Sporadic Hemangioblastoma of the Cavernous Sinus and Meckel's Cave.

Objectives To describe an extremely rare case of sporadic hemangioblastoma (HB) within the cavernous sinus and Meckel's cave with extension to the cerebellopontine angle (CPA) cistern. Methods A 73-year-old male presented with hearing loss, unilateral ptosis, and facial numbness. Results The imaging showed a complex cystic-solid mass centered at the left cavernous sinus and Meckel's cave with extension to the CPA cistern. Patient underwent retrosigmoid craniectomy for partial resection of the CPA angle component of the mass. Surgical pathology confirmed the diagnosis of HB and patient was scheduled for subsequent radiotherapy of the residual mass. Conclusions We present an exceptional case of supratentorial HB without associated von Hippel-Lindau (VHL) disease, which was predominantly located in the cavernous sinus and Meckel's cave and led to multiple cranial nerve symptoms. We describe imaging characteristics and radiologic-pathologic correlation of this atypically located HB, which can be difficult to consider in the differential diagnosis presurgically.

Pancreaticopleural Fistula: A Review of Imaging Diagnosis and Early Endoscopic Intervention.

A 49-year-old male with history of chronic alcohol-induced pancreatitis presented with one month of worsening left pleuritic chest pain and shortness of breath. Chest radiograph demonstrated bilateral pleural effusions. Thoracentesis revealed increased amylase in the pleural fluid. Computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) showed a fistula tract between the left pleural cavity and pancreas which was confirmed on endoscopic retrograde cholangiopancreatography (ERCP). Patient was treated with placement of a pancreatic stent with complete resolution of the fistula tract approximately in 9 weeks. A systematic literature search was performed on reported cases with pancreaticopleural fistula (PPF) who underwent early therapeutic endoscopy within the last 10 years. Imaging modalities, particularly CT and MRCP, play essential role in prompt preprocedural diagnosis of PPF. Early therapeutic ERCP is an effective and relatively safe treatment option for PPF, so invasive surgery may be avoided.

Lipoma arborescens of the bicipitoradial bursa.

Lipoma arborescens (LA) is a rare, benign articular lesion that is most commonly found within the suprapatellar recess of the knee. An extremely rare case of LA involving unilateral bicipitoradial bursa is described in this study. A 58-year-old male presented with a superficial soft mass on the volar aspect of the left forearm. The magnetic resonance imaging (MRI) examination demonstrated a lobulated complex mass containing multiple frond-like fatty nodules, along the distal biceps tendon in the bicipitoradial bursa. Ultrasound-guided biopsy of the lesion confirmed the diagnosis of LA and patient was scheduled for surgical excision. Recognizing the characteristic imaging of LA, particularly on MRI, is essential for accurate pre-procedural diagnosis.

Outcomes of assisted reproduction treatment after dopamine agonist -cabergoline- for prevention of ovarian hyper stimulation syndrome.

BACKGROUND: Release of vascular endothelial growth factor (VEGF) by ovaries in response to HCG administration is one of the main mechanisms of ovarian hyper stimulation syndrome. Since Dopamine/dopamine receptor2 (Dp-r2) pathway activity -mediated by VEGF/ Vascular endothelial growth factor receptor 2 (VEGFR- 2) signaling-, is associated with angiogenic events, dopamine agonists were used for the management of severe forms of OHSS. In order to assess the effects of Cabergoline on angiogenesis in the human endometrium, and subsequently its impacts on the implantation rate this study was conducted. METHODS: This historical cohort study was conducted based on existing data of 115 patients (20-40 years) whom underwent assisted reproductive treatment (ART) and with a high probability for developing OHSS between March 2007 and September 2008. Forty five cases received Cabergoline were compared to 70 control subjects. The statistical methods used were: Unpaired t-test for continuous variables and the chi-square test (or Fisher's exact test if required) for categorical variables. RESULTS: None of the patients (treatment or control group) developed OHSS. The etiologies of infertility and administration of GnRH agonist or antagonist protocols were similar in two groups (p>0.2). Number of transferred embryos and zygote intra-fallopian transfer (ZIFT) did not differ between the two groups (p≥0.06). Implantation rate in treatment (3.1%) and control (6.6%) subjects was similar (p=0.4). No significant difference was observed in fertilization rate, chemical, clinical and ongoing pregnancies between the two groups (p>0.5). CONCLUSION: Cabergoline can be safely administered in ART protocols to prevent OHSS, without compromising ART outcomes.

