The patient, a 75-year-old female, had a positive oncological anamnesis: her brother was affected by rectal neoplasm and her three aunts were affected by breast cancer, bladder cancer, and rectal cancer, respectively. The patient was under medical treatment for hypertension and due to the onset of general malaise, anemia, and asthenia, she performed a total body contrast enhanced computed tomography (CECT) scan. CECT total body scan was performed with a triphasic study of the abdomen and with a urographic phase. The scans showed the presence of an expansive solid growing mass with multilobulated shapes on the left kidney. The lesion was 90 mm (Longitudinal Diameter-LD) × 85 mm (Transversal Diameter-TD) × 96 mm (Anteroposterior Diameter-APD) sized (Figures , and ). The mass infiltrated the renal pelvis and the mid calyx (); proximal ureter was encased but not infiltrated (). The mass showed an inhomogeneous enhancement after contrast medium administration due to the presence of solid parts; many colliquative-necrtotic areas and intralesional calcifications were also found (Figures and ). An enlargement of the ipsilateral splenic vein, 9 mm sized (), and many intralesional collateral vessels were also observed (). No radiological signs of thrombosis of the main arteries or veins were detected. At the istm of the pancreas a nodular round-shaped lesion, 6 mm in size, was observed. The lesion, isodense in the precontrast scans (), after contrast medium administration, appeared hypervascularized in all the three phases of the study with a rich vascular supply (Figures , , , and ) and was referable, due to its contrastographic behavior, to a local metastasis or to a primitive neuroendocrine tumor. The pancreatic lesion showed on CECT scan unusual characteristics for a metastasis from renal clear cell carcinoma and, before considering surgical excision of the pancreatic mass, we decided that biopsy of the pancreatic lesion was needed in order to assess its nature. So the patient underwent a left nephrectomy with surrenalectomy and at the same time biopsy of the pancreatic lesion was performed. At the histological examination the renal mass resulted to be a clear cell carcinoma and the pancreatic lesion was found to be a metastasis of clear cell renal carcinoma.
Despite the guidelines indicated a local excision of the pancreatic lesion, we decided to start a systemic treatment, considering that the patient was not able to perform a second surgery because she had just undergone a major surgical intervention and she was not in good clinical condition at that time []. So, the patient started medical treatment with Sunitinib, 50 mg/day for two cycles. Due to the onset of grade III skin toxicity and gastrointestinal toxicity, the dose of Sunitinib was reduced to 37.5 mg/day for other six cycles []. After three months of treatment another CECT total body scan was performed. CECT scan showed in the posterior-basal segment of the inferior right lobe, close to the pleural wall, a solid nodular lesion, 10 mm (LD) × 8 mm (TD) × 8 mm (APD) sized (Figures and ). The hypervascularized pancreatic lesion was not more observed; a hypodense round-shaped lesion in the same region was observed (Figures and ) which was a colliquative-necrotic outcome of the therapy. Considering that the lung lesion appeared during treatment with Sunitinib we thought that the best therapeutic choice was to remove surgically the lung lesion, mainly for two reasons. The first reason was that the patient was in good general clinical condition with adequate lung function and the lesion was surgically resectable. So, according to guidelines, surgery was indicated []. The second reason was that the lung lesion appeared during treatment with Sunitinib, while the pancreatic metastases disappeared, so there was a possibility that the lung lesion was a second malignancy. In our case we decide to surgically remove the lung lesion in order to cure the patient and also because a histological characterization of the lung lesion was needed in order to differentiate the lung lesion from a second malignancy. Therefore, the patient performed surgical removal of the lung lesion. Histological examination deposed for metastasis from clear cell renal carcinoma.
After the surgery the patient performed two more cycles of Sunitinib, stopped due to poor tolerance, a total of eight cycles were administrated. The following CECT exams showed the outcomes, such as metallic clips, of the left nephrectomy and surrenalectomy (), and of the resection of the lung metastasis (); no local relapses were seen in both the organs. The previous hypodense lesion seen at the istm of the pancreas totally regressed and no other pancreatic lesions were observed (). Nowadays the patient is under oncological follow-up; she is in a good state of health, and she is disease-free for 47 months from the diagnosis of the tumour and for 20 months from the end of the treatment with Sunitinib.


In January 2013, a 28-year-old primigravida
woman with sever lower abdominal pain presented to the emergency room of our hospital (Tehran Women General Hospital, Tehran,
Iran). She suffered from vaginal spotting and lower abdominal pain for 5-6 consecutive days.
She revealed a history of primary infertility
with 3 years duration. Because of clinical and
paraclinical manifestations of polycystic ovarian syndrome, metformin (1500 mg/day) has
been prescribed for induction of ovulation since
8 months ago. After starting this medication,
she developed regular menstruation pattern.
Her last menstrual period was 25 days prior to
admission date. She had no previous history of
pelvic inflammatory disease, abdominal surgery, abortion, or use of any intrauterine contraceptive device (IUCD). Her hysterosalpingography (HSG) demonstrated an otherwise
normal image without uteroovarian fistula. On
general examination, she was pale. Abdominal
examination showed abdominal distention and
guarding. On vaginal examination, the uterus
was normal in size and the cervix was tender
in motion. There was a tenderous mass in deep
palpation of right fornix. Clinical investigation
showed hematocrit level of 25.2%, and betahuman chorionic gonadotropin (β-hCG) titer of
3569 m IU/mL. Vaginal ultrasonography demonstrated empty uterus with 6 mm endometrial
thickness, free fluid in the peritoneal cavity,
and a right sided heterogeneous adnexal mass
(52×61 mm) beside the uterus. These findings
were suggestive of ruptured ectopic gestation.
Based on the above findings, the patient underwent emergency laparotomy in which demonstrated an enlarged and bluish right ovary with
a 4 cm hemorrhagic and ruptured ovarian mass
and a leaking hematoma on its surface. A 3×3
cm multicystic structure was identified in the
other side of right ovary and was presumed to
be a tumorous lesion. The uterus, both tubes
and the left ovary appeared to be normal in appearance. The right tube had normal fimbriated
end without dilation. There was no obvious
evidence of endometriosis, metastatic lesions,
pelvic inflammation, or adhesion. We found
1500 mL bloody fluid in abdominal cavity.
The diagnosis of ovarian pregnancy was made.
Therefore, surgical resection of hemorrhagic
mass with conservation of the right ovary was
done carefully. Because of bad looking appearance of the concurrent cyst in the right ovary,
ovarian cystectomy and endometrial curetting
were performed, respectively. The final pathologic analysis revealed vascularized chorionic
villi and trophoblastic cells within ovarian paranchymal tissue (Figes, ). Histopathological
study demonstrated that the excised cyst was
a benign serous cyst adenoma (). The endometrial sample showed decidual change, but
no gestational tissue. The post-operative course
was uneventful. On monitoring of β-hCG lev-
els, they were undetectable (<5 m IU/ mL) on
the 21st
postoperative day. The patient menstru-
ated 37 days after the surgical operation.


The patient was a 52-year-old man who had proteinuria and hematuria that were diagnosed in his high-school days but for which he had not sought treatment. He subsequently experienced severe renal failure due to nephrosclerosis and was started on hemodialysis 2 years before this case report. Thereafter, he had a depressive mood, anxiety, and insomnia, and he subsequently presented with symptoms such as pressure of speech, flight of ideas, delusions of grandeur, thought dispersion, and irritability. Diagnosis was bipolar disorder according to DSM-IV-TR diagnostic criteria, and he was treated with olanzapine but showed no improvement. He was admitted to the psychiatric ward of our university hospital.
Because the patient continued to show a threatening attitude and irritability toward medical staff or other patients while being hospitalized, he was initially treated with VPA (400 mg/day) in addition to olanzapine (10 mg/day) and zopiclone (5 mg/day) and other drugs for general medical conditions [nifedipine(40 mg/day), artinolol hydrochloride (20 mg/day), valsartan (160 mg/day) and doxazosin mesilate (3 mg/day) for hypertension, and sodium polystyrene sulfonate (25 g/day) for hyperpotassemia, allopurinol (100 mg/day) for hyperuricemia and alfacalcidol (0.25 μg/day) for chronic renal failure] by the previous physician in charge. The dosage of VPA was increased up to 1,200 mg/day, and the manic symptoms gradually stabilized. The plasma level of VPA was 88.5 μg/ml.
However, approximately 3 weeks after increasing the dose of VPA to 1,200 mg (62 days after admission), the patient complained of severe abdominal pain. Blood amylase was found to be markedly high at 1,227 U/l (pancreatic amylase: 1,133 U/l) (Table ), and abdominal computed tomography revealed pancreatomegaly and an increased amount of peripancreatic fat (Figure ). Hence, acute pancreatitis was diagnosed. We believe that the pancreatitis was due to VPA because olanzapine, zopiclone and other drugs for general medical conditions had shown no particular adverse effects, and the patient had not consumed alcohol and did not have a history of biliary system disease such as gallstones. We promptly discontinued oral medication, and he was started on a pancreatic enzyme inhibitor, antibiotics, and transfusion, and he showed improvement for approximately 1 month (Figure ). Because the patient’s psychiatric symptoms were exacerbated, levomepromazine (50 mg/day) was then started without any adverse effects. He was discharged on Day 197 of the illness.
In 1979, Canfield et al. [] and Batalden et al. [] separately reported cases of acute pancreatitis caused by VPA. Since then, more than 100 such cases have appeared in the literature [], although most cases involved epilepsy, and only two cases involved bipolar disorder [,]. Ng et al. reported a female patient with epilepsy who was administered VPA and developed pancreatitis during hemodialysis []. Since then, the similar types of cases that have been reported thus far were cases with epilepsy [,-]. To the best of our knowledge, the present report is the first case of VPA-induced pancreatitis in a patient with bipolar disorder who was also undergoing hemodialysis.
Torelli et al. reported that the intermediate metabolites of VPA have toxic effects on pancreatic cells, and this is believed to be the main cause of VPA-induced pancreatitis [], although the definite metabolite has not been revealed yet. VPA is primarily metabolized as a glucuronic acid-conjugated compound []; however, several types of intermediate metabolites other than the glucuronic acid-conjugated compound are found in normal human urine. One possible etiology is that patients with renal failure are prone to pancreatitis because these intermediate metabolites accumulate due to renal failure leading to pancreatic cell damage.
When considering regimens of other mood stabilizers for patients with bipolar disorder who have renal failure, lithium carbonate does not bind to the plasma protein, and more than 95% of lithium is excreted by the kidney []. Therefore, patients with renal dysfunction would be expected to accumulate lithium and show a decline in renal function and serious lithium toxicity. In addition, maintaining adequate blood levels of lithium to achieve a response may be difficult because of the high dialyzability of lithium []. For administration of carbamazepine (CBZ), close monitoring of patients with renal failure is believed to be required because toxicity can readily occur as a result of accumulation of toxic CBZ-hydroxylated metabolites due to low albuminemia and inhibition of CBZ binding to the serum protein by a uremic toxin []. Therefore, using other mood stabilizers such as these for patients on hemodialysis requires close therapeutic drug monitoring. Physicians should be alert to signs of serious toxicity and the onset of adverse effects.
VPA is primarily metabolized by the liver; therefore, the management of the dose and blood level should be relatively easy because of its low dialyzability []. Therefore, VPA seems to be safe even for patients on hemodialysis. However, as in the present case, we must be cognizant of the possibility of serious adverse effects such as pancreatitis.


