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PubMed2005

Scoliosis correction in an adolescent with a rigid spine syndrome: case report.

STUDY DESIGN: Details of presentation and approach to the treatment of scoliosis in a case of a Rigid Spine Syndrome (RSS). OBJECTIVE: To report on the results of conservative and operative treatment of scoliosis associated with RSS, and, based on this, to propose an assessment and treatment protocol for this condition. SUMMARY OF BACKGROUND DATA: Congenital muscular dystrophies (CMD) are a group of disorders marked by hypotonia at birth and a generally nonprogressive course of muscle weakness. Spinal rigidity is present in a number of patients with CMD. RSS is classified as a CMD, and is characterized by early rigidity of the spine, limb contractures, and restrictive respiratory dysfunction. An approach to the treatment of scoliosis in RSS has not been established. METHODS: Details of history, diagnostic tests, and treatment of an adolescent with RSS associated with progressive scoliosis and cervical spine extension contracture is presented. The role of brace treatment, Botox (Allergan, Inc., Irvine, CA), and details of operative correction, including histology of back muscles, is defined. RESULTS: In this case of RSS with rigid scoliosis nonresponsive to brace therapy and Botox injections, an anterior thoracolumbar spine fusion with instrumentation was successfully performed for correction of the scoliosis, and the cervical spine was addressed through a posterior approach and an occiput-cervical fusion. We outline the details of surgical procedure and restoration of spinal balance in both sagittal and coronal planes. CONCLUSIONS: The chromosomal bases of CMD and characteristic features of RSS, including diagnostic tests, have been reviewed. Surgical intervention with spinal deformity fusion, correction, and instrumentation is indicated in RSS with progressive spinal imbalance not responding to conservative therapy is safe and can give promising results.

Adolescent↗
PubMed2005

Surfing for scoliosis: the quality of information available on the Internet.

STUDY DESIGN: A cross section of Web sites accessible to the general public was surveyed. OBJECTIVE: To evaluate the quality and accuracy of information on scoliosis that a patient might access on the Internet. SUMMARY OF BACKGROUND DATA: The Internet is a rapidly expanding communications network with an estimated 765 million users worldwide by the year 2005. Medical information is one of the most common sources of inquires on the Web. More than 100 million Americans accessed the Internet for medical information in the year 2000. Undoubtedly, the use of the Internet for patient information needs will continue to expand as Internet access becomes more readily available. This expansion combined with the Internet's poorly regulated format can lead to problems in the quality of information available. Since the Internet operates on a global scale, implementing and enforcing standards have been difficult. The largely uncontrolled information can potentially negatively influence consumer health outcomes. METHODS: To identify potential sites, five search engines were selected and the word "scoliosis" was entered into each search engine. A total of 50 Web sites were chosen for review. Each Web site was evaluated according to the type of Web site, quality content, and informational accuracy by three board-certified academic orthopedic surgeons, fellowship trained in spinal surgery, who each has been in practice for a minimum of 8 years. Each Web site was categorized as academic, commercial, physician, nonphysician health professional, and unidentified. In addition, each Web site was evaluated according to scoliosis-specific content using a point value system of 32 disease-specific key words pertinent to the care of scoliosis on an ordinal scale. A list of these words is given. Point values were given for the use of key words related to disease summary, classifications, treatment options, and complications. The accuracy of the individual Web site was evaluated by each spine surgeon using a scale of 1 to 4. A score of 1 represents that the examiner agreed with less than 25% of the information while a score of 4 represents greater than 75% agreement. RESULTS: Of the total 50 Web sites evaluated, 44% were academic, 18% were physician based, 16% were commercial, 12% were unidentified, and 10% were nonphysician health professionals. The quality content score (maximum, 32 points) for academic sites was 12.6 +/- 3.8, physician sites 11.3 +/- 4.0, commercial sites 11 +/- 4.2, unidentified 7.6 +/- 3.9, and nonphysician health professional site 7.0 +/- 1.8. The accuracy score (maximum, 12 points) was 6.6 +/- 2.4 for academic sites, 6.3 +/- 3.0 for physician-professional sites, 6.0 +/- 2.7 for unidentified sites, 5.5 +/- 3.8 for nonphysician professional sites, and 5.0 +/- 1.5 for commercial Web sites. The academic Web sites had the highest mean scores in both quality and accuracy content scores. CONCLUSION: The information about scoliosis on the Internet is of limited quality and poor information value. Although the majority of the Web sites were academic, the content quality and accuracy scores were still poor. The lowest scoring Web sites were the nonphysician professionals and the unidentified sites, which were often message boards. Overall, the highest scoring Web site related to both quality and accuracy of information was www.srs.org. This Web site was designed by the Scoliosis Research Society. The public and the medical communities need to be aware of these existing limitations of the Internet. Based on our review, the physician must assume primary responsibility of educating and counseling their patients.

