A 43-year-old female presented to our hospital with the complaints of progressive pain abdomen for 3 months. Abdominal pain was unrelated to food intake and was constant, dull-aching in nature. No lump was palpable on clinical examination and the abdomen was non-tender. Laboratory investigations including hematological and liver function tests were normal. On the basis of the above physical assessment, the patient was referred for cross-sectional imaging- sonography of the abdomen, to evaluate the cause of pain. The investigation revealed a hypoechoic retroperitoneal mass sandwiched between the pancreas anteriorly and the aortocaval region posteriorly, encasing the origin of common hepatic artery (). The patient further underwent a contrast-enhanced computed tomography (CECT) scan on a 64-slice CT scanner (Discovery750HD, General Electric, GE Healthcare, USA) to characterize the mass. CECT was performed by injecting 100 mL of intravenous contrast iomoperol (Iomeron, Bracco, Germany) using a pressure injector with bolus tracking technique and a dynamic triple phase study through an 18-gauge needle in the ante-cubital vein at a rate of 4 mL/s. In addition to a baseline non-contrast study, images were acquired during arterial phase (20 s), venous phase (70 s) and equilibrium phase (180 s). Thin reformatted and multiplanar images were studied before arriving at a differential diagnosis for the mass. The above CECT scan revealed a heterogeneously enhancing, lobulated solid mass lesion in the retroperitoneum with internal non-enhancing liquefied/necrotic areas anterior to the aortocaval region. The enhancement was persistent but equivalent to the phase of the dynamic study. The mass was seen to encase the celiac axis and its branches at their origin with extension along the common hepatic artery (CHA) for a small segment after origin () and along the hepatoduodenal ligament towards porta hepatis. It was seen to abut and partially encase the superior mesenteric artery (SMA) with circumferential contact of 180° () as well as compress and displace the main portal vein (). The mass was seen to cross the mid line towards the left side in the pre-aortic location (). Mass effect on the common duct was present with resultant minimal central bilobar intrahepatic biliary radicles dilatation. Fat planes with other neighboring structures such as caudate lobe of liver, right adrenal gland and the inferior vena cava (IVC) were maintained. There was no evidence of calcification within the tumor bulk. Based on the above features of soft tissue extension and insinuation along the retroperitoneal structures and partial encasement without obvious thrombosis of the affected vessels within the abdomen, a differential diagnosis of lymphoma or retroperitoneal mesenchymal tumor was made.
Surgical management, by resection was ruled out due to the extensive involvement of vital vascular structures within the retroperitoneum by the mass. Fine needle aspiration using endoscopic ultrasound (EUS) guidance was performed for tissue diagnosis which revealed malignant spindle cells on a hemorrhagic background (). Further cell block confirmed spindle cell tumor which was analyzed by immunohistochemistry (IHC) showing SMA negative and CD117 positive tumor cells. A histopathological diagnosis of malignant spindle cell tumor, likely, EGIST was made.
The patient was treated with imatinib mesylate for 3 months with regular follow-up at the hospital; however, she did not respond to therapy and succumbed to the rapid disease progression after 3 months.


A 50-year-old man has presented with enlarged gums in the upper and lower front jaws for 2 months. His medical history was significant for hypertension and diabetes mellitus type 2. He denied any bleeding from the gums, loosening of the teeth, use of any prosthesis, or dental braces. His medications included enalapril 10 mg bid (for 2 years), amlodipine 10 mg per day (for 6 months), sitagliptin 100 mg per day (for 1 year), and glipizide 20 mg/day (for 4 years). Oral examination revealed marginal and interdental gingival enlargement, predominantly involving maxillary and mandibular anterior teeth (Fig. ). The enlargement was firm, nontender with no bleeding on probing. Poor dental hygiene was noted. HIV and leukemia were excluded by appropriate laboratory testing. We diagnosed the patient with amlodipine-induced gingival enlargement, and he showed slight but notable improvement in 4 weeks after switching amlodipine to long-acting thiazide diuretic (Fig. ). He was also referred to see a dental hygienist.
The incidence of this side effect varies among different calcium channel blockers, for example, nifedipine (6.3%), amlodipine (1.7%), and diltiazem (2.2%) , . It may take from 1 to 8 weeks for resolution of gingival overgrowth after discontinuing the offending agent .


A 28-year-old male patient with the complaints of leg weakness, headache, refractory constipation, and numbness in the legs was initially seen abroad. A mass was detected between L3 and S2 on an MRI scan, and the patient underwent two operations under general anesthesia. Nonetheless, over time, his symptoms became more severe, and he was admitted to our clinic. The first pathological report from the foreign clinic was insufficient material, and the second reported as a malignant tumor. In the MRI scans, a contrast-enhancing intradural mass between L3 and S2 causing contrast enhancement of bony tissues was detected []. The patient was operated on under standard conditions with neuromonitoring. While in a prone position, a midline skin incision was made. A needle biopsy (Bx) was performed from a 1-cm area in the sacral region, which was disproportionate to the size of the previous skin incision. No laminectomy had been performed along the length of the tumor. Hence, a laminectomy was performed without damaging the facets from L2 until the interior end of S2 and the dura was reached. The dura was cut through the midline, pads were placed around the tumor, and the tumor was suspended laterally. Hemorrhagic dark-colored tumor tissue that was soft and fragile with dirty-gray-colored regionswas removed from between the fibers of the cauda from the upper margin of the end of the cord to the lower margin of S2 []. The primary dural closure was performed using hemostasis and SF. No abnormalities were detected in theneuromonitoring records []. At a postoperative follow-up, the patient could move his four extremities and had a GCS score of 15. On postoperative day 1, the wound was clean, headache relieved, and the patient could defecate comfortably. The patient was then discharged from the hospital and followed-up as an outpatient. Histopathological assessment of the Bx indicated that the tumor was an oligodendroglioma; the tissue had highcellularityand composed of two types of cell components with well-defined cell borders, friedegg-like cells showing round cell nuclei, aclear, and slightly basophilic cytoplasm. The nuclei were mildly atypical, and mitotic figures were scarce. No capillary clusters or plexiform capillaries were observed. Hematoxylin and Eosin staining demonstrated typical oligodendroglioma, which could be classified as the WHO classification Grade II. Immunohistochemical staining of the cytoplasm was negative, except for focal positivity for glial fibrillary acidic protein (GFAP). A low proliferation index (<5%) was observed using Ki-67 immunohistochemistry [].
The possibility of seeding was considered and brain, cervical, and MRI were performed for the brain and cervical and thoracic spinal cord. No pathologies were encountered []. Considering the current diagnosis of the patient, and that there were only sixty other cases in the literature, specialist radiotherapists were consulted. A 45 Gy dose of RT was applied. The patient had no complaints during follow-up other than occasional lower back pain. The patient's early control lumbosacral MRI scan revealed that the tumor was resected and, apart from a small contrast-enhanced region in the vicinity of S2, typical postoperative changes were seen []. In the patient's 6-month and 15-month control MRIimages, apart from a contrast-enhanced S2 region with changes in bone density, there were no radiological or clinical pathological findings [Figures and ].


The subject was a 70-year-old woman with RP and an ongoing history of posterior subcapsular cataract and visual discomfort from glare. The subject was targeted to receive palucorcel at a dose of 3.0×105 cells; however, the actual dose received was 2.22×105 cells, which was within specifications. A fluid–air exchange was not performed; however, a small air bubble remained at the retinotomy site opening at the completion of the injection and acted like a ball valve ().
We first noted the formation of the ERM and RD in this subject at 26 days after the administration of palucorcel. RD was considered related to the transvitreal delivery procedure and probably related to the treatment (palucorcel). Fundus photographs of the subject’s eye prior to the procedure are shown in , and fundus photographs at 5 weeks after surgery (showing the RD and ERM) are shown in .


A 46-year-old woman presented with spontaneous bruises and pancytopenia in 2008. Complete Blood Counts (CBC) measured showed a hemoglobin concentration of 10.7 g/dL, a leukocyte count of 1.2×106/mL (with differential of 30% neutrophils, 40% lymphocytes and 30% blasts), and a platelet count of 18×106/mL (). Screen coagulation was normal. Peripheral blood smear and bone marrow aspiration were suggestive of an infiltration of 90% of abnormal promyelocytes and blasts (). Bone marrow flow cytometry analysis confirmed the aberrant population (77.5% of viable cells) immunophenotyped as CD33+|CD45+ |CD117mid with a CD13+ homogeneous scattered pattern and MPO+, and negative for HLA-DR, CD34, CD123, CD14, CD15, CD64, CD42A, and GPA ( and data not shown). Cytogenetic analyses revealed t(15; 17) and PML/RARA rearrangement, without identifying other secondary genetic lesions. She completed chemotherapy treatment according to the Spanish Program of Treatments in Hematology (PETHEMA) 2005 protocol for patients with intermediate risk APL (induction with ATRA and Idarubicin, three cycles of consolidation and maintenance with Mercaptopurine, methotrexate and ATRA), after which complete hematological and molecular remission (CR) was observed ().
In September 2010, the patient presented with leucopenia and thrombocytopenia (). Cytology and flow cytometry of bone marrow aspirate showed 7% of blasts with dysplasia AREB-II versus AML that were immunophenotyped as CD117+|CD45+|CD33++ and negative for CD34 and HLA-DR, suggestive of an early stage of relapse (). The main differential diagnosis was established between a relapse of APL or a different subtype of AML secondary to treatment (t-AML). APL was discarded since t(15; 17) was not detected. A t-AML relapse was confirmed on peripheral blood smear cytology two weeks later () and bone marrow aspirate containing 70% of blasts. From the karyotype, a 46XX cell clone containing rearrangements on the MLL gene was identified, with breakpoints in chromosomes 20q12 and 11q23, corresponding to unfavorable European Leukemia NET (ELN) Score. With a diagnosis of t-AML in a patient with previous history of treated APL, she received two cycles of chemotherapy with Idarubicin and Ara-C (7+3 induction regimen), reaching CR with negative minimal residual disease (MRD), and without MLL gene rearrangements, which she maintained after consolidation with Ara-C at high doses. In March 2011, allogenic hematopoietic cell transplantation (HCT) was performed ().
After four years suffering serious symptoms related to chronic skin (grade II) and hepatic (grade III) GvHD, she presented with pancytopenia and a suspicion of relapse of t-AML in April 2015, later confirmed on bone marrow cytologic and immunophenotypic studies. She deceased due to a septic shock of respiratory origin before further treatment could be initiated.


