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Original Article
88 (
4
); 483-493
doi:
10.25259/IJDVL_1203_20
pmid:
35138068

Clinical and radiological assessment of rhinomaxillary syndrome in Hansen’s disease

Postgraduate Programme in Infectious Diseases, Universidade Federal do Espírito Santo, Vitória, Espírito Santo, Brazil
Department of Imaging, Hospital Israelita Albert Einstein, São Paulo, Brazil
Department of Internal Medicine, Universidade Federal do Espírito Santo, Vitória, Espírito Santo, Brazil
Department of Social Medicine, Universidade Federal do Espírito Santo, Vitória, Espírito Santo, Brazil
Department of Oral and Maxillofacial Surgery, University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom
National Infection Service, Public Health England, London, United Kingdom
Corresponding author: Prof. Patrícia Deps, Departamento de Medicina Social, Centro de Ciências da Saúde, Avenida Marechal Campos, 1468. Maruípe, Vitória, Espírito Santo, Brazil. patricia.deps@ufes.br
Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: do Espírito Santo RB, Serafim RA, Loureiro RM, Sumi DV, de Mello RA, Nascimento IF, et al. Clinical and radiological assessment of rhinomaxillary syndrome in Hansen’s disease. Indian J Dermatol Venereol Leprol 2022;88:483-93.

Abstract

Background

More than four million people today live with Hansen’s disease, and 200,000 new cases are diagnosed every year. Lifetime effects of Hansen’s disease manifest as changes to bones of the face, hands and feet, resulting in physical impairment, secondary complications and facial changes that can be detrimental to quality of life, particularly among the elderly.

Aims

This study aimed to perform a detailed characterization of rhinomaxillary syndrome and its clinical manifestations in older persons treated in the past for Hansen’s disease.

Methods

This was a cross-sectional study to characterize rhinomaxillary syndrome among older persons (age 60+ years) resident at Pedro Fontes Hospital, Cariacica, Espírito Santo, Brazil. Computed tomography images were examined with three-dimensional reconstructions to assess alterations to maxillofacial bones according to criteria for radiological rhinomaxillary syndrome. Participants were examined to assess facial alterations according to criteria for clinical rhinomaxillary syndrome.

Results

Rhinomaxillary syndrome was investigated in 16 participants (ten females and six males), median age 70 (range 60–89) years, age at diagnosis 20 (6–43) years and time since diagnosis 46 (26–70) years. Four participants fully met radiological rhinomaxillary syndrome criteria, four partially. All participants with full radiological rhinomaxillary syndrome presented with facial changes which met criteria for clinical rhinomaxillary syndrome, including “saddle nose” (loss of nasal dorsal height and shortened length of nose, due to cartilaginous and/or bone collapse), concave middle third of the face with sunken nose, maxillary retrognathia and inverted upper lip.

Limitations

Clinical histories were incomplete for some participants because records were lost at the hospital over time.

Conclusion

Until Hansen’s disease is eliminated from endemic countries, persons affected will continue to present with rhinomaxillofacial alterations caused by Mycobacterium leprae infection. Clinical protocols for assessment and long-term care need to include otorhinolaryngological evaluation, mainly to prevent secondary complications. When rhinomaxillofacial bone changes are suspected, this evaluation should be supported by computed tomography imaging, if available.