Cross-Sectional Studies↗
PubMed2006

Unilateral enucleation affects the laterality but not the incidence of scoliosis in pinealectomized chicken.

STUDY DESIGN: Randomized prospective study using an experimental scoliosis model in a pinealectomized chicken. OBJECTIVE: To investigate the effect of the side of visual impairment on the incidence and laterality of the curves on a pinealectomized chicken model. SUMMARY OF BACKGROUND DATA: Visual impairment has been shown to increase the incidence of idiopathic-like scoliosis in human subjects when compared to the incidence of the general population. However, an association between the laterality of the visual impairment and incidence or laterality of scoliosis has not been established. METHODS: A total of 60 newly hatched white leghorn chicks was divided equally into 3 study groups of no visual impairment (n = 20) (group 1), left-sided blindness by enucleation (n = 20) (group 2), and right-sided blindness (n = 20) (group 3). Pinealectomies and enucleations were performed on the second day after hatching. Anteroposterior radiographs were obtained at the 5th and 10th weeks, and the incidence, side, and magnitude of the resulting scoliotic curves were recorded. RESULTS: The pinealectomy model yielded a general scoliosis incidence of 60%. The occurrence of scoliosis was not different among the groups (65%, 55%, 60%, respectively, P = 0.812). The incidences at the 5th and 10th weeks were both 40%, as a result of the death of 6 chickens between the 5th and 10th weeks, as well as the appearance or disappearance of curves during this time, again not different among the groups. However, the laterality of the curves was significantly different (P = 0.045). The visually impaired groups tended to have left thoracic curves as frequently as the right thoracic curves (7 right and 4 left in group 2, and 7 right and 6 left in group 3), whereas in group 1, the thoracic curves were predominantly right sided (12 right and 1 left). The average magnitude of the curves was 30.47 degrees +/- 19.32 degrees , not significantly different among the groups (27.6 degrees +/- 16.7 degrees , 23.7 degrees +/- 21.5 degrees , 39.8 degrees +/- 17.7 degrees , respectively, P = 0.109). CONCLUSIONS: Unilateral visual impairment does not have a significant effect on the overall incidence and magnitude of scoliosis in the pinealectomized chicken. It does affect the laterality of the curves though, visually impaired subjects having a significantly higher likelihood of left thoracic curves, regardless of the side of blindness. It may be worthwhile to see if such an association is present in human beings as well.

Animals↗
PubMed2006

Operative treatment of scoliosis in patients with a Fontan circulation.

STUDY DESIGN: A retrospective chart and radiographic review of patients with a Fontan physiology who underwent surgical correction of their scoliosis. OBJECTIVE: To evaluate the operative and perioperative treatment of patients with a Fontan physiology and scoliosis. SUMMARY OF BACKGROUND DATA: The survival rates in patients with complex congenital heart disease are improving, thus creating a subset of patients with complex physiology who will require surgical treatment of their scoliosis. The Fontan physiology is created surgically in patients born with a single functioning ventricle. These patients represent a significant anesthetic risk during spinal surgery, given their physiologic need for a sustained systemic preload. Currently, to our knowledge, there are no published series in the literature regarding the operative treatment of scoliosis in these patients. METHODS: We reviewed our experience with the operative treatment of scoliosis in 7 patients with a Fontan circulation. There were 7 patients identified who were treated with correction and arthrodesis using modern segmental instrumentation. A radiographic and chart review was performed to assess the operative and perioperative treatment. RESULTS: Mean age at surgery was 14 years 9 months, and mean Cobb angle was 75 degrees . There were no intraoperative complications; however, postoperative complications were frequent and significant: 1 delayed paralysis, 2 superior mesenteric artery syndromes, 1 renal tubular necrosis, 2 pulmonary complications, 1 Horner syndrome, and 1 urinary tract infection. CONCLUSIONS: Operative treatment of scoliosis in these patients may be successful; however, complications are frequent and significant.