Patient 1 was a 55-year-old Caucasian female initially diagnosed with T2DM in 2005 with an A1C of 8.9%. She was referred to OEC after presenting to the ED with nausea and vomiting in January 2015. She was diagnosed with DKA and admitted to the intensive care unit (ICU). After obtaining a medication history at OEC, it was discovered that monotherapy with canagliflozin was initiated 2 months before the DKA episode. The Naranjo score for this adverse drug reaction (ADR) was possible. After resolution of DKA, insulin detemir and insulin lispro were started while canagliflozin was discontinued. In the following months, metformin, linagliptin, and empagliflozin were gradually initiated in addition to insulin. The patient was diagnosed with latent autoimmune diabetes in adults (LADA) based on a C-peptide of 0.4 ng/mL in April 2015. The patient's average self-monitoring blood glucose decreased from 205 mg/dL to 155 mg/dL in 5 months with a slight improvement in A1C to 8.6%. The patient has not had another episode of DKA.


Patient 2 was a 47-year-old Caucasian female initially diagnosed with T2DM in 2005 and her most recent A1C was 9.2%. She presented to the ED with DKA (blood glucose = 459 mg/dL CO2= 5, and pH = 6.9) in February 2014. On presentation of DKA, the patient was receiving metformin ER, liraglutide, and canagliflozin. Canagliflozin and fluconazole were added 6 days before ED presentation. The Naranjo score for this ADR was possible. In the ED, the patient was diagnosed with an underlying septic pneumonia. The patient was discharged on multi-daily insulin monotherapy with an A1C = 8% 2 months after discharge. Sitagliptin and metformin were added before the patient was lost-to-follow-up.


Patient 3 was a 56-year-old African–American female diagnosed with T2DM for an unknown duration with an A1C = 8.2%. The patient was admitted to the ICU with DKA in June 2014 after being started on dapagliflozin 2 months ago. The Naranjo score for this ADR was possible. The patient was also treated with glipizide and insulin glargine before admission. In the ED, the patient was diagnosed with an underlying electrolyte abnormality due to Fanconi syndrome. After resolution of DKA and discontinuation of dapagliflozin, the patient was started on saxagliptin.


A 3-year-old girl with SMA type I was referred to us because of dyspnea. She was the second child of non-consanguineous and healthy parents. The pregnancy was uneventful. She was born after 41 weeks of gestation by normal spontaneous vaginal delivery. Birth weight (3660 g), length (51 cm), and head circumference (36 cm) were within normal range. There was no family history of neuromuscular disorders, neurologic disorders, or congenital malformations. Both her vital signs and physical examination were normal. During the first months of life the child presented poorfeeding. At 1 months of age neurologic examination of the patient revealed tongue fasciculations, symmetric muscle weakness, generalized hypotonia and areflexia. Laboratory analyses showed normal complete blood counts, liver and kidney function tests and serum electrolytes. Creatine kinase levels were 344 U/L and serum lactate dehydrogenase levels were 676 U/L. Electromyography presented neurogenic changes, no spontaneous activity, and a markedly reduced innervation pattern more pronounced in distal muscle groups and in the lower extremities. Genetic analysis revealed a homozygous deletion of the SMN1 gene (exon 7). Therefore the diagnosis of SMA type I was made. During the following 3 years of life, the child performed a multidisciplinary disease’s specific followup, including chest X-ray, overnight oximetry, gastrointestinal, nutrition and orthopedic evaluations in order to prevent the complications of weakness in childhood spinal muscular atrophy such as respiratory, due to restrictive lung disease; gastrointestinal, in terms of dysphagia and constipation; and orthopedic, with progressive deformities. The child presented dyspnea, therefore she was referred to our pediatric pulmonary clinic by her pediatrician. On physical examination, the child was dyspneic and irritable; pulmonary auscultation revealed an asymmetry of breath sounds between the 2 hemithoraces, owing to decreased breath sounds in the right hemithorax. Oxygen saturation was 94% to 96% on room air. Therefore, a chest radiography was immediately performed. The results revealed a right pneumonia (). Laboratory analyses showed normal complete blood counts, such as liver and kidney function tests, serum electrolytes and C-reactive protein serum value. On the basis of the clinical, radiological and laboratory findings, the initial working diagnosis was a suspected mucus accumulation in the right hemithorax. Therefore, we started a treatment with Free Aspire. The child performed the treatment for 20 minutes three times a day. During the following days both her vital signs and physical examination returned normal. The oxygen saturation was always maintained at greater than 92%, and the objectivity of lung during the following days showed a clear improvement; no asymmetry of breath sounds between the two hemithoraces were found. Within a few days, we repeated chest radiography, and a significant change was found in comparison with the previous result. No right pneumonia was present as shown in . Subsequently the girl was discharged with a normal physical examination and X-ray study result.


A 21-year-old primigravida underwent an anomaly scan at 19 weeks of gestation. Her medical and surgical history was unremarkable. Her husband had NPS which was proven by mutation study. He was visually challenged secondary to congenital glaucoma and had undergone renal transplantation for end-stage renal disease due to focal segmental glomeruloscelrosis. He had antecubital pterygium; difficulty in abducting the shoulders; bilateral small and dislocatable patella; swan neck deformity of fingers; triangular lunules in the thumbs; dystrophy and longitudinal ridging in both thumb nails and absent skin creases on distal interphalangeal joints of the second, third and fourth fingers, and bilateral iliac horns.
Ultrasound evaluation showed a single live fetus with parameters corresponding to 19 weeks of gestation. The orientation of the long bones of the lower limbs was abnormal. There was rotational defect at the knee joint in both the lower limbs and feet were pointing towards the gluteal region. The extension at the knee joints was restricted [ and Videos , ]. Throughout the examination both the upper limbs were flexed at the elbow joint with limited extension in between []. A three-dimensional (3D) ultrasound scan was undertaken to better delineate the extent of the limb anomaly, which showed a clearer picture of the abnormal position of the limbs. The upper limb was kept flexed at the elbow and in the lower limbs the feet were pointing towards the gluteal region [Figures and ]. Based on these ultrasound findings the clinical diagnosis was NPS. When counseled regarding the findings, the couple opted for termination.
Post-abortal examination and infantogram confirmed the antenatal findings. In the lower limbs, there was a rotational defect at the knee joint and the feet were pointing posteriorly. The elbows could not be extended beyond 90° due to soft tissue contracture. In the infantogram, the tibia was seen laterally with a medial fibula due to torsion at the knee joint [].


A 69-year-old female patient, Eastern Cooperative Oncology Group performance status 2, with no history of autoimmune disorders, was treated with nivolumab as 3rd-line therapy for advanced lung adenocarcinoma with pleural dissemination and asymptomatic brain metastases. She complained of general malaise and double vision 1 week after her 3rd cycle of nivolumab administration and was subsequently admitted to our hospital because of progressive worsening of her symptoms.
On the 1st hospital day, diffuse ST segment elevation was found on electrocardiography, and her serum creatine kinase level was 1,156 U/L (normal range 41–153 U/L), with concurrent elevation of creatine kinase-MB isoenzyme and troponin I levels. She had no typical symptoms of angina pectoris, and ultrasound cardiography demonstrated good left ventricular ejection fraction with no segmental hypokynesis or pericardial effusion. Emergent coronary angiography and left ventriculography showed no abnormalities, thus excluding possible myocardial infarction. We therefore suspected immune-related myocarditis induced by nivolumab. After myocardial biopsy, pulse methylprednisolone was initiated at a dose of 1,000 mg for 3 days followed by 1 mg/kg/day.
On the 2nd hospital day, the patient suddenly complained of dyspnea due to congestive heart failure, requiring temporary noninvasive positive pressure ventilation in the intensive care unit. She showed decreased blood pressure with complete atrioventricular block and multifocal premature ventricular contractions on electrocardiography, and a temporary cardiac pacemaker was therefore implanted simultaneously. She gradually improved under these supportive-care measures and continued steroid therapy, and the pacemaker was removed on the 7th hospital day.
Pathological examination of a myocardial biopsy demonstrated lymphocytic and neutrophilic infiltrations, interstitial edema, and myocardial necrosis. Moreover, immunohistochemistry demonstrated CD8 and CD4 T cells within the myocardial tissue, as well as expression of HLA-ABC and HLA-DR antigens on the myocardial cells, suggesting an immune response on the basis of HLA associations (Fig. ). Serological and in situ analyses for cardiotropic viruses were negative.
Regarding the impaired adduction of her left eye, positivity for serum acetylcholine receptor antibody suggested MG. Furthermore, magnetic resonance imaging showed neither any new lesion in the brain nor the preexisting lesion in the medial longitudinal fasciculus. The patient was able to walk after rehabilitation training and continued steroid therapy, and left our hospital on the 36th hospital day.


A 19-year-old man complained about gynecomastia and the presence of a testicular lesion. In his recent pathological history the patient reported testicular inflammation 1 year earlier. Physical examination revealed a minimum increase of breast tissue bilaterally and centimetric mass in the right testis. The left testis was found to be normal.
Scrotal ultrasound revealed a solid testicular mass (18 × 13 mm) with nonhomogeneous ecotexture and calcifications. Abdominal and thorax computed tomography (CT) (Fig. ) revealed bulky abdominal and mediastinal lymphadenopathies (maximum diameter 44 mm).
High levels of lactate dehydrogenase (LDH) 835 mg/dL (normally 260–550 mg/dL) and α-fetoprotein (AFP) 306 ng/mL (normally <10 ng/mL) were found in the blood tests, while beta-human chorionic gonadotrophin hormone (b-hCG) 0.5 mIU/mL (normally <4 mlU/mL) was within normal limits.
On September 22, 2016, the patient underwent right orchiectomy, and histological specimens revealed testicular GCTs of 17 mm composed of mature teratoma (85%) and immature teratoma (15%). There was no other histological type of tumor cells, lymphovascular and rete testis invasion, pT1. The histopathological examination showed interstitial fibrosis with calcifications, hemosiderin depositions, and inflammatory cell infiltrate in the peritumoral area, indicative of partial regressed malignancy (“burned-out” phenomenon). The tissue around these aggregates was characterized by intratubular germ cell neoplasia and atrophy of the seminiferous tubules.
The patient arrived at our division after surgery. He had a good performance status and we repeated postoperative serum tumor markers that revealed LDH 932 mg/dL, AFP 803 ng/mL, and b-hCG 1 mIU/mL.
Due to the bulky and diffuse lymph node disease revealed at the preoperative CT body scan we decided to immediately start with first-line chemotherapy. A combination treatment with PEB (bleomycin, etoposide, and cisplatin) was given every 3 week for 4 courses.
After the first 2 courses, serum tumor markers dramatically decreased (LDH 203 mg/dL, AFP 7 ng/mL, and b-hCG 0.5 mIU/mL) and 1 month after the end of the 4th course, the patient underwent a positron emission tomography (PET) scan.
The PET/CT scan (Fig. ) showed dimensional reduction of abdominal and mediastinal lymphadenopathy (wider axis reduced from 44 to 34 mm), without pathological uptake in all the sites of disease. Similarly the values for AFP and others tumor markers returned to within normal limits.
After 3 months, the patient's repeated CT scan revealed further shrinkage of parameter lesions with extensive central necrosis. Surgical evaluation in the multidisciplinary unit was performed with no indication for surgery due to technical challenges. The patient underwent in laboratory and instrumental follow-up.


