Document Detail

Clinical presentation of epignathus teratoma with cleft palate; and duplication of cranial base, tongue, mandible, and pituitary gland.
MedLine Citation:
PMID:  23851840     Owner:  NLM     Status:  In-Data-Review    
A 2-day-old girl was diagnosed with an oral epignathus teratoma and an uncommon combination of orofacial malformations including cleft palate; tongue, mandible, cranial base, cervical vertebrae, lower lip, and pituitary gland duplications; and fistula of the glabella and lower lip. Computed tomography revealed that the mass within the nasal cavity had tooth-like calcifications and protruded into the nasopharynx and oral cavity. It was implanted on the anterior wall of the body of the sphenoid bone and was accompanied with mandibular duplication. Magnetic resonance imaging detected duplication of the pituitary gland and confirmed the absence of intracranial communication of the nasopharyngeal mass. The teratoma did not cause respiratory obstruction; however, the patient required continuous nasogastric tube feeding. Usually, an epignathus teratoma is associated with few midline defects and can be corrected with multiple interventions at different time points. The current study describes the surgical procedure comprising excision of the tumor along with reconstructive surgeries of the mandible, tongue, and fistulae undertaken when the infant reached 7 months of age. The cleft palate was repaired at 18 months of age using the Kaplan buccal flap method. Histopathologic examination confirmed a grade 0 teratoma covered with keratinized skin and containing pilosebaceous and sweat glands, adipose tissue, and smooth muscle. The long-term success of this intervention was determined at the follow-up examination conducted at 3 years of age, with no signs of the teratoma recurrence observed.
Yujiro Maeda; Hideyuki Suenaga; Madoka Sugiyama; Hideto Saijo; Kazuto Hoshi; Yoshiyuki Mori; Tsuyoshi Takato
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of craniofacial surgery     Volume:  24     ISSN:  1536-3732     ISO Abbreviation:  J Craniofac Surg     Publication Date:  2013 Jul 
Date Detail:
Created Date:  2013-07-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9010410     Medline TA:  J Craniofac Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1486-91     Citation Subset:  D    
From the Department of Oral-Maxillofacial Surgery, Dentistry and Orthodontics, The University of Tokyo Hospital, Tokyo, Japan.
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