Preauricular Sinus: A Tale of Forgetful Rediscovery

Boyko Matev,Nikolay R Sapundzhiev,Emran Lyutfi,George S Stoyanov

doi:10.7759/cureus.8885

Abstract

The preauricular sinus (PAuS) is a congenital foramen, opening or invagination, usually located on the crus of the auricular helix and is considered a congenital malformation and component of multiple syndromes. The structure can be present unilaterally or bilaterally, with the possibility of more than one fistula present on one ear, predominantly on the auricular tags. As a well-defined and established clinical entry, PAuS has a very strictly laid-out history. However, different works of art give us a glimpse into the structure before its first true clinical description, showing that the PAuS was known to man long before it was first clinically described, such as those of Hieronymous Bosch, with the first medical descriptions being attributed to Heusinger and Virchow. In modern times, the condition is considered both an individual malformation and a component of several genetic syndromes.

Highlights

BackgroundThe preauricular sinus (PAuS) is a congenital malformation characterized by a dent, dimple, or a foramen, usually located on the crus of the auricular helix [1,2]
Structures with the same characteristics and clinical course can be found on the pinna, tragus, or even in the postauricular area contradictory to the established name in modern times [1,2,3]
The PAuS, as it was first described, is often a component of several eponymous inherited syndromes such as Melnik-Fraser (BOR syndrome), Beckwith-Wiedemann, and Lachiewicz-Sibley, which include a variety of constant kidney and other distant variable malformities such as cysts, fistulae, facial and neural defects, and others

Summary

Introduction

The preauricular sinus (PAuS) is a congenital malformation characterized by a dent, dimple, or a foramen, usually located on the crus of the auricular helix [1,2]. F. Heusinger when describing the findings in a patient characteristic of the brachio-oto-renal (BOR) syndrome [13]. One of the most important contributions from that time period was in 1955 by Fourman and Fourman, who researched the inheritance of PAuS, constructing a genealogical tree, focusing on the inheritance of the structure [24]. They concluded that half of the siblings of the affected family have the structure and postulated that the sinus is inherited as a dominant trait, but with “incomplete penetrance” due to it skipping in generations or individuals [24]. Patients with PAuS, not associated with a genetic syndrome, undergo a safe intervention process with few described complications, whereas patients with genetic syndromes undergo the same curative modalities, and the excision of the PauS does not affect their overall clinical course [7,40,41,42]

Conclusions

Disclosures

20. Whitney DD