- Email: [email protected]
Lesions of the facial and trigeminal nerve in leprosy
i« J. Oral Surg, 1982: 11: 14-20 (Keywords: leprosy,'
tlellr%g)';
anaesthesia; nerve.facial; nerl'e, trigeminal)
Lesions of the facial and trigeminal nerve in leprosy An evaluation of 43 cases
PETER A. REICHART, SAMPAN SRISUWAN AND DATE METAH
Klinik fill' Mund-, Kiefer- und Gestchtschirurgie, Medizinische Hochschule Hannover, FRO and Faculty of Dentistry, Chiang Mai University, Thailand
Facial and trigeminal nerve involvement was studied in 43 leprosy patients, 5.7% of the 750 Thai national in-patients of a leprosy rehabilitation centre. Involvement of the facial nerve occurred late in the disease and had an average duration of 12.1 years. The zygomatic branches of this nerve were the most frequently affected. Hypaesthesia and anaesthesia were most often observed in the maxillary divisions. Periorbital surgical procedures to prevent severe ocular complications are evaluated and discussed. ABSTRACT -
(Receivedfor publication 25 September 1980, accepted 19 June 1981)
Leprosy, an infectious disease caused by Mycobacterium leprae, is characterised by
dermal and peripheral nerve lesions. Although there is little evidence ofleprous involvement of the central nervous system, affection of peripheral nerves, such as the facial and trigeminal nerves, is a frequent complicauon--v. DIWAN7 reported facial paralysis in 3% of leprosy patients and in 24.59% of those with the tuberculoid form, In a study of 70 cases of different types of leprosy", 19.8% were found to have facial paralysis, prevalence being amongst the borderline patients. Although affection of the facial nerve was also seen in. cases of lepromatous leprosy, this only occurred during the later stages of the disease, Whenever the nerve has been involved once, especially in the tuberculoid form", paralysis may result within 3 to 4 weeks", 0300-9785/82/010014-07$02.50/0
While all branches of the facial nerve may be affected during the course ofleprosy, especially during erythema nodosum leprosum reactions, the zygomatic branch has been seen to be the most often involved'. Since areas oflow surface temperature appear more prone to leprous affectiorr'>', late involvement or sparing of the deeply placed buccinator muscle is regarded as an example of the possible role of temperature in the affection of nerves". According to a WHO reporr", involvement of of the lower branches of the facial nerve appears to be more common in the yellow and white races. If the branches supplying the orbicularis oculi, frontalis and procerus muscles are affected, lagophthalmos and ectropion of the lower eyelid occur',S,I3,15,2'. The loss of corneal protection leads to exposure keratitis, corneal ulceration and perforation, hypopyon, en-
© 1982 Munksgaard, Copenhagen
15
NERVE LESIONS IN LEPROSY
dophthalmitis, secondary glaucoma and blindness. While lagophthalmos occurred in 14.2% of 70 Thai patients'S, this was seen in only 5.88% of 85 Indian patients', Since lagophthalmos and ectropion are generally considered to be particularly dangerous in leprosy, early surgical intervention is mandatory. Lateral or medial blepharoplasty does not lead to functional closure of the lids and these methods are thus considered only as a preparatory measure in advanced cases of ectropion':". The temporalis transfer operation which permits functional movement of the eyelids was first described by GILLIES9 , while modifications of the temporalismusculo-fascial-sling have been suggested by other authors 2 , 12, 13. Paralysis of the orbicularis oris muscle, although not as common as mid-facial paralysis, also results in serious functional disturbances'", Besides facial disfiguration, difficulties in phonation and, in advanced cases, drooling may be seen. Hypaesthesia or anaesthesia resulting from involvement of the trigeminal nerve have been observed in tuberculoid leprous macules, and infiltrations occur earlier than in borderline and lepromatous forms of leprosy', This team recorded the extent of facial sensory loss in II patients, and found that the maxillary region was always involved. Among 70 Thai patients,
hypaesthesia or anaesthesia was seen in only 5 cases'S. Affection of the sensory supply for the cornea, which comes from the ophthalmic division of the trigeminal nerve via the ciliary ganglion and the short ciliary nerve, can lead to impairment of corneal sensitivity. Anaesthesia of the cornea combined with facial paralysis of the zygomatic branches may give rise to severe ophthalmic complications'V",
Material and methods The study was undertaken at the McKean Rehabilitation Institute, Chiang Mai, Thailand, to determine the occurrence, prevalence and nature of facial and trigeminal nerve lesions in relation to type and duration ofleprosy and its treatment. Of750 inpatients, 43 with facial nerve lesions were examined. These cases were grouped according to the classification suggested by RIDLEY & JOPLlNG'o, using clinical examinations and skin smears (bacillary index; BI). Each patient was examined for changes as follows. I. Facial nerve. Its function was clinically tested by asking the patient to activate the muscles of facial expression. The functions of the frontal and zygomatic facial muscles, and of the muscles of the upper and lower lip were examined separately on the right and left side of the face. Facial paralysis was classified as unilateral, bilateral, complete or incomplete. The average duration of facial palsy, presence of lagophthalmos and blindness was registered. All functional impairments of the facial nerves were photographed.