Adolescent↗
PubMed2006

Geometric and electromyographic assessments in the evaluation of curve progression in idiopathic scoliosis.

STUDY DESIGN: The natural history of patients with idiopathic scoliosis was analyzed radiographically and electromyographically in a prospective longitudinal study. OBJECTIVES: To identify changes in geometric variables and the sequence in which these changes occur during curve progression in the natural history of patients with idiopathic scoliosis. In addition, to study the relationship between several geometric variables and electromyographic (EMG) measurements to determine their predictive value as risk factors to curve progression of the scoliotic deformity. SUMMARY OF BACKGROUND DATA: The main area of concern in treating children with adolescent idiopathic scoliosis is the unpredictability of curve progression during the early development of the deformity. METHODS: The changes in radiographic geometric and EMG variables between the first presentation and consecutive 4-6-month follow-up periods were analyzed in 105 patients with idiopathic scoliosis. Statistical analyses were performed to elucidate in more detail how spinal geometry evolves during curve progression. RESULTS: Curve severity was associated with remaining growth potential expressed as an increasing spinal growth velocity (SGV). With increasing SGV, an enhanced EMG activity at the lower part on the convex side of the curve expressed as EMG ratio was found. High EMG ratio was associated with increased axial rotation and diminished kyphosis before the rapid increase in Cobb angle. Lateral deviation, wedge angle, and axial rotation all increased during periods of progression. Changes in tilt angle and lordosis were not associated with curve progression. CONCLUSIONS: In the natural history of idiopathic scoliosis, SGV and EMG ratio at the lower end vertebra are prominent risk factors of curve progression. The asymmetric muscle activity is associated with increased axial rotation, which in its turn is associated with increasing Cobb angle and diminishing kyphosis. The combination of these variables provides insight in the physiologic and 3-dimensional biomechanical evolution of the natural history of curve progression in idiopathic scoliosis.

Adolescent↗
PubMed2006

The search for idiopathic scoliosis genes.

STUDY DESIGN: A cohort of 145 patients with adolescent idiopathic scoliosis (AIS) were identified and contacted to determine whether they had a family history of scoliosis. These results were submitted to an internal genealogical database to screen for potential connections to other AIS families. The severity and incidence of AIS in extended family groups were also analyzed. OBJECTIVES: Our objectives were to quantify the genetic effect in AIS, determine the expressivity and penetrance of AIS in large family groupings, and examine larger scoliosis pedigrees for evidence of multiple genes. SUMMARY OF BACKGROUND DATA: Previous reports have suggested an 80% connectedness among scoliosis families, but no clear evidence of multiple genes. It is not known if there are major gene(s). METHODS: A cohort of 145 AIS probands were identified and contacted to ascertain whether they had a family history of AIS. Their medical records and spine radiographs were reviewed to confirm the diagnosis and determine the disease severity. Using an internal genealogical database, the cases were screened for potential connections that would produce larger extended pedigrees. RESULTS: Overall, 131 of the probands were in the database and 127 showed connections to other scoliosis families, a 97% connectedness. These results suggest a major scoliosis gene, as more than 50% of the probands were connected by founders that all resided in England in the mid 1500s. The differences in penetrance (41% vs. 34%) and expressivity (38% vs. 61%) between seemingly unrelated large family groupings might suggest that two different genes are a major influence for AIS in these families. CONCLUSIONS: Nearly all (97%) AIS patients have familial origins. There appears to be at least one major gene, and the differences in penetrance and expressivity in two large unconnected pedigrees might suggest the presence of more than one gene.

Cohort Studies↗
PubMed2006

Surgical treatment of scoliosis in larsen syndrome with bilateral hip dislocation.

