Name: RACHEL BERTOLANI DO ESPIRITO SANTO
Publication date: 06/10/2022
Advisor:
Name |
Role |
|---|---|
| PATRÍCIA DUARTE DEPS | Advisor * |
Examining board:
| Name |
Role |
|---|---|
| CARLOS GRAEFF TEIXEIRA | Internal Examiner * |
| MOISES PALACI | Internal Examiner * |
| PATRÍCIA DUARTE DEPS | Advisor * |
Summary: Context: Hansen`s disease (HD) is a chronically progressive, infectious disease that accounts for more than 200,000 new cases per year worldwide, and is more frequent in developing countries such as India, Brazil, and Indonesia. Skeletal deformities of the face and extremities are historical markers and part of the stigma of this disease. However, the underlying mechanisms of HD induced bone damage are not completely known, although it is known that lesions in the nasal and oral mucosa are the starting point for the development and progression of facial bone changes. The term "Facies leprosa", coined in 1952 by Møller-Christensen, author of the first paleopathology studies on HD, denominated a set of changes in the facial skeleton, being, however, replaced by rhinomaxillary syndrome (RMS), as proposed by Andersen and Manchester (1992), who systematized such changes into seven criteria. According to
paleopathology, facial deformity as a sequela of HD is caused not only by the "saddle nose" but also by the regression of the maxilla. Objective: Based on this, this study aimed to evaluate the clinical and bone alterations of the rhinomaxillary region in people affected by HD through physical examination and tomographic images. Methods: In an analytical cross-sectional study, 3 groups of participants were evaluated, 2 with cases (Group 1: 16 participants with more than 35 years of HD diagnosis, treated; Group 2: 21 participants with less than 15 years of HD diagnosis, HD, treated or undergoing treatment) and 1 with controls (37 participants without a HD diagnosis). In the cases, an otorhinolaryngological evaluation was carried out with rhinoscopy and oroscopy, in addition to a clinical evaluation of the facial profile (this
one, exclusively in Group 1). Participants-cases and controls underwent evaluation of CT scan of the facial bones. Based on the paleopathological RMS criteria by Andersen and Manchester (1992), the tomographic criteria for defining radiological RMS (rRMS) were established. The evaluation of the facial profile, through photos of the participants affected by leprosy, allowed the description of clinical RMS (cRMS). Results: Of the 16 participants in Group 1, 4 fully met criteria for rRMS, all had substantial facial changes that met criteria for cRMS, and another 4 had partial rRMS and did not meet criteria for cRMS. All cases with total and partial rRMS had an original diagnosis of lepromatous HD. The investigation of rhinomaxillofacial bone manifestations of HD
revealed that the differences in bone changes between the three groups were mainly determined by: (i) severe resorption/atrophy, more frequent in Group 1, with the anterior alveolar process of the maxilla showing severe resorption/atrophy in 50% of the participants, a percentage that was 29% in Group 2 and 11% in the controls; (ii) nasal bones and nasal aperture with severe resorption/atrophy in 31% in Group 1, being absent in Group 2, and, in Group 3, 2.7% (nasal bone) and 5.4% (nasal opening);(iii) severe or mild to moderate changes in the nasal septum in 25% of the cases in Group 1 compared to 4.7% of the participants in Group 2 and 2.7% in Group 3. The
rhinomaxillofacial bone change score was higher among participants with more than 35 years of diagnosis (Group 1, median 7, IQR 5.5-10.5) compared with those with less than 15 years of diagnosis (Group 2, median 5, IQR 3-7) and with participants without HD (Group 3, median 5, IQR 2-7). These findings are consistent with the fact that most cases in Group 1 were diagnosed and had treatment started in the pre-Multidrug therapy (MDT) era, and a long time has elapsed since then, which allowed greater disease progression, despite later, some have received MDT. With respect to dental data, there were no differences between participants in Groups 1 and 2, but controls had less tooth loss (mean number of missing maxillary teeth 12, IQR 4-16, p
