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Seating for children with cerebral palsy.

Special seating enables children with even the most severe forms of cerebral palsy to sit comfortably. A straight spine and mobile hips are desirable. Prevention of hip dislocation by operation, or release of a hip extension contracture, is required for 40% of the younger children. Surgical correction of scoliosis is required for 20% of the older ones. Seating problems may be classified by (a) ability--hands free, hand dependent or propped--(b) the pattern of deformity--symmetrically slouched or windswept--and (c) severity of deformity--none, amenable to surgery, or beyond surgery. Each of these categories requires a different therapeutic approach.

Abnormalities, Multiple↗

Physical therapeutic and surgical management in spastic diplegia. A Japanese experience.

With the development of intensive neonatal care, spastic diplegia associated with prematurity has become the most common type of cerebral palsy. The principles of the present authors' treatment for children with the disease are physical therapy (involving neurodevelopmental treatment) and surgical treatment for contractures and deformities. The authors studied the long-term results of physical therapy and its effect on the necessity for surgery and the improvement of locomotor function. From 1973 to 1988, 355 children with spastic diplegia were treated at the authors' facility. From this group, the authors selected and studied 71 children who received systematic treatment for a minimum of one year and who could be followed at the age of six years or older. At the follow-up examination, 41 of these children were free ambulators, 28 were crutch ambulators, and two were not ambulatory. Of the 41 freely ambulatory children, four achieved free ambulation after the age of seven years. Of the 28 crutch ambulators, relatively small hip-flexion contractures were found in 11 children who had received the early developmental treatment program.

Cerebral Palsy↗

Surgical approaches for resection of heterotopic ossification in traumatic brain-injured adults.

The site of heterotopic ossification (HO) at the elbow or the hip dictates the surgical approach for resection. Three approaches are used for HO resection at the elbow: (1) a posterolateral approach for posterolateral HO; (2) an anterolateral approach for anterior HO; and (3) a medical approach for medial or posteromedial HO or anterior transfer of the ulnar nerve. Two approaches are recommended for resection of HO at the hip: (1) an anterior approach for anterior or inferomedial HO and (2) a posterior approach for posterior HO. Posterior HO is often associated with a hip-flexion contracture, and an anterior soft-tissue release may be necessary as well. Physical examination indicates the prognosis for functional improvement as well as recurrence. Patients with a near normal neurologic recovery have minimal to no HO recurrence with improved limb function and increased joint motion, whereas a poor neurologic recovery and persistent spasticity are associated with recurrence of HO and no functional limb improvement. Standard roentgenograms aid in selecting the appropriate surgical approach. Radiation, indomethacin, and diphosphonates have been administered for prophylaxis. Physical therapy is necessary until range of motion stabilizes.

Adult↗

[Report on the diagnosis and treatment of muscular contracture. The Ad Hoc Committee of the Japanese Orthopaedic Association of Muscular Contracture].

Muscular contracture due to repeated intramuscular injections raised urgent questions from the medico-legal standpoint when a large number of children with quadriceps contracture was found in Yamanashi Prefecture in 1973. In 1975, the Japanese Orthopaedic Association formed an Ad Hoc Committee on Muscular Contracture to investigate the diagnosis and treatment of this particular condition. Since then, the Committee has studied the symptomatology, diagnosis, natural history, orthotic and operative treatment of quadriceps, deltoideus and gluteus contractures. The results have been reported annually to the Japanese Orthopaedic Association, and guidelines for diagnosis and treatment have been made available to its members. Quadriceps contracture can be classified into three types: the rectus femoris, vastus, and mixed types. The rectus femoris type represents 80 to 90 per cent of cases with quadriceps contracture, while the vastus type is quite rare. In both the rectus femoris and mixed types, operative treatment is suggested when the knee flexion is limited to 30 degrees or less in the prone position. Transverse division of the rectus femoris at the muscle belly is the standard operative procedure recommended for the rectus femoris type and gives very satisfactory results in most cases. In the mixed type, an additional division of the scarred portion of the vasti is needed. The ideal age for such procedures is near or after the end of growth. The vastus type is difficult to cure, but to improve the condition to a certain extent an operation is suggested when the knee flexion is limited to 45 degrees or less in the supine position. The operative procedure recommended is either release of the affected vasti at their insertion to the patella or Z-lengthening of the common tendon of the quadriceps. In deltoideus contracture, the Committee proposed a scoring system for evaluating the severity with the grade of abduction contracture and the opposite shoulder test as parameters. A score of 5 points or more indicates operative treatment. The operative procedure recommended is release of both the acromial part and the anterior fibers of the spinal part of the deltoideus. The ideal age for this procedure is 12 years or older. A dent produced by the operation distal to the acromion, however, has to be regarded as a cosmetic complication. To avoid this complication, advancement of the severed fibers of the deltoideus from the scapular spine to the acromion is needed. Such an advancement procedure is suitable for children of 14 or 15 years of age and leaves the natural round contour of the shoulder intact.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

Abduction contracture: an unusual complication in the treatment of acute capital femoral epiphysiolysis.