STUDY DESIGN: This is a report of a 12-year-old girl treated surgically for scoliosis associated with bilateral hip dislocation in Larsen syndrome. OBJECTIVE: To describe a rare case of scoliosis associated with Larsen syndrome and bilateral hip dislocation that was treated surgically with follow-up for 15 years. SUMMARY OF BACKGROUND DATA: There are few reports of the long-term follow-up of cases involving surgically treated scoliosis associated with bilaterally dislocated hips. METHODS: The patient's spine showed a right thoracic curve (T5-T12) with a Cobb angle of 77 degrees and did not show pelvic obliquity on an anterior-posterior radiograph film. On the sagittal alignment of her spine, the thoracic spine showed an abnormal lordosis (T5-T12: 19 degrees), and the lumbar spine had a hyperlordosis (L1-S1: 57 degrees) with a large lumbosacral angle (72 degrees ). We performed a posterior spinal fusion between T4 and L2 using Cotrel-Dubousset Instrumentation, anticipating the restoration of normal lumbar and cervical lordosis, as well as thoracic kyphosis. RESULTS: The Cobb angle of thoracic scoliosis improved from 77 degrees to 28 degrees, and a thoracic kyphosis of 12 degrees (T5-T12) was obtained. Subsequently, on the sagittal plane, the lumbosacral angle (sacral anteflexion) decreased from 72 degrees to 52 degrees, comparable to that of patients with hip dislocation, and the lumbar lordotic angle increased from 57 degrees to 66 degrees. The restoration of thoracic kyphosis resulted in an increase of lumbar lordosis and decrease of sacral anteflexion. At the 15-year follow-up,although the thoracic scoliosis (T5-T12) had increased to 36 degrees, good coronal and sagittal balance had been maintained. The patient is asymptomatic in her spine and hip. CONCLUSIONS: A case of scoliosis associated with dislocated hips in a patient with Larsen syndrome was successfully treated with posterior correction surgery. Fusion surgery between T4 and L2 provided an ideal sagittal balance of the total spine, while preserving 4 lumbar mobile segments.

Child↗
PubMed2006

Screw position after double-rod anterior spinal fusion in idiopathic scoliosis: an evaluation using computerized tomography.

STUDY DESIGN: A retrospective evaluation of screw position after double-rod anterior spinal fusion in idiopathic scoliosis using computerized tomography (CT). OBJECTIVE: To evaluate screw position and complications related to screw position after double-rod anterior instrumentation in idiopathic scoliosis. SUMMARY OF BACKGROUND DATA: Anterior instrumentation and fusion in idiopathic scoliosis is gaining widespread use. However, no studies have been published regarding the accuracy of screw placement and screw-related complications in double-rod and double-screw anterior spinal fusion and instrumentation in idiopathic thoracolumbar scoliosis surgery. METHODS: CT examinations were performed after surgery in 17 patients with idiopathic scoliosis. At each instrumented level, the position of the screw and the plate relative to the spinal canal, relative to the neural foramen, and relative to the aorta was measured. Complications related to screw position were registered. RESULTS: A total of 189 screws in 17 patients were evaluated. Malposition occurred in 23% (16 patients) of the total number of screws. Three screws (2 patients) were partially in the spinal canal (1%). This resulted in pain in the right leg in 2 patients. However, electromyography showed no abnormalities. At three levels (3 patients), there was contact between the instrumentation and the aorta. However, no vascular complications occurred. A total of 113 screws (10 patients) were placed under fluoroscopic control and 76 screws (7 patients) were placed without use of fluoroscopy. Less screw malposition was observed in the group in which fluoroscopic control was used (19% vs. 30%, not significant). CONCLUSIONS: Screw placement in double-rod anterior spinal fusion in idiopathic scoliosis seems to be technically demanding, and the use of fluoroscopic control results in less frequent malposition. The risk of neurologic and vascular complications is low.

Adolescent↗
PubMed2006

Progression risk of idiopathic juvenile scoliosis during pubertal growth.

