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Spinal arthrodesis with instrumentation for thoracolumbar kyphosis in pediatric achondroplasia.

STUDY DESIGN: Retrospective radiograph and chart review. OBJECTIVES: To assess the safety, efficacy, and complications after posterior spinal arthrodesis with instrumentation for thoracolumbar kyphosis in the pediatric achondroplast. SUMMARY OF BACKGROUND DATA: The conventional approach of anterior and posterior arthrodesis has achieved minimal correction, avoided instrumentation, and had a high risk of neurologic deterioration. To the current authors' knowledge, there are only two reports of successful instrumentation with pedicle screw fixation for kyphosis in pediatric achondroplasts; the outcome of such procedures remains largely unknown. METHODS: The current authors evaluated the results of posterior spinal arthrodeses with instrumentation performed between 1998 and 2001 on 12 consecutive patients (mean age, 12 years). Indications for arthrodesis were concomitant laminectomy for neurologic symptoms or progressive deformity. Arthrodesis methods included anterior and posterior arthrodesis (5 patients) and posterior arthrodesis only (7 patients). Intraoperative somatosensory-evoked potentials, pedicle screw stimulation, and/or a wake-up test were used to confirm neurologic status in all patients. RESULTS: Successful fusion was obtained in all patients. No intraoperative or postoperative neurologic deterioration was encountered. Mean improvement in kyphotic deformity was 50%. Complications included three instrumentation fractures (2 patients) and one dural leak. Parents rated outcome as excellent (4), good (6), and fair (2). No patients have subsequently experienced deformity progression. CONCLUSIONS: Spinal arthrodesis with vertebral body and/or pedicle screw instrumentation was a reliable technique for treating thoracolumbar kyphosis in achondroplasia and did not precipitate any of the neuromonitoring difficulties or neurologic deficits that have been reported in previous studies.

Achondroplasia↗

Superior mesenteric artery syndrome following correction of kyphosis in an adult.

Superior mesenteric artery (SMA) syndrome is well described in the pediatric literature; however, to date, it has not been described in an adult individual undergoing correction of thoracic kyphosis. Medical history, physical findings, and the results of imaging studies were analyzed to describe the patient's onset of gastrointestinal obstruction following surgical correction of thoracic kyphosis. The postoperative course was marked by the onset of nausea and vomiting on postoperative day 24. The patient was admitted to the hospital, and the small bowel follow-through study showed blockage of the third portion of the duodenum at the level of the SMA. Oral intake was restricted, and total parenteral nutrition was instituted with complete resolution of her symptoms. At 1 year postoperatively, the patient was symptom-free. Although vascular compression of the duodenum with subsequent nausea and vomiting and electrolyte imbalance, caused by the relative lengthening of the spine, is most commonly associated with surgical correction of coronal plane deformities (ie, scoliosis), it may occur in patients undergoing correction of thoracic kyphosis as well. SMA syndrome is a well known potential complication in adolescents, and it should also be taken into consideration in adults undergoing deformity correction. Early institution of general medical measures of nasogastric suction, oral intake restriction, and intravenous alimentation can be successful.

Female↗

Lumbar kyphosis in Hunter's disease (MPS ii).

Although radiological involvement of the lower dorsal and upper lumbar vertebrae is common in the severe form of Hunter's disease (MPS II), there are reports in the literature that clinical kyphosis does not occur. We report a boy with marked clinical kyphosis in whom the diagnosis of MPS II was proved by demonstrating a severe deficiency of serum and leucocyte iduronate-sulphate sulphatase and an accelerated incorporation of radiosulphate into his cultured fibroblast glycosaminoglycans, which could not be corrected by the product of other typed reference MPS II cells. The existence of several other genetic diseases, sometimes complicated by kyphosis, was excluded by assay of fibroblast lysosomal enzymes.

Adolescent↗

Myelodysplasia: the natural history of kyphosis and scoliosis. A preliminary report.

