A Severe Form of Arterial Tortuosity Syndrome Presenting With Significant Airway Obstruction in an Infant

2019 ◽  
Vol 11 (2) ◽  
pp. 238-240
Author(s):  
Jannika Dodge-Khatami ◽  
Scott A. Simpson ◽  
Ali Dodge-Khatami
Keyword(s):  
Cardiopulmonary Resuscitation ◽  
Airway Obstruction ◽  
Clinical Course ◽  
Severe Form ◽  
Pulmonary Artery Sling ◽  
Arterial Tortuosity ◽  
Arterial Tortuosity Syndrome ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

We describe a severe form of arterial tortuosity syndrome in a newborn, in which the tortuous course of the aorta masqueraded as a pulmonary artery sling on fetal echocardiogram. The newborn presented with respiratory distress after birth. The clinical course was complicated by extrinsic airway obstruction requiring cardiopulmonary resuscitation. Timely diagnostic work-up in patients with arterial tortuosity syndrome is necessary to plan eventual intervention, and hopefully to prevent complications related to the abnormal vasculature.

scholarly journals A Newborn with Arterial Tortuosity Syndrome: The Importance of Timely Diagnostic Work-Up in Patients Presenting with Cutis Laxa

2016 ◽  
Vol 1 (2) ◽  
pp. 249-253 ◽  
Author(s):  
Karin Pichler ◽  
Elisabeth Ralser ◽  
Maria Resch ◽  
Elisabeth Steichen-Gersdorf ◽  
Kathrin Maurer ◽  
...  
Keyword(s):  
Cutis Laxa ◽  
Arterial Tortuosity ◽  
Arterial Tortuosity Syndrome ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

Pulmonary Artery Sling: A New Diagnostic Tool and Clinical Review

PEDIATRICS ◽  
1982 ◽  
Vol 69 (4) ◽  
pp. 472-475
Author(s):  
Karen S. Rheuban ◽  
Nancy Ayres ◽  
J. Gordon Still ◽  
Bennett Alford
Keyword(s):  
Computed Tomography ◽  
Pulmonary Artery ◽  
Diagnostic Tool ◽  
Vascular Ring ◽  
Vascular Anomaly ◽  
Pulmonary Artery Sling ◽  
Cardiovascular Malformation ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

The pulmonary artery sling is a vascular anomaly that produces severe respiratory disease in infancy. The differential diagnosis is lengthy, and the diagnostic work-up frequently includes such invasive maneuvers as bronchography, bronchoscopy, and cardiac catheterization. In this paper the use of a new noninvasive diagnostic tool, computed tomography, to aid in the diagnosis of this form of vascular ring, is discussed and a review of salient features of this cardiovascular malformation is presented.

scholarly journals 605 – The Clinical Course and Diagnostic Work-Up of Idiopathic Acute Pancreatitis, a Post-Hoc Analysis of a Prospective Multicenter Observational Cohort

2019 ◽  
Vol 156 (6) ◽  
pp. S-122
Author(s):  
Nora D. Hallensleben ◽  
Devica S. Umans ◽  
Stefan A. Bouwense ◽  
Robert C. Verdonk ◽  
Marc Besselink ◽  
...  
Keyword(s):  
Acute Pancreatitis ◽  
Clinical Course ◽  
Post Hoc Analysis ◽  
Observational Cohort ◽  
Idiopathic Acute Pancreatitis ◽  
Diagnostic Work ◽  
Post Hoc ◽  
Work Up ◽  
Diagnostic Work Up

Ectopic Thymus: An Unusual Case of Subglottic Mass

2019 ◽  
Vol 128 (12) ◽  
pp. 1182-1188
Author(s):  
Irene Paraboschi ◽  
Federica Fati ◽  
Francesca Rizzo ◽  
Oliviero Sacco ◽  
Nicola Stagnaro ◽  
...  
Keyword(s):  
Airway Obstruction ◽  
English Literature ◽  
Rare Condition ◽  
Thymic Tissue ◽  
Pediatric Airway ◽  
Ectopic Thymus ◽  
Narrative Literature ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

Objectives: The aim of our study is to report a case of a child with subglottic thymus that was suspected during diagnostic work-up for severe airway obstruction, excised surgically and confirmed with final histopathological examination. Moreover, we performed a narrative literature review to outline clinical and diagnostic features of this rare condition and to report suggestions for the management of subglottic masses. Methods: We report the case of a 7-month-old boy who was admitted to our Pediatric Airway Team Unit due to a history of worsening biphasic stridor and recurrent episodes of upper airway obstruction. The successful diagnostic work-up and a narrative literature of analogous cases of subglottic thymus were reported. Results: Ectopic thymus is a very rare condition in which thymic tissue is found outside the normal pathway of its embryonic migration. It usually presents as a cystic or, more rarely, solid mass, showing an indolent course toward spontaneous involution. In some cases, however, it becomes symptomatic exerting compression on surrounding vital structures. Due to its rarity, the initial diagnosis is normally mistaken with inflammatory diseases or malignancies and the definitive diagnosis is only achieved after histological examination of the excised specimen. To our knowledge, only four other cases of subglottic ectopic thymic tissue have been reported in the English literature so far and the diagnosis has never been suspected preoperatively. Conclusion: It is mandatory to consider ectopic thymic tissue in the differential diagnosis in children presenting with airways obstruction in order to prevent unnecessary, extensive, and exploratory surgery.

