Hand deformity and sensory loss due to Hansen's disease in American Samoa We report the prevalence of sensory loss and hand deformity in 63 patients with Hansen's disease in American Samoa. Open ulceration, the most common deformity, was present in 41 % of patients; sensory abnormalities were present in 54% and were bilateral in 65%. The presence of abnormal sensibility correlated with a high percentage of other deformities. Hand abnor- malities were most prevalent in lepromatous patients and were related to a prolonged duration of disease. Nerve thickening did not appear to be a helpful clinical finding to assess the degree of sensory loss. (J HAl'iD SURG 1988;13A:279-83.) Wiley BruneI, MD, William P. Schecter, MD, and Gisela Schecter, MD, San Francisco, Calif. Ha"nsen's disease is a chronic mycobac- terial infection that affects an estimated 10 to I I million persons. I It frequently involves the peripheral nervous system and skirr' and is thought to be the most common cause of peripheral neuropathy worldwide." Epidemi- ologic data on the neuropathy and hand dysfunction in Hansen's disease is not well known, and many persons with Hansen's disease have not been studied. In this study, we report the prevalence of specific hand ab- normalities found in patients with Hansen's disease in American Samoa. American Samoa is a group of volcanic islands lo- cated in the Pacific Ocean, 1600 miles northeast of New Zealand, 2700 miles east of Australia, and 2200 miles southwest of Hawaii. It is an unincorporated ter- ritory of the United States and consists of six islands that occupy a total land area of 76 square miles. The majority of the 33,000 inhabitants are Samoan, al- though small groups of Tongans, Korean fisherman, and mainland Americans also live there. Hansen's disease is endemic throughout the South Pacific and cases in American Samoa have been fol- From the Department of Surgery; University of California, San Fran- cisco at San Francisco General Hospital, and the Tuberculosis Clinic, San Francisco General Hospital, San Francisco, Calif. Received for publication April 20, 1987; accepted in revised form luly 22, 1987. No benefits in any form have been received or will be received from a commercial party related directly or indircctly to the subject of this article. Reprint requests: William P. Schecter, MD, San Francisco General Hospital, 1001 Potrero Ave., San Francisco, CA 94119. Table I. Factors associated with sensory loss Normal Abnormal All sensibiliry sensibility patients (n = 29) (n = 34) (n = 63) Patients (%) 46 54 100 Median age (yr) 30 40 33 Type (%) Borderline 21 15 18 Lepromatous 48 65 57 Tuberculoid 31 20 25 Average duration (yr) 4.5 13.5 9.6 lowed by the Hansen's Disease Control Clinic since 1972. Methods We attempted to interview and examine all patients with Hansen's disease, who were seen or were referred to the American Samoa Hansen's Disease Control Clinic to ascertain the nature and prevalence of hand impairment. An active effort was made to locate all patients who were listed in the Hansen's Disease Reg- istry between 1972 and 1983. A total of 150 patients were identified. Of those, 71 had either died, moved off the island, or could not be located. This resulted in a sample population of 79 patients of which 63 agreed to be interviewed and examined. Data were collected for age, sex, classification of Hansen's disease, duration of disease, and history of previous hand injuries. Occupational information was not obtained. Information regarding previous treatment was unreliable and therefore was not recorded. THE JOURNAL OF HAND SURGERY 279
Transcript
Hand deformity and sensory loss due to Hansen'sdisease in American Samoa
We report the prevalence of sensory loss and hand deformity in 63 patients with Hansen's diseasein American Samoa. Open ulceration, the most common deformity, was present in 41% ofpatients; sensory abnormalities were present in 54% and were bilateral in 65%. The presenceof abnormal sensibility correlated with a high percentage of other deformities. Hand abnormalities were most prevalent in lepromatous patients and were related to a prolonged durationof disease. Nerve thickening did not appear to be a helpful clinical finding to assess the degreeof sensory loss. (J HAl'iD SURG 1988;13A:279-83.)
Wiley BruneI, MD, William P. Schecter, MD, and Gisela Schecter, MD,San Francisco, Calif.
Ha"nsen's disease is a chronic mycobacterial infection that affects an estimated 10 to I I millionpersons. I It frequently involves the peripheral nervoussystem and skirr' and is thought to be the most commoncause of peripheral neuropathy worldwide." Epidemiologic data on the neuropathy and hand dysfunction inHansen's disease is not well known, and many personswith Hansen's disease have not been studied. In thisstudy, we report the prevalence of specific hand abnormalities found in patients with Hansen's disease inAmerican Samoa.
American Samoa is a group of volcanic islands located in the Pacific Ocean, 1600 miles northeast ofNew Zealand, 2700 miles east of Australia, and 2200miles southwest of Hawaii. It is an unincorporated territory of the United States and consists of six islandsthat occupy a total land area of 76 square miles. Themajority of the 33,000 inhabitants are Samoan, although small groups of Tongans, Korean fisherman,and mainland Americans also live there.
