Adrenal venous sampling in the diagnostic workup of primary aldosteronism

Primary aldosteronism (PA) is the primary cause of secondary hypertension. The prevalence of PA has probably been underestimated in the past and recent studies suggest that PA could be present in up to 10% of patients suffering from hypertension. Aldosterone excess in PA can be caused by unilateral adrenal disease, usually adrenal adenoma, or bilateral adrenal hyperplasia. Differentiation between unilateral and bilateral disease is clinically important as the former can effectively be treated by removal of the affected adrenal. CT or MRI cannot reliably distinguish unilateral from bilateral disease. Therefore, adrenal vein sampling (AVS) is an important step of the diagnostic work-up in patients with PA. Current guidelines recommend PA in virtually all patients with biochemically diagnosed PA who would undergo adrenal surgery if unilateral PA was diagnosed. In this narrative review, we give an overview of the current technique used for AVS with a focus on the experience with this technique at the University Hospital Basel, Switzerland.

Comprehensive Study of Relative Afferent Pupillary Defect in Kanyakumari Medical College, Tamil Nadu

BACKGROUND Relative afferent pupillary defect (RAPD) or Marcus Gunn pupil is a highly sensitive and significant objective clinical finding in eye examination. It can be detected by swinging flashlight test. Its presence indicates unilateral afferent sensory abnormality or an asymmetric bilateral disease. However, it can be determined even in an unconscious patient, requires no complicated gadgets, relies on clinical observation but can be a manifestation of a myriad of disorders affecting the eye or the brain. The goal of our study was to identify the aetiology of relative afferent pupillary defect and ascertain whether the grade of relative afferent pupillary defect correlated with the visual prognosis. METHODS This was a prospective observational hospital-based study. 25 consecutive patients who presented with a clinical diagnosis of relative afferent pupillary defect to the Ophthalmology Department of Kanyakumari Government Medical College in Tamil Nadu from February 2019 to January 2021 were included in the study. The patients were evaluated based on visual acuity, refraction, colour vision, slit lamp, fundus examination, intra ocular pressure (IOP) measurements & fields. Statistical analysis was done by trial version of statistical package for social sciences (SSPS) software. RESULTS Of the 25 cases analysed, 60 % (15) were male, 36 % (9) were females and 4 % (1) children. An analysis of the aetiology revealed that the optic nerve pathology was the predominant aetiology accounting for 60 % (15) of cases, followed by glaucoma 24 % (6) and retinal pathology in 16 % (4). Glaucoma though a bilateral disease has an asymmetrical presentation which led to RAPD. Grade of RAPD correlated well with the visual prognosis. CONCLUSIONS RAPD is a good and valuable clinical tool for any clinician, it is also a useful guide for assessing the management and response to treatment. KEYWORDS Relative Afferent Pupillary Defect, Optic Neuropathy, Retinal Detachment, Glaucomatous Optic Atrophy

PREVALENCE AND CLINICAL FEATURES OF BILATERAL OSTEOCHONDRITIS DISSECANS (OCD) OF THE KNEE IN PEDIATRIC AND ADOLESCENT PATIENTS PRESENTING WITH UNILATERAL SYMPTOMS

Background: Osteochondritis dissecans(OCD) is an idiopathic condition primarily involving the subchondral bone with secondary articular cartilage changes, commonly occurring in the knee. The true prevalence of bilateral OCD in patients presenting with unilateral OCD symptoms is unknown. Hypothesis/Purpose: The goals of this study are to determine the prevalence and characteristics of bilateral OCD in patients with unilateral symptoms and compare to those with unilateral disease. Methods: An electronic medical record database was queried from 2003-2016 to identify and retrospectively review patients 18 years or younger presenting to a single pediatric institution with a diagnosis of OCD of the knee and strictly unilateral knee pain. Contralateral knee imaging of the asymptomatic knee within one year of initial presentation was required. Lesion characteristics were evaluated on both x-ray and magnetic resonance imaging(MRI) assessing size, location, and Hefti staging. Treatment(both surgical and non-operative) and outcomes were recorded. Patients with unilateral OCD were compared with those with bilateral disease using appropriate statistical analyses. Results: Eighty consecutive patients, 63 males(79%) and 17 females(21%), average age of 13.1 years old (range:8-18), were included. 71% of symptomatic lesions were located on the medial femoral condyle and 14% on the lateral femoral condyle with 20 lesions(25%) deemed stable on MRI evaluation. A positive correlation was found between increasing lesion size and severity of MRI/Hefti grade(Figure-1). Twelve patients(15%) were found to have bilateral OCD on contralateral imaging, with five of the contralateral lesions(42%) considered stable on MRI. There was no significant difference in skeletal maturity between patients with bilateral vs. unilateral disease. Fifty-two patients(77%) with unilateral disease underwent surgical intervention, while 9(75%) of those with bilateral disease underwent a surgical procedure on either knee. In patients discovered to have an asymptomatic contralateral lesion, 67% ultimately underwent surgical intervention on the contralateral knee. Comparing patients with unilateral and bilateral disease, no statistical differences were found in terms of patient demographics or lesion characteristics. Conclusion: In patients presenting with unilateral OCD symptoms, there was a 15% prevalence of bilateral disease. There was no difference in age, sex, physeal status or lesion characteristics between patients with unilateral vs. bilateral OCD lesions, and we found no difference in rates of surgical intervention. A consistent relationship between lesion size and Hefti classification was appreciated. Given the prevalence of asymptomatic contralateral lesions and required intervention, our study supports the recommendation for bilateral radiologic knee evaluation for pediatric and adolescent patients presenting with unilateral knee OCD. [Figure: see text]