Analysis of urinary cathepsin C for diagnosing Papillon-Lefèvre syndrome.

Papillon-Lefèvre syndrome (PLS) (OMIM: 245000) is a rare disease characterized by severe periodontitis and palmoplantar keratoderma. It is caused by mutations in both alleles of the cathepsin C (CatC) gene CTSC that completely abrogate the proteolytic activity of this cysteine proteinase. Most often, a genetic analysis to enable early and rapid diagnosis of PLS is unaffordable or unavailable. In this study, we tested the hypothesis that active CatC is constitutively excreted and can be easily traced in the urine of normal subjects. If this is true, determining its absence in the urine of patients would be an early, simple, reliable, low-cost and easy diagnostic technique. All 75 urine samples from healthy control subjects (aged 3 months to 80 years) contained proteolytically active CatC and its proform, as revealed by kinetic analysis and immunochemical detection. Of the urine samples of 31 patients with a PLS phenotype, 29 contained neither proteolytically active CatC nor the CatC antigen, so that the PLS diagnosis was confirmed. CatC was detected in the urine of the other two patients, and genetic analysis revealed no loss-of-function mutation in CTSC, indicating that they suffer from a PLS-like condition but not from PLS. Screening for the absence of urinary CatC activity soon after birth and early treatment before the onset of PLS manifestations will help to prevent aggressive periodontitis and loss of many teeth, and should considerably improve the quality of life of PLS patients.

Conjunctival microvascular hemodynamics following vaso-occlusive crisis in sickle cell disease.

Painful vaso-occlusive crisis (VOC) is the clinical hallmark of sickle cell disease (SCD). Microcirculatory hemodynamic changes following painful VOC may be indicative of future development of VOC events in subjects with SCD. The purpose of the present study was to determine alterations in conjunctival microvascular hemodynamics during non-crisis state in SCD subjects with a history of VOC. Conjunctival microcirculation imaging was performed to measure conjunctival diameter (D) and axial blood velocity (V) in 10 control and 30 SCD subjects. SCD subjects were categorized into two groups based on their history of VOC within a 2-year period before imaging (with or without VOC-H) and also based on whether there was progression in the rate of VOCs during a 2-year period following imaging as compared to before imaging (with or without VOC-P). Conjunctival V was significantly higher in SCD subjects with VOC-H than in both control subjects and SCD subjects without VOC-H (P≤0.03). Conjunctival V was also significantly higher in SCD subjects with VOC-P compared with control subjects and SCD subjects without VOC-P (P≤0.03). Assessment of the conjunctival microcirculation may be useful for understanding hemodynamic changes that lead to VOC events in SCD subjects.

Changes in Conjunctival Hemodynamics Predict Albuminuria in Sickle Cell Nephropathy.

BACKGROUND: Albuminuria is an early manifestation of deterioration in renal function in subjects with sickle cell disease (SCD). Hyperfiltration may be an early mechanism for kidney damage in SCD. The purpose of the current study was to determine the association between conjunctival hemodynamics and albuminuria in SCD subjects with preserved glomerular filtration rate. METHODS: Conjunctival microcirculation imaging was performed to measure conjunctival diameter and axial blood velocity (V) in 35 SCD and 10 healthy control subjects. Albuminuria, defined as albumin excretion ratio (AER), was obtained from the medical charts. Based on the 95% CI of conjunctival V in control subjects (0.40-0.60 mm/s), SCD subjects were allocated to 3 groups: V1 <0.40 mm/s (n = 7), V2 of 0.40-0.60 mm/s (n = 18) and V3 ≥0.60 mm/s (n = 10). RESULTS: Mean log(AER) measurements in the V1, V2 and V3 groups were 1.08 ± 0.67, 1.39 ± 0.59 and 2.00 ± 0.91 mg/g creatinine, respectively, and followed a positive linear trend from the V1 to V3 groups (p = 0.01). By multivariate linear regression analysis, conjunctival V significantly correlated with albuminuria (p = 0.01) independent of age, blood pressure, α-thalassemia, hematocrit, white blood cell count and lactate dehydrogenase concentration. CONCLUSIONS: Increased conjunctival V is associated with albuminuria in SCD subjects. Assessment of conjunctival microvascular hemodynamics may improve our understanding of the pathophysiology and clinical management of sickle cell nephropathy.

Hemorrhagic Retinopathy after Spondylosis Surgery and Seizure.

