 mmol/L. There was no infection sign in bilateral testis, but their sizes were found smaller in scrotal ultrasound (right testis 25x20 mm, left testis 20x20 mm). Intravenous crystallized insulin therapy was given for decreasing serum glucose levels and intravenous imipenem 4x500 mg and Clindamycin 4x600 mg were started prophylactically to the patient according to the infectious disease consultation. All necrotizing tissues were debrided in right scrotum. Right hemiscrotectomy was performed and right testis had a normal blood supply appearance in operation (). Open wound dressing with the nitrofurazone and rifamycin was performed in first three days after operation. The vacuum-assisted closure technique (VAC) (a technique that keeps the wound environment under sterile condition and decreases the frequency of changing protective covers of wound) was performed to the patient in postoperative 3rd day to postoperative 15th day. VAC dressing makes its function by creating mechanical stress with negative pressure of the vacuum system; wound edges diminish, granulation formation accelerates, cellular proliferation and neoangiogenesis increases.
Pathological result revealed as Fournier gangrene and Rhizobium radiobacter was isolated from both of the tissue and abscess cultures. Rhizobium radiobacter was only resistant to trimethoprim sulfamethoxazole and sensitive to ceftazidime, cefepime, imipenem, meropenem, ertapenem, amikacin, gentamycin and tetracycline; therefore same antibiotics were continued to the patient. Control tissue culture was done at postoperative 13th day and by the results revealed as negative wound site was sutured primarily at postoperative 15th day. Patient was discharged at postoperative 18th day with the suggestion of endocrinology consultation for a new diagnosed diabetes mellitus. Patient had come to third month control, he had no symptoms and his scrotum was normal.


A 71-year old female, gravida 2, para 2 presented with abdominal distention which had worsened over the previous year. Her past medical history was appendicitis, and an appendectomy was performed in her thirties. Her past family history was unremarkable. A pelvic examination identified a very large mass in both hypochondrium which was hardly movable. Pelvic and abdominal ultrasonography showed a huge cystic mass with a solid component. Serum tumor marker levels were carcinoembryonic antigen (CEA): 2.0 ng/mL (normal < 37.0), CA19–9: 459.2 U/mL (normal < 37.0), SCC: 18.9 ng/mL (normal < 1.5), and CA125: 329.9 U/mL (normal < 35.0). Other blood examination results were unremarkable. A pelvic MRI showed a huge cystic mass with a nodular component which was enhanced and under diffused in diffusion weighted image (Fig. ). Computed tomography (CT) showed a 3 cm mass in the liver, and fluorodeoxyglucose-positron emission tomography (FDG-PET) showed FDG uptake not only in the pelvic tumor (SUVmax = 22.9) but also in the hepatic nodule (SUVmax = 13.7), thus suggesting metastases (Fig. ).
Ovarian cancer and liver metastasis was suspected on these data, and a subsequent percutaneous liver biopsy was performed. The pathology showed metastatic cells in normal hepatocytes, and a diagnosis of poorly differentiated carcinoma was made (Fig. ). Immunohistochemical staining showed that p40, p63, and hepatocytes were all negative, thus denying primary hepatocellular carcinoma. Therefore, a clinical diagnosis of ovarian cancer stage IVB with malignant transformation of the MCT was made.
A total abdominal hysterectomy, bilateral salpingo-oophorectomy and partial omentectomy was performed. The left ovary was enlarged (about 300 mm) and filled with 13,000 ml of yellowish fluid. Adhesion was not particular, and the uterus and right ovary appeared to have no remarkable changes (Fig. ). The cytology of ascites was negative. The patient’s postoperative course was uneventful.
The pathological findings revealed that most of the ovary consisted of an MCT with squamous cell epithelium, cutaneous appendage and cartilaginous tissue (Fig. ); however, some parts consisted of papillary or glandular lesion with hobnail-like atypical cells (Fig. ). We made a diagnosis of left ovarian clear cell carcinoma and malignant transformation of MCT based upon the proof of transition from simple squamous epithelium via simple glandular epithelium to papillary change with atypia (Fig. ). Histopathology showed no endometriosis in either ovary and, therefore, endometriosis-associated malignancy was denied. However, the uterus and omentum showed no signs of malignancy. The left fimbria of the fallopian tube had a lymphovascular space of invasion with calcifying carcinoma cells (Fig. ). However, the uterus and omentum showed no signs of malignancy. Immunohistochemical analysis of the left ovary indicated that AE1/AE3, CK7, PAX-8 and Napsin A were all positive, and that 34βE12 and P53 were slightly positive (Fig. ). Although CK20 and vimentin were negative, Ki-67 (MIB-1) index was 25% positive in the carcinomatous part. Moreover, PAS was positive in the stromal part, and PAS and ALB were both positive in some glandular lesions. This was classified as stage IVB (pT2aNXM1) according to the International Federation of Gynecology and Obstetrics (FIGO) 2014 classifications.
The patient subsequently underwent chemotherapy with 3 courses of paclitaxel and carboplatin. Afterward, CT scanning revealed a vanished hepatic metastasis and emerged swelling of the para-aorta lymph node. Although 3 courses of doxorubicin were administered, the para-aorta lymph node continued to enlarge. As chemotherapy was not effective due to her progressive disease, the patient began to receive the best possible supportive care. After 17 months from the operation, she passed away.


