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Cardiovascular Diagnosis and Therapy Oct 2021Thoracic outlet syndrome (TOS) is used to describe the constellation of symptoms arising from neurovascular compression of the thoracic outlet. The structures passing... (Review)
Review
Thoracic outlet syndrome (TOS) is used to describe the constellation of symptoms arising from neurovascular compression of the thoracic outlet. The structures passing through the thoracic outlet include the subclavian artery, subclavian vein and trunks of the brachial plexus. Patients may experience symptoms related to compression of any one or various combinations of these structures. Arterial pathology as the cause of TOS is rare, though repetitive overhead arm motion, such as seen in athletes, is a risk factor for developing arterial TOS (aTOS). Symptoms include chronic findings, such as pallor, arm claudication or cool arm. Currently diagnosis of aTOS is made using clinical and imaging parameters which include focused history and physical including provocative maneuvers and imaging follow-up ranging from angiography to MRI. Occasionally, acute thrombosis can result in limb threatening ischemia requiring emergent catheter directed thrombolysis. Outside of acute limb ischemia, management of aTOS is variable, however typically begins with conservative measures such as physical therapy. In patients who do not respond or progress on conservative management, surgical decompression may be performed. Open or endovascular treatment of subclavian artery pathology may be necessary for recalcitrant cases. In this article, the aim is to review the elements involving diagnosis and management of aTOS.
PubMed: 34815963
DOI: 10.21037/cdt-20-149 -
Eye and Brain 2021Optic nerve cupping or enlargement of the cup-to-disc ratio is widely recognized as a feature of glaucoma, however it may also occur in non-glaucomatous optic... (Review)
Review
Optic nerve cupping or enlargement of the cup-to-disc ratio is widely recognized as a feature of glaucoma, however it may also occur in non-glaucomatous optic neuropathies. The most well-recognized non-glaucomatous optic neuropathies that cause cupping include compressive optic neuropathies, arteritic anterior ischemic optic neuropathies, hereditary optic neuropathies, and optic neuritis. Cupping is thought to consist of two main components: prelaminar and laminar thinning. The former is a shallow form of cupping and related to loss of retinal ganglion cells, whereas the latter involves damage to the lamina cribrosa and peripapillary scleral connective tissue. Differentiating glaucomatous and non-glaucomatous optic nerve cupping remains challenging even for experienced observers. Classically, the optic nerve in non-glaucomatous causes has pallor of the neuroretinal rim, but the optic nerve should not be examined in isolation. The patient's medical history, history of presenting illness, visual function (visual acuity, color vision and visual field testing) and ocular examination also need to be considered. Ancillary testing such as optical coherence tomography of the retinal nerve fiber layer and ganglion cell layer-inner plexiform layer may also be helpful in localizing the disease. In this review, we review the non-glaucomatous causes of cupping and provide an approach to evaluating a patient that presents with an enlarged cup-to-disc ratio.
PubMed: 34934377
DOI: 10.2147/EB.S272343 -
Texas Heart Institute Journal 2000Neurally mediated syncope is a disorder of the autonomic regulation of postural tone, which results in hypotension, bradycardia, and loss of consciousness. A wide... (Review)
Review
Neurally mediated syncope is a disorder of the autonomic regulation of postural tone, which results in hypotension, bradycardia, and loss of consciousness. A wide variety of stimuli can trigger this reflex, the most common stimulus being orthostatic stress. Typically, a patient with neurally mediated syncope experiences nausea, lightheadedness, a feeling of warmth, and pallor before abruptly losing consciousness. If the cause of syncope is unclear, a stepwise approach is necessary to arrive at the diagnosis. The diagnosis of neurally mediated syncope can be confirmed by a head-up tilt-table test. Treatment options include behavioral modification and several pharmacologic therapies. For severe recurrent syncope unresponsive to conventional treatment, a pacemaker can be implanted.
Topics: Humans; Syncope, Vasovagal
PubMed: 11093411
DOI: No ID Found -
Paediatrics & Child Health Feb 2019
PubMed: 30792590
DOI: 10.1093/pch/pxy042 -
BMJ Case Reports Sep 2015A previously well 4-year-old boy presented to the emergency room with progressive cyanosis, pallor and vomiting over the last 5 h. Oxygen saturation on pulse oximetry...
