A 41-year-old woman presented with a 5-year history of right ear blockage and pain was diagnosed with a sympathetic chain schwannoma after imaging showed a mass along the sympathetic chain in the neck. A 43-year-old man with seizures was found to have a dural arteriovenous fistula involving the left sigmoid and transverse sinuses. A 15-year-old boy evaluated for a neck mass was diagnosed with pyriform sinus fistulae after follow-up imaging showed an abnormal tract extending from the pyriform sinus into the neck.

1. Unknown cases
Amy F Juliano, MD
Massachusetts Eye and Ear Infirmary
Harvard Medical School

2. 41 year-old woman with 5-yr history of
right ear blockage and intermittent pain

19. Diagnosis?

20. Sympathetic chain schwannoma

21. Sympathetic chain
• Travels up the neck in the carotid sheath
• Continues up through the carotid canal
• Forms plexus on ICA

22. Schwannomas in the head and neck
• Most common
– Vagus >> sympathetic chain
• Vagus – dysphagia, hoarseness
Sympathetic chain – Horner’s
• In most cases, patients present with a palpable mass or
have no symptoms

23. • Vagus
- Lateral to ICA
• Sympathetic chain,
cervical sympathetic
ganglion
- Posteromedial to
ICA

24. Treatment
• Observation
• Surgery
– Post-operative Horner’s
– First bite syndrome
• First bite syndrome
– Pain in the parotid area on the first bite of food
– Intense ear pain increased with strong sialogogues
– Due to loss of sympathetic input to parotid after severing the
chain
– Denervation hypersensitivity

25. 43 year-old man with seizures

62. Diagnosis?

63. Multichannel dural AVF of left sigmoid-transverse sinus
Venous infarction with vasogenic edema

64. Dural AVF
• Abnormal connection between dural arteries or
pachymeningeal branches of cerebral arteries and dural
veins
• Arteries can be recruited from branches of both the ECA
and ICA
• Venous drainage can occur through large dural venous
sinuses, and retrogradely through cortical cerebral veins
• If cortical cerebral veins are involved, there is a higher
risk of rupture and hemorrhage from the dAVF
• Frequently located in the transverse and sigmoid sinuses

65. Dural AVF
• Believed to result from thrombosis of a dural venous
sinus, with subsequent collateral revascularization
• Leads to venous hypertension, an initiating factor
opening up microscopic vascular connections within the
dura
• Results in abnormal fistulous connection between
arteries and veins in the walls of a dural sinus or
involving an adjacent cortical vein

66. Dural AVF
• Venous hypertension may be related to thrombosed
dural venous sinus or arterialized veins
– Pulse synchronous bruit
– Pulsatile tinnitus
– Headache
– Visual impairment
– Papilledema
– Venous infarct
– Cerebral hemorrhage (SAH, SDH, parenchymal)

67. Dural AVF
• Borden classification system
– Type I: antegrade drainage through a dural venous sinus or
meningeal vein (usually benign clinical behavior)
– Type II: antegrade drainage into dural venous sinus and
retrograde flow into cortical veins (may present with
hemorrhage)
– Type III: direct retrograde flow into cortical veins (causes venous
hypertension with risk of hemorrhage)

68. Dural AVF
• Cognard system
– Type I: normal antegrade flow into a dural venous sinus
– Type IIa: drainage into a sinus with retrograde flow within the
sinus
– Type IIb: drainage into a sinus with retrograde flow into cortical
vein(s)
– Type II a+b: drainage into a sinus with retrograde flow within the
sinus and cortical vein(s)
– Type III: direct drainage into a cortical vein without venous
ectasia
– Type IV: direct drainage into a cortical vein with ectasia > 5 mm
and 3x larger than the diameter of the draining vein
– Type V: direct drainage into spinal perimedullary veins

69. Dural AVF
• Treatment is indicated in aggressive cases, typically
those showing cortical venous reflux on angiography
• Treatment options include surgical and endovascular
approaches, or occasionally radiation

70. 15 year-old boy with neck mass

75. Follow-up US 3 weeks later

76. Follow-up CT 2 months after initial CT

78. Diagnosis?

79. Pyriform sinus fistulae

81. • Acute suppurative thyroiditis and thyroid abscess are
extremely rare.
• Acute suppurative neck infections are frequently
recurrent when associated with branchial fistulas (3rd or
4th)
• When an inflammatory infiltration or abscess is present
between the pyriform fossa and the thyroid bed in the
lower neck, esp on L, an infected 3rd or 4th branchial fistula
must be suspected

82. Pyriform sinus fistula

83. Pyriform sinus fistula
• The 3rd & 4th branchial pouches form the pyriform sinus
• Persistent ducts from either of these pouch sinuses may
drain into the pyriform sinus
• There are authors suggesting that there is wide
discrepancy between clinical/radiologic presentations
and the theoretical course of the 3rd and 4th branchial arch
anomalies
• They propose that persistence of the thymopharyngeal
duct of the 3rd pouch, frequently passing through or
adjacent to the thyroid gland, most often on the L side, is
the more suitable explanation embryologically

84. Pyriform sinus fistula
• >80% of cases are left-sided
• >60% of cases occurred after acute URI
• High rate of recurrence
• Children or young adults
• Pathway: pyriform sinus apex, anteroinferiorly through
the strap muscle layer, beside or through the thyroid
gland, into perithyroidal space

85. Pyriform sinus fistula
• CT is preferred imaging modality – can show air in sinus
or fistulous tract, thyroid gland involvement by loss of
normal high density, subtle infiltration or stranding
• Barium esophagram during active infection often do not
show the sinus or fistulous tract, perhaps because of
closure of the tract due to regional inflammation and
edema
• Perform barium swallow after infection has cleared

86. 46 year-old man with acute onset of vertigo

89. Diagnosis?

90. Endolymphatic sac tumor

91. Endolymphatic Sac Tumor
• Papillary epithelial neoplasm involving the
endolymphatic sac or duct
• Also been termed CPA ceruminoma,
adenocarcinoma, papillary adenomatous
tumor, etc
• Most occur sporadically; association with
von Hippel Lindau
• Papillary adenomatous architecture,
areas of hemorrhage, hemosiderin,
cholesterol clefts, giant cell reactions
• Slow-growing, may recur locally
• Involves posterior edge of petrous bone,
frequently involve dura

92. Endolymphatic Sac Tumor
• Hypervascular, locally-invasive, bone-
destroying; can have reactive new bone
formation
• CT: geographic, moth-eaten, intratumoral
bone reticular or spiculated, thin rim of
calcification
• MR: heterogeneous signal, areas of high
signal on T1-weighted sequence
• Late presentation: unilateral hearing loss,
vestibular dysfunction; facial nerve palsy
when tumor becomes large
• Duration of hearing loss 6 months to 18
years