Congenital bilateral zygomatico-maxillo-mandibular fusion associated with gumfusion: Case report

INTRODUCTION: Maxillomandibular sygnathia is a rare and severe craniofacial deformity defined by gingival mucosal fusion (synechia) or bony fusion (synostosis). CASE REPORT: We will present a case of complete closure of the mouth since birth from eastern Morocco treated in our department of maxillofacial surgery at the University Hospital of Casablanca. The patient was referred after two months by the pediatrician of the provincial hospital and following the placement of a nasogastric tube. On admission, she presented with facial dysmorphosis, signs of malnutrition with closure of the mouth and fusion of both gums with pro-alveoli and retromandibulia. CT scan with 3D reconstruction confirmed maxillomandibular synostosis. 5 days later, she underwent a fixed mucosal incision with osteotomy at the maxillomandibular joints with early and prolonged active physical therapy. The evolution was marked by the recurrence of mouth closure, the little girl was operated a second time and then she died by a mucous plug at the level of her tracheostomy cannula during resuscitation. DISCUSSION: Maxillomandibular sygnathia is a very rare pathology whose origin remains unknown. Very few cases published in the literature. CONCLUSION: The therapeutic difficulties encountered outside the ideal age of the operation and the management of recurrences were also linked to socio-economic factors making it difficult to ensure adequate postoperative follow-up.