Keywords

Facial bones
facial profile
Hansen’s disease
rhinomaxillary syndrome
tomography

Plain Language Summary

Hansen’s disease, also known as leprosy, is an infectious disease caused by a bacterium, Mycobacterium leprae. More than 200,000 new cases of Hansen’s disease are diagnosed each year worldwide. If the disease is diagnosed too late, it can alter bones in the skull, leading to facial changes and disfigurement. These are associated with stigma and with other medical complications. These bone alterations have been examined in skulls from archaeological sites, and the associated changes have been called “rhinomaxillary syndrome.” In living persons affected by Hansen’s disease, rhinomaxillary syndrome can be assessed by computed tomography imaging of maxillofacial bones, but a simpler method of looking at external changes to the face could also be useful in clinical practice, particularly in the countries where Hansen’s disease is endemic. The authors used both techniques in a group of elderly people from Brazil to evaluate a set of diagnostic criteria based on changes to the face which matched the underlying bone alterations. This simpler method could be incorporated into clinical guidelines to improve the assessment and care of persons affected by Hansen’s disease. When rhinomaxillofacial bone changes are suspected, clinical evaluation should be supported by computed tomography imaging where these facilities are available.

Introduction

Delayed diagnosis of Hansen’s disease increases the risk of disabilities.1-4 Hansen’s disease-related changes to bones, particularly of the hands, feet and face,5 impact on quality of life and contribute to stigmatization.6,7 Rhinomaxillofacial changes caused by Mycobacterium leprae infection of the nasal passages and the palate includes collapse of the bridge of the nose, resorption of the central part of the maxilla and inflammation of the floor and walls of the nasal cavity and hard palate.8,9 The degree of bone alteration correlates with the type of the disease at diagnosis, ranging from little or no detectable change in the tuberculoid type to severe changes at the “lepromatous” pole.8,9

Rhinomaxillary syndrome was defined in 1992 to identify Hansen’s disease in skulls from archaeological sites based on changes to the pyriform aperture, orbital bones and alveolar process of the maxilla.10-12 Rhinomaxillary syndrome criteria may have clinical utility in providing a standardized method for grading rhinomaxillofacial changes in persons affected by Hansen’s disease.13

The aim of our study was to perform the first in vivo investigation of rhinomaxillary syndrome using computed tomography imaging with three-dimensional reconstructions, to determine whether rhinomaxillary syndrome correlated with a clinical definition based on facial profile alterations.

Methods

Participants

Older persons (age 60+ years) affected by Hansen’s disease resident at the Hospital Colônia Pedro Fontes, Espírito Santo, Brazil, were invited to participate when they required medical assessment as part of routine care between September 2015 and December 2016. Demographic data and medical history were obtained by interview and from medical records. All participants received general medical, dermatological and otorhinolaryngological examinations.

Computed tomography imaging

Participants attended the Radiology and Computed Tomography Service at Hospital Universitário Cassiano Antônio Moraes, Espírito Santo, Brazil, for computed tomography scans of the head. Images were acquired in a 64-section multidetector computed tomography scanner (Aquilion, Toshiba Medical Systems Corp., Tochigi, Japan) with parameters 120 kVp, 100 mAs, 1.125 mm spiral pitch factor. Field of view was limited to the maxillofacial area. Three-dimensional reconstructions and cephalometrics were generated from volumetric data by DePuy Synthes TRUMATCH CMF Solutions (DePuy Synthes Inc., PA 19380, USA). Cephalometrics comprised maxillary height and width, nasofrontal angle, ANS angle (A=A point, N=nasion and S=Sella turcica mid-point) and maxillary volume measured at Le Fort 1 level.

Rhinomaxillary syndrome

Rhinomaxillary syndrome is defined by changes to: (I) anterior nasal spine; (II) alveolar processes of maxilla; (III) nasal surface of the palatine process of the maxilla; (IV) oral surface of the palatine process of the maxilla; (V) nasal turbinates and septum; (VI) pyriform aperture and (VII) posterior alveolar margins of the maxilla.11 To identify rhinomaxillary syndrome from computed tomography images, we combined criteria III and IV because both sides of the hard palate can be examined in physical remains but not in computed tomography images. To criteria VI, we added resorption of the nasal bones which are part of the pyriform aperture but usually absent from paleopathological specimens. Abnormalities were graded as “severe” or “mild to moderate” [Figure 1]. Full or partial radiological rhinomaxillary syndrome was based on assessment of the number and severity of alterations. “Fully met” required severe resorption of anterior nasal spine (criterion I) and severe loss of sharpness of pyriform aperture and/or resorption of nasal bones (criterion VI) plus at least one of the other criteria rated severe and one rated mild to moderate. Partial radiological rhinomaxillary syndrome typically required two–three severe plus two–three mild-to-moderate bone alterations. Computed tomography images were examined independently and in parallel by two radiologists.

