Robert N. Hensinger

Klippel-Feil syndrome; a constellation of associated anomalies.

Of fifty patients with the diagnosis of Klippel-Feil syndrome, less than half had the classic clinical triad of findings, while more than half had scoliosis, and a third had renal anomalies. All patients were at risk of having other serious, but less apparent, anomalies, including: Sprengels deformity (twenty-one patients), impairment of hearing (fifteen patients), synkinesia (nine patients), and congenital heart disease (seven patients). The discovery of one of these lesions should, therefore, stimulate a careful search for associated anomalies.

Emergency transport and positioning of young children who have an injury of the cervical spine: the standard backboard may be Hazardous

In ten children who were less than seven years old, an unstable injury of the cervical spine was found to have anterior angulation or translation, or both, on initial lateral radiographs that were made with the child supine on a standard flat backboard. In all ten patients, extension was the proper position for reduction of the injury of the cervical spine. Young children have a large head in comparison with the rest of the body. When a young child is positioned on a standard backboard, the neck may be forced into relative kyphosis. Supine and upright lateral radiographs that were made of seventy-two children who did not have a fracture also demonstrated more relative cervical kyphosis in younger children when they were in the supine position. Calculations from anthropometric data documented disproportionate rates of growth of the head and the chest. The circumference of the head grows logarithmically, but the circumference of the chest grows linearly. This disproportionate growth causes young children to have a relatively large head. When they lie supine, the neck is flexed. To prevent undesirable cervical flexion in young children during emergency transport and radiography, a standard backboard can be modified to provide safer alignment of the cervical spine. This can be accomplished by the use of a recess for the occiput to lower the head or of a double mattress pad to raise the chest.

Aneurysmal bone cysts in young children

We reviewed seven young children (< or = 10 years) with aneurysmal bone cysts. There were four girls and three boys. Six had involvement of the long bones and one had involvement of the clavicle. The average age was 5.5 years (range 2.9-10.6 years). Initial treatment was curettage and bone grafting. There were recurrences in five of the seven children (71%). This represented 100% of children with radiographically aggressive or active lesions. The recurrences appeared rapidly, at an average of 8 months from the first procedure. The mitotic index of the initial lesion did not correlate with that of the recurrent lesion. Surgical management of the recurrences must be handled individually, but repeat curettage and grafting is only recommended when surgical resection is not possible. This high rate of recurrence in radiographically aggressive or active aneurysmal bone cysts in young children should be considered when planning treatment, and in the preoperative counseling of parents.

Back pain and vertebral changes simulating scheuermann’s disease

Summary Nineteen adolescent patients who presented with mechanical-type back pain and vertebral changes consisting of intravertebral disc herniation, disc space narrowing, and minimal wedge deformity are described. The symptoms and signs were primarily located at the dorsolumbar junction. A specific strenuous activity or traumatic event was clearly associated with the onset of symptoms in 16 of the 19 patients. Spondylolysis or spondylolisthesis (grade I or II) at L5-S1 was also found in 32% of the patients. Most patients responded well to a program of rest, exercises, salicylates, and temporary avoidance of the particular activity involved. Some required orthoses to obtain relief. No patient had a progressive kyphotic deformity during the follow-up period. The relationship of this condition to increased stresses applied to the immature spine, particularly in a preflexed posture, is emphasized.

Complications of fusion to the upper cervical spine

Forty-seven operations for posterior fusion of the upper cervical spine were reviewed for complications. Alarmingly, only 11 patients had an entirely uncomplicated course. Most complications were minor, but there were four nonunions, one requiring reoperation. Although seven patients had increased neurologic deficits after surgery, only one was permanent. There was one death due to technical error. Patients with significant instability, myelopathy, prior failed fusions, or unreducible dislocations are at high risk for perioperative neurologic complications.

Osteoid osteoma and osteoblastoma of the spine

Eleven patients with spinal osteoid osteoma and six patients with spinal osteoblastoma treated between 1975 and 1990 were reviewed to characterize the tumors as they affect the spine and to define the important differences between the two tumors. All patients with cervical osteoid osteoma presented with pain, limited range of motion of the neck, and torticollis. Four osteoblastomas had soft-tissue components in the epidural space, necessitating dissection of the tumor from the dura. No soft-tissue component was found in any of the osteoid osteomas. Our results were similar to a metaanalysis of the clinical, radiographic, and surgical findings of all published cases of spinal osteoid osteoma and osteoblastoma. Important features that have not been emphasized in the literature are the high incidence of torticollis with cervical lesions and the frequent association of epidural invasion with osteoblastoma. Surgeons treating osteoblastoma of the spine should be prepared to dissect tumor from the dura.

Fusion of the upper cervical spine in children and adolescents. An analysis of 17 patients.

