Fatal acute retropharyngeal hemorrhage in neurofibromatosis type 1

Forensic Science, Medicine, and Pathology
Pierre-Antoine Peyron, Michael S Pollanen

Abstract

We report the sudden death of a woman with neurofibromatosis type 1 (NF1). The decedent developed acute respiratory distress and died rapidly despite an emergent cricothyroidotomy. An autopsy with postmortem CT scan was performed to determine the cause of the fatal respiratory collapse and to determine if death was related to neurofibromatosis. Postmortem examination revealed the classical external hallmarks of neurofibromatosis, including innumerable cutaneous neurofibromas. In addition, there was a massive retropharyngeal hematoma with fatal extrinsic compression of the airway. On macroscopic examination A localized 3 cm diameter tumor nodule was found in the soft tissues of the neck. Histologic examination of the nodule revealed a neurofibroma. In addition, histologic sections of the hematoma and surrounding soft tissues revealed plexiform neurofibroma with infiltration of the walls of small blood vessels and of the right vertebral artery. The fatal retropharyngeal hemorrhage was caused by diffuse infiltration of the blood vessels of the neck by plexiform neurofibroma. We concluded that the underlying cause of death was NF1. This case underscores the protean nature of neurofibroma, particularly when diffuse interstitial hemo...Continue Reading

References

Sep 1, 1974·Angiology·W R Salyer, D C Salyer
Oct 1, 1974·American Journal of Clinical Pathology·J F GreeneJ Burgess
Mar 1, 1972·Vascular Surgery·C V LeierL F Anatasia
Dec 1, 1984·Journal of Clinical Gastroenterology·J M Petersen, D R Ferguson
Sep 1, 1993·Pediatric Pathology·B KoszycaR W Byard
Jan 5, 2001·Clinical Genetics·S J Hamilton, J M Friedman
Jan 5, 2001·Clinical Genetics·V M Riccardi
Nov 5, 2002·Annals of Vascular Surgery·George L HinesManucher Mohtashemi
May 16, 2006·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·Akihiro IshizuTakashi Yoshiki
Jul 25, 2007·Journal of Forensic Sciences·Roger W Byard
Mar 11, 2008·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·Nora HinschKatharina Holzer
Dec 17, 2009·Emergency Medicine Australasia : EMA·M Omar Faruque, Fergus Davidson
Jun 17, 2010·Vascular and Endovascular Surgery·Brian J SantinMichael R Go
Jun 10, 2011·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Vasileios SouftasPanos Prassopoulos
Dec 14, 2011·Acta Neuropathologica·Steven L Carroll
Jan 28, 2014·International Journal of Surgery Case Reports·Bhupinder HoonjanAbdusalam Abu-Own
Sep 1, 2007·Forensic Science, Medicine, and Pathology·M KettnerS A Padosch
Apr 12, 2016·Auris, Nasus, Larynx·Stamatia DovaKonstantinos Markou

❮ Previous
Next ❯

Citations

Apr 21, 2021·Stomatologii︠a︡·F E SevrukovE M Kupriyanova
Oct 22, 2020·The American Journal of Emergency Medicine·Hiromi IhoriyaHiroshi Naitou

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery
M M GnuechtelK T Pitman
The Journal of the American Osteopathic Association
H Stein, S Herschberg
Clinical Neurology and Neurosurgery
S BhaskarAmit K Singh
© 2022 Meta ULC. All rights reserved