A 22-year-old Caucasian woman, gravida 3 para 2, was admitted to our unit at the 26th week of gestation for a detailed anatomy scan. Her pregnancy had been uncomplicated and an ultrasound examination at the 12th week of gestation had not revealed any anomaly. Fetal growth and the amniotic fluid volume were normal. The transabdominal ultrasound examination at the 22nd week of gestation revealed anhydramnios, which lead to the patient being referred to our clinic. A detailed transabdominal ultrasound examination was performed using Voluson E8 equipment with a 5.0MHz convex probe (GE Healthcare, Little Chalfont, UK). Anhydramnios and failure to visualize the kidneys, bladder and renal vessels were confirmed with the use of sonography in our department. Since the lack of amniotic fluid limited the acoustic window for fetal USG, an MRI scan was requested to confirm suspected renal agenesis. A fetal MRI scan was performed two weeks later at the 28th week of gestation and confirmed the suspected diagnosis (Figure ). No other anomalies were detected. MRI was performed using a General Electric Optima 360 1.5T scanner (GE Healthcare). Single-shot fast spin-echo sequences (SSFSE) were used, obtaining T2-weighted images in coronal, axial and sagittal planes. The MRI examination was well tolerated by our patient, and fetal movements did not alter the image quality, even though no maternal sedation was used.
A baby boy was born by breech vaginal delivery after spontaneous onset of labor at the 34th week of gestation. The boy weighed 1690g, with Apgar scores of 6 and 4, at two and five minutes, respectively. There was a respiratory effort observed and his heart rate was 50 to 60bpm. The parents requested no resuscitation, and the newborn died one hour after delivery. The diagnosis of bilateral renal agenesis was confirmed on autopsy.
Polish law prohibits termination of pregnancy after the second trimester. However, even when the decision to continue pregnancy is made, a precise diagnosis may help health-care professionals prepare the parents for the neonatal outcome and make a decision concerning postnatal management of the child.


In March 2011, a 44-year-old Caucasian male was evaluated for urolithiasis. He had no physical complaints, macroscopic haematuria, or weight loss. His medical and social history were unremarkable; he never smoked. His father had been treated for colorectal cancer; his mother had three episodes of spontaneous pneumothorax and had been treated for a renal tumour. Physical examination of the abdomen showed no abnormalities. Routine laboratory tests were normal. Computed tomography (CT) of the abdomen showed an interpolar tumour in the left kidney, diameter 14 mm (, arrow), and a second tumour in the upper pole of the right kidney, diameter 8 mm (, arrow). After a needle biopsy of the largest tumour, revealing a chromophobe renal cell carcinoma (Figures and ), the tumour in the left kidney was treated with radio frequency ablation (RFA). The CT findings in combination with the positive family history for renal cancer and his mother's episodes of pneumothorax suggested Birt-Hogg-Dubé (BHD) syndrome. Sequencing of the FLCN gene showed a pathogenic heterozygous frameshift mutation (c.155delc; p.Leu518Phefs∗19)—which has not been described before in literature—and confirmed the diagnosis of Birt-Hogg-Dubé syndrome. The index patient had neither siblings nor children. His parents died years ago; blood or tissue was not available for molecular testing. Frequent follow up by magnetic resonance imaging (MRI) will be performed for evaluation of the small tumour in the right kidney and possible recurrence of the tumour in the left kidney.


We report a case of a 66-year-old man who was diagnosed in 2010 of sigmoid-rectal adenocarcinoma.
At the moment of the diagnosis CT scan revealed a bulky mass (8 × 7 cm) in the sigmoid colon with no cleavage plane with the bladder and the abdominal wall and perilesional fat micronodulations. First operation was performed in another hospital. In that occasion the cancer was entirely removed and colostomy in the transverse colon was created. Local resection was followed by chemoimmunotherapy since there were no more signs of progression.
On December 2011 the patient came to our hospital. The cancer remained focal with no signs of metastasis. CT scan, indeed, showed a voluminous solid mass localised in the sigmoid colon that infiltrated the bladder and the abdominal wall including fascia and muscles up to the subcutaneous tissue. General surgeons proceeded with an anterior pelvic exenteratio removing the sigmoid colon en bloc, part of the ileum, the bladder, and the area of the abdominal wall infiltrated by cancer. Abdominal wall was reconstructed with a monofilament abdominal mesh in the lower abdominal quadrants, together with a ureterocutaneous stomy.
Afterwards, the presence of an enteric fistula caused a retroperitoneal pool and another surgery was needed. A relaparotomy was performed with abdominal mesh removal, enterorrhaphy, and an extended viscerolysis. Abdominal wall was finally closed with a macroporous polypropylene mesh.
Four days later signs and symptoms of peritonitis were diagnosed and the patient underwent a third surgery: the mesh was removed for the second time and another explorative laparotomy was made, followed by drainage of peritoneal cavity, viscerolysis, and creation of a lateral ileostomy in the left iliac fossa. Primary closure of the abdomen was not possible to achieve and an open abdomen negative pressure therapy system was applied over the peritoneal organs with a pressure of −125 mmHg in order to reduce edema. Dimensions of cutaneous dehiscence were, at the moment, 16 × 10 cm.
Three days later, a further valuation showed an improved clinical presentation of intestinal loops. The abdominal wall was then reconstructed using a biological porcine cross-linked mesh, Permacol (Covidien Inc., Norwalk, CT), of 20 × 15 cm overlapped and fixed to the fascial edges of the incision using nonabsorbable stitches. Biological mesh was used for its lower recurrence rates if compared with allograft and other biological meshes []. An advancement of the cutaneous tissue was made in order to reduce dehiscence as much as possible, obtaining a final dimension of 12 × 7 cm ().
Postoperative period was characterized by intestinal motility resumption through the ileostomy and conservation of renal function thanks to the ureterocutaneous stomy; oral alimentation was reintroduced. Patient was then transferred to our department.
He presented a 12 × 7 cm abdominal dehiscence which could not be closed neither directly nor with local advancement flaps because of excessive tension on wound edges. Moreover, patient clinical conditions made adoption of myocutaneous regional or distant flaps contraindicated. We subsequently considered NPWT as the best therapeutic option in order to reduce wound dimensions and to obtain secondary closure. For the first period negative pressure was set at −70 mmHg and was renewed twice a week (). We decided to adopt a lower pressure at the beginning as we were not confident to apply it to a biological mesh.
After four days of treatment the wound showed signs of local infection. Swab revealed the presence of Pseudomonas Aeruginosa and Corynebacterium species, two of the most common nosocomial pathogens in our experience. The patient was then treated with Ciprofloxacin (500 mg/bid) and Meropenem (1 g/tid). Locally the wound was disinfected with chlorhexidine and treated with a silver antimicrobial barrier dressing combined with NPWT. Infection was controlled and after 10 days of systemic and local therapies we obtained a negative swab for both pathogens.
Two weeks after NPWT application and once local infection has been controlled patient was discharged. At that point he still presented a 6 × 4 cm dehiscence of the abdominal wall. Moreover granulation tissue have been started to cover biological mesh and we decided to increase the negative pressure to −90 mmHg in order to further improve granulation tissue growth and to obtain a total coverage of the biological mesh.
Patient was therefore followed on outpatient basis twice a week. Wound dimension progressively decreased and granulation tissue flourished ().
NPWT was removed after a total therapeutic period of 43 days. During this period negative pressure was set at −70 mmHg for a total of 14 days and −90 mmHg for the remaining period. Our plan was to further increase negative pressure but the good clinical response allowed us to reduce time of NPWT. During all therapeutic period we adopted a polyurethane foam as dressing. Wound dehiscence starting dimensions were of 12 × 7 cm while, at the end of treatment, the final dimension was 3 × 5 cm ().
After NPWT removal wound bed was medicated with alginate and silver sulfadiazine for the following 14 days twice a week in order to prepare it for the final reconstructive step.
When biological mesh was completely overlaid with granulation tissue, the remaining loss of tissue in the lower abdominal area was closed with a split-thickness skin graft taken from the left thigh. The graft takes roots perfectly allowing total closure of abdominal dehiscence after 55 days from patient transferral to our department. No recurrence and local complications occurred with a postoperative follow-up of 6 months.