Table 1. Clinical data of 43 leprosy patients Race Thai: 38 Chinese: 3 Hill tribes: 2
Sex male: 31 female: 12
Age max: 89 years min: 28 years Mean: 51.2 years
Type of leprosy
Duration of leprosy max; 50 years min: 2 years mean: 29.7 years
Duration of treatment max: 30 years min: 3 months mean: 12.1 years
ENL (last 12 months) 7 cases
B.I. (positive) 6 cases (between 0.2 and 3.7)
IT: 8 BT: 20
BB: I BL: 6 LL: 8 Type of medication D.D.S.: 35 D.D.S./Lamprene®; 5 Lamprene'P: 3
16
REICHART, SRISUWAN AND METAH
2. Trigeminal nerve. Sensory charting wascarried out by using a pin-prick test and a pieceofcotton wool to grossly differentiate between anaesthesia and hypaesthesia. The area of reduced sensibility was marked on the face which was then photographed. The ears were not examined. 3. Periorbital surgical procedures were recorded and divided into blepharoplasty, temporalis-musculofascial-sling operation and a combination of both procedures. The effectiveness of each operation was assessed by asking the patient to closehis eyes. If the eyelids met, the effect of surgery was considered good. If the lids were not more than 2 mm apart, this was acceptable, and ifmore than 2 mm, the result was judged to be unsatisfactory.
Results Race, sex and age, type and duration ofleprosy, duration of treatment, type of medication, history of erythema nodosum leprosum in the 12 months prior to the study, and BI are shown in Table 1. A total of 43 patients was affected by facial paralysis. Unilateral involvement of the frontal right or left branches was seen as only partial paralysis in 5 patients; the others revealed bilateral partial and/or complete paralysis. The zygomatic branches were affected unilaterally in 11 cases (partial, 3; complete, 8). Unilateral paralysis of the upper lip was recorded in 10 patients (partial, 6; complete, 4) and unilateral
paralysis of the lower lip was seen in 6 cases (partial, 4; complete, 2). Only 4 patients showed affection ofjust one of the 8 possible sectors; in these, the zygomatic branch was involved by complete paralysis with Bell's phenomenon. The overall distribution of facial paralysis is given in Table 2. Complete paralysis of all facial nerve branches was seen in only one patient (case 6, 42-years-old, tuberculoid leprosy). This patient showed Bell's phenomenon and severe drooling; trigeminal nerve involvement was corn plete and a loss of taste was reported (Figs. 1 and 2). The average duration of facial palsy was 12.1 years, with a maximum of30years and a minimum of 1 month. Cases with the tuberculoid (TT), borderline-tuberculoid (BT) and borderline (BB) forms ofleprosy had facial
Table 2. Distribution of facial paralysis Right
Left
front
p: 11 c: 17
p: 12 c: 17
periorbit
p:
2
c: 29
p: 2 c: 32
p: 10 c: 12
p: 12 c: 7
p: 10 6
p: 10 c: 6
upper lip lower lip
c:
p: partial paralysis. c: complete paralysis.
Fig. 1. Case 26. All branches of the facial nerve are
affected. Facial anesthesia is complete and there is lossof taste. Inability to close the lips revealsmassive calculus accumulation and malposed teeth.
17
NERVE LESIONS IN LEPROSY
shown in Fig. 3.The frontal section of this nerve was affected in 13, the maxillary section in 21 and the mandibular section in 6 patients (Fig. 4). 3 patients with trigeminal nerve lesions had TT leprosy, 14had BT, 1 had BB, 4 had BL and 7 had the LL form. In contrast to the facial nerve lesions, little or no reliable information could be obtained concerning the onset of hypaesthesia and anaesthesia. A total of 17 patients had undergone periorbital surgery due to lagophthalmos and accompanying complications. In 7 of these, blepharoplasty had preceded the temporalismusculo-fascial-sling operation, while in 2 cases the latter had been performed twice. Two operations had been undertaken on one side in 5 patients. The average time interval between the operation and date of reexamination was 5.3 years for patients who had had one operation, and 9.5 years (I st operation) and 3.6 years (2nd
2. Case 26. Activation of the muscles of facial expression reveals Bell's phenomenon; drooling is observed. The upper left lip shows some residual muscularactivity. Fig.
paralysis with an average duration of I3.2 years, while for those with the borderline lepromatous (BL) and lepromatous (LL) types this was 10.3 years. Lagophthalmos with affection of one eye was seen in 10 cases and bilateral involvement was recorded in 16 patients. Blindness of one eye occurred in 6, blindness of both eyes in 4 cases. The average duration of blindness was 8.1 years. Five of these cases had TT and BT leprosy. One TT case (case 41) went blind without having any facial paralysis. In three cases, enucleation of the eye had been carried out (cases 7, 38, 39). Hypaesthesia and anaesthesia of the trigeminal nerve sectors were seen in 29 patients. The distribution of trigeminal nerve involvement is
39
3. Overall distribution of facial hypesthesia (stripes) and anesthesia (checks). Fig.
18
REICHART, SRISUWAN AND METAH
out of 9 cases showed good or acceptable closure of the eyelids (Fig. 5). The combination of temporalis-musculo-fascial-sling and blepharoplasty also showed poor results in 50% of the cases.
