ON HARELIP AND CLEFT PALATE.
ON
HARELIP AND CLEFT
PALATE.
BY
WILLIAM 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 Memory
OF
SIR 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 Remembrance
OF HIS
EXCEPTIONAL KINDNESS FOR MANY YEARS
BOTH AS A TEACHER AND PERSONAL FRIEND,
AND
In Humble Recognition
OF 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_).
31A. Head of fœtus at about 8 weeks, from the front (_Quain’s
Anatomy_).
31B. 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_).
46A and B. Author’s incisions for unilateral harelip, and
position of sutures.
47A and B. Graefe’s operation for unilateral harelip.
48A and B. Nélaton’s ditto.
49A and B. Malgaigne’s ditto.
50A and B. Giraldés’ ditto, or the mortise operation.
51A and B. Mirault’s ditto.
52. Stokes’s ditto (_Mason_).
53A and B. Collis’s ditto (_Mason_).
54A 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_).
61A. Fine hook forceps.
61B. Smooth-nosed forceps.
61C. 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_).
67A. Diagram to show extent and position of incisions in
uranoplasty.
67B. 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).
[Illustration: 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.
[Illustration: 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.
[Illustration: 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).
[Illustration: 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._)]
[Illustration: FIG. 10.
FIG. 10.—Complete cleft palate without alveolar or labial deformity; the
vomer is separate from the lateral segments. (_Mason._)]
[Illustration: 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).
[Illustration: 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.”
[Illustration: 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.
[Illustration: 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_”).
[Illustration: FIG. 18.—Oblique facial cleft, or rather cicatricial
deformity of face along the line usually traversed by such a cleft.
(_Tillmanns_, after _Kraske_.)]
[Illustration: 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_.)]
[Illustration: FIG. 20.—Double facial cleft with macrostoma.
(_Tillmanns_, after _Guersant_.)]
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.
[Illustration: FIG. 21.—Front view of a young puppy’s head from a
preparation in the Veterinary College of Brussels, showing double harelip
with double partial facial cleft. (After _Albrecht_.)
_a._ Central portion of upper lip, corresponding to internal nasal
process. _b._ Ala nasi, corresponding to external nasal process. _c._
Outer portion of upper lip, from superior maxillary process. _d._ Harelip
cleft. _e._ Facial cleft.]
[Illustration: FIG. 22.—Macrostoma, showing the cleft in the cheek
prolonged upwards and backwards by a reddish cicatrix to a lateral loss
of substance of the cranial wall. (_Sutton._)]
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.
[Illustration: FIG. 23.—Double macrostoma, showing the presence of
auricular appendages. (_Tillmanns._)]
[Illustration: FIG. 24.—Macrostoma with auricular appendages.
(_Fergusson._)]
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.
[Illustration: FIG. 25.—Mandibular cleft, showing the divided lower lip,
the segments being held together by cicatricial bands. (_Wölfler._)]
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.