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PubMed2002

Natural History of Untreated Scoliosis in beta-Thalassemia.

STUDY DESIGN: A prospective study was performed. OBJECTIVES: To investigate the natural history of untreated scoliosis in beta-thalassemia patients in a 10-year period. SUMMARY OF BACKGROUND DATA: Several previous studies have demonstrated the bone deformities, particularly the high incidence of scoliosis, that is associated with beta-thalassemia. However, little is known about the evolution of scoliosis curvatures, and almost nothing is known about the natural history of this type of scoliosis and its associated lateral spinal curvatures. METHODS: From a group of 115 patients with beta-thalassemia who were evaluated for scoliosis 10 years ago, 43 patients (37%) were re-evaluated 10 years later to study the evolution of the untreated scoliosis and lateral spinal curvatures. Scoliosis, thoracic kyphosis, thoracolumbar kyphosis, and lumbar lordosis were measured both in the initial and last observation and were compared with each other. All changes in scoliosis curve location, direction, and rate of resolving, as well as the changes in the magnitude of the lateral spine curves, were recorded. RESULTS: Scoliosis curves of more than 5 degrees were present in 34 (79%) of the 43 patients who were followed. Scoliosis progressed but was not statistically significant in 12% of the patients with thalassemia in a 10-year span. Ten years ago, 12 patients (28%) showed scoliosis of 10 degrees to 14 degrees, whereas in the last evaluation, 15 patients (35%) had scoliosis of 10 degrees to 19 degrees. The S-shaped scoliosis curve pattern was the most common (29%). In 10 years, the scoliosis curve pattern remained unchanged in 38% of the patients, and it changed in 38%, whereas 24% of the minor curves (5-9 degrees ) showed spontaneous "self-resolving" character. In the last evaluation, there were seven patients (16%) with new cases of scoliosis de novo. There was no sex-related predominance in prevalence of scoliosis in this series. There was a significant skeletal mature retardation in the patients with beta-thalassemia. There was a significant increase of thoracic and thoracolumbar kyphosis and a decrease of lumbar lordosis. The changes in the sagittal profile were not correlated to scoliosis in this group of patients. CONCLUSIONS: Scoliosis in beta-thalassemia appeared with increased prevalence but with small curves of 5 degrees to 19 degrees that did not need active orthopedic treatment. However, a few cases progressed to curves less than 20 degrees, and only one patient (2.9%) showed a severe curve that showed much progression. The behavior of scoliosis in beta-thalassemia differs significantly from that in idiopathic scoliosis. The skeletal disorders that are caused by this hematologic dysfunction seem to be responsible for the spinal deformities and their evolution.

Adolescent↗
PubMed1996

Prevalence of scoliosis in beta-thalassemia.

The objective of this study was to determine the prevalence and possible pathogenesis of scoliosis in beta-thalassemia in our country, and to compare its characteristics to those of patients with idiopathic scoliosis from the same geographic area. Twenty-four [13 male and 11 female thalassemic patients aged 16 +/- 7 years (range 7-32 years)] of 115 examined patients with beta-thalassemia showed scoliosis of 14 degrees +/- 11 (range 10-65 degrees) radiologically. The prevalence of scoliosis in the thalassemic population was 21% in this series, whereas the overall prevalence of scoliosis in the general Greek population was 6% (Smyrnis PN, Valavanis J, Alexopoulos A, Siderakis G, Giannestras NJ: School screening for scoliosis in Athens, J Bone Joint Surg 61B:215-217, 1979). The scoliosis prevalence in the general population was significantly higher in the females (5%) than in the males (1%), whereas no difference in prevalence was found between the two sexes in the thalassemic population. The most common curve pattern in thalassemia was the left lumbar (38%) followed by the right lumbar (21%), whereas in patients with idiopathic scoliosis the left thoracolumbar most commonly appeared (25%) followed by the left lumbar (14%). No patient with thalassemia showed radiographic signs of congenital spinal deformities and spinal fractures, whereas all patients showed a significant retardation of their skeletal maturation. The age of the thalassemic patients with scoliosis was significantly (p = 0.0003) higher than in patients without scoliosis. The hematocrit of the thalassemic patients with scoliosis was significantly (p = 0.0012) lower than in those without scoliosis, whereas the rate of transfusions was not correlated with the magnitude of the scoliosis. The level of ferritin was significantly (p = 0.025) higher in the thalassemic patients with scoliosis than in those without scoliosis. The duration of Desferal treatment was significantly (p = 0.0357) longer in thalassemic patients with scoliosis when compared with those without scoliosis. Thus, the prevalence, curve pattern, and etiology of scoliosis in beta-thalassemia differ from those of idiopathic scoliosis, indicating that the spinal deformities in thalassemia represent a distinct type of scoliosis.

