Biomedical subjects
Publications and source records attributed to G Bollini.
We report three cases of chronic osteoarticular infections associated with Q fever in children. In two of them, serologic diagnosis was confirmed by direct immunofluorescence of Coxiella burnetii in the synovial and bone biopsy specimen. We suspect that bone infections due to Coxiella burnetii could be underdiagnosed because this etiology is not frequently seen.
The clinical features of two children with myositis ossificans progressiva are described. Skeletal malformations can be observed in many sites: hand, femur, tibia and spine. Phalangeal abnormalities (shortened hallux, hallux valgus) are essential to the diagnosis. We recommend systematic roentgenographic examination to search for other skeletal malformations for congenital hallux valgus in young children because it can be the first sign of myositis ossificans progressiva. Progression of disability does not seem to be influenced by any form of medical treatment. Surgical removal of ectopic bone is thought to be followed inevitably by rapid recalcification at the original site.
MATERIAL AND METHOD: Acetabular anteversion angle (AAA) and orientation angle of the iliac bone (AOOI) determined by use of a CT scan were studied in CDH. 55 children with CDH were selected on hip arthrography for this study. Subluxated hips were excluded (i.e. opposite hip of a unilateral CDH is normal). 10 boys and 45 girls with a mean of age of 2 years 1 month (extremes from 1 to 4 years 3 months) were studied. CT scan was performed before any orthopaedic treatment in 3 cases of bilateral luxation and 14 cases of unilateral. In the other cases, time between the end of orthopaedic treatment and CT scan varied between 4 and 18 months. A group of 23 normal children, 10 boys and 13 girls, (mean age of 2 years 10 months) served as reference group. On the selected CT slide we measured AAA, AOOI, IAA and IAP (anterior acetabular index and posterior acetabular index as proposed by Guggenheim). RESULTS: We noted that the orientation of the iliac bone was variable in the two groups. This orientation angle could have higher or lower values. AAA: in bilateral luxation, this angle was higher (16 degrees +/- 5 degrees) than in reference group (13 degrees +/- 4 degrees), p < 0.005. In unilateral luxation there was no statistical difference (14 degrees +/- 4 degrees) with reference group, between normal and pathological side and when CT scan was performed before or after orthopaedic reduction. AOOI: there was no significant difference between bilateral, unilateral or reference group. Correlation analysis showed that AAA and AOOI moved in the same direction. IAA: in bilateral luxation this index was higher (p < 0.001); in unilateral luxation only right luxation showed an higher index (p = 0.002). IAP: no significant difference between the different groups. DISCUSSION: This study shows that there is any typical CT scan aspects of morphologic abnormality in CDH. The lesions of the anterior or posterior acetabular wedge are variable. The orientation of the iliac bone is also variable; we concluded that acetabular anteversion must be analysed depending on the morphologic aspects of the anterior and posterior extremities of the acetabulum and iliac bone orientation.
Twenty-two patients with metaphyseal primary malignant bone tumors (17 osteosarcomas, 5 Ewing's tumors) occurring before closure of the growth plate were examined with plain radiographs and MRI in order to determine the physeal or epiphyseal extent of the tumor. Results were correlated with the pathologic examination. Transphyseal spread was pathologically proven in 13 cases (59%): 12 cases of osteosarcoma and 1 case of Ewing's tumor (70% and 20%, respectively). There was no significant relation between epiphyseal invasion, age of patient, length of tumor or, in the cases of osteosarcoma, response to chemotherapy. Plain radiographs showed epiphyseal involvement in 4 cases and there were 10 false negatives. MRI revealed epiphyseal involvement in all cases; there were no false positives or false negatives. T1-weighted images in coronal or sagittal planes appeared to be sufficient. These findings are very useful in planning surgical limb salvage procedures and stress the ineffectiveness of the "barrier effect" of the growth plate against tumor spread.
The authors report a series of 19 children (ten boys and nine girls) presenting fibrous dysplasia with an average follow-up of 7 years 10 months. Twenty five bones were involved. The average age at diagnosis was 9 years (range 3 years to 14 years). Fatigue fractures and pains were the main reasons for consultation. The most frequent anatomic sites were the femur, the tibia and the humerus. In six cases it was decided to simply watch the children without any treatment. The clinical results were good for 18 children. In two cases the initial radiological lytic aspect remained at the longest follow-up. Nine complications (among which two iterative fractures and three recurrences) occurred. None of the cases developed malignant transformation. The authors discuss the different possibilities of treatment with a review of the literature.
