International Orthopaedics (SICOT) (1992) 16: 307- 310

International

Orthopaedics © Springer-Verlag 1992

Orthopaedic complications in sickle cell disease A comparative study from two regions in Saudi Arabia M. Sadat-Alil, S. S. Geeranavar2, and S. As-Suhaimi2 1Departement of Orthopaedic Surgery, College of Medicine and Medical Sciences, King Faisal University,Dammam, Saudi Arabia 2King Fahd Hospital, A1-Baha, Saudi Arabia

Summary. The haematological and orthopaedic complications of patients with sickle cell disease from two different regions of Arabia are presented. Nineteen patients from the South-western region were matched for age and sex with an equal number from the Eastern region. The mean sickle cell haemoglobin was 75.95% in those from the Southwest and 77.5% in those from the East. The haemoglobin concentration was marginally lower in the Eastern region patients at 9.19 g/% compared with 9.51 g/%. Bone and joint infections occurred in 17% of Eastern region patients and in 15% in those from the South-western region. There was no significant difference between the haematological parameters and the orthopaedic complications in the two regions. We conclude that these complications are severe in the Eastern region and the disease is not benign as previously thought. Aggressive treatment of orthopaedic complications is indicated in sickle cell disease in the Eastern region. R~sumC Les auteurs prdsentent les complications hdmatologiques et orthop(diques de la dr@anocytose chez des patients originaires de deux diffdrentes rdgions d'Arabie Saoudite. Ils ont compard 19 malades de la rdgion sud-ouest gt 19 autres malades de la rdgion est, du m~me glge et du mOme sexe. Une analyse minutieuse des donndes a montrd que le taux moyen d'hdmoglobine S dtait de 75.95% chez les patients de la r(gion sud-ouest et de 77.5% chez ceux de la r(gion est. La concentration d'hdmoglobine dtait l(gdrement plus basse chez les patients de la rdgion est, soit 9.19 g% contre

9.51 g%. 17 patients de la rigion est et 15 de la rdgion sud-ouest pr(sentaient une infection osseuse ou articulaire. Ces complications orthop(diques sont graves dans les deux rdgions. II n'y a pas de diffdrences notables ni entre les parambtres h6matologiques ni entre les complications orthop6diques. Nous concluons que ces complications sont aussi graves dans la rdgion est et que la maladie n 'est pas bdnigne lorsqu'elle est installde. Un traitement actif des complications orthop(diques de la dr6panocytose s'impose, quelle que soit la rdgion dont est originaire le malade.

Introduction Sickle cell disease was first described in the Kingdom of Saudi Arabia in 1963 [14]. Since then the understanding of the disease, its pattern and diagnosis has greatly improved [2, 3, 4, 9, 15, 18]. At first it was thought that sickle cell disease was milder in Saudi Arabia than in the rest of the world [13, 17], but this is now questioned [2, 20]. Reports in the past have suggested that sickle cell disease is mild in the Eastern region compared to the other regions [9, 10]. This view is now challenged because of the complications of the disease found in the Eastern region [1, 19]. We have carried out the present study with the object of assessing and comparing the orthopaedic complications in two regions of Saudi Arabia.

Patients and method Reprint requests to: M. Sadat-Ali, PO Box 40071, A1-Khobar 31952, King Fahd University Hospital, Saudi Arabia

Nineteen patients from the South-western region with orthopaedic complications were matched with patients from the Eastern region. In the South-west the King Fahd Hospital at

308

M. Sadat-Ali et al.: Orthopaedic complication in sickle cell disease

Table 1. Laboratory data of patients of both groups Southwestern region patients

Age HbS HbF Hb Cone Retic Count

Eastern region patients

Mean

Minimum

Maximum

_+SD

Mean

Minimum

Maximum

+ SD

11.32 75.95 22.88 9.51 10.5

3 62 4 6.3 1

22 96 36 12.1 24

6.36 9.32 8.9 1.79 8.29

11.95 77.25 21.79 9.19 7.99

3 68 6.4 5.6 2

22 98.1 32 10.9 20

6.59 8.83 8.47 1.16 5.27

HbS - sickle haemoglobin, HbF - foetal haemoglobin, Hb Conc - haemoglobin concentration in %, Retic Count - reticulocyte count