Adolescent↗
PubMed2005

Scoliosis in CHARGE: a prospective survey and two case reports.

CHARGE syndrome was first identified as a cluster of congenital anomalies in 1979 and has since undergone diagnostic criteria modifications to include the major and minor characteristics that occur during infancy and childhood. As the individuals with CHARGE syndrome have aged into their adolescents and adulthood, it has become increasingly common for them to develop scoliosis. This article presents an older population of individuals with CHARGE syndrome and describes the prevalence of scoliosis, and identifiable risk factors for scoliosis. Two case reports demonstrate the variability of scoliosis in CHARGE syndrome. A survey of adults and adolescents with CHARGE syndrome was completed to collect information about late onset medical issues, and those identifying scoliosis as an issue, were further followed for more information. The total population (n=31) and then the subgroup of individuals with scoliosis (n=19) were analyzed. Sixty one percent (19 of 31) of this population was diagnosed with scoliosis. The age of CHARGE syndrome diagnosis was later in the scoliosis population (6.3 years compared to 3.7 years in the no scoliosis population). Growth hormone use was reported in 7 of 31 of the individuals; 6 of these subsequently were diagnosed with scoliosis (32% of the scoliosis group). Of the scoliosis subgroup, most were mild scoliosis but eight were diagnosed with moderate to severe scoliosis, and all of these were treated with either a brace (n=5) or with surgical fusion (n=2) and one individual had both. Scoliosis in CHARGE syndrome individuals is more common than previously reported, and the age of onset is earlier than when routine monitoring for scoliosis is recommended. The prevalence of scoliosis in the CHARGE syndrome population is higher than in the general population therefore, it is very important for physicians to carefully monitor the spine for the development of scoliosis in children with CHARGE syndrome, especially if they are being treated with growth hormone.

Abnormalities, Multiple↗
PubMed1997

Does scoliosis have a psychological impact and does gender make a difference?

STUDY DESIGN: A population-based case-control study, we identified adolescents with and without scoliosis in Minnesota who were 12 through 18 years of age. Matched control subjects were randomly selected from school children who did not have scoliosis or any other condition. Information on scoliosis was obtained by a self-administered questionnaire, the Adolescent Health Survey. Collected on more than 75,000 school age adolescents, with established validity and reliability, a secondary analysis of adolescents with scoliosis was performed as compared with a normative peer group. OBJECTIVE: To describe and characterize the psychosocial impact of scoliosis on the areas of peer relations, body image, and health-compromising behavior, such as suicidal thought and alcohol consumption. SUMMARY OF BACKGROUND DATA: The impact of adolescent idiopathic scoliosis has not been assessed using generic health status measures appropriate for adolescents. Previous studies have concentrated on the health status of adults by measuring work status, marriage status, and other adult measures. The purpose of this study was to study the health status of patients with adolescent idiopathic scoliosis, using the Adolescent Health Survey, a generic health status measure with established validity and reliability. METHODS: Body image, peer relations, social and high-risk behavior, and comparative health were assessed to determine if scoliosis was an independent risk factor and to determine if scoliosis was associated with these psychosocial issues. RESULTS: Six hundred eighty-five cases of scoliosis were identified from the 34,706 adolescents. The prevalence was 1.97%. Of the 685 adolescents with scoliosis and their control subjects, the adjusted odds ratio for having suicidal thought among adolescent with scoliosis, compared to adolescents without scoliosis, was 1.40 (P value of 0.04) after adjustment for race, gender, socioeconomic status, and age. The adjusted odds ratio for having feelings about poor body development among adolescents with scoliosis was 1.82 (P value 0.001) compared with adolescents without scoliosis after adjustment for race, gender, socioeconomic status, and age. Scoliosis was an independent risk factor for suicidal thought, worry and concern over body development, and peer interactions after adjustment. CONCLUSION: Scoliosis is a significant risk factor for psychosocial issues and health-compromising behavior. Gender differences exist in male and female adolescents with scoliosis.