A 23-year-old man was admitted in the emergency department after a frontal car
collision. He had suffered severe blunt trauma, which included cervical
subcutaneous emphysema, bilateral pulmonary contusion, left hemothorax,
pneumomediastinum and complex fractures of both femurs. He was in hemorrhagic
shock and was immediately taken to the operatory room. After external fixation
of both femurs and reaching hemodynamic stability, he was transferred to the
Intensive Care Unit. The following morning the presence of a loud holosystolic
murmur was noted. The 12-lead electrocardiogram showed only sinus tachycardia. A
transthoracic and later a transesophageal echocardiogram (TEE) were performed
and both demonstrated a large muscular ventricular septal defect, located in the
mid anteroseptal segment with signs of dissection through the basal septum
(). It measured 19 mm on the
left ventricular (LV) side and 7 mm on the right ventricular (RV) side. The peak
left to right shunt gradient was estimated in 84 mmHg and the Qp/Qs ratio was
estimated in 1.8/1.0. Cardiac catheterization showed limited hemodynamic
repercussion (systolic pulmonary artery pressure of 35 mmHg and a Qp/Qs ratio of
1.9/1.0) and the patient remained clinically stable, so a conservative strategy
was decided at that time to allow the edges to heal and create a more delimited
defect.
He was released after recovering from orthopedic surgery.
Three months later the patient was reevaluated and remained asymptomatic. He
repeated cardiac catheterization, which showed a Qp/Qs ratio of 2.95/1.0.
Because the shunt increased significantly, it was decided to close the defect
percutaneously.
The procedure was done under general anesthesia and guided by transesophageal
echocardiography. Cardiac catheterization was performed using the right femoral
artery (6-Fr sheath) and vein (7-Fr sheath) and unfractionated heparin was
administered. Angiogram of the LV confirmed a VSD with an oblique entry from the
LV into the right ventricular outflow tract. The VSD was crossed using a
retrograde arterial approach with a floppy guidewire, which was advanced into
the pulmonary artery. The guidewire then snared and brought out the femoral
venous sheath. This created an arteriovenous loop to allow the delivery of the
closure device. A NuMed sizing balloon catheter was subsequently utilized to
measure the defect, but it was not possible to maintain it steady. Therefore,
the echocardiographic calculations were used to choose the device size. An 8-mm
Amplatzer septal occluder was first selected and loaded into the sheath. The
device was advanced across the VSD, but prolapsed back to the RV when it was
being released. After this failed attempt, a slightly different approach was
used. The VSD was crossed once more using the guidewire, this time in the
opposite direction into the right subclavian artery. Once again, it was snared
to make an arteriovenous loop, but on this occasion pulled out through the
femoral arterial sheath. For this second attempt, it was decided to employ a
10-mm Amplatzer septal occluder. The device was advanced through the venous
sheath and this time was successfully placed (). LV angiogram after the procedure revealed a mild residual
shunt and the Qp/Qs ratio reduced to 1.53/1.0.
A transesophageal echocardiography was repeated a month after the procedure,
which showed the device well adapted to the defect. Nevertheless, a residual
shunt remained in the superior border of the device with a peak gradient
estimated in 90 mmHg ().
Another complication of this procedure was the appearance of transient
self-limited hemolysis. Initial blood analysis showed a LDH value > 2000 UI/L
and haptoglobin < 6 mg/dL. The condition remained stable and resolved without
the need of blood transfusions.
The patient continued to be asymptomatic and has returned to his previous
professional life.


A seven and half year-old boy visited the outpatient clinic of Pediatric Dentistry Department, Faculty of Dentistry, Cairo University in June 2015 with a chief complaint of pain on the lower right molar area. The patient’s mother stated that the pain was at times throbbing in nature, and child is not able to chew on this side.
Clinical examination showed a badly decayed, lower second primary molar with related localized intraoral abscess, where the lower first primary molar was intact. The patient had poor oral hygiene; he had not received any professional dental care, and was very apprehensive.
Radiographic examination revealed root resorption and bone rarefaction related to lower second primary molar. The interesting finding was a considerable amount of root resorption of the distal root of the adjacent lower first primary molar (
).
The case was managed by performing pulpectomy
 to the lower second primary molar, with root canals filled with calcium hydroxide paste with iodoform (Metapex, Meta Biomed, Republic of Korea). The tooth was then restored with high viscosity glass ionomer (GC Fuji IX GP capsule, GC corporation, Tokyo, Japan) (
). The lower first primary molar was not touched and instead monitored. No antibiotics or analgesics was prescribed.
Unfortunately, the patient’s mother did not want follow-up appointments in person, however, she was contacted on the phone, after 2 weeks, 3 months and 6 months, and she said everything was fine and there was no swelling or pain.
At about 8 months from the treatment appointment, the patient’s mother visited the outpatient clinic with the patient for other reasons, and decided to pass by the Pediatric Dentistry Department for patient follow-up. Clinical examination showed no signs or symptoms, occlusal restoration was intact, and radiographic examination revealed arrested root resorption, on both molars, and an increase in the density of bone although this was not at a normal level yet (
).
shows the patient’s timeline of symptoms, treatment and follow-up.


A 49-year-old male patient was referred to our clinic with a prediagnosis of retinal vascular occlusion. In his medical history, he reported developing low vision after a traffic accident in childhood, having laser treatment in both eyes at 12 years of age and again 1 month earlier, and undergoing bilateral laser-assisted in situ keratomileusis in 2002. His medical and family histories were otherwise unremarkable. Following fundus examination and FFA, he was questioned again about his birth and he stated that he had been born full term by normal delivery. On ophthalmologic examination, his visual acuity was 0.4 in the right eye and 0.3 in the right eye, with mild cortical lens opacities. Fundus photography showed straightening of the temporal retinal vascular arcades and temporal dragging of the macula in both eyes ().
Previously applied laser spots with corresponding preretinal fibrosis were observed in the temporal periphery. Although the nonperfused areas in the temporal retina had been partially laser treated, FFA revealed leakage due to persistent retinal NVE in the right eye ().
Optical coherence tomography revealed disrupted macular contour associated with epiretinal membrane in the right macula and minimal perifoveal thinning in the left macula ().
Suspecting FEVR, the patient’s family members were invited for ophthalmologic examination. The patient’s father had normal ocular findings, while his brother showed straightening of the temporal vascular arcades in both eyes and excessive vascular branching and nonperfusing cord vessels in the peripheral vasculature, as well as temporal avascular areas ().
His brother was also not born prematurely. FFA was not performed for his brother because he did not return for the procedure. FFA in the patient’s father was within normal limits. Based on the brother’s findings and the revised Pendergast and Trese classification, the patient was diagnosed with stage 2A FEVR and his brother was diagnosed with stage 1A FEVR (). The patient’s other family members did not appear for examination. Additional laser therapy to the nonperfused areas was recommended due to persistent NVE. When obtaining his family history, it was learned that his parents were second-degree cousins.