A1-Baha and in the East the King Fahd University Hospital at A1-Khobar were chosen as both these hospitals are referral centres for their respective regions. Laboratory data compared were the level of sickle cell haemoglobin, foetal haemoglobin, haemoglobin concentration and the reticulocyte count. The clinical features taken into consideration were the number of episodes of osteomyelitis and the bones involved, the episodes of septic arthritis and the joints involved, and avascular necrosis and the bones involved. In all statistical analysis, the BMDP statistical software package was used [7].

Table 2. Orthopaedic complications in both groups Event

No. of patients

%

SWR

11

58

Bone and joint involved 13

ER SWR ER AVN SWR ER

13 4 4 7 7

68 21 21 36 36

17 5 4 9 15

OM SA

P value

NS NS NS NS

Southwestern Region, ER - Eastern Region, OM SWR Osteomyelitis, SA - Septic arthritis, AVN - Avascular necrosis -

Resul~ Table 1 gives the analysis o f laboratory data for both groups. A m o n g the S o u t h - w e s t e r n r e g i o n patients the m e a n sickle cell h a e m o g l o b i n and foetal h a e m o g l o b i n were 7 5 . 9 5 % and 2 2 . 8 8 % respectively w h i c h was close to the values for the Eastern r e g i o n patients. T h e significant difference was that the level o f h a e m o g l o b i n c o n c e n t r a t i o n and reticulocyte c o u n t w e r e l o w e r in patients f r o m the Eastern region. Osteomyelitis o c c u r r e d in 11 patients (58%) f r o m the S o u t h - w e s t c o m p a r e d to 13 (68%) f r o m the East

(Table 2). This m a y not be significant, but the n u m ber o f b o n e s i n v o l v e d in the Eastern g r o u p was 17 c o m p a r e d with 13 in the S o u t h - w e s t e r n group. Septic arthritis o c c u r r e d in the s a m e n u m b e r o f patients in each group. A v a s c u l a r necrosis was present in 7 patients in each group. T h e n u m b e r o f bones inv o l v e d was different, 15 in the Eastern and 9 in the S o u t h - w e s t e r n group.

Fig. 1. (a) Radiograph showing septic arthritis of both hips with dislocation of the femoral capital epiphysis in a patient from the Eastern region. (b) A similar condition in a patient from the South-western region

M. Sadat-Ali et al.: Orthopaedic complication in sickle cell disease

309 Eastern region patients similar to Asians. The production of foetal haemoglobin is clearly under genetic control [22] and if its level does not affect the severity of the disease there could be other factors which dictate the mildness of the disease, as suggested by other authors [8, 9]. Patients from the two regions may be genetically, socially and economically different, but the clinical presentation and the outcome appear to be similar (Figs. 1 and 2). W e conclude that orthopaedic complications are equally severe in patients from the South-western and Eastern regions. The old misconception of the mild nature of sickle cell disease in the East should be discarded. The patients in the Eastern region should be treated aggressively and appropriately if long term crippling disabilities are to be avoided.

References Fig. 2. (a) Radiograph of the radius and ulna showing sequestration of the shaft of the radius in a patient from the Eastern region. (b) Radiograph of the humerus in the same patient showing periosteal reaction affecting the whole shaft

Discussion Sickle cell disease is primarily a haematological disorder, but the skeleton bears the brunt of the complications due to marrow hyperplasia initially, and later due to vascular occlusion in up to 50% of the patients [5, 6, 11, 12, 16]. In bone and joint infection the diagnostic problem is to differentiate infection from infarction. M a n y patients were crippled due to delay in diagnosis and failure to institute prompt treatment because the physician had underestimated the severity of the disease. The comparative data in this study clearly indicate that the severity of orthopaedic complications from the two regions is not dissimilar, even though patients from the South-west were thought to suffer more severely than those from the East. Previously it had been believed that the high level of foetal haemoglobin in patients from the Eastern region was responsible for the mild clinical symptoms [13, 17, 21]. Roberts et al. reported that the high level o f foetal haemoglobin is not only confined to patients from the Eastern region [21]. E1-Hazmi and Warsi suggested that the level of foetal haemoglobin does not play a role in the severity of the disease [8], and this was later confirmed by E1-Mouzan et al. [10]. T h e y believed that genetic differences in the two regions played a role in the expression of the disease, one similar to African counterparts and the