Hansen's disease is endemic throughout the SouthPacific and cases in American Samoa have been fol-
From the Department of Surgery; University of California, San Francisco at San Francisco General Hospital, and the TuberculosisClinic, San Francisco General Hospital, San Francisco, Calif.
Received for publication April 20, 1987; accepted in revised formluly 22, 1987.
No benefits in any form have been received or will be received froma commercial party related directly or indircctly to the subject ofthis article.
Reprint requests: William P. Schecter, MD, San Francisco GeneralHospital, 1001 Potrero Ave., San Francisco, CA 94119.
lowed by the Hansen's Disease Control Clinic since1972.
Methods
We attempted to interview and examine all patientswith Hansen's disease, who were seen or were referredto the American Samoa Hansen's Disease ControlClinic to ascertain the nature and prevalence of handimpairment. An active effort was made to locate allpatients who were listed in the Hansen's Disease Registry between 1972 and 1983. A total of 150 patientswere identified. Of those, 71 had either died, movedoff the island, or could not be located. This resulted ina sample population of 79 patients of which 63 agreedto be interviewed and examined.
Data were collected for age, sex, classification ofHansen's disease, duration of disease, and history ofprevious hand injuries. Occupational information wasnot obtained. Information regarding previous treatmentwas unreliable and therefore was not recorded.
Patients were classified as having tuberculoid diseaseif there were three or less skin lesions, and lesions,with skin biopsy showing bacterial density less than Iper 10 high-powered fields. Lepromatous disease wasdefined by the presence of diffuse skin involvement,with skin biopsy showing 10 or more bacteria per highpowered field. Borderline patients fell between thesetwo extremes."
Sensory impairment was evaluated by examinationof digit two-point sensory discrimination. Pin prick andthermal testing were not performed as these tests werebelieved to be less helpful in the evaluation of nervedysfunction. ' Two-point discrimination less than 4 mmwas considered normal and two-point discriminationgreater than 4 mm was considered abnormal. Completeanesthesia was defined as the inability to detect bluntpressure on the fingertips."
All patients were examined to evaluate the degree ofmotor dysfunction. The presence of intrinsic minus fin-
gers, defined as hyperextension of the metacarpophalangeal (MP) joints and flexion of the proximal interphalangeal (PIP) joint and distal interphalangeal (DIP)joints of the ring or small finger, served as evidencefor motor impairment of the ulnar nerve . Thenar paralysis, with loss of opposition of the thumb to the ringfinger, served as evidence for motor impairment of themedian nerve. All patients were exjamined for the presence ofopen ulcers, joint subluxation, ulnar and mediannerve thickening, and finger absorption.
Results
Sixty-three patients with Hansen's disease were evaluated of which 79% were male and 21% were female(Fig. I) . The median age of the patients' was 33 yearsof age, with a range from 6 to 76 years. Borderlinedisease was present in 18% of the population, lepromatous disease in 57%, and tuberculoid disease in 25%.
Age appeared to play an important role in the expres-
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".n lenrs
# Pat ients
oss
Fig. 3. Duration of disease and sensory impairment.
De fo rmi ty
1-1 5 16· 20 21·25 26· 30 31-35Duration (Yrs)
Fig. 4. Duration of disease and deformity.
Hal/sen's disease 281
sion of the disease. Fig. 2 demonstrates that the diagnosis of Hansen's disease is frequently made at a relatively early age. The diagnos is was made in the majority of patients (83%) before the age of 40 years , withthe median age being 23 years of age. Sensory loss anddeformity were not apparent in patients who were lessthan 13 years old.
There appeared to be an association between the duration of the disease and hand dysfunction. The prevalence of both sensory loss and hand deformity increased with prolonged duration of the disease. Allpatients with a disease duration greater than 17 yearshad abnormal sensibility as measured by two-point discrimination (Fig. 3). Hand deformities were present inall patients with duration of disease greater than 21years (Fig. 4).
Table I shows the factors associated with sensory
loss as defined by abnormal two-point discrimination.Sensory abnormalities were present in 54% of patients.These patients' tended to be older, with a median ageof 40 years. They also tended to have the disease forit longer period of time; 13.5 years compared with 4.5years in patients with normal sensibility. From this data,it was difficult to determine whether or not gender correlated with sensory loss because of the maldistributionof males to females in our sample ;
Sensory loss occurred in an unequal nerve distribution (Table II). In the majority of cases (71%) sensoryloss occurred in the ulnar and median distribution. Inaddition, 21% of cases involved the ulnar nerve alone ,6% involved the median alone, and 3% involved theulnar, median, and radial nerves . Sensory loss was bilateral in 65% and unilateral in 35%.
