Frequent Presentation Research Articles

PAPILLARY FIBROELASTOMA: AN UNUSUAL CAUSE OF TRANSIENT ISCHEMIC ATTACK

Abstract Papillary fibroelastomas (PFEs) are small, avascular benign tumors. Despite that, PFEs could lead to important clinical consequences such as transient ischemic attack and myocardial infarction. A 65–year–old female patient was admitted to our ER presenting a transient ischemic attack characterized by retrograde amnesia lasting about an hour. She had a history of systemic scleroderma and left breast cancer treated with local radiotherapy. In our hospital she underwent a neurological examination and a head CT which were negative for ongoing acute pathologies. An electrocardiogram was performed which showed sinus rhythm at 65 bpm. A transthoracic echocardiogram was also performed revealing a floating mass arising from left ventricular outflow tract between aortic and mitral valves. The transesophageal echocardiogram confirmed a solitary lesion approximately 2 centimeters long localized between A2 and A3 mitral valve scallops. The patient was therefore referred for surgery. During surgery the mass was found to arise from anterior mitral annulus and was resected. The histopathological examination of the mass confirmed the morphologic diagnosis of papillary fibroelastoma. Histologically PFEs are made up of a single layer of endocardial cells covering a matrix of collagen. The main risk factors for the development of PFEs are iatrogenic causes, such as radiotherapy, and autoimmune diseases such as systemic scleroderma. In 54% of cases PFEs are asymptomatic at the time of diagnosis. Since over 95% of PFEs present on the left side of the heart, systemic embolism is the most frequent presentation typically as transient ischemic attacks. Initial evaluation is accomplished with echocardiography. PFEs appear as echo–dense and pedunculated masses. Cardiac computed tomography and magnetic resonance imaging are second line modalities in PFEs evaluation. Definitive diagnosis of PFE requires pathological confirmation. Surgical resection remains the treatment of choice for all symptomatic patients. For symptomatic patients who are not surgical candidate, long term antiplatelets or anticoagulation therapy should be initiated. Asymptomatic patients should have surgical resection if the size of the tumor is > 1 cm. PFEs are associated with significant morbidity and mortality. Echocardiography remains the most utilized diagnostic modality. Surgical treatment should always be considered. Antithrombotic therapy should be considered in non–surgical candidates.

Serious Failure Modes After EVAR Are Device Specific.

Type I and III endoleaks following endovascular aneurysm repair (EVAR) can lead to catastrophic events that require major re-interventions. We reviewed our experience with aortic endograft re-interventions for type I and III endoleaks and other serious failures among different devices. We retrospectively reviewed patients with a prior EVAR who underwent open conversion (OC) or major endovascular intervention (MEI) (re-lining, cuff/limb extension, parallel graft) for type I/III endoleaks at our institution from 2002 to 2019. Baseline characteristics, procedural details, re-interventions, and outcomes were collected. A total of 229 patients (194 men) underwent re-interventions for type I and III endoleaks after EVAR (90 OC, 139 MEI) for devices implanted between 1997 and 2019. Average age at re-intervention was 78±8.5 years. A total of 135 (59%) were implanted at our institution, whereas 93 (41%) were referred. Median time to re-intervention was 4 years with 25% to 75% interquartile range (IQR) of 2.2-6.6 years. There was no significant difference in baseline demographics or type of re-interventions (OC/MEI) between device types. 42/229 (18%) presented with ruptured aneurysms, 20/229 (9%) were symptomatic, whereas the rest presented with asymptomatic radiographic findings. Type 1A endoleak was present in 146/229 (63.8%-72 with proximal migration), type IB in 46/229 (20.1%), type IIIA in 37/229 (16.6%), type IIIB in 15/229 (6.5%), and persistent aneurysm sac growth with no radiographic evidence of an endoleak in 6/229 (2.6%). Devices included most commercial products: AFX, Excluder, AneuRx, Ancure, Endurant, and Zenith. A smaller number of investigational devices accounted for the rest. Type 1A endoleak was the most common indication for re-intervention among all devices except for AFX and ancure devices, proximal migration was a frequent presentation with AneuRx. AFX devices more frequently presented with a type III and ancure devices more frequently presented with a type IB endoleak. Serious failure modes after EVAR differ between endografts and occur throughout the follow-up period. This is important to guide targeted interrogation of surveillance studies and follow-up schedules, even for discontinued devices, as well as comparisons between various series and estimation of EVAR failure rates. Surveillance after EVAR is critical for long term success of the repair, understanding of the differential modes of failure of every graft available is important in the longitudinal evaluation of these endografts. Equally important is the understanding of the modes of failure of legacy endografts that are no longer on the market but still being followed, in order to be able to tailor a surveillance regiemn and the evntual repair if needed.

