First branchial cleft cyst

Changed by Bruno Di Muzio, 1 Dec 2016

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First branchial cleft cysts are a type of branchial cleft anomaly. They are uncommon and only represent ~7% of all branchial cleft cysts.

Epidemiology

They are usually manifest earlydiagnosticated in first decademiddle-aged women 3-4

Clinical presentation

Their presentation can re broad 3:

  • asymptomatic incidental finding on imaging
  • palpable lump or inflammatory mass in the parotid region
  • spontaneous fluid draining from a pit-like depression on the skin, which may mucus or pus depending or not on the presence of life.associated infection
  • facial nerve palsy 4

Pathology

First branchial cleft cysts develop as a result of the incomplete fusion of the cleft between the first and second branchial arches. They may have a sinus with drainage to the external ear or skin.

That typically occur within or close to the parotid gland or external auditory canal region.

Subtypes

They can be divided into three types based on location:

  • type I: inferoposteromedial to pinna
  • type II: angle of the mandible to the external auditory canal
  • type III: periparotidperi parotid

They can also be classified based on histology:

  • type I
    • purely ectodermal
    • extremely rare
    • appear histologically as cysts lined by squamous epithelium
    • clinically presents as a cystic mass or fistula posterior to the pinna and concha
    • usually located superior to the main trunk of the facial nerve and ends in a cul-de-sac on or near a bony plate at the level of the mesotympanum
  • type II
    • contain ectodermal and mesodermal elements
    • comparatively commoner
    • represent a duplication of both membranous and cartilaginous portions of the external auditory canal
    • contain skin as well as adnexal structures and cartilage
    • may be associated with the parotid gland
    • often associated with fistulae in the concha or external auditory canal +/- fistulous openings in the neck
    • incorporates some portion of the first and second arch as well as the cleft

Radiographic features

They are typically well-defined cystic masses located superficial, within, or deeper to the parotid gland. If they form a sinus, the sinus tract can be identified draining into the external auditory canal or even extending to the hyoid bone.

Ultrasound

Sharply demarcated thin-walled cyst with variable echogenicity: from anechoic (most common) do different degrees of heterogeneity depending on internal debris. 

When infected, sonographic features of parotitis may be present. 

CT

Sharply circumscribed, fluid density, and thin-walled mass. The wall thickness and enhancement are variable and tend to increase with recurrent infections 4.

MRI
  • T1: variable signal dependent on protein content
    • high protein content: high signal
    • low protein content: low signal
  • T2: high signal
  • T1 C+ (Gd): no enhancement in uncomplicated lesions
  • -<p><strong>First branchial cleft cysts</strong> are a type of <a href="/articles/branchial-cleft-anomaly">branchial cleft anomaly</a>. They are uncommon and only represent ~7% of all branchial cleft cysts.</p><h4>Epidemiology</h4><p>They usually manifest early in first decade of life.</p><h4>Pathology</h4><p>First branchial cleft cysts develop as a result of incomplete fusion of the cleft between the first and second branchial arches. They may have a sinus with drainage to the external ear or skin.</p><p>That typically occur within or close to the <a title="Parotid gland" href="/articles/parotid-gland">parotid gland</a> or <a title="External auditory canal (EAC)" href="/articles/external-auditory-canal">external auditory canal</a> region.</p><h5>Subtypes</h5><p>They can be divided into three types based on location:</p><ul>
  • +<p><strong>First branchial cleft cysts</strong> are a type of <a href="/articles/branchial-cleft-anomaly">branchial cleft anomaly</a>. They are uncommon and only represent ~7% of all branchial cleft cysts.</p><h4>Epidemiology</h4><p>They are usually diagnosticated in middle-aged women <sup>3-4</sup>. </p><h4>Clinical presentation</h4><p>Their presentation can re broad <sup>3</sup>:</p><ul>
  • +<li>asymptomatic incidental finding on imaging</li>
  • +<li>palpable lump or inflammatory mass in the parotid region</li>
  • +<li>spontaneous fluid draining from a pit-like depression on the skin, which may mucus or pus depending or not on the presence of associated infection</li>
  • +<li>facial nerve palsy <sup>4</sup>
  • +</li>
  • +</ul><h4>Pathology</h4><p>First branchial cleft cysts develop as a result of the incomplete fusion of the cleft between the first and second branchial arches. They may have a sinus with drainage to the external ear or skin.</p><p>That typically occur within or close to the <a href="/articles/parotid-gland">parotid gland</a> or <a href="/articles/external-auditory-canal">external auditory canal</a> region.</p><h5>Subtypes</h5><p>They can be divided into three types based on location:</p><ul>
  • -<strong>type II</strong>: angle of mandible to external auditory canal</li>
  • +<strong>type II</strong>: angle of the mandible to the external auditory canal</li>
  • -<strong>type III</strong>: periparotid</li>
  • -</ul><p>They can also be classified based on histology</p><ul>
  • +<strong>type III</strong>: peri parotid</li>
  • +</ul><p>They can also be classified based on histology:</p><ul>
  • +</ul><h4>Radiographic features</h4><p>They are typically well-defined cystic masses located superficial, within, or deeper to the parotid gland. If they form a sinus, the sinus tract can be identified draining into the external auditory canal or even extending to the hyoid bone.</p><h5>Ultrasound</h5><p>Sharply demarcated thin-walled cyst with variable echogenicity: from anechoic (most common) do different degrees of heterogeneity depending on internal debris. </p><p>When infected, sonographic features of parotitis may be present. </p><h5>CT</h5><p>Sharply circumscribed, fluid density, and thin-walled mass. The wall thickness and enhancement are variable and tend to increase with recurrent infections <sup>4</sup>.</p><h5>MRI</h5><ul>
  • +<li>
  • +<strong>T1:</strong> variable signal dependent on protein content<ul>
  • +<li>high protein content: high signal</li>
  • +<li>low protein content: low signal</li>
  • +</ul>
  • +</li>
  • +<li>
  • +<strong>T2:</strong> high signal</li>
  • +<li>
  • +<strong>T1 C+ (Gd): </strong>no enhancement in uncomplicated lesions</li>

References changed:

  • 3. D'Souza AR, Uppal HS, De R, Zeitoun H. Updating concepts of first branchial cleft defects: a literature review. International journal of pediatric otorhinolaryngology. 62 (2): 103-9. <a href="https://www.ncbi.nlm.nih.gov/pubmed/11788142">Pubmed</a> <span class="ref_v4"></span>
  • 4. Koeller KK, Alamo L, Adair CF, Smirniotopoulos JG. Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics : a review publication of the Radiological Society of North America, Inc. 19 (1): 121-46; quiz 152-3. <a href="https://doi.org/10.1148/radiographics.19.1.g99ja06121">doi:10.1148/radiographics.19.1.g99ja06121</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9925396">Pubmed</a> <span class="ref_v4"></span>

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