The results of this study support the concept that the levels of the bifid vertebral arch and of functional motor activity are significant influences on the development of scoliosis and kyphosis in patients born with myelodysplasia. 'Significant' degrees of scoliosis and kyphosis may appear at any time during life, but most frequently occur during late childhood or adolescence. Not all 'significant' curvatures require treatment, nor do all children with myelodysplasia and factors predisposing to kyphosis and scoliosis develop these complications. It is believed that the preliminary cumulative percentage curves presented in this study will be of value in counselling parents of afflicted newborn infants, and in planning the management of patients born with myelodysplasia.

Adolescent↗

Osteoporotic vertebral collapse: percutaneous vertebroplasty and local kyphosis correction.

Forty-six cases of osteoporotic vertebral collapse (27 thoracic, 19 lumbar) were treated by means of percutaneous vertebroplasty in a hyperlordosis position. Institutional review board approval and informed consent were obtained. Kyphosis reducibility was preprocedurally estimated from the angular difference between neutral and hyperlordosis positions. Effective reduction was the angular difference in neutral positions before and after vertebroplasty. Reduction (< or =14 degrees ; mean, 6.43 degrees) was obtained in cases with estimated reducibility greater than 5 degrees (31 cases, 67%), which is a 34% (6.5 degrees of 19.1 degrees ) mean reduction. A significantly greater level of kyphosis reduction was observed in cases with intravertebral clefts (20 cases, 43%) at hyperlordosis than in those without (7.2 degrees vs 4.9 degrees ; P < .01). Vertebroplasty may reduce kyphosis due to localized collapsed vertebrae; intravertebral mobility and cleft suggest this possibility.

Aged↗

Insufficiency fractures of the sternum: a consequence of thoracic kyphosis?

Osteoporosis often leads to thoracic compression fractures and progressive thoracic kyphosis. The kyphosis creates a deforming stress on the sternum that may eventuate in an insufficiency-type stress fracture. The findings in four women with osteoporosis, thoracic kyphosis, and insufficiency fractures of the sternum are reported.

Adult↗

Congenital kyphosis in myelomeningocele. The effect of cordotomy on bladder function.

To determine the effect of cordotomy on the function of the bladder during surgical correction of congenital kyphosis in myelomeningocele, we reviewed 13 patients who had this procedure between 1981 and 1996. The mean age of the patients at operation was 8.9 years (3.7 to 16) and the mean follow-up was 4.8 years (1.3 to 10.8). Bladder function before and after operation was assessed clinically and quantitatively by urodynamics. The mean preoperative kyphosis was 117 degrees (52 to 175) and decreased to 49 degrees (1 to 89) immediately after surgery. At the latest follow-up, a mean correction of 52% had been achieved. Only one patient showed deterioration in bladder function after operation. Eight out of the nine patients who had urodynamic assessment had improvement in bladder capacity and compliance, and five showed an increase in urethral pressure. One patient developed a spastic bladder and required subsequent surgical intervention. Cordotomy, at or below the level of the kyphosis, allows excellent correction of the structural deformity.

Adolescent↗

Simultaneous anterior-posterior approach through a costotransversectomy for the treatment of congenital kyphosis and acquired kyphoscoliotic deformities.

