Sisto Turra

Musculoskeletal manifestations in pediatric acute leukemia.

Background: In children, acute leukemia (AL) at presentation can mimic several orthopaedic pathologies, so that a variable delay of the correct diagnosis is often reported. Methods: To define more clearly the clinical and radiological musculoskeletal manifestations of leukemia in children, 122 affected children referred from 1984 to 1999 to our Pediatric Onco-Hematologic Clinic were retrospectively reviewed. Average age at diagnosis was 6.6 years (from 7 months to 17 years). Seventy-three (60%) were boys and adolescent boys, 49 (40%) were girls and adolescent girls. One hundred two (83.6%) had acute lymphoblastic leukemia, 20 (16.4%) had acute myeloid leukemia. The mean follow-up was 8.2 years for the 104 survivors and 2.5 years for the 18 nonsurvivors. The &khgr;2 test was used to perform the statistical analyses. Results: At presentation, complaints related to the musculoskeletal system were frequent (38.3%), including pain (34.4%), functional impairment (22.9%), limping (12.3%), swelling (10.6%), and joint effusion (5.7%). At presentation, 40.2% of children had at least 1 radiographic abnormality. In order, they were osteolysis (13.1%), metaphyseal bands (9.8%), osteopenia (9%), osteosclerosis (7.4%), permeative pattern (5.7%), pathological fractures (5.7%), periosteal reactions (4.1%), and mixed lysis-sclerosis lesions (2.5%). Different from previous reports, late radiographic lesions were uncommon (5.7%), probably because of milder newer medication protocols. They included avascular necrosis (3.3%), vertebral collapses (1.6%), and osteolysis (0.8%). Conclusions: Both clinical and radiological changes had various and no uniform localization. Poor correlation was found between symptoms and radiological lesions. Survival rates in children with AL were 95.8% at 1 year, 89.6% at 3 years, 85.8% at 5 years, and 83.4% at 10 and at 13 years. Radiographic abnormalities (P = 0.400), type of leukemia (P = 0.291), sex (P = 0.245), and white blood cell count at presentation (P = 0.877) were not prognostic factors. The presence of multiple bone lesions did not affect the survival rate (P=0.632). As early diagnosis significantly decreases morbidity and mortality of AL, the orthopaedist should suspect AL in any child with unexplained persistent skeletal pain or radiographic alterations. Accurate history, general physical examination, and complete blood cell count tests should address the suspicion, which is confirmed by a peripheral and/or iliac crest bone marrow biopsy. Level of Evidence: Retrospective comparative study III.

A 20-year follow-up study of a case of surgically treated massive osteolysis.

Massive osteolysis of the humerus occurred in a 19-year-old male. The lesion was successfully treated with an autogenous fibular shaft transplant. During the 20-year follow-up period, humerus function was restored. Roentgenograms showed incorporation of the graft without any recurrence of the disease. This experience and reports in the literature show that only predominantly cortical autogeneic bone grafting has been successful. This could be due to the intrinsic characteristic of cortical bone. In fact, it seems that cortical bone shows greater resistance to erosion than cancellous bone to the lymphangiomatous osteolytic tissue. Therefore, autogenous bone grafting could be considered as a reasonable alternative to radiotherapy, particularly in young people with monostotic disease localized in bones that are easily operable and have no soft-tissue involvement.

Prognostic value of Catterall and Herring classification in Legg-Calvé-Perthes disease: follow-up to skeletal maturity of 32 patients.

To compare the prognostic value of the Catterall and Herring classification, 32 patients, treated without surgery for unilateral Perthes diseases, were reviewed and classified at skeletal maturity according to Stulberg. The Catterall classification had no significant prognostic correlation with the final outcome. Only some head at-risk signs, such as lateral epiphyseal calcification and epiphyseal subluxation >4 mm, were prognostic. The Herring lateral pillar was not predictive when it was considered alone, but it became prognostic when related to age at onset. Moreover, in group C of the Herring classification (lateral pillar <50%), the final outcome was better in children younger than 6 years. This last observation strengthens the opinion that age and related residual skeletal moulding are also of prognostic value in Perthes disease.

Polydactyly of the foot.

Fifty-six patients with 72 duplicated toes were analysed. Postaxial duplication accounted for 79%, and the most common anatomical pattern was duplication of the proximal phalanx with a wide metatarsal head. Forty-two patients with 55 duplications were clinically and radiographically evaluated at long-term follow-up (mean 22.5 years). Results were satisfactory in 91% of the patients. Poor results were often associated with preaxial polydactyly because of persistent hallux varus. Surgical treatment is usually straightforward but must be individualized, and some anatomical and surgical details should be considered to obtain a better result.

Prevalence of unsuspected myopathy in infants presenting for clubfoot surgery

The objective of this study was an evaluation of the prevalence of myopathies in paediatric patients scheduled for orthopaedic surgery (clubfoot) performed under regional anaesthesia. Seventeen infants scheduled for lower limb orthopaedic surgery were studied to verify coexisting neuromuscular disorders with electromyography and muscle biopsy during surgery. All surgical procedures were performed under caudal block or spinal anaesthesia, associated with light general anaesthesia. No major cardiorespiratory, neurological or malignant hyperthermic complications (muscle rigidity, arrhythmias, hyperpyrexia) were observed. Combined neurological, electromyographic and biopsy studies showed a high rate of myopathic changes (70%). Performance of clubfoot surgery under light general anaesthesia with regional techniques was free from any problems. The high rate of myopathic changes (70%) observed in the muscle biopsies suggests that precautions should be taken with paediatric patients for clubfoot surgery and a regional anaesthesia technique with adequate monitoring may be helpful to prevent possible malignant hyperthermia related problems.