STUDY DESIGN: A retrospective study investigated the progression risk of juvenile scoliosis until skeletal maturity or spinal fusion. OBJECTIVES: To define risk factors of curve progression during pubertal growth and analyze the timing of arthrodesis. SUMMARY OF BACKGROUND DATA: Juvenile scoliosis is characterized by a major, extremely variable progression risk. Peak growth velocity is the most critical period. Curve progression related to growth needs to be analyzed critically for an adequate treatment. METHODS: A total of 205 patients, including 163 girls and 42 boys, with juvenile scoliosis were reviewed at skeletal maturity. The scoliosis was divided into juvenile I with an onset of 4-7 years (52 patients) and juvenile II with an onset of 8-10 years (153). Standing and sitting height, weight, Tanner signs, skeletal age, and menarche were regularly assessed. Topographies and Cobb angles of primary and secondary curves were referred to the pubertal growth diagram. RESULTS: Of 205 patients, 99 (48.3%) were operated on. Of 109 curves < or = 20 degrees at onset of puberty, 15.6% progressed > 45 degrees and were fused. Of 56 curves of 21 degrees to 30 degrees, the surgical rate increased to 75.0%. It was 100% for curves > 30 degrees . Curves > 20 degrees, which increased and were operated on, progressed significantly during peak growth velocity (P = 0.0014). Curves that progressed by 6 degrees to 10 degrees/y were fused in 70.9%, curves which increased > 10 degrees/y in 100% of cases (P = 0.0001). This risk was highest for primary thoracic curves: King V, III, and II (P = 0.0001). There was no difference between males and females or juvenile I and II. CONCLUSIONS: Curve pattern, Cobb angle at onset of puberty, and curve progression velocity are strong predictive factors of curve progression. Juvenile scoliosis > 30 degrees increases rapidly and presents a 100% prognosis for surgery (curve > 40 degrees to 45 degrees ). Anticipation is necessary if the scoliosis progresses during the first year of puberty. The prediction is difficult for curves of 21 degrees to 30 degrees during the first 2 years of puberty. Curve pattern and curve progression velocity are useful to detect which curves are likely to progress. From this retrospective analysis, spinal fusion could have been indicated earlier sometimes. An earlier intervention is probably preferable to obtain better curve reduction on a supple spine, even if a perivertebral fusion is necessary. We use the 3 parameters for operative indications. If an early spinal fusion leads to better curve correction needs to be verified on prospective data.

Bone Development↗
PubMed2006

Surgical correction of developmental scoliosis following cardiac transplantation: case report.

STUDY DESIGN: Case report. OBJECTIVE: To present a pediatric patient who underwent successful 2-stage anterior and posterior scoliosis surgery 20 months after cardiac transplantation. SUMMARY OF BACKGROUND DATA: Cardiac transplantation has increased the life expectancy in children with end-stage cardiac failure caused by congenital heart disease or cardiomyopathy. Scoliosis is commonly associated with congenital cardiac disease. Previous reports have suggested that anterior scoliosis surgery is contraindicated after heart transplantation. METHODS: We describe the case of a 13-year-old patient who underwent staged anterior and posterior spinal arthrodesis to correct a severe thoracic developmental scoliosis. RESULTS: The 2 procedures were performed 1 week apart. Following the first stage, pulmonary edema had developed, which required reintubation and administration of diuretics. Significant blood loss occurred during the posterior spinal fusion. The patient had a satisfactory correction of her scoliosis and made a good recovery. Three years after surgery, she had no complaints of her back and had a solid spinal fusion. CONCLUSIONS: Elective anterior and posterior spinal arthrodesis can be safely performed in cardiac transplant recipients with severe developmental scoliosis. This procedure can achieve optimum correction of the curvature and a successful outcome without long-term medical or technical complications.

Adolescent↗
PubMed2004

Radiographic and functional outcome after surgical management of severe scoliosis in skeletally immature patients with muscular dystrophy.

OBJECTIVE: Most patients with muscular dystrophy (MD) develop progressive scoliosis after losing ambulatory status, but some cases develop severe scoliosis at a skeletally immature age before losing ambulatory status. Only a few studies have been conducted in skeletally immature patients with severe scoliosis. The purpose of this study was to assess the functional and cosmetic outcome in skeletally immature patients with severe scoliosis. METHODS: Preoperative, immediate postoperative, and final follow-up radiographs were analyzed in 10 consecutive skeletally immature patients with respect to the Cobb angle degree and the pelvic obliquity angle correction, how long the correction was maintained, and the development of the crankshaft phenomenon. In the functional assessment, the ability to sit balanced, according to the Mulcahy method, and the ability to use hands, according to the Rhyu method, were evaluated. Furthermore, the degree of subjective satisfaction was evaluated in these patients. RESULTS: The average age of the patients was 10.4 years, and the average follow-up period was 33 months with minimum 2 years' follow-up. All 10 patients survived and were available at the follow-up. The mean Cobb and pelvic obliquity angles were 80 degrees and 17 degrees at the time of the surgery, 31 degrees and 3.7 degrees immediately after the surgery, and 35 degrees and 4.7 degrees at the time of the final follow-up, respectively. The initial mean Cobb angle correction averaged 61%, with 78% of pelvic obliquity corrected. These corrections were maintained over time in most cases. At the time of the surgery, the mean volume of blood loss was 1111 mL, with an average operation time of 411 minutes. There were no major complications. At the time of the last follow-up, no patient showed development of the crankshaft phenomenon. The average score for the ability to sit balanced improved from 4.4 to 6.6 according to the Mulcahy evaluation method. The scores for hand use were 2.2-2.7. However, the forced vital capacity of the lungs decreased from a preoperative 48% to 46.1%. CONCLUSIONS: These results indicate that even in very young MD patients with severe scoliosis, acceptable curve correction can be achieved and maintained with surgery. The improved pelvic obliquity and scoliosis angle stabilized the spine, freeing the upper extremities and allowing productive activities characteristic of childhood.

