Heritable disorders of connective tissue

Study design This study is a retrospective review of the initial enrollment data from a prospective multicentered study of adult spinal deformity. Objectives The purpose of this study is to correlate radiographic measures of deformity with patient-based outcome measures in adult scoliosis. Summary of background data Prior studies of adult scoliosis have attempted to correlate radiographic appearance and clinical symptoms, but it has proven difficult to predict health status based on radiographic measures of deformity alone. The ability to correlate radiographic measures of deformity with symptoms would be useful for decision-making and surgical planning. Methods The study correlates radiographic measures of deformity with scores on the Short Form-12, Scoliosis Research Society-29, and Oswestry profiles. Radiographic evaluation was performed according to an established positioning protocol for anteroposterior and lateral 36-inch standing radiographs. Radiographic parameters studied were curve type, curve location, curve magnitude, coronal balance, sagittal balance, apical rotation, and rotatory subluxation. Results The 298 patients studied include 172 with no prior surgery and 126 who had undergone prior spine fusion. Positive sagittal balance was the most reliable predictor of clinical symptoms in both patient groups. Thoracolumbar and lumbar curves generated less favorable scores than thoracic curves in both patient groups. Significant coronal imbalance of greater than 4 cm was associated with deterioration in pain and function scores for unoperated patients but not in patients with previous surgery. Conclusions This study suggests that restoration of a more normal sagittal balance is the critical goal for any reconstructive spine surgery. The study suggests that magnitude of coronal deformity and extent of coronal correction are less critical parameters.

Correlation of radiographic parameters and clinical symptoms in adult scoliosis

Low back pain is a symptom that cannot be validated by an external standard. It is a disorder with many possible etiologies, occurring in many groups of the population, and with many definitions. Low back pain is a common problem, with a prevalence in the United States ranging from 8% to 56%. It is estimated that 28% experience disabling low back pain sometime during their lives, 14% experience episodes lasting at least 2 weeks, 8% of the entire working population will be disabled in any given year, and the lifetime prevalence of low back pain is 65% to 80%. It is believed that most episodes of low back pain will be short-lived and that 80% to 90% of attacks of low back pain resolve in about 6 weeks, irrespective of the administration or type of treatment. However, multiple studies in the late 90s showed recurrent or chronic low back pain, evaluated at 3 months, 6 months, or 12 months, ranging from 35% to 79%. Risk factors of low back pain are multifactorial, with many possible etiologies. Multiple risk factors of low back pain and lower-extremity pain include physical factors, social demographic characteristics, habits, and psychosocial factors. This review will discuss the epidemiology of low back pain, with emphasis on frequency, causes, and consequences of low back pain; the influence of age, gender, morphologic characteristics, and genetics; and the influence of occupational, mechanical, social, habitual, and psychological factors.

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Epidemiology of low back pain.

https://www.ipd.gu.se/digitalAssets/1252/1252566_Artikel_sept_Aina_Lancet_2_.pdf

Adolescent idiopathic scoliosis.

From the material and data reviewed in our study of 405 patients, it appears that postoperative correction of the thoracic spine approximately equals the correction noted on preoperative side-bending roentgenograms. Selective thoracic fusion can be safely performed on a Type-II curve of less than 80 degrees, but care must be taken to use the vertebra that is neutral and stable so that the lower level of the fusion is centered over the sacrum. The lumbar curve spontaneously corrects to balance the thoracic curve when selective thoracic fusion is performed and the lower level of fusion is properly selected. In Type-III, IV, and V thoracic curves the lower level of fusion should be centered over the sacrum to achieve a balanced, stable spine.

The selection of fusion levels in thoracic idiopathic scoliosis

In seventy patients with 104 club feet that were treated at our hospital and followed for ten to twenty-seven years after treatment, the functional results were satisfactory according to our rating system in 88.5 per cent of the feet, and 90 per cent of the patients were satisfied with both the appearance and function of the club foot. However, in the majority of the patients, foot and ankle motion was limited and the talocalcaneal angles as seen on the anteroposterior and lateral roentgenograms were not fully corrected. The amount of motion in the joints of the foot and ankle and the correction of the lateral talocalcaneal angle correlated with the degree of patient satisfaction and the functional rating of the club foot. Transfer of the anterior tibial tendon to the third cuneiform appeared to prevent relapse.

Long-term results of treatment of congenital club foot.

