π Multiple Preauricular Tags β Minor congenital anomaly, association with hearing loss and renal anomalies, branchial arch derivativesπ Paeds Online β paeds.online
βοΈ OBSERVED STATION Β· CPSP FORMAT Β· 8 MINUTES Β· SEPARATE TABS Β· CLINICAL PHOTO INCLUDED
A 2-day-old term male infant is being examined in the newborn nursery. The mother had an uncomplicated pregnancy and spontaneous vaginal delivery. There is no family history of congenital anomalies or hearing loss. On examination, the infant is well-appearing with normal vital signs. He has three small, fleshy, skin-colored papules (tags) located anterior to the right ear (preauricular region). The external auditory canal is normal in appearance. The left ear is normal. The rest of the physical examination, including oropharynx, cardiac, abdominal, spine, and genitourinary systems, is unremarkable. The infant passes meconium and urinates normally.
A clinical photograph of the preauricular tags is shown below.
Task: Describe the findings, propose the most likely diagnosis, discuss the embryological basis, identify associated anomalies (hearing loss, renal anomalies, branchio-oto-renal syndrome), and outline the appropriate evaluation and parental counseling.
π Figure: Multiple preauricular tags (skin-colored pedunculated papules) located anterior to the right ear. These are minor congenital anomalies arising from the first and second branchial arches. Multiple tags (especially bilateral) warrant evaluation for hearing loss and renal anomalies (branchio-oto-renal syndrome).
π‘ Examiner instruction (interactive): The candidate will be asked to identify preauricular tags, explain their embryology (first branchial arch derivatives), differentiate from other ear anomalies, discuss associations with hearing loss and renal anomalies, recommend appropriate screening (audiology, renal ultrasound), and counsel parents regarding reassurance and follow-up.
π Examiner Questions (interactive) β Click to reveal model answers
β Q1 (Examiner): βDescribe the findings in the image. What is the most likely diagnosis?β
β Candidate's structured answer:
β’ Findings: Multiple (three) small, fleshy, skin-colored pedunculated papules (tags) located anterior to the right ear (preauricular region). The external auditory canal appears normal. No associated pits or other ear anomalies.
β’ Diagnosis: Multiple preauricular tags (also called preauricular appendages or ear tags). These are minor congenital anomalies.
β Q2 (Examiner): βWhat is the embryological basis of preauricular tags?β
β Candidate's answer:
β’ The external ear develops from the first (mandibular) and second (hyoid) branchial arches during the 5th-6th week of gestation.
β’ Preauricular tags result from accessory hillocks (supernumerary hillocks of His) β residual tissue from the fusion of the six auricular hillocks that normally form the auricle.
β’ Alternatively, they may represent remnants of the first branchial cleft.
β’ They can be isolated sporadic findings or part of branchial arch syndromes (e.g., branchio-oto-renal syndrome, Goldenhar syndrome, Treacher Collins syndrome).
β’ Multiple tags, especially bilateral, are more likely to be associated with systemic anomalies.
β Q3 (Examiner): βWhat are the differential diagnoses of a preauricular mass/tag in a newborn?β
β Candidate's answer:
β’ Preauricular tag (most common) β soft, pedunculated, skin-colored, no cartilage.
β’ Preauricular pit (sinus) β small depression anterior to ear, may be associated with branchio-oto-renal syndrome and hearing loss.
β’ Accessory tragus β a small cartilaginous elevation, often with a different histology, may be mistaken for a tag.
β’ Dermoid cyst β deeper, fluctuant, not pedunculated.
β’ Epidermoid cyst β deeper, can become infected.
β’ Branchial cleft remnant β usually along sternocleidomastoid, not in preauricular area.
β’ Hemangioma β red/purple, may appear later; not skin-colored at birth.
β’ Lymphatic malformation β softer, transilluminates.
β Q4 (Examiner): βWhat is the association between preauricular tags and hearing loss? What screening is recommended?β
β Candidate's answer:
β’ Increased risk of sensorineural and/or conductive hearing loss in children with preauricular tags (especially multiple or bilateral).
β’ Risk: Approximately 5-10% of children with preauricular tags have hearing loss (compared to <1% in the general population). Risk is higher if tags are bilateral, multiple, or associated with other ear anomalies (microtia, pits).
β’ Pathogenesis: Shared embryological origin β external ear, middle ear, and cochlea develop from branchial arches. Anomaly in one indicates possible anomaly in another.
β’ Screening:
- Newborn hearing screening (OAE and/or ABR) is mandatory for all newborns, but particularly important in these infants.
- If the newborn passes universal screening, clinical surveillance for hearing loss continues in infancy.
- If fails or has risk factors (bilateral tags, syndromic features), refer to audiology and otolaryngology for diagnostic ABR and long-term follow-up.
β Q5 (Examiner): βWhat is the association between preauricular tags and renal anomalies? When should renal ultrasound be performed?β
β Candidate's answer:
β’ Association: Preauricular tags (especially multiple or bilateral) are associated with renal anomalies in 1-5% of cases (compared to ~0.5% in general population).