Discussion The racial spectrum of patients in this study is representative for the northern part of Thailand. The majority are Thai (38), while minor ethnic groups are represented by the Chinese (3) and the hill tribes (2) of the
Fig. 4. Case 28, male, aged 57, BT type of leprosy.
Bilateral complete facial paralysis of the frontal and zygomatic branches has resulted in bilateral lagophthalmos and blindness of the left eye. Sagging of facial skin and open mouth posture is noted.
operation) for patients who had undergone two. Results ofsurgical procedures according to type are given in Table 3. Relatively poor results had been obtained for blepharoplasty alone; only 3 Table 3. Results of periorbital surgery
t-m-f-s t-m-f-s + BP BP orbicularis oris
Good
Acceptable
Poor
1 4 1 2
3 2
2 6 6
2
plasty t-m-f-s = temporo-musculo-fascial-sling. BP= blepharoplasty,
Fig. 5. Case 37, female, aged 67, BL type ofleprosy.
The facial skin shows wrinkling; loss of eyebrows and eyelashes is seen. The zygomatic branch is affected on the left side; Bell's phenomenon is present. A lateral blepharoplasty had been performed with acceptable results. There is weakness in the innervation of the right upper lip. The area of anesthesia is drawn on the skin infraorbitally (left).
NERVE LESIONS IN LEPROSY
mountain region of North Thailand. The sex ratio (male :female, 2.58: 1) is comparable to that of an earlier study (1.69:1)18. The more apparent male predominance in the present study may be explained by environmental, biological and genetic factors", The average age (51.2 years) is high compared to that of a previous study (42.3 years)". The spectrum of forms of leprosy includes all types and represents a cross-section of permanent residents and temporarily admitted out-patients at the institution. The 43 patients with facial paralysis were selected from a total of about 750 permanant residents and temporarily admitted outpatients. In this study, 5.7% of the leprosy cases had facial nerve involvement; this figure is high compared to other studies in which only 2.2%17 or 3%7 of patients with leprosy had facial nerve involvement. Compared to the polar types (IT and LL) alone, prevalence is low because a 24.59% incidence of facial nerve affection has been recorded for patients with TT'o, and an incidence of 19.8%for patients who were mostly of the LL type's. However, if the spectrum of types ofleprosy is analysed, it appears that most cases with facial nerve involvement are seen in the TT and BT forms of leprosy (Table 1). The average duration of leprosy was 29.7 years, which correlates with the relatively high average age of51.2 years. The duration of treatment was ]2.1 years and the main type of medication was D.D.S. (diaminodiphenylsulphone). These findings correlate with a former study's. Those cases under treatment with Lamprenef were positive for erythema nodosum leprosum; these were also the active cases (6) with a positive BI for acid-fast bacilli. The average duration of facial nerve lesions was 12.1 years. In the TT and BT forms of leprosy, facial paralysis occurred 2.9 years earlier than in the BL and LL types; that nerves are generally affected later in the LL type ofleprosy is an accepted fact' ,6 • The facial nerve lesions observed in the present study were of a rather symmetrical distribution. Complete paralysis was found more frequently
19
in the frontal and, almost exclusively, in the maxillary and periorbital sections. Compared to the other sections, the maxillary (zygomatic) portion was affected most often, an observation which has also been made by other authors'. In contrast to Bell's palsy which normally shows involvement of the frontal and zygomatic paralysis is more a result of peripheral branch involvement. Also, unlike Bell's palsy, lower facial paralysis is less frequently seen. The involvement of the frontal and zyomatic branches can be explained by the relatively thin layer of soft tissue which overlies the zygomatic bone, and temperature reductions which have been shown to be an important factor in development of nerve lesions':", Recovery of facial nerve paralysis is possible in the early stages but may, however, result in misreinnervation as shown by voluntary cocontractions of physiologically unrelated muscles'<" . Lagophthalmos of one or both eyes was seen in 26 out of 43 patients with facial nerve involvement (60.5%). This figure is high and may be explained by the high average age of the patients selected and the long average duration of both leprosy (29.7 years) and facial nerve affection (12.1 years). Of the 750 patients at the institution, about 3.5% had lagophthalmos; this % is comparable to findings in Indians (5.88%)'. Blindness was only observed in long-standing cases, most of which were of the TT and BT forms of leprosy. The pattern ofhypaesthesia or anaesthesia is comparable to that of facial nerve lesions, since the frontal and maxillary division was also the most often affected. While reduction of sensitivity of the whole face seems uncommon (4 cases), smaller areas of reduction or loss of sensitivity are observed more frequently. As in facial paralysis, most cases ofhypaesthesia and anaesthesia are seen in the BT and LL types of leprosy. Nothing can be said about the prevalence of trigeminal nerve involvement, because control patients without facial paralysis were not examined. Lesions of the facial and
20
REICHART, SRlSUWAN AND METAH
trigeminal nerves do not necessarily occur together. Evaluation of the results of periorbital surgery indicates that blepharoplasty alone does not lead to sufficient protection of the eye (Fig. 4) . Ectropion and lagophthalmos persist, thus leading to the severe ocular complications which are generally feared in leprosy. The insufficiency of this operation in advanced cases of lagophthalmos has been described previously':", However, if in permanent partial paralysis sufficient tone is present in the orbicularis muscle to support the tarsal plate, blepharoplasty may be used as a definite operation" , The relatively poor results obtained by combined temporo-musculo-fascial sling and blepharoplasty must be explained by the in ad eq u a t e cooperation of the patients after surgery. This cooperation is mandatory since only active muscular exercise (temporalis muscle) can lead to effective closure of the eyelids.