Adolescent↗
PubMed2005

A histomorphologic study of scoliosis in pinealectomized chickens.

STUDY DESIGN: Pinealectomy was used to induce scoliosis in Broiler chickens, and a histologic investigation of the pinealectomized chickens was performed. OBJECTIVES: To investigate the incidence of scoliosis in pinealectomized Broiler chickens and to determine histologic changes in vertebrae in the pinealectomized chickens before the occurrence of a secondary vertebral wedging deformity due to scoliosis. SUMMARY OF BACKGROUND DATA: White Leghorn chickens have previously been used as an experimental model of scoliosis, but the occurrence of scoliosis in these chickens following pinealectomy has varied in different reports and is not always high. It is accepted that the development of scoliosis has a close association with growth, but the etiology and mechanism of the disease remain unknown. Histologic findings in vertebrae after the occurrence of scoliosis include those caused by secondary changes due to mechanical compression, and no histologic data for the period before the occurrence of scoliosis are available. METHODS: Study 1. A total of 100 female "Broiler" chickens were pinealectomized, and the frequency with which scoliosis occurred was investigated. Study 2. Sixty female Broiler chickens were divided into three groups: the control group (Group C, n = 20), the sham operation group (Group S, n = 20), and the pinealectomy group (Group P, n = 20). Each group was then subdivided into two groups according to the time of death: 3 days after the operation (Group 3-C, 3-S, 3-P, n = 10), and 6 days after the operation (Group 6-C, 6-S, 6-P, n = 10). Decalcified thin sagittal sections were made using a tartrate-resistant acid phosphatase (TRAP) stain. Histologic examinations of the growth plate, trabecular structure, and osteoclast number were performed. RESULTS: The incidence of scoliosis in the pinealectomized Broiler chickens was 93.6%. Nine of the 10 chickens in Group 6-P showed scoliosis deformity, while the presence of scoliosis was unclear in chickens in Group 3-P. The osteoclast number increased significantly in Group 3-P, compared with Groups 3-C and 3-S, and the trabecular thickness was greater in Group 3-P than in Groups 3-C and 3-S. There was no significant change in the growth plate or in other aspects of the trabecular structure, except for trabecular thickness, in any of the groups. CONCLUSIONS: The results show that the incidence of scoliosis using pinealectomized Broiler chickens is sufficient to study histologic changes of the vertebral body before onset of scoliosis. We found that the osteoclast number and trabecular thickness increased in pinealectomized chickens after 3 days after surgery, just before scoliosis began to develop, and that no change in the growth plate occurred. This suggests that there is no association between changes in the growth plate and the development of scoliosis. However, the change in osteoclast number may have an association with the development of scoliosis, through changes in bone modeling.

Animals↗
PubMed2001

Scoliosis in patients with diastrophic dysplasia: a new classification.

