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- Herausgeber: Science & Technology Publishing
- Kategorie: Fachliteratur
- Sprache: Englisch
On Harelip and Cleft Palate is a comprehensive medical treatise authored by William Ferguson, first published in the 19th century. This seminal work delves deeply into the congenital deformities of harelip and cleft palate, conditions that affect the structure and function of the mouth and face. Ferguson meticulously describes the anatomical features, causes, and variations of these malformations, drawing on his extensive clinical experience and the prevailing medical knowledge of his time. The book offers a detailed exploration of the etiology and pathology of harelip and cleft palate, discussing both hereditary and environmental factors that may contribute to their development. Ferguson provides thorough guidance on the diagnosis and classification of these conditions, emphasizing the importance of early detection and intervention. A significant portion of the text is devoted to surgical techniques and treatment options. Ferguson outlines the principles of operative management, including preoperative preparation, anesthesia, and postoperative care. He presents step-by-step instructions for various surgical procedures, illustrated with case studies and practical observations. The author also addresses potential complications and the long-term outcomes of surgical intervention, offering advice on how to optimize functional and aesthetic results. In addition to surgical considerations, Ferguson discusses the broader implications of harelip and cleft palate for speech, nutrition, and psychosocial well-being. He advocates for a multidisciplinary approach to care, involving surgeons, dentists, speech therapists, and other specialists to ensure comprehensive rehabilitation for affected individuals. Rich in historical context and clinical insight, On Harelip and Cleft Palate stands as a valuable resource for medical professionals, historians, and anyone interested in the evolution of craniofacial surgery. Ferguson’s work not only reflects the medical practices of his era but also lays the groundwork for future advancements in the treatment of these challenging conditions.
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ON HARELIP AND CLEFT PALATE.
ON HARELIP AND CLEFT PALATE.
BYWILLIAM ROSE, M.B., B.S.Lond., F.R.C.S.,PROFESSOR OF SURGERY IN KING’S COLLEGE, LONDON, AND SURGEON TO KING’S COLLEGE HOSPITAL, ETC.
LONDON: H. K. LEWIS, 136 GOWER STREET, W.C. 1891.
To the MemoryOFSIR WILLIAM FERGUSSON, Bart., F.R.S., LL.D.,SERGEANT-SURGEON TO THE QUEEN; PROFESSOR OF CLINICAL SURGERY IN KING’S COLLEGE, LONDON, AND SENIOR SURGEON TO KING’S COLLEGE HOSPITAL, ETC. In Grateful RemembranceOF HIS EXCEPTIONAL KINDNESS FOR MANY YEARS BOTH AS A TEACHER AND PERSONAL FRIEND,ANDIn Humble RecognitionOF HIS MASTERLY SKILL AS A SURGEON,THIS SMALL VOLUME IS AFFECTIONATELY DEDICATED.
PREFACE.
In bringing this book before the notice of the profession I have given the results of my experience, which has now been considerable, in a department of surgery always of special interest to me, and to which I have devoted particular attention. The privilege enjoyed for many years of assisting the late Sir William Fergusson gave me an early insight into these operations, and an unusual opportunity of learning their details at the hands of so distinguished a surgeon. I have endeavoured to make the work as complete as possible, and to bring its contents up to date, and in this am greatly indebted to the assistance of my colleague Mr. Albert Carless, who has not only revised the whole work, indexed it, and seen it through the press, but has also written the chapter on Development. I gratefully acknowledge the kindness I have received from various sources in permitting the use of blocks for illustrations, notably from Mrs. Mason, the widow of the late Mr. Francis Mason, whose work on the subject is well known, from Mr. Oakley Coles, and many others, whose names will be found in the list of illustrations.
17, Harley Street, W.; 1891.
CONTENTS.
PAGE
CHAPTER I.
General Introduction.
Harelip—Cleft palate—Frequency—Occurrence in animals—Associated deformities—Median harelip—Facial clefts—Macrostoma—Mandibular clefts—Causes of these deformities
1-25
CHAPTER II.
Anatomy and Physiology of the Normal Palate.
The hard palate—The velum and its muscles—The mucous membrane—The blood supply—The shape and size of the hard palate—Functions
26-35
CHAPTER III.
Development.