PURPOSE: To report bilateral hemorrhagic retinopathy in an adult female subject after lumbar spinal surgery and seizure. CASE REPORT: A 38-year-old woman presented with bilateral blurry vision and spots in the visual field. The patient had lumbar spondylosis surgery that was complicated by a dural tear with persistent cerebrospinal fluid leak. Visual symptoms started immediately after witnessed seizure-like activity. At presentation, visual acuity was 20/100 and 20/25 in the right and left eye, respectively. Dilated fundus examination demonstrated bilateral hemorrhagic retinopathy with subhyaloid, intraretinal, and subretinal involvement. At 4-month follow-up, visual acuity improved to 20/60 and 20/20 in the right and left eye, respectively. Dilated fundus examination and fundus photography showed resolution of retinal hemorrhages in both eyes. CONCLUSIONS: The first case of bilateral hemorrhagic retinopathy after lumbar spondylosis surgery and witnessed seizure in an adult was reported. Ophthalmic examination may be warranted after episodes of seizure in adults.

Feasibility of assessment of conjunctival microvascular hemodynamics in unilateral ischemic stroke.

Since the internal carotid artery supplies blood to both the eye and the brain, ocular microvascular hemodynamics can be altered due to ischemic stroke. The purpose of the current study was to establish the feasibility of conjunctival microcirculation imaging for detection of inter-ocular differences in microvascular hemodynamics in subjects with unilateral ischemic stroke. Conjunctival microcirculation imaging was performed in both eyes of 15 healthy control subjects and 12 subjects following unilateral ischemic stroke. Diameter and axial blood velocity were measured in multiple conjunctival venules of each eye. A two-way repeated measures analysis of variance was performed to determine the effects of stroke (control vs. stroke) and side of stroke (ipsilateral vs. contralateral) on conjunctival diameter and axial blood velocity. There was no significant main effect of stroke on conjunctival diameter (P=0.7) or conjunctival axial blood velocity (P=0.9). There was no significant main effect of side of stroke on conjunctival diameter (P=0.8), but there was a significant main effect of side of stroke on conjunctival axial blood velocity (P=0.02). There was a significant interaction effect between stroke and side of stroke (P=0.04), indicating that conjunctival axial blood velocity was lower in ipsilateral eyes than in contralateral eyes of stroke subjects. Conjunctival axial blood velocity and internal carotid artery blood velocity were correlated in stroke subjects (r=0.75, P=0.01, N=10). Conjunctival microcirculation imaging is a feasible method to detect inter-ocular differences in microvascular hemodynamics in subjects with unilateral ischemic stroke.

Posterior segment toxicity after gemcitabine and docetaxel chemotherapy.

PURPOSE: To report outer retinal disruption and uveal effusion after gemcitabine and docetaxel combination therapy. CASE REPORT: A 78-year-old woman presented with blurry vision after two cycles of gemcitabine and docetaxel combination chemotherapy for stage IV sarcoma. At presentation, visual acuity was finger counting and 20/25 in the right and left eyes, respectively. Slit-lamp examination and B-scan ultrasonography revealed severe uveal effusion in the right eye and choroidal folds in the left eye. Spectral domain optical coherence tomography showed disruption of photoreceptor inner segment ellipsoid band in the right eye. The patient was monitored weekly with ophthalmic examination and B-scan ultrasonography, while continuing with gemcitabine monotherapy. At 8 weeks follow-up, uveal effusion improved considerably and visual acuity was 20/40 and 20/20 in the right and left eyes, respectively. CONCLUSIONS: Uveal effusion and outer retinal disruption were reported after gemcitabine and docetaxel chemotherapy. Early detection and close ophthalmic monitoring may allow concurrent cancer treatment and prevention of possible chemotherapy-induced ocular side effects.

Effects of optical blur reduction on equivalent intrinsic blur.

PURPOSE: To determine the effect of optical blur reduction on equivalent intrinsic blur, an estimate of the blur within the visual system, by comparing optical and equivalent intrinsic blur before and after adaptive optics (AO) correction of wavefront error. METHODS: Twelve visually normal subjects (mean [±SD] age, 31 [±12] years) participated in this study. Equivalent intrinsic blur (σint) was derived using a previously described model. Optical blur (σopt) caused by high-order aberrations was quantified by Shack-Hartmann aberrometry and minimized using AO correction of wavefront error. RESULTS: σopt and σint were significantly reduced and visual acuity was significantly improved after AO correction (p ≤ 0.004). Reductions in σopt and σint were linearly dependent on the values before AO correction (r ≥ 0.94, p ≤ 0.002). The reduction in σint was greater than the reduction in σopt, although it was marginally significant (p = 0.05). σint after AO correlated significantly with σint before AO (r = 0.92, p < 0.001), and the two parameters were related linearly with a slope of 0.46. CONCLUSIONS: Reduction in equivalent intrinsic blur was greater than the reduction in optical blur after AO correction of wavefront error. This finding implies that visual acuity in subjects with high equivalent intrinsic blur can be improved beyond that expected from the reduction in optical blur alone.