STUDY DESIGN: Scoliosis in patients with diastrophic dysplasia was analyzed. OBJECTIVES: To study the natural history of scoliosis and to classify the patients with different types of scoliosis. SUMMARY OF BACKGROUND DATA: Typical findings in diastrophic dysplasia are short-limbed short stature, multiple joint contractures, early degeneration of joints, and spinal deformities. The largest studies have reported scoliosis in 37% to 88% of the patients with this rare skeletal dysplasia. The natural history of the deformity is unknown. METHODS: Of the 130 unselected patients, 98 (75%) who were older than 16 years and/or had undergone surgery at the time of the last radiograph were included in the final analysis. These 98 patients included 37 males and 61 females. Their ages at the first radiograph ranged from newborn to 78 years (average, 21 years). The mean follow-up period was 20 years (range, 2-41 years) for 80 patients. Standard standing anteroposterior and lateral radiographs were taken. The degrees of scoliosis, kyphosis, and lordosis were measured according to the Cobb method. Classification of the scoliosis was based on the patient's age at onset of scoliosis, the rate of progression, the magnitude of the scoliosis at the end of growth, and the curve pattern. RESULTS: Of the 98 patients in this study, 86 (88%) had scoliosis. This difference was highly significant statistically (P < 0.001), as compared with the normal population. The frequency of scoliosis was 90% among females and 84% among males. Scoliosis can be divided further into three subtypes: early progressive (11 patients), idiopathic-like (41 patients), and mild nonprogressive (33 patients). One patient had a congenital scoliosis. CONCLUSIONS: Scoliosis is very common in patients with diastrophic dysplasia. The natural history of scoliosis varies from severe deformity with rapid progression to mild deformity without any progression. The authors suggest that the classification described in this report offers a tool for the predicting natural history of scoliosis in diastrophic dysplasia, and for adjusting the timing of surgery in individual patients.

Adolescent↗
PubMed1996

Incidence of scoliosis in beta-thalassemia and follow-up evaluation.

STUDY DESIGN: One hundred fifteen of 120 patients with beta-thalassemia followed in the thalassemia unit were studied for the presence of scoliosis. Forty-nine of these patients were reevaluated 1 year later. OBJECTIVES: To determine the frequency and the course of scoliosis in beta-thalassemia and to compare the findings with those of patients with idiopathic scoliosis. SUMMARY AND BACKGROUND DATA: There is only one report indicating increased frequency of scoliosis in a limited number of patients with thalassemia. In this study, the authors assessed the frequency of scoliosis in a large sample of patients and followed the evolution of this spinal deformity. METHODS: Patients with beta-thalassemia aged 3-35 years were examined clinically and radiologically for scoliosis. Forty-nine of them were reexamined 1 year later for determination of the evolution of scoliosis. RESULTS: Lateral curves of at least 5 degrees Cobb were found in 77 patients (67%), with a male-to-female ratio of 0.9. Scoliosis of at least 10 degrees was found in 21.7% of the male and 20% of the female patients with thalassemia. The ratio was 1.18 for curves of at least 10 degrees and 0.77 for curves of a smaller magnitude. The most common curve pattern was the left lumbar (35.1%), followed by the double-curve pattern (16.9%). Forty-nine randomly selected patients (42.6%) of the 115 included in the study were reexamined 1 year later. Seven male and 7 female patients (total, 28.6%) showed a progression of at least 5 degrees. Six patients (12.2%) experienced spontaneous improvement of less than 6 degrees. The pattern and the evolution of scoliosis observed in patients with beta-thalassemia differ from those found in Greek children with idiopathic scoliosis. CONCLUSIONS: The findings of this study show that the incidence, evolution, and etiology of scoliosis in beta-thalassemia differ from those of idiopathic scoliosis, indicating that the spinal deformities in patients with beta-thalassemia represents a distinct type of scoliosis. Longer follow-up is needed to investigate the natural history of this type of scoliosis.

Adolescent↗
PubMed2000

The estimated cost of school scoliosis screening.

