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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 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 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 reports a case of an old cured right-sided harelip in a man dead from apoplexy with infantile genital organs.

Broca describes a much deformed foetus, stillborn at seven months, which he dissected, showing a double harelip and cleft palate, but the os incisivum retained e is something ludicrous in being the hermit of a show-place, unlike St. Francis in his mountain-bed, where none but the stars and rising sun ever saw him.

There is also a "guide to the falls," who wears his title labelled on his hat; otherwise, indeed, one might as soon think of asking for a gentleman usher to point out the moon. Yet why should we wonder at such, when we have Commentaries on Shakespeare, and Harmonies of the Gospels?

I will here add a brief narrative of the experience of another, as being much better than anything I could write, because more simple and individual.

"Now that I have left this 'Earth-wonder,' and the emotions it excited are past, it seems not so much like profanation to analyze my feelings, to recall minutely and accurately the effect of this manifestation of the Eternal. But one should go to such a scene prepared to yield entirely to its influences, to forget one's little self and one's little mind. To see a miserable worm creep to the brink of this falling world of waters, and watch the trembling of its own petty bosom, and fancy that this is made alone to act upon him excites--derision? No,--pity."

As I rode up to the neighborhood of the falls, a solemn awe imperceptibly stole over me, and the deep sound of the ever-hurrying rapids prepared my mind for the lofty emotions to be experienced. When I reached the hotel, I felt a strange indifference about seeing the aspiration of my life's hopes. I lounged about the rooms, read the stage-bills upon the walls, looked over the register, and, finding the name of an acquaintance, sent to see if he was still there. What this hesitation arose from, I know not; perhaps it was a feeling of my unworthiness to enter this temple which nature has erected to its God.

At last, slowly and thoughtfully I walked down to the bridge leading to Goat Island, and when I stood upon this frail support, and saw a quarter of a mile of tumbling, rushing rapids, and heard their everlasting roar, my emotions overpowered me, a choking sensation rose to my throat, a thrill rushed through my veins, "my blood ran rippling to my fingers' ends." This was the climax of the effect which the falls produced upon me,--neither the American nor the British fall moved me as did these rapids. For the magnificence, the sublimity of the latter, I was prepared by descriptions and by paintings. When I arrived in sight of them I merely felt, "Ah, yes! here is the fall, just as I have seen it in a picture." When I arrived at the Terrapin Bridge, I expected to be overwhelmed, to retire trembling from this giddy eminence, and gaze with unlimited wonder and awe upon the immense mass rolling on and on; but, somehow or other, I thought only of comparing the effect on my mind with what I had read and heard. I looked for a short time, and then, with almost a feeling of disappointment, turned to go to the other points of view, to see if I was not mistaken in not feeling any surpassing emotion at this sight. But from the foot of Biddle's Stairs, and the middle of the river, and from below the Table Rock, it was still "barren, barren all."

Provoked with my stupidity in feeling most moved in the wrong place, I turned away to the hotel, determined to set off for Buffalo that afternoon. But the stage did not go, and, after nightfall, as there was a splendid moon, I went down to the bridge, and leaned over the parapet, where the boiling rapids came down in their might. It was grand, and it was also gorgeous; the yellow rays of the moon made the brokect. The palate was normal.

A more aggravated condition has been dissected by Witzel . 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" which has been applied to it.

FACIAL CLEFTS .

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 rather than actual clefts 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 . 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 fraenulum, often smaller, however, than the normal median fraenum.

This deformity may be unilateral or bilateral , more frequently the former; and is not uncommonly associated with macrostoma of the same or opposite side of the face , as seen in Fig. 20.

MACROSTOMA

Roulland 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 . 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 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.

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.

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 operated on a cleft extending to the chin, through which the saliva was continuously dribbling. Faucon and Lannelongue recorded clefts of the lip and mandible conjoined, and in both cystic swellings were found between the segments. Paris?'s and W?lfler'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 Paris?'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 fraenulum.

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.

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 foetus 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 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 foetus is not strange; but that the real shock and the so-called "Maternal Impression" are one and the same is more than doubtful.

ANATOMY AND PHYSIOLOGY OF THE NORMAL PALATE.

The palate is a more or less horizontal partition dividing the month from the nasal cavity, and consists of a firm bony plate in front with a freely moveable membrano-muscular velum behind , which under varying conditions of muscular action can either open or close the communication between the nose and pharynx.

The bony surface of the roof of the mouth is perforated by numerous small foramina for the transmission of the nutrient vessels to the body of the bone, pitted for the lodgment of mucous glands, and grooved longitudinally for the transit of vessels. At the postero-external corners the posterior and accessory palatine canals give entrance to the posterior palatine vessels, and nerves; and anteriorly in the median line is the anterior palatine canal transmitting the naso-palatine vessels and nerves.

The soft palate is a moveable curtain, consisting of a membranous expansion or aponeurosis attached to the posterior extremity of the hard palate by firm fibrous tissue. Incorporated with it are five pairs of muscles, controlling its movements; it is covered by a smooth thin mucous membrane, and terminates posteriorly in the uvula. The arrangement of these muscles is important, not only from their normal physiological functions, but also from their irregular action and effects in cases of cleft palate . They may be arranged in groups: two, the levator and tensor palati, form a superior group; the azygos uvulae is intermediate; and the palato-glossus and palato-pharyngeus form an inferior set. Arising from the extremity of the petrous bone, the levator passes downwards, and spreading out below unites with its fellow in the whole length of the median raphe. The tensor arises from the navicular fossa of the internal pterygoid plate, and after being reflected around the hamular process, its action there being assisted by the interposition of a bursa, is attached to the anterior portion of the aponeurosis and to the hinder part of the bony palate. The combined action of these muscles raises and makes tense the velum, and in addition influences the Eustachian tube; but the levator is by far the more important. The azygoi uvulae muscles arising from the median raphe and spine of the hard palate descend to the tip of that process, and are thus able to regulate its length.

