[The intraarticular osteoid osteoma : A diagnostic and therapeutic challenge]. / Das intraartikuläre Osteoidosteom : Eine diagnostische wie therapeutische Herausforderung.

Intra- and, respectively, periarticular osteoid osteoma are accompanied by sympathical arthritis which itself can irreversibly destroy the cartilage of the joint. In contrast to other locations, intra- and periarticular osteoid osteoma are rare. Reactive and symptomatic accompanying arthritis may lead to irreversible cartilage destruction especially in chronic courses. Therefore early diagnosis and therapy is crucial. We present a case report and discuss these rare tumors by reviewing the literature.

[Combined Salter-Pemberton pelvic osteotomy]. / Kombinierte Salter-Pemberton-Beckenosteotomie.

OBJECTIVE: In simple pelvic osteotomy in childhood the aim is for better lateral roofing, a safe centering of the femoral head and tuning between the volumes of the acetabulum and the femoral head. By the combination of anterior modified Pemberton osteotomy with dorsal osteotomy according to Salter these objectives can be achieved. INDICATIONS: Dysplasia of the acetabulum in patients between 2 years old and adolescence, subluxation and dislocation of the femoral head, also in neurological diseases as cerebral palsy and hypercontainment in Legg-Calvé-Perthes disease. CONTRAINDICATIONS: Critically small pelvic bones in toddlers younger than 18 months, children with a delay in skeletal formation. In patients aged more than 15 years if the symphysis is too taut for an effectual pivoting of the acetabulum and the acetabulum has no potential for future maturing. SURGICAL TECHNIQUE: Surgery is carried out by making a skin incision by the iliac crest ending in the middle of the groin, the cartilaginous iliac apophysis is split and the periosteum is elevated from the medial and lateral wall of the ilium to the inner pelvic ring. A K-wire is used to mark the level and the center of the osteotomy and dorsal to the K-wire a straight osteotomy is performed with a Gigli saw and anteriorly an arc-shaped and tilted cut is made with a chisel. The distal iliac fragment is rotated widely outwards and forwards and a triangular bone graft is removed from the anterior part of the iliac crest. The graft is inserted into the opened up osteotomy, three K-wires are used to fix the desired position of the iliac fragments and the two halves of the iliac apophysis are sutured together. POSTOPERATIVE MANAGEMENT: After the operation uncooperative children receive a scotch cast for 4 weeks. Cooperative children are mobilized after 3 weeks of bed rest. Partial weight-bearing is allowed after 6 weeks and full weight-bearing after 8-10 weeks. RESULTS: A total of 56 combined Salter-Pemberton pelvic osteotomies were performed in 49 patients from 1999 to 2008. The results of these studies demonstrate that this osteotomy is a safe and effective procedure which enables not only sufficient correction in classical dysplasia of the hip joint but also in high grade dislocation of the hip joint caused by cerebral palsy.

Verb generation in children and adolescents with acute cerebellar lesions.

The aim of the present study was to examine verb generation in a larger group of children and adolescents with acute focal lesions of the cerebellum. Nine children and adolescents with cerebellar tumours participated. Subjects were tested a few days after tumour surgery. For comparison, a subgroup was tested also 1 or 2 days before surgery. None of the children had received radiation or chemotherapy at or before the time of testing. Eleven age- and education-matched control subjects participated. Subjects had to generate verbs to blocked presentations of photographs of objects. As control condition, the objects had to be named. Furthermore, dysarthria was quantified by means of a sentence production and syllable repetition task. Detailed analysis of individual 3D-MR images revealed that lesions affected cerebellar hemispheres in all children and adolescents. The right cerebellar hemisphere was affected in four and the left hemisphere in five subjects. In the present study, naming and verb generation accuracy were preserved in the majority of subjects with cerebellar lesions. No significant signs of learning deficits were observed, as reduction of reaction times over blocks was not different compared to controls. There was a trend of children and adolescents with right-hemispheric lesions to perform worse compared to controls. In this group, however, significant signs of dysarthria were present. In sum, no significant signs of disordered verb generation were observed in children and adolescents with acute cerebellar lesions. Findings suggest that the role of the cerebellum in verb generation may be less pronounced than previously suggested. Findings need to be confirmed in a larger group of subjects with acute focal lesions.

Incidence of dysarthria in children with cerebellar tumors: a prospective study.