The efficacy of the use of atropine in children with reflex anoxic syncope during pallid breath-holding spells: can cardiac pacemaker implantation be avoided?

Reflex anoxic syncope is the result of an overreaction of the vagal system, resulting in hypotension and bradycardia or brief cardiac arrest. Because of the benign character and the absence of complications in short or long term, treatment is only necessary in case of frequent or severe clinical presentation. Treatment options are anticholinergic drugs or cardiac pacemaker placement. We investigated atropine treatment and aimed to examine if pacemaker placement can be avoided. We retrospectively reviewed patients treated with atropine for severe reflex anoxic syncope in our centre from January 2017 until May 2023, and compared our results to those in the literature. The study population consisted of 10 children, 70% female, with an age ranging from 5 months to 3 years (mean 14.5 months) when atropine treatment was started (dose 17-50 microg/kg/day). All patient's parents reported adequate symptom management during atropine treatment, with complete resolution in 10%. Minor side effects were reported in 60% (dry mucosa in 40%, obstipation in 20%, and nausea or blurry vision in 10%). We consider atropine a safe and effective treatment to manage reflex anoxic syncope with similar success rate to pacemaker implantation. However, pacemaker implantation entails substantial risk for complications (up to 25%) such as infection or technical problems and morbidity such as scar formation. This might be considered redundant for a benign and temporary condition, certainly given the possibility of other efficient treatment options. Consequently, we recommend atropine treatment over implantation of a cardiac pacemaker in children with severe reflex anoxic syncope.

Clinical characteristics of systemic lupus erythematosus patients with adrenal hemorrhage.

Adrenal hemorrhage (AH) is a rare condition and severe cases can lead to acute adrenal insufficiency with potentially life-threatening consequences. AH can be caused by a variety of etiologic factors, including systemic lupus erythematosus and antiphospholipid syndrome (APS). The early identification and treatment of these patients improves their prognosis. The aims of this study were to analyze and summarize the clinical characteristics of systemic lupus erythematosus patients with AH. The clinical characteristics of 6 systemic lupus erythematosus patients complicated with AH admitted to Peking Union Medical College Hospital and Beijing Shijitan Hospital from May 2004 to April 2022 were retrospectively analyzed. The diagnosis of AH was based on computed tomography (CT) findings. Two patients had bilateral lesions, and the other 4 patients had unilateral lesions. The symptoms of adrenal insufficiency were observed in 2 patients. The frequent presenting symptoms were abdominal pain, lower abdominal distension, vomiting, weakness, fever, arthrodynia, and skin rash. Four patients had APS. Five patients (4 patients with APS and 1 patient without APS) had thromboembolic events. All patients received glucocorticoid and immunosuppressant therapy. Five patients were treated with anticoagulant therapy. Follow-up imaging examinations showed a partial or total regression of the lesions after treatment. In the proper clinical setting, having high clinical suspicion for AH, early diagnosis and timely management is crucial to avoid life-threatening adrenal insufficiency. Key Points • AH is a rare condition and severe cases may lead to death. It can be caused by a variety of etiologic factors, including SLE. • In patients with SLE, especially combined with APS, if they complain of abdominal pain, particularly when common gastrointestinal involvement is difficult to explain, a high index of clinical suspicion is needed for the diagnosis of AH. • Early identification of AH in SLE patients can improve their prognosis.

Correlation of Ultrasound Guided (USG) Fine Needle Aspiration Cytology (FNAC) With Postoperative Histopathology in the Diagnosis of Thyroid Swelling

Background: Thyroid swelling is a common problem in Bangladesh. Early and systematic evaluation of a nodular thyroid swelling is necessary as it is a frequent presentation of thyroid neoplasia. USG Guided Fine needle aspiration cytology (FNAC) is one of the diagnostic tools for early evaluation of thyroid nodules. USG Guided FNAC is considered as a cost-effective, simple, minimally invasive and easily repeatable procedure for preoperative screening in the diagnosis for most of the thyroid lesions. The objective of this study is to determine the sensitivity and specificity of USG Guided fine needle aspiration cytology (FNAC) and compare the efficacy with histopathology in diagnosis of thyroid swellings. Objective: To evaluate the sensitivity and specificity of USG Guided fine needle aspiration cytology (FNAC) and histopathology for the diagnosis of thyroid swelling. Method: This Observational Prospective Study was performed in the department of Otolaryngology & Head Neck Surgery, Dhaka Medical College Hospital. Dhaka over 6 month’s duration from January 2019-June 2019. Total 50 patients with thyroid swelling was selected after taking informed written consent and fulfilling the inclusion criteria. Smears was taken by standard procedure and then was stained with papanicolaou stain and haematoxylin and eosine stain. The smears was classified into categories like benign, indeterminate, malignant and unsatisfactory. Sensitivity, specificity, positive and negative predictive value and accuracy of USG Guided FNAC was calculated and compared to already available data. Appropriate statistical analysis was done to make comparison between final histopathological findings with initial USG Guided FNAC findings. Result: During the study period a total 73 were found eligible for USG Guided FNAC. Most of the patients (52%) were from 20-39 years age group. Among them 67.12% were female and rest (32.87%) were male. In this government hospital 75.34% were from low income group. ......

