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. liii. no. 9 2 k 2 502 IAN AIRD and development of the pinna from the tubercles perhaps does not conform to the original description of His. Developmental Origin of Ear-pits.?There are three main hypotheses of the method of origin of auricular fistulas. (i) The Intertubercular Hypothesis of His.?Since the time of His, have been to ear-pits generally assumed originate in the grooves between the six primary tubercula, but high modern embryological to this authority is opposed hypothesis. G. L. Streeter (1922) in a of selected classical study specimens of the developing ear, observed of His are that the tubercles shallow, rudimentary, and evanescent,

s6 DtVEUOPfHEKT QFTHt PlHNO. A.NP IT* P>T?. ( AfTEft HltV I.Tubcrculum tV*qicum ? *Tr*qu%. 2.Tuberculum ?.nfcriu? hclicil. 3.Tubtrculum intermedium heliciv 4.Tuberculum fcnttieUcil ? AntiVitlix. 5Tuberculum &ntitri<]\cum ? AnTittagut. 6.Tubercu\um lobutar* 'Lobule. HM. Hyimwiibulw clefr. OV. Otic ve?icU. tlG.Fig. 3.?Development of the auricle, after His. disappearing soon after their formation, and replaced by a one-piece primitive embryological ear ; he challenges the importance of the tubercles in pinna development, too, by citing their occurrence in reptilian and avian embryos, which do not develop an external ear. Wood-Jones and I-Chuan agree with Streeter's estimate of the un- " importance of the tubercles of His, and conclude that the hillocks are of such low elevation that it is hardly possible to conceive of a " pit or fistula being formed at their sites ; they assume therefore that ear-pits take origin from unenclosed portions of the first branchial cleft itself. (2) The Branchial Hypothesis of Vircliow.?Virchow reported one fistula of obvious branchial origin, but the track in this instance opened superficially in the region of the angle of the jaw, and subsequent writers have hesitated to include Virchow's fistula in the auricular EAR-PIT 5o3

that a branchial and pre-auricular categories. Most authorities agree and anterior helieine origin can hardly be assumed for pre-auricular from the external tracks which run downwards and forwards, separated of the meatus (first branchial cleft) by the whole thickness tragus. line of the first Ihe line of the fistula is parallel and cephalad to the the external cleft. Pre-auricular fistulae never communicate with from the meatus as they might be expected to do if they took origin which is the first cleft, and they do not lie between tragus and lobule, the branchial line of the cleft. Frederici (1930) has recently applied hypothesis to marginal helieine sinuses, but to these only. it has been (3) The Facial Cleft Hypothesis.?From time to time of fusion suggested that pre-auricular fistulas are due to partial failure this were so, between maxillary and mandibular processes, but if the facial they might be expected to be distributed more evenly along literatuie line of fusion. The only sinus which I have found in the " " 1 011 this line anterior to the pre-auricular field outlined in Fig. is a con- that described by Schwendt (1890)?a sinus surrounded by the of the genital scar lying 3 cm. posterior to, and 5 mm. below angle mouth ; this might properly be assumed to be maxillo-mandibular in origin. of an 1 hus apparently the least objectionable hypothesis is that Stated inter-tubercular origin for auricular and pre-auricular fistulas. in its widest terms, this hypothesis implies the inclusion of the groove between two tubercles when the tubercles fuse, or the inclusion of pait of the angle between the base of the tubercle and the adjacent skin. the Even if accepted, however, this hypothesis does not explain quite of a fistula considerable distance which may separate the fundus hi from its eh usually lies close to the attachment of the pinna) the cutaneous orifice. Strecter has suggested that the direction and fistula be occasionally substantial length of a pre-auricular may stretches by the growth of the mandibular process, which c^plainedthe overlying skin and carries the mouth of the fistula downwards and forwards?the usual inclination of the track. A similar upward the cranium, stretching of the skin, accompanying the growth of would explain the direction of the anterior helieine fistulas. Ihe fixation of of the fundus might be explained by a close attachment ectoder m to mesoderm at the bottom of each groove. Iheiic ^- j* scarrinerscarring of thetl-w? skinct-;~ of the face, near y a wa the orifice of at the fistula to the point of the chin, wi right to failure angles that line, has been >y - ^ The of similarly explained has adjustment between ectodermal and mesoderma .re^ wt^ which the to produced fistula has been associated with a tU ^ ^ cctodcrm , adjust its )l ' r growth to that of the underlying man "j"1 process (01* and in cranium), the overstretched ectoderm has scarre nkus n)2-j described a ^ similar congenital scarring of the skin in ^jgh.) Further, not ^ so-Called infrequently, pre-auricular fistula is associa accessory ^ auricle?the little polypoid masses of carti a^ ^placed S04 IAN AIRD portions of tragus, which may hang or protrude from the skin in front of and below the external meatus ; growth of the mandibular arch has overstretched the superjacent ectoderm and a portion of the first tuberculum has been distracted from the main mass of the tragus. Fig. 4 shows an incompletely developed auricle, in a child four years of age, which seems to cast a substantial light on the development of the auricle and on the origin of ear-pits. (An almost identical deformity is illustrated in Schwartze's text-book.) Some of the elements of the distorted pinna are difficult to name, for the external meatus may be either of two tiny pits, but these definite statements seem to be justified :?(i) the tragus and the crus helicis are absent; (2) the lobule is well-formed, and above it a well-marked eminence represents the antitragus ; (3) the antihelix ascends from the antitragus and is attached to it at its edge, but is partly separated from its main mass