A 12-year-old Caucasian girl presented to our Department of Paediatric Surgery with a 30-day history of evening fever (38.5 to 39°C). Her general condition was good. After 7 days of fever, laboratory results showed a white blood cell count of 10,000/mm3 and a platelet count of 600,000/mm3 of blood. Inflammatory indices were increased: erythrocyte sedimentation rate was 56mm/hour, and C-reactive protein was 208mg/L; microhaematuria was also present (20/high-power field of red blood cells). The results of urinary tests and urine culture were negative. An abdominal ultrasound scan initially revealed no abnormalities and, although a focus of infection was not evident, antibiotics were administered as a preventive measure. After 3 weeks of fever and poor response to antibiotic therapy, prednisone was administered as it was suspected that she had rheumatoid arthritis. After this treatment, she had a remission, but in the following month she complained of suprapubic pain. A physical examination showed painful palpation of her hypogastria and no inflamed lesion on the surface of her skin. A repeat ultrasound evaluation of her abdominal wall revealed abscess formation without any communication with her bladder. In the absence of any umbilicus discharge, we suspected an infected urachal cyst (Figure ).
Afterwards, she underwent an immediate exploratory laparoscopy, which confirmed the presence of an infected urachal cyst. Because of inflammation and bleeding, we decided not to excise the cyst but to perform only a large incision and drainage (Figure ). Tissue specimens were taken for histological examination, which revealed inflammatory infiltrate with no evidence of malignancy and residual mucosa. There were no postoperative complications or recurrences after the incision and drainage of the infected urachal cyst. To our surprise, during the follow-up, echographic studies, performed after 3 and 6 months, showed a gradual reabsorption of the cyst; it was completely reabsorbed after 12 months. Six years later she is well and the urachal cyst has remained absent on magnetic resonance scans (Figure ).


On 18 December 2013, a 7-year-old, 38 kg, female, mixed-breed dog referred to the Small Animal Clinic of Tehran University with complaints of unthriftiness, lethargy and progressive weight loss together with a mass in the left upper quadrant. Clinical examination revealed a rapidly growing and progressive enlargement of the abdomen, periodic bloody discharge from the vulva and incontinence were observed.
At postmortem examination, a firm, slightly raised mass measuring 7 cm × 9 cm × 4 cm was identified on the left ovary; the contralateral ovary was not enlarged and measured 1 cm × 0.5 cm × 0.5 cm. The ovary surface was irregularly roughened with a papillary appearance.On cut surface, the left ovary contained multiple cysts, predominantly in the ovarian cortex, that were surrounded and separated by gray to tan thickened foci with scattered to coalescing hemorrhage and necrosis. The uterus and regional lymph nodes were uninvolved, but numerous, variably sized, firm nodular masses were scattered throughout the peritoneum, mesentery and were attached to the serosal and muscular layer surfaces of the small intestine. The mesenteric mass was multiple, red, multi-nodular and had foci of necrosis centrally, approximately 1.5 cm in length and 0.5 cm in diameter. The dog was not pregnant. No other abnormalities were found.
The samples were fixed in 10% neutral buffered formalin, processed by conventional methods, and paraffin embedded. Tumor, 2 to 4 paraffin blocks were available. Sample, 5-μm-thick sections were obtained and stained with hematoxylin and eosin. Canine POC were classified according to the human classification.
Immunohistochemical staining of both normal and neoplastic ovary was performed on deparaffinized sections by using the avidin-biotin-peroxidase complex technique procedure7 for CK20 (mouse monoclonal antibody, clone 20 diluted at 1:3,000), CK7 (mouse monoclonal antibody, clone OV-TL 12/30, diluted at 1:200). The primary antibodies were incubated overnight at 4°C and antigen retrieval was done for CK20 and CK7, by incubating for 15 min at 37°C with pepsin to differentiate between primary and metastatic ovarian tumor.
Histologically, the tumor is characterized by cysts and papillary endocystic projections with a multilocular cystic ovarian tumour which consisted of malignant serosal cystadenocarcinoma and infiterative tumor cells resembling the papillary ovarian tumor in the serosal surface of the small intestine along with an intact serosa (Figure B and C). In numerous foci the tumour invaded atretic follicles, displacing the serosal layer and forming papillary projections that extended into the follicular lumina (Figure A and D). On the other hand, the ovarian tumor was composed of numerous prominent cysts. Microscopically, the normal parenchyma was completely replaced by solid tumor with multiple areas showing a papillary pattern and the presence of necrotic and hemorrhagic foci. Neoplastic epithelial cells were arranged mainly the cystic and papillary structures and were supported by a fibrovascular stroma. The cysts were lined by single- to multi-layered cuboidal to columnar, pleomorphic, epithelial cells that occasionally formed arboriform papillae and these structures lined by clear cells arranged in nests separated by vascular stroma. In addition, papillae were arranged by cuboidal to columnar cells with abundant pale eosinophilic cytoplasm, distinct cell margins, ovoid and vesicular nuclei, and prominent nucleoli, moderate to severe nuclear and cellular pleomorphism and numerous mitotic figures were detected (Figure E and F). The surface epithelium of adjacent sections exhibited multiple foci of hyperplasia, forming small papillary projections. Numerous follicles were infiltrated by many neutrophil granulocytes that contained abundant clear cytoplasm with phagocytized lipid.
Representative sections from the ovary tumor was stained immunohistochemically with cytokeratin 7 (CK7) (1:100, OV-TL-12-30, DAKO, Glostrup, Denmark), CK20 (1:40, Ks 20.8, DAKO, Denmark). The results of the immunohistochemical stains were the same in the ovarian tumors. Serosal tumor cells showed negative immunoreactivity for CK20, but positive staining for CK7, leading to a final diagnosis of papillary ovarian cystadenocarcinoma and metastatic ovarian tumor (Figure ). The histology and the immunostaining profile were suggestive of a metastatic papillary ovarian cystadenocarcinoma to the small intestine serosal surface.


A 25-year-old male patient was admitted to our department, presenting progressive pain and numbness with a rapidly growing mass located in the upper part of the right arm for 2 months in 2005. At the time of the hospital admission, he had completely lost his occupational capacity of the arm (100% unable to work). Physical examination revealed a palpable mass on the upper part of his right arm with a size 13 × 12 cm. Radiographic examination demonstrated a large mass over 10 cm in diameter on the proximal humerus, with massive cortical erosion, extension of the tumor into soft tissues and indistinctness of the surface of the tumor (). A diagnosis of chondrosarcoma was made in view of the radiologic findings in combination with the histology by the needle biopsy. The specimen was histologically classified as grade II chondrosarcoma. The patient had a 20-year history of a painless and palpable bony mass on this location and had gradually increased in size. He first sought treatment at age 16 because he had a distal radius fracture. At that period of time, the plain radiographs demonstrated multiple cartilaginous dysplasia or multiple enchondromatosis (Ollier’s disease) in the humerus with most evidently involving the upper part of the humerus (), and the lower part of the radius () of the right upper limb. Soft tissue of hemangiomas was not detected. The patient had no family history of skeletal disorders and no other medical history of note.
A limb-salvage strategy with treatment of the large segmental defects following resection of the tumor and a prosthetic replacement of the proximal humerus was designed for the patient. The informed consent was obtained from the patient prior to the operation. The tumor resection prosthesis was applied in the same session following the tumor resection (). The tumor tissues were excised with maximum possible surgical safety margins. Frozen section biopsy was conducted during the surgery to ensure a safe surgical margin. Then the humeral head prosthesis (Waldemar LINK GmbH & Co., Germany) tailored for the patient was implanted, and the shaft preparation with cementing the prosthesis (, arrow indicated) was designed for supporting the reattachment of muscles and/or tendons to the prosthesis. This strategy was designed to get better active function, because inadequate reattachment of muscles and/or tendons to prosthesis cause considerable loss of function and loss of joint stability [, ]. No adjuvant chemotherapy and radiotherapy for the patient were given. Postoperative physiotherapy was applied to this patient. After surgery, the patient was to start active motion of the hand, wrist and elbow at 1 day even with mild tolerable pain. The patient could start to swing the shoulder joint at 4 weeks after surgery. Lifting and abduction of the shoulder joint were gradually started at 6 weeks after surgery. Early full weight bearing was started at 8 weeks postoperatively. Three months after surgery, the patient was almost 100% back to work and could perform all activities of daily life.
During the 8-year follow-up, clinical and radiologic examinations were done at the periodic controls. There are no signs of local recurrence and/or remote metastasis so far. The therapeutic results have been satisfied with a good functional recovery of the treated limb, enabling the patient to return to the pre-disease daily living and occupational activities. The overall functional outcomes were assessed by the musculoskeletal tumor society (MSTS) scoring system. The patient had the overall score 24 in the last examinations of the follow-up, and the ranges of motion of the shoulder joint are shown in .


A term baby girl was born to a 37-year-old Saudi woman, with a birth weight of 2600 grams. The mother had fever, body aches, and macular skin rash for a few days in the 3rd trimester. Antenatal scan was normal, and antenatal serology screening for congenital infections was not done. Six days after birth, the baby was admitted with fever and convulsions, She was diagnosed as neonatal sepsis, however; septic screening was unremarkable including a negative blood culture. Similar sepsis like episodes with fever and convulsions recurred at 3 and 6 weeks of life, which were treated with intravenous antibiotics, and the baby was discharged home in a good condition. At the age of 3 months, the diagnosis of DI was suspected when she presented with significant polyuria (urine output was around 15 ml/kg/hr), severe dehydration, and convulsion. Although systemic examination was normal at birth, she started to show up abnormal neurological features by 3 months of age including: bilateral nystagmus, significant head lag, hypertonia, brisk reflexes, and extensor planter response, along with failure to thrive. Examinations of the fundus showed bilateral chorioretinitis.
Renal function showed normal urea and creatinine, however; serum sodium was ranging between 147 and 150 mmol/L. Urine was diluted with urine osmolality between 100 to 110 mosm/L (normal >600 mmol/L) and she had low urine specific gravity of 1.005 (normal > 1.010). Thyroid function, growth hormone, luteinizing hormone (LH), follicle stimulating hormone (FSH), adrenocorticotrophic hormone (ACTH) and cortisol were within the normal limits (Table
). Computerized tomography (CT) of the brain demonstrated dilated ventricles with multiple subependymal and parenchymal calcifications (Figure
). Cerebrospinal (CSF) analysis showed high protein and cells (Table
). Electroencephalography (EEG) revealed multifocal epileptiform discharges on diffusely slow background. Electroretinography (ERG) illustrated evidence of retinopathy on the right eye.
Serology for toxoplasma specific immunoglobulin M (IgM), performed for this infant at 3 month of age using enzyme-linked immunosorbent assay (ELISA), was a 1370 unit/L (positive 120 (unit/L), and immunoglobulin G (IgG) was a 11128 unit/L (positive > 110 unit/L). As antenatal serology screening for congenital infection was not done, maternal serology for toxoplasma IgM and IgG was performed 3 month after delivery when CTox was suspected. This result showed that maternal toxoplasma specific IgM was a 630 (positive 120 (unit/L), and IgG was a 4320 unit/L (positive > 110 unit/L). Polymerase chain reaction (PCR) was positive for toxoplasma deoxyribonucleic acid (DNA) in both CSF and blood of the infant. The baby was commenced on pyrimethamine/sulphadiazine and leucovorin at 4 month of age aiming to continue this treatment for 1 year. A favorable response has been shown by regression of retinal lesions after few weeks of starting antimicrobial treatment. Also, she showed good response to nasal vasopressin with urine output reduced to 3.2 ml/kg/hr, urine osmolality increasing to 650 mosml/l and sodium controlled between 135 and 140 mmol/l. Monthly follow up routine blood tests including complete blood count (CBC), liver and renal functions showed satisfactory response to treatment and absence of side effects of antimicrobial therapy.
At age of 7 months and while still on pyrimethamine/sulphadiazine and leucovorin, the baby developed vesicular rash, high-grade fever, and convulsions. CBC was normal and CSF examination revealed protein of 1120 mg/dl, glucose of 65 mg/dl and 20 cells, mostly lymphocytes. CSF culture for bacteria as well as CSF polymerase chain reaction for Toxoplasma gondii was negative. Direct immunoflorescence test of a smear taken from the vesicular rash was positive for varicella. Based on this clinical data, in addition to having recent contact with a family member with active chicken pox, our patient was diagnosed as severe varicella meningoencephalitis. Compared to the previous images, CT brain in this acute stage showed that hydrocephalus was not progressive and hence ventriculoperitoneal shunt was not inserted. This patient underwent extensive immune work up including serum immunoglobulin, complement and neutrophil function that failed to identify evidence of immunodeficiency. She passed through a stormy course, became comatose with intractable seizures, irreversible shock and severe metabolic acidosis. She died despite full cardiorespiratory support with fluid, inotropes, hydrocortisone and mechanical ventilation.