Conjunctival microvascular haemodynamics in sickle cell retinopathy.

PURPOSE: To determine alterations in bulbar conjunctival microvascular haemodynamics in sickle cell retinopathy (SCR) subjects with focal macular thinning (FMT). METHODS: Conjunctival microcirculation imaging and spectral domain optical coherence tomography (SD-OCT) were performed in 22 subjects (eyes) diagnosed with SCR. Based on evaluation of SD-OCT retinal thickness maps, eyes were assigned to one of the two groups: with or without FMT. Conjunctival venular diameter and axial blood velocity were measured in multiple venules in each eye by customized image analysis algorithms. Measurements were then categorized into two vessel size groups (vessel size 1 and 2) and compared between FMT groups. A Pearson correlation coefficient was computed to assess the relationship between retinal thickness and axial blood velocity. RESULTS: Mean age, haematocrit, sickle cell haemoglobin type and median retinopathy score were not significantly different between the two groups (p ≥ 0.1). Retinal thickness in parafoveal and perifoveal temporal subfields was significantly lower in eyes with FMT as compared to eyes without FMT (p ≤ 0.04). There was a significant effect of FMT on axial blood velocity (p = 0.04), while the effect of vessel size was not significant (p = 0.4). In vessel size 1, axial blood velocity was lower in eyes with FMT than in eyes without FMT (p = 0.03), while in vessel size 2, there was no statistically significant difference between FMT groups (p = 0.1). In vessel size 1, there was a significant positive correlation between axial blood velocity and retinal thickness in the perifoveal (r = 0.48, p = 0.02) and parafoveal (r = 0.43, p = 0.04) temporal subfields. CONCLUSION: Conjunctival axial blood velocity in small venules is reduced in SCR subjects with focal macular thinning.

Conjunctival and pulmonary hemodynamic properties in sickle cell disease subjects with and without pulmonary hypertension.

Conjunctival microvascular hemodynamic alterations were reported for the first time in sickle cell subjects with and without pulmonary hypertension. Assessment of the conjunctival microcirculation using noninvasive imaging may improve understanding of microvascular hemodynamic alterations that occur due to pulmonary hypertension in sickle cell disease.

Enucleation and evisceration: indications, complications and clinicopathological correlations.

AIM: To assess main indications, postoperative complications and clinicopathological correlation of ocular enucleation-evisceration. METHODS: A total of 107 subjects who underwent enucleation and/or evisceration and received hydroxyapatite implants (Scleral wrap or mesh) were assessed. For each patient clinicopathological data was collected which included demographic information, clinical history, primary clinical diagnosis, main cause of ophthalmic surgery (traumatic, non-traumatic), type of surgical procedure (enucleation, evisceration) and pathological report. Patients' postoperative clinical visits were checked for procedure-related complications during first year after surgery. RESULTS: ONE HUNDRED AND SEVEN PATIENTS (MALE: 65.4%; mean age: 26y) underwent enucleation (n=100) or evisceration (n=7) due to traumatic (n=41) and non-traumatic (n=66) causes. Disfiguring painful blind eye was the most common indication of surgery (66.4%), followed by leukocoria (19.6%) and endophthalmitis (4.7%). The main types of injury included firecracker, traffic and work accidents, and sharp object perforating injury. In 53 (80.3%) subjects in non-traumatic group the initial clinical diagnosis matched the histopathological results. Malignant tumors (retinoblastoma: 47.5%, malignant melanoma: 27.3%) were the most common pathological diagnoses followed by phthisis bulbi (25.8%). The most common procedure-related complications were major eye discharge (39.6%), and implant exposure and discharge (20.8%). CONCLUSION: Trauma and malignant tumors are the leading causes of enucleation-evisceration. Despite developing new techniques and materials, enucleation is still associated with considerable postoperative complications.

Attenuation of Hemodynamic Responses to Laryngoscopy and Tracheal Intubation: Propacetamol versus Lidocaine-A Randomized Clinical Trial.