FIG.Fig. 4.?Deformed and partly deficient auricle in boy of four years. Two views of a piaster cast. A cartilage graft has been inlaid in the subcutaneous tissues as a preparation for subsequent plastic reconstruction. Detailed description in text. by a deep groove ; (4) the upper end of the antihelix is free, and bent backwards on itself like a closed fist, its curved extremity enclosing a deep pit ; (5) the antihelix is separated by a deep groove from the helix ; and (6) the helix is curved on itself too, like a fresh-sprouting bean, attached by a narrow base and its curved extremity bowing towards the antihelix. The individual parts of this defective auricle might be interpreted otherwise than I have interpreted them, but it is difficult, in viewing its parts, not to read in them the third, fourth, fifth and sixth tubercles of His. The deformity, indeed, could almost be deduced from the original diagram of His. Even if some other explanation than a tubercular origin of the pinna be advanced to explain this deformity, the illustrated specimen shows that, in a deformed auricle if not in an auricle otherwise normal, pits may arise by two separate mechanisms :?(1) by failure of fusion of tubercles or other component parts of the auricle ; and (2) by folding of each component part on itself. Incipient pits are discernible " " " " in the folded helix," between helix and antihelix," and between " " " " " antihelix and antitragus ; the folded extremity of the anti- EAR-PIT 505 lelix encloses a fully-formed pit. For completeness it should be added that a flat pigmented mole, 2 mm. in diameter, lay 3 cm. in lont of the midpoint of the auricular prominence. Inheritance of Ear-pit.?Ear-pit is inherited as an incomplete ominant of variable manifestation, and without sex difference C onnon, 1941). That is, the tendency to suffer from car-pit is dominant to the tendency not to suffer from it. There are thus, in any affected amily, considerable numbers of affected individuals and the variety ~es trough many generations. Subjects who inherit the tendency o t e deformity need not always develop it in the same degree (variable ?station). The majority of the members of an affected family su^er from the lesion , ? tendency to ear-pit manifest the typical 1 ^ ^ave described. Some, however, show a more severe deformity, n a and? few will have a gross abnormality such as partial or complete the auricle ; at the other end of the scale, some affectcd ^ meSCI)ce ^ave a scarcely perceptible ear-pit, and a few will show no^d 6 f ^^ere some the factor is indeed, evidence that genetic not a one concerned in the genesis of ear-pit; McDonough (1941) un*ovu^ar twins, only one of whom suffered from ear-pit ; the Cr oth'01"^6^ norma' but his son was born with a fistul- auricles, pre-auricular non"concordance of identical twins it can be con C l^HU ri'r?m w^e the tendency to ear-pit is inherited as a dominant, C man^estat'on can hardly be due to a genetic factor (OueT^H^rU ' but must , I94?)) depend upon pre-natal environment, using the term environment in its widest sense. future of the of recorded Edmonds and ttCUI7?us inheritance ear-pit, by *s ^at *n the unilaterally affected members of any giVe f6 006 S^G' t^le or is more affectcd than the"1^' left, frequently DlirffU^GERY .0F Ear_pit and Pre-auricular Fistula.?Uncom- ear-pit does not require treatment. 0f_ complications pre-auricularpi t-dui icuiux fistulausLuia. have already been mentioned, but are so fistulas they rare that the routine excision''^.Y^ninfected should not be acute recommended. Once occlirS) any abscess should be opened. Even a ter ful drainage, however,?luwever, infection oncennro 1 ^ A ?L ~1~ fistulous established is likely to recur until the whole track is eradicated. It should be obvious remembered that infection or ulceration may be more than, and may occur at a considerable distance from, t ie mouth of the fistula which is for it. This is tiue if the responsible particularly mouth of the u 01 abscess fistula is completely blocked. Any ce 1 is formation in c for the pre-auricular area should a seal the orifice, inspire A perhaps partly occluded, of a pre-auricular fistula. recurring ulcer in front of the even by signs of ear, though unaccompanie w ic infection, may be due to of the dermal scarring may fragility is accompany a fistula, even though the orifice of the fistu a unobtrusive and distant. 506 IAN AIRD

Acknowledgements The patient suffering from the auricular deformity illustrated in this articlc was referred by Dr Charles E. Scott for plastic correction to Mr A. B. Wallace, by whose permission I have recorded it. The plaster cast of the deformed auricle whose photograph is here reproduced was prepared by Mr A. S. Cleary. I am indebted to Mr J. J. Mason Brown for permission to photograph the pre-auricular fistula in Fig. i. REFERENCES

BATTLE, W. H. (1906), Tra?is. Clin. Soc. London, 39, 248. " Bezold, A. von (1885), Schuluntersuchungen iiber das kindliche Gehororgan," Wiesbaden : J. F. Bergmann. Bland-Sutton, J. (1888), Illustrated, Medical News, 1, 320. BREUER, F. (1927), Deutsch. Zeitschr.f. Chir., 202, 278. CALDERA, C. (1922), Arch. ital. di otol., 33, 55. Congdon, E. D., Rowmanavongse, S., and VARAMISARA, R. (1932), Atner. Journ. Anat., 51, 439. CONNON, F. E. (1941)) Journ. Heredity, 32, 413. DYER, E. (1885), Tran. Amer. Otol. Soc., p. 468. Edmonds, H. W., and Keeler, C. E. (1940), Journ. Heredity, 31, 507. EVANS, J. H. (1910), Brit. Journ. Child. Dis., 7, 490. " Eyle, P. (1891), Ueber Bildungsanomalien der Ohrmuschel," Zurich. Fischer, H. (1903), Arch. klin. Chir., 69, 263. FOUCAR, H. O. (1940), Canad. Med. Assoc. Journ., 43, 26. Fournier, G. (1919), Rev. de laryng., 40, 145. FREDERICI, F. (1930), Acta oto-largyngol., 14, 532. Gradenego, G. (1893), Arch. f. Ohrenheilk., 34, 218. Grunert, C. (1898), ibid., 45, 10. HADDA, S. (1909), Berl. klin. IVoch., 46, 711. " Hahne, A. (1889), Ueber fistula auris congenita," Gottingen : W. F. Kaestner. HEUSINGER, C. F. (1864), Virchows Arc/iiv. f. path. Anat. u.s.w., 29, 358. His, W. (d.e.) (1885), Co7npt. rend. IHme. congres internat. d'otol., Basle, p. 149. JENKS, A. E. (1916), Journ. Heredity, 7, 553. KEITH, A. (1909), Brit. Med. Journ., 2, 438. Koenig, H. (1895), Arch. klin. Chir., 51, 518. McDonough, E. S. (1941), Journ. Heredity, 32, 169. " Marx, H.(i926), Henke-Lubarsch: Handbuch der spez. path. Anat. u. Hist.," 12,632. " " Moldenhauer (1892), Die Missbildungen des menschlichcn Ohres ; im Hand- buch d. Ohrenheilkund. von H. Schwartze, 1, 154. Onodi, L. (1918), Archiv.f. Ohren-, Nasen-, u. Kehlkopfheilk., 102, 128. Paget, J. (1878), Med. and Surg. Trans., 61, 41. " PlNKUS, F. (1927), in Jadassohn's Handbuch d. Haut- u. Gcschlechtskrankh." QUELPRUD, T. (1940), Journ. Heredity, 31, 379. RUGANI, L. (1910), Arch, inter nat. de laryugol., 29, 119. SABRAZES, I. (1890), Journ. de med. de Bordeaux, 20, 483. SCHULLER J (1929), Miinchn. med. Woch., 76, 160. SCHWABACH, D. (1879), Zeitschr.f. Ohrenheilk., 