A 24-year-old lady was referred for presyncope and LV dysfunction and frequent PVC. The patient's chief complaint was palpitation and exertional dyspnea since two years ago. Her last delivery was four years ago and she has been well at routine activities ever since. The exacerbation of symptoms occurred within recent three months. Echocardiography had revealed mild LV dysfunction (EF:40%). Surface ECG showed repetitive nonsustained monomorphic VT three months ago and her cardiologist began Sotalol to control her arrhythmia. The symptoms was not relieved after three month of treatment for heart failure and arrhythmia. She was then referred to us due to frequent PVCs and runs of nonsustained VT. ECG recorded during the non-sustained VT showed a left bundle branch block pattern in the precordial leads and an inferior axis and early transition in precordial leads in V3-V4 (). This pattern fulfilled the criteria of VT originating from the aortic sinus cusp reported by Ouyang et al. (1); although this QRS morphology could be seen in PVC’s originating from Right ventricular outflow tract (RVOT)as well. Having obtained the informed written consent from the patient, she underwent Electrophysiologic study in fasting state, using the standard technique, with no antiarrhythmic agents. Quadripolar catheters were placed in the right ventricular apex and His bundle region via the right femoral vein. At first, using classic method, the right ventricular outflow tract was mapped, but pace map failed to find any matched QRS with the spontaneous PVCs. In addition the mapping of aortic cusps was performed.
The finding of some good potential in left aortic cusp encouraged us to seek the origin of PVCs therein. A contrast aortogram was obtained to define the anatomy of aortic root and localization of coronary arteries. Great care was taken to avoid the ostium of the left main coronary artery, and mapping and ablation was only performed with stable catheter contact. The catheter of ablation was then placed retrogradely from aortic root and careful mapping was performed in left and right aortic cusps and their commissure. Meanwhile the (Activated clotting time) ACT was maintained above 250s to prevent clot formation. The best potential was recorded in a region located at the commissure of left-right aortic cusps ().
The recorded potential at the tip of the ablation catheter begins 24 msec earlier than the onset of PVC on the surface ECG which was quite suitable. In the commissure, however, morphology of pacing QRS through ablation catheter was not completely similar to patient’s PVC and was not of any help (). The site was located more than 1.0 cm anteroinferior to the left coronary artery ostium in the left aortic sinus cusp (). Regarding good earliest activation a single radiofrequency energy with setting 30W, 40Cwas delivered that resulted in immediate elimination of PVCs at less than 10 seconds.
Following continued energy delivery up to 60 seconds VT was no longer inducible by RV extrastimuli or burst pacing before or after isoproterenol administration, and no PVC recurred during a 30- minute observation period in the EP lab. The patient was discharged the day after ablation without any recorded PVC on monitor. LVEF in echocardiography improved to normal level two months later, and no PVC was detected at serial holter monitoring.


Our patient was a 62-year-old male, 175 cm tall who complained about dyspnea on exertion (NYHA class III). The patient was on beta blocker, warfarin, and diuretic and his rhythm was atrial fibrillation. Transthoracic and transesophageal echocardiography was performed and severe mitral stenosis was established. Mitral valve area was 0.8 cm2 and no thrombus was found in the left atrium and its appendage. In addition, Wilkins score was 6 and systolic pulmonary artery pressure was 38 mmHg.
The patient was candidatedfor BMV.
Before attempting for valvulotomy, coronary artery status was determined. Selective coronary angiography demonstrated no significant obstruction in the coronary arteries. First, the right femoral vein was approached, but it could not be accessed; therefore, the left femoral vein was punctured. Then, the retrograde injection of the contrast media in the proximal of the right external illiac vein showed that the right femoral vein was occluded. Thus, we decided to continue the procedure from the left side.
Transseptal puncture of the interatrial septum was performed via the left femoral vein using Brocken Brugh needle. After dilatation of this puncture site, Inoue balloon 28 was used and graded inflation was done with 24, 26, and 28 cc. The mean transmitral valve gradient was declined from 16 mmHg to 3 mmHg and the left ventricudar injection revealed no mitral regurgitation. After all, the procedure was terminated without any complications.
The patient was discharged two days after the procedure with beta blocker and anticoagulant (warfarin) due to atrial fibrillation rhythm.


A 33-year-old male with PRS and severe intellectual disability was referred for dysmorphic assessment. He was seen in a secure psychiatric ward, where he was detained for reasons of aggressive behavior. A full examination and accurate growth measurements were not possible because of marked agitation. He had a long, thin face with a prominent nasal ridge, a small mouth, micrognathia (Fig. D and E) and bilateral mild arachnodactyly. The clinical similarity between this man and the previously reported SATB2 translocation cases was noted (see below and Table ) and a karyotype showed a de novo reciprocal translocation, 46XY,t(2;3)(q33.1;q26.33). Metaphase FISH mapping located the 2q33.1 BP to 760 kb 3′ (centromeric) of the SATB2 polyadenylation site within the gene desert between SATB2 and PLCL1 (chr2:199 380 000–199 390 000 hg19) ( and Fig. ). The 3q26.33 BP mapped to a gene desert 50–80 kb 3′ (telomeric) of SOX2 (chr3:181 460 016–181 526 605) () in a region that has not previously been implicated in human disease. SOX2 heterozygous loss-of-function (het-LOF) mutations are associated with a syndromic form of anophthalmia or severe microphthalmia ().


A 74-year-old Chinese man presented with a one-month history of recurrent headaches, vomiting and left limb atony. He had undergone a successful clipping operation through the pterional approach for an anterior communicating artery aneurysm five years previously. One month before presentation, he developed a headache without apparent cause that was especially severe in the right frontal region. He also had a small tumor on the right side of his forehead at the original incision site; the tumor had gradually increased from soybean size to egg size in one month. This was accompanied by nausea, projectile vomiting of gastric contents and left limb atony. Computed tomography demonstrated a subcutaneous tumor on the right side of his forehead, a scalp lesion complicated with right frontal lobe hemorrhage, and a right ventricular hematocele. We gave our patient oxiracetam (5.0g in 250mL of normal saline (0.9% weight to volume of sodium chloride)) for treatment of neurotrophy. On the third day after admission, he suddenly lost consciousness. Urgent head computed tomography showed an intracranial hemorrhage, the volume of which had increased from 40mL to 90mL as well as persistent bleeding and a midline shift of over 1.5cm (Figure ).
Our patient then underwent surgery to resect the subcutaneous tumor and evacuate the intracranial hematoma under general anesthesia. An incision was made along the one from the original surgery. During the operation, it became apparent that the main part of the tumor was located in the subcutaneous tissue of his right frontal region. The tumor was approximately 7cm×6cm×4cm (Figure ), fully enveloped, and characterized by abundant revascularization. The cyst of the tumor was intact, and his temporal muscle tissue was thin. His cranial periosteum and cerebral dura mater were invaded by the tumor, resulting in a thin, brittle skull (Figure ). His dura mater was also thin and light yellow in color. The tumorous hemorrhage extended into his intracranial tissue, but no tumor tissue was seen in his subdural area. The intracranial hematoma was successfully removed during the operation (70mL from the right frontal part, 20mL from the intraventricular hematoma). The subcutaneous tumor was totally resected along with some affected cranial tissues. The epidural bleeding was completely stopped. However, the temporal muscle fascia was incomplete because of the hemostasis. An artificial dura was created by relaxation sutures to prevent cerebrospinal fluid leakage. His residual temporal muscle was then sutured, and his galea aponeurosis and scalp were perfectly closed. Our patient’s postoperative recovery was good, and he was safely discharged 20 days after the surgery. He was free of recurrence over two years of follow-up.
A pathological microscopic examination of the resected tumor (Figure ) showed a malignant histiocytoma (undifferentiated sarcoma). It was gray-white, had no capsule, was gray on the cut section, had areas of hemorrhage and necrosis, and had a ductile texture. Microscopic examination showed pleomorphic tumor cells, specifically fibroblasts, histiocytes, multinucleated cells and some inflammatory cells infiltration, as shown in Figure . Immunohistochemistry results showed it was negative for keratin, S-100 protein, actin, desmin, homatropine methylbromide 45, cluster of differentiation 34 and b-cell lymphoma 2, and positive for vimentin and cluster of differentiation 99, with Ki-67 content over 60%.