A significant abduction contracture of the hip followed closed reduction and pinning of an acute slip of the proximal femoral epiphysis in a 13-year-old boy. The contracture was corrected by removal of this bony prominence. Over-reduction of the slipped epiphysis into valgus is thought to have stimulated bony overgrowth at the posteromedial aspect of the capital femoral epiphyseal-neck junction. This causes a block to adduction by abutting against the inferior lip of the acetabulum. To our knowledge, this complication has not been previously reported.

Adolescent↗

Vascular complications associated with orthopedic procedures.

Damage to vascular structures during orthopedic procedures occurs relatively infrequently; when it does occur, however, recognition and prompt intervention are essential. We report herein 11 vascular injuries secondary to orthopedic procedures encountered between 1978 and 1988. Two injuries occurred as a consequence of lumbar laminectomy, three as a result of total hip replacement, three secondary to open reduction and internal fixation of a fracture of a lower extremity, two secondary to attempted closed reduction of humeral fractures and one injury as a result of hip flexion contracture release. Injury occurred to three iliac arteries, three popliteal arteries, two brachial arteries, one femoral artery, one graft to femoral anastomosis and two iliac veins. Five arterial injuries were repaired primarily, one with a vein patch, while five required bypass grafts. One venous injury was repaired primarily and the other required placement of a Greenfield filter for thrombosis. Major complications were a result of diagnostic delay and subsequent ischemia in most patients. There were no deaths. We conclude that vascular injuries can occur as a result of laceration, compression or traction during orthopedic procedures as a result of the proximity of vascular structures to the spine, joints and long bones. Also, we conclude that injuries manifest themselves primarily as hemorrhage or ischemia; that excellent results can be obtained with prompt recognition and treatment; that angiography is useful in those with mild ischemia in whom diagnosis is delayed, and that preoperative documentation of the vascular status of patients is critical prior to orthopedic procedures.

Adolescent↗

Computed-tomography-based computer preoperative planning for total hip arthroplasty.

For precise preoperative planning in total hip arthroplasty (THA), we developed a technique of computed tomography (CT)-based computer preoperative planning and compared this technique with the single X-ray and template method generally used. The subjects of this study were 42 hips in 38 patients who underwent THA using a cementless total hip system. Preoperatively, a standard anteroposterior X-ray of the hip was taken, and conventional preoperative planning was done with a template of the total hip system. Transverse images were obtained using a helical CT scanner, and a CT-based computer preoperative plan was performed on true coronal slice images of the proximal femur reconstructed from CT data. Postoperatively, 29 hips (69%) showed good proximal fit of the femoral component to the medial endosteal line. Of the 20 hips with good proximal fit on preoperative X-ray planning, 12 hips had good proximal fit on postoperative X rays. Sensitivity and specificity of the proximal fit on X-ray templating were 41 and 23%, respectively. In 27 of 28 hips with good proximal fit on reconstructed CT images preoperatively, the postoperative X ray revealed good proximal fit. Sensitivity and specificity of the proximal fit on computer planning were 93 and 86%, respectively. Twelve hips with good proximal fit on preoperative templating, the reconstructed images, and the postoperative X ray had 20 degrees or less of combined femoral neck anteversion and external rotational contracture of the hip on the X-ray table. Eight hips with good proximal fit on preoperative templating and proximal poor fit on the reconstructed images had 17-65 degrees of combined version and rotational contracture. In 16 hips with poor proximal fit on preoperative templating and good proximal fit on the reconstructed images, the combined version and rotational contracture ranged from 17 to 69 degrees. When combined femoral neck anteversion and external rotational contracture of the hip is less than 15 degrees, the simple X-ray and template method might be sufficient for THA planning. Otherwise, the CT-based method of preoperative planning is recommended.

Arthroplasty, Replacement, Hip↗

Infant walkers and cerebral palsy.

We studied a 1-year-old infant with spastic cerebral palsy in its early stage. An infant walker was used by the mother to amuse the infant, but the walker was observed to produce a positive support reflex, perpetuating a primitive reflex that should fade during the first year of life. The walker also prevented the infant from practicing equilibrium reactions and protective responses that should be developing during this age. Positions assumed by the infant in the walker contribute to the development of common adverse sequelae of spastic cerebral palsy: heel cord contractures, sublocations and dislocations of the hips, and pronation contractures of the upper extremities.

Adolescent↗

Total hip replacement in children with arthritis.

