The patient is an African American male who was born at 40 weeks by vaginal delivery. He weighed 8 pounds at birth. His mother received prenatal care for the last 3 months of her pregnancy and she had no history of STDs. She was Group B Streptococcus positive but did receive antibiotics while she was in labor. There was no family history of facial deformities. The patient was hospitalized for 7 days at birth for feeding difficulties. He was then discharged home, where he lived with his parents and four siblings. He was readmitted at 15 days of age to the Pediatric service for stridor. He was noted to have persistent hypercarbia. His weight was in the 25th percentile, his height was in the 50th percentile, and his head circumference was in the 10-25th percentile. Nissen fundoplication and G tube were performed, and his stridor was noted to subsequently improve.
The Otolaryngology service was consulted for facial deformity, micrognathia, and microcephaly. On physical exam, he was noted to have a band of tissue that connected the right oral commissure to the oropharynx. It was felt that the vibrating of this band may have been contributing to his noisy breathing. The right auricle appeared normal, the mandible was intact, and there were no abnormalities of the facial nerve.
Echocardiogram was within normal limits. However he continued to have hypercarbia, and tracheostomy was performed at 4 months of age.
At 8 Β½ months of age, the patient underwent direct laryngoscopy, bronchoscopy, and examination of the ears. He was noted to have a 1.5 cm cleft at the right oral commissure, a 1 cm wide band of keratinized epithelium on the buccal mucosa extending to the superior pole of the tonsil and soft palate (Figure 3), and bilateral acute otitis media. The hard palate appeared intact. Bilateral PET were placed in at one year of age for recurrent otitis media. The right one spontaneously extruded. Modified barium swallow at 1 Β½ years of age showed no evidence of airway penetration or aspiration. He was seen in Craniofacial ENT clinic and noted to have 4+ tonsils and intact palate. At age 1 ΒΎ years, the G tube was removed and mother noted choking spells since that time. Modified barium swallow showed no signs of aspiration. At age 2 years, the patient underwent repair of facial cleft, myringotomy, and placement of bilateral PE tubes. The facial cleft was repaired using the Skoog technique. The intraoral skin was excised and the mucosa was closed primarily. Z-plasty was performed of the right cheek skin. On postoperative day 1, the patient was tolerating oral intake. He continues to have his tracheostomy and speaks well with a passey muir valve. At the age of 3 years, the patient underwent placement of right PE tube, direct laryngoscopy, and bronchoscopy to evaluate for decannulation. There were no noted abnormalities on exam and he was to be decannulated in the recovery room. However, he failed decannulation secondary to hypercarbia. Flexible scope exam one month later showed no laryngomalacia or anatomic defects.