Ear-Pit (Congenital Aural and Pre-Auricular Fistula) *
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EAR-PIT (CONGENITAL AURAL AND PRE-AURICULAR FISTULA) * By IAN AIRD, Ch.M., F.R.C.S.E. Fro?n the Department of Surgery, University of Edinburgh, and the Royal Edinburgh Hospital for Sick Children CONGENITAL aural fistula is a relatively rare deformity. Bezold (1885) and Urbantschitsch (1877) estimated its frequency at 0*19 per cent., and Eyle (1891) at 0-26 per cent. Rugani (1910) quoted a much and higher figure, apparently the frequency may rise to 5 per cent, in a small community which includes several affected families. Patho- logically there is nothing and surgically there is little of interest in the anomaly, but its illustration of certain genetic principles is illuminating, the understanding which it affords of the development and the of the pinna is valuable, contribution which it may make to chronic inflammations of the face is occasionally important. The affection was first described 'by Heusinger in 1864, and Virchow in the same year and Urbantschitsch subsequently speculated upon its possible origin from the first branchial cleft or the cleft between mandibular and maxillary processes. The first English description (apart from a translation of Urbantschitsch's paper) was that of Sir James Paget in 1878. Clinical Appearance.?The ear-pit is a narrow, blind fistula, as a rule a few millimetres deep (Gradenego, 1893 ; Marx, 1926), and will accommodate the lead point, and the point only, of a finely sharpened pencil. It may be unilateral or bilateral, and sometimes two or more pits occur together in the same ear (Fig. 1). Multiple pits may rarely communicate with each other (Schuller, 1929), though it is suspected that in such cases the communication is effected only by suppuration. Rarely also does a pit communicate with a branchial fistula (Thollon and Labernadie, 1919). The areas in which the pit may be found are indicated in Fig. 2. (1) The commonest site of those which become infected and require treatment is pre-aitricular, in a roughly triangular area based on the anterior border of the attachment of the auricle, and narrowing to an apex which lies on the line from the tragus to the angle of the mouth. They seldom lie more than an inch in front of the tragus. The truly auricular varieties are (2) anterior (;marginal) helicine, on the ascending limb of the helix, which includes 90 per cent, of all these fossae (Congdon et al., 1932) ; (3) crural, in the crus helicis, recorded once by Schwabach (1879), and eleven times by Congdon ; (4) posterior helicine, on the * Read at a meeting of the Edinburgh Medico-Chirurgical Society on 5th December 1946. 498 EAR-PIT 499 postero-lateral aspect of the descending limb of the helix, and illustrated only by five of Congdon's subjects, one of whom, a Chinese girl, aneMMSw istufistulas as in in Fig.Fig. i.?Combination i. Combination, of ofunilateral unilateral anterior anterior helicine helicine and and infected infected pre-auriculai pre-auricular uniunilateral atera a a andand one one brother,brother, present present girlgirl of ofnine nine years. years. TheThe child's child's father, father, youngeryounger andon on the the same same side. side. anterioranterior helicine helicine fistulas fistulas in inprecisely precisely the the same same situation situation and Fig.F IG" 2'-> Anatomical distribution of ear-pits, after Congdon. helic?-lobular> in the same ear ; ^ pitS J central and Breuer> r927) (6) very ' l889' or on rarelyTecorH^0/^!101116 of the lobule, lobular, on the mrri"lal or on the lateral aspect 5oo IAN AIRD both (Sabrazes, 1890; Fischer, 1903; Jenks, 1916; Edmonds and Keeler, 1940), and rarely piercing the entire thickness of the lobule (Bland-Sutton, 1887) as for an ear-ring ; (7) post-auricular, close to the root of the ear, and recorded only rarely (Battle, 1905, and Hadda, 1909). A pit or fossa has been described once in the floor of the inferior articular (triangular) fossa (Congdon, 1932), once in the concha (Caldera, 1872), and once in the antitragus (Dyer, 1885). It should not be assumed that every pit or fossa in the pinna is congenital. Artificial pits can be treated by children addicted to ear-picking, and this explanation should be considered especially if a pit or fossa is found in an unorthodox situation. Acquired pitting is common in the lobules of those races who practise the ear-piercing of the newly-born. The orifice of a congenital ear-pit is usually tiny in size, and either circular or lentiform in outline. The track is straight and can be conveniently probed by a bristle. It is lined by squamous epithelium only (Grunert, 1898 ; Steiner, 1925 ; Breuer, 1927), though rarely an island of cartilage may be present in its wall. The epithelium may be folded and valvular, and sometimes