[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._)]
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