Patient: A 76-year-old woman.
Chief complaint: To improve asthma management before undergoing neck surgery.
Past medical history: Diabetes, hypertension, pollen allergy.
Tobacco use: No.
Current medication (antiasthmatic drugs): Symbicort® Turbuhaler® (Symbicort), 2 inhalations twice daily up to a total of 8 inhalations per day (SMART); montelukast tablets 10 mg/day; theophylline sustained-release tablets 400 mg/day; ketotifen capsules 2 mg/day; salbutamol inhalation 0.5%, 0.5 mL/asthma attack; and prednisolone tablets 5 mg, to be taken at the patient’s discretion at the time of an attack.
Respiratory function test: Forced vital capacity, 2.74 L (129.2%); forced expiratory volume for 1 s (FEV1), 1.09 L (76.8%); FEV1, 39.8%, with findings of obstructive ventilatory defect.
Current medical history: Adult-onset asthma. Despite undergoing Step 4–5 (Global Initiative for Asthma 2017 [GINA2017]) therapy as a long-term management approach, wheezing persisted, and she had been admitted to a nearby hospital once every 2 years or so due to asthma attacks triggered by irregular weather conditions.
She was referred by a nearby otolaryngologic clinic to the Department of Otorhinolaryngology in our hospital for thorough examination of hoarseness lasting for 1–2 years. Examination findings revealed thyroid cancer with tracheal infiltration for which radical surgery was indicated. However, because wheezing increases surgical risk, she was referred to the Department of Internal Medicine for preoperative control of intractable wheezing. Consistent with her complaint of constant wheezing, initial examination revealed expiratory wheezing at rest.
To improve asthma management during a 4-week period before surgery, we prescribed Spiriva® Respimat® (Spiriva Respimat) 2.5 μg (two inhalations once daily) and subcutaneous injections of 300 mg anti-IgE antibody omalizumab every 2 weeks (determined based on the level of serum IgE [159 IU/mL IgE specific to Aspergillus fumigatus] and body weight [50.9 kg]).
Owing to these additions, wheezing disappeared after 1 week, and so the patient was able to undergo right thyroid lobectomy with tracheoesophageal resection as scheduled.
Considering that it would be difficult for the patient to use the inhalation powder Symbicort because of a permanent tracheostomy postoperatively, we switched from Symbicort to nebulized budesonide 2000 μg/day and a tulobuterol patch (2 mg). Spiriva Respimat and omalizumab were continued after surgery.
Approximately 3 weeks after surgery, the patient began to develop respiratory distress and wheezing early in the morning. According to the patient, these attacks would have been managed quickly as small attacks by additional inhalations of Symbicort, suggesting that the nebulized SABA that she had been taking postoperatively was not sufficiently effective. Because these symptoms used to be well managed when Symbicort was used, we considered switching back to Symbicort from nebulized budesonide and the tulobuterol patch. In Japan, however, Symbicort is available in only a DPI formulation, Turbuhaler®. In order to maximize the beneficial effects of Symbicort Turbuhaler, inspiratory flow rates need to exceed a certain level, but our patient would likely have had insufficient inspiratory flow rate due to the tracheostomy, and would thus need a device that does not require a high inspiratory flow rate, such as a pressurized metered-dose inhaler (pMDI). Adoair® and Flutiform® aerosols are commercially available pMDIs in Japan and contain ICS/LABA. Flutiform® aerosol was considered the most likely candidate because it contains the same LABA (formoterol), but not ICS, as in Symbicort, but Flutiform may leak out from the tracheostomy after being sprayed intraorally. Because the patient had previously been undergoing SMART with additional inhalations of Symbicort for acute exacerbations, it would be best to use Symbicort if possible. There seems to be no difference in the degree of distribution to the respiratory tract whether a tracheostomy is present or not if the patient is able to close the tracheostomy using his or her hand. However, the drug distribution in the respiratory tract is unknown when a patient with a tracheostomy uses an inhaled drug. Since the diameter of the tracheostomy is about 1 to 2 cm, the dead space can potentially be ignored. When the effect was found to be inadequate, we considered a change to Flutiform®, which has similar components in a different device (pMDI), because the tracheostomy was just under the skin in this patient.
Two types of devices are used to measure inspiratory flow rate: the whistle-type Turbutester (accessory for the Turbuhaler®) and the In-Check Dial®, an inspiratory flow measurement device. The Turbutester generates an audible whistling sound during inhalation, signifying that the patient has an adequate inspiratory flow rate to use Symbicort. The In-Check Dial, when used with an adapter tailored to conventional inhalers, functions as an inspiratory flow measurement device and expresses inspiratory flow rates in L/min. Our patient had an inspiratory flow rate barely sufficient to generate a whistling sound from the Turbutester during inhalation while simultaneously closing the tracheostomy with her hands. So, to select the best inspiratory flow rate, she was instructed to measure the rate three times using the In-Check Dial®, the Turbuhaler®, and the special adaptor while closing tracheostomy. The results were 40, 43, and 43 L/min. Several inspiratory flow rates have been suggested to be required to exert the beneficial effect of Turbuhaler®, but the consensus is that inspiratory flow rates above 30 L/min are sufficient to use Turbuhaler® [, ]. These findings, together with the findings from the Turbutester, suggest that the present patient could benefit from these devices.
Based on these findings, nebulized budesonide 2000 μg and tulobuterol patch 2 mg were switched to 2 inhalations of Symbicort twice daily up to a total of 8 inhalations/day (SMART). The antiasthmatic drugs Spiriva Respimat and anti-IgE antibody, which had been added before surgery, were continued. The patient’s symptoms improved quickly over the course of 2 days after starting 2 inhalations of Symbicort in the morning and evening plus additional inhalations as needed (usually 1 inhalation in the evening). Owing to stabilized asthma symptoms, she was discharged on the third day after switching drugs (Fig. ).


The patient is an 84-year-old woman with a long-standing history of mild leukopenia attributed to possible myelodysplastic syndrome, who presented with marked right leg swelling and hypercalcemia. Positron emission tomography (PET) scan showed FDG-avid extensive axillary, left hilar, pelvic and inguinal lymphadenopathy. She was admitted and a lymph node biopsy from the left inguinal region was performed.


This case describes a 29-year-old woman who was first diagnosed with ameloblastoma as a child at 7 years old. The lesion originated in the ascending branch of the left mandible, and the first surgical procedure was performed in March 1997 followed by disease recurrence in April 1999. A second resection was performed in May 1999, and during the next 16 years, the patient underwent several surgical approaches that were consistently followed by disease recurrence. Some of the procedures were conservative surgeries, but others were radical procedures that left her with several deforming scars. She presented to our clinic in January 2015 with a new magnetic resonance imaging (MRI) that evidenced a right, triangular aspect, paracellarlesion, extending to the homolateral cavernous sinus (13 × 9 mm), which was suspected to be a residual lesion that would have achieved the cavernous sinus by contiguity growth after several surgeries. Her last surgery had been performed in April 2014 and was followed by local radiotherapy in May 2014. She was asymptomatic and not willing to undergo a new invasive procedure. She decided to be followed without further intervention.
For the next 18 months, she was clinically stable and asymptomatic, but she returned in July 2016 with intense pain on the right side of her face that required multiple hospital visits for intravenous analgesia. MRI revealed an extensive heterogeneous lesion with contrast enhancement centered on the right cavernous sinus anterior to the cavus of Meckel and exhibiting anterior extension towards the upper orbital fissure (measuring approximately 19 × 15 × 16 mm). To identify new treatment possibilities, we decided to perform a new biopsy and conduct molecular testing (Fig. ). A BRAF mutational analysis by the allele-specific protein chain reaction (PCR) certified test revealed the presence of a BRAF c.1799 T > A;p.V600E mutation corresponding to a V600E amino acid substitution. After tumor board discussion and a careful conversation with the patient, she decided to undergo BRAF inhibitor therapy.
A treatment regimen with vemurafenib 960 mg PO twice daily was started on October 4, 2016. Prior to the initiation of therapy, a new MRI performed on September 24 revealed a lesion measuring 24 × 21 × 19 mm. After 2 weeks of therapy, the patient was asymptomatic and was not using any analgesic medication. During the course of therapy, she experienced grade one anorexia, nausea and fatigue, without any severe therapy-related adverse events. MRI performed in April 2017 revealed stable disease (24 × 18 × 15 mm), and her last MRI performed in September 2017 evidenced a reduction of the lesion size (18 × 13 × 14 mm) (Fig. ). The patient currently remains asymptomatic with excellent tolerance to the medication.


