Ernesto Ippolito

Treatment approaches for congenital pseudarthrosis of tibia: results of the EPOS multicenter study. European Paediatric Orthopaedic Society (EPOS).

&NA; This study was designed to analyze the different therapeutic methods used by European Paediatric Orthopaedic Society members from 13 countries for congenital pseudarthrosis of tibia. The treatment data of 340 patients who underwent 1287 procedures for this condition were analyzed. The essential findings were that the method of choice needed to approach the biological problem with the aims of: (1) resecting the pseudarthrosis to provide stability, the basic requirement for bony consolidation; (2) correcting length discrepancy and axial deformity; (3) achieving fusion; and (4) solving the additional problems around the main deformity such as alignment, leg length discrepancy and ankle valgus. The Ilizarov technique emerged as being the optimal method, having the highest rate of fusion (75.5%) of pseudarthrosis and rate of success in correction of the additional deformities. There was also consensus that surgery should be avoided before the third year of life.

Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data.

&NA; Congenital pseudarthrosis of the tibia is a rare disease with a variable history. The pseudarthrosis is usually not present at birth (and therefore is not truly congenital) but occurs during the first decade of life. Paget in 1891 was the first to describe a case. The etiology is unknown. Neurofibromatosis plays a role in approximately 50% of patients. In the others, hereditary and mechanical factors are debated. Various (morphologic) classification systems have been proposed (Andersen, Boyd, Crawford). Because the appearance changes during the course of the disease, all classification systems have limited value: the determining factor is the stage of the disease at which it was classified. Because of the rarity of the disease and the variability of its history, the European Pediatric Orthopaedic Society decided to carry out a multicenter study on this disease. This paper presents the epidemiologic data on the patients involved. Data have been gathered on 340 patients from 13 countries. Two hundred patients were male (58.8%), 140 were female (41.2%). The right side was affected in 165 patients (48.5%) and the left side in 172 (50.6%); 3 patients had bilateral disease. Symptoms of neurofibromatosis were present in 54.7%. Histologic examinations in 192 patients showed a nonspecific appearance in 45.3%; in 15.6% the ultrastructure resembled fibrous dysplasia, and in 39% there was histologic evidence of neurofibromatosis. Most of the lesions were initially localized in the middle or distal third of the tibia. In 29% the localization changed during the course of the disease.

Pathology of bone lesions associated with congenital pseudarthrosis of the leg.

&NA; Congenital pseudarthrosis of the leg remains one of the most controversial pediatric entities in terms of etiopathogenesis, pathology, treatment, and prognosis. The authors reviewed the pathologic material of 24 patients with congenital pseudarthrosis of the leg along with clinical and radiographic data. The tibia was affected in 22 patients; in two patients the disease was limited to the fibula. Fifteen patients were male and nine were female. Age at surgery ranged from 1 to 26 years. Nineteen patients were classified as having dysplastic type, one cystic, and four mixed. Clinical evidence of neurofibromatosis type I (NF‐I) was found in 17 patients. The main histopathologic change observed was the growth of a highly cellular, fibromatosis‐like tissue. In the dysplastic type, such tissue was associated with the periosteum. In the cystic type, a closely similar tissue occupied the lytic area. In cases classified as of mixed type, the coexistence of endosteal/medullary and periosteal involvement by the fibromatosis‐like tissue was observed. In the cystic lesion, evidence of de novo bone formation within the lesional tissue was obvious. Overall, the histologic features of the cystic lesion were similar to those of osteofibrous dysplasia. In the dysplastic type, the proliferation of the fibrovascular tissue was associated with active osteoclastic resorption of the cortex, which remodeled into a trabecular rather than a compact type of structure. Histologic comparison of the pathologic samples of patients with and without NF‐1 revealed no significant differences. The pseudarthrosis gap was continuous with periosteal soft tissues and filled by fibrous tissue, fibrocartilage, and hyaline cartilage with features of enchondral ossification. The authors suggest that the clinical diversity of congenital pseudarthrosis of the leg results from the diverse location of a single pathologic process—namely the growth of an abnormal, fibromatosis‐like tissue either within the periosteum or within the endosteal/marrow tissues. It is tempting to suggest that such an “osteofibromatosis” represent a skeletal expression of neurofibromatosis, either within the fully expressed syndrome (patients with known neurofibromatosis) or as isolated lesion (patients with unknown/cryptic neurofibromatosis).