Radiological Configuration of the Vestibular Aqueduct Predicts Bilateral Progression in Meniere's Disease

Objective: Meniere's disease (MD) progresses from unilateral to bilateral disease in up to 50% of patients, often chronically and severely impairing balance and hearing functions. According to previous studies, 91% of bilateral MD patients demonstrate bilateral hypoplasia of the endolymphatic sac (ES) upon histological and radiological examination of their inner ears. Here, we seek to validate a radiological marker for ES hypoplasia that predicts the risk for future progression to bilateral MD in individual patients.Methods: Patients with unilateral MD and radiological evidence for ES hypoplasia in either the clinically affected inner ear (cohort MDuni-hpuni) or both inner ears (cohort MDuni-hpbi) were included. Given our hypothesis that ES hypoplasia critically predisposes the inner ear to MD, we expected progression to bilateral MD only in the MDuni-hpbi cohort. To investigate eventual progression to bilateral MD, clinical, audiometric, and imaging data were retrospectively collected over follow-up periods of up to 31 years.Results: A total of 44 patients were included in the MD-hpuni (n = 15) and MDuni-hpbi (n = 29) cohorts. In line with our radiology-based predictions, none (0/15) of the MD-hpuni patients exhibited progression to bilateral MD, whereas 20/29 (69%) MD-hpbi patients have already progressed to bilateral MD. Using the Kaplan–Meier estimator, bilateral disease progression would be observed in 100% of MD-hpbi patients 31 years after the initial diagnosis with an estimated median time to bilateral progression of 12 years. The nine MD-hpbi patients who, so far, remained with unilateral disease demonstrated a median time since initial (unilateral) MD diagnosis of only 6 years and are thus still expected to progress to bilateral disease.Conclusion: Progression to bilateral MD adheres to predictions based on the radiological presence or absence of ES hypoplasia. This prognostic tool, if validated by prospective long-term studies, will provide clinically relevant information about a patient's future disease burden and will help to select more personalized treatment regimens.

Prognostic Significance of Computed Tomography Findings in Pulmonary Vein Stenosis

(1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five locations: right upper, middle, and lower; and left upper and lower. Severity of vein stenosis (0–4 = no disease–atresia) and lung segments (0–3 = unaffected–severe) were scored. A PVS severity score (sum of all veins + 2 if bilateral disease; maximum = 22) and a total lung severity score (sum of all lung segments; maximum = 15) were reported; (3) Of 43 CT examinations (median age 21 months), 63% had bilateral disease. There was 30% mortality by 4 years after CT. Individual-vein PVS severity was associated with its corresponding lung segment severity (p < 0.001). By univariate analysis, PVS severity score >11, lung cysts, and total lung severity score >6 had higher hazard of death; and perihilar induration had lower hazard of death; (4) Multiple CT-derived variables of PVS severity and lung disease have prognostic significance. PVS severity correlates with lung disease severity.

Predictors of Bilateral Disease in Pediatric Differentiated Thyroid Cancer

Context Total thyroidectomy is recommended for children with papillary thyroid carcinoma, partly because of a high prevalence of bilateral disease. Identifying characteristics that predict bilateral disease might identify candidates for more limited surgery. Objectives Investigate associations of preoperative or histopathological characteristics with bilateral disease in children with differentiated thyroid cancer. Design Retrospective cohort study (1998-2020) Setting Two academic hospitals Patients Patients &lt;19 years who underwent total thyroidectomy for differentiated thyroid cancer Interventions Description of clinical, sonographic, and histopathological characteristics Main outcome measure Presence of bilateral disease on histopathology, assessed by univariable analysis and multivariable logistic regression Results 115 subjects were analyzed (90% with papillary carcinoma). Median (range) age at diagnosis was 15.0 (8.1-18.9) years. Bilateral disease was present in 47/115 subjects (41%). Bilateral disease was associated with solid parenchyma, calcifications, irregular margins, and abnormal lymph nodes detected by ultrasound, Bethesda class V/VI cytology, papillary histology, tumor multifocality in the primary lobe, extrathyroidal extension, lymphovascular invasion, and nodal metastases. In multivariable analysis, only multifocality in the primary lobe was independently associated with bilateral disease (OR 7.61, 95% CI 2.44-23.8, p&lt;0.001). Among clinically node-negative subjects with papillary carcinoma who did not have tumor multifocality in the primary lobe, bilateral disease was present in 5/32 (16%). Conclusions In children with differentiated thyroid cancer, tumor multifocality in the primary lobe is associated with bilateral disease and should prompt consideration of completion thyroidectomy after initial lobectomy. Clinically node-negative children with tumors that are unifocal in the primary lobe have a low likelihood of contralateral disease.