Caffey's disease: an unusual cause for concern.

BACKGROUND: Caffey's disease or infantile cortical hyperostosis is a rare cause of irritability, bone pain, soft tissue swelling and fever in the infant. AIMS: To review the presentation and diagnosis of an 8-week old infant with focal tenderness of the tibia. METHODS: The symptoms, signs, laboratory work-up and radiology are reviewed. RESULTS: The child presented with focal bony tenderness and pyrexia. Laboratory work-up was inconclusive. The diagnosis of Caffey's disease was made following a skeletal survey to outrule non-accidental injury, which showed similar changes of cortical thickening in the mandible as well as the affected tibia. CONCLUSIONS: With our increasing immigrant population, an increasing number of differential diagnoses must be considered when evaluating patients presenting to our emergency rooms. Caffey's disease is an unusual cause of bone pain in the infant. Symptoms and signs are subtle and the diagnosis is generally made with plain X-rays.

Adolescent idiopathic scoliosis: metric analysis of the deformity.

In order to explore the concept that scoliosis is fundamentally a loss of left-right symmetry. surface topography was used to measure asymmetry in three dimensions at three levels on the back surface. Statistical analysis of prospectively collected topographic, radiographic and clinical data, in girls with adolescent idiopathic scoliosis, was carried out and comparisons were made with theoretically perfect symmetry (test value of zero). All scoliosis showed statistically significant differences in coronal dimensions, index points on the convex side of the scoliosis being further from the mid-line than those on the concave side. Primary thoracic scoliosis differed from thoracolumbar and lumbar in that they showed directional asymmetry at all levels and in all directions, the side of the scoliosis convexity being broader, taller and thicker. This asymmetry is not due to posture, spinal balance or trunk rotation, as left and right sides are being compared independently of their orientation in space. The asymmetry is of size in three dimensions and size is determined by growth. Growth is a three dimensional process, but does not necessarily occur equally in all three. Differential growth is both directional and regional, particularly during the pubertal growth spurt, when proportions change substantially, and is controlled by many genes, as well as by hormones and signalling molecules. The implication is that scoliotic deformity is the result of asymmetric growth, not confined to the vertebrae, but affecting the entire trunk. This is a developmental, rather than pathological, phenomenon. It makes questions of aetiology redundant and natural history logical.

Handedness and spinal deformity.

Biological lateralisation is clearly manifest in scoliosis, yet its relevance is unclear. Goldberg et al. (Spine. 15(2):61-64. 1990) found an association between curve pattern and hand-preference in a screened population, but no increase in sinistrality. Milenkovic et al, (European Journal of Epidemiology, 19:969-972,2004) concluded left-handedness was a risk factor in a screened group. The database was reassessed to determine whether clinically significant scoliosis was associated with sinistrality or differed from the population norm of 10%. Patients attending the scoliosis clinic were asked their preferred writing hand. 1,636 patients were identified with complete data. Overall, left handedness occurred in 11.5%, greater than the general population (p=0.04) Left hand preference was found to be increased in boys with infantile idiopathic scoliosis and in girls with infantile, juvenile, congenital and syndromic scoliosis, but was reduced in girls with adolescent idiopathic scoliosis. Scoliosis lateralisation was random in infantile and congenital scoliosis, while left curve patterns were decreased in girls with juvenile idiopathic scoliosis and increased in boys with syndromic scoliosis. Curve pattern and handedness correlated in asymmetry in boys and girls and in girls with radiologically confirmed adolescent idiopathic scoliosis, but not in any other type. This study cannot confirm findings of left-handedness as a risk factor for spinal deformity. Its incidence is reduced in girls with adolescent idiopathic scoliosis, and the increased sinistrality in infantile scoliosis is not a new finding (Rauterberg & Tonnis Ger. Z.Orthop. 109(14):676-689. 1971). Lateralisation is undoubtedly a factor in scoliosis, but does not have a simple causal relationship, probably deriving from the underlying scoliotic process, rather than contributing to it.

Surface topography and vectors: a new measure for the three dimensional quantification of scoliotic deformity.