Normal development of mouth, face, nose, and teeth—Ossification—Development of intermaxilla; old ideas (Goethe’s, &c.); Albrecht’s theory—Harelip; position of cleft in alveolus, and in lip—Dentition; accessory teeth—Development of other deformities
36-59
CHAPTER IV.
The Anatomy and Physiology of Harelip and Cleft Palate.
Harelip—Effect of labial muscles on deformity—Structure of os incisivum and labial segments.
Cleft palate—Arrangement and action of muscles—Shape of bony segments—Associated irregularity in shape of skull—Physiological effects in nutrition, articulation, &c.
60-71
CHAPTER V.
Operative Treatment of Harelip.
Period of operation—Statistics—Precautions to be adopted.
Operation for single harelip: incisions; sutures; dressing; after-treatment—Various plans adopted.
Operation for double harelip: treatment of os incisivum—extirpation or reposition; treatment of soft parts
72-100
CHAPTER VI.
Operative Treatment of Cleft Palate.
Period of operation—Preparation of patient—Anæsthesia—Duties of the assistant—Instruments—Description of uranoplasty; of staphyloraphy—After-treatment—Complications—Modifications of the operation
101-138
CHAPTER VII.
On Obturators and Artificial Vela
139-145
CHAPTER VIII.
Results of Treatment—After-treatment
146-153
CHAPTER IX.
Syphilitic Affections of the Palate
154-156
ADDENDUM.
Rectal Anæsthesia
157, 158
INDEX
159
LIST OF ILLUSTRATIONS.
1.
Hare’s lip (
Bland Sutton
).
2-6.
Different varieties of harelip (after
Tillmanns
).
7, 8.
Double harelip with projection of os incisivum, seen from the front and in profile (
Fergusson
).
9.
Complete cleft palate with double alveolar harelip (
Mason
).
10.
Complete cleft palate without alveolar or labial deformity (
Mason
).
11.
Complete unilateral cleft palate with vomer attached to one segment (
Mason
).
12-14.
Incomplete fissures of the palate (
Mason
).
15.
Cleft of lip, alveolus, and anterior part of palate only (
Mason
).
16.
Median harelip with absence of intermaxillæ (
Bland Sutton
).
17.
Median fissure of upper lip (
Bernard Pitts
).
18.
Oblique facial cleft (
Tillmanns
, after
Kraske
).
19.
Facial cleft in child (
Tillmanns
, after
Hasellmann
).
20.
Double facial cleft with macrostoma (
Tillmanns
, after
Guersant
).
21.
Puppy’s head with double alveolar harelip and double partial facial cleft (after
Albrecht
).
22.
Lateral aspect of a severe case of macrostoma (
Bland Sutton
).
23.
Double macrostoma with auricular appendages (
Tillmanns
).
24.
Ditto ditto (
Fergusson
).
25.
Mandibular cleft (
Wölfler
).
26.
Intermaxillary sutures of a tetraprodontous child’s jaw (after
Albrecht
).
27.
Intermaxillary sutures in a hexaprodontous jaw (after
Albrecht
).
28.
Muscles of palate dissected (
Fergusson
).
29.
Head of fœtus at 5 weeks (
Sutton
).
30.
Head of fœtus at about 6-7 weeks (
Sutton
).
31
A
.
Head of fœtus at about 8 weeks, from the front (
Quain’s Anatomy
).
31
B
.
Head of fœtus seen from below (
Quain’s Anatomy
).
32, 33.
Later stages of development of head (
Quain’s Anatomy
).
34.
Diagram of development of palatal processes and ethmo-vomerine plate (after
Gegenbaur
).
35.
Bony palate of a 6 months’ fœtus (after
Gilis
).
36.
Diagram illustrating “Goethe” theory of alveolar harelip.
37.
Diagram illustrating “Albrecht” theory of alveolar harelip in a tetraprodontous jaw.
38.
Diagram illustrating “Albrecht” theory of alveolar harelip in a hexaprodontous jaw.
39.
Adult upper jaw with right-sided alveolar harelip and cleft palate (after
Albrecht
).
40.
Child’s jaw with cleft palate and double alveolar harelip, showing the sockets of the teeth (after
Albrecht
).
41.
