Ear fistula and cyst, congenital Q17.0

Unilateral or bilateral (unilateral in about 85% of patients), congenital, manifesting in childhood or adolescence (less frequently in early adulthood), potentially combined with other anomalies, branchiogenic malformation of the 1st gill duct.

See also cysts and fistulas, branchiogenic.

Branchiogenic malformations result in cysts and sinui that drain outward through the skin at certain sites on one side and internally into the pharynx on the other. On the skin, they appear as red-brown, smooth-surfaced or verrucous papules or as inconspicuous indurations, but also as weeping, granulomatous inflammations (usually they then become clinically conspicuous).

m:w=1:1; congenital fistulas are usually discovered between the ages of 2 and 16 years.

The external fistula opening is located above or in front of the tragus or in the area of the ascending helix. It is perceived as a 0.3-0.5 cm large, reddish-brownish nodule resembling a foreign body granuloma; often with a visible central porus. Rare small ulcer. Usually the porus is not inflammatory. However, it can also develop folliculitis-like inflammatory symptoms with purulent secretion.

Accumulates stubborn auditory canal eczema.

Clinical diagnosis first, as an atheroma- to fibroma-like, occasionally pore-like structure can be diagnosed at a typical location. As the diagnosis can usually be made in infancy or early childhood, the caregivers should be informed as comprehensively as possible. This includes explaining that the change is harmless. However, it should be conveyed that under no circumstances should manipulative measures such as pressing, squeezing or improper surgical removal be carried out. The explanation must also include the procedure to be followed if secretions or inflammation occur, as it is usually necessary to prevent inflammation from progressing into the deep duct system. In this case, early external and internal antibiotics can be considered. Alternatively, radiographic imaging of the fistula tract after probing and injection of contrast medium can be considered, and appropriate surgery can be arranged by experienced colleagues.

Excision following radiographic imaging of the fistula tract after probing and injection of contrast medium. A specialist excision is recommended!

1. Ellies M et al (1998) Clinical evaluation and surgical management of congenital preauricular fistulas. J Oral Maxillofac Surgery 56: 827-830
2. Kuczkowski J et al (2011) Diagnosis and treatment of preauricular fistulas in children. Otolaryngol 65: 194-198
3. Martin-Granizo R et al (2002) Methylene blue staining and probing for fistula resection: application in a case of bilateral congenital preauricular fistulas. Int J Oral Maxillofac Surgery 31: 439-441
4. Nakano M et al (1999) Congenital cheek fistula: a report of three cases. Br J Plast Surgery 52: 311-313
5. Nicollas R et al (2000) Congenital cysts and fistulas of the neck. Int J Pediatr Otorhinolaryngol 55: 117-124
6. Song J et al (2015) Analysis of chromosome regions 8q11.1-q13.3, 1q32-q34.3 and 14q31.1-q13.3 in a Chinese family with congenital preauricular fistula. Zhonghua Yi Xue Yi Chuan Xue Za Zhi 32:472-475
7. Zou F et al (2003) A locus for congenital preauricular fistula maps to chromosome 8q11.1-q13.3 J Hum Genet 48: 155-158