β’ Pathogenesis: First and second branchial arches (ear) and the genitourinary system share developmental pathways (neural crest migration). Abnormalities in one may indicate abnormalities in the other.
β’ Indications for renal ultrasound:
- Bilateral preauricular tags.
- Multiple tags (>1 on same side).
- Associated with preauricular pits or other ear anomalies (microtia).
- Syndromic features (facial asymmetry, eye anomalies, cardiac defects).
- Family history of renal anomalies or hearing loss.
- In isolated unilateral single tag with normal examination, many experts do not recommend routine renal US (controversial). However, CPSP guidelines may suggest a low threshold for screening.
β’ If renal ultrasound performed, do it in the neonatal period or early infancy.
β Q6 (Examiner): βWhat is branchio-oto-renal (BOR) syndrome? How does it present?β
β Candidate's answer:
β’ Branchio-oto-renal (BOR) syndrome is an autosomal dominant disorder (EYA1 gene mutation, chromosome 8q13.3).
β’ Triad:
1οΈβ£ Branchial anomalies: Preauricular tags/pits (often bilateral, multiple), branchial cleft cysts/fistulae.
2οΈβ£ Oto (ear) anomalies: Hearing loss (sensorineural, conductive, or mixed), malformed pinnae (microtia), preauricular pits, ossicular chain anomalies.
3οΈβ£ Renal anomalies: Renal agenesis, hypoplasia, dysplasia, polycystic kidneys, vesicoureteral reflux.
β’ Other features: Lacrimal duct stenosis, palatal abnormalities.
β’ Inheritance: Autosomal dominant with variable expressivity; 50% recurrence risk for offspring.
β’ If suspected: Refer to genetics, audiology, renal ultrasound, and consider EYA1/SIX1/SIX5 gene testing.
β Q7 (Examiner): βWhat other syndromes are associated with preauricular tags?β
β Q8 (Examiner): βWhat is your recommended evaluation for this infant with multiple right preauricular tags (normal exam otherwise)?β
β Candidate's structured answer:
1οΈβ£ Complete physical examination β focus on: hearing (behavioral), ear canals, pits, facial asymmetry, eye anomalies (epibulbar dermoids), palate, cardiac exam, spine, kidneys (palpation), genitourinary.
2οΈβ£ Newborn hearing screen (OAE/ABR) β mandatory. If passes, no immediate audiology referral unless syndromic. If fails, refer to audiology/ENT.
3οΈβ£ Renal ultrasound β recommended for this infant because he has multiple (>1) tags on one side (three tags). Some guidelines suggest bilateral tags or associated anomalies; many experts would screen for renal anomalies given the increased risk.
4οΈβ£ Ophthalmologic exam β if Goldenhar syndrome suspected (epibulbar dermoids, coloboma).
5οΈβ£ Genetic evaluation β if dysmorphic features, family history, or suspicion of syndrome (BOR, Goldenhar, Treacher Collins).
6οΈβ£ Parental examination β check for preauricular tags/pits, hearing loss, or renal anomalies in parents (for autosomal dominant conditions).
β Q9 (Examiner): βDo preauricular tags need treatment? If removal is desired, when and how is it done?β
β Candidate's answer:
β’ No medical treatment needed β tags are benign and not harmful.
β’ Removal is cosmetic only β for parental preference or if the child experiences repeated trauma, irritation, or social stigma.
β’ Timing: Can be performed electively at any age. Many surgeons wait until 6-12 months of age to allow for better anesthetic safety (if general anesthesia required for multiple tags). Simple tags can be removed in the clinic under topical/local anesthesia.
β’ Methods:
- Snip excision with local anesthesia (simple tags with narrow peduncle).
- Ligation (tie-off) with suture β causes ischemic necrosis and fall-off in a few days (simple small tags).
- Electrocautery or laser excision.
β’ Post-removal: wound healing is excellent; recurrence rare.
β’ Important: If removal is performed, send tissue for histopathology (to rule out rare neoplasms).
β Q10 (Examiner): βHow will you counsel the parents about these preauricular tags? What reassurance and what red flags will you discuss?β
β Candidate's structured answer:
β’ Reassurance: βThese are extra skin tags (ear tags) that occur in about 1 in 100 babies. They are not dangerous and do not hurt the baby. They are completely benign.β
β’ No immediate treatment needed β they can be removed later for cosmetic reasons if the family wishes.
β’ Associated risks explained: βBecause these tags come from the same tissue that forms the ears and kidneys, there is a small chance (about 5%) of hearing issues or kidney anomalies. We will screen for these to be safe.β
β’ Planned investigations: βWe will do a newborn hearing test. We also recommend a kidney ultrasound to rule out any structural kidney problem.β
β’ Red flags to watch for: βIf you notice that your baby does not respond to loud sounds, or if he has any feeding difficulty, breathing issues, or unusual facial features, tell us right away.β
β’ Family screening: βWe should also examine the parents for ear tags or pits, as some conditions are inherited.β
β’ Prognosis: βMost children with isolated tags have completely normal hearing and kidney function. The tags do not affect development.β
β’ Removal option: βIf you wish to remove the tags for appearance, we can do a simple procedure when the baby is older, usually after 6 months.β
β Q11 (Examiner): βWhat is the long-term prognosis for an infant with multiple preauricular tags and a normal renal ultrasound and normal hearing screen?β
β Candidate's answer:
β’ Excellent prognosis β if renal ultrasound and newborn hearing screen are normal, the likelihood of significant associated anomaly is very low.
