Method of analysing the performance of self-paced and engine induced cycling in children with cerebral palsy.

PURPOSE: To develop a method of quantifying the features of cycling in children with CP by comparing them to typically developed children, and to demonstrate the applications of this tool for evaluating treatment effects in children with CP. METHODS: Twenty-seven typically developed children and 51 with CP, classified by their gross motor function levels, were studied. Angular velocities were measured during self-paced active cycling and during passive cycling imposed by an electrically powered stationary cycle. Angular velocities were compared with the gross motor function levels, Modified Ashworth Scale and the Adductor Tone Rating. RESULTS: Significant differences between children with CP and those typically developed demonstrated in passive mode that the cycling task is sensitive to differences in resistance offered by the subjects. Active and passive cycling velocities differ significantly between groups classified by their functional levels (p < 0.01). Children with CP in the mild group showed no differences from typically developed children. The correlations between passive cycling and clinical tests were significant and higher at higher speeds (r = 0.62). Correlations with anthropometric measurements for the typically developed group associated the cycling task with growth and development, and for children with CP with motor control adjustments and impairments. CONCLUSIONS: Measuring of these two cycling modes could be applicable in assessing lower extremity function in children with CP and changes following interventions.

Sonographic healing stages of Achilles tendon after tenomuscular lengthening in children with cerebral palsy.

Long-term follow-up of a tenotomized Achilles tendon reveals healing with formation of a tendonlike structure between the cut tendon ends. A prospective study to show sonographic healing stages was designed. Twelve patients (21 Achilles tendons) with the diagnosis of cerebral palsy, spastic diplegia, and shortening of their Achilles tendons underwent a tenomuscular release. The healing process was documented by monthly ultrasound studies. Overall, 102 examinations were done, with a mean follow-up of 5.3 months. The authors found six distinct healing stages, with no significant change occurring in the length of the primary gap at the tenotomy site throughout healing. Based on these results, the authors conclude that sonographic healing stages of the cut Achilles tendon can be defined and staged. They also suggest that transverse tenotomy at the tenomuscular junction is a safe method, with no risk of overlengthening.

Musculoskeletal deformities in Behr syndrome.

SUMMARY: Seventeen children with Behr syndrome were investigated, focusing on the musculoskeletal deformities and long-term outcome. Behr syndrome is characterized by optic atrophy beginning in early childhood associated with ataxia, spasticity, mental retardation, and posterior column sensory loss. The ataxia, spasticity, and muscle contractures, mainly of the hip adductors, hamstrings, and soleus, are progressive and become more prominent in the second decade. In 70% of the patients, contractures developed in the lower limbs, requiring surgery mainly for the Achilles tendon, hamstrings, and adductor longus. At last follow-up at an average age of 21.7 years (range, 8-31 years), 13 of the patients are housebound walkers, 2 are nonfunctional walkers, and 2 are nonwalkers.

Hereditary insensitivity to pain with anhidrosis.

Hereditary sensory neuropathy type IV is an autosomal-recessive disorder characterized by congenital insensitivity to pain and anhidrosis and resulting in recurrent hyperpyrexia, self-mutilation, recurrent infections, chronic osteomyelitis, bone and joint deformities, and limb amputations. Described is a child with signs as well as skin and nerve biopsy results compatible with this disease, emphasizing the importance of early diagnosis and appropriate medical and educational care to prevent complications.

Spondyloepiphyseal dysplasia associated with progressive arthropathy. An unusual disorder mimicking juvenile rheumatoid arthritis.

A family is presented with three affected siblings suffering from a rare form of spondyloepiphyseal dysplasia with progressive arthropathy affecting most joints of the body except for the craniofacial skeleton. This syndrome, probably of autosomal recessive inheritance, clinically presents diffuse and chronic joint involvement and should be differentiated from juvenile rheumatoid arthritis and other rheumatic disorders.

Computerized tomography in the diagnosis and treatment of osteoid osteoma.