References 1. ANISH, S. A.: The relationship between surface temperature and dermal invasion in lepromatous leprosy. Int . J. L epr. 1971: 39: 848-851. 2. ANTIA, N . H.: Reconstruction of the face in leprosy. Ann. Roy . Coil. Surg, Engl . 1963: 32: 7198. 3. ANTIA, N. H., D1VEKAR, S. C. & DASTUR, D. K.: The facial nerve in leprosy. Int. J. Lepr. 1966: 34: 103-177. 4. BRAND, P. W.: Temperature variation and leprosy deformity. Int. J. Lepr. 1959: 27: 1-6. 5. BROWNE, S. G .: Leprosy. Acta Clinica. J. R. Geigy S. A., Basle 1970. 6. BROWNE, S. G. : Leprosy - clinical aspects of nerve involvemen t. Contemp . Neural. Ser. 1975 : 12: 1-16. 7. DIWAN, V. S.: A survey of deformities in leprosy (with special reference to face). L epr. Re v. 1962 : 33: 255-262. 8. FRITSCHI, E. P.: Reconstructive surgery in leprosy. John Wright & Sons Ltd ., Bristol 1971. 9. GILLIES, H. D.: Plastic surgery of the eyelids and conjunctival sac. Trans . Ophthal. Soc. (UK) 1935 : 55 : 357-373.
10. HIDAKA, T .: Oral examination in leprosy patients. II. Leprous changes in the tissues of the oral cavity . Nagashirna Arch. Lepr. 1958: 4: 2842. 11. JOB, C. K. : Mechanism of nerve destruction in tuberculoid borderline Icprosy. An electron microscopic study. J . N eural. Sci. 1973: 20: 25-38. 12. JOHNSON, H. A. : A modification of the Gill ies' temporalis tr ansfer for the surgical treatment of the lagophthalmos of leprosy. Plast , Reconst. Sur g . 1962: 30: 378-382. 13. JOHNSON, H . A.: An operation to restore eyelid function lost in leprosy. Int. J. Lepr, 1965: 33: 8994. 14. LIGHTERMAN, J., WATANABE, Y. & HIDAKA, T.: Leprosy of the oral cavity and adnexa. Oral Surg, 1962 : IS: 1178-1194. 15. MANSON-BARR, P. : Manson's tropical diseases, 16th ed, Balliere, Tindall and Cassell, London 1966. 16. RANNEY, D. A., FURNESS, M . A. & SANTHANAKRISHNAN, C. K.: Misreinnervation in leprous neuritis affe cting the facial nerve. L ep R ev. 1972: 43: 151-158. 17. RANNEY, D . A . : The prevalence and consequences of mis-reinnervation of facial neuritis. Int. J. Lepr . 1974: 42: 316-322. 18. REICHART, P . : Facial and oral manifestations in leprosy. Oral Surg, 1976: 41: 385-399 . 19. REICHART, P.: Die chirurgische Korrektur des Lagophthalmus bei Leprapatienten. Fortsch r. Kiefer- Gesichtsch irurgie 1976 : 21: 27-29. 20. RIDLEY, D. S. & JOPLING, W. H. : Classification of lep rosy accord ing to immunity : a five-group system. Int . J. Lep r, 1966: 34: 255-273. 21. SABIN, T . D . & EBNER, J. D . : Patterns of sensory loss in lepromatous leprosy. Int. J. Lepr . 1969: 37: 239-248. 22. SEHGAL, V. N.: Occular changes in tuberculoid leprosy. Ind ian Dermatol. 1972: 17: 74-76. 23. WERNER, R. : Lepra im Mund-, Kiefer- und Gesichtsberelch. Disch. Zahnaeztl. Z. 1973: 28: 64-73. 24. WORLD HEALTH ORGANISATION, Scientific meeting on rehabilitation in leprosy. Veilore, India 1960. WHO Technical Report Series Nos. 221 , 1961. Address : Peter A. R eichart Klinik fiir Mund- , Kief er- und Gesicht schirurg ie Med iz inisclie Hoch schule Hannover 3000 Hanno ver 61 Karl- Wiechert-Allee 9' FRG
tlellr%g)';
anaesthesia; nerve.facial; nerl'e, trigeminal)
Lesions of the facial and trigeminal nerve in leprosy An evaluation of 43 cases
PETER A. REICHART, SAMPAN SRISUWAN AND DATE METAH
Klinik fill' Mund-, Kiefer- und Gestchtschirurgie, Medizinische Hochschule Hannover, FRO and Faculty of Dentistry, Chiang Mai University, Thailand
Facial and trigeminal nerve involvement was studied in 43 leprosy patients, 5.7% of the 750 Thai national in-patients of a leprosy rehabilitation centre. Involvement of the facial nerve occurred late in the disease and had an average duration of 12.1 years. The zygomatic branches of this nerve were the most frequently affected. Hypaesthesia and anaesthesia were most often observed in the maxillary divisions. Periorbital surgical procedures to prevent severe ocular complications are evaluated and discussed. ABSTRACT -
(Receivedfor publication 25 September 1980, accepted 19 June 1981)
Leprosy, an infectious disease caused by Mycobacterium leprae, is characterised by