A 56-year-old woman who had autosomal dominant RP with a heterozygous PRPH2 mutation (c.410G > A) complained of metamorphopsia in her left eye. Her best corrected visual acuity (BCVA) had declined from 1.0 (20/20) to 0.4 (20/50). Further examination revealed CNV with serous retinal detachment (Fig. ). She was treated with as-needed injections for 2 years; however, she experienced a recurrence during which her vision deteriorated to 0.2 (20/100). Therefore, we switched to a bimonthly regimen that continued for 6 years. No recurrence was noted during that time, and her left visual acuity remained 0.2 (20/100). In total, the patient received 34 anti-VEGF injections in 8 years (bevacizumab × 2, pegaptanib × 2, ranibizumab × 11, aflibercept × 19, in that order).
The patient’s central visual field was assessed using the mean deviation (MD) value on a Humphrey field analyser with a 10–2 SITA standard program (Carl Zeiss Meditec, Inc., Dublin, CA). The MD values decreased similarly in both eyes (Fig. ). The slope of the MD values during the 8-year treatment period was − 0.68 dB/year in the right eye (without CNV) and − 0.32 dB/year in the left eye (with CNV). Although her peripheral visual field loss was noted to have progressed based on Goldmann perimetry tests, her visual field in the left eye was preserved even after 8 years (Fig. ). No serious adverse events were observed during treatment.


A 33-month-old Caucasian boy presented with leukocoria right eye (OD). Fundus examination OD revealed a solid white predominantly endophytic retinal tumor filling most of the nasal aspect of the fundus (Fig. & ) and extensive vitreous seeding (Fig. ). The tumor extended to the posterior surface of the lens and exhibited preretinal neovascularization on its surface. B-scan ocular ultrasonography OD revealed dense intralesional particles consistent with calcific foci. Genetic testing demonstrated a homozygous non-germline RB1 nonsense mutation. Fundus examination of left eye (OS) revealed a broad-based flat melanotic area of the choroid extending from the subfoveal region to the ora serrata temporally (Fig. ). B-scan ocular ultrasonography OS showed no appreciable choroidal thickening corresponding to the melanotic patch. Anterior segment evaluation OS showed no iris or scleral melanocytosis. Our diagnoses were unilateral nonfamilial retinoblastoma OD and isolated choroidal melanocytosis1 OS. The child was treated by primary enucleation of the retinoblastoma-containing OD. Histopathologic evaluation confirmed the clinical diagnosis of retinoblastoma. The child has been followed for more than 4 years post-enucleation. Follow-up examinations of the fundus OS have shown no change in the patch of choroidal melanocytosis.


A 49-year-old female presented with a 2-month history of asymptomatic lesions on the left knee found incidentally on routine full skin examination. The patient was otherwise well, with no pulmonary or systemic symptoms.
She had a past history of breast cancer diagnosed 4 years ago, managed by lumpectomy and adjuvant chemoradiotherapy achieving remission. The patient had regular cancer surveillance and was currently on adjuvant tamoxifen, with a planned duration of 10 years. Her other notable medical history included lifelong asthma, gastrooesophageal reflux disease, depression, subacute thyroiditis and previous shoulder, and knee arthroscopies. Her regular medications included tamoxifen, pantoprazole, venlafaxine, budesonide/formoterol, and terbutaline. She was a lifetime non-smoker and rarely consumed alcohol. The patient had no family history of autoimmune conditions.
Examination revealed numerous erythematous-to-brown, non-tender papules occurring on the anterior left knee (). On the right foot, at the site of a scar from prior cryotherapy for plantar warts, the patient had a similar area of firm indurated erythematous-to-brown change. Dermoscopy of both sites showed orange and yellow translucent globules (“apple-jelly” sign). There were no skin lesions detected on full skin examination suspicious for malignancy. There was no lymphadenopathy and systemic examination was otherwise unremarkable.
Skin biopsy showed multiple, variably sized naked sarcoidosis type granulomas scattered throughout the dermis (). Chest radiograph showed bilateral hilar lymphadenopathy and serum angiotensin-converting enzyme was elevated at 107 U/L. Other laboratory tests were within normal limits (full blood count, liver and renal function tests, and calcium and inflammatory markers). Further investigations excluded systemic sarcoidosis (cardiac MRI and CT-PET scan). The CT PET ordered during systemic work-up, however, showed a solitary lesion in the T10 vertebra and subsequent biopsy proved recurrent metastatic breast cancer.
The patient's management was then deferred to a medical oncologist for ongoing care of her metastatic breast cancer. She received stereotactic radiation to her spinal lesion and was commenced on a special access program with ribociclib. Following breast cancer treatment, cutaneous sarcoidal lesions completely resolved.


A 22-year-old patient, Para 1, Gravida 2, presented to our Emergency Department of Gynecology and Obstetrics, at 35 weeks of gestation for acute onset of abdominal pain and uterine contraction. It was learned that the patient's history had no follow-up hypertriglyceridemia. On physical exam, her heart rate was at 100 pulses per minute, and her blood pressure was at 110/70 mm-Hg, respiratory rate 18 /min. Her abdomen was defensive. Her cervical os was dilated to 1-2 cm and minimal bleeding. The patient had mild epigastric tenderness. Decelerations were seen in pregnant cardiotocography follow-ups with abnormal abdominal pain and uterine contractions continued and simultaneous wide bleeding area (like abruptio placenta) was seen on the posterior part of placenta in ultrasound. Immediate cesarean section was performed under general anesthesia because of contraction of the tetanic type in the manual contraception. She gave birth to a healthy infant of 2980 g. Amylase, lipase, triglyceride, HDL, and LDL were studied in the patient's blood after emulsion of chylous fluid from abdomen during the cesarean section. Liver enzymes were high: ast: 241, sub. 147. It was observed that blood sample revealed a milky turbid serum. Laboratory finding included a triglyceride at 3297 mg/dl and amylase 827 U/L, lipase 1576 U/L. Abdominal ultrasound showed thickened pancreas without necrosis; acute pancreatitis compatible with diffuse edema was observed on pancreas. Biliary tract was naturally observed. Other causes of cholestasis of pregnancy, such as cholangitis, acute hepatitis, and hemophagocytic syndrome, were ruled out. Oral intake of the patient was stopped; intravenous fluid replacement therapy, antibiotherapy, proton pump inhibitor, insulin, and heparin therapy were started. She was discharged on the 10th day of treatment. Even though the patient did not have previous history of diabetes or gestational diabetes, the baby was born 4 to 3 weeks earlier. It was thought that this condition might be related to maternal hyperlipidemia for newborn's doctors.


