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Camptodactyly-arthropathy-coxa vara-pericarditis syndrome versus juvenile idiopathic arthropathy.

OBJECTIVE: The objective of our article was to highlight the important clinical and radiographic features of camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome. In particular, we emphasize those features that allow differentiation of CACP syndrome from juvenile idiopathic arthropathy. CONCLUSION: CACP syndrome should be considered in all patients who present with a noninflammatory arthropathy or with "atypical juvenile idiopathic arthritis," particularly if radiographs reveal an absence of erosions. In the correct clinical setting, large acetabular cysts on pelvic radiographs may be considered pathognomonic of CACP syndrome.

Adolescent↗

Lumbosacral agenesis. Three cases of reconstruction using Cotrel-Dubousset or L-rod instrumentation.

Three patients with lumbosacral agenesis underwent surgery to lock the lumbopelvic instability. All three patients had an unstable sitting position and a kyphotic bearing that impaired intestinal transit or hampered further colostomy or ureterostomy. Luque instrumentation with iliac fixation performed according to Galveston was used in one patient. Cotrel-Dubousset instrumentation was used for the two other patients. Autografts plus allografts provided sufficient bone for fusion without requiring lower leg amputations. Increased hip flexion was obtained after pelvic stabilization, but knee flexion contracture remained the same. All patients showed improved intestinal transit or decreased urinary infections, and two patients attained a stable sitting position without aid.

Adolescent↗

Musculoskeletal abnormalities and ichthyosis.

Musculoskeletal abnormalities and the associated findings of ichthyosis were seen in three patients between 1972 and 1975. Review of the literature revealed that musculoskeletal abnormalities had previously been recognized in patients with ichthyosis, but not emphasized because of the more frequent and profound neurological abnormalities. Interestingly, our patients had no neurological abnormalities. Ichthyotics should have thorough musculoskeletal as well as neurological and dermatological examinations.

Adolescent↗

Flexion contractures of the legs as the initial manifestation of adrenocortical insufficiency.

A 51-year-old woman presented with flexion contractures of the legs. Physical examination showed decreased passive movements of the bilateral hip and knee joints without muscle spasms or neurological abnormalities. Laboratory evaluation showed no response of ACTH or plasma cortisol to stimulation with CRH or insulin. Diagnosis of isolated adrenocorticotropic hormone deficiency was made. The patient was started on prednisolone 5 mg daily, and flexion contractures of the legs rapidly disappeared. Although the musculoskeletal manifestation of this patient is similar to that of stiff-person syndrome, flexion contracture of the legs associated with adrenocortical insufficiency seems to be a separate disease entity from stiff-person syndrome.

Adrenal Insufficiency↗

Distal hamstring lengthening in ambulatory children with cerebral palsy: primary versus revision procedures.

To document the benefits and limitations of distal hamstring lengthening (HL), 61 children (105 limbs) with cerebral palsy treated by distal HL with complete preoperative and postoperative evaluations were reviewed. There was significant improvement in popliteal angle, fixed knee flexion contracture, knee angle at foot contact (FC), and mid-stance knee extension after HL. On the other hand, the hip power generation peak decreased, and the anterior pelvic tilt increased. For the repeated HL (22 limbs), the fixed knee flexion contracture and knee flexion at FC improved. In a group of ten patients (17 limbs) with further postoperative follow up evaluations, the only significant clinical finding related to hamstring function between the first postoperative and the follow up evaluations with no intervening surgery was an increase in the popliteal angle of 20 degrees. Because the popliteal angle may increase over time after HL with no other directly related gait changes, the indications for repeated HL should include fixed knee flexion contracture and increased knee flexion at FC. The increase in the popliteal angle alone should not be considered an indication for repeated HL.

Cerebral Palsy↗

The influence of iliotibial tract on patellar tracking.

Thirty patients with 49 snapping hips and patellar malalignment underwent surgical release of the iliotibial tract contracture over the trochanteric area. Minimal follow-up was 2 years (average 4.6 years, range: 2-9 years). Eight patients underwent computed tomography (CT) preoperatively and 1 month postoperatively to investigate the patellar location in the patellofemoral articulation with knee bending at 0 degrees, 20 degrees, 45 degrees, 60 degrees, and 90 degrees. Significant improvements in the congruence angle and lateral patellofemoral angle were noted on Merchant radiograph for all knees (P<.01). On CT, at 20 degrees and 45 degrees knee bending, all congruence, lateral patellofemoral, and patellar tilt angles significantly improved postoperatively in 8 knees (P<.01). Iliotibial tract affects patellar tracking and dominates lateral patellar supporting structures.

Adult↗

Diabetic hand syndrome.

Diabetic hand syndrome is a condition affecting about 30% of patients with insulin-requiring juvenile diabetes. Characteristic findings in this syndrome are mild- to- moderately severe joint contractures of the fingers, particularly at the proximal interphalangeal joints in the ring and small fingers, and thickening of the skin of the dorsum of the hand. There is no evidence of palmar fascial thickening or Dupuytren's contracture. Occasionally other joints may be involved, such as the wrists, elbows, hips, knees, and toes. In the case presented in this report light and electron microscopic studies showed that the disease presents aspects similar to those of other "fibrotic diseases" as described by Kisher and Speer. Surgical treatment was only partially beneficial in this patient.

