On harelip and cleft palate

by William Rose

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.

Fig. 23.—Double macrostoma, showing the presence of auricular appendages. (Tillmanns.)

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.