Figure 1:: Grading by computed tomography imaging of maxillofacial bone alterations in rhinomaxillary syndrome

Clinical rhinomaxillary syndrome

Assessment of clinical manifestation of rhinomaxillary syndrome was based on clinical rhinomaxillary syndrome criteria comprising four facial profile alterations: (1) saddle nose, characterized by loss of nasal dorsal height and shortened length of nose, due to cartilaginous and/or bone collapse; (2) concave middle third of the face with sunken (retracted) nose, caused by erosion of the zygomatic process and enlargement and loss of the pyriform shape of the nasal aperture; (3) reduced maxillary projection (maxillary retrognathia/reduced ANS) and (4) inverted upper lip because of reduced maxillary height.13 Alterations were scored: saddle nose (no = 0, mild = 1, moderate = 2 and severe = 3); concave middle third of face with sunken nose (no = 0, either = 1 and both = 2); maxillary retrognathia (reduced ANS) (no = 0 and yes = 1) and inverted upper lip (no = 0 and yes = 1). The sum of scores was categorized: normal = 0, mild = 1, moderate = 2–4 and substantial (clinical rhinomaxillary syndrome) = 5–7.

Ethical Approval

This study was approved by the Research Ethics Committee of the Health Sciences Center of the Federal University of Espírito Santo (no. 4.248.419, August 31, 2020). Informed consent including for use of images was obtained from all participants.

Results

Participants

Sixteen former Hansen’s disease patients participated (ten females and six males) with median age 70 (range 60–89) years, age at diagnosis 20 (6–43) years and time since diagnosis 46 (26–70) years. Original Hansen’s disease diagnosis by Madrid Classification was the “lepromatous” form in 13 patients, “borderline” in two and “tuberculoid” in one patient [Table 1]. Most patients had received two–three courses of dapsone monotherapy during the 1960s to 1980s; seven had received multidrug therapy (dapsone, rifampicin and clofazimine). Type 1 and 2 reactions before, during or after Hansen’s disease treatment were recorded for ten patients.

Table 1:: Diagnosis, treatment history and reactions in older persons affected by Hansen’s disease
ID Age Sex Madrid classification at diagnosisa Age at diagnosis Delay (months)b Monotherapy start year (drug)c Months of monotherapy (drug)c Multidrug therapy start year Months of multidrug therapy Hansen’s disease reactionsd Reaction therapye
Before During After
P1 87 M L 42 1970 (D) 144 (D) 2000 12 II P, T
P2 77 M L 27 1977 (D) 156 (D) None III P
P3 89 M L 43 1978 (D) None II P, T
P4 65 F T 20 120 I P
P5 62 F L 25 2 1978 (D) 1990 (R) None II II P, T
P6 68 F B 13 1975 (D) None II T
P7 77 F L 31 60 1975 (C) 1979 (R) 1983 (D) 300 (D) Yes II II T
P8 76 M L 6 12 1969 (D) 1984 (C) 1984 (R) 252 (D) Yes
P9 79 F L 9 1960 (D) 1984 (C) 1990 (D) None
P10 79 F L 11 1959 (D) 1975 (C) 1979 (R) Yes
P11 60 F L 17 3 1976 (D) 1979 (R) 180 (D) 36 (R) Yes
P12f 70 F L 17 96 I P
P13 70 M L 26 1963 (D) 1984 (R) 1988 (C) 300 (D) 60 (R) 36 (C) Yes
P14 76 F L 23 1968 (D) 1984 (C) 1987 (R) 60 (D) n/r (C)
24 (R) Yes
P15 61 F L 15 48 1972 (D) 1975 (C) 1978 (D) 36 (D) 180 (C) 96 (D) None II T
P16 66 M B 40 36 1970 (D) 1980 (C) 120 (D) None II II P, T
Madrid classification: L=“lepromatous;” B= “borderline;” T= “tuberculoid”. bDelay in diagnosis was defined as being the time (in months) from awareness of the first symptom to the start of treatment. cDrugs: D=dapsone; R=rifampicin; C=clofazimine. dHansen’s disease reactions: type I (“reversal reaction”); type II (erythema nodosum leprosum); type III (isolated neuritis). eHansen’s disease reaction treatments: P=prednisone; T=thalidomide. fThis person came from Rio de Janeiro, therefore, data are incomplete

