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At least 433 records · Page 24Linked to original sources

Grass-skirt burns in Papua New Guinea.

A retrospective survey of burn cases admitted to Alotau Hospital, Papua New Guinea, over a four-year period showed that 48% were due to grass-skirt burns. Most of these occurred in young girls and usually caused full-thickness burns of the buttocks and thigh. The commonest long-term complications were contractures of the hips and knees. The next most common cause of burns was a fall into a fire during an epileptic fit (24%). None of the patients were on anticonvulsants. The population needs to be informed of the dangers of leaving children near open fires, of the value of bundling up children whose grass-skirts catch fire to smother the flames, of the importance of anticonvulsants to chronic epileptics, and of the advantages of seeking medical treatment when burns occur.

Accidents, Home↗

Dorsal longitudinal myelotomy.

An evaluation of dorsal longitudinal myelotomy in the management of spasticity of the lower limbs has been done in 17 patients. The follow-up has varied from 1 month to over 2 years. Most patients achieved satisfactory relief from spasticity. Eight patients have died after the operation in chronic care hospitals. In all except one, the spasticity had been relieved. Eight out of the nine living patients have benefited from myelotomy. Recurrence of spasticity following the first operation occurred in four patients, two of whom improved after a repeat myelotomy. In the presence of fixed contractures of the hip and knee joints, a satisfactory clinical result may not be obtained after the myelotomy.

Adult↗

Peroneal somatosensory evoked potentials in the "pure" form of hereditary spastic paraplegia.

Two patients suffering from the stationary form of hereditary spastic paraplegia, father and son, are described. While in the father the disease - probably because of congenital clubfeet - led to flexion contractures of the hip- and knee-joints, the son in contrary showed extreme genua recurvata. In spite of these contradictionary findings, neurological examination of both patients led to the diagnosis of stationary form of hereditary spastic paraplegia. Both patients had normal latencies of their somatosensory evoked peroneal cortical potentials (Peron-SEP). It is known that the progredient form of the disease leads to alterations of the Peron-SEP. So it is the opinion of the authors that this electrodiagnostic aspect might allow to distinguish between the "pure" and the progredient form of the disease in less clear cases, if these findings can be confirmed in other sibships with the "pure" form of hereditary spastic paraplegia.

Adult↗

Talectomy in the treatment of the myelomeningocele patient.

This paper is an analysis of a series of 11 pediatric myelomeningocele patients in whom 20 talectomies were performed for correction of rigid equinovarus of paralytic vertical talus deformities. In most of the patients talectomy achieved a satisfactory correction of the foot deformity with maintenance of the correction for up to 5 years of follow-up in this study. Failure of the procedure related to two factors: (1) severe external rotational contracture of the hips in patients with high spinal lesions; (2) incomplete removal of the talus because of extensive scarring and fibrosis due to prior infection or surgery. Correction of the external rotational deformity of the hip or a derotational supramalleolar osteotomy of the tibia can prevent the first cause of failure. An intra-operative X-ray of the foot can prevent the latter.

Arthrogryposis↗

Traumatic rupture of iliacus muscle with femoral nerve paralysis.

Two cases of traumatic rupture of the iliacus muscle are reported. The clinical picture of this condition is characterized by pain in the groin, a tender mass in the iliac fossa, flexion contracture of the hip, and a femoral nerve palsy. Presence of a bleeding tendency should be investigated. Operative treatment is recommended for large hematomas.

Adolescent↗

Scoliosis in arthrogryposis multiplex congenita.

Fourteen of 50 patients studied with arthrogryposis also had scoliosis, an incidence of 28%. Eight patients developed a severe curve associated with functional disability and poor health. Three patients were unable to walk independently, and 2 died in childhood. Seven patients had congenital scoliosis; 4 had long "C" neuromuscular curves, and 3 had scoliosis associated with pelvic obliquity caused by unilateral hip dislocation or contracture. The natural history of congenital and neuromuscular curves was progression to an extreme and rigid scoliosis. The authors urge others to look for scoliosis in their patients with arthrogryposis and to anticipate its progression.

