Oral and Maxillofacial Management in Patients With Congenital Insensitivity to Pain and Anhidrosis (CIPA).

PURPOSE: This retrospective cross-sectional study aims to elucidate the challenging oral and maxillofacial management associated with congenital insensitivity to pain with anhidrosis (CIPA), emphasizing the need for early intervention and specialized treatment approaches.

METHODS

Conducted at the Soroka University Medical Center, this study spans from 2005 to 2024, examining a significant cohort within a high-prevalence Bedouin community. We analyzed clinical outcomes focusing on dental extractions, incidence of osteomyelitis (OM), and subsequent mandibular fractures.

RESULTS

The study included 36 patients diagnosed with CIPA, highlighting a high incidence of early-onset dental extractions and OM leading to various complications, including pathological mandibular fractures. Specifically, 63.9% of the patients underwent tooth extractions, primarily due to self-mutilation and infection. The average age at first tooth extraction was remarkably young at 2.23 years. Pathological fractures were observed in several cases, emphasizing the progressive and destructive nature of the disorder's impact on oral structures.

CONCLUSION

Given the escalating nature of oral and maxillofacial manifestations in CIPA, there is a critical need for early dental intervention and the adoption of preventive strategies. This study advocates for a multidisciplinary approach to enhance long-term outcomes, integrating oral and maxillofacial surgeons, pediatricians, geneticists, and rehabilitation specialists in the care of these patients.