Tessier 30 facial cleft.

A case of midline cleft of the lower lip with cleft of the mandible and complete duplication of the tongue is reported here. Median cleft of the lower lip, mandible and bifid tongue with ankyloglossia is reported in the literature, but complete duplication of the tongue as part of the Tessier 30 cleft is not yet reported.

INTRODUCTION

Tessier 30 cleft, or lower midline facial cleft, also known as the median mandibular cleft, is a rare anomaly. Median cleft of the lower jaw was first reported in 1819 by Couronne.[1] In 1976, Tessier published a detailed description of the classification of craniofacial clefts,[2] wherein the lower midline facial cleft was numbered as 30. It is a rare anomaly, which may be limited to a defect in the soft tissue of the lower lip. However, in the more severe form, it may extend into the bony mandibular symphysis. In some cases, the neck structures like the hyoid bone, thyroid cartilage and even the strap muscles of the front of the neck may be involved. The anterior portion of the tongue may be bifid, which may be associated with ankyloglossia.[3] Total absence of the tongue is also reported, at least in one case.[4] The lower midline facial cleft defect is due to failure of midline union of the first branchial arch. The associated deformities of the neck are thought to be due to failure of fusion of the lower branchial arch elements.

CASE REPORT

A 5-month-old male baby was brought with midline cleft of the lower lip and the mandible [Figure 1]. On initial examination, the tongue appeared bifid, but examination under anesthesia revealed complete duplication of the tongue [Figure 2]. The radiograph of the mandible showed a bony defect in the midline [Figure 3]. The chest radiograph, echocardiography and routine blood examination were normal. The case was taken up for staged repair of the defect. In the first stage, repair of the soft tissues in the midline with bone graft to join the split mandible was performed [Figure 4]. During the procedure, it was noticed that there was a small piece of mandible with attached tooth bud that was not in continuity with the rest of the mandible. This piece of bone was dissected out, denuded of soft tissues and utilized as a bone graft to unite both halves of the mandible in the midline. The lower lip was reconstructed with multiple Z-plasties. No attempt was made to reconstruct the tongue at this stage. The patient is currently being followed-up for assessment of the mandibular growth and midline union, assessment of dentition and planned for further management with secondary repair of the lower lip and repair of the duplicated tongue at a later stage.

Figure 1

The child with Tessier 30 defect

Figure 2

Examination under anesthesia showing complete duplication of tongue

Figure 3

X-ray of the mandible showing the bony defect

Figure 4

Result after the first stage of repair

DISCUSSION

Since the first report of midline mandibular cleft by Couronne in 1819, 66 cases have been reported worldwide till 2007.[5] Documented anomalies are mandibular cleft, ankyloglossia and abnormal dentition. Associated findings are thinned-out strap muscles, split hyoid, split sternum, split tracheal cartilages, thyroglossal cyst, dermoid cyst, etc.[1] Sternal cleft was described in only three cases and foregut duplication cyst was reported by Senan et al.[5] The rare association of bifid tongue with ankyloglossia was reported by Chidzonga[6] and Tiwari.[7] Rana et al.[8] reported a case of median cleft of mandible and lower lip with ankyloglossia and ectopic minor salivary gland on the tongue.

The case reported here had midline cleft of the lower lip with cleft of the mandible, along with complete duplication of the tongue. Although Britto et al.[9] reported a case of double tongue with cleft palate, there has been no case report with Tessier 30 cleft with complete duplication of the tongue. There was no ankyloglossia in our case. The case was taken up for staged repair. In the first stage, the two halves of the mandible were approximated with a bone graft, and the soft tissue of the lower lip was repaired. Secondary repair of the lower lip to improve the cosmetic appearance and fusion of the two halves of the tongue will be taken up at subsequent stages.

The treatment, as advocated by Armstrong and Waterhouse,[10] is to tackle the condition in a staged manner. In the first stage, soft tissue correction including Z-plasty in chin, lip and neck is performed. Mandible reconstruction is done after 10 years of age to minimize damage to the developing tooth buds. However, earlier treatment may be indicated when the segments are hypermobile, causing respiratory or feeding difficulty.

In conclusion, a rare case of Tessier 30 cleft is reported and the literature is reviewed. A hitherto unreported association of this anomaly with complete duplication of the tongue is described. Staged repair of the case is being undertaken.