Arthrogryposis↗

Musculoskeletal consequences of near-drowning in children.

Nearly drowned children sustaining cardiac or respiratory arrest or altered neurologic status developed anoxic encephalopathy (30%), died in the ensuing days (36%), or were discharged neurologically normal (34%). In encephalopathic children, spasticity was more malignant than in children with other causes of spasticity. The most common musculoskeletal problem was contracture, especially equinus, hip adductor, hamstring, and quadriceps. Hip subluxation or dislocation occurred in 34% as early as 1 month after injury. Scoliosis developed in 18%, with some requiring bracing and surgery. Thirty-one percent ambulated, and the rest were quadriplegic. Independent sitting or better function by discharge was a predictor of ambulation potential. Of the quadriplegics, 66% were dependent sitters.

Child↗

Freeman-Sheldon syndrome (whistling face syndrome) and cranio-vertebral junction malformation producing dysphagia and weight loss.

The whistling face syndrome has been reported, with complications of the respiratory system and feeding problems due to microstomia, since 1938. Dysphagia, however, has not previously been reported as a complication of whistling face syndrome. We report a 13-year-old with the whistling face syndrome and dysphagia of late onset who has the typical whistling face, scoliosis and multiple joint contractures. Dysphagia occurred as a result of compression at the cervicomedullary junction by a cranio-vertebral junction malformation and impingement by the tip of the odontoid process. This child had undergone multiple surgical procedures for cleft palate, clubfeet, contractures of the hip and knee and scoliosis. Halo traction and posterior occipital-cervical decompression and fusion successfully treated the dysphagia.

Adolescent↗

Pseudomalignant heterotopic ossification ("myositis ossificans"). Recurrence after excision with subsequent resorption.

An eight-year-old boy presented with massive pseudomalignant heterotopic ossification around the upper femur. The mass was completely excised because of severe pain, systemic illness and a flexion contracture at the hip. Symptomatic improvement was swift, but two weeks later the mass had recurred and was even more extensive. During the subsequent 18 months of conservative management he has been free of pain and there has been progressive resorption and remodelling of the heterotopic bone. There is now no limitation of physical activity and movement at the hip is full.

Bone Resorption↗

Paralytic drop foot and gluteal fibrosis after intramuscular injections.

Eight children with paralytic drop foot after intramuscular injections later developed gluteal fibrosis. Sciatic palsy, presenting as equinovarus or equinus deformity, was diagnosed on average 3.8 months after the intragluteal injections, but gluteal fibrosis was not diagnosed until 5.1 years after the injections. In three patients the equinovarus recurred after surgical correction due to persistent muscle imbalance and the effect of the external rotation contracture of the hip.

Buttocks↗

Pediatric reference data for dual X-ray absorptiometric measures of normal bone density in the distal femur.

OBJECTIVE: Many children at risk for osteoporosis have substantial hip and knee contractures that prevent assessment of bone mineral density in the "usual" region, the proximal femur. As an alternative, bone density may be measured in the distal femur projected in the lateral plane. The purpose of this study was to provide normative reference data useful for interpretation of bone density measures in the distal femur of children and adolescents. SUBJECTS AND METHODS: The study was a cross-sectional, single-observational assessment of 256 healthy children and adolescents between the ages of 3 years and 18 years 6 months (mean, 10 years 5 months). Bone mineral density was measured in the nondominant proximal femur, lumbar spine, and both distal femurs using dual X-ray absorptiometry. RESULTS: We found that bone mineral density increases with age in the cortical, cancellous, and mixed regions of the distal femur, similar to the findings with other regional analyses of bone density. Bone density in the distal femur correlates very highly with bone density in the proximal femur and slightly less well with bone density in the lumbar spine. CONCLUSION: In pediatric patients who have deformities, have experienced trauma, or have undergone surgical procedures that prevent reliable measures of bone density in the proximal femur, bone mineral density may be measured in the distal femur and interpreted relative to the bone mineral density findings in healthy age- and sex-matched controls.