A 40-year-old man with a history of end-stage renal disease on hemodialysis, hypertensive cardiomyopathy, and poorly controlled hypertension presented to the emergency department with a sudden onset of 48-hour right visual blurring and headache. On physical examination, he was alert, oriented to time, place and person, with a blood pressure of 200/124 mmHg, and a heart rate of 88 beats/minute. Neurologic examination was only significant for decreased right visual acuity. The electrocardiogram revealed normal sinus rhythm.
Computed tomography of the head revealed focal area of hypoattenuation in the left cerebellar hemisphere (Figure ).
Magnetic resonance imaging of the brain revealed multiple new regions of restricted diffusion within the left frontal, parietal and occipital lobes, consistent with an embolic stroke (Figure ).
A carotid duplex ultrasound was unremarkable for carotid artery stenosis. A two-dimensional transthoracic echocardiography revealed a large calcified mass measuring 24.5 mm x 16.0 mm (Figure , asterisks; Video ).
A three-dimensional transesophageal echocardiogram of the mitral valve revealed two discrete nonmobile calcified masses, with central areas of echolucency consistent with CCMA (Figure , asterisks; Video ).
As no other potential sources of embolism were identified, CCMA lesion was postulated as the possible source of embolism. Dual antiplatelet therapy (aspirin 81 mg daily and clopidogrel 75 mg daily) and high-intensity atorvastatin 80 mg daily were initiated. Surgical excision of the CCMA lesion was considered but not performed immediately due to the potential risk of hemorrhagic conversion of the ischemic stroke.


A 68-year-old Japanese man presented at our hospital for evaluation and treatment of rectal cancer. He had no history of rectal cancer. He had been diagnosed with rectal cancer using colonoscopy for screening of rectal bleeding; following biopsy, the tumor was confirmed to be moderately differentiated adenocarcinoma. Barium enema showed a filling defect at the rectum below the peritoneal reflection (Fig. ). Contrast-enhanced CT (CECT) revealed swollen lateral lymph nodes (Fig. ). We diagnosed him as having rectal cancer cT4aN1M0 clinical stageIIIa (UICC 8th). We decided to perform neoadjuvant chemotherapy for locally advanced rectal cancer for the purpose of the prevention of postoperative local recurrence. We performed CapeOX therapy 1 course until the RAS results were out. Since the RAS gene was confirmed as the wild-type, we changed the regimen and performed FOLFOX + cetuximab therapy 1 course to reduce the tumor size. The reason why we chose cetuximab is we can perform chemotherapy with cetuximab until just before surgery. On day 14, he visited the emergency room due to perineal pain. Physical findings showed BT 38.0 °C, HR 117/min, BP 79/57 mmHg, tenderness, redness, and swelling of the perineum and scrotum (Fig. ). He had no tenderness of the abdomen. CECT revealed extensive emphysema in the scrotum and gluteus maximus muscle (Fig. ). We diagnosed as Fournier’s gangrene caused by penetration of a rectal cancer. We performed debridement, left orchiectomy, transverse colostomy with double orifices. Abscess cultures yielded Escherichia coli. Intravenous antimicrobial treatment was administered using cefmetazole (1 g every 6 h).
After the first operation, we cleaned the wound every day. Because his right testis was in poor condition, we performed right orchiectomy under local anesthesia on postoperative day (POD) 7. Once the granulation tissue had formed (Fig. ), we performed abdominoperineal resection (APR) and dissected the left lateral lymph nodes. on POD30 (Fig. ). The amount of blood loss was 320 ml, and the operation time was 269 min. Pathological findings were type3, 35 × 30 mm, tub2, ly1, v2, ypT3N1a(263), and M0 Stage IIIa (UICC 8th). Histological grade was 1a (RECIST v1.1). The tumor left side wall was thin. Because the effect of chemotherapy was poor, we diagnosed as Fournier’s gangrene caused by penetration of rectal cancer.
After the second operation, we cleaned the wound every day. He left the hospital on POD59. On POD92, the wound was healed like that in the picture (Fig. ). Since he wanted to leave the hospital, we showed him the treatment procedure for scar healing. We performed CapeOX therapy eight courses as adjuvant chemotherapy. The patient had no recurrence for 1 year and 2 months after the operation.


An 18-year-old female was referred to our institution complaining of pain in the left mid-forearm. Previous medical history included a fracture of both radius and ulna 12 years previously, which had been successfully treated with reduction and by intramedullary pinning (). Several months before the current referral, there was an insidious onset of pain, which was gradually increasing, more intense at night and relieved by salicylates. On physical examination, there was moderate swelling at the old fracture site. There were no signs of local or systemic inflammation. Conventional radiographs of the left forearm revealed a lucent area adjacent to the previous fracture site with surrounding sclerosis and cortical thickening (). Subsequent MRI showed an oval intracortical lesion in the ulna, with central hypointensity to skeletal muscle on both T1 and T2 weighted images. After administration of gadolinium contrast, there was marked peripheral enhancement of the lesion with perilesional bone marrow and soft-tissue oedema (). Because imaging characteristics were highly suggestive of an OO, an additional CT scan was performed (). This examination showed pathognomonic features of an OO with a central calcified nidus at the site of the previous fracture.


A 29-year-old male presented with progressively worsening symptoms, including altered mental status, lower extremity weakness, nausea and vomiting for 6 months, with symptomatology extending back more than 6 years prior to this decline. He also reported headaches, dizziness, multiple falls, and right facial numbness and tingling during his 6-month decline. Previously, he had had seizures that were controlled with medication. Prior to referral to neurosurgery, his presumptive diagnosis was hydrocephalus, for which he had a ventriculoperitoneal shunt placed. Neurological examination was significant for mild dysarthria, a non-reactive left pupil, nystagmus on primary gaze of the left eye, bilateral nystagmus on horizontal gaze and mild action tremors in both hands.
Initial pre- and post-contrast-enhanced CT scans demonstrated diffuse enhancement of the basimeninges as well as heterogeneously enhancing cystic and solid lesions in the right temporal lobe and left cerebellum. Subsequent contrast-enhanced T1 weighted MRI of the brain demonstrated extensive post-contrast nodular enhancement of the basimeninges associated with an approximately 5 × 4 cm solid and cystic mass in the right temporal lobe (). An additional elongated enhancing mass was seen on the right side of the prepontine cistern measuring 2.3 × 1.1 cm in size, with an associated cystic component that extended towards the midline from this mass (). The left cerebellum was also involved, with a solid, enhancing mass that crossed the midline. On T2 weighted imaging, the cystic components of these lesions demonstrated extremely high signal intensity () that was suppressed on fluid-attenuated inversion-recovery (FLAIR) imaging, while T1 weighted imaging demonstrated hypointense signal.
Initial laboratory findings were significant only for an elevated erythrocyte sedimentation rate and C-reactive protein. CT of the chest was performed, demonstrating no evidence of pulmonary sarcoidosis, and subsequent biopsies of the right temporal lobe and dura were negative for neoplasm or any other abnormality. In addition, analyses of aspirated cyst contents were negative for inflammation or malignancy. A presumptive diagnosis of neurosarcoidosis was considered and the patient was started on prednisone. He had symptomatic improvement with the prednisone and was discharged following recovery from the biopsy.
Follow-up MRI 11 months later demonstrated progression of his disease with an interval increase in size of the intracranial lesions. Views of the spinal axis demonstrated diffuse nodular meningeal enhancement (). The patient underwent a repeat ultrasound-guided brain biopsy. This time, histopathological analysis of the biopsy specimen showed an astrocytoma with protoplasmic features (). Immunohistochemistry for Ki-67 (a cell proliferation marker) showed a labelling rate of approximately 5%, which was somewhat higher than the 0–2% commonly seen in low-grade tumours, suggestive of more aggressive behaviour ().
Owing to the multicentric nature of the tumour, craniospinal radiation therapy was recommended instead of radical surgical resection. After multiple cycles of radiation therapy with cumulative doses totalling 1800 cGy to the brain and 1620 cGy to the spine, the patient underwent repeat MRI. The studies showed a slight interval increase in size of the brain lesions but decrease in nodular enhancement of the spinal meninges. A whole body PET-CT was performed to assess disease status and demonstrated a standard uptake value of 13 in the right temporal lesion and 12 in the left cerebellar lesion, which was consistent with a highly metabolically active tumour. No definite increase in uptake was seen in the spinal region or elsewhere.
Following treatment, the patient continued to take dexamethasone for symptomatic relief. He reported a much improved condition despite mild difficulty ambulating, short-term memory loss, occasional euphoria and swollen tongue. However, his disease progressed () and did not respond to further medical or surgical management, and he ultimately passed away.