Physeal Fractures of the Distal Radius and Ulna: Long-term Prognosis

OBJECTIVES The long-term prognosis of injuries to the distal physis of forearm bones, including complications such as radioulnar length discrepancy and styloid nonunion, has not been extensively studied. Reliable radiographic prognostic criteria to predict physeal disturbance at trauma are also lacking. The aim of this study is to investigate both issues. DESIGN Retrospective study. SETTING University hospital. PATIENTS/PARTICIPANTS One hundred sixty-three lesions to the distal physis of the forearm bones in 157 patients were available for a long-term follow-up. Seventy-seven injuries of the distal radial physis were radiographically isolated, 54 were associated with a fracture of the ulnar styloid, and 26 with a fracture of the distal ulnar metaphysis. Of the six injuries of the distal ulnar physis, five were associated with a fracture of the distal radial metaphysis, and one was an isolated injury of the distal ulnar physis. INTERVENTION Treatment consisted of wrist immobilization in a long-arm plaster cast for 6 weeks. Dorsal or volar displacement was reduced using general anesthesia. MAIN OUTCOME MEASUREMENTS All patients had both clinical and radiographic evaluation, with an average follow-up of 25.5 years (range 14-46 years). The average age of the patients at injury was 11.6 years (range 5-17 years), whereas their average age at follow-up was 35.5 years (range 22-56 years). Both the Salter and Harris and the Ogden classifications were used to classify physeal injuries. RESULTS According to Salter and Harris, of the 157 radial lesions, 18 were type 1 and 139 type 2. According to Ogden, 14 were type 1A, 4 type 1C, 84 type 2A, 13 type 2B, 17 type 2C, and 25 type 2D. Of the 6 ulnar lesions, 2 were Salter and Harris type 1 (Ogden type 1A), 3 type 2 (Ogden type 2A), and 1 type 4 (Ogden type 4A). Fifty-four radiographically evident fractures of the ulnar styloid associated with injuries of the distal radial physis were classified as Ogden type 7A. At follow-up, all of our patients were fully asymptomatic, except for those who had forearm bone growth failure of more than 1 cm. Shortening of the previously injured forearm bones ranging from 1 to 6.5 cm was observed in 2 open and subsequently infected lesions as well as in 5 uncomplicated lesions of the 157 distal radial physeal injuries (4.4%), and in 3 of the 6 distal ulnar physeal injuries (50%). Shortening of 1 cm or more was observed in the uncomplicated lesions of radial physeal injury with Ogden type 1C, 2B, and 2D lesions, and in ulnar physeal injuries Ogden type 1A, 2A, and 4A. Thirty-eight additional patients had radioulnar length discrepancy that ranged from 2 to 9 mm, and 53 patients had styloid nonunion, but all of them were asymptomatic. CONCLUSIONS None of the patients reviewed at follow-up, including those with radioulnar length discrepancy of less than 1 cm and those with styloid nonunion, complained of any symptom related to their previous injury, not even those engaged in heavy manual labor. Of the 10 patients with either radial or ulnar shortening of more than 1 cm, only 2 with radial growth arrest and marked radioulnar length discrepancy had severe functional problems. Growth disturbances of more than 1 cm following distal radial physeal injury occurred only in Ogden type 1C, 2B, and 2D lesions, whereas in distal ulnar physeal injuries, growth disturbances occurred regardless of the Ogden classification type.

Free vascular fibular transfer in congenital pseudoarthrosis of the tibia: results of the EPOS multicenter study. European Paediatric Orthopaedic Society (EPOS).

&NA; This paper presents a review of the literature, describes the principal authors (B.R.) personal experience and provides the results of the European Paediatric Orthopaedic Society (EPOS) multicenter study. The objective is to evaluate the present status and future role of free vascular fibular transfer in treating congenital pseudoarthrosis of the tibia. Variables such as the selection of cases, age at operation, technical surgical details and postoperative results will be considered. The data on the EPOS study were incomplete at the time of writing, but the considerable amount of information already amassed is a valuable contribution to this updated report.

The surgical management of fibrous dysplasia of bone

The surgical management of Polyostotic Fibrous Dysplasia (FD) of bone is technically demanding. The most effective methods to manage the associated bone deformity remain unclear. The marked variation in the degree and pattern of bone involvement has made it difficult to acquire data to guide the surgeon’s approach to these patients. In light of the paucity of data, but need for guidance, recognized experts in the management of these patients came together at the National Institutes of Health in Bethesda, Maryland as part of an International meeting to address issues related to fibrous dysplasia of bone to discuss and refine their recommendations regarding the surgical indications and preferred methods for the management of these challenging patients. The specific challenges, recommended approaches, and “lessons learned” are presented in hopes that surgeons faced with typical deformities can be guided in the surgical reconstruction of both children and adults with FD.

The fibula in congenital pseudoarthrosis of the tibia: the EPOS multicenter study. European Paediatric Orthopaedic Society (EPOS).