The two descending muscles are placed in the pillars of the fauces, forming the lateral prolongations of the velum, and the tonsils lie in a recess between them. The palato-glossi arising from the tongue ascend in the anterior pillars of the fauces, and spreading out on the anterior surface of the velum unite in the median raphe. The palato-pharyngei start from the median raphe in two lamellae enclosing the termination of the levator muscle; they descend in the posterior pillars of the fauces, and being attached to the pharyngeal wall between the superior and middle constrictors, by their contraction assist in raising the pharynx during deglutition.

The nervous supply of these muscles requires little notice here; suffice it that the superior set and the azygos are supplied by the facial nerve, the inferior set from the pharyngeal plexus.

The normal shape of the palate is a regular arch, bounded laterally by the gums and alveoli into which the teeth are implanted so as to describe a parabolic curve, being normally uninterrupted at any spot by spaces or diastemata. The height and curvature of the palate vary considerably in different individuals, not only from inherited peculiarities, but also from acquired conditions dependent on the teeth. A person with a good set of sound teeth will probably own a regular well-formed palate; whilst if sundry of the upper permanent teeth are lost during the stage of adolescence, the palate is likely to become high and narrow from the falling in of the jaw. This is especially the case if the incisor teeth are lost.

The shape of the palate in a child of two years does not differ so markedly as one would at first expect from that of an adult except in length, and the reason for this is plainly the existence in the latter of three additional teeth on each side. Its increase in length is from 20 to 30 millimetres, whilst its breadth is only augmented by 10 to 15 mm., and this mainly posteriorly. When once the permanent incisors, canines, and premolars are developed, the anterior portion of the palate alters but little in shape, unless any of these teeth be lost, and the gaps not artificially maintained.

Dr. Ehrmann states that the alveolar border in front of the canine teeth forms a nearly regular semicircle, with a posterior transverse diameter of 22-26 mm.; thence the alveoli diverge regularly, adding to the diameter about 2-4 mm. for each tooth. He gives the following measurements as the mean of many observations:

Oakley Coles has carefully investigated the size of the palate in several series of skulls in the Museum of the College of Surgeons, and gives the results as follows:

Of 34 adult skulls of European origin, the average length was 49 mm., the average width at the second bicuspid was 35 mm., and the average height from the margins of the alveoli 9 mm.

Of 32 adult skulls of mixed races, the average length was 54 mm., the width 35 mm., and the height 12 mm.

Dr. Langdon Down had previously noticed and pointed out this frequent relationship, remarking that as the result of a large number of careful measurements of the mouths of the congenitally feeble-minded and of intelligent persons of the same age, he found with few exceptions a marked diminution in the transverse measurement between the posterior bicuspids, resulting in an inordinate vaulting of the palate. There was often noticed an actual deficiency in the bony structures of the posterior part of the hard palate, causing the velum to hang down abnormally, interfering with phonation.

The functions of the soft palate are mainly related to the acts of respiration, deglutition, phonation, and articulation.

DEVELOPMENT.

Before discussing from an embryological standpoint the various deformities which we have already described, it is essential for us to consider the normal process of development of the parts entering into their formation.

At the same time that this mandibular arch is being developed, other changes are occurring around the upper part of the stomodaeum, viz. the shutting off of sacs lined with epiblast to assist in the formation of the organs of special sense, and the outgrowth between them of fleshy processes which by their later amalgamation form the facial skeleton and coverings. Three of these involutions of epiblast occur, two communicating more or less with the stomodaeum, viz. the nasal and ocular; whilst the third, or auditory, is separate. Expansions from the cerebral vesicles meet them, and by further changes, unnecessary to particularize here, the organs of special sense are elaborated.

The upper or nasal cavity is again subdivided into lateral halves by the growth downwards from the under surface of the fronto-nasal process of a central vertical septum, to become in time the cartilaginous septum nasi and the bony ethmo-vomerine plate, uniting at its lower border with the primary fleshy palatine processes .

The tongue grows as a fleshy protuberance from the floor of the stomodaeum; antero-lateral segments on either side from the conjoined second and third branchial arches unite with a central posterior growth from the tuberculum impar in an inverted Y-shaped manner. At the point of junction of the segments is a depression from which the thyroid gland develops, indicated in later life by the foramen caecum.

The palate bone develops from a single centre appearing about the eighth week at the junction of the horizontal and perpendicular portions. The superior maxilla is supposed to arise from four separate foci of ossification, viz. for the alveolar arch, for the palate, for the orbito-malar portion, and for the naso-facial segment. All these are united together by the third month.

The question as to the ossification of the intermaxilla has been purposely omitted hitherto, that the subject and its morphological relationship to congenital deformities might be more fully discussed.

Much controversy has been lighted up by this pronouncement, but here only a few of the points of interest and importance will be discussed.

The development of the intermaxilla from two centres on each side may be accepted as a proven fact. It was first maintained by the late Mr. Callender, who stated that these bones have a lateral wedge-shaped sutural surface, fitting into a groove in either superior maxilla, and that the alveolar processes of the latter extend forwards, forming the anterior walls of the sockets of the central incisors, and so fix the bones in position. A confirmation of the idea that the anterior alveolar walls of the incisor teeth are formed in this way was sought in the well-established fact that these particular parts are very imperfectly developed in those cases of alveolar harelip in which the os incisivum is isolated from the superior maxillae; but such is probably due to the abnormal condition and position in which the bone is developed, rather than to the loss of the maxillary "clip." And certainly the most recent researches tend to prove that the maxillae have no share in the formation of the alveoli of the incisor teeth.

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