β’ However, mild or late-onset hearing loss (especially conductive loss due to ossicular anomalies) can develop later in childhood. Therefore, continued clinical surveillance of hearing and language development is recommended.
β’ Follow-up plan:
- Routine well-child visits with developmental and hearing checks.
- Formal audiology testing if the child has speech delay, recurrent otitis media, or parental concern.
- No further imaging for kidneys if initial ultrasound is normal and no urinary symptoms develop.
- Parents should be informed of signs of hearing loss (failure to respond to sound, delayed speech).
- Genetic evaluation if additional features develop.
β’ Cosmetic: Tags can be removed electively.
β’ No activity restrictions.
β Q12 (Examiner): βWhat is the difference between a preauricular tag and a preauricular pit? Which carries higher risk of syndromic associations?β
β Candidate's answer:
β’ Preauricular tag: A fleshy, pedunculated skin-colored papule (accessory hillock). Contains skin Β± cartilage. Usually benign. Can be isolated or syndromic.
β’ Preauricular pit (sinus): A small depression (<1 mm) anterior to the ear. May be a blind-ending sinus tract. Can become infected (cellulitis, abscess). Associated with higher risk of branchio-oto-renal syndrome and hearing loss, especially if bilateral or associated with tags.
β’ Both are associated with hearing loss and renal anomalies β but pits may have a slightly higher association with syndromic causes (especially BOR).
β’ Management difference: Pits require counselling about risk of infection (if debris collects). Infected pits may need antibiotics or surgical excision. Tags do not get infected spontaneously.
β’ Both warrant audiology and renal ultrasound if multiple, bilateral, or syndromic features present.
π£οΈ Examiner's probing / high-yield points:
β’ "What is the embryological origin of preauricular tags?" β First and second branchial arches (hillocks of His).
β’ "What is the association with hearing loss?" β ~5-10% risk; mandate newborn hearing screen.
β’ "When should you do a renal ultrasound?" β Bilateral, multiple, syndromic features, or family history of renal anomalies.
β’ "What is branchio-oto-renal syndrome?" β Autosomal dominant (EYA1); triad: branchial anomalies (tags/pits), hearing loss, renal anomalies.
β’ "Do tags need treatment?" β No; removal is cosmetic and elective.
β’ "What is the difference between a tag and a pit?" β Tag = fleshy papule; pit = depression. Pits carry higher infection risk.
β’ "What is the prognosis with normal screening?" β Excellent, but monitor hearing clinically.
π Preauricular Tags β Core Revision for TOACS
π Definition Small, fleshy, pedunculated skin-colored papules located anterior to the external ear. Accessory hillocks of His (first and second branchial arches).
π Epidemiology ~1 in 100 live births. Usually unilateral (80%), can be multiple or bilateral. Sporadic or familial (autosomal dominant).
βοΈ Evaluation Newborn hearing screen (OAE/ABR). Renal ultrasound if bilateral, multiple, or syndromic features. Examine for dysmorphic features. Family history.
πͺ Treatment No medical treatment needed. Cosmetic removal (snip excision, ligation) can be done after 6-12 months.
π Prognosis Excellent if isolated and screening normal. Monitor hearing and language development. No risk of malignant transformation.
β High-yield pearls for TOACS (Preauricular Tags):
β’ Embryology: First and second branchial arches (hillocks of His).
β’ Associated anomalies: Hearing loss (5-10%) and renal anomalies (1-5%).
β’ Bilateral or multiple tags = higher risk β need renal ultrasound and audiology.
β’ Branchio-oto-renal (BOR) syndrome: Autosomal dominant (EYA1) β tags/pits + hearing loss + renal anomalies.
β’ Parental counseling: Benign, cosmetic only, no urgency. Screen hearing and kidneys if indicated. Removal is elective.
β’ Difference from pit: Pit is a depression, higher infection risk, also needs screening.
π£οΈ Candidate's role-play & examiner feedback
π¬ To the candidate (roleβplay): You will be asked the 12 questions from the Examiner Q&A tab (including clinical recognition, embryology, associated anomalies, screening recommendations, syndromes, and parental counseling). Provide concise, evidenceβbased answers. Examiner may ask about the indications for renal ultrasound or the features of branchio-oto-renal syndrome. Use structured points and demonstrate reassurance to parents.
β Lists other syndromes: Goldenhar, Treacher Collins, CHARGE, 22q11.2
β States that treatment is cosmetic only (snip excision or ligation)
β Provides appropriate parental reassurance (benign, no urgency, screening for hearing/kidneys)
β Recognizes good prognosis with normal screening; recommends clinical hearing surveillance
π Key references: Nelson Textbook of Pediatrics 22e (Chapter 654 β Ear Abnormalities), Branchio-oto-renal syndrome literature, CPSP guidelines on newborn examination, American Academy of Pediatrics (hearing screening).