A 2-week-old male came to clinic for his well child check. He was breastfeeding well and gaining 21 grams/day since his 1 week examination. He was urinating and stooling well and had no jaundice concerns. His parents had normal newborn concerns. The past medical history showed a full term male born by vaginal delivery without complications. He had passed a newborn screening hearing evaluation during his hospitalization. The family history was positive for Parkinson’s disease in a paternal great-grandmother. The review of systems was negative.
The pertinent physical exam showed an alert male with weight of 3.68 kg (50%), length of 49 cm (25%) and head circumference of 35.5 cm (65%). HEENT showed a left 2 mm preauricular skin tag without a pit, and a normal shape to both ears. The rest of his examination was negative.
The diagnosis of a healthy newborn with an isolated preauricular skin tag was made. The resident seeing the patient was concerned about the skin tag and wanted to order a renal ultrasound because of potential renal abnormalities. The attending physician discussed with her that the infant had no other risk factors and therefore the risk of renal disease was basically the same as the general population. Later the attending physician was able to search the literature and find several papers that supported non-evaluation of simple isolated preauricular abnormalities.
In 1946, Dr. Edith Potter described renal agenesis along with flattened ears (i.e. Potter’s Syndrome). Isolated preauricular tags (IPT) have an incidence of ~5-10/1000 live births. These are the most common minor external ear abnormalities and are often noted incidentally on physical examination. Renal malformations have an incidence of ~1-3/100 live births. They can be seen together in a variety of genetic diseases including:
- BOR syndrome – brachio-oto-renal abnormalities
- CHARGE association
- Diabetic embryopathy
- Epstein Syndrome
- Miller syndrome
- Muckle-Wells syndrome
- Nager syndrome
- Oculoauriculovertebral syndrome
- Townes-Brocks syndrome
Ear and renal tissue arise embryologically at similar but different times and therefore some of these associations are best described by gene expression and not specific insults during the embrologic time period.
Patients with multiple congenital anomalies or syndromic external ear anomalies should be investigated for potential renal abnormalities. This is particularly true in the setting of a family history of potential genetic syndrome or known renal or hearing disease. While there have been some studies that show an increased association with ear and renal abnormalities, there have been many studies which show that patients with isolated IPTs and preauricular pits having the same risk of significant renal abnormalities as the general population and therefore IPT and pit existence alone does not warrant additional evaluation of the renal system.
Questions for Further Discussion
1. What are indications for renal ultrasonography?
2. What are indications for hearing rescreening in neonates?
3. What are indications for removal of preauricular skin tags?
- Disease: Isolated Preauricular Ear Tag | Ear Disorders
- Symptom/Presentation: Health Maintenance and Disease Prevention
- Age: Newborn
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Information prescriptions for patients can be found at MedlinePlus for this topic: Ear Disorders
To view current news articles on this topic check Google News.
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Kugelman A, Tubi A, Bader D, Chemo M, Dabbah H. Pre-auricular tags and pits in the newborn: the role of renal ultrasonography. J Pediatr. 2002 Sep;141(3):388-91.
Izzedine H, Tankere F, Launay-Vacher V, Deray G. Ear and kidney syndromes: molecular versus clinical approach. Kidney Int. 2004 Feb;65(2):369-85.
Deshpande SA, Watson H. Renal ultrasonography not required in babies with isolated minor ear anomalies. Arch Dis Child Fetal Neonatal Ed. 2006 Jan;91(1):F29-30.
Lizama M, Cavagnaro F, Arau R, Navarrete O, Fontanaz AM, Garcia CJ. Association of isolated preauricular tags and nephrourological anomalies: case-control study. Pediatr Nephrol. 2007 May;22(5):658-60.
Firat Y, Sireci S, Yakinci C, Akarçay M, Karakas HM, Firat AK, Kizilay A, Selimoglu E. Isolated preauricular pits and tags: is it necessary to investigate renal abnormalities and hearing impairment? Eur Arch Otorhinolaryngol. 2008 Sep;265(9):1057-60.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital