The congenital fissures and deformities of the mouth and lips form a group which is considerably larger than might be imagined from the scanty notice given them in ordinary text-books; and although many are extremely rare, yet possibly if more attention were drawn to them, fresh cases would be noted and recorded, and the somewhat scanty materials from which we have to work out their development and characteristics would be increased. In order to facilitate subsequent description, I append a classified list of the deformities which we shall pass under notice, premising that the more practical part of this work will be occupied exclusively with two of them.

Six different classes may be described:—

(1) Median harelip (inter-intermaxillary).

(2) Ordinary harelip (intermaxillary).

(3) Facial cleft (maxillo-intermaxillary).

(4) Buccal cleft, or macrostoma (maxillo-mandibular).

(5) Mandibular cleft, or median fissure of the lower lip.

(6) Cleft palate.

Inasmuch as ordinary harelip and cleft palate are the conditions most commonly met with, it will be convenient to describe them first, alluding subsequently to the others.

Harelip.

French, bec-de-lièvre. German, Hasenscharte—or if with complete cleft palate, Wolfsrachen (wolf-jaw).

Harelip is a congenital deformity of the upper lip, characterised by a cleft extending for a variable depth, either through the soft tissues of the lip only, or implicating in addition the alveolus, floor of the nose, and palate. No mention of this condition is made by Hippocrates, Galen, or any of the fathers of medicine; and so far as I can discover the name is first used by Ambrose Paré, who probably initiated the treatment by pin and figure-of-8 suture. The name is really a misnomer, in that the condition (as has been many times pointed out, but notably by Fergusson) does not simulate a hare’s lip except in the fact of being cleft, for the natural cleft in the animal’s lip is always in the median line below, bifurcating above to reach either nostril (Fig. 1), whereas in the abnormal human lip the cleft lies to one or the other side. Instances of median defect are known, but they are extremely uncommon, and consist often of more than a simple fissure.

The deformity may exist as a simple notch in the soft tissues of the lip, unilateral (Figs. 2 and 3) or bilateral (Fig. 4); when more decided, it may implicate one or both nostrils (Figs. 5, 6, and 7). In mild cases the alveolus is intact; in others, cleft, constituting the variety known as alveolar harelip, and the line of fissure may, or may not, extend backwards into the palate. In all cases of double alveolar cleft, the palate is also involved, and the central parts of the lip and intermaxilla tend to project forwards; in the severest forms these portions are completely isolated from the maxillæ, and, supported by the vomer and septum nasi, form a proboscis-like appendage to the end of the nose, which is excessively disfiguring. (Figs. 7 and 8 illustrate this deformity as seen from the front and in profile.)

The shape of the nose in unilateral harelip is very characteristic, being broad and flattened out from the deficiency of the floor and posterior wall of the anterior nares.

Harelip seems to occur more commonly in boys than in girls. According to Müller, out of 270 cases, 170 were boys, and 100 girls.

Unilateral harelip is more commonly met with on the left side than on the right; probably 60-70 per cent. of the cases are left-sided. Thus Müller reports 142 left-sided against 62 right-sided clefts; Mason, out of 65 cases, found 54 to be unilateral, and of these 35 left-sided to 19 on the right; Kölliker mentions that in 165 unilateral clefts, 113 were on the left side, and 62 on the right. My own experience quite coincides with these figures. At present, no satisfactory explanation of this preponderance of left-sided clefts has been given. One solution suggests itself, but we have no facts of importance to support it, viz. that, inasmuch as the majority of people are from heredity or education right-handed, Nature devotes more energy to completing her developmental processes on that side than on the left, and any check to this would be more likely to happen on the left side. It would be valuable and interesting to know in what proportions other unilateral deformities occur on the left and right sides respectively.

Occasionally one sees in the upper lips of children a congenital red line apparently cicatricial, occupying the position of the normal harelip fissure, and which has been supposed to indicate a natural cure of a temporary defect of development. My colleague, Mr. Carless, has recently shown me a case of this character under his care in a child a few weeks old. There was a well-marked red line extending from the lip margin to the nostril; but there was no irregularity in the red border, and no evidence of cicatricial contraction; the tissue of the lip, moreover, seemed quite soft and normal, not fibrous or hard. These points seem to bear out fully Trendelenburg’s opinion[1] that the name “intra-uterine cicatrisation or cure of a harelip” is incorrect, and that such cases are simply due to the raphe of union remaining evident instead of disappearing as usual; and he quotes the normal appearance of the raphes in the scrotum and perinæum as similar conditions. In this child there was no evidence of any groove or depression in the alveolus; but other deformities were present, viz. a very definite post-anal dimple, the cicatrix being adherent to the tip of the coccyx, a slight condition of hypospadias, and a congenital hydrocele. There was no history of deformity in the family, nor of maternal impression.

Cleft Palate.

This is a congenital deformity due to non-closure of the horizontal palatine outgrowths extending inwards from the maxillary processes. The name must not be applied to acquired fissures or defects of the palate due to injury or to disease of the bones later in life. (See Chap. IX.)

As with harelip, so with cleft palate, the extent of the defect varies greatly in different cases. Thus in the most severe forms, there is a total mesial longitudinal cleft, extending forwards from the tip of the uvula to the level of the anterior palatine canal, thence bifurcating to communicate anteriorly with a double alveolar harelip, the os incisivum or central portions of the intermaxilla being usually displaced forward (Fig. 9). Such a condition is known by German authors as “Wolfsrachen,” or wolf-jaw. The vomer descends in the median line usually into close quarters with, but separate from the margins of the cleft, and the os incisivum is attached to its anterior extremity. When the vomer comes far down and is well developed and prominent, and the palatal outgrowths small, the cleft appears to be double, but is not so in reality (Figs. 9 and 10).

Not unfrequently the vomer is attached to one of the margins of the cleft, this condition being usually associated with unilateral alveolar harelip. Such attachment always occurs on the side opposite to the fissure in the alveolus; that is to say, since unilateral harelip is more common on the left, the vomer is usually attached to the right side of the cleft. Fig. 11 indicates the less common condition of attachment of the vomer to the left palatal segment. Rouge[2] and Oakley Coles[3] fully confirm this statement. The cleft may, however, merely implicate the soft and hard palate, leaving the alveolus and lip perfect, and does not then extend further forward than the site of the anterior palatine canal, and is strictly median (Fig. 12); or it may be still more limited, involving more or less of the velum, perhaps only the uvula, or extending a variable distance into the hard palate (Figs. 13 and 14).

Other less common congenital deformities have been recorded, and amongst them may be noted a case lately seen by myself in a girl of four years, in whom there existed an oval opening at the junction of the hard and soft palate, separated by a narrow bridge of normal palatal tissue from a cleft of the posterior half of the velum and uvula, showing intermissions of development; a congenital aperture in the soft palate at its junction with the hard, or in any part of the velum, but with no defect of either uvula or palate bones (Dieffenbach[4]); a defective development of the palate bones alone, the mucous membrane remaining intact from side to side, and hence no cleft resulting (Trélat, Notta, Langenbeck); or again, as in Fig. 15, a cleft only of the anterior portion of the palate, extending through the alveolus, and for a short distance behind it (Mason[5]). Inasmuch as the union of the two halves of the velum occurs subsequently to that of the alveolar arch, it appears that this last rare defect must have been due to an intermission of development, which was felt only at the anterior portion, whilst that of the posterior part proceeded normally at a later date.

Mason[6] records a curious case worth mentioning of a girl under his care in 1877, who had a fissure extending through the velum, and for a short distance into the hard palate, but there was no trace of uvula on either side, and the soft palate was continuous on both sides with the pharyngeal wall.

The width of the cleft varies as much as the extent, and is a matter of great importance prognostically, as the broader clefts are much more difficult to close. The direction or slope of the segments of the bony palate also differs considerably, in some instances being more or less horizontal and following the normal curve; in others one or both of the segments is much more nearly vertical, a condition which is not at all unsatisfactory, for, as will be explained hereafter, the more horizontal the palatal processes, the more difficult is it to gain satisfactory closure by operation (p. 65).

The frequency of the occurrence of harelip and cleft palate cannot accurately be ascertained, inasmuch as statistics are not readily to be found. In the ‘St. Thomas’s Hospital Reports’ the number of malformations of the children born is noted in some of the years. Thus the aggregate number of living children born in their maternity department in the years 1875, 1877-1880, and 1883 was 10,653, and of this number there was only one case of harelip, with two cases of cleft palate, and three of the combined deformity, i. e. about one case in every 1800 infants born; but if the silence of the reports for subsequent years means absence of deformity, then this proportion may be much too great.

On the Continent some old records are obtainable. Thus, according to Grenser, of 14,466 infants born living at the Maternity at Dresden from 1816 to 1864 there were sixteen cases of simple harelip, and nine with fissures of the palate. Credé states that amongst 2044 infants examined at birth, only one case of simple harelip was observed, and one of complete division of the hard and soft palate.

Occurrence in Animals.

These conditions obtain not only in the human subject, but also in animals, though not so commonly.

Thus Sutton figures a right-sided harelip in a slink calf, and mentions a specimen of a harelip in a lamb in the museum of the Odontological Society; and in our museum at King’s College there is a specimen of a right-sided harelip in a kitten with a cleft alveolus, but the palate is intact.

Cleft palate occurs more frequently in animals, particularly in those born in a state of captivity. Thus it appears that from statistics taken ten years ago 99 per cent. of the lion cubs born in the London Zoological Gardens had cleft palates, indicating that either the food-supply of these animals was not all that was requisite for perfect development, or that enforced confinement has a deleterious effect upon the multiplication of the species. It is a curious fact that in the Dublin Zoological Gardens the deformity was rarely noticed amongst the lion cubs, and the reason for this was supposed to be the supply of such food that the mother could eat both flesh and bone. Since the same practice has been followed in London, viz. giving the lions twice a week a young goat which they can eat, bones and all, the proportion of cleft palates in the young subsequently born has become considerably diminished.

Association with other Deformities.

Occasionally, besides the fissured palate or lip, other deformities are noted in the same patient, but not so often as one might be led to expect. Mason records two or three cases as having come under his notice, the coincident deformities being respectively fistulous openings of buccal glands in an everted lower lip, congenital fissure of the lobe of the right ear, congenital talipes calcaneus and hypospadias. Dr. F. Warner records in the ‘Medical Times and Gazette,’ January, 1882, some cases of cleft palate associated with congenital defects of the heart and smallness of head, and also notes in his more recent report[7] that in 117 cases of malformations of the palate, other than cleft, only 42 were not in combination with other defects. Thus in 55 cases there were abnormalities in the shape of the cranium, in 16 cases defective development of the ear, in 12 the existence of an epicanthic fold, and in 15 cases other defects not tabulated.

Clutton[8] records and pictures a curious development of a flap of mucous membrane on the lower lip of a woman with a cleft palate; it was triangular in shape, and with overhanging projecting angles. The teeth in this case were likewise badly developed, and were all extracted at the age of nineteen.

Binet[9] reports a case of an old cured right-sided harelip in a man dead from apoplexy (æt. 53 years) with infantile genital organs.

Broca[10] describes a much deformed fœtus, stillborn at seven months, which he dissected, showing a double harelip and cleft palate, but the os incisivum retained its usual position, thanks to its mucous covering. The dentition,[11] as noticed elsewhere, was also interesting, and the buccal deformity was associated with a congenital diaphragmatic hernia, and an abnormal condition of the heart and great vessels.

Other associated malformations are on record, e. g. an extra thumb on each hand; and Sir Morell Mackenzie has reported a case in which there was a congenital fissure between the arytænoid cartilages with a trilobate epiglottis, occurring in conjunction with harelip and cleft palate.[12]

The remaining deformities to be noticed here are much less common, but demand attention by their rarity and interest, and on account of the light they throw on the embryology of the lip and mouth.

Median Harelip.

This is an exceedingly rare phenomenon, and for long the possibility of the existence of such a condition was doubted. Supposed cases were explained by imagining that from some unknown cause a lateral fissure had been drawn over to the median line. But at the present time there are records of several, mainly, however, in German works; in our own literature there are but few references to the subject. Two varieties of median defect have been described; and the distinction between these has been carefully and thoroughly made by Trendelenburg.[13]

1. Double cleft of the upper lip with failure of development of the intermaxilla. Some half-dozen cases of this are indicated in his work; but perhaps one of the best descriptions is that recently given by Bland Sutton,[14] and from whose paper the accompanying picture is obtained (Fig. 16). It occurred in the practice of Mr. Treves, and died within a few weeks of birth. There was a broad median defect, flanked laterally by the curved convex borders of the maxillary processes; the intermaxillæ were entirely absent, and the nose quite flat. In addition to this the eyes were affected with coloboma, the right eye presenting other serious defects. “On examining the child before its death,” the author states, “I felt convinced that there was no ethmo-vomerine plate, and this conviction was strengthened by the peculiar shape of its forehead. When the child died, this opinion was fully confirmed; there was no ethmo-vomerine plate, consequently no nasal septum, and what is more important, the premaxillary bones were absent.”

But according to the cases reported by Trendelenburg the defects do not stop here. There is usually in addition a broad median palatal cleft, and an absence of nasal bones and muscles; but Kundrat records two cases where the palatal processes of the superior maxillæ and palate bones were well developed, and united in the middle line. The skull itself has been found defective occasionally, the whole cranial portion being small, and the lamina cribrosa and crista galli of the ethmoid absent; in place of these was a fossa between the orbital plates of the frontal bone with no bony basis, but only dura mater covered with mucous membrane. No openings for the passage of the olfactory nerves were found.

Hadlich has also described changes in the brain in two cases occurring in Langenbeck’s clinique, consisting mainly in the amalgamation, more or less, of the two hemispheres; the corpora striata and optic thalami were united in the middle line, and the third ventricle, fornix, corpus callosum and olfactory nerves were absent. It is interesting to note the association of such an abnormal fusion of the lateral parts of the brain in the median line with the defective development of the median parts in the skull and face.

But the separation of the facial elements is not always maintained; sometimes they fall or are drawn together by the united palate, resulting in the so-called congenital atresia of the nose (“angeborene Atresia der Choanen”), cases of which have been recorded by Luschka, Bitot and Engel. The latter states that in an infant’s skull examined, only 2 or 3 mm. of space existed between the orbits, and 4 mm. between the optic foramina.

2. True median cleft of the upper lip with development of the intermaxilla is an excessively rare occurrence, but a few cases have been now recorded.

The simplest type consists of a cleft in the soft portions of the upper lip with no other deformity, but a more complete variety of the defect includes a median division of the nose.

Mr. Pitts, in the Medical Society’s ‘Proceedings’ (vol. xii, p. 304), reported a case in a boy aged five months (Fig. 17). The cleft was median, extending halfway up to the columna. The premaxilla was centrally grooved but otherwise perfect. The palate was normal.

A more aggravated condition has been dissected by Witzel (in the Rostock Collection). Behind the cleft in the upper lip was found a median division of the premaxilla, each half of which was firmly united to the adjacent superior maxilla. The vomer was single, but broader than usual, and the palate cleft throughout; the two halves of the nose were bounded internally by separated plates of the divided cartilaginous nasal septum. There was also a defect of the frontal bone giving rise to a meningocele. This flattening of the nose, combined with separation of the anterior nares, gave such an appearance to the face as seemed to warrant the term “dog’s nose” (Doggennase) which has been applied to it.[15]

Facial Clefts (German, “Schräge Gesichtsspalte”).

These are seldom seen, but a sufficient number are now recorded and figured to enable us to study the nature of the defect. Sir W. Fergusson seems the only English surgeon who has observed this rare condition, the majority of recorded cases hailing from Germany or France. As we shall see hereafter, this defect is due to the non-closure of the cleft between the outermost part of the intermaxilla and the maxilla itself, and occupies the position which was claimed up to recent years as that of an ordinary harelip.

In several of the cases noted red cicatrices (Fig. 18) rather than actual clefts (Figs. 19 and 20) were present. The defect begins at the free margin of the upper lip, and usually at the spot whence starts the ordinary harelip cleft; but occasionally from the angle of the mouth. It then trends upwards and outwards, leaving the nose entire, and skirts round the ala nasi to reach its upper limit at the middle of the lower eyelid which is cleft, or at the inner canthus. The eye itself may show a coloboma iridis, usually downwards and inwards. The facial skeleton may be divided or not; sometimes a large opening into the antrum exists (Hasellmann,[16] Kraske[17]). No incisor teeth are developed on the outer side of the cleft, the first tooth seen being the canine. On the inner border of the cleft lip there is usually a marked frænulum, often smaller, however, than the normal median frænum.

This deformity may be unilateral or bilateral (Guersant, Meckel), more frequently the former; and is not uncommonly associated with macrostoma of the same or opposite side of the face (Guersant, Pelvet[18]), as seen in Fig. 20.

Albrecht[19] records a most interesting case in a newly born pup (Fig. 21) of double clefts extending from the lip margin upwards not only into the nostril, but also towards the eye on either side, i. e. a double associated harelip and facial cleft. The specimen is taken from the Royal Veterinary College of Brussels.

Macrostoma

Or commissural harelip (French, bec-de-lièvre genien; German, Quere Gesichtsspalte, Wangenspalte, or Grossmaul) is a less uncommon condition, evidenced by an increased transverse diameter of the mouth. The oral aperture extends into one or both cheeks, and, if unilateral, is more frequently on the right side. The cleft extends upwards and backwards towards the auditory meatus, and sometimes towards the temple to a variable extent. It may merely be manifested by a slight increase in the breadth of the mouth, or may extend to a considerable distance, as in a case reported by Rynd,[20] where the mouth-opening extended as far as the first molar on the right side, and to the last molar on the left. Sutton[21] has published drawings of a child (Fig. 22) in which a very large cleft existed, the angles of which gradually passed into a red cicatrix. This scar ended in a gaping recent wound over the temporal region, extending to the dura mater, and through this, after death, the convolutions of the brain were visible. The condition was symmetrical, and he suggests that the wound in the skull was probably brought about during parturition. The same author records a condition the very opposite of this, where the defective closure of maxillary and mandibular processes was reduced to a minimum, the deformity amounting to nothing more than a fistulous opening through the cheek, with a small tumour representing an accessory auricle just in front of the tragus.

Roulland[22] has recently reported an instructive case in which double macrostoma existed with accessory auricular appendages, but this was also complicated with an entire absence of the middle ear and of the Eustachian tube, with defective development and absence of the temporo-maxillary joint on the left side. Such a deformity is probably to be explained by an excessive obliteration or partial development of the maxillo-mandibular cleft at its posterior extremity, and a defective obliteration of the same anteriorly.

Associated with macrostoma is often to be noticed some abnormal condition of the external ear, either defective development or the production of accessory auricles (Figs. 23 and 24). In a case of bilateral macrostoma recently under my own care, there was a well-marked accessory auricle. This complication was first pointed out by M. Debout.

One or two observers (Morgan, Colson[23]) have noticed a small papillary projection on the red margin of the cleft, indicating the position where the true mouth ended, and due to the insertion thereat of the divided orbicularis oris.

For long the very existence of this macrostomatous deformity was doubted, but cases have been recognised more or less since 1715, when Muralt pictured it for the first time. A résumé of all the earlier cases has been made by M. Debout,[24] whilst Roulland[25] and Pilz[26] have gathered together some of the later.

Macrostoma is not only attended by great disfigurement, but is also troublesome from the impossibility of the child retaining its saliva, and the food escaping during mastication. Suckling can be performed if the nurse’s nipple be long, but is difficult otherwise. This deformity is, perhaps, more frequently associated with defective cerebral power than any other of the facial clefts, a large proportion of the subjects having been idiots.

Mandibular Cleft.

This condition is one of the rarest that we have had to describe, so much so that Roux and Cruveilhier denied its existence, and Fergusson had seen but one case. Bouisson[27] in 1840 mentions some three or four earlier cases, and records one that he had seen post mortem himself. Since that date some six or eight instances have been noted, and the latest, with some excellent pictures, is described by Wölfler[28] (Fig. 25).

The cleft extends in different cases to a variable extent. Thus Nicati, Couronue, F. Petit, and Ammon saw clefts implicating the lower lip alone. Ribell[29] operated on a cleft extending to the chin, through which the saliva was continuously dribbling. Faucon (1868) and Lannelongue (1879) recorded clefts of the lip and mandible conjoined, and in both cystic swellings (presumably of the dermoid type) were found between the segments. Parisé’s (1862)[30] and Wölfler’s cases were also associated with cleft of the tongue, through its whole thickness in the former, and only at its tip in the latter.

In Parisé’s case the child was fourteen days old. The lower lip was cleft through its whole thickness in the median line. The free edges were rounded as in harelip, and the cleft was continued below as a cicatricial band in the middle line of the neck as far as the sternal notch. The mandible was in two portions, which were separated from one another by a distance of two or three millimetres, bridged across by connective tissue. The tongue was entirely divided, the cleft extending back to the glosso-epiglottic ligament, and downwards between the genio-hyo-glossi muscles; each half was covered throughout with mucous membrane, and was bound to the corresponding side of the jaw by a mucous ligament or frænulum.

As to the ætiology of these defects, but little is known.

Heredity is an undoubted factor in their production, and an investigation of the family history will in many cases elicit a confirmation of such an idea. Thus in two instances in my own practice I have been able to determine that the father, grandmother, and great-grandfather had all suffered from harelip to a greater or less extent. Mason in his book mentions several other illustrations of this fact. Liston operated on four members of one family for harelip. M. Demarquay[31] related a case in the Surgical Society of Paris, in which, from the grandparents downwards, eleven children had been born with harelip. In the ‘British Medical Journal’[32] a correspondent related his own family history, stating that it had occurred in some branch or other for the past hundred years.

An examination of the parents’ mouths should always be made when possible, and very commonly it will be found that one or both possess a short upper lip, and a high arched narrow palate. In others there is a slight groove in the alveolar process between the central and lateral incisors. I have also observed a small symmetrical crease on either side of the median line in the upper lip, indicating a tendency to, if not a natural intra-uterine cure of, a double harelip.

In some instances the deformity dies out of families, possibly from the fact that the defective condition in one parent is remedied by a more perfect development in the other; whilst in others the tendency distinctly increases, and a father or a mother with harelip will beget a family where three out of the four or five children will be similarly affected. By a proper selection of mates this deformity could probably be bred out, as well as bred up to.

The so-called Maternal Impression is looked on, especially by the laity, as another common cause of these deformities. Medical men will usually receive histories of such with a smile of incredulity, and rightly so; but some recorded cases, if true, are so definite that to condemn such an explanation too dogmatically seems scarcely to indicate a scientific spirit. The usual type of history given is that after the mother has seen the defect in the newly-born infant, she looks back over the preceding nine months to see if there were any apparent cause for the trouble, and seeking out particularly some shock or fright produced by seeing something resembling the defect in her infant often selects something trivial and irrelevant. The following authentic case is worthy of mention:[33]

A child was born deformed by a left unilateral harelip. The mother immediately asked to see the infant, declaring she was afraid it was marked, and on seeing it manifested no surprise at the appearance of its lip, stating that when about four months pregnant she received a fright, from the shock of which she had not yet fully recovered. Startled by a boy running almost into her arms, from whose face blood was streaming, she had seen a cut in the left side of the upper lip, extending through its substance into the nostril, laying bare the gums and teeth. She turned faint with fright, and could not banish the thought even after reaching home. The lad was subsequently examined, and the scar of a cut was found in that position.

In spite of such facts, however, one hesitates somewhat in accepting the antecedent alarm and the subsequent deformity in the relationship of cause and effect. The imaginary “maternal impression” probably in nine cases out of ten has nothing to do with the defect; whilst a real “maternal shock” which possibly led to the production of the deformity passes unnoticed. Mr. Carless tells me of a case recently seen by him of a cleft of the soft palate in a child, whose mother, without asking any leading questions, gave a history of a sharp attack of febrile disturbance keeping her in bed two or three weeks at a period when the fœtus could not have been more than two months old. This is the type of maternal shock we should possibly look for, rather than the more out-of-the-way maternal impressions commonly suggested.

The union of the parts entering into the formation of the palate, alveolus, and lip is normally completed by the eighth to the tenth week, and when once this has occurred in these parts no maternal impression (such as seeing a gashed lip) could, as far as we know, bring about a retrogressive change. Should some shock occur to the mother prior to that period, we can fully appreciate the possibility of its interfering with the typical growth of the parts then being produced; and the fact that the due adjustment and union of so many component parts is requisite for the normal development of the mouth and face explains why these defects are relatively so common. That a severe shock to an infant may produce coincidently a lamellar cataract and defective development of dentine is well recognised; that a similar type of shock acting on the mother should result in defective union of parts developing at that period in the fœtus is not strange; but that the real shock and the so-called “Maternal Impression” are one and the same is more than doubtful.