A 68-year-old man had an acute onset of truncal ataxia, dysarthria, and diplopia in March 2009. He developed dysphagia the next day and underwent a magnetic resonance imaging (MRI) scan, which was normal. He presented to our neurology outpatient department with truncal ataxia, opthalmoplegia, and areflexia five days later.
Upon admission, he had a normal blood pressure of 136/96 mmHg, a regular heart rate of 98 beats per minute (bpm), and a peripheral capillary oxygen saturation (SpO2) of 97% in room air. Neurological examination revealed mydriasis, absent pupillary light reflex bilaterally, total ophthalmoplegia with both eyes fixed centrally, bulbar palsy with nasal voice, dysphagia, absent pharyngeal reflex, and absent deep tendon reflexes. There was no limb weakness. Finger-to nose and heel-to-shin tests showed no dysmetria or decomposition. Romberg’s test and Mann’s test were both positive. He was unable to walk unaided due to the truncal ataxia. There were no sensory abnormalities, orthostatic hypotension, and dysfunction of the bladder and bowel.
His blood count and serum biochemical parameters were normal. Antinuclear, antideoxyribonucleic acid (DNA), and antiacetylcholine receptor (Ach R) antibodies were all negative. Vitamin B1 levels were normal (46.3 ng/mL). Cerebrospinal fluid analysis was normal with a cell count of 1/μL (mononuclear leukocytes), protein level of 33 mg/dl, glucose level of 66 mg/dl (serum glucose 97 mg/dl), and an IgG index of 0.52. However, he tested positive for serum anti-GQ1b IgG, an autoantigenic antiganglioside antibody. His repeated head MRI and chest X-rays were normal.
Given his presentation with acute onset total ophthalmoplegia, ataxia, and areflexia, he was diagnosed with MFS. He had a Functional Systems Score (FSS) of 3 upon admission. Treatment with intravenous immunoglobulin (IVIG; 0.4 g/Kg body weight/day for 5 days) was initiated on day six. He underwent 24-hour Holter recording on day seven because he had exhibited bradycardia (heart rate under 40 bpm), sinus arrest with the P wave falling off, and a 2.94-second duration of the greatest R-R interval (). There were five pauses daily. The 24-hour Holter recording also displayed atrioventricular dissociation with junctional escape beats. After commencing IVIG therapy, his bulbar palsy improved first, followed by the internal ophthalmoplegia and truncal ataxia. In addition, a repeat 24-hour Holter recording on day 19 showed that the sinus arrest had resolved. He was discharged with an FSS of 2 on day 32. Approximately two months after the onset of symptoms, the diplopia secondary to the ophthalmoplegia had resolved.


An 83-year-old man who underwent a right carotid endarterectomy (CEA) with a Dacron patch in 2011 following an ipsilateral right hemispherical transient ischaemic attack, presented 4 years later in 2015, with bleeding and pus discharging from a sinus tracking down to the wound. His background included a 3-year history of left internal carotid artery (ICA) occlusion with bilateral high-grade vertebral artery stenosis of more than 70%. He had a left hemispherical stroke in 2008 and a long-standing history of hypertension for which he takes two antihypertensive medications.
In September 2015, the patient reported a 12-month history of discharge from his CEA scar, initially managed by his general practitioner with local dressings and oral antibiotics. Examination revealed a sinus at the distal end of his scar that was expressing haemoserous fluid. No underlying fluctuant collection was palpable, but the overlying region was mildly tender with no surrounding erythema. The patient was haemodynamically stable and did not exhibit any fevers or rigours. A CT angiogram (CTA) demonstrated a mural atheroma at the right proximal ICA with no pseudoaneurysm or collection noted. A whole body labelled white cell study with positron emission tomography (PECT)/CT (technetium colloid) was normal with no objective evidence of graft infection related to the previous CEA. Surgical management was offered, but the patient declined. Consequently, the wound was monitored closely in the outpatient clinic, and he was managed on broad-spectrum antibiotics for 12 weeks.
The patient presented again to the vascular department in June 2016 after he noted approximately 500 mL of haemoserous liquid discharging from the inferior portion of his CEA wound. He was subsequently admitted and investigated with another CTA scan. The CTA demonstrated some irregularity of the proximal right ICA at the region of surgical interest, with mild enhancement of the surrounding soft tissue. But there was no definite evidence of a pseudoaneurysm formation or dissection. The left ICA remained completely occluded with a complete circle of Willis. However, the carotid duplex scan demonstrated a sinus tract extending down to the anterior inferior margin of the Dacron carotid patch (). There was a focal outpouching in the contour of the inferior aspect of the lumen covered by the patch. A Tc-99m labelled leucocyte scan supplemented with localised planar single photon emission computed tomography (SPECT)-CT of the neck demonstrated active infection in the soft tissue of the sinus track at the right upper neck with involvement of the anterior aspect of the right CEA patch just superior to the hyoid bone ().


A 40-year-old male presented with 2-week history of subjective headache, nausea, and blurry vision. MRI brain [] revealed a large right frontal lobe neoplasm consistent with high-grade glial neoplasm. Craniotomy was performed and postoperative imaging demonstrated gross total resection with marginal enhancement along the posterior margin of the surgical cavity thought to imply postoperative blood and hemostasis product []. Pathology confirmed glioblastoma WHO grade IV with primitive neuroectodermal tumor-like components (MG-PNET) [Figure –]. Immunohistochemical staining demonstrated abundant GFAP immunoreactivity and frequent p53 nuclear staining. Fluorescence in-situ hybridization (FISH) analysis was negative for MYC rearrangement, but did confirm extra copies of the MYC region in 70% of the tumor nuclei. MIB-1 nuclear labeling approached 100% in solid component regions of the mass with abundant EGFR expression. 1p 19q were intact. Isocitrate dehydrogenase 1 (IDH1) was wild type, and unmethylated MGMT was observed.
Due to the PNET-like component of the tumor, MRI of the spinal axis was performed and was without evidence of drop metastasis. Subsequent treatment with radiation therapy and concurrent temozolomide chemotherapy was completed. Twelve weeks after the initial resection and post radiation therapy/temozolomide, surveillance MRI scan revealed multiple new enhancing foci within the left frontal lobe []. Due to the MGMT unmethylated status and the areas of new enhancement, open resection was performed, with pathology demonstrating cortex and white matter with very mild hypercellularity; no obvious tumor was recognized []. Chemotherapy was continued. Seven weeks later (and prior to his next planned MRI scan), the patient presented to the emergency department with acute onset of worsening headaches, nausea, vomiting, and left-hand weakness. He proceeded to develop status epilepticus. MRI brain revealed a diffusion restricting subdural collection overlying the prior craniotomy site [Figure –]. Considering the possibility for empyema, the patient was taken to the operating room for evacuation with intraoperative inspection of the subdural space revealing a thick, gelatinous mass adherent to the pia mater and invading the brain parenchyma. Biopsy results demonstrated PNET-like only recurrence of the previously treated MG-PNET []. The small blue cell tumor component was histologically identical to the original MG-PNET tumor. Due to mixed-glial nature of the original tumor, biomarker molecular profiling was performed to assess the molecular characteristics of the tumor to determine chemotherapy sensitivity and provide targeted treatment. Considering that gliomas can exhibit multiple histologic and molecular subtypes with different clinical phenotypes and responsiveness to treatment, molecular profile testing provides the ability to evaluate tumor cell genetic characteristics and biomarkers. Specifically, molecular profiling evaluates gene amplification, deletion, and methylation-specific PCR to determine MGMT promoter methylation status and FISH for 1p and 19q deletion status. Though not routinely performed at our institution on gliomas demonstrating a homogenous histology, this study can be obtained and analyzed at an outside facility to assist in developing focused treatment regimens for mixed-gliomas. In our patient, Caris Life Sciences comprehensive molecular tumor profiling test was conducted and included analysis of DNA, RNA, and proteins utilizing immunohistochemistry, in-situ hybridization, next-generation sequencing, and pyrosequencing techniques. Based on molecular testing results, chemotherapeutic agents with potential or lack of benefit have been suggested in clinical trials. In our patient, biomarker results were positive for PTEN, TOPO1, and TOP2A; chemotherapeutic agents with potential benefit included carboplatin, cisplastin, and irinotecan.
Because PNET is typically treated with platinum-based chemotherapy, studies confirmed MGMT unmethylated status and demonstrated potential sensitivity to platinum-based chemotherapy. In addition, biomarker results demonstrated topoisomerase activity. Given these findings and that PNET is traditionally treated with platinum-based chemotherapy, the patient was treated with salvage cisplatin and irinotecan. These agents were selected as cisplatin gains superior central nervous system penetration and irinotecan has been used in this setting on primary brain tumors. Given the toxicity associated with the selected regimen, a long discussion was held with the patient prior to initiating therapy. Initially, the patient responded well both clinically and radiographically to therapy; however, surveillance imaging at 8 weeks demonstrated significant tumor progression []. Following discussion, platinum-based chemotherapy was discontinued as the tumor demonstrated resistance, and salvage therapy with bevacizumab was undertaken. The patient continued to decline clinically and eventually succumbed to the disease burden 8 months following the initial diagnosis.


A 66-year-old male manifested psychiatric symptoms such as anxiety, insomnia, depressed mood, hypochondriasis, and hand tremors. He had been receiving bupropion 150 mg per day and clonazepam 1 mg per day for 6 months, since he was 65 years of age, because of the diagnosis of MDD. The patient’s mood symptoms such as depression and anxiety partially improved with bupropion treatment. He had no other physical illness and had not been taking other medications except bupropion and clonazepam. The patient worried about the possibility of PD and underwent neurological examination and brain images tests. The neurological examination revealed bilateral hand tremors that presented during both resting and voluntary movement without other typical signs for PD such as pill-rolling tremor, bradykinesia, rigidity or postural instability. The neurological examination did not favor PD. The SPECT using Tc-99m TRODAT-1 showed decreased DAT binding capacity in bilateral striata (uptake ratio of striata: right 0.31; left 0.27, ) and immeasurable visual score of striata due to an atypical pattern of striatal uptake. The uptake ratio was calculated by the following equation: (The average count density of striatum - the average count density of the reference regions)/the average count density of the reference regions. The reference regions were the cerebellar and the occipital cortex. The striatum is marked by the dotted line in . Under the impression of atypical PD, the patient was advised to take levodopa combined with benserazide by the neurologist. Discontinuation of bupropion for 14 days for the confirmation of the test was suggested by the psychiatrist. The follow-up SPECT after 14 days showed improvement in the uptake ratio of striata (right 0.47; left 0.56, ) and the visual score of striata (Score 0 with intact striatal shape). The patient’s gait was steady, and his muscle power of limbs was normal. Due to the lack of typical sign for PD, anti-Parkinson’s drug was not used. During the follow-up period, the patient’s mental and physical status remained stable without worsening of hand tremors.