A 55-year-old female with no significant past medical history initially presented in 2006 with vague abdominal pain. Her past social history was negative for alcoholism or smoking. Work up of the patient including physical examination and laboratory indices were all normal. A multi-detector CT of the abdomen and pelvis utilizing a pancreatic mass protocol (arterial, portal venous and delayed phases) was performed which revealed a diffusely enlarged pancreas with extensive parenchymal calcifications (). No discrete mass was identified in the pancreas. Minimal pancreatic ductal dilatation was noted on the CT examination. The patient was diagnosed with chronic pancreatitis and followed clinically for several years. In 2015, the patient presented with recurrent abdominal pain. A CT of the abdomen and pelvis with pancreatic mass protocol was repeated (), which revealed new hypoattenuating masses in the pancreatic neck and tail. Some of the smaller tumours were hypervascular on the arterial phase imaging. There was redemonstration of extensive parenchymal calcifications. No pancreatic atrophy was identified. The pancreatic duct remained minimally dilated. Given the new masses many of which were arterially enhancing, the possibility of neuroendocrine tumour was raised. A MRI of the abdomen with pancreatic mass protocol (T2 with fat saturation, MRCP, in and out of phase T1 and unenhanced T1/arterial/portal/ 5 min delayed post-contrast T1) was performed (). The MRI confirmed multiple well-circumscribed masses throughout the pancreas, many of which had increased T2 signal with cystic change. The pancreatic duct was at most mildly prominent, and no lesions were identified outside of the pancreas. The largest pancreatic mass in the tail measured approximately 3.5 cm, with peripheral enhancement and central hypointense signal (). An endoscopic ultrasound was performed, which confirmed a hypoechoic mass in the tail of the pancreas, two isoechoic masses in the head of the pancreas, and diffuse parenchymal calcifications suggestive of chronic pancreatitis. Fine needle aspiration of the pancreatic masses in the head was performed, with cytology returning as concerning for neuroendocrine tumour. Given the suspicion for pancreatic neuroendocrine tumour, an indium-111 Octreotide scan was requested for further characterization. Fused SPECT-CT imaging was also performed for improved uptake localization (). The indium-111 scan revealed diffuse intense uptake of radiotracer throughout the entire pancreas. No extrapancreatic foci of uptake was identified. On the grounds of the clinical and imaging findings, it was decided the best course of action would be to perform a pylorus-preserving pancreaticoduodenectomy with total resection of the pancreas, splenectomy and cholecystectomy. Sectioning of the pancreas revealed numerous well-circumscribed, solid and tumoural masses ranging from minute up to the largest grossly identified lesion measuring 3.5 cm in diameter (). Many of the nodules were coalescing with only a scant amount of intervening normal pancreatic parenchyma present. The cut surfaces of the nodular masses were solid and showed a variegated pink to orange-red colour. No gross areas of necrosis were identified. Numerous representative histologic sections of the nodular masses were examined. The nodules were comprised of numerous insular nests and trabecular cords of fairly uniform epithelioid neoplastic cells with oval nuclei and speckled chromatin. Many of the nodules showed numerous calcifications and localized amyloid deposition (). Immunohistochemical stains were performed and the neoplastic cells marked strongly for the neuroendocrine markers chromogranin A and synaptophysin (). Multiple immunostains for pancreatic peptides were performed. The neoplastic cells were positive for pancreatic polypeptide and negative for insulin, glucagon and somatostatin. Only a rare mitotic figure was identified but the Ki-67 mitotic index marker was calculated at 5% as measured by the Aperio image analysis system. The findings were consistent with numerous neuroendocrine tumours of the pancreas, Grade II, as per the 2010 WHO criteria for neuroendocrine tumours of the pancreas. The neuroendocrine neoplastic nodules were all confined within the pancreatic parenchyma and all pancreatic resection margins were free of neoplasia. All regional lymph nodes sampled were negative for metastatic disease.


A 48-year-old healthy male presented with a hemoglobin level of 21 mg/dl and an elevated hematocrit (63%). The patient had an increased hematocrit (64%) 12 years ago, leading to clinical suspicion of polycythemia, although the patient was quickly lost to follow-up. At that time, JAK2 mutational testing was negative. At the current presentation, the patient reported fatigue, headache, blurred vision, and excessive sweating. He declined both a history of living at high altitude and smoking. Exogenous EPO use was also excluded. Past medical history includes atrioventricular block requiring pacemaker insertion, hypertension controlled with lisinopril, mild depression managed with citalopram, and erectile dysfunction treated with sildenafil. On physical examination, the patient's vital signs were as follows: blood pressure: 140/100 mmHg; heart rate: 97; respiratory rate: 19 (with excessive redness in the face); BMI: 25.85 kg/m2; SpO2: 100%.
WBC: 3.6 x 109/L HGB: 19.4 g/dL Hct: 63 % MCV: 85 fl/cell MCH: 26 pg MCHC: 31 g/dL RDW: 25.7 % PLT: 132 x 109/L Creatinine: 1.2 mg/dl (normal range: 0.6-1.2) Carboxyhemoglobin: 1.5% (normal range: 0-1.4%) EPO: 687 mU/ml (normal range: < 29.5)
CT scans of the chest and abdomen showed no evidence of malignancy, while MRI of the head was negative. Subsequent genetic/biochemical testing ruled out congenital polycythemias.
Due to the polycythemia having no obvious etiology, a bone marrow aspiration and biopsy were performed (Figures and ). The core biopsy showing a markedly decreased myeloid: erythroid ratio with CD71-positive erythroid precursors comprising ~80% of marrow cellularity () and MPO-positive myeloid cells comprising < 10% of cells (). Surprisingly, the touch prep revealed increased plasma cells (16%, ), and CD138 immunohistochemical staining highlighted the increased plasma cells arranged in clusters in the core biopsy (~15% of cellularity, ). Flow cytometry demonstrated the bone marrow aspirate contained a monotypic lambda-restricted CD38/CD138-positive plasma cell population (). Karyotype analysis of the aspirate revealed a normal male 46,XY karyotype, while FISH revealed an IGH/CCND1 rearrangement (t(11;14)), solidifying a diagnosis of plasma cell myeloma. Subsequent serum studies showed increased serum lambda light chains (41.58 mg/dl; normal range: 0.57-2.63 mg/dl) and a kappa: lambda ratio of 0.02. Immunofixation revealed an IgD lambda paraprotein that was too small to quantitate.
The patient was phlebotomized until Hct dropped below 50, which led to the resolution of symptoms. The patient has not been treated for the smouldering PCM.