The monitoring of spinal deformity uses many techniques: clinical history and physical examination for patient status, radiography for precise spinal delineation and Cobb angle, topography to quantify cosmesis and to approximate the Cobb angle. Experience with a system based on Raster photography has shown that adequate correlation with the Cobb angle is achieved, but that the relationship between spinal curvature and cosmetic effect is not simple. A measure was developed to quantify the asymmetry of the back, making it available to statistical analysis, without expressing it in terms of the Cobb angle or referring to trunk balance or rotation. The calculation expresses symmetry about the median saggittal plane (first thoracic vertebra to natal cleft), reflecting the right half onto the left and measuring the three-dimensional displacement between corresponding fixed points on the trunk. Tolerance limits were calculated and correlation with Cobb angles using routine scans was analysed. There were statistically significant correlations between the Cobb angle and all vectors except the middle antero-posterior. All vectors correlated with each other, except again for the middle Z or anteroposterior which correlated only with the middle and lowermost sets. Applied to natural history and to surgical outcome, this new parameter provides a different quantification of back shape which can be used both for patient assessment and monitoring, for the evaluation of the cosmetic (as opposed to the radiological) effect of treatment, and for aetiology and natural history studies.

Minor head injuries in children.

Traumatic head injury continues to be a major problem facing the pediatric specialists despite efforts to reduce its incidence. Aims of our study were assessment of the incidence of hospital admissions of children with minor head injuries, their treatment and outcome. In this prospective study we included 101 patient with head injury. We assessed the hospital admission criteria of children with minor head injuries, their length of stay, symptoms and neurological status, in-hospital imaging and consultations to other medical specialities, together with the outcome of patients on their discharge from the hospital. Of 101 patient 54 (53.46%) were male and 47 (46.53%) female. Average age was 3 years with age range from 5/52 to 12 years. Majority of patients (46.53%) were less than 1 year old. Fall was the most common mechanism of injury (92.07% of all patients). Injuries were witnessed in 51 case (50.49%), unwitnessed in 19 cases (18.81%) and unknown in 31 cases (30.69%). 95 patients did not have associated extracranial injuries, while 5 patients had associated lacerations and one had associated extracranial fracture. Length of stay was from 1 day in 80.19% of all cases to more than 2 days in 6.93%. GCS was recorded on admission and discharge and majority of patients were discharged with GCS of 15. 93% of admissions had no neurological deficit. 79% had 1 or more symptoms including vomiting, sleepiness, LOC and headaches. Over half of patients (55%) had no imaging done. 45 patients (45%) had skull x-ray. CT was performed in 7 (15%) patients and 7 fractures were found on x-ray and CT. 3 patients were admitted to ICU. All injuries were closed and all received conservative treatment. In less than 1 year old group of patients 95% of them were also seen by medical team and social worker. The outcome was good for all patients. We conclude that majority of patients with minor head injury could be supervised and observed at home by a competent care giver. Admitted patients need radiological evaluation, preferably a CT scan.

Osteoid osteoma: painful enlargement of the second toe.

Osteoid osteomas involving the phalanges of the toes are a rare occurrence. We report a case of an osteoid osteoma of the distal phalanx of the second toe which was treated successfully with surgical excision. Although soft-tissue swelling with a typical clinical history is suggestive of the diagnosis, differentiation from subacute infection is subtle and may ultimately depend on tissue analysis.

Kyphectomy in children with myelomeningocele: a long-term outcome study.

Twenty-four children with myelomeningocele and a kyphotic deformity treated by surgical correction between 1980 and 1994 were reviewed. Different techniques of instrumentation and spinal fusion were used. The mean age at surgery was 9.5 years. The mean kyphotic angle was 121 degrees preoperatively, 50 degrees postoperatively, and 57 degrees at final follow-up. The postoperative complication rate was high. Delayed wound healing and late skin breakdown with exposure of instrumentation were common problems. Further surgery to remove protruding hardware was necessary in 18 patients. Long posterior instrumentation with fixation to the pelvis had significantly better stability on follow-up than other methods. Despite the prolonged postoperative morbidity in the majority of the study group, the long-term clinical and radiologic outcome at a mean follow-up of 10 years was excellent.