A 72-year-old female was presented with a 3-week history of blurred vision in her right eye. The baseline best-corrected visual acuity (BCVA) was counting fingers at a distance of 2 m. The intraocular pressure (IOP) was 17 mmHg. A fundus examination, an optical coherence tomography (OCT), and a fundus fluorescein angiography revealed hemiretinal vein occlusion with diffuse ME []. The injection of a dexamethasone implant was planned, and the inferotemporal quadrant of the right eye was injected. The steroid implant must be the first option in patient with RVO according to Social Security Institution in Turkey. Therefore, we performed dexamethasone implant as the first treatment approach. The needle was introduced 4.0 mm posterior to the limbus. This was the first Ozurdex® injection for the surgeon. After 1 week of routine follow-up visit and during a slit-lamp examination, it was observed that the dexamethasone rod was impeded into the inferonasal quadrant of the lens. The rare event of an Ozurdex® being inadvertently placed in the lens was diagnosed. No inflammatory reaction was seen, and IOP remained normal. The patient was followed without repositioning the implant and was monitored closely for complications related to Ozurdex® such as progression of a cataract, steroid-induced glaucoma, and retinal detachment. After 4 weeks, the IOP rose to 26 mmHg, which was managed well with topical antiglaucoma medications. In addition, ranibizumab (Lucentis®, Genentech, Inc., USA) injection was performed for ME after 1 month. The macular edema had already started to subside, and conservative management was continued. After 3 months, the implant did not show any notable signs of degeneration []. The IOP was 8 mmHg, and antiglaucomatous treatment was stopped. During the 9-month follow-up, the anterior segment of the right eye remained uneventful with no progression of cataract; the implant was still seen in the crystalline lens. The IOP remained within normal limits. The cataract had progressed; phacoemulsification was planned 12 months later for mild posterior subcapsular cataract formation and dexamethasone implant in the crystalline lens []. The implant did not show any notable signs of degeneration []. There was a remarkable improvement in ME in OCT. The BCVA was 0.1 before the surgery.
A cataract extraction was performed through a 2.8 mm clear temporal corneal incision. The anterior chamber was filled with dispersive viscoelastic. Standard capsulorhexis, hydrodissection, and phacoemulsification were performed gently. At the end of the nucleus removal, a small defect in the inferior site of posterior capsule was identified – there was no vitreous on the capsule surface. The Ozurdex® implant was aspirated using a phacoemulsification probe, and a three-piece intraocular lens (AcrySof SA60, Alcon Laboratories, Inc., Fort Worth, TX, USA) was implanted in the sulcus with optic captured within the intact anterior capsule. Ranibizumab was injected after cataract surgery into the vitreous for persisted ME related to RVO in the same session. Regular follow-up visits were performed, and no further complications were registered. The intraocular lens position was normal [] despite the posterior capsular rupture []. At 2 months, BCVA was 0.7 and the macula was dry [].


A 77-year-old Caucasian female with ulcerative colitis, diverticulosis, and liver cysts underwent elective surveillance colonoscopy. Two <1 cm cecal polyps were removed using cold snare with subsequent placement of 3 hemostasis clips. Four 2- to 4-mm ascending colon polyps were removed using cold biopsy forceps. A sessile 1-cm transverse colon polyp was raised with injection of normal saline and removed using cold snare (Fig. ) with the subsequent placement of 2 hemostasis clips. The colon was fixed and tortuous; however, the mucosa was normal except for mild erythema of the ascending colon and rectum without ulceration, and moderate pan-colonic diverticulosis. Following the procedure, the patient experienced sharp, left-lower quadrant abdominal pain. She was directed to the Emergency Department for further evaluation. An abdominal CT scan showed a new 1.8 cm × 2.9 cm fluid collection between the inferior pole of the spleen and the splenic flexure without contrast extravasation or abdominal free air (Fig. ), suggestive of a bowel wall hematoma. Vital signs were stable and initial lab results, including hemoglobin, were normal. The patient was admitted for close hemodynamic monitoring and pain management. She was discharged home the following day, and at outpatient follow-up 2 weeks later she was completely pain-free. Repeat abdominal CT 4 months later showed complete resolution of the bowel wall hematoma.


A 57-year-old man was admitted to our department on 16 July 2007 with a cough, expectoration, and pressure and suffocation in the chest, which had been all present for over six months, but were exacerbated over the last month. He had smoked 10 cigarettes every day for more than 30 years. Chest computed tomography (CT) performed at Jixian County People's Hospital showed space-occupying lesions in the middle lobe of the right lung and lung cancer was suspected, with mediastinal lymph node metastasis (data not shown). Furthermore, slight intumescentia was found on the right wall of the trachea near the carina and cauliflower-like neoplasms were detected on both the right main bronchial wall and the middle lobe of the right lung by fibrobronchoscope. Neoplasms also obstructed the bronchial lumen. Tracheal ACC was diagnosed by means of pathological hematoxylin and eosin (H&E) staining and immunohistochemistry (Fig ). The tumor was classified as T4N3M0, stage IIIB.
Because the patient and his family were unwilling to proceed with surgical resection, he was treated with cisplatin (60 mg days 1–2, 30 mg day 3) and paclitaxel (270 mg day 2) in a cycle. Treatment for hepatoprotection, acid inhibitors, and antiemetics were simultaneously administered to reduce adverse effects. However, because of economic reasons, the patient did not undergo regular examinations or continuous treatment, and was treated with four rounds of chemotherapy on July 25, August 15, and November 6 in 2007 and June 3 in 2008. Upon chest CT reexamination in November 2007 (after two cycles of chemotherapy) (Fig ), the patient's pulmonary lesions had improved slightly and the therapeutic evaluation was stable disease (SD).
Nevertheless, in June 2008, CT reexamination showed that the pulmonary lesions had progressed. Although the patient was still evaluated as having SD, further treatment was required. Therefore, the patient visited the local county hospital for primary pulmonary tumor lesion and metastatic lymph node radiotherapy in July 2008 and was treated with 60 Gy in 30 fractions (2 Gy per fraction). Thereafter, his symptoms of cough and suffocation improved and another chest CT revealed that the tumors had shrunk.
The patient had not received any type of therapy for five years after the first course of radiotherapy until March 2014. As a result of disease progression, the patient visited the local county hospital once again for a second round of pulmonary tumor radiotherapy with a dose of 60 Gy in 30 fractions (2 Gy per fraction). Symptoms of suffocation and cough thereafter improved and chest CT revealed that the tumors had once again shrunk.
A severe cough, and pressure and suffocation in the chest had once again worsened by December 2016. Chest CT results showed space-occupying lesions in the trachea and the right main bronchus with left pulmonary metastasis (Fig a,b), and the tumor was T4NxM1, stage IV. In February 2017, the patient was administered 500 mg/day apatinib mesylate. Symptoms including the cough, and pressure and suffocation in the chest gradually decreased. However, after administration of apatinib mesylate, the patient experienced a headache and his blood pressure increased to a maximum of 160/100 mm/Hg. Valsartan was administered and as a result his blood pressure returned to a normal level. Other adverse reactions included loss of appetite and desquamation of fingers and toes. Chest CT reexamination in April and June 2017 showed that the tumors in the trachea and right main bronchus were reduced. The metastatic foci in the left lung also shrank and cavitation developed (Fig c–f).


A 57-year-old male patient who had been diagnosed with HIV infection 23 years previously
presented with a mildly burning and pruritic hypochromic rash of three-months duration.
The first lesions emerged on shoulders and proximal arms, but soon progressed to the
chest, abdomen, flanks, and dorsum. He informed that, when the illness was first
noticed, the lesions were mildly erythematous, but he could not recall if the palms and
soles were also affected. Sun exposure seemed to worsen the overall aspect. The rash was
taken as an allergic reaction. Within a few days, the erythematous lesions were replaced
by hypomelanotic patches, seemingly on the sites of the preceeding exanthema.
On clinical examination, the patient was mildly overweigh and apparently in good health.
Several round to oval, non-scaling, slightly raised and well-demarcated hypochromic
papules were scattered over the trunk, abdomen, dorsum, and arms (). At times, lesions were confluent, giving an overall
reticulate pattern. There was no ulceration or alopecia. The palms, soles, and mucous
membranes were spared. He was offered a first-generation antihistamine, without
relief.
The patient was on antiretroviral therapy for over two decades. His current regimen was
lamivudine, tenofovir, atazanavir, and ritonavir. The CD4 cell count was 151
cells/mm and the plasma HIV viral load was 2,378 copies/mL. An HIV genotyping study was
performed to investigate the presence of mutations associated with reduced
susceptibility to HIV reverse transcriptase and protease inhibitors. The only mutation
of this type was found in the reverse transcriptase codon 70, which probably emerged due
to the prior use of zidovudine. The genotypic pattern was suggestive of non-adherence to
treatment.
There was no serologic evidence of hepatitis B or hepatitis C infection. Previous
routine non-treponemal tests had been negative for syphilis, but novel studies yielded
positive results. The Venereal Disease Research Laboratory (VDRL) titer was positive at
a titer of 1/2,048. On further history taking, the patient could not recall having had
any prior chancre, but was sexually active and engaged in unprotected sex. Skin lesions
slowly regressed and the hypochromic areas repigmented a few weeks after two consecutive
weekly administrations of 2.4 million units of intramuscular benzathine penicillin G
(). No Jarisch–Herxheimer reaction
occurred. A clinical diagnosis of secondary syphilis and LS was made. A biopsy was not
performed.


A 20-year-old young man presented with progressive areflexic quadriparesis on the 5th day of illness. His MRC sum score was 13 and HD Grade was 4 at admission. His conduction study revealed pure motor demyelinating neuropathy. The patient was treated with five cycles of LVPP. HD Grade remained as 4. On day 18 of illness, after completion of LVPP, he developed new onset asymmetric lower motor type of facial palsy. The patient was given one cycle of additional LVPP and he subsequently made gradual improvement to HD Grade 2 at 3 months and Grade 0 at the end of 3-year follow-up. Interestingly, the patient had past history of GBS in his childhood.


