Diaphragm Sellae Meningioma: Distinct Clinical, Anatomic, and Surgical Considerations: 2-Dimensional Operative Video

2021 ◽  
Author(s):  
Ketan R Bulsara ◽  
Walid Ibn Essayed ◽  
Emad Aboud ◽  
Ossama Al-Mefty

Abstract Nestled in the parasellar region, surrounded by critical neurovascular structures, diaphragm sellae meningiomas although rare present distinct clinical, radiological, and surgical considerations.1-3 Consequently, they present surgical challenges that could be overcome with technical nuances. The origin of this meningioma on the diaphragm creates a distorted anatomy, which must be comprehended for the safe approach and resection.  Three distinct subtypes of diaphragm sellae meningiomas are described, each with distinctive clinical presentations and surgical treatment implications.2 Type A originates from the upper leaf of diaphragm sellae pushing the stalk posteriorly. It usually presents with unilateral visual loss. Type B originates from the upper leaf of the diaphragm sellae pushing the stalk anteriorly. It presents with few visual symptoms, but memory disturbance and hypopituitarism are common. Type C originates from the inferior leaf of the diaphragm sellae (intrasellar meningioma) presenting with bitemporal hemianopsia and hypopituitarism. Recognizing these variations in this rare tumor subtype is critical to minimizing potential adverse outcomes associated with operative treatment. The cranial approach has been the recommended route for these lesions with an exception of the intrasellar type.1,3  In this article, we depict the pathological anatomy and demonstrate the surgical nuances in handling diaphragm sellae meningioma resection through a cranio-orbital approach4 in a patient who had an unsuccessful trans-sphenoidal resection attempt. The patient consented for the procedure.  Image at 1:38 from Al-Mefty O, Operative Atlas of Meningiomas, © LWW, 1997, with permission. Image at 8:56 from Kinjo et al,2 Diaphragma sellae meningiomas, case reports, Neurosurgery, 1995, 36(6), 1082-1092, by permission of the Congress of Neurological Surgeons.

2012 ◽  
Vol 50 (05) ◽  
Author(s):  
Z Visnyei ◽  
E Schafer ◽  
K Kardos ◽  
L Szentpétery ◽  
A Iványi ◽  
...  

2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Jeff John ◽  
Ken Kesner ◽  
John Lazarus

Abstract Background Squamous cell carcinoma (SCC) of the scrotum was the first malignancy known to be associated with exposure to an occupational carcinogen—in this case, soot trapped in the breeches of chimney sweeps. Better civil rules and regulations and the replacement of hearths with other forms of heating have rendered SCC of the scrotum a rarity. We report two cases of scrotal SCC with vastly differing clinical presentations and management. Case presentation Case 1 had T1 N0 M0 disease and presented with a small (< 2 cm), innocuous-looking, non-healing ulcer of eight years duration. A punch biopsy revealed a superficially invasive SCC confirmed on immunohistochemical profiling. A wide local excision of the lesion was subsequently performed. Follow-up at three years showed no signs of recurrence. Case 2 presented with T4 N1 M1 disease and rapidly progressing locally destructive mass. A punch biopsy of the scrotal lesion confirmed invasive moderately differentiated focally keratinising SCC. The metastatic evaluation confirmed the presence of metastatic, extensive para-aortic lymphadenopathy. He was managed with cisplatin-based chemoradiotherapy. Conclusion Early detection and management of patients with SCC of the scrotum are essential. If the diagnosis is delayed, treatment options become limited, and the prognosis is poor. Notwithstanding the rarity of this disease, multicentre trials are needed to provide more precise guidelines as to the optimal management of these patients.


2020 ◽  
pp. 1753495X2097079
Author(s):  
Niccole Ranaei-Zamani ◽  
Mandeep K Kaler ◽  
Rehan Khan

Proteus syndrome is a rare, multi-system, genetic syndrome characterised by atypical and excessive growth of skeletal tissue. Clinical presentations include abnormal musculoskeletal growth and cutaneous lesions. Due to its rarity, there have been a limited number of published case reports of Proteus syndrome. This is the first case report on the management of Proteus syndrome in pregnancy. We present the case of a pregnant woman with Proteus syndrome in her first pregnancy in a large teaching hospital and discuss the considerations and challenges faced in her antenatal, intrapartum and postnatal care.


BMJ Open ◽  
2018 ◽  
Vol 8 (12) ◽  
pp. e022743 ◽  
Author(s):  
Debora Farias Batista Leite ◽  
Aude-Claire Morillon ◽  
Elias F Melo Júnior ◽  
Renato T Souza ◽  
Ali S Khashan ◽  
...  