A previously healthy 14 year-old boy presented to the Emergency Department (ED) for evaluation of right eye injury that was sustained one to two hours prior to arrival. According to the patient, he was standing on a street corner on his way to school when he was struck in the right eye with a rock thrown from a passing car. He reported no loss of consciousness and no other injury. He denied nausea or vomiting. His past medical history and family history were not significant. The patient did not have any prior ocular injuries, history of ocular disease, or prior ocular surgeries. The patient reported that he does not wear glasses.
In the ED, he was alert, awake, answering questions, and following commands with a GCS of 15. His only complaint was pain to the right eye. His vital signs were as follows: temperature 98°F, heart rate 79 beats/minute, respiratory rate 18 breaths/minute, blood pressure 151/73 mmHg, and oxygen saturation 99% on room air. On examination, blood was oozing and pooling in his right eye with eye lid swelling and surrounding abrasions ().
The patient expressed severe photophobia in his right eye and was unable to open it. Detailed examination of the right eye could not be performed due to the possibility of right ocular globe rupture. A metal eye shield was placed on the patient's right eye for protection. His left eye refractory status was normal with a visual acuity of 20/20. The patient had a negative battle sign, no hemotympanum, no rhinorrhea, and no cervical spine tenderness. The remainder of his examination was noncontributory.
A non-enhanced CT scan of the orbits was obtained and revealed rupture of the right globe with associated orbital and periorbital swelling with intraconal hematoma ().
An ophthalmology consultation was obtained. He was then taken to the operating room where a 20 mm linear laceration was noted crossing the iris and sclera with uvea protruding. The laceration was repaired. The patient tolerated the procedure well and was followed-up in the clinic on the next day. The patient received an explanation regarding the poor prognosis of his right eye vision. The open globe injury caused blindness in his right eye. The patient was advised to wear protective eye gear to prevent future eye injuries while participating in activities that carry a risk of eye injury.


A 26 years old primigravida unbooked case belonging to lower socioeconomic status, presented in antenatal clinic with 23 weeks of pregnancy with distension of abdomen. She had past history of left salpingo-oopherectomy done two years back for left ovarian mucinous adenocarcinoma. Postoperatively, she had received six cycles of combined chemotherapy with carboplatin and paclitaxel and was advised contraception. Subsequently she was lost to follow up until 23 weeks of period of gestation. Per abdomen examination revealed ascites and 24 weeks size uterus. with palpable fetal parts.CA-125 was 80 IU/ml. Ultrasound examination revealed intra-uterine pregnancy of23 weeks anda right ovarian mass of 12.8 x8.8 cm with solid and cystic areas containing free fluid with internal echoes. Diagnosis of recurrent ovarian cancer in pregnancy was made and she was advised surgical management which was declined by the patient. Hence two courses of chemotherapy with single dose carboplatin 450mg i.v. was started at three weekly intervals. However, third course of chemotherapy could not be administered due to derange liver functions tests. In spite of chemotherapy, ascites was progressive, leading to maternal respiratory distress and fetal growth restriction and oligohydroamnios ensued. Hence, elective Cesarean section with staging laparotomy was planned at 34 weeks for breech presentation with decreased liquor and gross ascites, after completion of steroid cover. Intraoperatively, 20litersof hemorrhagic ascetic fluid was drained, omentum was agglutinated over intestine and deposits were present over uterus. Right ovary was replaced by a tumor of 15x15cm with rupture and hemorrhage ().
A live healthy baby girl 2.3kg with APGAR 6/10 and 8/10 at 1 and 5minute respectively was delivered. The placenta appeared normal at the time of delivery. Total abdominal hysterectomy with right salpingo-oopherectomy, infracolic omentectomy, and appendectomy was done. Peritoneal washings were collected and multiple peritoneal biopsies were taken. Palpation of pelvic and para-aortic lymph nodes was negative. The patient had an unremarkable postoperative course. Histopathology revealed mucinous adenocarcinoma of ovary. She was given six cycles of chemotherapy (carboplatin and paclitaxel) commencing from postoperative day 14.At 6 month follow up, mother and baby are healthy.


We report a 24-year-old Caucasian woman who presented to the hematology clinic with a history of easy nontraumatic bruising on both thighs and legs. She had a recent history of fall, with bruises on the left hip, bilateral arms, and the lower left quadrant of the abdomen. Her family history was negative for easy bruisability, bleeding, or clotting disorder. She denied any bleeding gums, heavy menstrual blood flow, nasal bleeds, blood in stool, or blood in urine. She also had an unexplained loss of appetite and loss of weight over the last six months. Her medication history included inhaled albuterol as needed. She has not had any surgeries in the past. She smokes a pack of cigarettes per day and drinks a pint of vodka every night as well as three cans of beer per week. She is unmarried and has no children. She is sexually active with male partners. Her maternal grandmother had lung cancer.
The physical examination was unremarkable except for few bruises on bilateral thighs. The computed tomography (CT) scans of the chest, abdomen, and pelvis were ordered to screen for her unexplained weight loss which was unremarkable. The complete blood count, comprehensive metabolic panel, factor VIII, prothrombin time and partial thromboplastin time, D-Dimer, and antithrombin activity were normal. Her von Willebrand factor activity was much lower compared to the antigen (Table ). From the reports, it was established that her von Willebrand factor activity was 20% (reference range is 40%-163%) whereas the antigen was low normal at 45% (reference range is 45%-150%) and the ratio is less than 0.5 (normal ratio is more than 0.7). Repeat testing in a week consolidated the previous findings with the factor activity and the antigen as less than 19% and 37%, respectively. The reports of her von Willebrand factor multimer analysis were normal. The platelet count was normal.


