UNASSIGNED: Accessory maxilla is a rare condition often associated with Tessier type-7 clefts with fewer than 25 cases recorded in the literature. This manuscript reports a unilateral accessory maxilla with six supernumerary teeth.
UNASSIGNED: A 5-1/2-year-old boy, a treated macrostomia case, on follow-up visit showed evidence of accessory maxilla with teeth on radiological examination. The structure was interfering with growth, and hence, surgical removal was planned.
UNASSIGNED: Based on clinical history, diagnosis and imaging, accessory maxilla with supernumerary teeth was diagnosed.
UNASSIGNED: The accessory structures and teeth were removed surgically via an intraoral approach. Healing was uneventful. The growth deviation was arrested.
UNASSIGNED: Intraoral approach is a good option to remove an accessory maxilla. Tessier type-7 cleft may be accompanied by type-5 clefts and such accessory structures when impinging on vital structures such as temporomandibular joint or facial nerve should be immediately removed to facilitate proper form and function.

Tessier no. 7 clefts are characterized by macrostomia, facial muscular diastasis and maxillary and zygomatic bone abnormalities. It is caused by a lack of ectomesenchyme formation or penetration of the maxillary and mandibular processes during the fourth and fifth weeks of development. A case of bilateral transverse facial cleft with an accessory maxilla and an osseous choristoma is presented. The diagnosis of accessory maxilla was based on clinical findings due to the inaccessibility of orthopantomography and computed tomography scan. Orbicularis oris muscle reconstruction, cheiloplasty and excision of accessory maxilla were done. Histopathological examination of the bony lesion showed an osseous choristoma. There were no postoperative complications or local recurrence of the lesion excised. This case report demonstrates the importance of early diagnosis and intervention in maxillofacial congenital anomalies. Cheiloplasty restores function and gives the patient a natural appearance. The excision of accessory bone prevents further complications in the child\'s growth.

Bilateral Tessier no. 7 clefts are rarely reported in the literature. Here, we describe the presence of accessory maxilla with supernumerary teeth in a patient who exhibited bilateral Tessier no. 7 clefts; the diagnosis was established based on the patient\'s history, clinical presentation, and computed tomography images. A review of the available literature revealed 24 patients with Tessier no. 7 clefts from 2000 to 2020, including our patient. The most common clinical manifestation in patients with Tessier no. 7 clefts comprises bilateral facial clefts. Additionally, Tessier no. 7 clefts are more frequently found in boys or men, rather than in girls or women. The presence of an accessory maxilla with supernumerary teeth in a patient with bilateral Tessier no. 7 clefts is extremely rare. Early detection of craniofacial abnormalities is important, because it may influence patient prognosis and management.

Cleft lip and palate (CLP) are commonly occurring birth defects which occur due to failure of fusion of various developmental processes of the face, leading to a visible defect in the lip and palate. In severe cases, this defect may extend over the face, thus causing facial clefts. The present case of a 7-year-old girl is unique in the sense that she has right and left side facial clefts of different severity with bilateral accessory maxilla, extra set of dentition, unilateral CLP of the right side, and a double soft palate. Other less associated findings in the case are severe maxillary protrusion, complex open bite, downward and backward rotation of mandible, and incompetent lips with apparently no limb deformities. With some relevant data in hand, we present this case for various suggestions and best possible treatment plan.

A Tessier no. 7 cleft is a lateral facial cleft which originates from the oral cavity and extends towards the tragus, involving both soft-tissue and skeletal components. A male patient presenting with both maxillary jaw duplication and bilateral Tessier no. 7 clefts, which has been reported only twice in the literature, is described. Bilateral facial clefts, macrostomia and chondro-cutaneous remnants were noted, which were repaired and resected. With further growth, facial asymmetry and asymmetric facial nerve dysfunction became apparent. Radiographic examination showed an accessory maxillary jaw and a flattened and hypoplastic right coronoid process. A maxillary alveolar cleft was also present between the left second bicuspid and the second permanent molar. This case may represent an under-recognized phenotype with an unusual combination of maxillary jaw duplication, macrostomia, Tessier no. 7 clefts, and chondro-cutaneous remnants. A long-term follow-up of these patients is recommended as they often develop craniofacial deformities later in life.