Absorptiometry, Photon↗

Comparison of clinical characteristics between congenital fiber type disproportion myopathy and congenital myopathy with type 1 fiber predominance.

Congenital myopathies are clinical and genetic heterogeneous disorders characterized by skeletal muscle weakness and specific structural changes in muscle fiber. Congenital myopathy with fiber type disproportion (CFTD) is an established disorder of congenital myopathy. CFTD is characterized by non-progressive childhood neuromuscular disorders with a relatively good prognosis and type 1 fiber predominance and smallness. Congenital myopathy with type 1 fiber predominance (CMT1P) is also a distinct entity of congenital myopathy characterized by non-progressive childhood neuromuscular disorders and type 1 fiber predominance without smallness. Little is known about CMT1P. Clinical characteristics, including dysmorphic features such as hip dislocation, kyphoscoliosis, contracture, and high arch palate, were analyzed along with laboratory and muscle pathologies in six patients with CMT1P and three patients with CFTD. The clinical manifestations of CFTD and CMT1P were similar. However, the frequency of dysmorphic features is less in CMT1P than in CFTD. Long term observational studies of CMT1P are needed to determine if it will change to another form of congenital myopathy or if CMT1P is a distinct clinical entity.

Adult↗

[A patient of Charcot-Marie-tooth disease with rigid spine and respiratory failure].

This report describes a case of a 17-year-old girl with Charcot-Marie-Tooth disease (CMT) representing rigid spine and respiratory failure. At age 11, she tended to walk on her toes and had difficulty in getting up from the floor without support. She became aware of flexion limitation of the neck at the age of 12. At 15 years of age, She began to have dyspnea on effort. When she was 17 years old, neurological examination revealed mild weakness of the upper extremities and severe weakness of the distal lower extremities, generalized wasting and areflexia. Superficial sensation was mildly impaired distally, and vibration sensation was severely impaired in the lower extremities. Motor and sensory nerve conduction velocities were mildly reduced, and compound muscle action potential of the tibial and peroneal nerves and sensory nerve action potential on ulnar and sural nerves were absent. Electromyography showed neurogenic changes with denervation potentials. Sural nerve biopsy revealed severe loss of myelinated fibers without any onion-bulb formation. As for family history, her elder sister showed moderate loss of vibration sensation in the lower extremities. On the basis of these findings, she was diagnosed as having CMT type 2, though a mode of inheritance was uncertain. She also had peculiar findings of flexion limitation of the spine (rigid spine), contracture of the hip joint, and fatty degeneration of paraspinal muscles on CT. Percent vital capacity (VC) was 22.5%, and arterial blood gas analysis showed PaO2 of 60.5 mmHg and PaCO2 65.0 mmHg. To our knowledge, this is the first case of CMT accompanied by rigid spine and respiratory failure. Motor and sensory neuropathy combined with rigid spine also have not been reported previously. The relationship between rigid spine syndrome with neurogenic muscular atrophy and CMT type 2C with the clinical characteristics of diaphragm and vocal cord paresis is discussed.

Adolescent↗

Schwartz-Jampel syndrome: report of one case.

We report a case of Schwartz-Jampel syndrome in a 2-year-9-month-old Taiwanese girl and her clinical response to treatment. She had a history of generalized muscle stiffness and hypertrophy since birth. Clinical and electromyographic myotonia were noted. Other clinical features included short stature, unusual facial appearance, ophthalmoplegia, elbow joint contractures, and developmental hip dysplasia. Muscle stiffness and myotonia improved after infusion of lidocaine. She then received oral mexiletine and the symptoms significantly improved.

Child, Preschool↗

[New therapeutic methods for spasticity and dystonia in children with cerebral palsy require multidisciplinary team work. Comprehensive approach yields good results].

