Median cleft is rare among facial clefts, including bilateral and unilateral clefts. Median upper cleft lip and median alveolar cleft correspond to Type 14 and Type 0, respectively, in Tessier’s classification system. Some authors have reported surgical procedures for median cleft. In the case of median alveolar cleft, bone grafting to the cleft side and orthodontics are generally applied, similarly to bilateral or unilateral cleft. Median alveolar cleft is usually accompanied by median upper cleft lip, the degree of which differs in each case. The symptoms include, but are not limited to, median lip defect, wide philtrum, and vermilion notch. However, an isolated alveolar cleft is extremely rare. We encountered a patient with an isolated alveolar cleft who did not have a light median upper cleft lip, such as a wide philtrum or vermilion notch. We herein report this case and describe its treatment.
Median alveolar cleft is usually accompanied by median upper cleft lip, the degree of which differs in each case.
We reported an extremely rare case of isolated alveolar cleft without median upper cleft lip.
A good result was gained by bone grafting and corrective orthodontics.
An examination of the filling mass with cleft revealed that it was mostly composed of normal tissue.
In comparison to bilateral and unilateral cleft, median cleft is a rare deformity. Davis and Fogh-Anderson reported that the median cleft was observed in 0.73% and 0.43% of cleft lips, respectively [ , ]. Another study reported that the frequency of rare craniofacial clefts was 1.4–4.9 per 100,000 live births [ ]. Various classifications are used for median clefts. Veau described three varieties of median cleft: a notch, a median cleft extending to the columella, and a defect due to lack of development of the whole medial element [ ]. However, Millard proposed that vertical cleft through the center of the upper lip, regardless of the extent, should be classified as a median cleft lip [ ]. The median cleft is distinguished into two types: true median cleft and false median cleft. True median cleft is accompanied by other abnormalities, such as polydactyly and hypertelorism. False median cleft was proposed that was associated the deformities of median cleft lip, nasal malformation, and orbital hypotelorism [ ]. The false type is a kind of holoprosencephaly and it is difficult for patients to achieve long-term survival [ ]. In this case report, we describe the treatment of a case of median alveolar cleft with a palatal mass and without an upper cleft lip.
A 4-year-old girl with median alveolar cleft visited the department of Plastic and Reconstructive Surgery in Kyoto University Hospital. She had the large space between the central maxillary incisor and a palatal mass in the cleft ( Fig. 1 A and B) without a median upper cleft lip ( Fig. 2 A). She had a past history of epilepsy, but no other diseases or abnormalities. The patient sometimes complained of feeling uncomfortable because of the mass. We planned to perform combination therapy with corrective orthodontics and surgical bone grafting.
The patient underwent surgery at 9 years of age. The median distance between the incisors was narrower than at 4 years of age after applying corrective orthodontics ( Fig. 2 B). The surgical operation was carried out under general anesthesia; 1% lidocaine with 1:100,000 epinephrine was injected into the gingiva and mucosa around the alveolar cleft. First, the palatal mass was excised. Then, the mucosa-periosteum flaps on both the lip and palatal side were elevated to expose the maxillary bone in the cleft. A pocket and space surrounding the periosteum was made to transfer iliac cancellous bone ( Fig. 2 C). The incised mucosa at the median line was sutured completely and mucosal grafting was required on the palatal side ( Fig. 2 D). There were no complications (e.g., infection or wound dehiscence) on the oral or iliac sides. The macro and microscopic findings of the mass are shown in Fig. 3 A and B. The lesion was covered with squamous epithelium. Furthermore, collagenous fiber and an increased number of vessels were observed between the superficial epithelial side and the deep side, including the small salivary gland ( Fig. 3 B).