A retrospective review of 17 immature patients who underwent posterior spinal fusion of C1–C2 or C1–C3 was performed to establish the efficacy of the procedure, to determine the incidence of complications, and to identify those at high risk for complications. All had radiographic C1–C2 instability or dislocation. Etiologies included os odontoideum, fixed rotatory subluxation, atlantoaxial subluxation, type II dens fracture nonunion, and nonunion of a Hangmans fracture. Before surgery, 10 patients were neurologically intact, and seven had neurologic findings. Associated diagnosis included Downs syndrome in three and Ehler–Danhlos in one. Follow-up averaged 25 months. One neurologically intact patient had a C2 radiculopathy that resolved by 1 year. Three patients with Downs syndrome had complications: one with a preoperative Brown-Sequard syndrome had transient worsening in the immediate postoperative period, one with a preoperative myelopathy developed a late recurrence of a severe myelopathy that required odontectomy, and another sustained an intraoperative spinal cord contusion followed by postoperative quadriplegia and death due to respiratory failure. Of seven reporting neurologic symptoms before surgery, two had residual deficit in the late postoperative period. These two represented preventable technical errors. There were two nonunions, one of which required occiput to C2 fusion. In general, posterior spinal fusion of the upper cervical spine was found to be a reliable, safe, and predictable procedure, but extra caution should be employed when considering arthrodesis in patients with ongoing spinal cord compression, fixed dislocations, and inherited ligamentous laxity.

The role of melatonin in the pathogenesis of adolescent idiopathic scoliosis

Study Design. A matched, case‐control study comparing melatonin production in female patients with and without adolescent idiopathic scoliosis. Objectives. To determine whether melatonin production is decreased in adolescent idiopathic scoliosis. Summary of Background Data. A central etiology for idiopathic scoliosis has never been established. Previous authors have produced experimental scoliosis in chickens after pinealectomy, preventable by administration of melatonin. They suggested that a defect in melatonin synthesis might be involved in the pathogenesis of human idiopathic scoliosis. Methods. Nine female adolescents with no medical problems, normal neurologic examinations, radiographic idiopathic scoliosis of 15‐40°, and Risser Stage I‐III were in the patient group. Eighteen healthy adolescent girls with no medical problems, a negative school screening, and no family history of scoliosis were control subjects. Patients and control subjects were matched for age, weight, Tanner stage, sleep duration, and light exposure by multiple linear regression. Nighttime and daytime urine samples were analyzed for melatonin by high‐performance liquid chromatography. Results. Although nighttime melatonin levels were significantly higher than daytime levels in all volunteers (P < 0.00002), there were no significant differences in nighttime (P > 0.63) or daytime (P > 0.78) melatonin levels between patients and control subjects, even after matching by multiple linear regression analysis. A statistical analysis demonstrated that if a melatonin deficiency of 25% or more did exist in patients with scoliosis compared with control subjects, the likelihood that it would have been detected in this study was more than 98%. Conclusion. Although melatonin deficiency may cause scoliosis in the chicken, this study suggests that it is not a mechanism in the pathogenesis of adolescent idiopathic scoliosis in humans.

Angular and torsional deformities of the lower limbs in children

Angular and torsional malalignments of the lower extremity in children are common problems for which parents seek orthopedic advice. Calcaneal valgus of the foot, internal tibial torsion, genu varus, genu valgus, and femoral anteversion are normally observed in infants and usually are corrected with growth. The majority of angular deformities are physiologic; however, it is important to distinguish those requiring further evaluation and treatment. Accurate examination and knowledge of the natural history of rotational and angular deformities allow accurate assessment of the child and sound advice to the parents. There is a trend away from the use of shoe modifications, Denis-Browne-type bars, and twister cables in treating these deformities. Surgical treatment is occasionally required, but the prerequisites in each case should be carefully observed.

Management of scoliosis due to syringomyelia in childhood and adolescence

Summary: To determine the effect of syringomyelia treatment (drainage) on the efficacy of orthotic management and safety of operative management of an associated scoliosis, the records of four boys with scoliosis due to syringomyelia were reviewed. The average follow-up was 8 years. On presentation, all had an abnormal neurologic examination. Each syrinx was treated by laminectomy and insertion of a syringosubarachnoid shunt. After drainage, three of four improved neurologically. Two older patients underwent uneventful posterior spinal fusion with Harrington distraction instrumentation after their drainage procedures. One boy developed kyphosis at the site of his laminectomy. Two young patients, whose curves were initially well controlled with bracing, developed continued curve progression over time. Drainage of the syrinx delayed but did not prevent curve progression in immature patients, but did allow use of distraction instrumentation without complication for operative management of the associated scoliosis.