Factors Associated With Extrapulmonary Tuberculosis in Southeastern Mexico: A Case-Control Study.

Extrapulmonary tuberculosis (EPTB) is a disease that can affect any organ or tissue. Due to its potential to cause more dangerous sequelae and the barriers to its timely diagnosis, greater clinical awareness of this disease is crucial.This study aimedto identify the factors associated with EPTB in the population of Oaxaca, Mexico. This is an unpaired case-control study. The cases were patients with EPTB+ while the controls were patients with pulmonary tuberculosis (PTB+) registered in the Tuberculosis Epidemiological Surveillance System. Sociodemographic, clinical, and microbiological variables were recovered. Bivariate analyses were performed and logistic regression analyses were performed to calculate the odds ratio (OR). A total of 75 EPTB+ cases and 300 PTB+ controls were included. Of the total sample, 57.1% were men and 60.3% indigenous. The most frequent clinical presentations of EPTB+ were nodal (21.3%), miliary (21.3%), and breast (20.0%). According to logistic regression analysis, age <40 years (OR: 2.25(95% CI: 1.13-4.49), female sex (OR: 1.92(95% CI: 1.03-3.56)], urban residence (OR: 2.25 (95% CI: 1.11-4.55)), comorbidity with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) (OR: 3.46 (95% CI: 1.31-9.10)), dyspnea (OR: 2.67 (1.22-5.82)), and adenopathy (OR: 3.38 (95% CI: 1.42-8.06)) were positively associated with EPTB+. These results can serve as a basis for screening EPTB+, thus improving the preventive and diagnostic capacity of local health services, taking as a starting point women under 40 years of age and patients with HIV/AIDS in urban areas, as well as the presence of adenopathy and dyspnea as clinical characteristics of the disease.

Spectrum of respiratory distress and outcome in neonates admitted in National Institute of Child Health, Karachi, Pakistan.

Objective: To find out the spectrum of respiratory distress and outcome in neonates admitted to Neonatal Intensive care unit (NICU) of National Institute of Child Health (NICH), Karachi, Pakistan. Study Design: Cross-sectional study. Setting: NICU of NICH, Karachi, Pakistan. Period: June 2022 to May 2023. Methods: We analyzed 148 neonates of either gender presenting with respiratory distress. At the time of enrollment, demographic and clinical information was noted in all neonates along with detailed maternal history. Relevant laboratory and radiological studies were performed. Final outcomes were recorded in terms of discharged, expired or referred. Results: In a total of 148 neonates, 96 (64.9%) were boys. The mean age was 4.04±5.65 days. Tachypnea, nasal flaring, intercostal recession, subcostal recession, grunting, and cyanosis 148 (100%), 146 (98.6%), 138 (93.2%), 133 (89.9%), 97 (65.5%), and 83 (56.1%) were the most frequent clinical presentations among neonates with respiratory distress. The most frequent causes behind respiratory distress were noted to be respiratory distress syndrome, sepsis, and meconium staining of labour in 31 (20.9%), 25 (16.9%), and 24 (16.2%) neonates. Mortality was observed in 21 (14.2%) neonates. Significant association of neonates presenting with congenital pneumonia (p=0.048), or congenital anomalies (p&lt;0.001) were found to with poor outcomes. Mechanical ventilation was found to have significant relationship with poor outcomes (p&lt;0.001). Conclusion: Tachypnea, Nasal flaring, intercostal and subcostal recessions, grunting, and cyanosis were the primary clinical presentations among the neonates with respiratory distress. Congenital pneumonia, and congenital anomalies emerged as key predictors of poor outcomes.

Pediatric arterial ischemic stroke; Risk factors, clinical presentation and short-term outcome of patients presenting at a tertiary care hospital.