A 53-year-old female was referred to our centre because of a two-month lasting exhaustion, abdominal distension, early satiety and fever of up to 39°C. Her medical history was not significant. She had been travelling for a longer periods of time in the countries of the Middle and Far East, especially to Indochina and India. Physical examination showed splenomegaly and hepatomegaly, palpable at 15 and 6 cm below the costal margin, respectively. Peripheral lymph nodes measured up to 1 cm in the largest diameter. Petechiae and suffusions were present on the skin of the thoracic region and flanks. Laboratory tests demonstrated: (1) white blood count 3.40 × 109/l (Neu 2,01 (59%) , Ly 0,99 (29%), Mono 0,17 (5%), Eos 0,03 (1%)), platelet count 64 × 109/l, red blood cells 3,66 × 109/l, haemoglobin level 115 g/l (2) liver function tests were raised: AST 2.21 IU/L, ALT 0.95 IU/L and γGT 1.30 IU/L with bilirubin within normal ranges-bilirubin 17 IU/L (3) LDH was raised to 15,68 IU/L; raised was also CRP to 35 (4) serology for HAV showed reactive IgG anti-HAV antibodies, whereas IgM anti-HAV, HbsAg, anti- HBc, anti-Hbs, anti-HCV and CMV were negative.
The Giemsa-stained smears from bone marrow and peripheral blood showed presence of medium-sized atypical lymphoid cells. These cells slightly differed in size and shape, being round, oval or droplet-like with irregular cell borders. The cytoplasm was scant and basophilic, containing few small vacuoles. The nuclei were round or oval. Chromatin was immature, finely granular, with small inconspicuous nucleoli (Figure A, B, D). On flow cytometric immunophenotyping (FCI) of bone marrow and peripheral blood, these cells had immunophenotypic profile consistent with gamma-delta T-cell lymphoma (CD3 dim +, CD7 dim+, CD2+, TCRγδ dim +, CD52 dim +, CD56+, CD5-, CD4- and CD8-) (Figures A, B). Besides T-cell lymphoma, a small population of monoclonal B-cells with immunophenotype characteristic of HCL (CD103+, CD25+, CD22+, surface-kappa+) (Figures C, D) was also present in the bone marrow and peripheral blood. After careful re-examination of peripheral blood smears, only few hairy cells were found. These cells were medium-sized and oval in shape, their cytoplasm was abundant, pale basophilic, with circumferential “hairy” projections (Figure C).
Biopsy of a slightly enlarged left inguinal lymph node and bone marrow trephine biopsy were also performed. Histology of the lymph node showed partial, predominantly intrasinusoidal infiltration by medium-sized, atypical lymphoid cells that were immunohistochemically positive for CD3, CD56, and TIA, while other T- and B-cell markers were negative. Bone marrow biopsy (Figures ,, and ) revealed hypercellular bone marrow with reduced orthotopic haematopoiesis and intrasinusoidal infiltrates consisting of medium-sized lymphocytes with the same immunohistochemical profile as was observed on lymph node infiltrates. To continue, patchy interstitial infiltrates were also present, consisting of medium-sized lymphocytes with a round nuclear profile and fine chromatin, and they were widely separated from each other by abundant clear cytoplasm. These cells were positive for CD20, HCL, Cyclin D1 and TRAP (Figures and ). There was also a marked increase in reticulin fibres. According to the morphology, the immunophenotype and the infiltration pattern of lymphoid infiltrates, the diagnosis of coexistent HSTCL and HCL was established.
The patient was treated with multivalent chemotherapy including rituximab. The effect of treatment was monitored by clinical and ultrasound examination, leukocyte and lymphocyte counts and FCI of peripheral blood. The patient received the first immunochemotherapy regimen with R-VACPE (rituximab-vincristine, doxorubicin, cyclophosphamide, prednisone, etoposide) and splenectomy (Figures , and ). After that HCL regressed completely while HSCTL was refractory to the treatment. R-VACPE was therefore changed to R-DAHP (rituximab, dexamethasone, cytarabine, cisplatin) and then to R-EPOCH (rituximab- etoposide, vincristine, doxorubicin, cyclophosphamide, prednisone). However, all these treatment regiments turned out to be only partially effective. The disease progressed again in the liver, and the patient’s general condition worsened. Therefore, only palliative treatment with liver irradiation was continued. The patient died soon because of haematemesis and Mallory-Weiss syndrome, 7 months after the initial diagnosis.


A 26-year-old Caucasian woman presented with a chief complaint of left-sided maxillary pain with intermittent, discoloured nasal drainage. Seven years prior to current presentation, the patient had reported a history of headaches, nasal congestion and bilateral discolored drainage refractory to prednisone, antibiotics, and endoscopic sinus surgery at an outside facility. Revision endoscopic sinus surgery one year after the initial surgery provided partial relief. Two years prior to current presentation, her left maxillary pain recurred, and a computed tomography (CT) scan revealed a left wisdom tooth projecting into her maxillary sinus. Following wisdom teeth extraction, she had marked improvement in pain and nasal drainage; however 8 weeks later, left maxillary pain returned and was associated with left-sided yellow nasal discharge. Her oral surgeon discovered an infection in the molar adjacent to the extracted wisdom tooth and performed a root canal.
Two weeks following the root canal, she presented with continued left maxillary tooth pain and left-sided discolored nasal discharge. Extraction of the molar failed to resolve her pain, and she was subsequently referred to the facial pain/headache clinic by her dentist where she was prescribed gabapentin 300 mg TID, which was ineffective. Her dentist also prescribed her multiple courses of clindamycin 150 mg TID and guaifenesin 600 gm QID, which would improve her pain and discoloured drainage. However, her symptoms would return after completing the antibiotics. On examination with rigid endoscopy, she had widely patent maxillary, ethmoid, and frontal sinus ostia with no purulence or polyposis. On flexible endoscopic examination of the floor of her left maxillary sinus, white to slightly yellow mucus was found. Aspiration of the mucus relieved the patient’s discomfort. Maxillary sinus cultures revealed few polymorphonuclear leukocytes, few mononuclear cells, and no microorganisms. Her most recent CT scan from two years prior to presentation at our facility was remarkable for minimal mucosal thickening of the floor of the left maxillary sinus was otherwise normal (
). An occult dental infection was considered high in the differential diagnosis; however, because no actual dental infection could be demonstrated, a medial maxillectomy was considered in order to facilitate topical washing of the left maxillary sinus. Follow-up appointments in the facial pain/headache clinic found “some features of migraine, but it is unclear whether headaches represent primary or secondary headaches with migraine features”. Repeat evaluations by her dentist found no evidence of a dental infection. Because of past improvement on an 8-week course of clindamycin 300 mg TID, she was prescribed a 12-week course of clindamycin 300 mg TDI before surgery was considered, and she was referred to an infectious disease specialist.
Clindamycin initially improved her symptoms, but midway through the course her congestion returned. A bone scan was positive in the region of the left maxilla; however, a repeat indium scan was negative. Her CT scan was repeated and she was referred to an oral surgeon for evaluation (
–
).
The CT scan was evaluated by an oral and maxillofacial surgeon who noted that there were two small remnants of the prior left maxillary root canal that had been performed two and half years ago (
–
). A combined endoscopic and Caldwell-Luc approach under computer-assisted navigation to drill out retained gutta-percha in the maxillary sinus resolved the patient’s pain and drainage immediately, without recurrence at her three month follow-up. No pathological specimens were available for analysis as the gutta-percha remnants were drilled out during the surgery.


A 79-year-old Japanese man with acute vision loss in the left eye was referred to us in February 2015. He had a 24-year history of Graves’ disease and was taking MMI (starting dose of 30 mg/day in 1991, which was gradually reduced to 5 mg/alternate day by the time of his first visit) and levothyroxine (25 µg/day since 2013). He also had chronic obstructive pulmonary disease with pulmonary emphysema. The best-corrected visual acuity (BCVA) was 0.8 in the right eye and light perception in the left eye, and he had a relative afferent pupillary defect in the left eye. Extraocular muscle movements were normal, and exophthalmometer (Hertel) measurements were 20 mm bilaterally. There were no findings indicative of vision loss in the intermediate optic media or fundus of the left eye (Fig. ). Although his thyroid-stimulating hormone level was low [0.11 µIU/mL (reference value: 0.50–5.00 µIU/mL)], his thyroid function was almost normal [free T3: 2.55 pg/mL (2.3–4.0 pg/mL), free T4: 1.73 ng/dL (0.9–1.7 ng/dL)]. Although plain magnetic resonance imaging (MRI) demonstrated mild enlargement of the extraocular muscles in both eyes, there was no compression or stretching of the optic nerve, which would have been suspicious of thyroid optic neuropathy (Fig. A). A computed tomography (CT) scan of the head showed the left ethmoid sinus to be filled with soft tissue and destruction of the upper ethmoid sinus wall, which included the anterior cranial base (Fig. B). A contrast-enhanced MRI revealed thickened mucous membranes in the left ethmoid sinus, enhanced dura from the left orbital apex to the anterior cranial base, and a mild enhancement of the optic nerve surrounded by enhancement at the orbital apex (Fig. C, D). We suspected optic neuropathy due to HP and infectious rhinogenic optic neuropathy due to infectious sinusitis. Inflammatory markers were high based on the laboratory data: white blood cells 8680/µL (3500–8500/µL) with 76.1% neutrophils (40–70%); C-reactive protein (CRP) 2.40 mg/dL (≤ 0.3 mg/dL); erythrocyte sedimentation rate 31 mm/hour (≤ 10 mm/hour) and 53 mm/2 hours (≤ 25 mm/2 hours); however, markers related to infection did not show a remarkable change [procalcitonin: 0.07 ng/mL (≤ 0.49 ng/mL), β-d-glucan: < 6.0 pg/mL (0–10.9 pg/mL)]. We performed further examinations for suspected HP, and MPO-ANCA was 19.5 U/mL (< 3.5 U/mL), proteinase 3-ANCA was <1.0 U/mL (< 3.5 U/mL), and IgG-4 was 42.1 mg/dL (4.8–105 mg/dL). A whole-body CT scan showed no malignancy, but pulmonary nodule lesions and a blood clot in the left lower leg were detected. Proteinuria and hematuria were both positive, and he had bloody nasal discharge. We consulted rheumatologists and pulmonologists, who indicated that the findings were consistent with ANCA-associated vasculitis. After ruling out diseases that could lead to HP, such as syphilis, aspergillosis, and tuberculosis. Serological tests and interferon gamma release assays established a diagnosis of optic neuropathy secondary to HP with ANCA-associated vasculitis. The clinical findings met the diagnostic criteria for GPA [, ] involving the upper respiratory tract, respiratory organs, and kidneys. We diagnosed optic neuropathy due to HP secondary to GPA. High-dose steroid pulse therapy (1000 mg of daily methylprednisolone for 3 days) was initiated, followed by 50 mg (1 mg/kg body weight) of daily oral prednisolone (PSL), which was subsequently tapered. MMI was discontinued, since ANCA-associated vasculitis could be induced by antithyroid drugs. Six days into treatment, the patient’s BCVA recovered to 0.03. Nine days into treatment, Goldmann perimetry (GP) showed a central scotoma in the left eye (Fig. A). Visual recovery was insufficient, so an endoscopic sinus biopsy was done on the same day to rule out malignancy and confirm the diagnosis. A biopsy of the posterior ethmoid sinus mucosa and nasal septal mucosa showed severe infiltration of chronic inflammatory cells, including neutrophils. There were no findings indicative of malignancy. While perivascular hyperplasia of connective tissue was observed, no apparent vasculitis or granuloma was seen. The BCVA of the left eye recovered to 0.5 within 3 weeks after the start of the treatment, and a contrast-enhanced MRI revealed rapid regression of enhanced dura from the left orbital apex to the anterior cranial base and enhancement of the optic nerve became unclear, however, enhancement surrounding the optic nerve remained (Additional file : Figs. A and B). The BCVA of the left eye recovered to 0.9 within 6 weeks after the start of treatment, and the central scotoma resolved 7 weeks following surgery (Fig. B). Thyroid function was stable despite discontinuation of MMI.
Six months after starting the treatment, when PSL was tapered to 15 mg, the patients BCVA decreased and GP revealed scotomas in the left eye (Fig. C). A contrast-enhanced MRI, taken for follow-up 1 week before the patient realized his vision had deteriorated, showed thickened enhanced dura from the left orbital apex to the anterior cranial base, and the optic nerve surrounded by enhancement at the orbital apex (Additional file : Figs. C and D). Furthermore, mild enhancement of the optic nerve appeared to recur (Additional file : Figs. A and B). There was no remarkable change in laboratory findings, including MPO-ANCA and CRP levels. We started a second round of high-dose steroid pulse therapy (1000 mg of daily methylprednisolone for 3 days), followed by a PSL taper. We added 4 mg/week of methotrexate (MTX) to the PSL taper and discontinued it after 3.5 years. He had no relapse after the addition of MTX, and MPO-ANCA levels were within normal limits until 4 years after onset. Three months after his 4-year follow-up, he died from hypoxic ischemic encephalopathy due to pulmonary emphysema.