1. Abu-Srair HA, E1-Bashier AM, A1-Dabous, A1-Khater M (1991) Incidence of major infection in sickle cell pediatric patients at Qatif central hospital. Ann Saudi Med 12: 267 - 270 2. Acquae JK, Omer A, Ganeshaguru K, Sejeny SA, Hoffbrand V (1985) Non-benign sickle cell anemia in Western Saudi Arabia. Br J Haemato160: 99-108 3. A1-Awamy BH, E1-Mouzan M, Altorkl MT, Serjean GR (1984) Neonatal screening for sickle cell disease in Eastern Provitace of Saudi Arabia. Trans R Soc Trop Med Hyg 78: 792-794 4. A1-Awamy BH (1987) Sickle cell anemia: its clinical manifestations and their management. Saudi Med J 8(6): 553-562 5. Bohrer SP (1981) Bone ischemia and infarction in sickle cell disease. Warren H Green, St Louis 6. Diggs LW (1967) Bone and joint changes in sickle cell disease. Clin Orthop 52:119-143 7. Dixon WJ, Brown MB, Engelman L, Jennrich RI (1990) BMDP statistical software manual. University of California Press, Berkeley 8. E1-Hazmi MAF, Warsy AS (1986) On the nature of sickle cell disease in the South-western Province of Saudi Arabia Acta Haemat 212-216 9. E1-MouzanMI, A1-AwamyBH, Altorki MT (1990) Clinical features of sickle cell disease in Eastern Saudi Arab Children. Am J Pediatr Hematol Oncol 12(1): 51-55 10. E1-MouzanMI, A1-AwamyBH, Absood G (1989) Infections and sickle cell disease in Eastern Saudi Arab children. AJDC 143:205-207 11. Gaston MJ (1987) Sickle cell disease. An overview. Semin Roentgenol 22: 150-159 12. Golding JSR, MacIver JE, Went LN (1959) The bone changes in sickle cell anemia and its genetic variants. J Bone and Joint Surg [Br] 41:711-718 13. Gelpi AP (1970) Sickle cell disease in Saudi Arabs. Acta Hemato143:89-99 14. Lehmann H, Maranjian G, Mourant AE (1963) Distribution of sickle cell hemoglobin in Saudi Arabia. Nature 198: 492-493 15. Mallouh AA, Salamah MM (1985) Pattern of bacterial infections in homozygous sickle cell disease. AJDC 139: 820-822

310 16. Middlemiss JH, Raper AB (1966) Skeletal changes in the haemoglobinopathies. J Bone and Joint Surg [Br] 48: 693 -702 17. Perrine RP, Brown MJ Clegg GB et al (1972) Benign sickle cell anemia. Lancet 2:1163-1167 18. Sadat-Ali M, A1-Salem AH (1991) Orthopaedic complications in sickle cell disease in eastern Saudi Arabia. Indian Prac 54: 215-220. 19. Sadat-Ali M (1990) Severe orthopaedic complications of the benign sickle cell disease. First scientific symposium of sickle cell anemia, Dammam, Saudi Arabia

M. S adat-Ali et al.: Orthopaedic complication in sickle cell disease 20. Sadat-Ali M, Sanakaran-Kutty M, Kutty MK (1985) Recent observations on osteomyelitis in sickle cell disease. Internat Orthop 9(2): 97-99 21. Roberts GT, E1-Badawi SB, Padmos MA, Sackey K (1988) Regional variations in sickle cell anemia in Saudi Arabia. Ann Saudi Med 8(5): 320-328 22. Weatherall D J, Wainscoat JS, Thein SL, Old JM, Wood WG, Higgs DR, Clegg JB (1985) Genetic and molecular analysis of mild forms of homozygous beta-thalassemia. Ann NY Acad Sci 445:68-80