Maxillofacial bone changes

All participants had alterations to the alveolar process of maxilla to varying degrees (eight severe and eight mild to moderate) [Table 2]. The second most frequent alteration was resorption of the anterior nasal spine, observed in 12/16 participants (six severe). Resorption of nasal bones was observed in ten/16 participants (five severe), loss of sharpness of the pyriform aperture in ten/16 (five severe) and atrophy of the inferior and middle nasal turbinates in eight/16 (one severe) and seven/16 (two severe). Three participants had severe perforation of the nasal septum and eight had thinning of the hard palate with bony discontinuities [Table 2]. None had perforated palate.

Table 2:: Evaluation of maxillofacial bone alterations and diagnosis of radiological rhinomaxillary syndrome in older persons affected by Hansen’s disease
ID Frontal,
zygomatic,
lacrimal
bones
Anterior nasal spine Alveolar
process of maxilla (anterior)
Hard palate (palatine process of maxilla) Nasal
septum
Inferior
nasal
turbinates
Middle
nasal
turbinates
Pyriform
aperture
Nasal
bones
Alveolar
process of maxilla (posterior)
Radiological
rhinomaxillary
syndromea
Figure
I II III + IV V VI VII
2.1.1 2.1.2 2.1.3 (nasal)
2.1.4 (oral)
2.1.5 2.1.6 2.1.7
Osteitis Resorptionb Resorptionb Thinning with bony discontinuities Perforationb Atrophyb Atrophyb Loss of sharpnessb Resorptionb
P1 Yes ++ ++ Yes ++ ++ ++ ++ ++ + Full Figure 2
P2 No ++ + No ++ + + ++ + - Full
P3 No + + No - + + - - + No
P4 No - ++ No - + - - - + No
P5 No + ++ Yes - - - - - ++ No
P6 No - ++ Yes - - - - - ++ No
P7 No ++ + Yes ++ + + ++ + + Full Figure 3
P8 No ++ + No + + + ++ ++ ++ Full Figure 4
P9 No ++ ++ Yes - + + + + ++ Partial
P10 No ++ ++ No - - - + ++ + No Figure 5
P11 No + ++ Yes - - - + ++ ++ Partial
P12 No + + No - - - - - + No
P13 Yes - + No - - ++ + - + Partial
P14 No - ++ Yes - - - - + ++ No
P15 No + + No - - - ++ ++ - Partial
P16 No + + Yes - + - + + ++ No
Radiological rhinomaxillary syndrome adapted from criteria defined by Anderson and Manchester (see Methods). b-None, + mild to moderate, ++ severe

Clinical rhinomaxillary syndrome and radiological rhinomaxillary syndrome

Four participants (P1, P2, P7 and P8) fully met the criteria for radiological rhinomaxillary syndrome [Table 2], all of whom had facial profile changes which met criteria for clinical rhinomaxillary syndrome [Table 3]. Four participants (P9, P11, P13 and P15) had partial radiological rhinomaxillary syndrome, of whom three had moderate and one had mild facial profile changes. Three cases (P1, P7 and P8) with bone alterations meeting criteria for full radiological rhinomaxillary syndrome are illustrated in Figures 2-4, together with one case (P10) with bone alterations which did not meet radiological rhinomaxillary syndrome criteria [Figure 5]. Cephalometrics for P1, P7, P8 and P10 are reported in Table 4.