Respiratory function and cosmesis at maturity in infantile-onset scoliosis.

STUDY DESIGN: Retrospective review of patient records, clinical and radiographic, and patient recall for full pulmonary function studies and surface topography. OBJECTIVES: Assessment of outcome of treatment policy after age 15 during the previous 30 years to establish the efficacy of management protocols in a group that is too small and too varied for more formal assessment. BACKGROUND DATA: Spinal deformity presenting during infancy or early childhood poses a clinical problem caused by small numbers, long growth period, variable presentation and treatment methods, and, finally, the length of time that must pass before meaningful outcome results can be assessed. The aims of treatment are to preserve respiratory function and cosmetic appearance. MATERIALS AND METHODS: The records of patients with infantile onset, nonsyndromic, and noncongenital scoliosis were reviewed. Thirty two were at least age 15 years at the time of review and 21 of these agreed to attend for full pulmonary function testing (spirometry, lung volumes, gas diffusion) and surface topography, whereas two more had recent spirometry results available in their record. Treatment had been serial casting with Risser jacket, bracing, or surgery. RESULTS: Those whose scoliosis resolved or was stabilized by nonoperative means (N = 6) at an acceptable Cobb angle had normal cosmesis and pulmonary function (mean FEV1 = 98.7%, mean FVC = 96.6%). Those who were managed by casting or bracing and underwent surgery after age 10 (N = 6, mean age at surgery 12.9 y) had variable cosmesis and acceptable pulmonary function (mean FEV1 = 79%, mean FVC = 68.3%). Those whose deformity necessitated early surgery (N = 11, mean age at surgery 4.1 y) had recurrence of deformity and diminished respiratory function (mean FEV1= 41%, range 14%-72%, mean FVC = 40.8%, range 12%-67%). CONCLUSIONS: Although these are small numbers and treatment methods have changed since the beginning of the series, the results indicate that this condition is not simple to treat and for some children still has the risk for serious deformity and respiratory compromise. There is, as yet, no evidence that early surgical intervention in this group of patients with infantile scoliosis has altered their prognosis in any meaningful way.

The rib hump after surgery for early onset spinal deformity.

Spinal deformity presenting early in childhood has a poor prognosis, in that progression is probable and severe respiratory compromise is a real possibility. Treatment is difficult, since these patients frequently do not respond to bracing, and surgery is sometimes performed in childhood in an attempt to control relentless progression. This carries the risk of continued deformation during subsequent growth, and the surgical procedures have been adapted in an attempt to minimise this. 25 children undergoing spinal fusion for progressive and severe deformity have sequential topographic scans which show that, despite measures to control the rib hump, progression after surgery is the rule.

The relationship between minor asymmetry and early idiopathic scoliosis.

School scoliosis screening programmes have consistently produced large numbers of referrals with minimal trunk asymmetry which then must be assessed, evaluated and perhaps investigated and monitored over a period of time before it is felt safe to discharge them as 'non-progressive.' Beyond measures to reduce their number, they have received less attention than the more interesting cases requiring treatment or surgery, which they outnumber to a significant extent. Examination of the figures from a historical database shows that the patients have no greater probability of developing significant scoliosis than their 'normal' peers . So they should not be classed as scoliosis but they are still relevant to the broader picture of aetiology.

Surface topography and the several components of scoliotic deformity.

Using previously established values for the smallest detectable difference in topographic parameters, the effectiveness of the Quantec system for monitoring progress in scoliosis was investigated. It was found that, while a significant change in Cobb angle was always accompanied by a meaningful change in at least one topographic parameter, the pattern of shape change was highly individual. It is concluded that deformity in scoliosis is not determined exclusively by the spinal curve.

Adolescent idiopathic scoliosis: is the search for aetiology constrained by the orthosis?