The present study investigated dysarthric symptoms in children with cerebellar tumors. Ten children with cerebellar tumors and 10 orthopedic control children were tested prior and one week after surgery. Clinical dysarthric symptoms were quantified in spontaneous speech. Syllable durations were analyzed in syllable repetition and sentence production tasks. Localization of the cerebellar lesions were defined after manual transfer from individual 2D-MR images onto 3D images of a spatially normalized healthy brain. Cerebellar children showed few and mild clinical signs of dysarthria. No difference was present in the sentence production task compared to controls. In five cerebellar children, syllables were prolonged in the syllable repetition task after surgery. Syllable duration normalized in an additional four-week session in all but one case. The MR-analysis showed that superior paravermal cerebellar areas likely involved in dysarthria in adults (paravermal lobules HVI, Crus I) were not significantly affected. In children, speech impairments appear to be rare after cerebellar surgery because tumors most commonly affect posterior-inferior and medial parts of the cerebellum while critical cerebellar regions are likely spared. The results suggest a similar localization of speech functions in the cerebellum in children and adults.

Do children with focal cerebellar lesions show deficits in shifting attention?

More recent findings suggest a possible role of the cerebellum in nonmotor functions. Disability of individuals with cerebellar damage in rapidly shifting attention is one frequently used example to support cerebellar involvement in mental skills. The original proposal was based on findings in five children with chronic surgical lesions of the cerebellum and a young adult with a degenerative disorder. The aim of the present study was to repeat Akshoomoff and Courchesne's initial findings in a larger group of children with focal cerebellar lesions. Ten children with cerebellar lesions and 10 age- and sex-matched controls were tested. Neocerebellar areas were affected in all children with cerebellar damage except one based on detailed analysis of MRI scans. Subjects had to perform a focus and a shift attention task. Two visual and two auditory stimuli were presented in a pseudorandom order. An ellipse and a high-pitched tone were presented less frequently than a circle and a low-pitched tone. Rare stimuli were presented at five different time intervals. In the focus tasks, subjects had to react to the same rare stimulus of one of the two modalities. In the shift task, subjects had to switch between the two rare stimuli. Motor deficits based on reaction times were small in cerebellar children compared with controls. The ability of target detection did not significantly differ in the children with cerebellar lesions compared with the control children in both the focus and the shift attention task. In particular, children with cerebellar damage showed no significant impairment in rapid (<2 s) shifts of attention. The present findings indicate that the cerebellum may be less critical in attention related processes than suggested previously.

Resistance to rocuronium in a child with Schwartz-Jampel syndrome type 1 B.

In Schwartz-Jampel syndrome micrognathia and jaw muscle rigidity may result in difficult or impossible tracheal intubation. Since the dose-response relationship to muscle relaxants is unknown in this rare disease we assessed by mechanomyography the neuromuscular response to the rocuronium in a two-year-old child with Schwartz-Jampel syndrome (SJS) Type 1 B. Rocuronium's dose-response curve was markedly shifted (3.5-fold dose) to the right when compared to healthy children and intubation conditions were improved. This resistance to NDMR may result from a lower acetylcholine degradation rate suggested as being the consequence of mutation of the gene encoding perlecan (HSPG2) in SJS. Thus, considerably higher doses of NDMR than usual may be required for facilitation of tracheal intubation in patients with SJS. Since evidence for genetic heterogeneity of SJS exists we also recommend incremental doses of a rapidly acting NDMR with continuous monitoring of neuromuscular function so as to assess the optimum relaxant dose.

[Implant positioning in epiphysiolysis capitis femoris. Sequelae of poor outcome and consequences]. / Zur Implantatpositionierung bei der Epiphysiolysis capitis femoris. Folgen des schlechten Verlaufs und Konsequenzen.

Which consequences can be ascribed to the intraarticular position of devices in the operative treatment of a slipped capital femoral epiphysis? Which steps are to define as a standard of a careful procedure? The clinical and radiological results of five cases of a pin or nail penetration after the operative treatment of a slipped capital femoral epiphysis are described, a possible connection of casualties is investigated. The intraarticular position of devices in most cases goes along with an unfortunate clinical outcome and leads to a higher risk of developing chondrolysis. Because of the radiologic overprojection with the femoral head it is possible to oversee the malposition of the pin. Any operative treatment of a slipped capital femoral epiphysis requires a careful intraoperative X-ray examination combined with documentation. With this procedure the bad results of an intraarticular implant position must be ascribed to the reminding risk of a fateful development.