The purpose of this study is to assess the effects of propacetamol on attenuating hemodynamic responses subsequent laryngoscopy and tracheal intubation compared to lidocaine. In this randomized clinical trial, 62 patients with the American Anesthesiologists Society (ASA) class I/II who required laryngoscopy and tracheal intubation for elective surgery were assigned to receive propacetamol 2 g/I.V./infusion (group P) or lidocaine 1.5 mg/kg (group L) prior to laryngoscopy. Systolic and diastolic blood pressures (SBP, DBP), mean arterial pressure (MAP), and heart rate (HR) were recorded at baseline, before laryngoscopy and within nine minutes after intubation. In both groups P and L, MAP increased after laryngoscopy and the changes were statistically significant (P < 0.001). There were significant changes of HR in both groups after intubation (P < 0.02), but the trend of changes was different between two groups (P < 0.001). In group L, HR increased after intubation and its change was statistically significant within 9 minutes after intubation (P < 0.001), while in group P, HR remained stable after intubation (P = 0.8). Propacetamol 2 gr one hour prior intubation attenuates heart rate responses after laryngoscopy but is not effective to prevent acute alterations in blood pressure after intubation.

Therapeutic effects of oral zinc supplementation on acute watery diarrhea with moderate dehydration: a double-blind randomized clinical trial.

BACKGROUND: To assess the therapeutic effects of oral zinc supplementation on acute watery diarrhea of children with moderate dehydration. METHODS: All 9-month to 5-year-old children who were admitted with acute watery diarrhea and moderate dehydration to the Children Ward of Motahari Hospital, Urmia, Iran in 2008 were recruited. After the application of the inclusion and exclusion criteria, the patients were randomly allocated to two groups: one group to receive zinc plus oral rehydration solution (ORS) and the other one to receive ORS plus placebo. All the patients were rehydrated using ORS and then receiving ORS for ongoing loss (10 ml/kg after every defecation). Additionally, the patients in the intervention group received zinc syrup (1 mg/kg/day) divided into two doses. A detailed questionnaire was filled daily for each patient by trained pediatrics residents; it contained required demographic characteristics, nutrition and hydration status, and disease progression. The primary outcome (frequency and consistency of diarrhea) and the secondary outcomes (duration of hospitalization and change in patients' weight) were compared between the two groups. RESULTS: The mean diarrhea frequency (4.5±2.3 vs. 5.3±2.1; P=0.004) was lower in the group receiving zinc +ORS; however, the average weight was relatively similar between the two groups (10.5±3.1 vs. 10.1±2.3; P=0.14). The qualitative assessment of stool consistency also confirmed earlier improvement in the treatment group in the first three days of hospitalization (P <0.05). The mean duration of hospitalization was significantly lower in the patients receiving zinc supplements (2.5±0.7 vs. 3.3±0.8 days; P=0.001). CONCLUSION: Our results imply the beneficial effects of therapeutic zinc supplementation on disease duration and severity in patients with acute diarrhea and moderate dehydration in Iran. TRIAL REGISTRATION NUMBER: IRCT201201241580N2.

A Case of Colorectal Cancer during Pregnancy: A Brief Review of the Literature.

The incidence of colorectal cancer (CRC) during pregnancy is so rare. Herein we present a case of colorectal cancer that was missed by pregnancy all over the pregnancy period. The patient was a 37-year-old woman (gravid 4, para 2) referred with the complaints of vaginal discharge and suspicious rupture of membrane (ROM). The patient was pale and the initial physical examination revealed dilation of two fingers, effacement about 30%. She underwent emergent cesarean section which showed adhesions surrounding the uterus, the bladder, and the abdominal wall. Forty days postoperatively, the patient presented with abdominal pain in the left upper quadrant (LUQ). Imaging confirmed a mass in LUQ. Partial colectomy of transverse colon (20 cm) was performed. Postoperative histopathologic study revealed a 7 ∗ 6 ∗ 5 cm mass in transverse colon compatible to stage IIa of the Duck class (T3, N0, Mx). Adjuvant chemotherapy was applied and the patient showed improvements during 7 months followup after surgery. Colorectal cancer in pregnancy is associated with diagnostic and therapeutic challenges which mostly lead to late diagnosis in advanced stages and poor prognosis. A targeted program to improve the general population knowledge and the establishment of a national consultant and screening program particularly for women with a planned pregnancy in the high risk group might be beneficial.