A 50-year-old male presented with complaints of chronic, intermittent abdominal pain. The patient had a 20-year history of mild-to-moderate amount of alcohol consumption. There was no history of jaundice, fever or weight loss. His laboratory tests revealed no significant abnormal findings. Serum amylase and lipase, and tumour markers [CA 19-9, carcinoembryonic antigen (CEA)] were within normal limits. The patient was subjected to a CT examination. On unenhanced CT image, a poorly defined soft tissue mass was seen in the pancreaticoduodenal (PD) groove with a hypodense cystic lesion within the mass (). On post-contrast study, the lesion showed minimal enhancement in the portal venous phase ( and ), but delayed imaging at 2–3 min showed mild persistent enhancement of the lesion compared with the pancreatic parenchyma that was consistent with scar tissue () and a non-enhancing cystic lesion within the lesion. The lesion showed poorly defined fat planes with the adjacent second part of the duodenum on its right side and pancreatic head on the left side. Mild thickening of the wall of the second part of the duodenum adjacent to the lesion with variable luminal narrowing was noted. The common bile and pancreatic ducts appeared grossly normal. The pancreatic body and tail were normal. In order to clearly delineate the ductal system and the periampullary region, and to know the extension of the lesion, the patient was also subjected to an MRI examination. Axial two-dimensional fast imaging employing steady-state acquisition () sequences showed soft tissue signal intensity lesion in the PD groove with a cystic lesion within the lesion and the aforementioned CT scan findings. MR cholangiopancreatography sequence () revealed smooth and regular tapering of the pancreatic and common bile ducts. The gallbladder was distended and the cystic duct was normal. Incidentally, a few simple cysts (Bosniak 1) were noted in both the kidneys. The patient was advised further surgical intervention but he refused and was managed with conservative treatment. At present, the patient is asymptomatic.


A 62-year-old woman diagnosed with pancreatic cancer T3N0M0 Stage IIA [] underwent pylorus-preserving pancreatoduodenectomy (PPPD) + D2. The pathological findings revealed invasive ductal carcinoma of the head of the pancreas, nodular and well-moderately differentiated type with a 3.8 cm diameter, with direct cancer cell invasion of the duodenal mucosa and extra-pancreatic nerve plexuses, as well as lymph node metastasis (#13b involving five nodes). The dissected peripancreatic tissue margin was positive for pathological stage III pT3N2M0 cancer [] (Fig. a–c). The patient provided written, informed consent to undergo adjuvant treatment using gemcitabine, tegafur, and uracil (a 5-fluorouracil prodrug). However, this therapy was stopped after only 14 days as the patient developed Grade 4 neutropenia. Three months after surgery, the patient was enrolled in a clinical study of SVN-2B peptide vaccination. The vaccine at 1 mg/mL was mixed with the incomplete Freund’s adjuvant, Montanide ISA 51 (Seppic, Paris, France), emulsified, and then immediately injected subcutaneously once every 2 weeks. Human IFN-α at a dose of 3,000,000 IU (Dainippon-Sumitomo Pharmaceutical, Osaka, Japan) was also injected subcutaneously near the SVN-2B peptide injection site on days 1, 4, 8, and 11 (Fig. ) [, ]. Serum CA19-9 levels did not increase over a period of 12 years postoperatively (Fig. a).
A follow-up CT at 82 months after the surgery detected three small lesions (Fig. a–c) that were removed by thoracoscopic excisional biopsy. All of them were pathologically well-differentiated adenocarcinoma and immunohistochemically positive for CK7 and negative for TTF-1 and CK20, indicating that they were pancreatic tumor metastases (Fig. d–g). Twelve years after the primary operation, periodic computed tomography (CT) and positron emission tomography did not uncover any new local recurrences or metastases. The clinical course did not include severe adverse events, although low-grade adverse events included transient headache, nausea, fatigue, and a persistent red flare with induration at the injection site (Grade 1).


A 43-year-old female presented with a 3-month history of right-sided facial swelling associated with dry eyes, dysphagia with dry foods, fevers, and weight loss. On examination, there was right-sided facial swelling, nontender. Serology for tuberculosis, human immunodeficiency virus, antinuclear, anti-Ro, and anti-La antibodies was all negative. A chest radiograph showed bilateral hilar adenopathy. MRI (Figure ) revealed asymmetric right parotid enlargement involving both superficial and deep lobes. The parotid tail was excised, demonstrating non-necrotizing granulomas (Figure ). Closer examination (Figure ) showed a foreign body giant cell with Schaumann body present. These findings were consistent with sarcoidosis, presenting as Heerfordt syndrome.
Sarcoidosis is a granulomatous disorder of unknown etiology and one of the “great masqueraders” of medicine. Although most patients have thoracic involvement, more than half do not have respiratory symptoms at presentation. Up to 30 percent of patients present with extrathoracic manifestations. Heerfordt syndrome was first described by the Danish ophthalmologist Christian Heerfordt in 1909, and the association with sarcoidosis was made by the Swedish internist Jan Waldenstro¨m in 1937. Due to its rarity, the exact prevalence is unknown and physicians should include the disease in the differential of parotid swelling. As in this case, chest radiography is helpful and definitive diagnosis can be made by histopathology.


We report a case of 38-year-old Greek woman who presented to our Emergency Department complaining of abdominal pain during the last week without any other symptoms. Her clinical history was clear and she had not noticed the occurrence of the same symptoms before. A clinical examination revealed only focal tenderness in the left part of her abdomen. Laboratory results were within normal limits. During an ultrasound examination of her abdomen, an anechoic lesion in her upper left abdomen was revealed. In a further investigation with computed tomography (CT), a well-defined hypodense cystic 7.08 × 6.05 cm mass with mild enhancement was noted (Fig. ). Surgical approach was decided after a thorough examination and our patient gave her consent for surgery. A cystic lesion sized 7.08 × 6.05 cm appeared between the layers of small bowel mesentery (Fig. ). The cystic lesion was excised within healthy borders and sent for further pathologic examination (Fig. ). On macroscopic examination, the cyst sized 7.08 × 6.05 cm was unilocular and contained a white, milk-like viscous fluid (chylous), which was drained out by incision. Histopathological investigation showed a thick fibrous wall, pervaded by chronic inflammatory cells (lymphocytes and plasma cells) and lymphoid aggregates. Variously sized vessels could also be observed while immunohistochemically CD31 (platelet endothelial cell adhesion molecule) was positive (Fig. ). CD31 is used primarily to demonstrate the presence of endothelial cells and can help to evaluate the degree of tumor angiogenesis. A definitive inner epithelial lining was not found. On the inner surface, multiple aggregates of foamy macrophages as well as focal foreign-body giant cells were present. (Fig. ) The thickness of the wall varied trivially with small parts of mature fat tissue toward the outer surface, indicating the mesentery. The findings were found to be most consistent with a simple lymphatic (chylous) cyst of the mesentery in combination with features of a non-pancreatic pseudocyst. Her postoperative course was uneventful and patient feeding was started on the second postoperative day. She was discharged on the fifth postoperative day while she had a low-grade fever. A 6-month follow-up with abdomen ultrasound and 1-year CT imaging showed no signs of recurrence.