A 52-year-old male presented with acute areflexic quadriparesis on day 20 of illness. His MRC sum score was 6 and HD Grade was 4. He was in plateau stage for 8 days. His conduction studies revealed pure motor demyelinating neuropathy. He was treated with three cycles of LVPP. On day 28 of illness (after 16 days of stable course), he developed paralytic ileus. His serum potassium was normal, and his X-ray abdomen showed dilated bowel loops with fluid levels. He was evaluated by general surgeons who excluded surgical causes. He was initiated on parenteral feeding, treated conservatively, and subsequently improved over the next 4 days. Subsequently, the remaining two cycles of LVPP were continued, and the patient stabilized. He remained to be in HD Grade 4 at 3-month follow-up and had incomplete recovery (foot drop) with HD Grade 2 at the end of 4-year follow-up.


An 18-year-old boy presented with pain and paresthesias of proximal lower limb followed by upper limbs and mild breathing difficulty of 4 days' duration. His MRC sum score was 12 and HD Grade was 4. He progressively worsened over the next 3 days to limb power of 0/5, MRC sum score 0 and required ventilator support. His conductions were suggestive of pure motor axonal neuropathy. He was treated with five LVPP and gradually started improving and MRC sum score became 4 with HD still 5 by day 12 of illness.
On day 17 of disease onset (four days after fifth LVPP), he developed asymmetric bifacial weakness despite his limb power showing improvement (MRC sum score of 6 and HD Grade 5). He was given additional three cycles of LVPP plasmapheresis, and he gradually showed minimal improvement in limb power with HD Grade 4 and was extubated on day 46. At follow-up, 3 months later, his sum score was 36 and Hughes Grade was 3.


A 28-year-old young man presented on day four of illness with a stable plateau phase of two days. He had minimal limb weakness, mild gait ataxia (cerebellar type) and was ambulant. His nerve conductions showed evidence of motor sensory demyelinating and axonal neuropathy with sural sparing pattern. His HD Grade was 2 and MRC sum score was 58. With a clinical diagnosis of GB variant, he was treated with injectable methyl prednisolone. By day 11, his facial weakness resolved, ataxia and proximal weakness improved, and he had MRC sum score of 60. However, on day 13, he worsened in HD Grade from 2 to 3. His MRC sum score was 54. The worsening persisted for 4 days. LVPP was initiated along with pulse methylprednisolone, and he significantly improved 4 days later (MRC sum score was 60 and HD was 3). He improved in facial weakness, was able to walk, although ataxia persisted during discharge and had HD Grade 2 at 3 months, Grade 1 at 1 year, and Grade 0 at the end of 3 years.


A 72-year-old male presented with 2 weeks history of progressive tingling paresthesia of upper limbs, areflexic quadriparesis, and bulbar weakness of three days' duration. He had bilateral lower motor facial weakness, binocular mild abduction restriction, and limb incoordination (cerebellar type). His admission MRC sum score was 56 and HD Grade was 4. He was diagnosed as GBS variant and started on LVPP. He started showing improvement in limb power, walked with two-person support, 3 days after first LVPP and his bulbar symptoms started gradually improving. He developed hyponatremia due to syndrome of inappropriate antidiuretic hormone secretion and developed altered sensorium and seizures 2 weeks after admission. After a stable period of 7 days after completion of fifth LVPP, his lower limb weakness worsened, and he became bedbound with difficulty in flexing limbs in bed. His MRC sum score became 42 and HD was 4. Because of worsening in MRC sum score and HD Grade around 62 days ADO, possibility of TRF was considered and five more LVPP cycles were given. After two cycles of repeat LVPP, he could move limbs in bed and facial weakness started improving. His MRI showed enhancement of lumbosacral roots and serial CSF protein done in view of diagnostic uncertainty was 270 mg% on day 28 with five cells,422 mg% on day 46 with 15 cells, 239 mg% with six cells on day 52 of illness, and increased to 546 mg% with three cells on day 62 after worsening on day 62 after worsening.
His conduction studies revealed motor and sensory demyelinating polyneuropathy with sural sparing pattern. He showed gradual improvement and could walk with walker support at the end of 5-month follow-up.


A 50-year-old male presented with tingling paresthesia of soles and progressive weakness of limbs of 40 days' duration. His disease onset to peak of illness interval was 15 days and had plateaued for 25 days at presentation. He had poliomyelitis in childhood with deformed atrophic and short left lower limb but could walk independently and could work as driver before the illness onset. On examination, he had deformed left lower limb with contracture, had distal upper limb weakness, distal more than proximal weakness of lower limbs, impaired proprioception and sluggish to absent reflexes, and was wheelchair bound. His MRC sum score was 44 and HD Grade was 4. His conduction studies revealed demyelinating and axonal motor and sensory neuropathy and his CSF protein was elevated (98 mg %). As he had not improved 40 days after onset of illness and 25 days of plateau phase, he was treated with LVPP and subsequently discharged. The patient improved gradually after 15 days of completion of five cycles of LVPP. He had improvement in his distal upper and lower limb weakness, could button unbutton, feed himself, could dorsiflex his right foot, and was ambulant with crutches support (MRC sum score was 46 and HD was 3). However, he presented again on day 80 of disease onset (day 34 after start of treatment) with worsening of ten days' duration, had paresthesia of both hands, his distal upper limb and ankle power worsened to around 0–1 power, and became bedbound. He was diagnosed as TRF, and LVPP was initiated again. After 2 weeks, he reported dramatic improvement of distal upper limb and lower limb weakness and could walk with support (HD Grade was 4 and MRC sum score was 40). He showed steady improvement and is independent for ambulation at almost 8½ months follow-up.


A 49-year-old premenopausal female presented with abdominal fullness and vomiting. She had a performance status-1, and clinical examination was suggestive of ascites and decreased air entry in right lower lobe. Blood investigation showed a creatinine of 5.81 mg/dl and uric acid >15 mg/dl. Her cancer antigen (CA)-125 was 3240 U/ml, carcinoembryonic antigen of 0.70 ng/ml, alpha-fetoprotein – 4.80 ng/ml, and beta human chorionic gonadotropin – 5.05 mIU/ml. Noncontrast computed tomography thorax and abdomen showed mild ascites, thickened infiltrated omentum forming omental caking, and the presence of moderate right pleural effusion. Both kidneys, ovary, uterus were normal. Pleural fluid cytology was suggestive of malignant cells. Fine needle aspiration cytology from omental cake was suggestive of metastatic adenocarcinoma. Diagnosis of stage IV peritoneal carcinomatosis/carcinoma ovary in clinical TLS with renal failure was made. She received a single dose of 1.5 mg rasburicase along with aggressive hydration at 3 L/m2. Urine output and daily creatinine and uric acid were monitored daily. The blood investigation on diagnosis and subsequent to diagnosis are mentioned in . A decision to start neoadjuvant chemotherapy (NACT) was made in view of advanced stage. Paclitaxel and carboplatin could be given postpresentation day 4 after reversal of TLS; however, hydration for 2 days' postchemotherapy and allopurinol for 7 days was given. She underwent cytoreductive surgery post three cycles NACT. Presurgery CA-125 was 24.20 U/ml, and contrast-enhanced computed tomography abdomen showed resolution of pleural effusion and partial response in omental deposits and no ascites. Her surgical pathology report showed marked response to chemotherapy with minimal residual poorly differentiated serous carcinoma involving left ovary and omentum and single pelvic lymph node. The patient then went on to complete three cycles of adjuvant chemotherapy. The patient is now apparently healthy, and her posttreatment ultrasonography abdomen pelvis and CA-125 are normal.


An 8-year-old female presented with the right cheek swelling for 2 months with no additional symptoms. The swelling was ill-defined, firm, and nontender. Her general and systemic examinations were normal. Positron emission tomography (PET) scan with F-18 fludeoxyglucose (FDG) revealed an FDG-avid soft tissue mass measuring 3.6 cm × 3.5 cm with maximum standardized uptake value (SUV) of 11.88 in the right maxillary sinus with complete opacification and erosion of the adjacent superior alveolus with thinning of the walls of the maxillary sinus with an extramaxillary soft tissue component. Hematology values were as follows: hemoglobin (Hb) concentration – 13.1 g/dl; white blood cell (WBC) count – 7.8 × 109/L; platelets – 400 × 109/L; and peripheral blood smear (PBS) examination did not reveal any abnormal cells or blasts. Subsequent BM examination revealed normocellular to mildly hypocellular BM with trilineage hematopoiesis. No blasts or abnormal cells were identified. Cytology of cerebrospinal fluid also did not reveal any abnormal cells.
The right maxillary sinus biopsy showed diffuse proliferation of medium size cells with scant cytoplasm and blastic chromatin []. Mild pleomorphism in the size of the tumor cells with high mitotic activity was noted. On immunohistochemistry (IHC) [], the tumor cells were positive for CD99 (Mic2), diffusely positive for B cell markers such as CD10, CD79a, PAX5 and also expressed MPO and weakly CD45 (LCA) but negative for CD20, CD3, CD5, desmin, and chromogranin. In addition, nearly all tumor cells showed nuclear positivity for terminal deoxynucleotidyl transferase (TdT). Fluorescence in situ hybridization on interphase cells of formalin-fixed paraffin-embedded tissue revealed negative results for BCR/ABL using locus-specific identifier (LSI) BCR/ABL dual fusion translocation probe and for MLL using LSI MLL (11q23) break apart rearrangement probe; however, nearly 40% of cells revealed trisomy of chromosome 22. Thus, histological and IHC findings were consistent with bi/mixed phenotypic blastic hematolymphoid neoplasm with B-lymphoblastic lymphoma with coexpression of the myeloid lineage-specific marker and MPO.
She was started on treatment with institutional acute lymphoblastic leukemia protocol called MCP-841 with standard 4-drug induction followed by a consolidation block with intermediate-dose cytarabine known to be active in myeloid neoplasms, interim maintenance, and delayed intensification. After the end of induction, she had a good response in maxillary mass with complete metabolic and a partial morphological response with calcification of mass. She was continued on postinduction chemotherapy and received consolidative radiotherapy to the right maxilla in a dose of 25.2 grays in 14 fractions during interim maintenance. Her postradiation contrast-enhanced computed tomography scan revealed only thickening and sclerosis of the wall of maxillary sinus suggestive of healing. She is currently continuing on oral maintenance chemotherapy and is doing well after 3 maintenance cycles at 18-month follow-up.