Objective: To determine the risk factors, clinical presentation and short term outcome in children presenting with arterial ischemic stroke at a tertiary care hospital. Study Design: Prospective Observational study. Setting: Medical Units of National Institute of Child Health, Karachi, Pakistan. Period: November 2022 October 2023. Methods: Children of either gender aged between 1 month to 12 years and admitted with the diagnosis of pediatric arterial ischemic stroke during the study duration were analyzed. Detailed medical and neurological examinations were performed. Routine work up and laboratory investigations were done. Modified Rankin Scale (MRS) scores at the time of enrollment, discharge and after 3 month follow-up were recorded. Results: In a total of 35 children, 21 (60.0%) were boys the mean age was 6.89±3.63 years. The most frequent presenting clinical features were focal neurological deficits noted in 29 (82.9%) children whereas fever, and fits were reported in 26 (74.3%), and 22 (62.9%) children, respectively. Right hemiplegia was observed in 15 (42.9%) children. Stroke was secondary to moyamoya 8 (22.9%), infections in 6 (17.1%) children. Mortality was reported among 3 (8.6%) children while 3 others lost during the follow ups. Comparison of mean mRS scores at the time of admission, discharge and after 3 months showed significant reduction (p&lt;0.001). Conclusion: Focal neurological deficits were the predominant clinical features, with a significant incidence of associated fever and seizures. Moyamoya and infections emerged as primary causative factors. Despite a mortality rate of 8.6%, there was an overall improvement in outcomes, as evidenced by a significant reduction in Modified Rankin Scale scores at admission, discharge, and after three months.

Spectrum of skeletal dysplasia in short stature children in tertiary care hospital.

Objective: To evaluate the spectrum of skeletal dysplasia in short stature children at National Institute of Child Health (NICH), Pakistan. Study Design: Case Series study. Setting: Department of Endocrinology, NICH, Karachi, Pakistan. Period: January 2022 to November 2023. Methods: Short statured (height &lt; -2.0 SD) children of either gender aged between 1 month up to 16 years and diagnosed with skeletal dysplasia were analyzed. At the time enrollment, gender, age, anthropometric measures, antenatal history, and family history were noted. Complete skeletal survey was performed. Results: In a total of 131 short statured children with skeletal dysplasia, 77 (58.8%) were male. The mean and median age were 5.54±4.33 and 5.0 (1.5-8) years. Consanguinity was reported in 85 (64.9%) cases whereas siblings were affected among 9 (6.9%) cases. The most frequent presenting complaints and clinical features were joint pain, facial dysmorphism, movement limitations, and infections, reported by 67 (51.1%), 67 (51.1%), 65 (49.6%), and 63 (48.1%) children respectively. Mucopolysaccharidosis (29.0%), achondrodysplasia (13.7%), and osteogenesis imperfecta (10.7%) were the most common types of skeletal dysplasia. Conclusion: Mucopolysaccharidosis, achondrodysplasia, and osteogenesis imperfecta were the most frequent types of skeletal dysplasia. The most frequent presenting complaints and clinical features were joint pain, facial dysmorphism, movement limitations, and infections. The high prevalence of consanguinity and familial history emphasizes a probable genetic basis for skeletal dysplasia.

COVID-19 in pregnancy: A cross-sectional study on clinical features, disease severity, and health outcome.

Assessing the impact of coronavirus disease 2019 (COVID-19) reveals unique challenges for pregnant women, who experience distinct clinical manifestations and health outcomes compared to their non-pregnant counterparts. We aimed to evaluate the clinical features, disease severity, and health outcomes of COVID-19 in pregnant women and compare them to those of non pregnant women. In this population-based study, we included all women diagnosed with COVID-19 across the province of Tehran during the first two years of the epidemic. Descriptive statistics, the chi-squared test, and the logistic regression model were applied. Overall, 79,338 non-pregnant women and 3249 pregnant women diagnosed with COVID-19 were included. Pregnant women were most commonly in the age group of 25 - 34 years (54%, n = 1758), while the age group of 34-44 had the highest representation among non-pregnant women (56%, n = 44,492). After accounting for age and comorbidities, pregnancy was associated with an increased risk of requiring intensive care (odds ratio [OR] 1.38, confidence interval [CI] 1.223 - 1.564). However, the probability of dying due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection was lower in pregnant women compared to non-pregnant women (OR 0.55, CI 0.394-0.793). Cough (41%) and fever (30%) were the most frequent clinical presentations in pregnant women, whereas cough (57%) and muscle ache (38%) were the most common symptoms in non-pregnant women. Furthermore, diarrhea (P < 0.001) and skin lesions (P < 0.001) were reported more frequently by pregnant patients than non-pregnant patients. A significant prevalence of diabetes (P < 0.001), hypertension (P < 0.001), cancers (P < 0.001), and chronic hematological diseases (P < 0.001) was observed in pregnant patients. In conclusion, COVID-19-infected pregnant women exhibit different clinical manifestations and a more severe clinical course but have better health outcomes compared to their non-pregnant counterparts.

