Surgical Management of Duplication of the Pituitary Gland-Plus Syndrome With Epignathus, Cleft Palate, Duplication of Mandible, and Lobulated Tongue. Issue 2 (March 2017)

• Record Type: Journal Article
• Authors: Noguchi, Tadahide; Sugiyama, Tomoko; Sasaguri, Ken-ichi; Ono, Shigeru; Maeda, Kosaku; Nishino, Hiroshi; Jinbu, Yoshinori; Mori, Yoshiyuki
• Is Part Of: Journal of craniofacial surgery. Volume 28:Issue 2(2017)
• Publication Date: 2017-03
• Subjects: Cleft palate -- duplication of mandible -- duplication pituitary gland-plus syndrome -- epignathus -- lobulated tongue; Facial bones -- Surgery -- Periodicals; Skull -- Surgery -- Periodicals; Face -- Surgery -- Periodicals; Surgery, Plastic -- Periodicals; 617.52
• DOI: 10.1097/SCS.0000000000003324
• Languages: English
• ISSNs: 1049-2275

Abstract

Abstract : 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.