A 21-year-old male was being investigated for an acute onset of pain involving the left hip for 6–8 weeks. The magnetic resonance imaging revealed a permeative lesion involving the head and upper shaft of the femur. The needle core biopsy from the lesion was performed in another hospital which was reported as suspicious of a primitive neuroectodermal tumor and was referred to our institute. His laboratory workup in our hospital revealed normal complete blood count with Hb concentration – 14.2 g/dl; WBC count – 5.7 × 109/L; platelets – 278 × 109/L; and PBS did not reveal any abnormal cells or blasts. The serum lactate dehydrogenase was mildly raised to 216 U/L. PET scan with F-18 FDG revealed hypermetabolic disease involving the head, neck, and proximal one-third shaft of the left femur with maximum SUV of 7.6 with no obvious cortical destruction or soft tissue mass. In addition, the proximal end of the left fibula also showed an active disease with maximum SUV of 3.59. There was no evidence of active disease elsewhere in the body.
A biopsy of the lesion was reviewed which showed sheets of medium-sized tumor cells with scant cytoplasm and blastic chromatin with few crushing artifacts. Mild pleomorphism of tumor cells with focal apoptosis was noted. These features were consistent with a high-grade blastic neoplasm. On IHC, the tumor cells showed diffuse membranous expression of MIC2, whereas TdT showed weak, but distinct nuclear reactivity. In B-lymphoid lineage markers, CD10 and CD20 were diffusely positive while PAX5 was negative. In addition, blasts expressed MPO; however, CD117 did not reveal any positive expression.
The BM study was omitted initially as there was no evidence of any other sites or marrow involvement or uptake by PET scan. However, BM was done after a month of diagnosis, and it revealed 54% of blasts which were negative for cytochemical MPO. We performed eight-color flow cytometric immunophenotyping (FCI) using a comprehensive antibody panel []. Cells were acquired on Navios (Beckman Coulter [BC]) flow cytometer using Navios software, and data were analyzed using Kaluza software (BC).
Immunophenotypic analysis [] showed approximately 43% of viable cells were abnormal blasts. The abnormal blasts revealed a B-lymphoid lineage origin expressing CD10, CD19, CD20, and CD22 but negative for cytoplasmic CD79a. It also showed expression of CD34 and human leukocyte antigen–antigen D. In addition, they showed cytoplasmic MPO expression but were negative for rest of the other markers tested. BM trephine biopsy also showed a sheet of blasts expressing CD10, CD20, and MPO. Cytogenetics revealed no evidence of BCR/ABL fusion: t(9;22), AML1/ETO: t(8;21), and MLL rearrangement.
All features were consistent with bi/mixed phenotypic blastic hematolymphoid neoplasm as B-lymphoblastic lymphoma with coexpression of the myeloid marker and MPO that progressed to mixed phenotypic acute leukemia (B/myeloid leukemia) during a course of time.


A 9-year-old female patient with progressive spinal deformity and shoulder imbalance visited our scoliosis research institute. At the index surgery, her Cobb's angle was 71° with Risser stage 0 and an open triradiate cartilage []. She was skeletally immature with height and weight of 134.7 cm and 25 kg, respectively. Considering the risk of progression, corrective surgery was done using the modified guided growing rod system as described by hat McCarthy et al. Through a posterior approach, mono-directional screws (maintain alignment) and rods were placed from T3 to L2, with tightening of the inner cap done only over the T8 and T9 (the apex of the spinal curvature). Rest of the screws were not tightened so that the rod could slide through the unlocked gaps. Posterior fusion was not done. The postoperative Cobb's angle was 13°.
After the index surgery, the 5 years followup was uneventful, except for persistent complains of recurrent irritation by the rod and gradual loss of correction after the 1st year []. Her Cobb's angle was 30° by 2 years, 40° by 4 years with a final Cobb's angle of 57°. During the 5-year followup period, the truncal height (from T1 to T12) increased from 146 to 203 mm with an increase in lung volume from 658,540 to 994,533 mm3 and 502,246–886,303 mm3 on the concave and convex side, respectively []. Considering unsuccessful spinal correction and symptoms of recurrent skin irritation, she was readmitted for a revision surgery for posterior instrumentation and fusion. At this juncture, her Cobb's angle was 57° with a height of 161.1 cm (an increase of 26.4 cm from index surgery) and Riser stage 4.
At the revision surgery, the rod and pedicle screws were exposed by an incision through previous healed surgical scar. Preoperatively, the proximal and distal pedicle screws, which had space for sliding of the rod, showed significant changes of implant wear and metallosis []. Whereas, no such changes were observed in the T8 and T9 segment screw-rod construct (apex), which were previously fixed and tightened with the inner cap. The effects of implant wear and metallosis at proximal and distal pedicle screws were more apparent after the removal of the rod. Considering the significant findings, the tissue sample was sent for a culture and biopsy, and loose pedicle screws were removed, followed by thorough irrigation and debridement. New pedicle screws replaced the loosened ones, and additional fixations at L3 to L5 level were implemented to correct the deteriorated scoliotic deformity. Finally, contoured rods were inserted with inner caps and tightened with deformity correction through derotation maneuver, followed by the tightening of the inner caps was done. In addition, thoracoplasty was done to correct the thoracic rib hump. The postoperative Cobb's angle was 23°. The histological examination of the tissue sample sent was reported as chronic inflammation, fibrosis with foreign body granules []. Postoperatively, the patient's hair was examined for the titanium concentration in order to rule out the systemic spread of metallosis. The hair examination manifested a normal range of titanium concentrations. The patient was discharged without any complications. A radiograph of 2 years followup shows corrected vertebral bodies, maintaining Cobb's angle of 23°, without an observable sign of skin irritations [].


A 68-year-old female on HD for 1 year as a result of glomerulonephritis was seen for a malfunctioning proximal radiocephalic AVF, created by an interventional nephrologist of the VA team. Diagnostic angiography showed a patent arteriovenous (AV) anastomosis and perianastomotic vein and chronic occlusion of a long segment of the cephalic vein (). An “early cannulation graft” (Acuseal, Gore, Flagstaff USA) was implanted as venous bridge on the cephalic vein, maintaining her native AV anastomosis as inflow. After 2 months, she was newly admitted for VA dysfunction. US-guided phlebography showed low flow along the graft (). No definite opacification of the AV anastomosis was obtained. A 0.014″ guide wire was negotiated up the arterial side via a retrograde approach. A 4F angled diagnostic catheter was exchanged on the wire for diagnostic angiography. The previously unknown high takeoff of the radial artery from the axillary artery, an anatomical variation referred to as brachioradial artery, was discovered. A critical stenosis of >90% on the arterial anastomosis was clearly depicted. Initially, repeated long inflation angioplasty (percutaneous transluminal angioplasty (PTA)) with high-pressure low-profile catheter balloons of 4 and 5 mm was attempted, but immediate elastic recoil occurred with persisting stenosis. A 6 mm × 100 mm Viabahn stent graft (Gore, Flagstaff, USA) was thus deployed through a 6F sheath from the radial artery across the anastomosis and the swing vein, to land in the healthy zone of the cephalic vein. Trans-anastomotic intrastent post dilation to 6 mm was carried out along the entire length of the stent graft. The side-to-end AVG was transformed into an end-to-end anastomosis with the exclusion of the distal radial artery. Thrill was perceived immediately, and the postprocedure period was uneventful. The patient underwent efficient HD the following day without problem to the hand that presents a good ulnar artery flow. A computed tomography angiography (CTA) performed 4 months later to study central veins confirmed the neo-anastomotic target lesion patency. It revealed a stenosis on the cephalic arch, which was treated by PTA with drug eluting balloon 1 month later (). Circuit access and target lesion were patent at the 13-month follow-up.


A 75-year-old man on HD for 7 years was referred to us as tertiary center for a malfunctioning right radiocephalic fistula. Comorbidities included coronary artery disease (CAD) with previous aortic coronary bypass and peripheral arterial disease (PAD). Access history included implanted cardiac defibrillator–related chronic occlusion of the left central venous axis, right brachiocephalic vein angioplasty and the implantation 3 years earlier of a 8 mm × 50 mm Viabahn stent graft at the elbow for a relapsing obstructive lesion of the basilic vein without necessity for further intervention. On admittance, the patient presented an abrupt occlusion of the stent graft with fresh thrombus spreading extensively above the elbow detected at ultrasound and confirmed by fistulography. A 4-mm skin ulcer overlying the stent was present. Skin swab culture was negative and other signs of infection were absent (white blood cell count (WBC) of 6160 µL, HB of 10.5 g\dL, erythrocyte sedimentation rate (ESR) of 37 mm\h, C-reactive protein (CRP) of 3.1 mg\dL). The thrombus was aspirated percutaneously and a new stent graft (8 mm × 100 mm) was placed overlapping the previous one () gaining AVF’s thrill. Patient refused surgical revision and returned to the referring dialysis center. After 3 months, the patient was readmitted with fever (WBC of 13,170 µL, ESR of 62 mm\h, CRP of 8.3 mg\dL). A mild edema from the hand to the elbow was appreciated on the AVF side. Another skin ulcer, found to be infected with Gram positive bacteria, was present at a HD puncture site. Broad spectrum antibiotic therapy was started. The AVF anastomosis was pulsing and still patent on ultrasound examination but thrill was absent. Phlebography revealed complete occlusion of the stent grafts with an extended thrombosis of 14 cm along the basilic vein above the elbow (). After VA team evaluation, a surgical prosthetic bridge was deemed unfeasible at the time due to the extended basilic thrombosis. A two-stage hybrid repair was opted for. First, a new percutaneous recanalization as endovascular bridge was performed. Manual thrombus aspiration and multiple high-pressure PTAs were done through a 8Fr sheath. Due to unsuccessful hemodynamic result, a third 8 mm × 50 mm Viabahn stent graft was finally released to restore AVF flow. Thrill reappeared and patency of the circuit access including the basilic vein was confirmed at completion angiography. The patient underwent efficient HD through the AVF the same day. After 3 days, he underwent the planned surgical removal of the three overlapping infected stent grafts. Because an “early cannulation” graft was not available, surgical rerouting was done with a polytetrafluoroethylene (PTFE) graft (Propaten, Gore, Flagstaff, USA) to bridge the forearm AVF efferent vein and the basilic vein. During the graft maturation time, HD puncture was allowed on the proximal and distal vein segments (). The association of endovascular and surgical treatment gave a double benefit: the salvage of the last VA and the excision of the infected stent grafts avoiding the placement of a temporary venous access. The patient remained asymptomatic at 4 months control.


