title: Surgical Management of Duplication of the Pituitary Gland-Plus Syndrome With Epignathus, Cleft Palate, Duplication of Mandible, and Lobulated Tongue.
abstract: A 1-day-old male infant was referred to our department for evaluation of multiple malformations in his oral cavity. He was diagnosed duplication of the pituitary gland-plus syndrome with epignathus, cleft palate, duplication of the mandible, and a lobulated tongue. A thumb-sized mass lesion was visible on the hard palate. The duplicated mandible and lower lip was fused at the midline. The alveolar ridge was protruding through a wide-cleft soft palate involving the uvula. Further examination showed a lobulated tongue, which was seen behind the duplicated part of the mandible. Five days after birth, tracheotomy and epignathus resection were performed. At 7 months of age, the excess tissue of the duplicated mandible was resected at the area of adhesion on the lingual side, and the duplicated tongue and lip were reconstructed. A palatoplasty was performed at 20 months of age. Thereafter, the patient's progress was uneventful, with no abnormality in swallowing. No recurrence of epignathus has been observed during 2 years of follow-up.
authors.affiliation: *Department of Dentistry, Oral and Maxillofacial Surgery, Jichi Medical University †Department of Pediatric Surgery, Jichi Medical University, Shimotsuke-shi ‡Department of Pediatric Surgery, Kobe Children's Hospital, Kobe §Department of Otolaryngology, Head and Neck Surgery, Jichi Medical University, Shimotsuke-shi, Japan.
authors.forename: Tadahide
authors.initials: T
authors.lastname: Noguchi
authors.forename: Tomoko
authors.initials: T
authors.lastname: Sugiyama
authors.forename: Ken-Ichi
authors.initials: KI
authors.lastname: Sasaguri
authors.forename: Shigeru
authors.initials: S
authors.lastname: Ono
authors.forename: Kosaku
authors.initials: K
authors.lastname: Maeda
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nishino
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Jinbu
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Mori
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery*; D002675:Child, Preschool; D002972:Cleft Palate / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008046:Lip / Q000002:abnormalities / Q000601:surgery; D008297:Male; D008334:Mandible / Q000002:abnormalities / Q000601:surgery*; D010157:Palatal Neoplasms / Q000601:surgery*; D021362:Palate, Hard / Q000473:pathology / Q000601:surgery; D010160:Palate, Soft / Q000002:abnormalities / Q000601:surgery; D010902:Pituitary Gland / Q000002:abnormalities*; D013577:Syndrome; D013724:Teratoma / Q000601:surgery*; D014059:Tongue / Q000002:abnormalities / Q000601:surgery*