dermal and peripheral nerve lesions. Although there is little evidence ofleprous involvement of the central nervous system, affection of peripheral nerves, such as the facial and trigeminal nerves, is a frequent complicauon--v. DIWAN7 reported facial paralysis in 3% of leprosy patients and in 24.59% of those with the tuberculoid form, In a study of 70 cases of different types of leprosy", 19.8% were found to have facial paralysis, prevalence being amongst the borderline patients. Although affection of the facial nerve was also seen in. cases of lepromatous leprosy, this only occurred during the later stages of the disease, Whenever the nerve has been involved once, especially in the tuberculoid form", paralysis may result within 3 to 4 weeks", 0300-9785/82/010014-07$02.50/0
While all branches of the facial nerve may be affected during the course ofleprosy, especially during erythema nodosum leprosum reactions, the zygomatic branch has been seen to be the most often involved'. Since areas oflow surface temperature appear more prone to leprous affectiorr'>', late involvement or sparing of the deeply placed buccinator muscle is regarded as an example of the possible role of temperature in the affection of nerves". According to a WHO reporr", involvement of of the lower branches of the facial nerve appears to be more common in the yellow and white races. If the branches supplying the orbicularis oculi, frontalis and procerus muscles are affected, lagophthalmos and ectropion of the lower eyelid occur',S,I3,15,2'. The loss of corneal protection leads to exposure keratitis, corneal ulceration and perforation, hypopyon, en-
© 1982 Munksgaard, Copenhagen
15
NERVE LESIONS IN LEPROSY
dophthalmitis, secondary glaucoma and blindness. While lagophthalmos occurred in 14.2% of 70 Thai patients'S, this was seen in only 5.88% of 85 Indian patients', Since lagophthalmos and ectropion are generally considered to be particularly dangerous in leprosy, early surgical intervention is mandatory. Lateral or medial blepharoplasty does not lead to functional closure of the lids and these methods are thus considered only as a preparatory measure in advanced cases of ectropion':". The temporalis transfer operation which permits functional movement of the eyelids was first described by GILLIES9 , while modifications of the temporalismusculo-fascial-sling have been suggested by other authors 2 , 12, 13. Paralysis of the orbicularis oris muscle, although not as common as mid-facial paralysis, also results in serious functional disturbances'", Besides facial disfiguration, difficulties in phonation and, in advanced cases, drooling may be seen. Hypaesthesia or anaesthesia resulting from involvement of the trigeminal nerve have been observed in tuberculoid leprous macules, and infiltrations occur earlier than in borderline and lepromatous forms of leprosy', This team recorded the extent of facial sensory loss in II patients, and found that the maxillary region was always involved. Among 70 Thai patients,
hypaesthesia or anaesthesia was seen in only 5 cases'S. Affection of the sensory supply for the cornea, which comes from the ophthalmic division of the trigeminal nerve via the ciliary ganglion and the short ciliary nerve, can lead to impairment of corneal sensitivity. Anaesthesia of the cornea combined with facial paralysis of the zygomatic branches may give rise to severe ophthalmic complications'V",
Material and methods The study was undertaken at the McKean Rehabilitation Institute, Chiang Mai, Thailand, to determine the occurrence, prevalence and nature of facial and trigeminal nerve lesions in relation to type and duration ofleprosy and its treatment. Of750 inpatients, 43 with facial nerve lesions were examined. These cases were grouped according to the classification suggested by RIDLEY & JOPLlNG'o, using clinical examinations and skin smears (bacillary index; BI). Each patient was examined for changes as follows. I. Facial nerve. Its function was clinically tested by asking the patient to activate the muscles of facial expression. The functions of the frontal and zygomatic facial muscles, and of the muscles of the upper and lower lip were examined separately on the right and left side of the face. Facial paralysis was classified as unilateral, bilateral, complete or incomplete. The average duration of facial palsy, presence of lagophthalmos and blindness was registered. All functional impairments of the facial nerves were photographed.