scholarly journals Stroke masquerading as cardiac arrest: the artery of Percheron

2021 ◽  
Vol 14 (1) ◽  
pp. e238681
Author(s):  
Megan Quetsch ◽  
Sureshkumar Nagiah ◽  
Stephen Hedger
Keyword(s):  
Cardiac Arrest ◽  
Clinical Assessment ◽  
Altered Consciousness ◽  
Cognitive Changes ◽  
Consciousness Level ◽  
Artery Of Percheron ◽  
Conscious Level ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

The artery of Percheron (AOP) is a rare arterial variant of the thalamic blood supply. Due to the densely packed collection of nuclei it supplies, an infarction of the AOP can be devastating. Here we highlight a patient who had an AOP stroke in the community, which was initially managed as cardiac arrest. AOP strokes most often present with vague symptoms such as reduced conscious level, cognitive changes and confusion without obvious focal neurology, and therefore are often missed at the initial clinical assessment. This case highlights the importance of recognising an AOP stroke as a cause of otherwise unexplained altered consciousness level and the use of MRI early in the diagnostic work-up.

scholarly journals Death of unknown cause? Post-mortem diagnosis of fulminant course of an EBV-associated secondary hemophagocytic lymphohistiocytosis

2021 ◽  
Author(s):  
Josia Fauser ◽  
Stefan Köck ◽  
Eberhard Gunsilius ◽  
Andreas Chott ◽  
Andreas Peer ◽  
...  
Keyword(s):  
Immune Response ◽  
Case Report ◽  
Macrophage Activation ◽  
Post Mortem ◽  
Life Threatening ◽  
Appropriate Therapy ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up ◽  
Secondary Hemophagocytic Lymphohistiocytosis

SummaryHLH is a life-threatening disease, which is characterized by a dysregulated immune response with uncontrolled T cell and macrophage activation. The often fulminant course of the disease needs a fast diagnostic work-up to initiate as soon as possible the appropriate therapy. We present herein the case of a 71-year-old patient with rapidly progressive hyperinflammatory syndrome, which post mortem resulted in the diagnosis of EBV-associated HLH. With this case report, we intend to highlight the relevance of the HScore in the diagnosis of HLH, to create a greater awareness for EBV as a trigger of HLH, and to demonstrate the importance of treating EBV-associated HLH as early as possible.

scholarly journals COVID-19 lung injury as a primer for immune checkpoint inhibitors (ICIs)-related pneumonia in a patient affected by squamous head and neck carcinoma treated with PD-L1 blockade: a case report

2021 ◽  
Vol 9 (2) ◽  
pp. e001870
Author(s):  
Angelo Dipasquale ◽  
Pasquale Persico ◽  
Elena Lorenzi ◽  
Daoud Rahal ◽  
Armando Santoro ◽  
...  
Keyword(s):  
Lung Injury ◽  
Head And Neck ◽  
Checkpoint Inhibitors ◽  
Experimental Therapy ◽  
Common Mechanism ◽  
Nasopharyngeal Swab ◽  
Immune Mediated ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

By the beginning of the global pandemic, SARS-CoV-2 infection has dramatically impacted on oncology daily practice. In the current oncological landscape, where immunotherapy has revolutionized the treatment of several malignancies, distinguishing between COVID-19 and immune-mediated pneumonitis can be hard because of shared clinical, radiological and pathological features. Indeed, their common mechanism of aberrant inflammation could lead to a mutual and amplifying interaction.We describe the case of a 65–year-old patient affected by metastatic squamous head and neck cancer and candidate to an experimental therapy including an anti-PD-L1 agent. COVID-19 ground-glass opacities under resolution were an incidental finding during screening procedures and worsened after starting immunotherapy. The diagnostic work-up was consistent with ICIs-related pneumonia and it is conceivable that lung injury by SARS-CoV-2 has acted as an inflammatory primer for the development of the immune-related adverse event.Patients recovered from COVID-19 starting ICIs could be at greater risk of recall immune-mediated pneumonitis. Nasopharyngeal swab and chest CT scan are recommended before starting immunotherapy. The awareness of the phenomenon could allow an easier interpretation of radiological changes under treatment and a faster diagnostic work-up to resume ICIs. In the presence of clinical benefit, for asymptomatic ICIs-related pneumonia a watchful-waiting approach and immunotherapy prosecution are suggested.

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