Frequent Presentation of Counterfeit Gunshot Wounds in a Tertiary Care Hospital in North India: A Case Series

Introduction: Self-inflicted wounds, or wounds produced by another acting in accord with him, are wounds inflicted by a person on his own body. The purpose of fabricating injuries is to file a false claim of assault against an opponent, to alter the appearance of a minor injury in order to attract more attention and a harsher punishment for the alleged accused. Someone may also pretend these as defence wounds, so as to conceal homicide.Result: In a short period of 1 year, i.e. from August 2020 to July 2021 in a single unit of Department of General Surgery, 8 cases of Counterfeit Gunshot Wounds are there which depicts that the foul play is at large scale and these gunshot wounds are not present over the vital organs of the body.Conclusion: The combined efforts of all investigating authorities and doctors can help to avoid injury fabrication, which will not only decrease wasteful litigation in the courts, but will also help to avoid harassment of innocent individuals.

The treatment of behavioural and psychological symptoms in dementia: pragmatic recommendations.

Behavioural and psychological symptoms of dementia (BPSD) are a clinical challenge for the lack of a sound taxonomy, frequent presentation with comorbid BPSD, lack of specific pharmacologic interventions, poor base of methodologically sound evidence with randomized clinical trials, contamination from the treatment of behavioural disturbances of young and adult psychiatric conditions, and small efficacy window of psychotropic drugs. We present here a treatment workflow based on a concept-driven literature review based on the notions that (i) the aetiology of BPSD can be mainly neurobiological (so-called 'primary' symptoms) or mainly environmental and functional ('secondary' symptoms) and that this drives treatment; (ii) the clinical efficacy of psychotropic drugs is driven by their specific profile of receptor affinity; (iii) drug treatment should follow the rules of 'start low-go slow, prescribe and revise'. This article argues in support of the distinction between primary and secondary BPSD, as well as their characteristics, which until now have been just sketchily described in the literature. It also offers comprehensive and pragmatic clinician-oriented recommendations for the treatment of BPSD.

Use of the Canadian CT head rule for patients on anticoagulant/anti-platelet therapy presenting with mild traumatic brain injury: prospective observational study.

Background and importanceMild traumatic brain injury (mTBI) is a frequent presentation in Emergency Department (ED). There are standardised guidelines, the Canadian CT Head Rule (CCHR), for CT scan in mTBI that rule out patients on either anticoagulant or anti-platelet therapy. All patients with these therapies undergo a CT scan irrespectively of other consideration.ObjectiveTo determine whether standard guidelines could be applied to patients on anticoagulants or anti-platelet drugs.Design, settings, and participants1,015 patients with mTBI and Glasgow Coma Score (GCS) of 15 were prospectively recruited, 509 either on anticoagulant or anti-platelet therapy and 506 on neither. All patients on neither therapy underwent CT scan following guidelines. All patients with mTBI on either therapy underwent CT scan irrespective of the guidelines.Outcome measure and analysisPrimary endpoint was the incidence of post-traumatic intracranial bleeding in patients either on anticoagulants or anti-platelet drugs and in patients who were not on these therapies. Bayesian statistical analysis with calculation of Confidence Intervals (CI) was then performed.Main resultsSixty scans were positive for bleeding: 59 patients fulfilled the criteria and 1 did not. Amongst patients with haemorrhage, 24 were on either therapy and only one did not meet the guidelines but in this patient the CT scan was performed before 2 h from the mTBI. Patients on either therapy did not have higher bleeding rates than patients on neither. There were higher bleeding rates in patients on anti-platelet therapy who met the guidelines vs. patients who did not. These rates overlapped with patients on neither therapy, meeting CCHR.ConclusionThe CCHR might be used for mTBI patients on either therapy. Anticoagulants and anti-platelet drugs should not be considered a risk factor for patients with mTBI and a GCS of 15. Multicentric studies are needed to confirm this result.