STUDY DESIGN: This is a population-based, longitudinal retrospective study of one community's school-based scoliosis screening program. OBJECTIVE: To report the estimated costs of mass school-based screening for scoliosis. SUMMARY OF BACKGROUND DATA: School scoliosis screening has been reported to cost from as little as $0. 06 to as much as $194 per child. The lower estimate considered only the cost to the school, and the higher estimate defined all children with curves of 5 degrees or more as cases. METHODS: School scoliosis screening results were linked with the medical and chiropractic care records of all referred children to identify outcomes and scoliosis-related health service utilization from Grade 5 through graduation or age 19 years. Costs are presented per child screened, per child with a spinal curve of 20 degrees or more, and per child treated for scoliosis and are based on scoliosis-related health care utilization and school costs. RESULTS: Ninety-two (4.1%) of 2197 children screened were referred for further evaluation of possible scoliosis, and 68 (74%) of those had documented evaluation for possible scoliosis. Five of the 92 children referred were treated for scoliosis by age 19. Sixty-six scoliosis-related primary care visits, 79 scoliosis-related orthopedist visits, and 79 full spine radiographs followed referral but preceded treatment. Case-finding costs for screening were $24.66 per child screened (n = 2197), $3, 386.25 per child with a curve of 20 degrees or more (n = 16) and $10, 836.00 per child treated for scoliosis (n = 5). CONCLUSION: School scoliosis screening is significantly more costly than has previously been reported.

Adolescent↗
PubMed2003

Sagittal plane deformity in the thoracic spine: a clue to the presence of syringomyelia as a cause of scoliosis.

STUDY DESIGN: A retrospective review of scoliosis radiographs of 93 patients with either idiopathic scoliosis or syringomyelia-associated scoliosis were assessed, defining their sagittal alignment. OBJECTIVE: To validate an observation regarding the absence of Dickson's sagittal deformity of the thoracic spine in patients with syringomyelia-associated scoliosis. SUMMARY OF BACKGROUND DATA: Patients with adolescent idiopathic scoliosis have a classic sagittal deformity. Dickson described that patients with adolescent idiopathic scoliosis (AIS) have an associated lordotic deformity at the apex of their coronal deformity. MATERIALS AND METHODS: Retrospective reviews of standard scoliosis series radiographs of 93 patients with idiopathic or syringomyelia-associated scoliosis from two institutions were compared. Particular attention was given to the lateral radiograph of the spine assessing presence or absence of Dickson's apical lordosis. Nine patients had to be excluded because of inadequate imaging. The study group consisted of 30 patients with scoliosis from TSRH with documented syringomyelia identified between 1985 and 1997. The demographic and radiographic features of this group were compared with those of a control group consisting of a consecutive series of 54 patients from HSS with adolescent idiopathic and normal MRI. The groups were comparable for age (mean age: control 13 y; syrinx 12 y) and curve pattern but differed in curve magnitude (mean Cobb: control 50 degrees; syrinx 40 degrees ). RESULTS: Apical lordosis was present in 97% of patient with AIS and a normal MRI but was absent in 75% of patients with syringomyelia-associated scoliosis (P < 0.0001). The results also confirmed that male patients with scoliosis and left-side curves have a predisposition to having a syringomyelia (P < 0.0001). CONCLUSIONS: Sagittal plane deformity in scoliosis can be an indicator of the presence of a syringomyelia. Our results reinforce the necessity of assessing sagittal plane deformity when treating scoliosis. If apical lordotic deformity is absent, a diagnosis of idiopathic scoliosis should be made with caution.

Adolescent↗
PubMed2003

Value of treating primary causes of syrinx in scoliosis associated with syringomyelia.