Table 1. Clinical data of 43 leprosy patients Race Thai: 38 Chinese: 3 Hill tribes: 2
Sex male: 31 female: 12
Age max: 89 years min: 28 years Mean: 51.2 years
Type of leprosy
Duration of leprosy max; 50 years min: 2 years mean: 29.7 years
Duration of treatment max: 30 years min: 3 months mean: 12.1 years
ENL (last 12 months) 7 cases
B.I. (positive) 6 cases (between 0.2 and 3.7)
IT: 8 BT: 20
BB: I BL: 6 LL: 8 Type of medication D.D.S.: 35 D.D.S./Lamprene®; 5 Lamprene'P: 3
16
REICHART, SRISUWAN AND METAH
2. Trigeminal nerve. Sensory charting wascarried out by using a pin-prick test and a pieceofcotton wool to grossly differentiate between anaesthesia and hypaesthesia. The area of reduced sensibility was marked on the face which was then photographed. The ears were not examined. 3. Periorbital surgical procedures were recorded and divided into blepharoplasty, temporalis-musculofascial-sling operation and a combination of both procedures. The effectiveness of each operation was assessed by asking the patient to closehis eyes. If the eyelids met, the effect of surgery was considered good. If the lids were not more than 2 mm apart, this was acceptable, and ifmore than 2 mm, the result was judged to be unsatisfactory.
Results Race, sex and age, type and duration ofleprosy, duration of treatment, type of medication, history of erythema nodosum leprosum in the 12 months prior to the study, and BI are shown in Table 1. A total of 43 patients was affected by facial paralysis. Unilateral involvement of the frontal right or left branches was seen as only partial paralysis in 5 patients; the others revealed bilateral partial and/or complete paralysis. The zygomatic branches were affected unilaterally in 11 cases (partial, 3; complete, 8). Unilateral paralysis of the upper lip was recorded in 10 patients (partial, 6; complete, 4) and unilateral
paralysis of the lower lip was seen in 6 cases (partial, 4; complete, 2). Only 4 patients showed affection ofjust one of the 8 possible sectors; in these, the zygomatic branch was involved by complete paralysis with Bell's phenomenon. The overall distribution of facial paralysis is given in Table 2. Complete paralysis of all facial nerve branches was seen in only one patient (case 6, 42-years-old, tuberculoid leprosy). This patient showed Bell's phenomenon and severe drooling; trigeminal nerve involvement was corn plete and a loss of taste was reported (Figs. 1 and 2). The average duration of facial palsy was 12.1 years, with a maximum of30years and a minimum of 1 month. Cases with the tuberculoid (TT), borderline-tuberculoid (BT) and borderline (BB) forms ofleprosy had facial
Table 2. Distribution of facial paralysis Right
Left
front
p: 11 c: 17
p: 12 c: 17
periorbit
p:
2
c: 29
p: 2 c: 32
p: 10 c: 12
p: 12 c: 7
p: 10 6
p: 10 c: 6
upper lip lower lip
c:
p: partial paralysis. c: complete paralysis.
Fig. 1. Case 26. All branches of the facial nerve are
affected. Facial anesthesia is complete and there is lossof taste. Inability to close the lips revealsmassive calculus accumulation and malposed teeth.
17
NERVE LESIONS IN LEPROSY
shown in Fig. 3.The frontal section of this nerve was affected in 13, the maxillary section in 21 and the mandibular section in 6 patients (Fig. 4). 3 patients with trigeminal nerve lesions had TT leprosy, 14had BT, 1 had BB, 4 had BL and 7 had the LL form. In contrast to the facial nerve lesions, little or no reliable information could be obtained concerning the onset of hypaesthesia and anaesthesia. A total of 17 patients had undergone periorbital surgery due to lagophthalmos and accompanying complications. In 7 of these, blepharoplasty had preceded the temporalismusculo-fascial-sling operation, while in 2 cases the latter had been performed twice. Two operations had been undertaken on one side in 5 patients. The average time interval between the operation and date of reexamination was 5.3 years for patients who had had one operation, and 9.5 years (I st operation) and 3.6 years (2nd
2. Case 26. Activation of the muscles of facial expression reveals Bell's phenomenon; drooling is observed. The upper left lip shows some residual muscularactivity. Fig.
paralysis with an average duration of I3.2 years, while for those with the borderline lepromatous (BL) and lepromatous (LL) types this was 10.3 years. Lagophthalmos with affection of one eye was seen in 10 cases and bilateral involvement was recorded in 16 patients. Blindness of one eye occurred in 6, blindness of both eyes in 4 cases. The average duration of blindness was 8.1 years. Five of these cases had TT and BT leprosy. One TT case (case 41) went blind without having any facial paralysis. In three cases, enucleation of the eye had been carried out (cases 7, 38, 39). Hypaesthesia and anaesthesia of the trigeminal nerve sectors were seen in 29 patients. The distribution of trigeminal nerve involvement is
39
3. Overall distribution of facial hypesthesia (stripes) and anesthesia (checks). Fig.
18
REICHART, SRISUWAN AND METAH
out of 9 cases showed good or acceptable closure of the eyelids (Fig. 5). The combination of temporalis-musculo-fascial-sling and blepharoplasty also showed poor results in 50% of the cases.