Figure 2a:: Patient (P1), male, age 87, with full rhinomaxillary syndrome: frontal facial aspect showing facial profile changes comprising severe saddle nose which was sunken in concave middle third of face and maxillary retrognathia with inverted upper lip, meeting clinical rhinomaxillary syndrome criteria
Figure 2b:: Patient (P1), male, age 87, with full rhinomaxillary syndrome: coronal computed tomography image showing atrophy of the middle and inferior nasal turbinates (long arrows), a large perforation in the nasal septum (star), thinning of the hard palate with focal discontinuities (short arrows), as well as thickening and sclerosis of zygomatic and frontal bones, consistent with osteitis (arrowheads)
Figure 2c:: Patient (P1), male, age 87, with full rhinomaxillary syndrome: three-dimensional reconstruction computed tomography image (anterior view) showing loss of sharpness of the pyriform aperture, deformity and resorption of the nasal bones (arrow) and marked, crescentic resorption of the central part of the alveolar process of the maxilla (arrowhead)
Figure 2d:: Patient (P1), male, age 87, with full rhinomaxillary syndrome: lateral facial aspect showing substantial facial profile changes including severe saddle nose
Figure 2e:: Patient (P1), male, age 87, with full rhinomaxillary syndrome: sagittal computed tomography image showing marked resorption of the anterior nasal spine (arrowhead) and a large perforation in the nasal septum (star)
Figure 2f:: Patient (P1), male, age 87, with full rhinomaxillary syndrome: three-dimensional reconstruction computed tomography image (lateral view) showing marked resorption of the anterior nasal spine (arrowhead) and nasal bones (arrow), as well loss of sharpness of the pyriform aperture (dashed line)
Figure 3a:: Patient (P7) female, age 77, with full rhinomaxillary syndrome: frontal facial aspect showing facial profile changes including moderate saddle nose which was sunken in concave middle third of face, and maxillary retrognathia with inverted upper lip, meeting clinical rhinomaxillary syndrome criteria
Figure 3b:: Patient (P7) female, age 77, with full rhinomaxillary syndrome: coronal computed tomography image showing resorption of the middle and inferior nasal turbinates (arrows) and a large perforation in the nasal septum (star)
Figure 3c:: Patient (P7) female, age 77, with full rhinomaxillary syndrome: three-dimensional reconstruction computed tomography image (anterior view) showing resorption of the nasal bones (arrow) and alveolar process of the maxilla (arrowhead)
Figure 3d:: Patient (P7) female, age 77, with full rhinomaxillary syndrome: lateral facial aspect showing substantial facial profile changes including a sunken and folded nose
Figure 3e:: Patient (P7) female, age 77, with full rhinomaxillary syndrome: sagittal computed tomography image showing marked resorption of the anterior nasal spine (arrowhead) and a large perforation in the nasal septum (star)
Figure 3f:: Patient (P7) female, age 77, with full rhinomaxillary syndrome: three-dimensional reconstruction computed tomography image (lateral view) showing marked resorption of the anterior nasal spine (arrowhead), resorption of the nasal bones (arrow) and loss of sharpness of the pyriform aperture (dashed line)
Figure 4a:: Patient (P8) male, age 76, with full rhinomaxillary syndrome: frontal facial aspect showing facial profile changes including moderate saddle nose which was sunken in concave middle third of face, and maxillary retrognathia with inverted upper lip, meeting clinical rhinomaxillary syndrome criteria
Figure 4b:: Patient (P8) male, age 76, with full rhinomaxillary syndrome: coronal computed tomography image showing variable atrophy of the middle and inferior nasal turbinates (arrows)
Figure 4c:: Patient (P8) male, age 76, with full rhinomaxillary syndrome: three-dimensional reconstruction computed tomography image (anterior view) showing resorption of the nasal bones (arrow) and alveolar process of the maxilla (arrowhead)
Figure 4d:: Patient (P8) male, age 76, with full rhinomaxillary syndrome: lateral facial aspect showing substantial facial profile changes including a sunken and folded nose
