BACKGROUND

The tongue is an essential organ accounted for proper deglutition and articulation. Surgical repair should be planned soon after diagnosis of any structural abnormality to prevent later speech and swallowing disorders. The lobulated tongue could be isolated (sporadic) or in association with other disorders. Pierre Robin Sequence (PRS) consists of the clinical trial of congenital micrognathia, glossoptosis, and airway obstruction with variable inclusion of a cleft palate. We present the case of a rare congenital tri-lobed tongue with Pierre Robin sequence and its surgical management in our hospital setting.

CASE PRESENTATION

Six-month-old boy presented with severe retrognathia, high arch, complete isolated cleft palate, and a bizarre mass in the oral cavity instead of his tongue that led to disruption of his swallowing. The mass (deformed tongue) check clearly, and the normal shape of the tongue was restored through multiple local randomized flaps. Dramatic improvement in swallowing was noticed 6 months after surgery during postoperative follow-up.

DISCUSSION

We present the case of a patient with a tri-lobed tongue with Pierre Robin sequence characterized by severe retrognathia, high arch, and complete isolated cleft palate. This seems to be the first reported case of this particular craniofacial anomaly.

CONCLUSION

The management of infants with the Pierre Robin sequence is complex, and much still needs to be learned and practiced. Congenital tri-lobed tongue with a cleft as part of the Pierre Robin sequence is a very rare malformation. Early repair of the tongue is important to assist the baby in adapting to speech and swallowing as they grow.