Child↗

[Clinical aspects and treatment of arthrogryposis multiplex congenita. Lower extremity].

This is a report about 25 patients of 28 AMC patients with affected lower limbs. From distal to proximal the joints are less attacked. The club-foot is most frequent. It nearly always needs operative treatment. The best results can be obtained by bony operations (exstirpation of the talus, lateral resection of the cuboid, subtalar arthrodesis). In knee joints the frequent flexion-gryposis is functionaly disturbing. It can be eliminated in most of the cases only by operation. The supracondylar extension-osteotomy has to be rejected in children in favour of a dorsal arthrolysis. Flexion-gryposis preponderate in hip joints. Conservative treatment is most often successful, when flexion is not supported by flexion-gryposis of the knee. Frog like contractures are very obstinate and need operative treatment. There exist different opinions whether open reduction of bilateral complete luxation of the hip is necessary or not. Our 3 patients with complete luxation of both hips had a satisfying function of hips after false external rotation was eliminated by osteotomy of the femur. Unilateral luxation of a hip needs open reduction even if a live in sitting position has to be expected. Our own experience is compared with reports of other authors and guiding principles for treatment are presented.

Arthrogryposis↗

Arthrography of the hip in children. Technique, normal anatomy and findings in unstable hip joints.

A method by which bilateral hip arthrography was performed under general anaesthesia in 130 infants and children 1 month to 7 years of age is described. Significant contractures of the adductor muscles had been corrected before the examination. Most of the children were girls with unilateral congenital idiopathic hip instability detected before the age of one year. From films exposed in standardized projections valuable information was obtained about the configuration of the joint cavity and the size and shape of the femoral head. Criteria for normal appearance in infants of ages 2 to 12 months are presented. More reliable details on the relation between the articular surfaces, the radiographic anatomy of the acetabulum and the distribution of the contrast medium were yielded by stereoradiography. Closed reduction was usually accomplished successfully during the arthrography, which made beginning of definitive treatment under the same anaesthesia possible.

Anesthesia, General↗

Cup arthroplasties in teen-agers and children.

Review of cup arthroplasties performed prior to 1970 in sixty hips of fity-one consecutive patients less than twenty years old, with follow-up on fifty-three hips, revealed that fifteen (28%) of the cup arthroplasties were followed by other salvage operations, usually total hip arthroplasty. Of the remaining thirty-eight hips, only twenty-four (63%) were rated satisfactory clinically, although most of the patients considered their cup arthroplasty an improvement over their preoperative status. Patients with bilateral hip disease did very poorly, and results were inconsistent in others. The final range of motion was frequently poor. Results were improved in hips in which acetabular reaming was not required.

Adolescent↗

Management of neglected joint contractures.

This review is based on the author's experience treating over 5,000 joint contractures of the lower limbs in developing countries in the tropics and subtropics over the past 40 years. It includes realistic methods of correction of neglected, untreated joint contractures mainly due to poliomyelitis and other paralytic conditions by general duty medical officers. Corrections were performed in centers where medical facilities were often limited and where millions of untreated patients still await treatment. The treatment includes percutaneous correction of flexed hips, knees and equinus ankles and rotational osteotomy of the distal femur for severe knee contractures. Surgery is followed by simple cheap braces made out of local materials. These methods will usually allow early mobility, and in severe cases survival instead of early death.

Contracture↗

Postoperative migration of the adductor tendon after posterior adductor transfer in children with cerebral palsy.

The adductor longus tendon was tagged with metallic markers after posterior adductor transfer in 17 children (33 hips) with cerebral palsy: 10 had spastic diplegia and seven had spastic quadriplegia. The average age at operation was 5 years 3 months, time of postoperative immobilization 5.7 weeks and average follow-up 1 year 7 months. In 19 hips the tendon remained attached to the ischium, in 11 hips it pulled away from the ischium and in three hips the result was equivocal. The incidence of detachment was higher among diplegic children than quadriplegic children.

Adolescent↗

[Significance of the sagittal profile and reposition of grade III-V spondylolisthesis].

The deformity in severe spondylolisthesis consists of two components: the parallel anterocaudad slip of the spondylolisthetic vertebra, and its tilt into kyphotic malposition. The influence of the two components is very different: the anterocaudad slippage has not much impact on the sagittal profile of the spine and is easily compensated for by a slight increase in lumbar lordosis. The kyphotic deformity has a high impact on trunk imbalance and the sagittal profile. There are two compensation mechanisms: hyperlordosis of the lumbar spine to its anatomical extremes and-if that is not sufficient-verticalisation of the sacral bone, performed by contracture of the hamstrings and uprighting of the pelvis around the hip joints. The latter mechanism is followed by functional disadvantages. Therefore, correction of the kyphosis of L5 may be considered during operative treatment if the lumbosacral kyphosis (angle delta) is less than 85 degrees and the sacral inclination less than 35 degrees.

Adolescent↗