Bilateral Maxillary Duplication in Tessier No. 7 Cleft: An Uncommon Congenital Deformity with a Challenging Radiological Diagnosis

TL;DR

This paper presents a rare case of bilateral maxillary duplication in a patient with Tessier No. 7 cleft, emphasizing the importance of radiological diagnosis for effective treatment.

Contribution

The study highlights key radiological features of a rare bilateral maxillary duplication in Tessier No. 7 cleft, offering insights for accurate diagnosis and treatment planning.

Findings

Bilateral maxillary duplication in Tessier No. 7 cleft is extremely rare, with only two prior cases reported.

Radiological imaging is crucial for diagnosing complex facial clefts when clinical evaluation is inconclusive.

The case underscores the need for detailed radiological assessment to guide therapeutic approaches in such rare deformities.

Abstract

Tessier No. 7 cleft, known as lateral facial cleft, is a rare and understudied entity with an incidence of 1/80,000–1/300,000 live births. Besides perioral tissue abnormalities manifesting as macrostomia, Tessier 7 cleft also involves anomalies of the underlying bony structures. It can appear as part of a syndrome, such as Treacher-Collins syndrome or Goldenhar/Orbito-Auriculo-Vestibular Spectrum, or as an isolated form (unilateral or bilateral) with variable expressions. Bilateral maxillary duplication in Tessier 7 cleft is considered extremely rare, accounting for only two previously presented cases. Given that the cases presented in the literature mainly focus on clinical appearance and surgical treatment, without providing sufficient imaging, we aim to present key radiological features of Tessier 7 cleft in terms of evaluating the involved structures, which is essential for the therapeutic approach and final outcome. A 17-year-old male with incompetent lips and orthodontic abnormalities was referred to our Radiology Department for orthopantomography (OPG) and CT examinations. Hetero-anamnestic data revealed a history of surgical treatment of the commissural cleft conducted 2 months after the birth to enable feeding. Intraoral examination showed a maxillary cleft and supernumerary teeth. Since the given clinical presentation was inconclusive, radiological diagnostics took precedence in elucidating this complex entity.

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