A previously well 4-year-old boy presented to the emergency room with progressive cyanosis, pallor and vomiting over the last 5 h. Oxygen saturation on pulse oximetry was 87-89% despite 9 L/min of supplemental oxygen. He was tachypnoeic and had a systolic heart murmur, with no other findings on clinical examination. In his medical history, there was record of a restrictive atrial septal defect, with a normal echocardiogram from 3 years before. He had no relevant family history. His shoes appeared to have been recently painted, which raised the suspicion of methaemoglobinaemia, presumptively caused by aniline-containing shoe dye. The shoes were removed promptly and his feet washed profusely. After confirming the diagnosis, methylene blue was started. The level of methaemoglobin decreased rapidly and the boy made a full recovery.
Topics: Child, Preschool; Cyanosis; Enzyme Inhibitors; Ethanol; Humans; Male; Methemoglobinemia; Methylene Blue; Oximetry; Paint; Shoes; Tachypnea; Treatment Outcome; Vomiting
PubMed: 26338243
DOI: 10.1136/bcr-2015-210619 -
Journal of Clinical and Diagnostic... Nov 2016Senior Loken Syndrome (SLS) is a rare genetic disorder having juvenile nephronophthisis and retinal degeneration progressing to blindness and end stage renal disease....
Senior Loken Syndrome (SLS) is a rare genetic disorder having juvenile nephronophthisis and retinal degeneration progressing to blindness and end stage renal disease. The present case report is about two sisters who presented with decreased visual acuity and end stage renal disease. Both had decreased vision, pallor, deranged renal function test and chronic malnutrition. Investigations revealed anaemia, uraemia, raised creatinine, low Glomerular Filteration Rate (GFR). Ophthalmology examination revealed nystagmus, retinal examination depicted pale optic disc and pigmentary changes in the retina. Renal ultrasound showed grade III renal parenchymal changes and bilateral cortico-medullary cysts. These cases are presented to highlight the importance of timely recognition of renal derangement in patients with retinal disease to delay end stage renal disease.
PubMed: 28050464
DOI: 10.7860/JCDR/2016/21832.8816 -
Cureus Aug 2023Rectus sheath hematoma (RSH) is one of the surgical emergencies that mimics peritonitis or other causes of acute abdominal pain. It is usually seen in old age,...
Rectus sheath hematoma (RSH) is one of the surgical emergencies that mimics peritonitis or other causes of acute abdominal pain. It is usually seen in old age, post-trauma, anticoagulation therapy pregnancy, chronic cough, and liver disease. Nevertheless, RSHs can be spontaneous without any underlying predisposing factors. Here, we present a 51-year-old female with sudden onset abdominal pain, abdominal distention, hypotension, and severe pallor. After initial resuscitation, the patient underwent radiological imaging. This suggested an RSH with active bleeding from the inferior epigastric artery or profunda femoris artery. The patient underwent digital subtraction angiography and angioembolization of the profunda femoris branch. After a few days, the patient continued deteriorating and succumbed to acute respiratory distress syndrome (ARDS).
PubMed: 37753012
DOI: 10.7759/cureus.44138 -
Acta Informatica Medica : AIM : Journal... Dec 2022Retinitis pigmentosa (RP) is a set of inherited rod-cone degenerative diseases that clinically presents with similar signs and symptoms. Mutations in one of more than 70... (Review)
Review
BACKGROUND
Retinitis pigmentosa (RP) is a set of inherited rod-cone degenerative diseases that clinically presents with similar signs and symptoms. Mutations in one of more than 70 genes are involved. Patients will commonly present with bone-spicule pigment formation, waxy optic nerve pallor, and attenuated blood vessels in the posterior pole.Symptoms often begin with progressive night blindness, mid-peripheral visual field defects, and eventual tunnel vision. Central vision loss will ultimately occur following loss of rod function. Complete blindness is uncommon.
OBJECTIVE
The aim of this article is to present two cases of retinitis pigmentosa (mother and daughter) trough optalmologic exams in our clinic. The next aim it to show how to menage a low vision service and to treat cystoid macular oedema as a complication of retinitis pigmentosa.
