Vito Pavone

Total condylar knee arthroplasty: a long-term followup.

Between July 1977 and December 1983, 80 patients underwent 120 arthroplasties using a total condylar knee prostheses. Forty-one patients (68 knees) died and 13 patients were lost to followup. Twenty-six patients with 34 total condylar replacements were available for clinical followup. During the followup, 10 knees in nine patients (8.3%) from the overall 80 patients (120 knees) underwent revision; three (four knees) for aseptic loosening, one for periprosthetic fracture, three for infection, and two patients underwent revision for pain. Three revisions (three patients) occurred in the group of 26 patients available for followup. The average age of this group of patients at followup was 78 years (range, 53–94 years). There were 10 men and 16 women. Considering the high mean age of the patients in the series and patients’ overall health status, the clinical results were extremely good. Kaplan-Meier analysis showed a survivorship of 91% at 23 years followup, considering revision as an end point. Although there have been several changes in total knee replacement designs, materials, and implantation techniques, the long-term outcome of the original total condylar knee prosthesis is excellent.

Perioperative morbidity in bilateral one-stage total knee replacements.

A retrospective review was done on 501 patients who had bilateral sequential one-stage total knee replacements from September 1995 to April 2000 to evaluate perioperative (inhospital) morbidity. One thousand two knee replacements were done with the patients receiving regional anesthesia, on 286 women and 215 men with an average age of 66 years. The average transfusion requirement was 2.8 units of blood per patient. There were no deaths, myocardial infarctions, or cerebrovascular accidents. The mean length of hospital stay was 7.2 days. One hundred forty-four perioperative complications were observed in 109 patients (21.8%). These complications included 27 arrythmias (5%), one congestive heart failure (0.2%), 65 lower extremity deep venous thromboses (13%), 14 fat emboli (3%), and two pulmonary emboli (0.4%). Other major complications were pneumonia (1%), acute renal failure (0.4%), ileus (2%), and mental status changes (2%). Wound complications included two deep infections (0.4%), three hematomas (0.6%), and five delayed wound healings (0.9%). According to the current study the perioperative morbidity and mortality is acceptable if the procedure is used for selected patients. Patients with significant comorbidities should have a staged bilateral total knee replacement.

Congenital bone malformations in patients with neurofibromatosis type 1 (Nf1).

To address the prevalence of congenital bone malformations in neurofibromatosis type 1 (Nf1; compared with Nf1 bone anomalies and tumors), we studied the Nf1 population of 135 children (70 boys, 65 girls) seen at the neurofibromatosis clinic, University of Catania, Italy, in the period 1990 through 1996. Twelve (8.8%) of the 135 children had congenital bone malformations: unilateral or bilateral postaxial polydactyly type A (n = 1) and type B (n = 1), bilateral postaxial polydactyly type A in association with preaxial polydactyly type 1 (n = 1) and bilateral preaxial polydactyly (n = 1); clynodactyly (n = 1); multiple segmentation anomalies of the vertebrae (n = 6), and complex costovertebral anomalies (n = 1). We found a higher frequency of polydactyly (2.9%) in comparison to that in the general population (0.014-0.12%) and in our national (0.027%) and regional (0.066%) population, and a higher frequency of vertebral (5.1%) and costovertebral (0.7%) malformations. This is the first study to state the overall prevalence of congenital bone malformations in an Nf1 population. Interestingly, polydactyly (postaxial type) and Nf1 was previously reported only once.

Bone ultrasonometry, bone density, and turnover markers in type 1 Gaucher disease

Abstract. In 12 patients (mean age, 33 ± 13 years) with type 1 Gaucher disease (GD), we evaluated bone mass by broadband ultrasound attenuation (BUA) of the calcaneus and dual X-ray absorptiometry (DXA) of the total body, lumbar spine, and hip. In all patients, we measured serum levels of osteocalcin (OC) and bone-specific alkaline phosphatase (BAP) and urinary excretion of pyridinoline (Pyr/Cr) and deoxypyridinoline (D-Pyr/Cr) cross-links. Compared to age- and sex-matched healthy controls, patients with GD showed marked osteopenia at all measuring sites as expected. Values of BUA (67.25 ± 15.83 dB/MHz) were also significantly reduced. OC and BAP concentrations were within the normal range. Pyr/Cr and D-Pyr/Cr were significantly higher than in controls. Calculating T- and Z scores, we found a significant correlation between the Bone Severity Score Index (BSSI) and both BUA and BMD measurements. A significant correlation was also found between pyridinoline urinary excretion and both BSSI and BUA at the calcaneus. Our data suggest that type 1 GD in adulthood is associated with increased bone resorption and that BUA at the calcaneus may be a relevant tool in the assessment of bone status in these patients.

Blood management after bilateral total knee arthroplasty.

