Pediatric Surgery: Branchial Cleft Anomalies

Basics

• From Incomplete Branchial Cleft Obliteration During Embryogenesis
• 10% are Bilateral
• Risk for Recurrent Infection & Later Malignancy
• 20% Become Infected Prior to Excision

Presentation

• Classic Presentation: Lateral Mass Along the SCM
• Presentations:
  • Cartilaginous Remnant
  • Cyst – No External Opening
  • Draining Sinus – Opening to Either External Skin or Internal Pharynx
    • More Common than in Thyroglossal Duct Cysts
  • Fistula – Communication Between Both External Skin & Internal Pharynx

First Branchial Cyst

• Location: From Angle of Mandible to External Acoustic Meatus
• Often Closely Associated with Facial Nerve & Parotid Gland
  • At Risk During Excision
• May Have Connection to External Acoustic Meatus (Otorrhea/Infection)
• Subtypes:
  • I: Lateral to Facial Nerve
  • II: Medial to Facial Nerve

Second Branchial Cyst

• Most Common Type
• Location: From Supraclavicular Fossa to Tonsillar Pillar
  • Tract Travels Through the Carotid Bifurcation
• Subtypes:
  • I: Anterior Border of SCM
    • Most Superficial – Lowest Risk of Nerve Injury
  • II: Adjacent to Internal Carotid Artery & Adherent to Internal Jugular Vein
    • Most Common Subtype
  • III: Between Internal & External Carotid Arteries within the Carotid Bifurcation
  • IV: Deep to Carotid Sheath; Adjacent to Pharynx

Piriform Sinus Tract

• Generic Term for Both Third/Fourth Tracts
  • May Be Difficult to Distinguish
  • Third Enters Above the Superior Laryngeal Nerve
  • Fourth Enters Below the Superior Laryngeal Nerve
• Location:
  • Third: Posterior Cervical Space by Lower SCM
  • Fourth: Descend into Mediastinum Along Tracheoesophageal Groove
• Left Sided Most Common

Treatment

• Definitive Tx: Excision of Entire Tract
  • Transverse Cervical Incision Along a Skin Crease
    • Elliptical Incision Over Draining Sinus
  • Piriform Sinus Tracts Often Require Ipsilateral Thyroid Lobectomy
• Infected: Antibiotics & Delayed Excision
  • Antibiotics Should Cover Skin Flora (Add Broad Spectrum if an Oropharynx Connection is Suspected)
  • May Require Aspiration or Incision & Drainage if Abscess Develops

Associated Syndromes

• Branchio-Oto-Renal (BOR) Syndrome
  • Autosomal Dominant Condition
  • Presentation:
    • Branchial Cleft Cysts (May See “Preauricular Pits/Tags”)
    • Ear Abnormalities (Hearing Loss & Malformation)
    • Renal Abnormalities
  • Branchio-Otic Syndrome (BOS)
    • Similar to BOR Syndrome but No Renal Abnormalities
• Branchio-Oculo-Facial Syndrome
  • Autosomal Dominant Condition
  • Presentation:
    • Branchial Cleft Cysts (May See “Preauricular Pits/Tags”)
    • Eye Abnormalities (Microphthalmia & Obstructed Lacrimal Ducts)
    • Facial Abnormalities (Cleft Lip/Palate)