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Giant epignathus with midline mandibular cleft: Insights in embryology and management.
MedLine Citation:
PMID:  23483138     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
A congenital teratoid tumor arising and protruding through the mouth is classified as epignathus or fetus in fetu. On review of literature, we found various reports of midline mandibular and lower lip cleft associated with flexion contracture of neck, midline cervical cord, but there is only one report of association with midline dermoid. We present an unusual case of midline cleft of mandible with an epignathus. A 2.3-kg male child, delivered transvaginally in the 38(th) gestational week, was referred to us for management of a large irregular growth hanging outside the mouth. On examination, he had a wide median cleft of the mandible with tongue adherent to the "V"-shaped defect in the area of lower lip. A midline irregular mass of size 12 × 8 × 5 cm with variegated consistency was arising in the midline from the floor of the mouth between the tongue and lower lip. X-ray and computed tomography scan showed a rounded soft tissue mass arising from the alveolus with multiple calcifications within it along with a large triangular calcification and absence of hyoid bone. The mass was excised by mobilizing the tip of tongue. Staged repair was planned for the defect in the mandible. Unfortunately, the baby succumbed postoperatively to complex congenital heart disease. Histopathology was suggestive of epignathus. We discuss hereby the embryology and current management strategies of the problem.
Authors:
Advait Prakash; Sandesh V Parelkar; Sanjay N Oak; Rahul K Gupta; Beejal V Sanghvi
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Annals of maxillofacial surgery     Volume:  2     ISSN:  2231-0746     ISO Abbreviation:  Ann Maxillofac Surg     Publication Date:  2012 Jan 
Date Detail:
Created Date:  2013-03-13     Completed Date:  2013-03-14     Revised Date:  2013-04-18    
Medline Journal Info:
Nlm Unique ID:  101598423     Medline TA:  Ann Maxillofac Surg     Country:  India    
Other Details:
Languages:  eng     Pagination:  56-9     Citation Subset:  -    
Affiliation:
Department of Pediatric Surgery, King Edward Memorial Hospital, Parel, Mumbai, India.
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