The authors have analysed a series of aneurysmal bone cysts (A.B.C.) in children and adolescents which were reviewed at an average follow-up of 4 years 2 months (6 months-15 years). The average age at diagnosis was 8 years (3-19 years). Pathological fractures were the main reason for consultation, probably due to the predominance of central lesions. If conventional radiology remains indispensable to diagnosis, magnetic resonance imaging is nevertheless the most important examination in determining the extent of the affection. The diagnosis remains anatomopathological, even if this may be difficult due to associated lesions (A.B.C. illness and A.B.C. symptoms). In long bones lesions, we observed recurrence after curettage in 3 out of 7 cases. For this reason the recommend simple resection or reconstructive resection rather than curettage whenever possible. 5 lesions in contact with growth plate were observed. In such cases we recommend its conservation by careful curettage, especially in young children. We feel that subsequent recurrence is easier to treat than an epiphysiodesis bridge. The surgical techniques employed to conserve the plate are described along with methods of bone reconstruction after surgery.
This retrospective review included 133 patients with one or several of the following defects: diastematomyelia, neurenteric cyst, dermal sinus or cyst, meningeal malformation, sacral agenesis, tethering of the spinal cord, and lumbosacral lipoma. Physical evaluation readily identified most lumbosacral lipomas, as well as most cases of diastematomyelia since hypertrichosis over the defect was common. Some patients developed life-threatening clinical manifestations, e.g., meningitis due to a dermal sinus. On the basis of this retrospective review and a review of the literature, the clinical and therapeutic aspects of each defect are discussed.
The diagnosis of Ewing's malignant tumor in the young still raises major problems, either from a clinical point of view because of its rarity, its pluri-potentiality and various symptoms, or on imaging because of its numerous pitfalls. Accordingly the disease is often misdiagnosed as osteomyelitis. Only a high quality biopsy can determine histological diagnosis of undifferentiated small round cell tumor. The chromosomic study shows a specific (11;22)(q24;q12) translocation, and immunocytochemistry and molecular biology show the tumor's neuroectodermal origin. For 20 years, therapy tended toward first line tumor chemoreduction, followed, when appropriate, by complete resection and orthopedic reconstruction of the bone. Radiotherapy, which is responsible for long-term sequelae is now increasingly restricted to inaccessible or incompletely excised tumors. More intensive chemotherapy is being examined in patients with poor prognosis factors such as a negative response to induction chemotherapy, a significant mass or metastases. As a result of new strategies, disease-free survival rate is now between 60-70%. The management of this disease is highly multidisciplinary and patients will now be included in multicentric controlled therapeutic trials. Long term follow-up has to be carried out following completion of treatment.
We present two cases of vitamin D-dependent rickets type II, a rare disease caused by a hereditary disorder of the receptor for 1,25 dihydroxyvitamin D (1,25 (OH) 2 D). The first patient was seen for the first time at the age of 2 years with florid rickets, almost complete alopecia, and an elevated plasma level of 1,25 (OH) 2 D; there was no improvement following treatment with 1 alpha-hydroxy-vitamin D3). The second patient, a boy whose parents are first cousins, was seen at the age of 7 years with severe rickets, partial alopecia and an elevated serum level of 1,25 (OH) 2 D; the rickets disappeared after administering high doses of 1 alpha hydroxyvitamin D3 and vitamin D.
The authors reviewed a series of 72 hips in 60 children with sequelae of septic arthritis of the hip to evaluate the long term evolution. The average follow up was 7 years 8 months (Range 1Y-17Y). 40 hips were operated and 32 were not. The results were analysed according to clinical and radiological criterions in an attempt to propose a guide for treatment. Sequelae might also occur after both initial treatment with open drainage or aspiration. The study of sequelae according to the factors of severity (organism, age when infected) showed that the evolution of these cases of arthritis cannot be forecast. The authors noticed that very severe roentgenographic lesions could be well tolerated clinically, at least on a medium term basis. They advise great care before operating on dislocated hips when they are clinically well tolerated. Finally the authors insist on 2 little known lesions: caput valgum and caput antetorsum.
