Tessier 30 Facial Cleft with Duplication of Tongue.

A case of midline cleft of the lower lip, mandible, and the neck with complete duplication of the tongue repaired at neonatal period is reported here. Median cleft of the lower lip, mandible, and bifid tongue with ankyloglossia is reported in the literature, but cleft of the neck with complete duplication of the tongue as a part of the Tessier 30 cleft is very rare. We could not find such report in the available English literature.

INTRODUCTION

Lower midline facial cleft, or median mandibular cleft, is a rare anomaly. Although several literature mentioned this before,[1] in 1976, Tessier published a detailed description with the classification of the condition.[2] The defect may range from the involvement of soft tissue of the lower lip, teeth, mandible, and neck. The tongue may be bifid and can be associated with ankyloglossia.[3] The total absence of the tongue has also been reported.[4] Delayed repair of these cases is usually practiced. Some authors have opined that the early repair of this condition should be done to avoid feeding problem.[1] We are reporting a case of midline facial cleft that was repaired in the neonatal period with good functional outcome.

CASE REPORT

A 5-day-old female was admitted with a midline cleft of the lower lip, mandible, and tongue along with midline deficiency of the muscles and skin of the neck region [Figure 1]. No other congenital anomaly was detected.

Figure 1

The initial appearance of the patient

Computed tomography scan with three-dimensional reconstruction and magnetic resonance imaging scan of the face and neck region was done. The soft tissue gap was from tip of the tongue to its base along with the submandibular region. Hyoid bone and muscle and skin of the neck were deficient in the midline. Trachea and larynx were normal. The mandible was separated wide at symphysis, but there was no bony deficiency. To prevent respiratory distress the repair was planned in two stages.

In the first stage, repair of bifid tongue and midline cervical cleft was done. Neck skin was repaired by multiple Z-plasties. The bifid tongue was repaired by approximating both halves after excising the excess mucosa. Nasogastric tube feeding started from the 2nd postoperative day and after 5th day breastfeeding was initiated. The patient gradually learnt the sucking and swallowing and was out of tube feeding from the 10th postoperative day.

Second stage operation was done 2 weeks after the first. Here tip of the tongue was released from the floor of mouth, both halves of the mandible were united by stainless steel wire suturing and the soft tissue over the mandible was repaired in a straight line [Figure 2]. Nasogastric tube feeding was started from the 2nd postoperative day and the baby was able to drink milk by spoon from the 7th postoperative day. Breast feeding was started from the 12th postoperative day.

Figure 2

After repair of the defect

The patient was discharged 14 days after the second operation. At that time, the baby was on breastfeeding. At 3 weeks follow-up, the patient was thriving well and was on exclusive breastfeeding.

DISCUSSION

Couronne in 1819 first reported a case of midline mandibular cleft and till 2012, a total of 67 cases were reported.[5] In 2014, Guttikonda et al. reported a case of the mandibular cleft, ankyloglossia, and abnormal dentition. The case was repaired at 1 month and again at 16 years of age.[6] Association of thinned-out strap muscles of the neck, split hyoid, split sternum, split tracheal cartilages, thyroglossal cyst, dermoid cyst, etc., are also documented.[7] Senan et al. described three such cases with a sternal cleft. One of these had duplication cyst of the foregut.[8] Rana et al.[9] reported a case of the median cleft of the mandible and lower lip with ankyloglossia and ectopic minor salivary gland on the tongue.

Almeida et al.[10] reported a case with fusion defect with deficiency of the mandible. In their case tongue, lip, and neck structures were normal. They repaired the mandible at 10 years of age with bone graft and plate fixation.

So far, there is only one case report of Tessier 30 cleft with complete duplication of the tongue.[5]

The case reported here had midline cleft of the lower lip, mandible, and floor of the mouth with the involvement of skin and superficial muscles of the neck. There was a complete duplication of the tongue but without the involvement of trachea and sternum.

Armstrong and Waterhouse,[1] advocated a staged repair of the defect. They suggested that in the first stage, soft tissue correction including Z-plasty in chin skin, lip, and neck is to be performed. Mandible reconstruction was advised at 10 years of age to minimize the damage to the developing tooth buds. However, they advocated earlier fixation of mandible when the segments are hypermobile causing respiratory or feeding difficulty.

The present case had mobile halves of the mandible, and it caused a feeding problem. After the first stage repair, the baby could suck breast milk. Early second stage operation was done for cosmetic and functional improvement of the baby. The baby tolerated the fixation of mandibular halves well. As there was the least bone handling it is expected that there will be no defect in the dentition.

In conclusion, a rare case of Tessier 30 cleft is reported, and the literature is reviewed. In this case, there was cleft of the tongue, mandible and the soft tissue of the neck. Staged repair of the case was undertaken at the neonatal period. An early result of repair is encouraging. Long-term follow-up regarding dentition and scar contracture is to be observed.

DECLARATION OF PATIENT CONSENT

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FINANCIAL SUPPORT AND SPONSORSHIP

Nil.

CONFLICTS OF INTEREST

There are no conflicts of interest.