Thirteen cases of osteoid osteoma demonstrated with computerized tomography are reported. Twelve patients underwent definitive surgery and were relieved of their symptoms. The histological examination confirmed the preoperative diagnosis in 11 cases. Six lesions were located in the proximal femur, three in the neck of the talus, two in the proximal tibia, and one each in the proximal humerus and the calcaneus. Radionuclide technetium 99m MDP scans were performed in 11 cases and showed an increased uptake in the vicinity of the lesions. Computerized tomography showed the precise location of the nidus, thus helping in the preoperative planning of the surgical approach and enabling us to perform a precise and economic resection.

Multiple exostoses-mental retardation syndrome. A case report and review of the literature.

A girl seven years ten months of age with multiple exostoses-mental retardation (MEMR) syndrome was treated by bilateral supracondylar osteotomies at the age of six years 11 months for correction of severe genu valgum. The case is the 14th to be described in the English-language literature and seems to be the first on record in which the deformity was corrected by surgery. Typical findings in this syndrome include unusual facial features with bulbous nose, sparse scalp hair, large ears, microcephaly, mental retardation, cone-shaped epiphyses of the digital phalanges, and multiple exostoses. Each of these features may also appear in other constitutional and genetic disorders, and only their combination points to a definite diagnosis of MEMR syndrome. Other features, e.g., joint laxity and loose skin, are transient and may cause some confusion in diagnosis, sometimes leading to a mistaken diagnosis of cerebral palsy or Ehlers-Danlos syndrome. Thus, care must be taken in consecutive examinations to seek and identify each of the above mentioned typical features of the disorder.

Local penetration of gentamicin into the femoral cortex of rats.

To investigate the extent of local penetration of antibiotic into the bone, a single dose of 10 mg/kg gentamicin sulphate was injected over the midshaft of the left femur in 12 young and 14 old rats. In some of the rats the injected gentamicin was dissolved in saline while in the others it was dissolved in dimethylsulphoxide (DMSO). Up to 120 minutes after the injection gentamicin concentrations were measured by the fluoroimmunoassay method in the serum, cortex and spongiosa of both femurs. In young rats gentamicin was shown to have penetrated directly into the bone and its presence was limited to the femoral cortex. In the old rats, on the other hand hematogenic was found to be the antibiotic spread in the femurs. No significant difference was observed between DMSO and saline as solvents in facilitating gentamicin penetration into the bone or capillaries in either age group.

Anterior cruciate ligament insufficiency syndrome.

Corrective surgery for anterior cruciate ligament (ACL) lesions was performed in 64 patients. Approximately two-thirds of the patients who presented with anteromedial instability were approached through the medial aspect of the knee. In all cases the instability was improved but not entirely eliminated. This was not in itself a disabling problem, as 44% of these patients had good subjective results. However, pivot-shift tests at follow-up examination clearly indicated the presence of anterolateral instability in most, if not all, of these patients. This did not necessarily preclude a self-evaluation of "good"; conversely, a self-evaluation of "fair" (33% of patients) was always related to a positive pivot-shift phenomenon. The authors' current surgical policy is, therefore, to regard the two types of instability as related aspects of a single ACL insufficiency syndrome, and to correct both the anterolateral instability (by the MacIntosh procedure) and the anteromedial instability (by suturing the posterior medial capsule to the semimembranosus tendon together with pes transfer). Poor subjective results (22% of patients) were always associated with the patellofemoral pain syndrome, which could be traced to rotational instability leading to patellar malalignment.

Radiating leg pain and positive straight leg raising in spondylolysis in children.

Three children presented with low back pain radiating to the leg and with spasm of the hamstring and paravertebral muscles. Since the pain could not be ascribed to trauma, it was necessary to exclude the presence of infection or tumors. All the signs--localization of the pain, tenderness on one side of the back, X-ray film findings of unilateral or bilateral spondylolysis, and localized positive bone scan--pointed to spondylolysis as the cause of pain. All three children exhibited symptoms resembling those found in the facet syndrome described by Mooney and Robertson.