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Triple osteotomy of the innominate bone. A procedure to accomplish coverage of the dislocated or subluxated femoral head in the older patient.

The triple osteotomy of the innominate bone is a total redirectional reconstruction and is obtained by circum-acetabular osteotomies through the ilium, ischium and pubis. The method achieves coverage of a dislocated or subluxated femoral head where other iliac osteotomies are ineffective or incomplete. Triple osteotomy was performed on 175 hips including 121 congenital dislocation-subluxations. The patients' ages ranged from 6 to 35, with 70 per cent falling between 9 and 12. Eighty-six per cent had a satisfactory result. Most of the unsatisfactory results were in cerebral palsy, myelodysplasia and peroneal muscular atrophy and associated with uncontrolled muscle contractures or progressive disease.

Adolescent↗

The camptodactyly-arthropathy-coxa vara-pericarditis syndrome: clinical features and genetic mapping to human chromosome 1.

OBJECTIVE: To delineate the clinical features in patients with the autosomal recessive camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) and to determine the location of the involved gene. METHODS: Eight affected individuals (ages 2-15 years) with CACP from 4 consanguineous kindreds were clinically evaluated. Four patients are newly described and 4 have been reported previously. Findings were compared with those in 21 other previously reported cases. DNA obtained from the 8 affected patients and their available siblings and parents was used in a genome-wide search for linkage. RESULTS: Congenital camptodactyly and childhood-onset noninflammatory arthropathy were present in all affected patients. Seven patients developed bilateral coxa vara deformity, and 1 developed coxa magna with cystic erosions. Two of the patients also had symptoms or signs of pericarditis. A genome-wide search for linkage identified homozygosity for a series of genetic markers on human chromosome 1q in all affected patients. The marker D1S191 yielded a maximum logarithm of the odds ratio (LOD score) of 3.3 at theta = 0. The CACP gene lies within a 1.9-cM candidate interval defined by the markers D1S2107 and D1S222. CONCLUSION: The principal features of the CACP syndrome are congenital or early-onset camptodactyly and childhood-onset noninflammatory arthropathy. Coxa vara deformity or other dysplasia associated with progressive hip disease may develop over time. Clinical pericarditis may also occur. A locus responsible for causing CACP syndrome is assigned to a 1.9-cM interval on human chromosome 1q25-31 by homozygosity mapping. This now facilitates the identification of the responsible gene and permits testing for locus homogeneity in other CACP kindreds.

Adolescent↗

Proximal femoral resection for older children with spastic hip disease.

Thirteen patients (19 hips) who underwent proximal femoral resection arthroplasty for symptomatic subluxation or dislocation of the hip were reviewed retrospectively. All had severe neurological involvement, and when older were non-ambulatory with spastic quadriplegia or myelodysplasia. Resections were either at the level of the neck (nine hips) or in the subtrochanteric region (10 hips). The latter provided a better operative result than did the former. Poor clinical results noted were continued pre-operative pelvic obliquity, postoperative adduction contracture, and bony contact between the pelvis and the remaining proximal femur or its heterotopic ossification.

Adolescent↗

Different methods of treatment related to the bilateral occurrence of Perthes' disease.

We treated 98 consecutive patients with Perthes' disease by a unilateral brace in external rotation, flexion and abduction and a further consecutive 110 by a bilateral cast with the hips in internal rotation and abduction. During treatment in the unilateral brace, six (6.1%) hips on the opposite side developed evidence of Perthes' disease and one developed this after the brace had been removed. In children managed in bilateral casts, no contralateral Perthes' disease was seen. Adequate containment of the femoral head may prevent subsequent changes in the opposite hip.

Braces↗

Maintenance of ambulation in Duchenne muscular dystrophy. The role of the orthopedic surgeon.

As the processes of pseudohypertrophic muscular dystrophy advance, contractures about joints in the lower extremities progress until an unstable base of support secondary to equinovarus and weakened pelvic balance produced by hip flexion prohibit ambulation. Properly timed surgery and bracing have helped some patients to extend significantly their ability to ambulate. Because severe weakness can result from prolonged restraint, operative procedures must permit immediate mobilization without fear of excessive pain or wound dehiscence. The primary aims of surgery are to maintain standing balance and prolong independent ambulation. Conditions requiring surgical correction are lower extremity flexion contracture, metatarsus adductus, and rigid forefoot equinocavovarus. Techniques designed to improve these deformities and permit early postoperative mobilization include subcutaneous release of contracted tendons and percutaneous removal of cancellous bone with corrective manipulation of the feet. Postoperative plastic bracing enhances balance through mild knee flexion and ischial seating. Maintenance of the upright posture extends the ability of these patients to attend to their tasks of daily living. In addition, these techniques offer a method of prolonging ambulation in the patient with pseudohypertrophic muscular dystrophy. In so doing, confinement to a wheelchair with its inevitable downhill course may be significantly postponed.

Braces↗

A randomized controlled trial of early surgery in Duchenne muscular dystrophy.

We performed a randomized controlled trial of early surgical treatment of contractures in 20 boys with Duchenne muscular dystrophy, age 4-6 yr. Surgery consisted of release of hip flexors, removal of iliotibial bands, and lengthening of tendo Achilles bilaterally. All patients were monitored for at least 12 months post-randomization, and assessed quantitatively for muscle strength and function. Surgery corrected the deformities, but had no beneficial effect on strength or function. Indeed, data in the second year showed more rapid deterioration of function in some of the operated boys. There appeared to be continued evolution of pathology following surgery, as assessed by sequential muscle ultrasound and muscle biopsy. We cannot recommend this type of surgery as a routine treatment.