While the aetiology of adolescent idiopathic scoliosis is unknown, it is widely believed that the deformity can be controlled by mechanical forces applied by a corset and acting, via skin and soft tissues, to exert pressure on vertebral growth-plates (Hueter-Volkmann Law). The reported efficacy of bracing in reducing the incidence of small degrees of progression reinforces this concept. The supposed mode of action conditioned the model of pathomechanism and precludes other hypotheses. Since the critical Cobb angle changes are usually less than the probable measurement error, another criterion (treatment outcome) is also relevant. At our centre, bracing for adolescent idiopathic scoliosis has not been practised for 9 years. Out of the 153 untreated, unselected patients presenting to the general scoliosis clinic in that time and who were at least 15 years old at the last review, 43 (27.6%) of them have undergone surgery. This does not differ statistically from 22.45% (229 of 1,020) braced patients reported by Lonstein and Winter in 1994. If bracing does not significantly improve prognosis, its efficacy cannot be accepted and the hypothesis of aetiology which it underpins has no greater standing than any other.

Adolescent idiopathic scoliosis: natural history and prognosis.

Retrospective review of the scoliosis database showed adolescent idiopathic scoliosis to be the most common but least significant variety of spinal deformity. Data from 112 girls diagnosed on repeat screening showed the importance of menarche as a date in the natural history. In the whole database, there were 514 aged at least 15 years at last review, 45 boys and 469 girls. Braces were prescribed for a total of 38, mostly during the early part of the period and gradually abandoned without an increase in surgical rate. Progression depended on the age and maturity of the patient as well as the initial Cobb angle. Surgery was recommended for 27% overall. A sub-group with more complete data diagnosed after bracing had been discontinued confirmed the findings.

The natural history of early onset scoliosis.

A retrospective analysis of the natural history and treatment outcome of scoliosis, both idiopathic and syndromic, presenting before age 10 years was undertaken. The pattern was generally one of relentless progression, with little discernible benefit from orthotic treatment and surgical correction as the outcome for the majority. This result makes imperative a properly constructed study on the evolution and long-term outcome of this less common but more significant type of spinal deformity.

Growth patterns in patients with unoperated congenital vertebral anomaly.

Retrospective analysis of height and weight data recorded during routine clinic visits of children with congenital vertebral anomaly were related to decimal age and compared with national centiles. Individuals were dropped from the study at surgery. Growth followed a normal trajectory until puberty, although girls tended to be smaller than average. At puberty, they lagged behind their peers and at maturity were shorter than average. This does not appear to be a hormonal problem, and suggests a fundamental failure of growth.

Bilateral wrist dislocation in trisomy 21: a case report.

Ligamentous laxity is common in Trisomy 21 (Down's syndrome). The literature has numerous reports of atlanto-axial instability, patellar instability, carpal instability and other manifestations of joint instability. To date, no report has been published in the English literature describing non-traumatic unilateral or bilateral wrist dislocation either in trisomy 21 or in any other condition.

Neck-shaft angle remodelling after derotation varus osteotomy for severe Perthes disease.

Sixteen patients with a severe form of Perthes disease who underwent containment derotation varus osteotomy (DVO) of the proximal femur were studied retrospectively for the neck-shaft angle (NSA) remodelling. Analysis of results after a mean follow-up of 7 years (minimum of 5 years and maximum of 8.7 years) showed mean preoperative, immediate postoperative and the latest follow-up neck-shaft angles (NSA) as 135 degrees, 110 degrees (none had varus less than 90 degrees) and 125 degrees, respectively. A statistically significant relationship between NSA remodelling and the immediate post-op NSA (P = 0.0035) was established. Patients with smaller postoperative NSA showed better remodelling compared with those who had higher degrees of NSA immediately after the surgery. No significant relationship was found between the degree of NSA remodelling and the patient's age at diagnosis, age at surgery, severity of Perthes or the Stulberg grading at maturity. The authors conclude that up to 90 degrees or more varization after DVO for Perthes disease can remodel with time, and the remodelling process is independent of the patient's age at diagnosis, age at surgery, or severity of Perthes or Stulberg grading at maturity.