Adolescent↗
PubMed1994

Comparison of curve shape between children with cerebral palsy, Friedreich's ataxia, and adolescent idiopathic scoliosis.

Fourteen patients with cerebral palsy (CP), 12 with Friedreich's ataxia (FA) and 26 with adolescent idiopathic scoliosis (AIS) were studied to determine whether the shape of the scoliosis curve differs between these categories. The slope of the regression relationship between vertebral rotation and lateral deviation was greater for the CP group compared with the FA and AIS groups. The authors conclude that the scoliosis curve pattern of children with Friedreich's ataxia and adolescent idiopathic scoliosis is similar. In contrast, the scoliosis curve of children with CP was distinctly different, with more rotation of the apical vertebrae into the convexity of the scoliosis curve (transverse plane deformity) in relation to the amount of lateral deviation of the apical vertebrae from the spinal axis (coronal plane deformity).

Adolescent↗
PubMed1994

Resting energy expenditure and nutritional state of patients with increased oxygen cost of breathing due to emphysema, scoliosis and thoracoplasty.

BACKGROUND: Weight loss is a well recognised feature of patients with emphysematous chronic obstructive pulmonary disease (COPD). It has been suggested that this weight loss could be due to a hypermetabolic state resulting from the increased oxygen cost of breathing (OCB). To clarify the relation between resting energy expenditure (REE), nutritional state, and OCB these indices were measured in patients with respiratory impairment and an increased OCB due to COPD, scoliosis, and thoracoplasty. METHODS: Eighteen patients (six COPD, six scoliosis, six thoracoplasty) of mean (SD) age 59.9 (8.6) years (8M, 10F) and six controls (45.5 (9.9) years; 2M, 4F) were studied. OCB was estimated by the addition of dead space to the breathing circuit and REE was measured by indirect calorimetry using a ventilated canopy system. Height, arm span, weight, triceps skin fold thickness (TSF), mid-arm muscle circumference (MAMC), forced expiratory volume in one second (FEV1), and vital capacity (VC) were measured in all study subjects. RESULTS: OCB was elevated in all patient groups (mean 7.0 ml/l) compared with controls (1.9 ml/l). All patients with COPD, four with scoliosis, three with thoracoplasty, and none of the controls were < 90% ideal body weight. Mean (SD) measured REE as % predicted (Harris-Benedict equation) was 103.8 (7.6) in patients with COPD, 105.5 (10.9) in those with scoliosis, 106.3 (6.9) in the thoracoplasty patients, and 103.3 (3.4) in controls. One patient with COPD, two with scoliosis, two with thoracoplasty, but no controls were hypermetabolic (REE > 110% predicted). In all groups there was a negative relation between OCB and lung function (OCB v FEV1 r = -0.83 in COPD, -0.62 in scoliosis, -0.67 in thoracoplasty, and -0.76 in controls). There was no correlation between REE and OCB or MAMC. CONCLUSIONS: In patients with respiratory disease OCB (augmented ventilation) is related to lung function but not to REE. This is evidence against the hypothesis that hypermetabolism due to increased oxygen cost of breathing at rest is the sole or major cause of malnutrition in patients with lung disease.

Aged↗
PubMed2003

Results of surgery for scoliosis in Rett syndrome.