a tiny diverticulum branches from the lumen. The direction of the track varies with the situation of the fistula. A bristle introduced in a pre-auricular fistula passes backwards, inwards and slightly upwards towards the helix ; the fundus of the anterior helicine variety lies almost immediately below its mouth ; the crural track lies along the line of the crus, and the other varieties are usually directed medially. In a unique example observed by Fournier (1919) the track passed through the parotid gland and coursed downwards to open again on the skin surface just below and behind the angle of the mandible, but in this case it is presumed (Congdon) that a true pre-auricular fistula had acquired a connexion with a cavity derived from a branchial groove or pouch. In no instance has a true pre-auricular or auricular fistula been shown to communicate with the external meatus ; one fistula, described by Virchow (1864) did communicate with the external meatus, but it is doubtful whether this particular example was a true pre-auricular fistula. On the skin of the cheek, below and in front of the orifice of a pre-auricular or anterior helicine fistula, there may be a curious area of patchy scarring of the skin, elongated from above downwards and likened by Streeter to striae gravidarum in miniature. The scarring may be superficial to the deeper part of the track or may lie in front of the position of the fundus, showing no relation whatever to the track. This scarring appears in an illustration in the paper of Stammers (1926) who ascribes it to suppuration in the deeper part of a pre-auricular fistula, but it has been observed even in early infancy, when suppuration has not had time to become established, and Heusinger (1864) recorded its symmetrical bilateral occurrence in front of marginal helicine sinuses. EAR-PIT 501 A pigmented mole, often tiny, is not uncommonly detected in front of the fistulous opening-, and a few centimetres from it. Only occasionally does stagnation of inspissated sebum occur, sometimes with the production of a musty odour unpleasant to the patient, sometimes with suppuration, recurrent abscess-formation, facial oedema, and, exceptionally, the appearance of secondary fistulae. Relation to Other Anomalies.?In a demonstration of con- genital deformities of the face at the Royal College of Surgeons of England in 1909, Sir Arthur Keith illustrated the concurrence of pre-auricular fistula with cleft palate, spina bifida, and imperforate anus. Other deformities of the ear?accessory auricle, partial absence of the auricle, and incomplete development of the ear in its various degrees may be associated with fistula. The vast majority of congenital instances more recently reported, however, particularly those ascribed by anatomists and geneticists, have occurred in otherwise normal people. Congdon and his fellow-workers found only two associated abnormalities of ear or face in 518 Asiatics and white and co oured Americans who presented congenital aural fistulas. The stula itself is rarely the subject of a surgical consultation, while any associated abnormality often is, and the association therefore seems frequent to the surgeon. The simplicity of the defect is so absolute that it is difficult to reco ect any pathological structure of less complexity, unless it be ^sence of an organ or of part of an organ, yet similar fossae elsewhere t e are body not numerous. The rare mucous pits of the lower lip, ^nsituated on its buccal aspect and usually bilateral, are identical in ructure , they are said to be due to incomplete fusion between tuber- cu um and impar the mandibular processes. The pits of the upper p w ich are sometimes associated with cleft palate and seem to be ue> 1 e hare-lip, to non-fusion of the philtrum and the maxillary P ocesses, are similar also, as are the rare fistulae of the columella. EVELOPment of the Pinna.?The pinna develops around the Sa of the extremity first branchial cleft (Fig. 3). Its first appearance by His (1885) as of six low hillocks or tubercula, se es^1"it)edfr?m each other by shallow depressions. The first two tur" a on tjleercu *.e the branchial edge of the first arch, the third embraces from^h^1?1^- ^rSt c'e^' and the fourth, fifth and sixth arise margin of the second arch. Streeter (1922) describes two h'lT C^an^^ S' Pos^on ?f the sixth tubercle of His. Knowled? contribution made each individual tubercle to the full by fG *?neC* aur^c^e is still to His inexact and incomplete. According t0 Ihe^fi S^Xt^ tubercles fuse to form the the crus ^ respectively tragus, hV the the but helix? antihelix, and antitragus and lobule, Wood6/018' and instance of rnmni I-Chuan mention a remarkable /on0sa (1934) .SGnce the structures derived from the first arch, in whi c h e pinna lacked so only its tragus,8 that the arrangement vol.