Comorbidity of depression and anxiety with obstructive sleep apnea in a sample of Egyptian patients

Abstract Background Over the past 2 decades, sleep researchers have made significant advances in understanding sleep disorders including obstructive sleep apnea (OSA). OSA commonly coexists with psychiatric disorders encompassing mental health issues like depression, anxiety, bipolar, schizophrenia an others. This study aims to assess the comorbidity of OSA with depression and anxiety disorders among Egyptian patients. During the study, we conducted a cross-sectional study involving 92 adults diagnosed with OSA. Those patients were interviewed using a checklist to gather information regarding their chief complaints and other associated symptoms. The psychological status was assessed utilizing the Structured Clinical Interview for DSM-IV Axis I Disorders (SCID I), Beck Anxiety Inventory (BAI), and Beck Depression Inventory (BDI) scoring. The severity of the breathing disorder was classified as mild, moderate, or severe based on the respiratory disturbance index (RDI), which was confirmed through overnight polysomnography. Daytime sleepiness was assessed by the Epworth Sleepiness Scale (ESS). Results The sample of patients as a whole shows that witnessed apneas represent the highest percentage (70.7%), followed by fatigue (69.6%), then non-refreshing sleep (67.4%), and snores (63.0%). The least frequent presenting symptom is frequent naps. The majority of OSA patients have a comorbid psychiatric disorder (65.0%). Among these, depressive disorders are the most prevalent (31.5%), followed by anxiety disorders (23.9%) and psychoses (6.5%). It is worth noting that 38% of OSA patients do not have any psychiatric disorders. In terms of anxiety disorders, the most common subtype observed is OCD (8.7%), followed by GAD (6.5%), panic (5%), and PTSD (3.3%). As regards depressive disorders, the predominant subtype present is depressive disorder not otherwise specified (11.9%), followed by dysthymic disorder and major depressive disorder (8.7% for each), and then bipolar disorder (depression) at 2.2%. Conclusions We conclude the following: 1. OSA patients are likely to present with comorbid depression and/or anxiety disorder(s). 2. Fatigue was found to be the most prevalent presenting symptom in OSA patients with comorbid anxiety compared to those without anxiety.

Long-Term Surgical Outcome of Class A and B Tympanomastoid Paragangliomas.

To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients. Retrospective study. The medical records of patients with surgically managed class A and B tympanomastoid PGLs between 1983 and 2023 were thoroughly evaluated. Our center has treated a total of 213 cases of tympanomastoid PGL surgically. The mean age of patients was 54, and the male-to-female ratio was 1:6. The most common symptoms at presentation were hearing loss (80%), pulsatile tinnitus (77%), and vertigo (15%). According to the modified Fisch classification, 45% of the cases were classified as class A (A1 and A2), while 55% were classified as class B (B1, B2, and B3). All class A and most class B1 and B2 tumors were removed either with transcanal or retroauricular-transcanal approaches. However, more advanced class B3 lesions were removed with subtotal petrosectomy (SP) along with middle ear obliteration. Facial nerve outcome was excellent in all class A and B cases, while chances of postoperative paresis slightly increased with the size and extent of the tumor (p < 0.05). The hearing outcome is excellent for class A1, A2, B1, and B2 tumors, whereas more advanced class B3 cases have a loss of air conduction (AC) and increased bone conduction (BC) threshold (p < 0.05). Complete surgical removal was achieved in 97% of our cases. The most common late complication was permanent TM perforation (7%), and the recurrence rate was 3%. Tympanomastoid PGL represents the most common neoplasm of the middle ear space. The most frequent presenting symptoms include pulsatile tinnitus and hearing loss, whereas the presence of retrotympanic mass was evident in all cases at the time of initial otoscopic evaluation. Proper documentation of facial function and audiometric evaluation are crucial elements of preoperative workup. The most preferred preoperative radiologic examination is high-resolution computer tomography (HRCT), whereas magnetic resonance imaging (MRI) with or without gadolinium enhancement is reserved for cases with a dilemma of carotid artery or jugular bulb involvement. The main goal of tympanomastoid PGL treatment is complete disease removal with preservation of hearing and facial functions. Surgical treatment remains the preferred treatment modality with the benefits of complete disease removal, lower rate of recurrence and complication, and acceptable postoperative hearing level. Here, we present our 40 years of experience, which, to the very best of our knowledge, is the largest series of tympanomastoid PGL in the English literature.

Paediatric onset lymphomatoid papulosis: results of a multicentre retrospective cohort study, on behalf of the EORTC Cutaneous Lymphoma Tumours Group (CLTG).

Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population is scarce. To characterize epidemiological, clinical, histopathological, and prognostic features of paediatric LyP. This was a retrospective, multicentre international cohort study including 87 cases of children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years old at disease onset were included. Diagnosis was made in each centre based on clinical-pathological correlation. Eighty-seven patients from 12 centres were included. The mean age at onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2:1. The mean time between onset of first cutaneous lesions and diagnosis was 1.3 years (range 0-14 years). Initial misdiagnosis concerned 26.4% of patients. Initially, LyP was most often misdiagnosed as Pityriasis lichenoides et varioliformis acuta (PLEVA), insect bites, or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned for 20.7% of patients. The main histological subtype was type A in 55.1% of the cases. If analysed, monoclonal TCR rearrangement was found in 76.5% of the skin biopsies. The overall survival rate was 100% with follow up at 5 years available for 33 patients and at 15 years for 8 patients. A development of associated haematological malignancy (HM) occurred in 9.6% of the cases (7/73), including four mycosis fungoides (MF) cases, one primary cutaneous anaplastic large cell lymphoma (pc-ALCL), one systemic ALCL and one case of acute myeloid leukaemia. If we compare incidence rates of cancer with the world 0-19 years old population from 2001-2010, we estimate a significantly higher risk of associated malignancy in general, occurring before the age of 19 years old with incidence rate ratio of 87.49 (CI 86.01-88.99). We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall the prognosis of the disease is good, with excellent survival rates for all patients. Due to increased risk of associated HM, a long-term follow-up should be recommended for LyP patients.

Ultrasound features using MUSA terms and definitions in uterine sarcoma and leiomyoma: cohort study.

Timely and accurate preoperative diagnosis of uterine sarcoma will increase patient survival. The primary aim of this study was to describe the ultrasound features of uterine sarcoma compared with those of uterine leiomyoma based on the terms and definitions of the Morphological Uterus Sonographic Assessment (MUSA) group. A secondary aim was to assess the interobserver agreement for reporting on ultrasound features according to MUSA terminology. This was a retrospective cohort study of patients with uterine sarcoma or uterine leiomyoma treated in a single tertiary center during the periods 1997-2019 and 2016-2019, respectively. Demographic characteristics, presenting symptoms and surgical outcomes were extracted from patients' files. Ultrasound images were re-evaluated independently by two sonologists using MUSA terms and definitions. Descriptive statistics were calculated and interobserver agreement was assessed using Cohen's κ (with squared weights) or intraclass correlation coefficient, as appropriate. A total of 107 patients were included, of whom 16 had a uterine sarcoma and 91 had a uterine leiomyoma. Abnormal uterine bleeding was the most frequent presenting symptom (69/107 (64%)). Compared with leiomyoma cases, patients with uterine sarcoma were older (median age, 65 (interquartile range (IQR), 60-70) years vs 48 (IQR, 43-52) years) and more likely to be postmenopausal (13/16 (81%) vs 15/91 (16%)). In the uterine sarcoma cohort, leiomyosarcoma was the most frequent histological type (6/16 (38%)), followed by adenosarcoma (4/16 (25%)). On ultrasound evaluation, according to Observers 1 and 2, the tumor border was irregular in most sarcomas (11/16 (69%) and 13/16 (81%) cases, respectively), but regular in most leiomyomas (65/91 (71%) and 82/91 (90%) cases, respectively). Lesion echogenicity was classified as non-uniform in 68/91 (75%) and 51/91 (56%) leiomyomas by Observers 1 and 2, respectively, and 15/16 (94%) uterine sarcomas by both observers. More than 60% of the uterine sarcomas showed acoustic shadows (11/16 (69%) and 10/16 (63%) cases by Observers 1 and 2, respectively), whereas calcifications were reported in a small minority (0/16 (0%) and 2/16 (13%) cases by Observers 1 and 2, respectively). In uterine sarcomas, intralesional vascularity was reported as moderate to abundant in 13/16 (81%) cases by Observer 1 and 15/16 (94%) cases by Observer 2, while circumferential vascularity was scored as moderate to abundant in 6/16 (38%) by both observers. Interobserver agreement for the presence of cystic areas, calcifications, acoustic shadow, central necrosis, color score (overall, intralesional and circumferential) and maximum diameter of the lesion was moderate. The agreement for shape of lesion, tumor border and echogenicity was fair. A postmenopausal patient presenting with abnormal uterine bleeding and a new or growing mesenchymal mass with irregular tumor borders, moderate-to-abundant intralesional vascularity, cystic areas and an absence of calcifications on ultrasonography is at a higher risk of having a uterine sarcoma. Interobserver agreement for most MUSA terms and definitions is moderate. Future studies should validate the abovementioned clinical and ultrasound findings on uterine mesenchymal tumors in a prospective multicenter fashion. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

Clinical profile, treatment modalities and outcomes among patients with upper aero-digestive tract emergencies at Bugando Medical Centre, Mwanza, Tanzania