Biopsy↗

Adapting pediatric DXA scanning to clinical orthopaedics.

Osteoporosis and low bone density for age is increasingly recognized in children yet poorly understood and often overlooked. DXA scanning in children is problematic due to lack of guidelines and inexperience of most DXA practitioners. The International Society for Clinical Densitometry recommends spine or total body scans as the preferred skeletal sites for study in children, yet we find multiple regional scans to be far more useful in the clinical practice of pediatric DXA scanning, especially in a pediatric orthopaedic setting. DXA scans of 119 children are reviewed: 20% of the children had orthopaedic hardware or other artifact that would have rendered the whole body scan unusable. In 20 of 119 patients, the only scan that could be obtained, either due to contracture or patient cooperation, was the distal lateral femoral scan. Scanning multiple regions of interest including spine, hip, and/or distal lateral femora as indicated is the recommended protocol for assessment of BMD in the pediatric orthopaedic setting.

Absorptiometry, Photon↗

[Orthopedic aspects of Rett syndrome].

Rett syndrome is a degenerative neurological disorder with onset between 6 and 18 months of age. Severity of motor impairment is variable; some patients lose the ability to walk whereas others walk nearly normally. Orthopedic problems, which mainly involve the lower limbs and spine, can increase functional impairment and cause discomfort. Abnormal joint alignment in the lower limbs due to muscle contractures requires physiotherapy and orthopedic appliances. Surgery may be necessary, in particular to prevent dislocation of the hip or to correct talipes equinus. Scoliosis is common and unresponsive to conservative therapy and should be treated surgically if severe.

Child, Preschool↗

Microdissection thinning of a pedicled deep inferior epigastric perforator flap for burn scar contracture of the groin: case report.

A severe burn scar contracture of the groin in a 25-year-old woman was successfully reconstructed with microdissection and thinning of a pedicled deep inferior epigastric perforator (DIEP) flap. This perforator flap is a relatively thin cutaneous flap, but may still require secondary defatting procedures, especially when used for coverage of mobile anatomic areas such as the groin and axilla. A deep inferior epigastric perforator flap was radically thinned by excising the subcutaneous fatty layer very carefully under loupe magnification, to preserve the subdermal vascular plexus. One year after surgery, the patient can abduct and rotate the hip freely without limitation.

Adult↗

[Psoas abscess: report of a case].

The retrofascial space is a concealed one located behind the transversalis fascia in the posterior peritoneal cavity. It contains mainly muscles and fasiae and extends from the mediastinum to the pelvis. Clinical manifestations of an abscess are usually obscure except for hip joint problems when the abscess developed. Computed tomography is done immediately if an abscess is suspected, and there have been good results under incision with drainage and antibiotics. It is different from retroperitoneal abscess anatomically, pathologically, bacteriologically and clinically, so we report a case for reference. The patient, a 43-year-old female worker, had suffered from disability of her left lower limb for more than 5 months before admission. She even visited her local medical doctor, but in vain. In the week prior to admission, left hip swelling with awkwardness in walking was felt, and she called on an orthopedist. After brief examination, impaired hepatic and renal function was noted, so she was referred to our hospital. The chief complaint on admission was left hip swelling and pain with radiation to the inguinal and knee area for one week. The past history was insignificant except for a cesarean section and an appendectomy done more than 10 years before. Upon physical examination, the patient was found to be pale, emaciated, mildly icteric and had a flexion contracture of the left thigh. Laboratory data revealed anemia, leukocytosis, impaired hepatic and renal function. The chest X-ray was normal, and KUB demonstrated left hip joint flexion and external rotation. A sonogram showed a localized hypoechoic lesion in the left posterior peritoneal cavity 2 days later.(ABSTRACT TRUNCATED AT 250 WORDS)

Abscess↗

Reliability and validity of visual assessments of gait using a modified physician rating scale for crouch and foot contact.

This study evaluates the visual assessment of gait using portions of the Physicians' Rating Scale (PRS). Thirty children with pathologic gait were evaluated "live" and using full- and slow-speed video. Interobserver reliability (weighted kappa) was 0.57 to 0.74 for foot contact, 0.69 to 0.71 for crouch, 0.30 to 0.40 for hip flexion, 0.57 to 0.65 for knee flexion, and 0.42 to 0.52 for dorsiflexion in stance. Intraobserver reliability (comparing the three conditions) was 0.50 to 0.78 for foot contact, 0.71 to 0.80 for crouch, 0.26 to 0.44 for hip flexion, 0.60 to 0.86 for knee flexion, and 0.39 to 0.61 for dorsiflexion. Observers were correct only 12% to 32% of the time when reporting less than 0 degrees of dorsiflexion and 0% to 29% of the time when reporting more than 20 degrees of hip flexion due to overestimation of hip flexion and underestimation of ankle dorsiflexion. These errors could lead some clinicians to presume the presence of contractures that do not actually exist. Visual assessment using the PRS does not appear to accurately measure what it is most commonly used to assess: ankle position in stance.

Adolescent↗