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.