Recurrent congenital haemangiopericytoma in a child.

A five-day-old boy was referred with a soft-tissue mass in his right upper arm. Plain radiographs and ultrasound demonstrated a lesion extending from the axilla to the elbow on the posterolateral aspect of the humerus. Open biopsy confirmed the diagnosis of congenital haemangiopericytoma. After MRI and selective angiography, excision biopsy was carried out, but no adjuvant therapy was administered. At further examination, four years and ten months later, he was noted to have three small nodules at the site of the original tumour. Excision biopsy confirmed this to be a local recurrence, although the lesion was less cellular with no appreciable mitotic activity. Congenital haemangiopericytoma is a rare cause of a soft-tissue mass in children. Most tumours are benign, and recurrence is uncommon. The treatment is controversial, but most centres recommend the use of adjuvant chemotherapy, combined with complete excision. We recommend treatment with doxorubicin. Orthopaedic surgeons should be familiar with this tumour since 30% to 50% of cases occur in the limbs.

Surface topography, Cobb angles, and cosmetic change in scoliosis.

STUDY DESIGN: Preliminary analysis of the clinical value of surface topography in a spinal deformity clinic. OBJECTIVES: The Cobb angle is the gold standard for the monitoring of scoliosis. This study was designed to determine whether surface topography would reflect Cobb angle status with sufficient reliability to permit its safe use as an alternative means of documentation in some circumstances. SUMMARY OF BACKGROUND DATA: Surface topography offers the possibility of describing spinal deformity more fully than radiographic measures alone. To be useful, it must ignore changes due to varying posture and reliably detect differences that are clinically significant, while broadening the ability to assess deformity. METHODS: Surface topography using Quantec () was obtained routinely in all patients attending a spinal deformity unit. Intrasubject variation was reduced by taking the mean for each parameter of four repositioned scans, which gives a smallest detectable change on all measures of approximately 10 U. Fifty-nine patients with two sets of radiographs and topography scans were studied to determine the ability of the different measurements to detect significant change. RESULTS: There was a significant correlation between Cobb angle and Quantec spinal angle. A significant change in Cobb angle could be identified by associated change in at least one topographic measure in a significant proportion of cases. CONCLUSIONS: It is unlikely that topography will supplant radiography for the ascertainment of Cobb angles, because the error margins of both are wide, and the two are not measuring the same aspect of the deformity. The Quantec system is useful in patient monitoring as an alternative to radiography, without diminishing the standard of care.

Adolescent idiopathic scoliosis: the effect of brace treatment on the incidence of surgery.

STUDY DESIGN: Retrospective analysis of outcome in terms of incidence of surgery for adolescent idiopathic scoliosis during a period when bracing was not practiced. OBJECTIVES: To determine whether centers with an active bracing policy have lower numbers undergoing surgery for adolescent idiopathic scoliosis than a center where nonintervention is the practice. BACKGROUND DATA: Two major recent publications have claimed that bracing significantly improves the outcome in adolescent idiopathic scoliosis. However, one had no control subjects and the other did not examine the final status of the subjects under review. While statistically significant differences in progression have been observed, what will convince patients to submit to an onerous treatment is the conviction that it will make a substantial difference, such as the avoidance of surgery. METHODS: Since 1991, bracing has not been recommended for children with adolescent idiopathic scoliosis at this center. The scoliosis database was searched for patients with adolescent idiopathic scoliosis who were at least 15 years of age at last review and who had adequate documentation of curve parameters. The incidence of surgery was compared with that of published data from other centers. RESULTS: A total of 153 children, 11 boys and 142 girls, fitted the criteria. Forty-three of these (28.1%) have undergone surgery. This was not statistically different from the surgery rate reported from an active bracing center. CONCLUSIONS: If bracing does not reduce the proportion of children with adolescent idiopathic scoliosis who require surgery for cosmetic improvement of their deformity, it cannot be said to provide a meaningful advantage to the patient or the community. Recent studies notwithstanding, the question of the efficacy of orthoses in idiopathic scoliosis remains unresolved.