Forty-six hips treated by a shelf operation in childhood and adolescence have been reviewed. The mean follow-up was 8 years 1 month (4Y-26Y) after operation. The indications were residual dysplasia of the acetabulum, neurologic hips and sequelae of osteochondritis. All patients were operated after the age of 8.5 years except 2. In poliomyelitis and sequelae of osteochondritis the results were good. In dysplasia we had two bad results in 18 cases (1 painful hip after operation and 1 ankylosing hip) and in cerebral palsy 4 bad results in 13 cases, due to difficulty of maintaining a correct reduction for a long period. After a review of the literature to compare the results of this intervention with pelvic osteotomies we conclude that the shelf procedure is beneficial in childhood and adolescence, due to the quality of acetabular cover, to its improvement in time and to ease of intervention.
CT-scan examinations have been performed in 25 children with slipped capital femoral epiphysis (SCFE) and in 127 children in an out-patients orthopaedic consultation. A low femoral anteversion (FA) was present in SCFE. This morphological abnormality, in association with other factors, could explain the production of SCFE at the moment of the most important fragility of the upper femoral growth plate, which is puberty. A statistical analysis showed that the risk of SCFE was distinctly greater, even for the contralateral hip when FA was low.
The authors have made a review of the literature about two cases of osteomyelitis of the iliac ala. They insist on rarity of the disease and how often the right diagnosis may be delayed because of misleading clinical symptoms. Any tumefaction of the buttock associated with pelvic pain and with an infectious syndrome necessarily evoke an iliac osteomyelitis. The bone scan may be negative which need not exclude the possibility. The evolution may be the formation of intra or extra or sometimes simultaneous sub-periosteal abscesses which must be drained. This is the reason why the authors advise to verify systematically the other side of the ilium by its surgical trepanning in case of a sub-periosteal abscess.
Fifteen pediatric cases of vertebral involvement in histiocytosis X are reported. The study of these cases and a review of the literature shows that every patient with histiocytosis X should have a local and systemic staging work-up to differentiate isolated eosinophilic granulomas of the bone, multiple eosinophilic granulomas of the bone, and visceral disseminated disease. Management varies according to the extension of the disease. Systemic chemotherapy may be indicated in patients with extraosseous lesions. Local investigations should be performed to look for instability of the spine, which may require surgery, and above all for spread to the soft parts indicating chemotherapy with or without surgery. Forms which are strictly confined to the bone and cause no instability, such as vertebra plana, require no treatment.
The MR aspects of spondylodiscitis in pediatric patients were studied with a 0.5-Tesla supraconductive magnet: 12 patients were divided in two groups; acute disease (9) and period of sequelae (3). The first group was divided into two subgroups according to the age and the clinical findings: infants (2) and children (7). In infants, MR gave good visualization of the destruction of vertebrae and discs. Presuppurative abscesses appeared as light signals, their extent and position relative to the cord being clearly visualized in frontal and sagittal planes in T1-weighted images. In older children, there were a low vertebral signal and disappearance of the disc-vertebra borders on T1-weighted images and a high vertebral signal with a decreased and flattened disc signal on T2-weighted images. Frontal and sagittal planes were used. During the first month of antibiotic therapy, the follow-up scans showed no changes, and even disclosed a spread of the abnormal signal in the vertebral body. At the stage of sequelae, on T1 the spinal signal was normal with a decreased disc width and on T2 the disc signal was low while the vertebrae appeared normal.
The authors report two rare cases of pygomelus monster. The appearance and operative procedures undertaken are described. Pygomelus and caudal duplication are often mixed in literature. This monstruosity is classified in the twin monsters, but minor forms any be discussed with sacro-coccygeal teratomas and reduplication of lower limbs.
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Although the Chiari osteotomy is usually effective in reducing pain, many patients are left with a long-term limp. The postoperative limp can at times be caused by hip abductors that have strength insufficient to counteract the torque from body weight during single-leg stance. To study how the surgical technique affects the hip abductor muscles, a biomechanical model was developed that computes the postsurgery pelvic geometry and the resulting hip abductor torque given three surgical parameters: angulation of the osteotomy, distance of medical displacement, and angle of internal rotation. The computer simulations of the Chiari osteotomy showed that some sets of surgical parameters conserve abductor torque while others greatly reduce it. Simulated surgeries with high angulation and large medial displacement reduce gluteus medius abductor torque by up to 65%. Therefore, this combination of surgical parameters may account for some instances of the postoperative limp. In the model, high angulation reduces the length of the gluteus medius and is the primary cause of reduced abductor strength. Simulated horizontal osteotomies (0 degrees to 10 degrees) were found to best conserve both muscle length and abductor torque.