A 5-month-old girl, born at term with a birth weight of 3750 g, was presented to our outpatient department with the chief complaint of deviation of both eyes to the left side by the parents. The baby had history of oxygen therapy in neonatal intensive care unit (NICU) for neonatal pneumonia for three days after birth, there was no other systemic abnormalities. The exact duration and mode of oxygen inhalation was not known. Except for the deviation of both eyes to the left, there was no other abnormal facial features of the baby. A preliminary torch light examination was done at the time of admission later we examined the child under anesthesia under an operating ophthalmic microscope. The anterior segment of the right eye and fundus were within normal limits (Fig. ). The left eye cornea and lens were transparent, there was a whitish star shaped tissue close to posterior lens capsule (Fig. A). Vitreous cavity was anechoic. Fundoscopy revealed attached retina, a large excavated dysplastic disc with multiple vessels radiating from the disc margin. The peripapillary area showed chorio-retinal pigmentary changes. The macula was dragged to the temporal side. (Fig. B). Fluorescein angiography showed multiple radiating retinal vessels from the disc to periphery. The disc and its surrounding annular area showed hyper fluorescence without leakage (Fig. ). The retinal vessels showed abnormal branching with bulbous terminals and leakage of fluorescein dye at the periphery. The peripheral retina showed 360 degree non perfusion with a defined border between vascular and avascular retina (Fig. ). The B-scan ultrasonography of the left eye revealed disc excavation. MRI brain and orbit showed no obvious abnormality in brain parenchyma, normal right orbit, and right optic nerve with surrounding ring of enhancing cerebrospinal fluid (CSF). But in the left eye, the optic nerve head was excavated with scleral defect (Fig. A). The intra-orbital part of optic nerve in left side was tortuous-thickened and this was extending up to the chiasma (Fig. B). The absence of CSF enhancement surrounding the left optic nerve revealed abnormal development of the optic nerve sheath (Fig. C). The child received barrage laser photocoagulation to the edge of optic disc excavation of left eye to prevent retinal detachment due to dysplastic disc. In the follow-up visit after 1 week, laser scar surrounding the disc could be appreciated in fundoscopy. At last follow up after 9 months of laser intervention, the left eye fundus was stable (Fig. ).


A 46-year-old male presented with severe intermittent claudication and dyspnea. His medical history included severe aortic valve stenosis, bicuspid aortopathy, and uncontrolled hypertension. He had aortic coarctation repair at the age of 11 years with a 16-mm Dacron graft. Computed tomography (CT) scan revealed a 5-cm ascending aortic aneurysm and a severely kinked Dacron graft in the descending aorta (
). A one-stage open procedure was indicated to fix both ascending and descending aortic problems given endografting was unsuitable. A median sternotomy was performed and cardiopulmonary bypass was established. Beating heart techniques allowed us to expose the descending thoracic aorta through the posterior pericardium by retracting the heart cephalad with a heart positioner. The aorta was controlled with a partially occluding vascular clamp, and an end-to-side graft anastomosis was completed (
). The graft was routed posterior to the inferior vena cava and lateral to the right atrium. Then a supracoronary aortic graft (26 mm) and a mechanical aortic valve were implanted. The descending graft was then anastomosed to the lateral aspect of the proximal graft (
). Recovery was uneventful with discharge home in 4 days and resolved claudication.


A 69-year-old hypertensive female presented with recurrent hemoptysis and pneumonias. She had a history of isthmus coarctation as a child repaired with a patch aortoplasty followed by aneurysm formation repaired with a Dacron tube graft at 46 years of age. She developed severe hemoptysis at 57 years of age, requiring a left subclavian-carotid bypass and stent endografting and fungal suppression therapy for a positive cryptococcus bronchial culture. She was admitted to our service 12 years later with hemoptysis and malaise. The chest CT angiography (
) revealed mediastinal contrast extravasation, false aneurysm, and lung parenchyma involvement confirmed later with a bronchoscopy demonstrating blood in the left lower lung bronchi. A two-stage open procedure was performed. Through a median sternotomy, she underwent ascending aorta to left carotid bypass with a 10-mm Dacron graft, ascending-to-descending aorta bypass with a 16-mm Dacron graft using cardiopulmonary bypass and then aortic arch transection (
). A week later, she underwent third time left thoracotomy with mediastinal debridement and removal of the aorta and stent-grafts above the new Dacron graft (
). Aortic stump coverage with a diaphragmatic flap and segmental lung resection were required. Cultures were negative but she was placed on oral fluconazole suppression therapy. Postoperatively, she developed a chylothorax treated conservatively and was discharged home 21 days after initial surgery. At 2-year follow-up, she was without signs of recurrent infection.


A 75-year-old male with history of CLL/SLL presented to the emergency department (ED) with complaints of left knee pain and swelling for one week. Patient had undergone left total knee arthroplasty (TKA) for osteoarthritis twelve years prior without post-operative complications. Three months prior to presentation, he was started on ibrutinib for his CLL/SLL due to worsening anemia, leukocytosis, and lymphadenopathy. In the ED, his physical exam was notable for a left knee effusion with tenderness and limited range of motion. His effusion was aspirated, and synovial fluid analysis revealed a total WBC count of 6,404 cells/mm3 with 53% monocytes, 29% neutrophils, and 19% lymphocytes. On presentation, his WBC was 7.4 × 109/L (normal 4–12 × 109/L), with 57% lymphocytes and 30% neutrophils, with a hemoglobin of 9.6 g/dl (normal male 13.5–17.5 g/dL) and platelet count of 131 × 109/L (normal 150–450 × 109/L). His erythrocyte sedimentation rate (ESR) and c-reactive protein (CRP) were both elevated at 74 mm/h (normal male 0–20 mm/hr) and 76.9 mg/L (normal male <10 mg/L), respectively. He was started on intravenous (IV) vancomycin and cefepime and his ibrutinib was held.
The next day he underwent left knee incision and drainage with synovectomy and exchange of his tibial polyethylene component with orthopedic surgery with intraoperative specimens sent off for Gram stain and cultures. Forty-eight hours post-operative fungal stains turned positive for mold, and he was started on IV liposomal amphotericin B 5 mg/kg daily and antibiotics were discontinued.
Patient reported he was originally born in Southern California and lived there for 25 years. In his early 20's, he had acute onset of left knee pain and swelling, underwent left knee aspiration, and was told he had “Valley Fever” in his knee. Records from 1970s were not available; however, as per the patient, he was given 2 intra-articular injections followed by one year of antifungal treatment with complete resolution of his symptoms. He reported no problems with his knee until 2008 when he underwent left TKA for osteoarthritis. A CT scan of his chest four months prior to presentation for his CLL workup was noted to have a calcified granuloma in his left lower lobe suggestive of possible previous coccidioidomycosis inoculation.
After 12 days of incubation, the cultures were visualized in the lab (Figures and ) and confirmed to be Coccidioides immitis. He underwent explantation of his left total knee arthroplasty with extensive synovectomy and conversion to a rigid spacer. He was switched to oral fluconazole 400 mg daily on post-operative day one after receiving 12 days of IV liposomal amphotericin B. Coccidioides complement fixation came back positive at 1:16 (normal <1:2) and patient was discharged on hospital day 15 with his ibrutinib on hold.
Six months later his left knee was re-aspirated which demonstrated no fungal growth and his ESR and CRP had also normalized. He then underwent second-stage revision of his left knee with reimplantation of hardware without complications. He is currently following the expected post-operative course and will be continued on lifelong suppressive fluconazole therapy.


