1999 Volume 45 Issue 3 Pages 211-213
The authors describe a typical case of Treacher-Collins syndrome with bilateral cleft lip and palate and polypoid lesions in the retromolar region.A 2-month-old boy had an antimongoloid slant of the eyes with colobomas, depressed cheeks, small jaws, auricular anomalies, conductive deafnesscleft lip and palate, and a hairy tongue.The family history was negative.A tracheostomy was performed 1 month after birth to manage airway obstruction due to TMJ-mandibular deficiency.A Hotz-type plate was used to improve nursing 2 months after birth.Cleft lip was repaired 6 months after birth.Bilateral polypoid lesions (20×10×7mm), in the retromolar region were removed at the same time.They consisted of unkeratinized squamous epithelium, submucosa, salivary glands, and striated muscles, similar to soft palate tissues.Treacher-Collins syndrome is generally characterized by bilaterally symmetrical abnormalities of structures within the first and second branchial arches. Polypoid lesions in the retromolar region have not been reported so far. Cleft lip is found only in 2% to 6% of patients with this syndrome.The findings in this case may have resulted from severe deformities in the first and second branchial arches.