Figure 4e:: Patient (P8) male, age 76, with full rhinomaxillary syndrome: sagittal computed tomography image showing marked resorption of the anterior nasal spine (arrowhead) and a perforation in the nasal septum (star)
Figure 4f:: Patient (P8) male, age 76, with full rhinomaxillary syndrome: three-dimensional reconstruction computed tomography image (lateral view) showing marked resorption of the anterior nasal spine (arrowhead) and nasal bones (arrow), as well loss of sharpness of the pyriform aperture (dashed line)
Figure 5a:: Patient (P10) female, age 79, without rhinomaxillary syndrome: frontal facial aspect showing normal facial profile
Figure 5b:: Patient (P10) female, age 79, without rhinomaxillary syndrome: coronal computed tomography image showing normal middle and inferior nasal turbinates (arrows)
Figure 5c:: Patient (P10) female, age 79, without rhinomaxillary syndrome: three-dimensional reconstruction computed tomography image (anterior view) showing resorption of the nasal bones (arrow) and the anterior region of the alveolar process of the maxilla (arrowhead)
Figure 5d:: Patient (P10) female, age 79, without rhinomaxillary syndrome: lateral facial aspect showing normal facial profile
Figure 5e:: Patient (P10) female, age 79, without rhinomaxillary syndrome: sagittal computed tomography image showing marked resorption of the anterior nasal spine (arrowhead) and no perforation in the nasal septum
Figure 5f:: Patient (P10) female, age 79, without rhinomaxillary syndrome: three-dimensional reconstruction computed tomography image (lateral view) showing marked resorption of the anterior nasal spine (arrowhead), resorption of the nasal bones (arrow) and loss of sharpness of the pyriform aperture (dashed line)
Table 3:: Diagnosis of clinical rhinomaxillary syndrome in older persons affected by Hansen’s disease
ID 1. Saddle nose 2. Concave middle third of the face with sunken nose 3. Maxillary retrognathia (reduced ANS)a 4. Inverted upper lip Overall grading of facial changes (score) Clinical rhinomaxillary syndromeb Radiological rhinomaxillary syndromec
P1 Severe Yes Yes Yes Substantial (7) Yes Full
P2 Severe Yes No No Substantial (5) Yes Full
P3 No No Yes Yes Moderate (2) No No
P4 No No Yes No Mild (1) No No
P5 Mild No Yes No Moderate (2) No No
P6 No No Yes Yes Moderate (2) No No
P7 Severe Yes Yes Yes Substantial (7) Yes Full
P8 Moderate Yes Yes Yes Substantial (6) Yes Full
P9 Mild Yes Yes No Moderate (4) No Partial
P10 No No No No Normal (0) No No
P11 Mild No Yes Yes Moderate (3) No Partial
P12 No No No No Normal (0) No No
P13 Mild No No No Mild (1) No Partial
P14 No No Yes Yes Moderate (2) No No
P15 Moderate Yes No No Moderate (4) No Partial
P16 No No No No Normal (0) No No
ANS measures maxillary projection/retrusion relative to skull base, where A=A point (maximum concavity of maxilla, viewed sagittally), N = Nasion, S = Sella turcica mid-point (pituitary fossa). bClinical rhinomaxillary syndrome criteria as defined by the authors (see Methods). cRadiological rhinomaxillary syndrome adapted from criteria defined by Anderson & Manchester (see Methods)
Table 4:: Cephalometric measurements in three dimensional computed tomography images of older persons affected by Hansen’s disease
ID Age Sex Maxillary height (mm) Maxillary width (mm) Maxillary volume (cm3) Nasofrontal angle (°) ANSa (°) Clinical RMSb Radiological RMSc
P1 87 M 6.5 62.6 19.3 151.8 84.0 Yes Full
P7 77 F 19.8 53.9 9.0 150.3 82.4 Yes Full
P8 76 M 16.4 56.8 16.4 164.8 81.2 Yes Full
P10 79 F 24.5 61.4 29.6 152.7 85.2 No No
Maxillary projection/retrusion relative to skull base, where A = A point (maximum concavity of maxilla, viewed sagitally), N = Nasion, S = Sella turcica mid-point (pituitary fossa), bClinical rhinomaxillory syndrome criteria as defined by the authors (see Methods), cRadiological rhinomaxillory syndrome adapted from criteria defined by Anderson & Manchester (see Methods). Figures from left to right illustrating: maxillary height (vertical) and width (horizontal); nasofrontal angle; ANS angle (reduced ANS angle = maxillary retrognathia); maxillary volume (blue) measured at le fort 1 level (horizontal through piriform aperture)