A 70-year-old woman presented for evaluation of a tender lesion on her left lower back in April 2017 (Fig. ). There was no fever or associated systemic symptoms. Her past medical history was significant for basal cell carcinoma, melanoma in situ, and seronegative rheumatoid arthritis. She was currently being treated with methotrexate.
Nine days prior to presentation, she was at the beach and felt her back being bitten by an insect. The affected area became red and painful and increased in size over the next few days.
Cutaneous examination showed an erythematous patch—the presumed bite site—with an underlying cystic component on the left lower back (Fig. ). A small amount of pus was expressed from the lesion and cultured. The bacterial culture grew S. lugdunensis (Table ) and antibiotic susceptibility testing was performed (Table ).
She was treated with incision and drainage; empiric treatment with cephalexin 500 mg, four times daily for 10 days, was also initiated. A wound check performed 5 days later did not show any significant improvement. Therefore, doxycycline 100 mg, twice daily for 10 days, was added. The infection resolved during the subsequent 3 weeks. There was no recurrence of the infection within 60 days following treatment.


A 30-year-old man presented for evaluation of a tender lesion on his right great toe in September 2015. There was no fever or associated systemic symptoms. His past medical history was significant for psoriasis and psoriatic arthritis. He was currently being treated with adalimumab and methotrexate.
Cutaneous examination showed erythema of the medial right great toe nail fold with an underlying abscess. A small amount of pus was expressed from the abscess and cultured. The bacterial culture grew S. lugdunensis, Staphylococcus aureus (S. aureus), and Streptococcus agalactiae (S. agalactiae) as the pathogens (Table ). Antibiotic susceptibility panels were performed (Table ).
He was treated with incision and drainage. Empiric oral antibiotic treatment with cephalexin 500 mg, four times daily for 10 days, was initiated. Six days after starting the antibiotic, the patient mentioned that the infection had improved by 70%. The cephalexin treatment was extended for another 20 days, for a total of 30 days of treatment. One month after the initiation of antibiotic therapy, the patient presented to the office for a wound check; the infection had resolved. After an additional 30 days, there were no signs of infection.


A 67-year-old man presented for the evaluation of a tender lesion on his left upper back in February 2015. There was no fever or associated systemic symptoms. His medical history was significant for elevated fasting glucose levels. The lesion initially appeared a few months earlier.
Cutaneous examination showed an inflamed cystic lesion with surrounding erythema on the left upper back. A small amount of pus was expressed from the lesion and cultured. Bacterial culture grew S. lugdunensis (Table ) and antibiotic susceptibility tests were performed (Table ).
He was treated with incision and drainage; empiric oral antibiotic treatment with cefdinir 300 mg, twice daily for 10 days, was initiated. Two days later, the patient reported that he experienced severe diarrhea. Therefore, the cefdinir was stopped and doxycycline 100 mg, twice daily for 10 days, was initiated. The infection resolved within the 10 days of treatment. There was no clinical evidence of recurrent infection 2 months after completion of treatment.


A healthy 80-year-old man presented with right index finger pain and swelling in March 2016. There was no fever or associated systemic symptoms. His past medical history was significant for nodular basal cell carcinoma.
The patient stated that a few days prior to the presentation he was doing some gardening and scraped his finger on a plant. He subsequently developed a large blister with localized throbbing, numbness, and redness.
Cutaneous examination showed an abscess with surrounding erythema on the nail fold of the radial aspect of the right index finger. The finger was cleaned and a small amount of pus was expressed from the abscess and cultured. The bacterial culture grew S. lugdunensis and Streptococcus intermedius (S. intermedius) (Table ). Antibiotic susceptibility tests were performed (Table ).
He was treated with incision and drainage; empiric oral antibiotic treatment was initiated with cephalexin 500 mg, four times daily, and sulfamethoxazole–trimethoprim 800–160 mg, twice daily, for 10 days. The infection resolved within 10 days of treatment. Sixty days following completion of treatment, there were no clinical signs of recurrent infection.


An 82-year-old man presented for evaluation of a tender lesion on his left lower back in May 2017. There was no fever or associated systemic symptoms. His past medical history was significant for diabetes mellitus and metastatic lentigo maligna melanoma. He was being treated with pembrolizumab.
Cutaneous examination showed an inflamed cyst with surrounding erythema on the left lower back (Fig. ). A small amount of pus from the cyst was expressed and cultured. Bacterial culture grew S. lugdunensis (Table ) and antibiotic susceptibility tests were performed (Table ).
He was treated with incision and drainage. Empiric oral antibiotic treatment with cephalexin 500 mg, four times daily for 30 days, was initiated. The infection cleared within the 30 days of the antibiotic regimen (Figs. , ). There was no recurrence of the infection 60 days after completion of treatment.
Informed consent was obtained from the patients who were included in the study.


A 19-year-old female presented with dimness of vision in the right eye for 5 years following blunt trauma to the eye. Her best-corrected visual acuity (BCVA) was 20/30 (−2.00 DS − 1.00 DC 90°), N6 in the right eye and 20/20, N6 (Plano) in the left eye. Color vision and pupillary reflexes were normal in both eyes. Anterior segment was normal in both eyes, and there was no evidence of inflammation. Fundoscopy of the right eye showed a choroidal rupture in the juxtafoveal region. Left eye macula was normal. The optic discs of both eyes appeared hyperemic with blurring of disc margins [Fig. and ]. B-scan ultrasonography (USG) showed a hyperechoic clump over the optic nerve head (ONH) in both eyes which persisted at low gain confirming ONHD [Fig. and ]. Spectral domain optical coherence tomography (SD-OCT) of the ONH showed elevation with an irregular internal optic nerve contour and hyporeflective spaces below the surface suggestive of ONHD. Composite MC image of the right and left eyes showed a hyperreflective greenish ring with discrete margins and clearly demarcated disc and retinal vasculature [Fig. and ]. Infrared reflectance (IR) images of the right and left eye show clearly demarcated disc vasculature. Green reflectance (GR) and blue reflectance (BR) images of the right and left eyes showed hyperreflectivity limited to the optic disc margin [Fig. - and -].


A 63-year-old male patient, known diabetic, presented with sudden and painless diminution of vision in the right eye for the last 10 days and gradual painless diminution of vision in the left eye over the last 10 years. BCVA was 20/80;N12, 20/125;N12 in the right and left eyes respectively. The color vision score was 4 out of 21 Ishihara plates in the right eye whereas the left eye had a score of 11 out of 21. Anterior segment examination was unremarkable. Dilated fundoscopy of the right eye showed disc edema with prominence of the inferotemporal sector and splinter hemorrhages whereas that of the left eye showed temporal disc pallor []. The vessels, macula, and peripheral retina were normal in both eyes. Humphrey 24-2 visual field analysis of both eyes revealed an inferior altitudinal field defect. A diagnosis of the right eye nonarteritic anterior ischemic optic neuropathy was made. MC image of the right eye showed a greenish hue with irregular margins extending beyond the disc margins and obscuration of disc vasculature [Fig. -].


A 38-year-old male patient, known case of diabetes mellitus (uncontrolled), presented with sudden painless diminution of vision of both eyes (OS > OD) for 1 month. BCVA was 20/25;N6, 20/125;N12 in the right and left eyes respectively. RAPD was present in the left eye. Color vision test with Ishihara plates revealed a score of 21/21 in the right eye and 1/21 in the left eye. The remainder of the anterior segment examination was normal. On dilated fundoscopy, both eyes showed gross disc edema with splinter hemorrhages [Fig. and ]. Macular edema was also noted in the left eye. MRI brain done elsewhere showed diffuse mild cerebral cortical atrophy with no evidence of an intracranial space-occupying lesion. The patient was lost to follow-up thereafter. MC images of both eyes showed a diffusely greenish hyperreflectant ring with blurry irregular borders. The left eye also showed extension of this greenish hue temporally to involve the macula [Fig. -].


A 35-year-old female of the Indian subcontinent, from a rural background, was referred to our center complaining of pain in left hip and limp since 1 year. The pain was insidious in onset and was associated with difficulty in squatting and sitting cross-legged. The patient had anorexia and undocumented weight loss.
There was no other significant past medical history. Family history revealed that her husband had been previously treated for pulmonary tuberculosis 10 years back.
The hip was tender on palpation and associated with fullness in the iliac fossa. She had flexion deformity of 30°, with only jog of movement at the hip joint, and true shortening of 3 cm.
Plain radiographs () showed obliteration of joint space with the destruction of femoral head and acetabulum. Osteolytic lesions were seen in the supra-acetabular region and superior pubic ramus. Laboratory investigations revealed an elevated erythrocyte sedimentation rate (ESR) of 52 mm/h and eosinophilia, with absolute eosinophil count (AEC) of 930/mL (30-350/mL). Based on this, a provisional diagnosis of tuberculosis of the hip was made. Baseline liver function tests (LFT) before starting anti-tubercular therapy (ATT) revealed mild hyperbilirubinemia (T. Bil -2.1 mg/dL) and elevated serum transaminases (alanine aminotransferase [ALT] -136 U/L, aspartate aminotransferase [AST] -145 U/L). Within 1 week of starting ATT, the patient developed abdominal pain with vomiting and icterus. LFT revealed a further rise in bilirubin levels (T. Bil -3.2 mg/dL) and serum transaminases (ALT -286 U/L, AST -312 U/L). ESR also increased to 64 mm/h and eosinophilia persisted (AEC -740/mL). ATT was withheld; however, derangement of LFT persisted.
This prompted an ultrasound of the hepatobiliary system which revealed a well-circumscribed multilobulated lesion in segment V of the right lobe of the liver with peripheral daughter cysts. There was an associated large exophytic component extending medially beyond the hepatic capsule, suggestive of hydatid cyst.
Henceforth, abdominal computed tomography (CT) scan with contrast () was done revealing 10.2 cm × 5.6 cm exophytic multiseptated thick-walled cystic lesion arising from the right lobe of the liver. There was an associated smaller (3.3 cm × 2.3 cm) multiseptated cystic lesion with evidence of rim calcification.
CT scan of the pelvis () revealed a large multiseptated cystic lesion with the osseous destruction of the left iliac bone, acetabulum, and proximal femur. It showed complete destruction of the acetabulum with evidence of protrusio acetabuli. The cystic component of the lesion involved muscles around the hip joint and left hemipelvis. This extended into the ischiorectal fossa, gluteal region, and iliopsoas, with a cold abscess in pelvis tracking along the anterolateral aspect of the thigh. Multiple small bony fragments were seen in the cystic lesion.
Surgical debridement was done 1 week after commencing oral treatment with albendazole 10 ?mg/kg/day. A lateral approach was used to expose the hip joint through an incision starting from the highest point of iliac crest extending distally along the posterior border of the greater trochanter (). On separating the fibers of gluteus medius, hydatid cyst, and debris from destroyed hip joint were encountered. The cyst was injected with 50 mL 10% povidone- iodine solution. Carefully, the extraosseous part of the cyst was dissected and excised. Using a bone saw, osteotomy of the femoral neck was made at the upper border of the lesser trochanter. The diseased and deformed femoral head was removed revealing an enlarged acetabulum filled with osseous debris, cartilage flakes and hydatid cyst. The cyst was seen to be communicating to the endopelvic. Again, 50 mL of 10% povidone-iodine solution was injected into the cyst.
For endopelvic exploration, the anterior approach was made through an incision from pubic symphysis to anterior superior iliac spine and along the iliac crest to join the first incision at the highest point of the crest. The femoral neurovascular bundle was identified and preserved. Anterior abdominal wall muscles were reflected off the superior pubic ramus, inguinal ligament, and anterior portion of the anterior iliac crest. Dissection was continued in the extraperitoneal plane by retracting the peritoneum away from the affected side. Lesser pelvic wall and quadrilateral plate were identified and were observed to be involved in the parasitic infestation. Cysts were found bulging into the endopelvic from the medial acetabular wall. This cyst was communicating across the acetabulum. Careful excision of the cyst was performed. Surgical wounds were thoroughly washed with 0.9% saline and 10% povidone-iodine mixture (2:1) and closed in layers over suction drains. Fistulae were debrided and excised. No anaphylactic reaction has been observed during the surgery. Femoral head and synovial biopsy confirmed hydatid disease. Since the disease was infective in nature, we did not opt for reconstruction of the hip joint with endoprosthesis for fear of surgical site infection.
Albendazole was continued for 3 months after the surgery. Skeletal traction through upper tibial Steinman pin was applied postoperatively. Gentle hip adduction/abduction skateboard exercises were initiated 2 weeks postoperatively. Hip flexion/extension exercises were added at 3 weeks. Surgical wound dehiscence occurred at 2 weeks after the surgery, but with regular dressing and oral antibiotic coverage, the wound healed within 3 weeks. At 3 months postoperatively, the patient was allowed crutch-assisted full weight- bearing ambulation.
At 6-month follow-up, the patient was able to walk with the help of a stick, squat, and sit cross-legged (). At 3-year follow-up, the patient was walking pain- free and unaided. There was no clinical evidence of disease recurrence.


