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.
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.