Background: Upper aero-digestive tract emergencies (UADTEs) are common in our setting and can be life-threatening and challenging to otolaryngologists. However, there is a paucity of clinical studies on these emergencies. This study aimed to determine the clinical profile, treatment modalities and outcomes of upper aero-digestive tract emergencies at Bugando Medical Centre (BMC), Mwanza, Tanzania. Methods: Between January and May 2019, a cross-sectional study involving patients presenting with a clinical diagnosis of UADTEs was conducted at BMC. Results: Of 487 ENT emergencies seen during the study period, 128 (26.3%) patients had UADTEs. Males outnumbered females by a ratio of 1.5: 1. Their median age at presentation was 5 [range, 2-40] years. The major causes of UADTEs were foreign body ingestion, head and neck tumours, foreign body aspiration and cut-throat injuries that were seen in 56(43.8%), 33(25.8%), 25(19.5%) and (3.9%), respectively. The most frequent presentations were dysphagia 81(63.3%), difficulty in breathing 61(47.7%) and odynophagia 56(43.8). Age ≤ 40 years (p=0.02), prolonged duration to treatment (p=0.04) and low blood oxygen saturation (SPO2) (p=0.04) were significantly associated with poor outcomes following esophagoscopy. In addition, delayed duration to treatment (p=0.01) and foreign body ingestion (p=0.001) were significantly associated with prolonged hospital stay. Conclusion: UADTEs are common at BMC and constitute a significant cause of otorhinolaryngological admission with foreign bodies in the aerodigestive tract as the most typical cause of these emergencies. Most of these injuries can be prevented through public enlightenment campaigns. Early detection and management of UADTEs is essential to reduce morbidity and mortality associated with these emergencies.

A 6-year Retrospective Study on the Clinical Profile and Outcomes of Postrenal Transplant Patients in the Emergency Department

Abstract Background: Kidney transplantation is widely recognized as the primary treatment for those with end-stage renal disease. Our goal was to provide an overview of the clinical characteristics of postrenal transplant patients (commercial vs. noncommercial) seeking care in the emergency department (ED). Methodology: The study was conducted at a leading Omani ED from 2015 to 2021; this study included all postrenal transplant patients. Various variables were categorized, coded, and analyzed. Results: A total of 55 postrenal transplant patients were included in our study. Of these, 60.0% were male (n = 33). Among the participants, 14.5% (n = 8) had undergone noncommercial renal transplants, whereas the remaining 85.5% (n = 47) had undergone commercial renal transplants. Among noncommercial postrenal transplant patients, the most common reasons for ED visits were fever (5), gastrointestinal symptoms (4), and cardiac symptoms (3). They also presented with pain at the surgical site (2) and urological symptoms such as dysuria and hematuria (2). In contrast, the most frequent clinical presentations among commercial postrenal transplant patients were cardiac symptoms (13) and pain at the surgical site (13), followed by fever (12). Noncommercial renal transplant patients had a high discharge rate of 62.5% (5) and a low admission rate of 37.5% (3). On the other hand, commercial renal transplant patients necessitated a high admission rate of 59.6% (28) and a low discharge rate of 40.4% (19). Conclusion: The majority of patients had received commercial kidney transplants, and the most common complaints upon their presentation to the ED were cardiac symptoms and pain at the surgical site. Patients undergoing commercial transplants had higher rates of admission.

A study on risk factors, clinical profile, spectrum of comorbidities in children with cerebral palsy and efficacy of early intervention by rehabilitation units on neurodevelopmental outcome in these children

Background: Cerebral palsy (CP) is one of the leading causes of childhood disability worldwide with the greatest burden found in developing countries. This study is undertaken as there is limited data available regarding CP children in the Andhra Pradesh region. This study was done to evaluate the clinical spectrum, risk factors, and comorbidities associated with CP that will help in better understanding of etiology and for early intervention by District early intervention centers. Methods: This is a hospital-based prospective observational study conducted over 18 months among children from 6 months to 5 years of age visiting a tertiary care hospital. Results: Among 65 enrolled children majority of the children (74%) were between 2 to 5yrs with a male-to-female ratio of 4:1. Consanguinity was observed in 33.8% and the majority were from rural areas (71%). The most frequent presenting complaint was delayed milestones (33.4%). 67.6% of subjects were born at term with the most common risk factor being birth asphyxia (27.6%). The most common type was spastic CP (87%) in whom the Developmental quotient (DQ) was severely affected (56.7%) and 32.3% of children belong to GMFCS level III. Comorbidities like feeding difficulties and GERD (47.6%), visual problems (24.8%), and hearing problems (30%) were commonly associated. At the time of enrollment, only 21.5% of children had normal nutritional status which improved to 30.7% after 12 months of follow-up. Conclusions: Efforts should be made to increase awareness among parents regarding the availability of encouraging utilization of the services to relieve the burden of disorder.