BACKGROUND: Congenital kyphosis and acquired kyphoscoliotic deformities are uncommon but are potentially serious because of the risk of progressive deformity and possible paraplegia with growth. Our current approach for the treatment of these deformities is to use a single posterior incision and costotransversectomy to provide access for simultaneous anterior and posterior resection of a hemivertebra or spinal osteotomy, followed by anterior and/or posterior instrumentation and arthrodesis. To our knowledge, this approach has not been reported previously. METHODS: The medical records and radiographs for sixteen patients who had been managed at our institution for the treatment of congenital kyphosis and acquired kyphoscoliosis between 1988 and 2002 were analyzed. The mean age at the time of surgery was twelve years. The diagnosis was congenital kyphosis for fourteen patients and acquired kyphoscoliotic deformities following failed previous surgery for two. The mean preoperative kyphotic deformity was 65 degrees (range, 25 degrees to 160 degrees ), and the mean scoliotic deformity was 47 degrees (range, 7 degrees to 160 degrees ). Fifteen patients were managed with vertebral resection or osteotomy through a single posterior approach and costotransversectomy, anterior and posterior arthrodesis, and posterior segmental spinal instrumentation. The other patient was too small for spinal instrumentation at the time of vertebral resection. A simplified outcome score was created to evaluate the results. RESULTS: The mean duration of follow-up was 60.1 months. The mean correction of the major kyphotic deformity was 31 degrees (range, 0 degrees to 82 degrees ), and the mean correction of the major scoliotic deformity was 25 degrees (range, 0 degrees to 68 degrees ). Complications occurred in four patients; the complications included failure of posterior fixation requiring revision (one patient), lower extremity dysesthesias (one patient), and late progressive pelvic obliquity caudad to the fusion (two patients). The outcome, which was determined with use of a simplified outcomes score on the basis of patient satisfaction, was rated as satisfactory for thirteen patients, fair for two patients, and poor for one patient. CONCLUSIONS: A simultaneous anterior and posterior approach through a costotransversectomy is a challenging but safe, versatile, and effective approach for the treatment of complex kyphotic deformities of the thoracic spine, and it minimizes the risk of neurologic injury. LEVEL OF EVIDENCE: Therapeutic Level IV.

Adolescent↗

Surgical treatment of congenital kyphosis associated with progressive spastic paralysis in an adult patient.

A 38-year-old man presented with untreated congenital kyphosis associated with progressive spastic gait. To prevent progression of the spastic paralysis, rigid correction of the severe spinal deformity arising from the congenital kyphosis was performed by one-stage posterior closing-wedge osteotomy, without occurrence of neurological complications. Progression of the paralysis has not been identified for 30 months after the operation and a slight improvement in gait was recognized. The current case is categorized as type I deformity of congenital kyphosis in the upper thoracic spine, which is normally treated surgically before the adolescent growth phase begins.

Adult↗

Effects of sex hormones on congenital kyphosis in Ishibashi rats.

We examined the influence of estrogen and testosterone on the development of congenital kyphosis in Ishibashi rats. Neither hormone caused a growth spurt. Estrogen suppressed the spinal growth and the progression of kyphosis in both sexes, and accelerated narrowing of the epiphyseal plates, which became apparent at 6 weeks after birth in females and 8 weeks in males. In both sexes, maturity of the spine was accelerated and trabeculae were hypertrophied by estrogen. Testosterone suppressed the progression of kyphosis in males, but not in females. Histologically, testosterone had no effects on growth cartilage, but did produce thinning of the bone trabeculae in males.

Animals↗

Surface stereophotogrammetry of thoracic kyphosis.

The thoracic kyphosis angles of 16 normal individuals, 10 patients with Scheuermann's disease and 11 with adolescent idiopathic scoliosis were measured both radiographically and from Integrated Shape Imaging System (ISIS) scans obtained by surface stereophotogrammetry. There was a high correlation between the two measures. The method of kyphosis measurement from ISIS scans was then used for 30 patients with adolescent idiopathic scoliosis who underwent corrective surgery. A significant reduction in thoracic kyphosis was observed postoperatively. In another group of 28 patients managed conservatively by bracing, some hypokyphosis developed after treatment. However, we found no association between hypokyphosis and curve progression.

Adolescent↗

[Influence of kyphosis on the age-related decline in pulmonary function].

To examine the influence of kyphosis on pulmonary function in the elderly, 300 consecutive healthy subjects (aged 20-94 years old) were evaluated using pulmonary function tests (TLC, VC, FEV1, RV) and static maximal pressures (PImax and PEmax). Kyphosis of the thoracic bones was measured at Cobb's angle. VC, FEV1. PImax and PEmax showed a significant age-dependent decline. Cobb's angle had a positive relationship with age and was negatively correlated with TLC, VC, FEV1 and PImax. Further, RV/TLC strongly correlated with Cobb's angle and PImax. Stepwise regression analysis revealed that the kyphotic angle was an independent determinant for PImax and RV/TLC. These results indicate that kyphosis significantly affects lung volume and maximal inspiratory pressure in the elderly and those affected should have careful assessment of their pulmonary function and respiratory pressures.

