Branchial cleft anomaly

Background

During the 4th week of embryonic development, there are five branchial arches that grow into distinct parts of the head and neck, all of which consist of arteries, nerves, muscles, and skeletal tissue. If the branchial arches fail to fuse, a soft tissue anomaly on the lateral aspect of the neck may form, which is called a branchial cleft anomaly. The range of anomalies include cysts (most common), fistulas, and sinus tracts. The second branchial cleft anomalies are the most common.

Clinical Features

• Typically asymptomatic unless superinfected causing cellulitis or abscess formation

• First branchial cleft cyst
  • Lump in parotid/auricular region
  • Facial nerve palsy
  • May drain through neck and external auditory canal

• Second branchial cleft cyst
  • Most common
  • Present in late childhood or early adulthood
  • Swelling below angle of mandible and anterior to sternocleidomastoid
  • Sinus tracts travel into the deep neck structures and drain into tonsillar fossa
  • Fistulae cause mucous drainage from cutaneous opening at lateral neck
  • Very rarely become squamous cell carcinoma

• Third and fourth branchial cleft cyst
  • Difficult to differentiate between the two
  • Located lower in neck, anterior or posterior to sternocleidomastoid
  • Preference for left side

Differential Diagnosis

Acute

• Reactive lymphadenopathy- most common
  • Viral URI
  • EBV
  • CMV
  • Strep/staph
  • HIV
  • Toxoplasmosis
  • Bartonella henselae- kitten or flea exposure
  • Tuberculous lymphadenitis (scrofula)
• Descending infections from oral cavity
  • Ludwig's angina
• Sialoadenitis (can also be chronic)
• Trauma-related
  • Hematoma
  • Pseudoaneurysm or AV fistula

Subacute (weeks to months)

• Cancer
  • HPV-related squamous cell carcinoma
  • Upper aerodigestive tract squamous cell carcinoma
  • Metastatic disease
  • Lymphoma
  • Parotid tumors
• Systemic diseases
  • Amyloidosis
  • Sarcoidosis
  • Sjögren syndrome

Chronic

• Thyroid nodules or cancer
• Goiters
  • Graves' disease
  • Hashimoto thyroiditis
  • Iodine deficiency
  • Lithium use
  • Toxic multinodular
• Congenital cysts
  • Thyroglossal duct cyst- 2nd most common benign neck mass
  • Dermoid cyst
• Carotid body tumor
• Glomus jugulare or vagale tumor
• Laryngocele
• Lipoma/liposarcoma
• Parathyroid cysts or cancer

Evaluation

• Ultrasound
• CT
• MRI

Management

• Surgical excision is definitive treatment (high risk of recurrence of not completely excised)
• Antibiotics if superinfection present

Disposition

• Discharge with outpatient surgical referral (if no systemic signs of infection present)
• Admit for sepsis

See Also

• cellulitis
• neck mass