On harelip and cleft palate

by William Rose

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.)

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.

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.

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.)