ContextPrevious long-term studies of idiopathic scoliosis have included patients
with other etiologies, leading to the erroneous conclusion that all types
of idiopathic scoliosis inevitably end in disability. Late-onset idiopathic
scoliosis (LIS) is a distinct entity with a unique natural history.ObjectiveTo present the outcomes related to health and function in untreated
patients with LIS.Design, Setting, and PatientsProspective natural history study performed at a midwestern university
with outpatient evaluation of patients who presented between 1932 and 1948.
At 50-year follow-up, which began in 1992, 117 untreated patients were compared
with 62 age- and sex-matched volunteers. The patients' mean age was 66 years
(range, 54-80 years).Main Outcome MeasuresMortality, back pain, pulmonary symptoms, general function, depression,
and body image.ResultsThe estimated probability of survival was approximately 0.55 (95% confidence
interval [CI], 0.47-0.63) compared with 0.57 expected for the general population.
There was no significant difference in the demographic characteristics of
the 2 groups. Twenty-two (22%) of 98 patients complained of shortness of breath
during everyday activities compared with 8 (15%) of 53 controls. An increased
risk of shortness of breath was also associated with the combination of a
Cobb angle greater than 80° and a thoracic apex (adjusted odds ratio,
9.75; 95% CI, 1.15-82.98). Sixty-six (61%) of 109 patients reported chronic
back pain compared with 22 (35%) of 62 controls (P =
.003). However, of those with pain, 48 (68%) of 71 patients and 12 (71%) of
17 controls reported only little or moderate back pain.ConclusionsUntreated adults with LIS are productive and functional at a high level
at 50-year follow-up. Untreated LIS causes little physical impairment other
than back pain and cosmetic concerns.

/pdf/health-and-function-of-patients-with-untreated-idiopathic-4scgnvdoyg.pdf

Health and function of patients with untreated idiopathic scoliosis: a 50-year natural history study.

Objectives. The purpose of this study was to evaluate the efficacy of the Ponseti method in reducing extensive corrective surgery rates for congenital idiopathic clubfoot. Methods. Consecutive case series were conducted from January 1991 through December 2001. A total of 157 patients (256 clubfeet) were evaluated. All patients were treated by serial manipulation and casting as described by Ponseti. Main outcome measures included initial correction of the deformity, extensive corrective surgery rate, and relapses. Results. Clubfoot correction was obtained in all but 3 patients (98%). Ninety percent of patients required ≤5 casts for correction. Average time for full correction of the deformity was 20 days (range: 14–24 days). Only 4 (2.5%) patients required extensive corrective surgery. There were 17 (11%) relapses. Relapses were unrelated to age at presentation, previous unsuccessful treatment, or severity of the deformity (as measured by the number of Ponseti casts needed for correction). Relapses were related to noncompliance with the foot-abduction brace. Four patients (2.5%) underwent an anterior tibial tendon transfer to prevent further relapses. Conclusions. The Ponseti method is a safe and effective treatment for congenital idiopathic clubfoot and radically decreases the need for extensive corrective surgery. This technique can be used in children up to 2 years of age even after previous unsuccessful nonsurgical treatment.

Radical reduction in the rate of extensive corrective surgery for clubfoot using the Ponseti method.

One hundred and thirty-three curves in 102 patients who were followed for an average of 40.5 years were evaluated to quantitate curve progression after skeletal maturity and for prognostic factors leading to curve progression. Sixty-eight per cent of the curves progressed after skeletal maturity. In general, curves that were less than 30 degrees at skeletal maturity tended not to progress regardless of curve pattern. In thoracic curves the Cobb angle, apical vertebral rotation, and the Mehta angle were important prognostic factors. In lumbar curves the degree of apical vertebral rotation, the Cobb angle, the direction of the curve, and the relationship of the fifth lumbar vertebra to the intercrest line were of prognostic value. Translatory shifts played an important role in curve progression. Curves that measured between 50 and 75 degrees at skeletal maturity, particularly thoracic curves, progressed the most.

Curve progression in idiopathic scoliosis.

Two hundred and nineteen patients with untreated adolescent idiopathic scoliosis who were seen at the University of Iowa between 1932 and 1948 were studied, and recent information was available on 194 of the patients. The mortality rate was 15 per cent. Backache was somewhat more common in these patients than in the general population, although it was never disabling. The backache was unrelated to the presence of osteoarthritic changes on roentgenograms. Many curves continued to progress slightly in adult life, particularly thoracic curves that had reached between 50 and 80 degrees at skeletal maturity. The lumbar components of combined curves between 50 and 74 degrees also tended to progress. Pulmonary function was affected only in patients with thoracic curves.

Ignacio V. Ponseti

Papers

Long-term results of treatment of congenital club foot.

Health and function of patients with untreated idiopathic scoliosis: a 50-year natural history study.

Radical reduction in the rate of extensive corrective surgery for clubfoot using the Ponseti method.

Curve progression in idiopathic scoliosis.

Idiopathic scoliosis: long-term follow-up and prognosis in untreated patients.