METHODS
All medical reports are shown in this article. Every diagnostic tool as well as report is a part from our archived history of the patients and has been throughly analysed. We also reviewed available literature using the key words retinitis pigmentosa, cystoid macular oedema, gene therapy.
CASE PRESENTATION
A 38 year old female patient for a low vision consultation. The patient was legally blind secondary to retinitis pigmentosa, which was diagnosed in her late 20s. She reported gradually progressive hazy central vision and decreasing peripheral vision in both eyes as well as severe night blindness. Other than the diagnosis of retinitis pigmentosa in both eyes,the patient had no other remarkable ocular conditions. Findings at that visit included unaided distance visual acuities VOD: 0,04 VOS: 0,06. Pupils were round with brisk responses. Extraocular muscle motility was full in both eyes. Confrontation methode visual fields were noted as temporal loss in the right eye and superior and temporal loss in the left eye. The perimetry test could not be performed due to the lack of correspondece of the patient even after a couple repetitions of the perimetry. She had normal ocular adnexa and quiet lids, conjunctiva, and sclera in both eyes. Corneas in both eyes were noted as clear epithelium, clear stroma, and clear endothelium. Anterior chambers had normal depth, iris with no pathological findings in both eyes; lens incipient sclerotic. Intraocular pressures were noted as 22 mmHg in both eyes with Icare, 21mmHg and 19 mmHg with aplanation tonometry; pahimetry corretional factor was +1 on both eyes. The vitreous was clear in both eyes. Both optic nerves were measured as 0.4 cup-to-disc ratios with no disc edema, disc hemorrhages, notching, or thinning noted.Waxy disc pallor and attenuated blood vessels were observed in both eyes. The macula in both eyes had retinal pigment epithelium (RPE) changes with no edema or hemorrhages. Bone spicule changes were noted 360 in the periphery of both eyes with no holes or tears(Figure 1a+1b+1c+1d).
CONCLUSION
We presented two cases of retinitis pigmentosa - the mother with diagnosed RP more than 15 years ago in need for low vision rehabilitation service and the daughter that got diagnosed after our initial examination and with complications in visual impairment through cystoid macular oedema.
PubMed: 36467319
DOI: 10.5455/aim.2022.30.329-333 -
The Journal of Maternal-fetal &... Dec 2023Congenital segmental dilatation of the intestine (CSDI) is a rare gastrointestinal condition. We conducted a scoping review through MEDLINE and Google Scholar,... (Review)
Review
OBJECTIVE
Congenital segmental dilatation of the intestine (CSDI) is a rare gastrointestinal condition. We conducted a scoping review through MEDLINE and Google Scholar, collecting data from 1959 through August 2020 to better understand this peculiar disease.
METHODS
The clinical and pathological features of 150 patients were reviewed.
RESULTS
The mean age was 25.9 days, and 61.3% of patients were male. An antenatal diagnosis was made in 15.3% of patients. Predominant symptoms included abdominal distension (83.9%) and vomiting (61.3%). Pallor and anemia were associated with ileal CSDI. The most common sites of the lesion were the ileum (56%) and colon (27.3%). Associated anomalies occurred in 57.3% of the patients, of which the most common included other abnormalities of the digestive system (69.8%), abdominal wall (19.8%), and cardiovascular system (11.6%). Resection and anastomosis was performed in 83.3% of patients. Postoperative complications occurred in 10%. Normal ganglion cells were commonly found (97.3%), while muscle layer hypertrophy and atrophy were found in 14.7% and 13.3% of the patients, respectively. Abnormal interstitial cells of Cajal were identified in four patients. Death occurred in 12.7% of patients. Demise was significantly associated with the duodenal location of CSDI (Mantel-Cox test, = 0.002).
CONCLUSION
CSDI remains poorly understood, and mortality is associated chiefly with its duodenal location. Further research is needed, and biorepositories should be promptly set up to study this disease in the future better.
Topics: Pregnancy; Humans; Female; Male; Adult; Dilatation; Intestinal Diseases; Colon
PubMed: 37726217
DOI: 10.1080/14767058.2023.2259047