Ulnar and medianUlnarMedianUlnar, median, and radialBilateralUnilateral
Table II. Patterns of sensory loss
__1--
Table III. Deformity and sensory loss
Discussion
The hand is one of the most frequent areas of involvement in Hansen's disease," and sensory perceptionhas been reported to be one of the earliest functionslost." In American Samoa, upper extremity sensory andfunctional abnormalities are frequent findings in patients with Hansen's disease.
Some reports have suggested that deformity ratesincrease with advancing age (15, 18,21), while othersshow no correlation.'? Since hand deformity is rarely
three patients. In these patients, the duration of diseasewas 16, 20, and 26 years. Two of the patients hadlepromatous disease and one had borderline disease.
Hand deformities were present in 56% of the patients(Table III). Open ulceration was the most common deformity. The presence of intrinsic minus fingers wasthe second most common deformity. All observed deformities occurred most frequently in lepromatous patients as compared with the other types of disease.Overall, 46% of patients had a history of hand injury(Table IV).
There was no clear relationship between the presenceof nerve thickening and the extent of sensory impairment. Fifty-six percent of patients overall were foundto have peripheral nerve thickening. There was no difference in the prevalence of nerve thickening betweenpatients with normal and abnormal sensibility.
Finding
Open ulcersIntrinsic minusJoint subluxationThenar paralysisFinger absorption
Normalsensibility(n == 29)
1473oo
Abnormalsensibility(n == 34)
6544413826
Total% (n == 63)
4127242114
seen in young patients." 8, II, 12 it might be thought thatincreasing deformity and neurologic loss reflects prolonged duration of disease in older patients." In thisstudy, older patients did not necessarily have a prolonged duration of disease. We observed that 30% ofpatients more than 50 years of age had the disease lessthan 10years. Forty percent of these patients had intactsensibility and absence of deformity. This suggests thatage may not be as important as duration of disease inthe development of hand dysfunction.
There is no agreement regarding the type of Hansen'sdisease that causes maximal deformity. Lepromatous,borderline, and tuberculoid types have all been associated with maximum deformity rates." 7, 8, 10. 12 InAmerican Samoa, upper extremity deformity and sensory abnormalities are the most prevalent in lepromatous patients. However, lepromatous patients were alsoobserved to have the longest duration of disease. It isnot clear from this study which is more important asboth appear to playa major role in the expression ofthe disease.
Previous reports have suggested that nerve swellingin Hansen's disease occurs in an uneven distribution. 13
It has also been suggested that nerve swelling is closelyassociated with paralysis of limbs,14 and that this findingmay be used diagnostically in early stages of the disease. 13 From our experience we have found that the clinical assessment of nerve thickening is difficult. A nervethat appears to be only "palpable" to one group of investigators may represent a thickened nerve to another.Therefore, interpretation and comparison of our findingsof nerve thickening with other studies should be donewith caution. Our results suggest that nerve dysfunctiondoes not correlate well with nerve thickening.
Conclusion
Hand deformity and sensory loss is frequently observed in patients with Hansen's disease in American
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Samoa. These abnormalities are most prevalent in lepromatous patients' and appear to be related to a prolonged duration of disease. In this patient population,nerve thickening does not appear to be a useful indicatorof nerve dysfunction. In this study we did not addressthe impact of medical intervention on hand dysfunctionobserved in these patients. Further research is neededto determine whether or not treatment affects the se- ·verity of upper extremity sensory loss and deformitycaused by Hansen's disease.
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Compression of the deep branch of the ulnarnerve at the adductor hiatus producing painwithout muscle atrophy
Compression of the deep branch of the ulnar nerve distal to the pisohamate hiatus is rare. Twocases of compression of the deep branch at the adductor hiatus are presented. These cases areunique because of pain in the hand without muscle atrophy. One patient has had surgicaltreatment, and the other patient's symptoms are not severe enough to warrant surgical treatment.(J HA!"D SURG 1988;13A:283·6.)
Michael A. Milck, MD, and J. David Thompson, MD, Nashville, Tenn.
From the Department of Orthopaedics and Rehabilitation. VanderbiltUniversity Medical Center, Nashville . Tenn.
Received for publication June 30. 1987; accepted in revised formJuly 22. 1987.
No benefits in any form have been received or will be received froma commercial party related directly or indirectly to the subject ofthis article.
Reprint requests: Michael A. Milck, MD, Department of Orthopaedics and Rehabilitation, 0-4213 Medical Ctr., N., VanderbiltUniversity Medical ce., Nashville, TN 37232.
Compression of the deep branch of theulnar nerve at the wrist is a well-described entity, having been summarized by Shea and Mcfllain' and otherauthors.' A variety of etiologic factors have been implicated. Ganglion cysts and vascular lesions within thecanal of Guyon and compression at the pisohamatehiatus':" have been the factors most frequently reported.
Compression of the deep motor branch distal to thepisohamate hiatus and within the palm itself is rarely