A 69-year-old male patient presented with stuporous mentality after fall from 2-meter height. On admission, the patient's Glasgow Coma Scale (GCS) score was 7/15. Neurological exam revealed no further abnormalities. Brain computed tomography (CT) scan revealed an ICH in the left temporal lobe with diffuse SAH and subdural hemorrhage (). A brain CT angiography revealed a pseudoaneurysm of MMA (). Sequentially, the patient was submitted to cerebral angiography and the left external carotid angiogram revealed a pseudoaneurysm arisen from the anterior branch of the MMA accompanied by dAVF at the middle segment of sphenoid ridge (). Through the right common femoral artery, a guiding catheter (Envoy 6 Fr; Cordis/Johnson & Johnson, Miami, FL, USA) was positioned in the left external carotid artery. We carefully naviated a microcatheter pre-shaped 45 degrees (Excelsior SL-10; Stryker Neurovascular, Fremont, CA, USA) under the guidance of a 0.014-inch microguidewire (Synchro 14; Stryker Neurovascular) to reach the juxtaproximal to the pseudoaneurysm via MMA. After the confirmation of the appropriate position of the microcatheter via repetitive superselective angiography (), embolization of a pseudoaneurysm and MMA was performed using 25% n-butyl cyanoacrylate (NBCA) (Hystoacryl; B. Braun, Melsnagen, Germany) mixed with iodized oil (Lipiodol; Guerbet, Aulnay-sous-Boid, France). We achieved angiographically complete occlusion of a pseudoaneurysm and dural AVF (). Follow-up brain CT scan showed no significant hematoma enlargement and well penetrated glue cast at the all the vascular lesions ().
The patient presented an improvement in consciousness for GCS score 15 after 12 days. However, at the 18-day after the procedure, the right side weakness and dysarthria were developed. Brain CT angiography and subsequent left internal carotid angiography revealed cerebral vasospasm at the distal segment of MCA with multiple low densitied at the left MCA territories (). Cerebral vasospasm was treated using intra-arterial (IA) nimodipin infusion, and it was improved. At discharge, dysarthria was improved, but right side weakness was remained (Grade 3).


The propositus was a 2-day-old male, born at 39 gestation weeks, weighing 3610 g. He was the sixth child of non-consanguineous parents. All previous pregnancies were normal, and all children are healthy. No previous abortions have occurred. The family history was negative for metabolic diseases. During the first hours of life, the health of the newborn male worsened due to vomiting, lethargy and focal seizures. He was immediately transported from a local hospital to University Children’s Hospital for further examinations and treatment. Initial laboratory analyses showed an absence of hypoglycemia, total bilirubin level 42 μmol/L, conjugated fraction 28 μmol/L, alanine aminotransferase (ALT) level of 987 IU/L, aspartate aminotransferase (AST) level of 4165 IU/L and lactate dehydrogenase level 4760 IU/L. Ammonia level was 823 μmol/L. The coagulation test displayed extended partial thromboplastin time (PTT) of 59.5 s, extended prothrombin time (PT) of 22.2 s and international normalized ratio (INR) of 1.75. Fibrinogen level was normal (2.24 g/L). Having completed the routine screening of iron concentration (performed for neonatal iron overload), the results showed elevated iron serum concentration (30.7 μmol/L), very high ferritin (7145.6 ng/L), decreased serum transferrin concentration (1.35 g/L), TIBC 34.1 μmol/L and transferrin saturation 90% accordingly. A biopsy of submucosal oral salivary glands was done as well. Without knowing of histological findings of biopsy specimen, the patient was diagnosed as having NH. It was decided to treat the patient with blood exchange transfusion and intravenous immunoglobulin, but there was no improvement. Two days later, the pathologist reported that the biopsy specimen stained for iron deposits, was negative. The diagnosis of NH was immediately suspended and patient’s blood and urine samples were urgently sent to another medical institution for metabolic testing. The results of that testing showed: High citrulline (328 μmol/L, normal 10-21 μmol/L) and high alanine (847 μmol/L, normal range 274-384 μmol/L) levels, as well as high urine orotic acid excretion (0.9, normal < 0.14). This metabolic profile finding gave the final diagnosis of citrullinemia type I, a defect in the urea cycle, caused by the deficiency of argininosuccinate synthetase. The treatment was continued according to the rules for the treatment of acute hypeammomiemia[]. It included prompt removal of ammonia from the body and providing the organism with adequate calories and essential amino acids to halt further breakdown of endogenous proteins. The patient showed visible clinical and laboratory improvement. The ammonia level fell to 46 μmol/L, ALT level 70 IU/L, AST 86 IU/L and LDH level 538 IU/L. Unfortunately, on the 20th day of the recovery process in the intensive care unit, the patient contracted sepsis caused by the multi-resistant hospital species of Enterobacter. Despite vigorous antimicrobial and supportive therapy, the patient started to suffer from gastrointestinal, intracranial and pulmonary hemorrhage, accompanied by the failure of vital functions. Ten days later the patient died.


A 60-year-old male presented with a history of decreased urine output and pedal edema of 1-week duration. He noted a decrease in urine output about 1 week back, which progressed to complete anuria in 2–3 days. There was no history of fever or rash but he developed edema in the course of 3-day period. There was no history of dysuria, gross hematuria, or abdominal pain. He was a known case of Type 2 diabetes mellitus (Type 2 DM) and systemic hypertension for 3 years and he was on tablet metformin (500 mg) and glimiperide (2 mg) combination twice a day and tablet atenolol (50 mg) and amlodipine (50 mg) combination once in a day for 2 years. He denied the intake of nonsteroidal anti-inflammatory drugs (NSAIDs) or any other over-the-counter drugs but has taken hyponidd one tablet once daily for 7 days before the onset of his symptoms. The physical examination revealed normal temperature, pulse rate of 90/min, and blood pressure (BP) of 170/100 mmHg in all the four limbs with bilateral pitting pedal edema. Systemic examination was normal. Fundus examination did not revealed any abnormality.
At the time of admission, urea was 177 mg/dl and serum creatinine was 9.1 mg/dl. There was no previous laboratory record available for renal function. Urine analysis showed numerous red blood cells (RBCs), with numerous white blood cells (WBCs) with no WBC or RBC cast and no eosinophills. Proteinuria was present, 2+ on dipstick, but as his output was <10 ml/day, quantification of proteinuria was not done. A renal ultrasound demonstrated normal-sized kidneys (right 10.1 cm and left 10.3 cm) without evidence of hydronephrosis or nephrolithiasis. Other investigations were as follows: hemoglobin (Hb): 10.9 g/dl, total leukocyte count: 12450/cmm, differential leukocyte count: P88 L10E2, peripheral platelet count: 4.1 lakhs, bilirubin: 0.8, serum albumin: 3.6, serum globulin: 3, serum glutamic pyruvic transaminase: 42, serum glutamic oxaloacetic transaminase: 32, serum calcium: 7.8, serum phosphate: 4.4, serum uric acid: 4.8, HIV negative, Hepatitis B surface antigen negative, and anti-HCV negative. Urine culture was sterile. Anti-neutrophil cytoplasmic antibodies were negative, and C3, C4 levels were normal, and antinuclear antibody and antiglomerular basement membrane antibody testings were not done. As the patient is a known diabetic and there were no previous renal function reports, our first differential diagnosis was acute on chronic kidney disease.
We kept the second possibility as severe acute tubular necrosis, and as urine showed proteinuria and RBCs, rapidly progressive, glomerulonephritis was considered as the third possibility.
BP control was achieved over the next 2 days with oral medications and hemodialysis was done. A kidney biopsy was performed on the 3rd day of admission. The biopsy showed areas of interstitial edema with marked infiltration composed of mononuclear cells, eosinophils, few polymorphs, and macrophages []. The infiltrates surrounded individual tubules. Evidence of tubulitis and destruction of tubular epithelium with detached necrotic tubular cells and casts in lumen of tubules were consistent with acute tubulointerstitial nephritis. Mild interstitial fibrosis (15%–20%), along with patches of atrophic tubules and hyalinosis of arterioles, were also present. The glomeruli were unremarkable in light microscopy except for mild mesangial hypercellularity, and immunofluorescence microscopy was negative for IgG, IgM, IgA, C3, C4, C1q, kappa, and lambda. Offending drug was withdrawn immediately, and renal replacement therapy was started in the form of hemodialysis. The patient remained anuric, so on the 6th day, oral steroids were started at 1 mg/kg dose, 60 mg/day for 1 week, and then tapered and stopped at 28 days. After about 20 days of the onset of symptom (after 1 week of steroids), he started to have urine output, which progressively increased. The patient was discharged after 1 more week when he became dialysis independent and his urine output became normal. At discharge, his creatinine was 3.1 mg/dl and urine was showing 1+ protein and 5–10 WBCs, and there was no RBCs. At 2 months, his creatinine is still 1.8 mg/dl.