Placenta
Umbilical cord
Necrotizing funisitis

Author: Paul J. Kowalski, M.D. (see Authors page)

Revised: 13 February 2016, last major update February 2016

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Necrotizing funisitis [title]
Cite this page: Necrotizing funisitis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/placentanecrotizingfun.html. Accessed December 8th, 2016.
Definition / General
  • Definition: in long standing inflammatory processes, a fetal inflammatory response resulting in tissue necrosis and the accumulation of cellular debris that extends into the substance of the umbilical cord
Terminology
  • The use of "funisitis" as a generic descriptor of inflammation, without further comment as to its location (umbilical artery or vein, Wharton’s jelly, etc.), is generally discouraged
  • Inflammation may be localized to umbilical vein (phlebitis) or umbilical arteries (arteritis)
  • Inflammation at the periphery or surface of the umbilical cord may also be a primary pattern (peripheral funisitis), distinct from necrotizing funisitis
Epidemiology
  • Not well documented, but seen as a sequela in a minority of severe cases of chorioamnionitis or umbilical vasculitis
Pathophysiology
  • Microorganisms gain access to the placenta or umbilical cord either by ascending from the endocervical canal, by the maternal bloodstream or by direct inoculation, such as from a diagnostic procedure
  • In response, cytokines IL8, interferon gamma, complement components or leukotriene B4 are released by endothelium, mast cells and macrophages, creating a chemoattractant chemical gradient for neutrophils
  • The chemical gradient may be augmented by certain peptides released by microorganisms
  • Activated neutrophils marginate against the vascular endothelium and transmigrate through the vascular wall into the connective tissue of Wharton's jelly
  • Eventually, immune complexes from microbiologic antigens precipitate and elicit a maternal antibody response through the transplacental passage of antibodies
Etiology
  • Due to various longer term infections involving a wide range of bacteria, viruses, fungi or parasites
  • Classically described with congenital syphilis infection
Clinical Features
  • Typically seen in association with chorioamnionitis (maternal inflammatory response including fever, tachycardia, leukocytosis or foul smelling amniotic fluid)
Radiology Description
  • Inflammation induced calcifications within Wharton’s jelly can be distributed as a spiraled 'barber's pole' on maternal radiographs
Prognostic Factors
  • More severe inflammatory response is seen when funisitis is present in preterm infants, possibly accounting for some differences in gestational age related morbidity (Hum Pathol 2001;32:623)
  • Histopathologic severity may help predict certain neonatal outcomes (Placenta 2015;36:1490)
Case Reports
Treatment
  • Antibiotic therapy, particularly if symptoms of chorioamnionitis are present
Gross Description
  • Cut surface of an involved umbilical cord may show concentric circles or chalky calcified arcs surrounding one or more umbilical vessels, distributed in successive 'waves'
  • External surface of an involved umbilical cord is generally unremarkable, unless accompanying peripheral funisitis is present
Gross Images

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"Barber pole"

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Waves of inflammation

Micro Description
  • Tissue necrosis and the progressive accumulation of inflammatory cells, cellular debris and fluid into the umbilical cord substance
Micro Images

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Cellular debris in Wharton jelly

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Various images

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Concentric, partially calcified

Electron Microscopy Description
  • Necrotic debris and inflammation can be seen surrounding one or more umbilical vessels, often appearing as concentric 'waves'
  • The presence of immune complexes aggregated as arcs around vessels are analogous to the precipitin arcs seen in an Ouchterlony immunodiffusion assay
  • Calcification of the immune complexes or cellular debris can be partially or entirely encircling the vessel(s)