Caroline Racz1, Stéphanie Dakpé2, Natacha Kadlub3, Sylvie Testelin4, Bernard Devauchelle4, Martin Rachwalski5, Arnaud Picard3. 1. Department of Maxillofacial Surgery, University Hospital Center, Place Victor Pauchet, 80054, Amiens Cedex 1, France; University of Picardie Jules Verne, Pôle santé 3, Rue des Louvels, 80036, Amiens Cedex 1, France. Electronic address: racz.caroline@chu-amiens.fr. 2. Department of Maxillofacial Surgery, University Hospital Center, Place Victor Pauchet, 80054, Amiens Cedex 1, France; Facing Faces Institute, Amiens University Hospital Center, Place Victor Pauchet, 80054, Amiens Cedex 1, France. 3. Department of Maxillofacial and Plastic Surgery, National Reference Center for Cleft Lip and Palate, Hôpital Universitaire Necker-Enfants Malades, 149 rue de Sèvres, 75015, Paris, France; University Paris Descartes, 12 rue de l'Ecole de Médecine, 75006 Paris, France. 4. Department of Maxillofacial Surgery, University Hospital Center, Place Victor Pauchet, 80054, Amiens Cedex 1, France; University of Picardie Jules Verne, Pôle santé 3, Rue des Louvels, 80036, Amiens Cedex 1, France; Facing Faces Institute, Amiens University Hospital Center, Place Victor Pauchet, 80054, Amiens Cedex 1, France. 5. Department of Maxillofacial and Plastic Surgery, National Reference Center for Cleft Lip and Palate, Hôpital Universitaire Necker-Enfants Malades, 149 rue de Sèvres, 75015, Paris, France; Department of Pediatric Neurosurgery, National Reference Center for Craniofacial Malformations, Hôpital Universitaire Necker-Enfants Malades, 149 rue de Sèvres, 75015, Paris, France.
Abstract
INTRODUCTION: Craniofacial clefts belong to the most disfiguring and rare congenital malformations of the face and among these, orbito-facial clefts constitute approximately 0.22 % of the cases with Tessier cleft number 5 being the least common. Our aim was to define the phenotypic spectrum for this subgroup to improve clinical management. METHODS: Our study group consisted of four patients which were treated at two different cleft centers. Retrospective chart review and anatomical analysis were conducted for each patient based on clinical evaluation and imaging studies. Morphological anomalies including soft tissue, bone and oral components were recorded. RESULTS: Based on our analysis and literature review, we could define two subtypes of Tessier facial cleft number 5. (1) Medial clefts are the more severe subtype, creating a significant soft tissue and bone defect that runs vertically, through the eyelid, infraorbital rim, maxillary sinus and cheek. They have the poorer esthetic and functional prognosis, due to orbital dystopia and absence of lower eyelid. (2) Lateral clefts are a less severe subtype characterized by the presence of a vertical furrow of the cheek running laterally to the maxillary sinus. CONCLUSIONS: We identified two subtypes of facial cleft number 5 which require an individualized surgical management.
INTRODUCTION:Craniofacial clefts belong to the most disfiguring and rare congenital malformations of the face and among these, orbito-facial clefts constitute approximately 0.22 % of the cases with Tessier cleft number 5 being the least common. Our aim was to define the phenotypic spectrum for this subgroup to improve clinical management. METHODS: Our study group consisted of four patients which were treated at two different cleft centers. Retrospective chart review and anatomical analysis were conducted for each patient based on clinical evaluation and imaging studies. Morphological anomalies including soft tissue, bone and oral components were recorded. RESULTS: Based on our analysis and literature review, we could define two subtypes of Tessier facial cleft number 5. (1) Medial clefts are the more severe subtype, creating a significant soft tissue and bone defect that runs vertically, through the eyelid, infraorbital rim, maxillary sinus and cheek. They have the poorer esthetic and functional prognosis, due to orbital dystopia and absence of lower eyelid. (2) Lateral clefts are a less severe subtype characterized by the presence of a vertical furrow of the cheek running laterally to the maxillary sinus. CONCLUSIONS: We identified two subtypes of facial cleft number 5 which require an individualized surgical management.