The British Isles Survey for Rett Syndrome stores longitudinal health data from clinical examinations and postal questionnaires to monitor health and severity in Rett syndrome, including the presence and severity of scoliosis and the effects of corrective surgery. Scoliosis is rarely present at birth (3% before regression) but usually appears by 25 years (87%). The degree tends to increase with growth and by 16 to 20 years, 43% (75 of 173) of cases with classic Rett syndrome reported severe or operated scoliosis. Surgical correction was reported in 91 classic cases. Following initial postoperative recovery, families considered that the operation had improved general well-being for 84% of individuals (42 of 50 classic cases with postoperative health reports). Thirteen of 50 patients walked independently before surgery, and 12 patients did so following surgery; 2 stopped walking, and 1 who had not walked began to do so. Scoliosis surgery usually benefited sitting posture (82% better, 10% worse), chest episodes (52% better, 6% worse), and digestion of food (42% better, 6% worse). However, toilet function was improved in only 10% and deteriorated in 20%. Families reported short-term problems at operation in 48% (24 of 50) and minor recurrence of scoliosis in 22% (11 of 50). Surgery in a specialized unit is satisfactory management for severe scoliosis in Rett syndrome. Recommendations include planning for surgery when the curve passes 40 degrees, ensuring optimal nutrition before and after surgery, robust fixture of the whole spine in two stages, familiarization of the surgical team with the individual and the disorder before the operation, and inclusion of the main carer in the hospital care team. Parents form an important part of the management team. Families also require support during and after this stressful major procedure.

Adolescent↗
PubMed2006

Predictors of scoliosis in Rett syndrome.

Scoliosis is a common clinical manifestation of Rett syndrome, a neurodevelopmental disorder that almost exclusively affects girls. Following apparently normal development, these girls typically regress and lose previously attained cognitive, social, and motor skills. Severe intellectual and physical disabilities remain throughout life. Mutations in the methyl-CpG-binding protein 2 gene, MECP2, are detected in approximately 80% of cases and are associated with phenotypic variability. Population-based data on Australian cases were used to study the association between early developmental and genetic factors and the onset of scoliosis. The median age at scoliosis onset was 9.80 years, and three quarters of subjects had developed scoliosis by 13 years of age. Children with compromised early development before 6 months, those who were less mobile at 10 months, and those who never walked were more likely to have an earlier onset of scoliosis. When seven common point mutations and large genomic and C-terminal deletions were compared, the R294X mutation appeared to provide some protective effect against the development of scoliosis.

Adolescent↗
PubMed1980

Scoliosis in symptomatic spondylolisthesis.

The association between spondylolisthesis and scoliosis was studied in 84 patients who presented during a 30-year period with symptomatic spondylolisthesis. The incidence of scoliosis was 42 per cent, the majority of cases being lumbar or thoracolumbar curves of less than 15 degrees. The incidence was highest in the group of patients with spondylolisthesis at L4--5 where all except one had scoliosis. Scoliosis was present in 47 per cent of patients with dysplastic spondylolisthesis at the lumbosacral junction; in this group, the incidence of scoliosis was greater where the displacement exceeded 25 per cent. The lowest incidence (25 per cent) was found in the group with isthmic spondylolisthesis at the lumbosacral junction. There appeared to be no relationship between excessive lumbar lordosis or tightness of the hamstrings and scoliosis.

Adolescent↗
PubMed1983

Infantile idiopathic scoliosis: can it be prevented?

The changing incidence of idiopathic scoliosis in 672 patients who attended the Edinburgh Scoliosis Clinic between 1968 and 1982 inclusive is reported. Of these patients, 144 had infantile, 51 juvenile and 477 adolescent idiopathic scoliosis. Thirty-seven of the infantile curves progressed and 107 resolved. The relative frequency of both progressive and resolving infantile idiopathic scoliosis (given as a percentage of the total number of patients with idiopathic scoliosis) declined from 41.75 per cent in the four years from 1968 to 1971, to four per cent in the three years from 1980 to 1982. It is suggested that infantile idiopathic scoliosis is a preventable deformity and that the position in which the infant is laid may be a causative factor.

Adolescent↗
PubMed1993

Scoliosis in children after thoracotomy for aortic coarctation.

A follow-up study was performed to assess the prevalence of scoliosis in 160 patients in whom aortic coarctation had been treated operatively through a left posterolateral incision in the fourth intercostal space. Scoliosis had not been seen in any patient before the thoracotomy, but a scoliosis of 10 degrees or more was observed in thirty-five patients (22 per cent) at the follow-up examination, which was performed an average of seven years after the thoracotomy. The scoliosis was a thoracic curve and was directed to the left in twenty-six of the thirty-five patients. Most of the curves were mild (between 10 and 20 degrees) and hardly progressed during the follow-up period. The high-risk period for the onset of scoliosis began about three years after the operation. The prevalence of scoliosis did not correlate significantly with the age or sex of the patients.

Adolescent↗