Oral and Maxillofacial Manifestations of Kallmann Syndrome: A Systematic Analysis of the Literature.

Abstract

Aims: Kallmann syndrome (KS) is a rare genetic disorder characterized by congenital hypogonadotropic hypogonadism and varied clinical features. Despite its recognition, the oral and maxillofacial manifestations remain poorly understood. This study synthesized clinical aspects and management of KS-related oral and maxillofacial alterations.

Methods: Searches were conducted in the PubMed, Web of Science, Scopus, Embase, and LILACS databases, supplemented by manual scrutiny and gray literature. Case series and/or case reports were included. The Joanna Briggs Institute tool was employed for critical appraisal of the studies.

Results: A total of 46 studies comprising 108 cases were included. The mean age of individuals was 19.8 (±12.6) years, and there was a marked predominance of males (79.3%). Cleft lip/palate (32.7%) was the predominant oral condition, followed by high-arched palate (21.7%), and dental agenesis (19.8%). Oral treatment consisted of corrective surgery of the cleft lip and/or palate (n = 9), myoplasty (n = 1), and tooth extraction/orthodontic treatment (n = 1). Hyposmia/anosmia (71.3%) was the most frequently reported manifestation.

Conclusion: Early diagnosis and interdisciplinary collaboration are essential for addressing the complex nature of KS-related oral and maxillofacial alterations and improving patient outcomes. The scarcity of data on oral treatment underscores the need for additional research and clinical attention in this field.