STUDY DESIGN: Retrospective chart and radiologic analysis. OBJECTIVES: To analyze the value and risks of neurosurgical procedures for syringomyelia and of fusion procedures for scoliosis. SUMMARY OF BACKGROUND DATA: There are few reports on the benefit of neurosurgical procedures on scoliosis, as well as the risk of fusion procedures on scoliosis related to syringomyelia. METHODS: The 105 patients who had both scoliosis and syringomyelia were subdivided into three groups: (I) 59 patients without congenital scoliosis or myelomeningocele, (II) 20 patients with congenital scoliosis and syringomyelia, and (III) 26 patients with myelomeningocele and syringomyelia. The study evaluated: (1) The effect of either suboccipital craniectomy or direct shunting on the syrinx, the curvature, and the neurologic status; (2) the neurologic risk of scoliosis fusion surgery; and (3) the curve status after fusion surgery. RESULTS: In Group I (patients without congenital scoliosis or myelomeningocele), suboccipital craniectomy resulted in curve improvement in seven, worsening in three, and no change in two, whereas direct syrinx shunting gave curve improvement in none, curve worsening in six, and no change in two. In Groups II and III, no patient had curve improvement after neurosurgery. Group III had the highest rate of subsequent neurosurgery (50%). The subsequent neurosurgery was nearly always suboccipital craniectomy or detethering of the cord. Three of 38 patients (8%) had neurologic worsening when scoliosis fusion surgery was done without previous syrinx decompression. CONCLUSIONS: In patients without myelomeningocele or congenital scoliosis, but with Arnold-Chiari malformation and syringomyelia, suboccipital craniectomy gave the best chance for syrinx reduction and scoliosis improvement, particularly in children younger than 10 years. Syrinx shunting improved none of the scolioses. For syrinxes in patients with congenital scoliosis or myelomeningocele, neither neurosurgical procedure resulted in curve improvement, as other causes of scoliosis (vertebra anomalies, paralysis) remained untreated. Patients with myelomeningocele require a multipronged surgical approach to address all causes of syrinx, thus minimizing the potential need for repeat neurosurgery. Scoliosis correction without prior syrinx decompression carries a high neurologic risk.

Adolescent↗
PubMed2003

Pediatric scoliosis and the lung.

OBJECTIVE: To determine the pattern of scoliosis in the pediatric age group and to determine the course of the disease and the result of surgical intervention. METHODS: A retrospective review of all charts of patients referred to the Pulmonary Clinic of the King Faisal Specialist Hospital, Riyadh, Kingdom of Saudi Arabia (KSA), for scoliosis and preoperative evaluation from the period November 1992 to October 2001. Demographic and clinical data, preoperative and postoperative pulmonary function test (PFT) and effect of surgery on the lung were presented. RESULTS: A total of 66 patients with scoliosis were followed. The age at diagnosis was 8.5 +/- 4.4 years. Thirty-four (52%) males and 32 (48%) females. Fifty-seven (86%) were alive and 9 (14%) died. A period of follow up was 5.3 +/- 3.7 years. Idiopathic scoliosis was found in 20 patients (30%), congenital anomalies associated with scoliosis constituted 35 (53%) of the total scoliosis referral and secondary type due to trauma or malignancy formed 11 (17%). Mean Cobb s angle of 70 +/- 30 degrees. The most common curvatures were: thoracic 31 (47%), lumbar 12 (18%) and thoracolumbar 30 (45%). Thirteen patients (20%) had 2 or 3 types of curvatures simultaneously. Baseline PFT showed moderate restrictive lung disease in approximately two thirds of the patient population. Scoliosis surgery was carried out in 34 (52%) of the patients. Posterior approach was more commonly used in 30 (88%) of the patients. Pneumonia and atelectasis were the most common complication encountered in 15 (44%) of the patients. Recurrence of scoliosis after operation recurred in 12 (35%) of the population. Comparisons between both groups have shown that patients with congenital anomalies and scoliosis were diagnosed earlier, had significant postoperative complications and higher mortality compared to those with idiopathic scoliosis (p<0.05). Changes in PFT for patients who underwent surgery for scoliosis with Cobb s angle >40 degrees showed less deterioration compared to those with angle <40 degrees (p=0.004), due to progressive deterioration without surgery. CONCLUSION: Scoliosis is a common problem in KSA. Patients need to be referred early for proper intervention before it becomes a severe magnitude and difficult to be fixed and to prevent permanent complications. Patients with congenital anomalies and scoliosis had poor prognosis compared to those with idiopathic scoliosis.

Child↗