Discussion The racial spectrum of patients in this study is representative for the northern part of Thailand. The majority are Thai (38), while minor ethnic groups are represented by the Chinese (3) and the hill tribes (2) of the
Fig. 4. Case 28, male, aged 57, BT type of leprosy.
Bilateral complete facial paralysis of the frontal and zygomatic branches has resulted in bilateral lagophthalmos and blindness of the left eye. Sagging of facial skin and open mouth posture is noted.
operation) for patients who had undergone two. Results ofsurgical procedures according to type are given in Table 3. Relatively poor results had been obtained for blepharoplasty alone; only 3 Table 3. Results of periorbital surgery
t-m-f-s t-m-f-s + BP BP orbicularis oris
Good
Acceptable
Poor
1 4 1 2
3 2
2 6 6
2
plasty t-m-f-s = temporo-musculo-fascial-sling. BP= blepharoplasty,
Fig. 5. Case 37, female, aged 67, BL type ofleprosy.
The facial skin shows wrinkling; loss of eyebrows and eyelashes is seen. The zygomatic branch is affected on the left side; Bell's phenomenon is present. A lateral blepharoplasty had been performed with acceptable results. There is weakness in the innervation of the right upper lip. The area of anesthesia is drawn on the skin infraorbitally (left).
NERVE LESIONS IN LEPROSY
mountain region of North Thailand. The sex ratio (male :female, 2.58: 1) is comparable to that of an earlier study (1.69:1)18. The more apparent male predominance in the present study may be explained by environmental, biological and genetic factors", The average age (51.2 years) is high compared to that of a previous study (42.3 years)". The spectrum of forms of leprosy includes all types and represents a cross-section of permanent residents and temporarily admitted out-patients at the institution. The 43 patients with facial paralysis were selected from a total of about 750 permanant residents and temporarily admitted outpatients. In this study, 5.7% of the leprosy cases had facial nerve involvement; this figure is high compared to other studies in which only 2.2%17 or 3%7 of patients with leprosy had facial nerve involvement. Compared to the polar types (IT and LL) alone, prevalence is low because a 24.59% incidence of facial nerve affection has been recorded for patients with TT'o, and an incidence of 19.8%for patients who were mostly of the LL type's. However, if the spectrum of types ofleprosy is analysed, it appears that most cases with facial nerve involvement are seen in the TT and BT forms of leprosy (Table 1). The average duration of leprosy was 29.7 years, which correlates with the relatively high average age of51.2 years. The duration of treatment was ]2.1 years and the main type of medication was D.D.S. (diaminodiphenylsulphone). These findings correlate with a former study's. Those cases under treatment with Lamprenef were positive for erythema nodosum leprosum; these were also the active cases (6) with a positive BI for acid-fast bacilli. The average duration of facial nerve lesions was 12.1 years. In the TT and BT forms of leprosy, facial paralysis occurred 2.9 years earlier than in the BL and LL types; that nerves are generally affected later in the LL type ofleprosy is an accepted fact' ,6 • The facial nerve lesions observed in the present study were of a rather symmetrical distribution. Complete paralysis was found more frequently
19
in the frontal and, almost exclusively, in the maxillary and periorbital sections. Compared to the other sections, the maxillary (zygomatic) portion was affected most often, an observation which has also been made by other authors'. In contrast to Bell's palsy which normally shows involvement of the frontal and zygomatic paralysis is more a result of peripheral branch involvement. Also, unlike Bell's palsy, lower facial paralysis is less frequently seen. The involvement of the frontal and zyomatic branches can be explained by the relatively thin layer of soft tissue which overlies the zygomatic bone, and temperature reductions which have been shown to be an important factor in development of nerve lesions':", Recovery of facial nerve paralysis is possible in the early stages but may, however, result in misreinnervation as shown by voluntary cocontractions of physiologically unrelated muscles'<" . Lagophthalmos of one or both eyes was seen in 26 out of 43 patients with facial nerve involvement (60.5%). This figure is high and may be explained by the high average age of the patients selected and the long average duration of both leprosy (29.7 years) and facial nerve affection (12.1 years). Of the 750 patients at the institution, about 3.5% had lagophthalmos; this % is comparable to findings in Indians (5.88%)'. Blindness was only observed in long-standing cases, most of which were of the TT and BT forms of leprosy. The pattern ofhypaesthesia or anaesthesia is comparable to that of facial nerve lesions, since the frontal and maxillary division was also the most often affected. While reduction of sensitivity of the whole face seems uncommon (4 cases), smaller areas of reduction or loss of sensitivity are observed more frequently. As in facial paralysis, most cases ofhypaesthesia and anaesthesia are seen in the BT and LL types of leprosy. Nothing can be said about the prevalence of trigeminal nerve involvement, because control patients without facial paralysis were not examined. Lesions of the facial and
20
REICHART, SRlSUWAN AND METAH
trigeminal nerves do not necessarily occur together. Evaluation of the results of periorbital surgery indicates that blepharoplasty alone does not lead to sufficient protection of the eye (Fig. 4) . Ectropion and lagophthalmos persist, thus leading to the severe ocular complications which are generally feared in leprosy. The insufficiency of this operation in advanced cases of lagophthalmos has been described previously':", However, if in permanent partial paralysis sufficient tone is present in the orbicularis muscle to support the tarsal plate, blepharoplasty may be used as a definite operation" , The relatively poor results obtained by combined temporo-musculo-fascial sling and blepharoplasty must be explained by the in ad eq u a t e cooperation of the patients after surgery. This cooperation is mandatory since only active muscular exercise (temporalis muscle) can lead to effective closure of the eyelids.