Adult↗

Cervical corpectomy and plate fixation for postlaminectomy kyphosis.

Between 1987 and 1991, 20 patients with symptomatic postlaminectomy kyphosis were treated with anterior decompression, bone graft, and anterior cervical plate. The patients were predominantly male (14:6) with a mean age of 58 years. The initial laminectomy was performed for either spondylosis (80%) or spinal tumor (20%). All patients had anterior compressive pathology, which was associated with instability (45%), neck pain (75%), myeloradiculopathy (90%), or severe neck deformity (30%). The mean degree of kyphosis was 38 degrees. Treatment consisted of a trial of cervical traction (75%), anterior corpectomy (95%), intersegmental decompression (5%), bone fusion (100%), and fixation with either Caspar (85%) or Synthes (15%) anterior plating at a mean of 3.8 levels. Halo fixation was used in 10% of patients. Postoperative complications included vocal cord paresis (15%), pneumonia (10%), wound dehiscence (5%), and screw pull-out (5%). At follow-up evaluation, a mean of 28 months after treatment, all patients had a solid fusion and a mean curvature improvement to 16 degrees residual kyphosis. Neurologically, 10% were cured, 55% were improved and returned to premorbid function, 30% were stable, and 5% had late progression. These data suggest that immediate fixation with anterior plating facilitates solid fusion, maintains spinal curvature, and promotes neurological improvement.

Adult↗

Correlation of anomalous vascular and osseous anatomy in congenital kyphosis type I. Case report.

Congenital kyphosis Type I results from failure of formation of the vertebral body (VB). The clinical course is characterized by progressive deformity and neurological dysfunction unless the patient undergoes fusion. It has been postulated that failure of VB formation may be secondary to anomalies of the segmental arteries arising from the aorta. There is limited clinical evidence in the literature, however, to support this hypothesis. The authors report on the case of an adult patient with congenital kyphosis Type I in whom spinal angiography correlated vascular with vertebral anomalies. This 32-year-old man presented with back pain, lower-extremity paresthesias, and incomplete voiding, which had progressed over several years. Imaging revealed that the L-1 and L-2 vertebrae had not fully formed, causing kyphotic deformity such that the anterior portion of T-12 nearly articulated with that of L-3; this anomaly produced significant mobility in flexion and extension. Magnetic resonance imaging revealed distal spinal cord atrophy and signal change on T2-weighted images. Spinal angiography showed that the right and left segmental arteries at both L-1 and L-2 arose from a common trunk from the aorta. This was confirmed during subsequent T12-L3 anterior-posterior fusion. Postoperatively the patient experienced relief of pain and remained neurologically stable throughout a 3-year follow-up period. The presence of corresponding anomalies of the segmental arteries and vertebrae in this case of congenital kyphosis Type I indicates that a relationship may exist in the genesis of aberrant spinal vertebral and vascular structures.

Adult↗

Scheuermann's kyphosis in adolescents and adults: diagnosis and management.

Scheuermann's thoracic kyphosis is a structural deformity classically characterized by anterior wedging of 5 degrees or more of three adjacent thoracic vertebral bodies. Secondary radiographic findings of Schmorl's nodes, endplate narrowing, and irregular endplates confirm the diagnosis. The etiology remains unclear. Adolescents typically present to medical attention because of cosmetic deformity; adults more commonly present because of increased pain. The indications for treatment are similar to those for other spinal deformities, namely, progression of the deformity, pain, neurologic compromise, and cosmesis. The adolescent with pain associated with Scheuermann's kyphosis usually responds to physical therapy and a short course of anti-inflammatory medications. Bracing has been shown to be effective in controlling a progressive curve in the adolescent patient. For the adult who presents with pain, the early mainstays of treatment are physical therapy, anti-inflammatory medications, and behavioral modification. In patients, either adolescent or adult, with a progressive deformity, refractory pain, or neurologic deficit, surgical correction of the deformity may be indicated. Surgical correction should not exceed 50% of the initial deformity. Distally, instrumentation should be extended beyond the end vertebral body to the first lordotic disk to prevent the development of distal junctional kyphosis.