New effective methods to reduce spasticity and dystonia are now included in the treatment of children with cerebral palsy (CP): selective dorsal rhizotomy (SDR), continuous intrathecal infusion of baclofen by an implanted pump or intramuscular injections of botulinumtoxin A. SDR is the only method that can reduce the spasticity permanently. Strong evidence exists for a positive impact of SDR on the function of the children. A multidisciplinary approach is required as it is essential to choose the best treatment for each child in each developmental period. In Lund physicians and physiotherapeuts from different disciplines such as paediatric orthopaedic surgery, neurosurgery and neurology form a central spasticity team which co-operates with the local child habilitation services. We have found that a common structured and standardized follow-up programme with early intervention against muscle hypertonia and imbalance can prevent hip luxation and contractures in children with CP.

Cerebral Palsy↗

[A clinical course and autopsy results of an 8-year-old severely handicapped girl with marked periventricular leukomalacia].

We reported a clinical course and autopsy results of an 8-year-old severely handicapped girl with marked periventricular leukomalacia. She was well until 3 days prior to first admission in local hospital. Two days prior to admission, she began to vomit. Twelve hours later, she was noted to be lethargic and developed malaise with frequent vomiting. At physical examination on admission, she had frequent fits and her posture was decerebrate rigidity. Consciousness disturbance continued for two weeks. Thereafter, she became severely handicapped with spastic quadriplegia, mental retardation and intractable epilepsy. She was transferred to our hospital one month later. We cared her totally and carefully with our rehabilitation staff, but during her course several rare happening occurred; she suffered from subdural hemorrhage due to hypocupremia and received an operation for the release of contracture of her hips. She died of acute cardio-respiratory failure at 8 years and 5 months of age. Her autopsy findings were characteristic of the damage to an immature brain during development; cactus formation of cerebellar cortex and periventricular leukomalacia.

Acute Disease↗

Sagittal plane alignment of the spine and gravity: a radiological and clinical evaluation.

Analysis of the sagittal balance of the spine includes the study of the spinal curves and of the pelvis in the sagittal plane. It therefore requires full-spine lateral radiographs. The sagittal balance of the spine was studied in forty-nine young adults. Strong correlations were observed between parameters related to the pelvis ("pelvic incidence angle", "sacral slope" and "pelvic tilting"), and the sagittal spinal curves ("lordosis" and "kyphosis"). We therefore propose to begin the evaluation of the sagittal plane alignment of the spine in clinical practice with measurement of the pelvic incidence angle. The relationship between the pelvic incidence angle and the sacral slope, as well as between the sacral slope and lordosis, is then assessed, and these are related to each other. The use of a graphic abacus facilitates assessment of the physiological comparison of the measured values and of the relationship between pelvic and spinal parameters, within their range of physiological variability. This analysis of the sagittal alignment of the spine also considers its dynamic aspect and the importance of gravity load and of muscular contraction on the lumbar structures. These data have been published previously and are recalled here. Three basic patterns of disruption of the relations between parameters may be encountered: a sacral slope angle exceeding the value expected considering the measured pelvic incidence angle (owing to fixed flexion contracture of the hips), excessive lordosis with regard to the observed sacral slope angle (with hyperkyphosis at the thoracic level) and stiff hypolordosis with pelvic retroversion. These three conditions are analysed in the light of the repercussions of the gravity load on the lumbar structures. A convenient method is thus available for functional analysis of the sagittal balance of the spine.

Adult↗

Orthopedic assessment of young children: developmental variations.

Parents often seek orthopedic evaluation of their young children because of apparent abnormalities. However, many of these are simply developmental variations that are part of normal growth and development. Pes planus, or flat foot, is one of the earliest and most common concerns. Torsional variations are also often seen; the presenting complaint may be intoeing (metatarsus adductus, tibial torsion and increased femoral anteversion) or out-toeing (pes calcaneovalgus and external rotation contractures of the hips). Angular variations (genu varum and genu valgum) are also seen frequently in young children. In assessing each finding, consideration must be given to the age at which the finding may be considered within normal limits, methods of examination and documentation, the expected course, findings that may signify abnormality, and appropriate follow-up and referral. An understanding of these common developmental variations in the orthopedic assessment of young children will enable the health care provider to respond to parents' concerns with accurate information and counseling.

Child↗