Os incisivum (
Fergusson
).
42, 43.
Diagrams illustrating the effect of differing slopes of the palatal segments (
Mason
).
44.
Teat of feeding bottles adapted for feeding infants with cleft palate (
Mason
).
45.
Coles’s nipple shield for ditto (
Coles
).
46
A
and
B
.
Author’s incisions for unilateral harelip, and position of sutures.
47
A
and
B
.
Graefe’s operation for unilateral harelip.
48
A
and
B
.
Nélaton’s ditto.
49
A
and
B
.
Malgaigne’s ditto.
50
A
and
B
.
Giraldés’ ditto, or the mortise operation.
51
A
and
B
.
Mirault’s ditto.
52.
Stokes’s ditto (
Mason
).
53
A
and
B
.
Collis’s ditto (
Mason
).
54
A
and
B
.
Author’s incisions for double harelip.
55.
Sédillot’s operation for ditto (
Mason
).
56.
T. Smith’s operation for ditto (
Mason
).
57.
T. Smith’s gag with tongue plate (
Messrs. Arnold and Son
).
58.
Mason’s gag (
Matthews Bros.
).
59.
Rose’s gag (
Matthews Bros.
).
60.
Various forms of raspatories (after
Durham
).
61
A
.
Fine hook forceps.
61
B
.
Smooth-nosed forceps.
61
C
.
Knife for paring edges of cleft (
Mason
).
62.
Angular long-handled catch forceps.
63.
Various forms of needles.
64, 65.
Diagrams to illustrate the effects of tightening the sutures on the needle-tracks.
66.
Wire twister (
Messrs. Maw, Son, and Thompson
).
67
A
.
Diagram to show extent and position of incisions in uranoplasty.
67
B
.
Diagram of position of sutures and condition of palate after operation.
68.
Double-curved raspatories.
69.
Loop method of passing sutures (
Mason
).
70.
Method of tying slip-knot (
Fergusson
).
71.
T. Smith’s palate needle (
Messrs. Arnold and Son
).
72-74.
Various form of artificial vela (
Coles
).
75.
Appearance in profile after operation for double harelip, including removal of os incisivum (
Coles
).
ON HARELIP AND CLEFT PALATE.
CHAPTER I.GENERAL INTRODUCTION.
Harelip—Cleft palate—Frequency—Occurrence in animals—Associated deformities—Median harelip—Facial clefts—Macrostoma—Mandibular clefts—Causes of these deformities.
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).
Fig. 1.—Hare’s lip to show the median cleft in the lower part prolonged upwards into either nostril. (Sutton.)
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.
Fig. 2.Fig. 3.Fig. 4.
Fig. 5.Fig. 6.
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.
Figs. 7 and 8.—Double harelip with projection of the os incisivum, as seen from the front and in profile. (Fergusson.)
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).
Fig. 9.
Fig. 9.—Complete cleft palate and double alveolar harelip; simulating a double lateral cleft, due to the vomer being seen free between the palatal segments. (Mason.)
Fig. 10.
Fig. 10.—Complete cleft palate without alveolar or labial deformity; the vomer is separate from the lateral segments. (Mason.)
Fig. 11.—Complete unilateral cleft palate without alveolar deficiency; the vomer is attached to the left palatal segment. (Mason.)
Figs. 12, 13, 14.—Various degrees of simple fissure of the palate. (Mason.)
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).
Fig. 15.—Unusual form of cleft involving the alveolar arch, and the anterior portion of the palate only. (Mason.)
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.”
Fig. 16.—Median harelip showing total absence of the central portion of the upper lip and of the intermaxillæ, and flattening of the nose from absence of the ethmo-vomerine plate. (Bland Sutton.)
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.
Fig. 17.—Median harelip, showing a mesial cleft in the soft structures of the upper lip. (Pitts.)
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”).
Fig. 18.—Oblique facial cleft, or rather cicatricial deformity of face along the line usually traversed by such a cleft. (Tillmanns, after Kraske.)
Fig. 19.—Facial cleft in a child, implicating the lower lid and eye, and with a development of accessory teeth along the cleft margins. (Tillmanns, after Hasellmann.)
Fig. 20.—Double facial cleft with macrostoma. (Tillmanns, after Guersant.)