&NA; Fibular involvement in congenital pseudoarthrosis of the tibia (CPT) can be either a rare isolated pathology or is in association with the tibial changes. Out of 282 patients with CPT who had complete radiographic work‐up, 62% (almost two thirds) demonstrated fibular pathology: 36% had true fibular pseudoarthrosis and the rest, i.e., 26% had fibular hypoplasia or dysplastic fibula. Neurofibromatosis was found in 62% of the patients with fibular pathology. The typical radiological features of tibial pseudoarthrosis are often missing in patients with fibular pathology. It is most probably because fibular changes precede the tibial involvement. In 250 patients with tibial surgical treatment, the highest rate of fusion occurred in patients with fibular involvement compared with those with normal fibula.

Primary subtalar arthrodesis for the treatment of comminuted intra-articular calcaneal fractures

We report the short- and mid-term results in six patients (seven feet) affected by markedly comminuted intra-articular calcaneal fractures (Sanders type IV), treated by primary subtalar arthrodesis. The average age at surgery was 40 years. In all patients, arthrodesis of the subtalar joint was performed using a limited lateral approach to the calcaneus; it was stabilised with two or three cannulated screws. No patient had a preliminary reduction and internal fixation of the fracture. The time from injury to surgery averaged 20 days because all of the patients had associated visceral and/or other skeletal injuries. All of the patients were followed up clinically and radiographically 2 times, at an average of 12 months and 53 months after surgery. At the short-term follow-up, the mean AOFAS score was 70 points; the X-rays showed a complete fusion of the subtalar joint in all seven feet, without any sign of osteoarthritis of the calcaneo-cuboid and the talo-navicular joints. In all cases, an altered shape of the calcaneus was present. At the mid-term follow-up, the mean AOFAS score increased to 85 points; in one patient, radiographic signs of osteoarthritis of the calcaneo-cuboid and the talo-navicular joints were present and, in another patient, only talo-navicular joint was present, although both patients were free from pain. The difference between the two AOFAS scores was statistically significant. We believe that primary subtalar arthrodesis performed for markedly comminuted Sanders type IV calcaneal fractures yielded good mid-term results, and it is especially indicated when surgical treatment is delayed for whatever reason. A preliminary open reduction and internal fixation to restore the normal height of the calcaneus before performing the subtalar arthrodesis, as suggested by several authors, does not seem indispensable to obtain good clinical results.

Epiphyseal chondroblastoma of bone. Long-term effects on skeletal growth and articular function in 15 cases treated surgically.

SummaryThe authors describe the long-term sequelae of chondroblastoma in 15 patients with open growth plates, whose age at operation ranged from 8 to 15 years. At follow-up, the youngest patient was 21 and the oldest 48 years old. Upper limb length discrepancy ranging from 2 cm to 10 cm was present in the four patients who had a proximal humeral epiphyseal location; in three of them, the range of motion of the shoulder was also limited and X-rays showed marked irregularities of the humeral head. Lower limb length discrepancy ranging from 0.5 cm to 2 cm was found in five of the eight patients in whom the tumor affected lower limb epiphyses. One patient with proximal tibial epiphyseal involvement also had mild genu valgum. Radiographic osteoarthritis was present only in the trapeziometacarpal joint of a patient in whom the first metacarpal bone affected by the tumor was replaced by a free fibular graft. The abnormalities observed did not cause important functional loss in either the everyday or the working activities of any of our patients.

ChevronOsteotomy with Lateral Release and Adductor Tenotomy For Hallux Valgus

Background: Distal chevron osteotomy is a procedure widely performed for the surgical treatment of painful hallux valgus. The risks and benefits of a lateral capsular release and adductor tenotomy combined with chevron osteotomy are still debated. The aim of our study was to report the clinical and radiographic outcomes of this combined procedure in mild and moderate incongruent bunion deformities, with a hallux valgus angle (HVA) up to 40 degrees and an intermetatarsal angle (IMA) up to 20 degrees. Materials and Methods: Forty-two patients (52 feet) who consecutively underwent chevron osteotomy combined with lateral release and adductor tenotomy were reviewed 24-36 months after surgery. The mean age of the patients was 53.5 (range, 43 to 64) years. All the deformities were mild to moderate, with a mean preoperative value of 28 degrees in the HVA (range, 16 degrees to 40 degrees) and of 13 degrees in the IMA (range, 9 degrees to 20 degrees). Results: At followup, the AOFAS hallux score improved from an average of 46 to an average of 88. The HVA and IMA had an average postoperative decrease respectively of 12 degrees and 6 degrees; lateral sesamoid displacement decreased by a mean of 15%. In no case did we observe infection or nonunion of the osteotomy. In one case, painless avascular necrosis of the first metatarsal head developed. Conclusion: Our short-term results show that distal chevron osteotomy combined with lateral release and adductor tenotomy is a feasible surgical option to address mild to moderate hallux valgus deformity, even with an IM angle between 15 and 20 degrees. Clinical and radiographic outcomes are generally good and patient satisfaction is generally high.