References 1. ANISH, S. A.: The relationship between surface temperature and dermal invasion in lepromatous leprosy. Int . J. L epr. 1971: 39: 848-851. 2. ANTIA, N . H.: Reconstruction of the face in leprosy. Ann. Roy . Coil. Surg, Engl . 1963: 32: 7198. 3. ANTIA, N. H., D1VEKAR, S. C. & DASTUR, D. K.: The facial nerve in leprosy. Int. J. Lepr. 1966: 34: 103-177. 4. BRAND, P. W.: Temperature variation and leprosy deformity. Int. J. Lepr. 1959: 27: 1-6. 5. BROWNE, S. G .: Leprosy. Acta Clinica. J. R. Geigy S. A., Basle 1970. 6. BROWNE, S. G. : Leprosy - clinical aspects of nerve involvemen t. Contemp . Neural. Ser. 1975 : 12: 1-16. 7. DIWAN, V. S.: A survey of deformities in leprosy (with special reference to face). L epr. Re v. 1962 : 33: 255-262. 8. FRITSCHI, E. P.: Reconstructive surgery in leprosy. John Wright & Sons Ltd ., Bristol 1971. 9. GILLIES, H. D.: Plastic surgery of the eyelids and conjunctival sac. Trans . Ophthal. Soc. (UK) 1935 : 55 : 357-373.
10. HIDAKA, T .: Oral examination in leprosy patients. II. Leprous changes in the tissues of the oral cavity . Nagashirna Arch. Lepr. 1958: 4: 2842. 11. JOB, C. K. : Mechanism of nerve destruction in tuberculoid borderline Icprosy. An electron microscopic study. J . N eural. Sci. 1973: 20: 25-38. 12. JOHNSON, H. A. : A modification of the Gill ies' temporalis tr ansfer for the surgical treatment of the lagophthalmos of leprosy. Plast , Reconst. Sur g . 1962: 30: 378-382. 13. JOHNSON, H . A.: An operation to restore eyelid function lost in leprosy. Int. J. Lepr, 1965: 33: 8994. 14. LIGHTERMAN, J., WATANABE, Y. & HIDAKA, T.: Leprosy of the oral cavity and adnexa. Oral Surg, 1962 : IS: 1178-1194. 15. MANSON-BARR, P. : Manson's tropical diseases, 16th ed, Balliere, Tindall and Cassell, London 1966. 16. RANNEY, D. A., FURNESS, M . A. & SANTHANAKRISHNAN, C. K.: Misreinnervation in leprous neuritis affe cting the facial nerve. L ep R ev. 1972: 43: 151-158. 17. RANNEY, D . A . : The prevalence and consequences of mis-reinnervation of facial neuritis. Int. J. Lepr . 1974: 42: 316-322. 18. REICHART, P . : Facial and oral manifestations in leprosy. Oral Surg, 1976: 41: 385-399 . 19. REICHART, P.: Die chirurgische Korrektur des Lagophthalmus bei Leprapatienten. Fortsch r. Kiefer- Gesichtsch irurgie 1976 : 21: 27-29. 20. RIDLEY, D. S. & JOPLING, W. H. : Classification of lep rosy accord ing to immunity : a five-group system. Int . J. Lep r, 1966: 34: 255-273. 21. SABIN, T . D . & EBNER, J. D . : Patterns of sensory loss in lepromatous leprosy. Int. J. Lepr . 1969: 37: 239-248. 22. SEHGAL, V. N.: Occular changes in tuberculoid leprosy. Ind ian Dermatol. 1972: 17: 74-76. 23. WERNER, R. : Lepra im Mund-, Kiefer- und Gesichtsberelch. Disch. Zahnaeztl. Z. 1973: 28: 64-73. 24. WORLD HEALTH ORGANISATION, Scientific meeting on rehabilitation in leprosy. Veilore, India 1960. WHO Technical Report Series Nos. 221 , 1961. Address : Peter A. R eichart Klinik fiir Mund- , Kief er- und Gesicht schirurg ie Med iz inisclie Hoch schule Hannover 3000 Hanno ver 61 Karl- Wiechert-Allee 9' FRG