Four hundred sixty-one patients who had bilateral one-stage total knee replacements were reviewed to evaluate their blood management. Overall, patients received an average of 2.1 units of autologous blood and 0.9 units of allogenic blood. Seventy-six percent of the patients who preoperatively donated one unit of autologous blood required allogenic blood transfusions compared with 51% of patients who donated two units, 29% of patients who donated three units, and 27% of patients who donated four units of autologous blood. Ninety-eight percent of the patients who did not donate autologous blood required allogenic blood. Donating two units of autologous blood in combination with a perioperative cell saver reduced the incidence of allogenic blood transfusions to 8% but increased the amount of unused autologous blood to 54%. If the indication for wound drainage recovery is guided by the preoperative hematocrit (≤ 40%) or postoperative hemoglobin (≥ 11 mg/dL) the incidence of allogenic blood transfusions decreased to 17% and 13%, respectively and the amount of unused autologous blood decreased to 39% and 30%. There is no statistical difference among the three protocols regarding the need for allogenic blood transfusions and associated costs. Based on this retrospective evaluation the combination of preoperative donation of two units autologous blood and use of a postoperative cell salvage system in all patients is recommended.

Tuberculosis of the Ankle in Childhood: Clinical, Roentgenographic and Computed Tomography Findings

We report on three Italian children who presented with unilateral ankle tuberculosis (TB) consecutively during a short time period and in the same geographical area. A 6-year-old-girl with a family history of TB had limited mobility of the right leg at age 9 months; Mantoux test and radiographs at that age yielded normal findings. When severe right tibiotarsal swelling, reddening, pain and restriction of motion became apparent at age 4.6 years, the typical lesions of TB were evident on radiographs and computed tomographic (CT) scans. Mantoux test and synovial biopsy confirmed TB. A three-drug regimen of treatment proved useless: articular cartilage destruction and diffuse osteosclerosis ensued. Only a four-drug prolonged regimen of treatment proved to be somewhat effective. A 5-year-old girl had a 6-month history of painless swelling and limited mobility of the ankle; radiographs and CT showed osteopenia with marginal erosion of cartilages. A 14-month-old boy presented with a 2-week history of painless swelling ankle. Radiographs showed decreased bone density of talocalcanear bones. Mantoux test and synovial biopsy confirmed TB in both patients; treatment with a three-drug regimen greatly reduced symptoms. A careful suspicion of the diagnosis of tuberculosis is paramount in children with chronic or subacute monoarticular arthritis, even in absence of a positive tuberculin test or abnormalities on chest radiograph. When negative early on, the tuberculin test should be repeated after 6 weeks of arthritis, and a needle biopsy of the synovium is required in those children with monoarticular arthritis and a positive tuberculin test. Careful therapy is necessary to avoid sequelae that may lead to severe osteoarticular damage.

Congenital talipes equinovarus: an epidemiological study in Sicily

Background and purpose Congenital talipes equinovarus (clubfoot) can present in 2 forms: “syndromic”, in which other malformations exist, and the more common “idiopathic” form, where there are no other associated malformations. We analyzed the epidemiology of congenital talipes equinovarus in the Sicilian population, looking for potential etiological factors. Patients and methods Among the 801,324 live births recorded between January 1991 and December 2004, 827 cases were registered (560 males; M/F sex ratio: 2.1). Control infants were randomly selected from a historical cohort of live births without any major congenital malformations. Results A positive family history of clubfoot, gender, and maternal smoking were found to be risk factors for clubfoot. Patients with clubfoot were born most frequently during the period January–March. No association was found between clubfoot and reproductive history, peri-conceptional maternal drug exposure, maternal education, or ethnicity. Interpretation Our findings emphasize the importance of birth defects surveillance programs and their usefulness in investigating potential risk factors.

Autosomal dominant and sporadic radio‐ulnar synostosis

We report on seven cases of congenital radio-ulnar synostosis (RUS). Five were found in the same family and two were sporadic. In six the synostosis was bilateral and consistently involved the proximal end of the radius and ulna. In the familial cases the anomaly was inherited as an autosomal dominant trait and was associated with a Dubois sign and relative shortness of metacarpals number 4 and 5 in two patients, and of number 2 in another patient, and of all phalanges of the 5th fingers. These observations suggest involvement of an ulnar developmental field. RUS does not seem to be rare in the Sicilian population.

Spectrum of skeletal abnormalities in a complex malformation syndrome with "cutis tricolor" (Ruggieri-Happle syndrome).

Background:  The term cutis tricolor describes the combination of congenital hyper‐ and hypopigmented skin lesions in close proximity to each other in a background of normal complexion. This phenomenon has been reported: (i) as a purely cutaneous trait; (ii) as a part of a complex malformation syndrome (Ruggieri‐Happle syndrome ‐ RHS); (iii) as a distinct type [cutis tricolor parvimaculata]; (iv) in association with other (e.g. vascular) skin disturbances.

Primary osteoma cutis--multiple café-au-lait spots and woolly hair anomaly.

An 11-year-old girl with typical features of primary osteoma cutis is reported. She demonstrated multiple areas of subcutaneous, asymptomatic, slow-growing nodules, mostly localized at the extremities. Radiographic evidence of soft tissue calcification and histologic confirmation of ectopic bone formation was obtained. She also showed café-au-lait spots, woolly hair and intrauterine growth deficiency.