IntroductionFetal growth restriction (FGR) is a relevant research and clinical concern since it is related to higher risks of adverse outcomes at any period of life. Current predictive tools in pregnancy (clinical factors, ultrasound scan, placenta-related biomarkers) fail to identify the true growth-restricted fetus. However, technologies based on metabolomics have generated interesting findings and seem promising. In this systematic review, we will address diagnostic accuracy of metabolomics analyses in predicting FGR.Methods and analysisOur primary outcome is small for gestational age infant, as a surrogate for FGR, defined as birth weight below the 10th centile by customised or population-based curves for gestational age. A detailed systematic literature search will be carried in electronic databases and conference abstracts, using the keywords ‘fetal growth retardation’, ‘metabolomics’, ‘pregnancy’ and ‘screening’ (and their variations). We will include original peer-reviewed articles published from 1998 to 2018, involving pregnancies of fetuses without congenital malformations; sample collection must have been performed before clinical recognition of growth impairment. If additional information is required, authors will be contacted. Reviews, case reports, cross-sectional studies, non-human research and commentaries papers will be excluded. Sample characteristics and the diagnostic accuracy data will be retrieved and analysed. If data allows, we will perform a meta-analysis.Ethics and disseminationAs this is a systematic review, no ethical approval is necessary. This protocol will be publicised in our institutional websites and results will be submitted for publication in a peer-reviewed journal.PROSPERO registration numberCRD42018089985.


2016 ◽  
Vol 33 (S1) ◽  
pp. 1-1 ◽  
Author(s):  
J. Rebelo ◽  
M. Oliveira ◽  
P. Nunes

IntroductionNiemann-Pick type C disease (NPCD) is a rare metabolic illness, with autosomal recessive inheritance. NPCD has a heterogeneous presentation, with non-specific psychiatric symptoms, mostly affective and psychotic features and also cognitive deficits.Objectives and methodsWe present the case reports of two brothers with an adolescent-adult onset and discuss the evolution of their neuropsychiatric manifestations.ResultsThe patients have now 35 and 31 years old and the youngest was the first to develop clinical manifestations of the disease. From 16 years old, he developed unspecified neurological impairment with gait imbalance. In the next years, the neurologic manifestations exacerbated, with dysarthria, ataxic gait, and his academic performance declined. With 24 years old, he presented acute psychosis, with unstructured delusion and auditory hallucinations. The acute psychotic symptomatology remitted with olanzapine but he revealed social withdrawal, apathy and progressive cognitive decline that persist until now. His brother, whose diagnosis was made in the course of the family genetic study, developed the first signs of the NPCD with 19 years old. He presented neuropsychiatric compromise, with impaired learning, social isolation and insomnia. They are receiving specific treatment with miglustat and symptomatic treatment for the psychiatric manifestations.ConclusionsNPCD is a rare metabolic disease, with neuropsychiatric compromise. No general psychopathological profile has been associated to NPCD. Sometimes psychiatric symptoms dominate the initial clinical presentation, with neuro-visceral signs appearing later. An atypical psychiatric symptomatology should be extensively investigated in order to exclude organic causes, including metabolic diseases like NPCD.Disclosure of interestThe authors have not supplied their declaration of competing interest.


2020 ◽  
Vol 20 (5) ◽  
pp. 404-414
Author(s):  
May Yung Tiet ◽  
Rita Horvath ◽  
Anke E Hensiek

Ataxia telangiectasia is an autosomal recessive DNA repair disorder characterised by complex neurological symptoms, with an elevated risk of malignancy, immunodeficiency and other systemic complications. Patients with variant ataxia telangiectasia—with some preserved ataxia telangiectasia-mutated (ATM) kinase activity—have a milder and often atypical phenotype, which can lead to long delays in diagnosis. Clinicians need to be aware of the spectrum of clinical presentations of ataxia telangiectasia, especially given the implications for malignancy surveillance and management. Here, we review the phenotypes of ataxia telangiectasia, illustrated with case reports and videos, and discuss its pathological mechanisms, diagnosis and management.


2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Rafael Aporta Rodriguez ◽  
Mariola García Montero ◽  
Jose Pablo Lorente Aporta ◽  
Carolina Gallego Luque ◽  
Alfonso Chacón Mayor ◽  
...  

Iron deficiency and iron deficiency anemia during pregnancy call for safe treatment options that raise maternal hemoglobin levels and counterbalance iron demand and blood volume expansion while minimizing risks for the growing fetus. This retrospective study describes experience with intravenous ferric carboxymaltose given to pregnant women in a tertiary hospital in Spain. In a 5-year period, 95 pregnant women who had pretreatment hemoglobin <10 g/dL and at least one time of ferric carboxymaltose administration during pregnancy were included. Main outcome measures were week of pregnancy at iron administration, Hb levels before and after treatment, neonatal 5-minute Apgar scores, and birth weight. The majority received one dose of ferric carboxymaltose (1000 mg iron) during advanced pregnancy (median 31 weeks; interquartile range [IQR]: 27; 37 weeks) with minor to no adverse outcomes. Overall, median Hb increased from 8.5 g/dL (8.1; 8.9 g/dL) before treatment to 11.0 g/dL (9.9; 11.7 g/dL) after treatment. Normal Apgar scores were observed in all 97 infants (median birth weights 3560 g, 3270, and 3798 g). Four women received ferric carboxymaltose in the first trimester and twenty-eight during the second trimester without adverse outcomes for mother or child. These cases add to the evidence that ferric carboxymaltose administration during pregnancy is effective and safe.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
R Murphy ◽  
R K Kerley ◽  
P K Kearney