Fourteen children with juvenile rheumatoid arthritis and two with ankylosing spondylitis received 29 total hip replacements (THR). The ages at THR were 12 to 18 years, the median duration of prior hip disease was 7.1 years, and the minimum followup was one year (range 1--4 years, median 2.2 years). The primary indication for THR was pain in 5 hips and severe malposition or flexion contractures in 24 hips. (Sixteen of these also had some degree of pain.) All 29 hips demonstrated improved postoperative range of motion, and all were free of pain. In children, active rheumatic disease in other joints, prosthesis longevity, and possible lack of adequate motivation all merit special consideration prior to THR, but the results are encouraging.

Adolescent↗

[Contraction of the hip and fecal drainage via a fistula tract 30 years after "appendectomy"].

A 46-year-old female was admitted with increasing fecal drainage via a fistula tract in the right inguinal region. She had a history of surgery for appendicitis 30 years previously, from which there was disturbed wound healing resulting in a blunt fistula, and the patient suffered from contraction of the right hip. Computed tomographic scan and ultrasound demonstrated an inflammatory mass in the right inguinal region. Colonoscopy demonstrated a stenosis of the rectosigmoid junction but did not provide any further specific information. Surgery revealed the presumed diagnosis of complicated Crohn's disease, but an advanced squamous cell carcinoma was also identified. The patient died 23 months later due to generalized tumor. Although malignant transformation of a fistula tract is rare, this case demonstrates that long-standing fistulas should be cured as far as possible without significant morbidity. In the case of incurable fistulas, malignancy must definitely be excluded if the clinical appearance of the fistula changes.

Appendectomy↗

Lumbar hyperlodosis in cerebral palsy: anatomic analysis and surgical strategy for correction.

INTRODUCTION: We report our experience of five cases in adolescents with spastic quadriplegia who had primarily sagittal plane deformity with hyperlordosis of the lumbar spine, an uncommon condition in cerebral palsy spinal deformity. METHODS: Three boys and two girls, 13 to 19 years old, were surgically treated for excessive lumbar lordosis which made sitting difficult. Bilateral hip flexion contractures were present in all cases with a very horizontal sacrum and, in four cases, associated with pelvic anteversion. Two patients were surgically treated by posterior spinal fusion and instrumentation, and three patients had anterior discectomy and fusion prior to posterior fusion and instrumentation. CONCLUSION: Hyperlordosis was reduced by 26 to 48 degrees , and the horizontal sacrum was corrected by 15 to 35 degrees , which enabled the patients to sit comfortably.

Adolescent↗

Unilateral cryptophthalmia.

Two patients had the variable clinical features of unilateral cryptophthalmia. A 5-month-old boy had isolated unilateral cryptophthalmia: a small boney orbit, deformed optic canal, and a small amorphous mass with no normal intraocular tissue representing the globe. No extraocular muscles or optic nerve were identified by B-scan ultrasound or by computed axial tomography x-ray techniques. The second patient, a 13-year-old girl, had unilateral cryptophthalmia, and numerous systemic abnormalities including a head circumference less than the third percentile, severe mental retardation, hypoplasia of the left side of the head, and a left facial cleft deformity. She also had contractures of hips, knees and ankles, and bilateral spasticity and jerky movements. The left boney orbit was contracted and deformed and contained a small amorphous tissue with no ocular detail, as revealed by B-scan and computed tomography scan.

Abnormalities, Multiple↗

The phenotype of chromosome 2p-linked limb-girdle muscular dystrophy.

This study reports on a detailed clinical, electrophysiological, muscle computed tomography (CT) and laboratory investigation carried out on five families with definite linkage to chromosome 2p. Some clinical and laboratory features were common to most of the patients, such as the very high serum creatine kinase (CK) levels (mean 43.70 times the normal). The onset was most frequently in the late teens or early twenties with weakness and wasting of the pelvic girdle muscles. All patients had normal motor milestones and had not complained of any symptoms of muscle disease in early childhood. The clinical course was variable both between and within some families, but was most often slowly progressive. Some variability in the pattern of muscle involvement between the different families has also been observed.

Adolescent↗

Rehabilitating patients with hepatopulmonary syndrome using living-related orthotopic liver transplant: a case report.

The objective of this study was to rehabilitate a patient with hepatopulmonary syndrome (HPS) who underwent living-related orthotopic liver transplantation (LT). HPS is rare; it presents severe complication in patients with liver disease. A 17-year-old woman with HPS developed portal hypertension after undergoing Kasai's surgery for congenital biliary atresia and underwent a living-related orthotopic LT. After LT, her allograft functioned well, but she continued to have hypoxemia and orthodeoxia. She was referred for rehabilitation for disuse atrophy, contracture of hip and shoulder joints, left common peroneal nerve palsy, and rehabilitation for respiratory dysfunction. By day 106 after LT, her orthodeoxia and disuse atrophy had improved because of daily exercise training and active joint range of motion exercises. Patients with HPS have orthodeoxia and poor responsiveness to oxygen therapy, and correction of hypoxemia after LT may be delayed. Therefore, rehabilitation approaches for patients with HPS should be based on the pathophysiology and characteristics of HPS.

Adolescent↗