Unilateral Craniofacial Microsomia with Severe Tessier No. 7 Cleft and Mandibular Agenesis: A Rare Case Report.
Chi Hyun Lee, Hyun Seung Lee, Kyung Hee Park et al.
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Case in brief
A neonate with unilateral craniofacial microsomia had a severe Tessier No. 7 cleft, complete mandibular agenesis, ear deformities, and extensive intraoral adhesions. Tongue-to-mucosa adhesions left little reconstructable soft tissue and made release and surgical timing challenging. Respiratory distress required tracheostomy, while severe oral-feeding difficulty necessitated nasogastric feeding. Surgery on day 70 released the adhesions, separated the oral cavity from the external face, and reconstructed the oral commissure. Oral feeding was then initiated, with aesthetic improvement and no reported complications.
What made this case unusual
The combination of complete mandibular agenesis and a severe Tessier No. 7 cleft with tongue-to-mucosa adhesions created a marked soft-tissue deficiency that complicated both adhesion release and the timing of reconstruction.
Diagnostic / clinical pearl
In severe craniofacial microsomia, assess airway and feeding needs alongside the extent of intraoral adhesions and available reconstructable tissue when planning intervention.