Serum insulin-like growth factors IGF-I and IGFBP-3 in children with slipped capital femoral epiphysis.

Hormonal imbalance in puberty and biomechanical overload due to obesity have been implied in the still unknown cause of slipped capital femoral epiphysis (SCFE). Local mediators of growth hormone (GH) action, such as insulin-like growth factor I (IGF-I), play a crucial role in the development of the growth plate cartilage. Concentrations of IGF-I and its binding protein 3 (IGFBP-3) were measured in the serum of 19 SCFE children without endocrine disorders. Standing height and body weight were determined. The results were related to the bone age. Concentrations for IGF-I and IGFBP-3 were predominantly within the normal ranges for chronologic age and bone age. The correlation of IGF-I and IGFBP-3 serum levels was high. Standing height and body weight showed a tendency toward the higher percentile ranges. Ten of 19 patients were above the 97th percentile concerning their weight for height. Bone age did not differ significantly from chronologic age. Serum concentrations of IGF-I and IGFBP-3 provided no evidence of a disturbance of the somatotropic axis in SCFE children. Increased body weight associated with normal skeletal maturation implies a mechanical stress factor in the cause of SCFE in these children.

The treatment of Legg-Calvé-Perthes disease. To contain or not to contain.

In order to evaluate a treatment protocol involving a non-containment method and femoral osteotomy when lateral subluxation of the femoral head develops, 39 children with 42 affected hips were examined radiologically an average of 7 years (4-16 10/12) following the onset of Legg-Calvé-Perthes disease. Twenty-six hips were treated conservatively with an ischial-bearing, Thomas-type splint and 16 with a femoral varus derotating osteotomy. Both Catterall and Herring gradings were used. The end result was evaluated according to the Stulberg classification. All Catterall grade I (n = 8) hips had a satisfactory result regardless of age at onset and method of treatment. In Catterall groups II-IV (n = 34), age at onset influenced the treatment outcome. In children affected before the age of 8 years, results of conservative treatment were originally satisfactory but were seen to deteriorate with advancing age. In children over 8 years old (n = 13), the results clearly favour operative containment.

Skeletal immaturity, IGF-I and IGFBP-3 serum concentrations in Legg-Calvé-Perthes disease (skeletal immaturity, IGF-I and IGFBP-3 in LCPD).

BACKGROUND: Skeletal immaturity is a major feature in Legg-Calvé-Perthes disease (LCPD). Evaluation of growth hormone concentration, somatomedin activity, or insulin-like growth factor I (IGF-I) concentration revealed inconsistent results. Recently, IGF-binding protein 3 (IGFBP-3) was found normal in relation to chronological age in LCPD patients. PATIENTS: In this study IGF-I and IGFBP-3 were measured in the serum of 23 children with unilateral LCPD and in 23 sex and age matched controls. METHODS: IGF-I and IGFBP-3 were measured with radioimmunoassays, using an IGF binding site-blocked assay for IGF-I. The results were related to the chronological age in all and to the bone age in 19 of the patients. RESULTS: Bone age was retarded in 16 of 19 patients with a delay of one year or more in twelve children (mean 14.75, range 2-35 months). Chronological age and bone age related IGF-I and IGFBP-3 serum concentrations were predominantly within the normal ranges and did not differ significantly from the matched controls. IGF-I and IGFBP-3 serum levels showed a high correlation, which was similar in LCPD (r = 0.7; p < 0.0001) and in the control group (r = 0.8; p < 0.0001). CONCLUSIONS: Our data confirm that most children with LCPD are skeletally immature. However, IGF-I measured with IGF-II-blocked IGFBP binding sites, and IGFBP-3 serum concentrations analysed with respect to bone age show no evidence for a disturbance of the hypothalamo-pituitary-somatomedin axis in these children.

[Operative therapy in tumor involvement of the spine]. / Operative Therapie bei tumorösem Befall der Wirbelsäule.

We report on 56 patients, who underwent operative treatment of tumorous lesions of the spine. We indicated dorsal instrumentation in 14 cases, ventral tumorectomy in 11 cases and a combined dorsal and ventral instrumentation in 31 cases. The median survival time was 14.8 months. Reduction of pain and the improvement of the preexisting neurological symptoms were the most important postoperative factors. According to our follow-up study, the combined dorsal and ventral instrumentation seems to be the best method for an operative treatment of tumorous spine lesions.