The patient is a 38-year-old female who experienced sudden onset blurred vision bilaterally followed by a focal headache over the right occipital projection one day prior to admission, which responded favorably to analgesics. Blurred vision recurred the next morning with left-sided predominance. She also experienced a sudden onset thunderclap headache after sneezing, which was followed by the same right-sided headache (VAS 3) as one day before. Her previous history was positive for infrequent migraine attacks without aura, smoking (10 P/years), and a second shot of the Moderna SARS-CoV-2 vaccine 18 days prior to symptom onset. A clinical neurological exam on admission revealed blotchy scotoma in the left eye exclusively. Cerebral MRI revealed an acute cortical ischemic lesion in the territory of the right posterior cerebral artery (PCA) (Figure ). Perimetry disclosed patchy visual field deficits in the left upper quadrant. Work-up for the etiology of the stroke excluded venous sinus thrombosis (VST; normal D-dimer, normal venography), cerebral vasculitis, antiphospholipid antibody syndrome, arterial hypertension, hyperlipidemia, diabetes, and atrial fibrillation. EEG revealed focal slowing and epileptiform discharges over the right occipital projections. RCVS was suspected and the patient was treated with an anti-seizure drug and nimodipine. CT angiography, seven days after admission, revealed a normal-sized right PCA, which was physiologically filled via the basilary artery, confirming the diagnosis RCVS. The patient was discharged nine days after admission with only mild visual impairment with a treatment of levetiracetam (1000 mg/d) and nimodipine (90 mg/d for another five weeks).


An 11-year-old boy, weighing 50 kg, poorly commutative with a history of autism spectrum disorder (ASD), learning disorder, and attention-deficit hyperactivity disorder (ADHD), was brought to our ED with a pencil embedded in his right foot. His full-scale intelligence quotient (FSIQ) was 76, and his intellectual function is further impaired by an inability to concentrate at times. He, apparently, was feeling testy and irritated at school, so he began to stab at his shoe repeatedly with a pencil, resulting in the pencil piercing through the sole of the shoe and impaling his foot. His vital signs on arrival at the ED were normal. The broken portion of the pencil was completely embedded in his right foot, with only mild bleeding and soft tissue trauma (Figure ). The pencil could not be extracted readily because it was completely embedded within the foot, with the outer edge nearly flush with the skin, and he complained of significant pain. Plain film radiograph (X-ray) of the right foot revealed a stick-like foreign body in the right foot (Figure ), although the anterior (AP) view did not show the foreign body (Figure ). The patient was anesthetized with 30 mg ketamine hydrochloride administered intravenously to allow for removal of the pencil. However, the pencil could not be extracted easily because it was embedded and wedged deeply, and only a small fragment could be removed with mosquito forceps (Figure ). The pencil was then twisted in a gentle back-and-forth motion, which successfully freed up the large piece of the broken pencil (Figure ). After confirming that the tip of the removed pencil was intact, thorough lavage of the site was performed. Ultrasound of the right foot did not show evidence of any pencil remnant. The boy was able to walk without pain or disability at his follow-up in clinic one week later.


A 23-year-old woman was diagnosed with severe and extensive fungal sinusitis as a complication of root canal therapy of the left upper molar tooth. She was posted for functional endoscopic sinus surgery (FESS) for management of her extensive fungal infection that caused destructive soft tissue lesions in the left maxillary, ethmoidal and sphenoid sinus with the destruction of the left orbit floor. FESS was done under general anesthesia with a regular endotracheal tube and pharyngeal pack inserted by an anesthesiologist. The regular pharyngeal pack was unavailable, so packing was made using four pieces of unfolded gauze (size 4 by 4 inches) that was tied end to end making a modified pack of 120 cm length as shown in Figure .
A documentation of the pharyngeal pack placement and removal was missed during the surgery. Upon the take over of the next anesthesia team , the presence of the pack was not signed over. Before extubation, the pharyngeal pack was checked for by direct laryngoscopy twice, but it was not visualized. Due to lack of documentation, the presence of a pharyngeal pack by the second anesthesia team was missed. The patient was extubated and moved to the post-anesthesia care unit (PACU). After discussion with the first anesthesia team, an alert about insertion of a modified pharyngeal pack was made. An x-ray was deferred as the modified pharyngeal pack lacks a radiopaque line and hence it would not be visualized on a plain radiograph. As the pack was not visualized in the nasopharynx the possibility of migration into the gastrointestinal tract was considered. An emergency esophago-gastro-duodenoscopy (OGD) was performed under sedation and the pack was visualized in the stomach (Figure ). Pharyngeal pack was extracted eventually endoscopically, and the patient remained clinically stable and had an uneventful recovery after surgery.


A 42-year-old female presented with enlarged lymphadenopathy and a clinically suspicious painful lump in the right breast. The patient reported no family history of breast or ovarian cancer. The mammogram revealed diffusely spread microcalcification in the right breast (Figure ). Breast ultrasound showed diffuse hypoechoic abnormality occupying the entire right breast, with several abnormal ipsilateral axillary lymph nodes (Figure ). In addition, there was a 7 mm indeterminate lesion in the left breast upper outer quadrant (UOQ) area (Figure ). A breast magnetic resonance imaging (MRI) confirmed these radiological findings (Figure ). The bilateral breast core biopsy was consistent with grade I invasive lobular carcinoma with ER8, PR8, and Ki-67 of 10% (Figure ). The human epidermal growth factor receptor 2 (HER2) was borderline (Figure ). However, fluorescence in situ hybridization (FISH) was not amplified, and the right axillary lymph node biopsy showed metastatic disease (Figure ).
The staging computed tomography (CT) revealed prominent supraclavicular, bilateral axillary, and para-aortic lymph nodes in addition to multiple lytic and sclerotic lesions in the thoracic spine and pelvic bones (Figure ). A whole-body bone scan showed increased uptake in the left side of the pelvis, upper left femur, right scapula, skull, and ribs, suggestive of metastasis (Figure ), and an MRI showed lytic lesions in the surgical neck of both femurs. A multidisciplinary team advised hormonal manipulation using tamoxifen and bisphosphonate (zoledronic acid).
In addition, the patient underwent palliative radiotherapy to the left proximal femur and pelvis. After nine months and due to disease progression, her treatment was switched to letrozole and Zoladex. The treatment goal for metastatic breast cancer in this patient was to maintain a reasonable quality of life.
Due to stable disease and to avoid the side effects of Zoladex, bilateral salpingo-oophorectomy was advised and performed. The post-operative histology showed widespread infiltration by malignant cells involving both ovaries. Immunohistochemistry was positive for mouse monoclonal anti-cytokeratin, pan antibody (MNF116), Cam5.2, cytokeratin 7 (CK7), gross cystic disease fluid protein 15 (GCDFP-15), and GATA binding protein 3 (GATA-3); however, it was negative for ER, progesterone receptor (PR), E-cadherin, CK20, cancer antigen 125 (CA125), and thyroid transcription factor-1 (TTF-1). HER2 was borderline and FISH was not amplified. The findings confirm the diagnosis of metastatic pleomorphic lobular carcinoma originating from the breast.


A 46-year-old woman suffered multiple pelvic fractures in March 2016. In November 2016 she was referred to the pain unit presenting chronic low back pain for more than 1 year, worse since the accident (vehicle collision), and CPP for more than 6 months. The latter was comprised of a somatic pain localized on the pelvic girdle and a neuropathic pain based on the association of paresthesia in both groins and legs (L2 dermatome on the ventral side). There were also additional lancinating symptoms when urinating. Mean visual analogue scale (VAS) on her baseline pain intensity was 7/10, and increased to 10/10 in movement. The patient presented severe gait problems and a reactive depression. There was a relevant deterioration in the patient's quality of life (QoL).
Computed tomography and lumbosacral nuclear magnetic resonance imaging revealed pelvic fractures (left ilio-pubic branch fracture, a consolidated left ischiopubic branch fracture, an oblique fracture that affected the right ischiopubic branch to the pubic symphysis) in addition to a severe lumbar facet arthrosis. Sphincter abnormalities were not detected. There was evidence of less amplitude in the left femoral nerve, possibly secondary to muscle hypotrophy due to analgesic disuse, and also a demyelinating neuropathy in the left pudendal nerve.
Pharmacologic treatment consisted of the combination of non-steroidal anti-inflammatory (celecoxib 600 mg per day), antidepressant (fluoxetine 30 mg per day), anticonvulsant (gabapentin 1800 mg per day), and oral morphine 90 mg equivalents per day. Pain meant that this therapy remained refractory.
Three caudal epidural blocks were performed, with a reported 20% of pain relief. The patient subsequently underwent infiltration of both third sacral roots and also the ganglion impar with attainment of less than 50% pain relief.
SCS was proposed to the patient in January 2019 to reduce lower lumbar spine pain and neuropathic pain in both legs. Spectra WaveWriter System (Boston Scientific) was selected because of its multiple stimulation waveforms and field shapes.
After psychiatric evaluation, the procedure was performed in April 2019. Access to the posterior epidural space was at L1-L2 level. Two octopolar linear leads were placed over the bilateral T8 and T9 (Fig. ). Three-dimensional neural targeting programming facilitated the optimal location for leads by attaining appropriate anatomic coverage of the pain area (lower back and legs – dermatome L2 ventral side).
After 2 weeks, the patient achieved 80% pain relief, even with CPP symptoms. Opioids were also withdrawn. The patient was implanted with the leads, which were placed in the same location.
At the time of writing this manuscript, the patient was approximately 18 months postimplant. VAS was reported as 3/10 and several medications have been weaned off.
Her QoL was also assessed using the EuroQOL-5D (EQ-5D) test. In 2016, the EQ-5D resulted in –0.0757, meaning “worse than death”. Nowadays, the EQ-5D value is +0.6454, and the patient reports 80% pain relief (VAS 3/10). Medical treatment has been weaned off, and QoL improved.
The patient selected program number 7 (Fig. ) from 16 programs provided by the Spectra system. Therapy consisted of a tonic stimulation whose paresthesia covers the entire area of pain including the lower back, pelvic region, and both legs. This program was combined simultaneously with a 1000 Hz contour high frequency tonic stimulation.