Discussion

This group of elderly adults previously treated and cured of Hansen’s disease displayed at least one maxillofacial bone alteration consistent with the pathophysiological effects of clinical Mycobacterium leprae infection. All participants had alterations to the alveolar process of the maxilla and most presented with resorption of the anterior nasal spine, but the number of other bones affected and the degree of alterations varied widely. Maxillofacial bone alterations in four participants met the criteria for radiological rhinomaxillary syndrome fully, while four participants had partial radiological rhinomaxillary syndrome. Participants with full radiological rhinomaxillary syndrome presented with substantial facial changes including saddle nose, concave middle third of face with sunken nose, inverted upper lip and maxillary retrognathia. In all four full radiological rhinomaxillary syndrome patients, these alterations met a set of criteria defining “clinical RMS.”

Maxillofacial bone changes assessed in vivo by computed tomography imaging have been described in only one previous study.14 This study of ten former Hansen’s disease patients residing in a National Sanatorium in Japan reported a range of alterations similar to those observed in our participants with four of ten participants having severe maxillary changes and saddle nose, and three of ten having no bone alterations.

Strengths and weaknesses

A limitation of our study is the lack of a control group to differentiate age-related changes to the maxilla. However, Kasai et al. reported that Hansen’s disease-related changes occur predominantly in the median frontal part with characteristic interruption of the U-shaped process, as we also observed in our participants, compared with the typical horizontal and vertical age-related atrophy of the maxillary alveolar processes.14 Furthermore, reported evidence of substantial age-related maxillary bone loss is limited, although some may be secondary to poor nutrition, loss of teeth (all participants had a partially or completely edentulous maxilla) and changes in mechanical function.15 In our cases of full and partial rhinomaxillary syndrome, maxillofacial bone changes were consistent with those considered to be pathognomonic of Hansen’s disease.8

We had incomplete clinical histories for some participants because records were lost over time. Resorption and atrophy of nasal bones can be a consequence of trauma which may not have been recorded in medical histories. Similarly, we did not have complete records to support our assessment of differential diagnoses such as syphilis and mucocutaneous leishmaniasis,11 and less common rhinological diseases such as granulomatosis with polyangiitis and nasal extranodal lymphoma.16 Although two researchers assessed and graded facial changes independently, this remains a subjective process, and our clinical rhinomaxillary syndrome criteria require validation.

Conclusion

Availability of computed tomography scanning even in endemic countries means that assessment of rhinomaxillofacial bone changes, where suspected, could become a component of specialist care, primarily to prevent secondary complications. We propose that clinical protocols for Hansen’s disease are extended to include otorhinolaryngological evaluation, supported by imaging where necessary. In the meantime, practical steps need to be taken at the health system level to ensure timely diagnosis and prompt treatment initiation to prevent Hansen’s disease-related disability.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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