8, 103. " Schwartze, H. (1886), Die Chirurgischcn Krank. d. Ohres," p. 06. SCHWENDT, A. (1890), Archiv.f. Ohren-, Nasen-, u. Kehlkopfheilk., 32, 37. STAMMERS, F. A. R. (1926), Brit. Journ. Surg., 14, 359. STEINER, A. (1925), Derm. Woch., 86, 325. STREETER, G. L. (1922), Carnegie Institute Contributions to Embryology, No. 1O9. THOLLON and Labernadie (1919), Rev. de laryugol., 40, 129. Urbantschitsch, V. (1877), Monatschr. f. Ohrenheilk., 11, 85 (translation Edin. Med. Journ., 1877-78, 23, 690). Virchow, R. (1864), Virchows Archiv. f. path. Anat. u.s.w., 30, 221. Wood-Jones, F., and I-Chuan, W. (1934), Journ. Anat., 68, 525. ear-pit 5075

Discussion Dr Traquair referred to the fact that m there was one was c this condition deformity, there usually ^^"^ereas' of the ear was an isolated defonnity. He \vas stehearing appeared to be of secon ary

Where - the meatus was too narrow to allow ^?am}nation of the ear, X-ray might be helpful in deciding whether the nu internal ears were developed. These patients may show complete ab e^ external ear, or from vary one extreme to the other?where no externa ^ present to where the external ear is developed and there may e "essory auricles or nodules. Dr Martin referred to the faet that it was difficult to, be? ' condition was always developmental and not the resu o ^ ? or an Childhood, infection of the auricle. In adults; the presence the ear often very gave rise to a condition almost 1 entica wi ofa^ his """in opinion the condition was fairly common but rarely recognised as a has ft been that ear defects are much on suggested congenital the side and right also familial. A fair percentage o peop The otologist was interested, not so much in the actual because that could be with or e put right plastic surgery prosde^1_tyb?^t^ , presence or absence of hearing, and over 50 per cent, o e cas jtaj malformation of the ear had also labyrinthine malformation. Dr Farguharson, referring to the treatment of the condition, the cauterising opening with the cautery was not satisfactory. said^ ^ to a tendency recurrence of the condition. ow ^ quite Diathermy, , good results and was easy of application. Mr Aird, in reply, said?There is no difference in the sex ear-pit; inheritance is not sex-linked. I was interested m r \ experience that artificially-produced ear-pits may close y resem cQn_ genital varieties. Recent observers have not noticed a prepon er on the side . right ; perhaps the earlier German authors drew or ei on a population which included large affected families wit a t :