A 48-year-old female complained of defective vision in the right eye immediately after drainage of liver abscess. Her best corrected visual acuity was 8/400 in the right eye and 20/20 in the left eye. There was no evidence of anterior chamber cells or keratic precipitates, and the intraocular pressure was normal. Dense vitreous opacities obscured the visualization of fundus of the right eye. Chorioretinitis with exudation and hemorrhage were present in the macular and inferonasal peripheral retina (Figures and ). The fundus of the left eye is normal (). Optical coherence tomography (OCT) showed epiretinal membrane on the inner retinal surface of macular foveal and pigment epithelial detachment (PED) (Figures and ). The patient's blood culture was positive for Klebsiella pneumoniae. Rheumatoid factor (RF), antistreptolysin antibody (ASO), human immunodeficiency virus (HIV), TP particle agglutination assay, and purified protein derivative (PPD) tuberculin skin test were all negative. The possibility of endogenous endophthalmitis was considered in ophthalmologic consultation. Taking into account the patient's poor general condition, systemic cephalosporin therapy combined with periocular triamcinolone injection was given to her.
Four months later, the patient returned to the ophthalmology department with stable condition after the antibiotic therapy. Her BCVA was 16/400 and vitreous inflammatory cells in the right eye were decreased; flaky yellowish-white elevated lesions surrounded by hemorrhages can be visualized in the macular and inferonasal peripheral retina. The crooked retinal arterioles and retinal venules extended into the outer nuclear layers of the macula of the right eye (). OCT showed that a dense epiretinal membrane was attached in the inner surface of the retina, hyperreflective materials were located at the level of the outer retinal layers, and vascularized PED was found in the macular fovea of the right eye (Figures and ). Optical coherence tomography angiography (OCTA) detected abnormal vascular networks in the superficial, deep retinal capillary plexus/inner retina, outer retina, and choroidal layers (Figures –). Fundus fluorescein angiography (FFA) showed that an intraretinal vascular complex was formed by retinal artery and retinal vein anastomosis in the macular, and a hyperfluorescent lesion was detected at the inferonasal peripheral retina of the right eye (Figures –). During the course of the fluorescein angiography, the vascular complex, abnormal dilation of the capillaries, and hyperfluorescent lesion had progressively increased levels of leaked dye. Vitreous humor testing was performed, and the results showed that interleukin-6 (IL-6) was 33 mg/ml, which was slightly higher than normal, suggesting that intraocular inflammation was present. However, polymerase chain reaction (PCR) showed that the pathogen was negative. The ratio of interleukin-10 (IL-10) and IL-6 was less than 1, indicating that the diagnosis of lymphoma was not supportive. Based on the symptoms and clinic tests, the clinical diagnosis was endophthalmitis with RAP. Considering that the patient had an infection history and presented chorioretinitis, empirical treatments such as intravitreal vancomycin injection combined with periocular triamcinolone injection were recommended. After treatment, her vitreous inflammatory cells of the right eye were decreased; the patient's BCVA was improved to 20/400.
Five months later, the inflammation was under control (), but her RAP was progressed and resulted in serious damage in her central vision. Her BCVA was decreased to count finger (CF/30 cm). OCT showed that intra- and subretinal fluids were increased (Figures and ). Areas of abnormal vascular network on OCTA were enlarged (Figures –). Anti-VEGF therapy was given to her. After three intravitreal injections of ranibizumab (once a month), the lesions shrunk compared to before the VEGF treatment (Figures and ). OCT revealed that sub- and intraretinal fluids were partly absorbed than before in the right eye (Figures and ). The patient's BCVA was improved to 5/400.


A 40-year-old woman has been hospitalized in our department of infectious diseases in February 2018 for fever and cough. Her medical history included CMC since 7 years of age which had never been explored, and esophageal candidiasis in 2015 showing recurrence despite treatment with fluconazole. She had a 3-year-old healthy boy, and no other case was reported in her family.
Upon admission, the patient was diagnosed with pneumonia associated with bronchiectasis via clinical and radiological examinations. Microbiological examination revealed Streptococcus pneumoniae in the sputum. On clinical exam, marked oral candidiasis and diffuse inflammatory papules on the face associated with bilateral blepharitis were noted (). The patient was successfully treated with ceftriaxone but experienced another episode of pneumonia in June 2018, which resolved after further treatment with ceftriaxone. The patient denied receiving any treatment for CMC.
A direct examination of the eyelashes revealed the presence of Demodex folliculorum (, ). Skin biopsy also revealed non-granulomatous peripilar inflammation with the presence of numerous Demodex within the follicles (). A diagnosis of florid rosacea-like demodicosis was therefore established. Treatment with oral doxycycline (100 mg/day) led to the partial improvement of the lesions. Similarly, oral ivermectin (200 µg/kg, thrice a week) in combination with a daily topical application of ivermectin led to the partial improvement of the lesions. Finally, lasting remission was achieved with the resumption of doxycycline in combination with the local application of 1.5% metronidazole.


A 44 years old man electrician by profession, right-handed, with no particular pathological history. was brought by his coworkers to our emergency department after he had an accident at work by electrocution to a high voltage current with direct cranial contact point at the vertex, causing an initial loss of consciousness without associated head trauma. There is no history suggestive of any mental or physical illness.
At admission, his GCS scores were E4V5M6. Neurological examination revealed muscle power was 3/5 on right side, 5/5 on left side, no sensitive deficiency with 2nd and 3rd degree burns on his face and scalp (, ). The rest of the exam was normal.
A cerebral CT scan was performed at the ER 4 hours after the accident objectifying an extradural hematoma of the vertex more marked on the left associated with a left frontal pneumocephaly there was no sign of trauma the bone structure was intact (, ).
The diagnosis of extradural hematoma was obvious. We hospitalized the patient in the neurosurgical intensive care unit after being conditioned, the patient underwent a preoperative assessment, then was sent for surgical management. The surgery was performed under general anesthesia by our chief resident. After removal the bone flap we discovered an extradural hematoma which was evacuated, the origin of the bleeding was the superior longitudinal sinus there were no fracture in the bone.
On the follow-up, there was total recovery from the motor deficiency, conscious; the control CT scan was satisfying (). The patient was later transferred to the burn center for additional care. the outcome was unfavorable the patient was in acute renal failure due to rhabdomyolysis associated with a lung infection unfortunately the patient developed septic shock which was the cause of his death.
This case has been reported in line with the 2020 SCARE guidelines [].


A 56-year-old male with a past medical history of diabetes mellitus and bilateral knee osteoarthritis, presented to the emergency department with severe abdominal pain for five hours, radiating to the back, associated with nausea and five episodes of non-bloody, non-bilious vomiting. He otherwise denied any other significant symptoms. He reported tobacco smoking of 25 pack-years, social drinking 1-2 beers on weekends, and denied any illicit drug use. The patient also denied any significant family history. On presentation, he was afebrile, hemodynamically stable, awake, alert, and oriented; however, on physical examination, he had abdominal distension and generalized tenderness. Lab work was remarkable for leukocytosis, with white blood cells (WBC) 14.7 k/ul (normal range: 4.8-10.8 K/UL), hemoglobin (Hb) 16.5 gm/dl (normal range: 12-17 gm/dl) with haematocrit (Hct) 49.8 % (normal range: 37-51%), creatinine 1.6 mg/dl (normal range: 0.7-1.2 mg/dl), blood urea nitrogen (BUN) 27 mg/dl (normal range: 8-20 mg/dl) and lipase 4200 U/L (normal range 0-160 U/L). The patient was admitted to the medical floors for IV fluids and analgesic therapy for acute pancreatitis management. Further studies were done, showing unremarkable lipid panel with normal liver function tests. As seen in Figure & 2, gallbladder ultrasound showed no gallstones or common bile duct (CBD) dilatation, respectively.
CT abdomen showed acute peripancreatic inflammatory changes as seen in Figure , supporting the diagnosis of acute pancreatitis.
Upon further interrogation, the patient reported recent daily use of different kinds of herbs including fenugreek, turmeric, and Gymnema, to decrease his weight and blood sugar. Throughout the hospital stay, the patient's clinical status improved and he tolerated oral intake. He was recommended to stop taking the aforementioned herbs and discharged with instructions to follow up outpatient.


An 8-month-old girl was admitted to our hospital because of a 6-day history of fever and cough. Shortness of breath lasted for 2 days. The infant is the second child of a nonconsanguineous Chinese family and has a healthy older brother. The baby was born at full term and had no history of asphyxia due to birth injury. She had a history of whooping cough and pneumonia at the ages of 4 and 6 months, respectively. There was no history of exposure to endemic water and schistosomiasis sources. Her parents and brother were clinically normal, and their family history of hereditary diseases was denied. This study was approved by the Medical Ethics Committee of Hunan Provincial People’s Hospital. Written informed consent to participate in this study was provided by the legal guardian.


The index patient was a 57-year-old male who presented with vague, intermittent, recurrent abdominal pain. The pain evolved into a worsening epigastric pain radiating to the back with associated vomiting. The supine plain radiograph of the abdomen () demonstrated a well-circumscribed mass projected in the left upper quadrant of the abdomen. Free intraperitoneal air was excluded on the erect radiograph (not shown). Additionally, ultrasound excluded cholelithiasis, peripancreatic fluid, a left renal lesion and obstructive uropathy. Markers of infection were minimally increased. Amylase levels were normal.
Given the presence of a left upper quadrant mass on plain radiography that was suboptimally appreciated on ultrasound, computed tomography (CT) was requested. Multiphase contrasted CT imaging was performed with prior administration of oral contrast (). A cluster of jejunal bowel loops was noted within a sac in the left upper quadrant of the abdomen. Dilatation of the proximal duodenum and jejunal loops within the sac was evident with loss of the normal duodenal and jejunal configuration. Failure of transit of oral contrast into the terminal ileum was noted. Engorgement of the mesenteric vessels entering the hernial sac was visualised with surrounding fat stranding. There were no features of pneumatosis intestinalis. The inferior mesenteric vein and the ascending branch of the left colic artery were seen along the anterior margin of the hernial sac (). These collective findings were in keeping with a left PDH and bowel obstruction.
Intraoperative findings () confirmed the congenital defect (white arrow), presence of fossa of Landzert, and jejunal bowel loops within the left PDH. The patient recovered without further complications or recurrence of symptoms.