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.

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.

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.

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.

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.

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.

Prevalence of oral mucosal lesions in an adult Iranian population.

BACKGROUND: Nowadays the importance of oral health to life quality is not obvious to anyone in our world. Oral lesions can interfere with daily social activities in involved patients through impacts on mastication, swallowing and speech and symptoms like xerostomia, halitosis or dysesthesia. OBJECTIVES: To assess the prevalence and types of oral lesions in a general population in Rasht, Northern Province of Iran. PATIENTS AND METHODS: 1581 people aged > 30 years old who were inhabitant of Rasht, Iran, enrolled in a cross-sectional study. For each individual a detailed questionnaire based on the world health organization (WHO) guidelines in order to diagnosis of the lesions was filled and it contained all the required data. Participants were divided into two groups with and without oral mucosal lesions and oral mucosal lesions were divided into two groups with and without. Demographic characteristics and clinical information including age, sex, smoking (cigarette and tobacco), opium consumption, medication and oral and dental hygiene were collected and compared between these two groups. RESULTS: The prevalence of mucosal lesions in our study was 19.4%. Our data demonstrated higher prevalence of oral mucosal lesions in males and young adults (30-40 years). The most common mucosal lesion among our participants was Fissured tongue(4%), followed by Fordyce granules(2.8%), geographic tongue(2.6%) , Pigmentation(2.5%), Candida(1.8%), Smoker Plate(1.6%), lingual Varices(1.5%), Petechiae(1.4%) and lingual labial(1.4%) . Leukoplakia was observed only in two people (0.1%).No case of malignant lesions was detected. No statistically significant difference was confirmed between the two groups regarding smoking, opium consumption, medication and oral and dental hygiene. CONCLUSIONS: Our data has provided baseline information about epidemiologic aspects of oral mucosal lesions which can be valuable in organized national program targeting on oral health and hygiene in the society.

Clinical responses to rituximab in a case of neuroblastoma with refractory opsoclonus myoclonus ataxia syndrome.

Opsoclonus myoclonus ataxia syndrome (OMS) is a rare neurologic syndrome. In a high proportion of children, it is associated with neuroblastoma. The etiology of this condition is thought to be immune mediated. In children, immunotherapy with conventional treatments such as corticosteroids, intravenous immunoglobulin, adrenocorticotropic hormone, and even antiepileptic drugs has been tried. Recently rituximab has been used safely for refractory OMS in children with neuroblastoma. Our patient was a 3.5-year-old girl referred for ataxia and dancing eye movements starting since 1.5 years ago. She was diagnosed with neuroblastoma on imaging studies on admission. The OMS was refractory to surgical resection, chemotherapy, corticosteroids, and intravenous immunoglobulin. Patient received rituximab simultaneously with chemotherapy. The total severity score decreased by 61.1% after rituximab. Patient's ataxia markedly improved that she was able to walk independently after 6 months. Our case confirmed the clinical efficacy and safety of rituximab in a refractory case of OMS.

Estradiol supplementation during the luteal phase in poor responder patients undergoing in vitro fertilization: a randomized clinical trial.

PURPOSE: This study was designed to evaluate the effects of adding Estradiol (E2) supplementation to progesterone (P) on improvement of pregnancy outcomes in poor responder patients who underwent in vitro fertilization (IVF). METHODS: In a prospective randomized clinical trial, 118 poor responder patients, older than 38 years without contraindications of estradiol consumption from Infertility clinic of a university hospital were randomly divided (by computerized software) into two groups. Control group (59 patients) received only P and intervention group (59 patients) received P and E2 (4 mg/d). Supplementation was done with 4 mg E2 in the luteal phase. Fertilization rate, implantation rate, biochemical and clinical pregnancy rates, abortion rate, ongoing pregnancy, multiple pregnancy and ectopic pregnancy rates were documented for those who completed the study protocol in each group (per protocol analysis) and compared between groups. RESULT: Fifty five patients in control group and 53 patients in intervention group successfully completed the study protocol. Treatment outcomes were not significantly different between two groups. CONCLUSION: For poor responder women who underwent IVF, addition of E2 to P supplementation could not significantly improve pregnancy outcomes.

The protective effect of vitamin E on locus coeruleus in early model of Parkinson's disease in rat: immunoreactivity evidence.

BACKGROUND: Free radical formation and oxidative stress might play an important role in the pathogenesis of Parkinson's disease (PD). In vitro data indicate that neuromelanin (NM) pigment is formed the excess cytosolic catecholamine that is not accumulated into synaptic vesicles via the vesicular monoamine transporter 2 (VMAT2). We designed this study to investigate the neuroprotective effects of vitamin E in the early model of PD. METHODS: Male rats (n = 40) with unbiased rotational behavior were randomly divided into five groups: sham operated group (SH, n = 8), vehicle-treated SH group (SH + V, n = 8), vitamin E-treated SH group (SH + E, n = 8), vehicle-treated lesion group (L + V, n = 8) and vitamin E-treated lesion group (L + E, n = 8). Unilateral intrastriatal 6-hydroxydopamine (12.5 microl) lesioned rats were treated intramuscularly with alpha-tocopherol acid succinate (24 I.U/kg, intramuscular [i.m.]) 1 h before surgery and three times per week for 2 month post-surgery. To evaluate the vitamin E pretreatment efficacy, tyrosine hydroxylase (TH) immunoreactivity and immunostaining intensity (ISI) for monoamine transporter 2 were used. RESULTS: TH immunohistochemical analyses showed a reduction of 20 percent in locus coeruleus (LC) cell number of vitamin E pretreated lesioned group but the cell number dropped to 60 percent in the lesioned group. The ISI of the cells was measured for VMAT2 in LC. Lesioned groups: 1) had the lowest VMAT2 ISI of all neurons; 2) There was an inverse relationship between VMAT2 ISI and NM pigment in the locus and 3) Neurons with the highest VMAT2 ISI also had high TH ISI. CONCLUSION: The data support the hypothesis that repeated i.m. administration of vitamin E exerts a protective effect on the LC neurons in the early model of PD.