Abstract Background Cardiac implantable electronic devices (CIEDs) were long considered a contraindication for magnetic resonance imaging (MRI). Modern devices are now MRI conditional, but still the majority of CIEDs in the population are legacy devices and are classified as unsafe for MRI. There is growing consensus that MRI is also safe in these patients. Purpose The purpose of this study was to perform an up to date systematic review of the evidence evaluating the use of MRI in patients with non-conditional CIEDs. Methods Searches of the PubMed, CINAHL and Embase databases were performed. Studies that assessed the rate of adverse outcomes after MRI in patients with non-conditional CIEDs were included. Studies were excluded if they did not disclose the conditionality of patients CIEDs. Case reports or case series were not included. Results 36 cohort studies were identified. No patient in these studies died during or immediately after MRI. Symptom associated with either torque or heating occurred in &lt;1% of patients. Electrical resets occurred 1–2% of patients. There were no cases of non-conditional lead or generator failure. Inappropriate pacing occurred in &lt;1% of patients. No ICD shocks occurred during MRI. Changes in CIED parameters occurred in 1–4% of patients. Conclusions This systematic review highlights the relative safety of the use of MRI in patients with non-conditional CIEDs. Demonstrated be the fact that no deaths or device shocks have been suffered as a consequence of MRI in any of these studies, and the extremely low incidence of device or lead related complications. Still strict selection and monitoring protocol should be used when imaging these patients. FUNDunding Acknowledgement Type of funding sources: None.


2020 ◽  
Vol 25 (4) ◽  
pp. 336-339
Author(s):  
Jeffrey J. Cies ◽  
Wayne S. Moore ◽  
Adela Enache ◽  
Arun Chopra

Pharmacokinetic data regarding ceftaroline fosamil (CPT) penetration into cerebrospinal fluid (CSF) are limited to a rabbit model (15% inflamed) and adult case reports. We describe serum and CSF CPT concentrations in a 21-year-old, 34.8 kg female, medically complex patient presented with a 4-day history of fevers (Tmax 39.2°C), tachypnea, tachycardia, fatigue, and a 1-week history of pus and blood draining from the ventriculopleural (VPL) shunt. A head CT and an ultrasound of the neck revealed septated complex fluid collection surrounding the shunt. Therapy was initiated with vancomycin and ceftriaxone. Blood and CSF cultures from hospital day (HD) 1 were positive for methicillin-resistant Staphylococcus aureus with a CPT MIC of 0.5 mg/L and a vancomycin MIC range of 0.5 to 1 mg/L. On HD 3, CPT was added. On HD 7, simultaneous serum (69.4, 44, and 30.2 mg/L) and CSF (1.7, 2.3, and 2.3 mg/L) concentrations were obtained at 0.25, 1.5, and 4.75 hours from the end of an infusion. Based on these concentrations, CPT CSF penetration ratio ranged from 2.4% to 7.6%. After addition of CPT, the blood and CSF cultures remained negative on a regimen of vancomycin plus CPT. On HD 14, a new left-sided VPL shunt was placed. The patient continued on CPT for a period of 7 days after the new VPL shunt placement. This case demonstrated CPT CSF penetration in a range of 2.4% to 7.6%, approximately half of the rabbit model. This allowed for CSF concentrations at least 50% free time &gt; 4 to 6× MIC of the dosing interval with a dosing regimen of 600 mg IV every 8 hours in a 34.8 kg chronic patient and resulted in a successful clinical outcome with no identified adverse outcomes.


2017 ◽  
Vol 14 (04) ◽  
pp. 204-208
Author(s):  
Joana Aquino ◽  
Sónia Lemos ◽  
Inês Balacó ◽  
Gabriel Matos ◽  
Graça Rocha ◽  
...  

Introduction Osteoarticular infections caused by atypical mycobacteria are uncommon, with osteitis being more common and arthritis being rare. We describe two cases of subacute osteomyelitis and one of chronic osteomyelitis with arthritis. Case Reports Three boys, aged between 7 and 20 months. Clinical presentations: case 1—swelling of right forearm; case 2—limping and swelling of right hallux; case 3—pain and restriction of motion of right lower limb with effusion. None had fever. All had received Bacillus Calmette–Guérin (BCG) and none had contact with tuberculosis. All had thrombocytosis, and erythrocyte sedimentation rate was between 17 and 44 mm/1st hour. All had osteolytic lesions on X-ray with a sclerotic halo and swelling of the surrounding soft tissues, and invasion of the metaphysis and/or epiphysis. Bone histopathological studies revealed granulomatous inflammation or epithelioid cells in all cases. Microbiological identification was positive by culture in one case (Mycobacterium bovis) and by polymerase chain reaction in two cases (Mycobacterium spp. and M. avium). Immunodeficiency screening was negative in all cases. All were treated with antituberculous agents and made good clinical and radiological recoveries. Discussion Osteolytic lesions should raise suspicion of mycobacterial infection, including BCG vaccine, particularly in the presence of granulomatous inflammation. The prognosis was favorable in all patients. Although immune deficits were not identified in any of our three patients, it should be routinely tested for in such cases.


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