Adolescent↗

Increased rate of fractures and severe kyphosis: sequelae of living into adulthood with cystic fibrosis.

BACKGROUND: Osteoporosis occurs in patients with cystic fibrosis as they age, but its clinical implications are not well defined. OBJECTIVE: To determine the clinical effect of decreased bone mineral density in adults with cystic fibrosis and to assess possible clinical predictors of osteoporosis. DESIGN: Retrospective cohort study. SETTING: Academic cystic fibrosis center. PATIENTS: 70 adults with late-stage cystic fibrosis who were referred for lung transplantation. MEASUREMENTS: Bone mineral density was measured with dual-energy x-ray absorptiometry, patient-reported fracture events were confirmed by radiography, and kyphosis angles were measured by using the Cobb method. RESULTS: Mean bone mineral densities for the spine, femur, and total body were severely depressed in patients with cystic fibrosis, averaging 2 SDs below those of age-matched normal controls (P < 0.001). Patient interviews showed that 54 fractures had occurred over 1410 patient-years, and chest radiographs showed evidence of 14 additional rib and 62 additional vertebral compression fractures. The database (which covered 1410 patient-years) showed that fracture rates were approximately twofold greater in women with cystic fibrosis aged 16 to 34 years (P = 0.015) and men with cystic fibrosis aged 25 to 45 years (P = 0.04) than in the general population. Vertebral compression and rib fractures were 100- and 10-fold more common than expected, respectively (P < 0.001 for both comparisons). The mean kyphosis angle (+/- SD) for this group was markedly abnormal (44 +/- 14 degrees; 62% > or = 40 degrees) and probably contributed to diminished stature (mean height loss, 5.8 cm in men with cystic fibrosis and 5.9 cm in women with cystic fibrosis). Cumulative prednisone dose, body mass index, and age at puberty were the strongest predictors of bone mineral density. CONCLUSIONS: Osteoporosis is universal in adults with late-stage cystic fibrosis, and its complications include increased fracture rates and severe kyphosis.

Adult↗

[The influence of kyphosis on lung function in young scoliotic patients (author's transl)].

By assessment of partial correlation in the field of lung function in kypho-scoliosis, the direct influence of kyphosis on the upward-shift of the mid-respiratory level (FRC% TLC) can be documented. It is shown that several other lung function parameters are dependent more or less on a change of volume balance in the deformed chest and, thus, indirectly on the degree of kyphosis. The degree of scoliosis which was the presenting symptom in our series, shows, of course, a highly significant direct correlation to vital capacity, which in itself is the source of changes in several other lung function parameters. The role of kyphosis in the development of lung function during therapy is being discussed.

Adolescent↗

Estimated kyphosis and lordosis changes at follow-up in patients with idiopathic scoliosis.

The objective of this study was to verify the accuracy of surface measurements to estimate the magnitude of sagittal curvature changes at follow-up. Ninety-seven patients with idiopathic scoliosis were evaluated at two different visits (interval: 15.7 months). Kyphosis and lordosis were measured on the lateral radiograph. Surface measurements rely on localization of spinous process landmarks using a video-based system. Multiple regression analyses were performed to estimate the sagittal curvatures on the second visit. The regression was significant for both kyphosis and lordosis. The mean absolute difference between the estimate and the radiologic measurement was 3.3 degrees for kyphosis and 3.2 degrees for lordosis. The difference between the estimated change and the observed change between visits showed mean absolute differences of 3.4 degrees and 2.7 degrees, respectively. The proposed strategy could be used during follow-up to reduce patient irradiation without loss of sagittal information.

Adolescent↗