Unicameral bone cysts treated by injection of bone marrow or methylprednisolone.

In 79 consecutive patients with unicameral bone cysts we compared the results of aspiration and injection of bone marrow with those of aspiration and injection of steroid. All were treated by the same protocol. The only difference was the substance injected into the cysts. The mean radiological follow-up to detect activity in the cyst was 44 months (12 to 108). Of the 79 patients, 14 received a total of 27 injections of bone marrow and 65 a total of 99 injections of steroid. Repeated injections were required in 57% of patients after bone marrow had been used and in 49% after steroid. No complications were noted in either group. In this series no advantage could be shown for the use of autogenous injection of bone marrow compared with injection of steroid in the management of unicameral bone cysts.

Glucocorticoids decrease interleukin-6 levels and induce mineralization of cultured osteogenic cells from children with fibrous dysplasia.

Fibrous dysplasia (FD) is a progressive bone disease in which abnormal fibroblast proliferation results in the replacement of normal cancellous bone with an immature fibrous tissue that is poorly mineralized. The disease manifests itself in the monostotic form in which only one bone is involved and the polyostotic form in which multiple bones at different sites are affected. The McCune-Albright syndrome is a variation of the polyostotic form in which patients demonstrate a greater extent of bone involvement and a variety of endocrinopathies. Somatic activating mutations in the GNAS gene have been demonstrated in the fibrotic lesions of patients affected with either monostotic or polyostotic FD. The increased cAMP levels caused by the G-protein mutations lead to increased interleukin-6 (IL-6) levels in the affected tissues, resulting in abnormal osteoblast differentiation and increased osteoclastic activity. Utilizing cell culture techniques that have been developed for mammalian bone marrow stromal cells, we have successfully cultured osteogenic stem cells from the affected stroma of 11 FD patients. Cells cultured from patients with polyostotic FD showed a high frequency of the Gsalpha mutation, whereas cells from monostotic FD patients showed a low frequency of the mutation. Both the normal and FD cells displayed the osteogenic phenotype when exposed to medium containing glucocorticoids. Glucocorticoids also caused a dramatic inhibition of IL-6 mRNA and protein levels in osteogenic cells cultured from the FD patients. These findings suggest that chemical alteration of cellular function may lead to new treatment options for patients with FD.

Surgical management of calcaneal unicameral bone cysts.

Unicameral bone cysts are not seen commonly in the calcaneus. Little is known about the etiology and natural history of these lesions. Calcaneal cysts often are symptomatic, although some of these lesions are detected as incidental findings. Treatment has been advocated based on the fear of pathologic fracture and collapse. Several published series have been divided in their favor for either open treatment or injection management. These series are small, and the optimal treatment is still in question. The current study compared the efficacy of methylprednisolone acetate injection treatment with curettage and bone grafting in the treatment of unicameral bone cysts of the calcaneus. All patients treated for unicameral bone cysts of the calcaneus during the past 7 years at two institutions were reviewed. Eleven patients met inclusion criteria. All diagnoses were confirmed radiographically or histologically. Demographic information, presenting complaints, diagnostic imaging, treatment modalities, and outcome were analyzed. Long term radiographic and subjective followup was obtained. Eighteen surgical procedures were performed on 11 patients with 12 cysts. Nine injections performed on six patients failed to show healing of the cyst. Nine cysts treated with curettage and bone grafting showed cyst healing. At mean followup of 28 months (range, 12-77 months), all 11 patients had no symptoms; there were no recurrences of the cyst in the nine patients who underwent bone grafting and persistence of the cyst in the two patients who underwent injection therapy. This review reports one of the largest series of cysts in this location. The results indicate that steroid injection treatment, although useful in other locations, may not be the best option for the management of unicameral bone cysts in the calcaneus. Curettage and bone grafting yielded uniformly good results.

Slipped capital femoral epiphysis associated with radiation therapy.

We reviewed 32 children with 41 radiation-therapy associated slipped capital femoral epiphyses (RTASCFE). Ten were from the authors' institutions and 22 from the literature. Gender distribution was equal. The age at diagnosis of the malignancy was 4.3 +/- 3.1 years; the amount of radiation was 4,240 +/- 1,445 rads. Children with RTASCFE presented younger (10.4 +/- 3.2 years) than a routine SCFE. The average symptom duration was 5 +/- 6 months. Children with RTASCFE are usually thin (median weight, 10th percentile) in contrast to children with typical SCFE, who are usually obese (<95th percentile). The majority (82%) of the slips were mild, compared to routine SCFEs (approximately 50%); 28% were bilateral. There was a positive linear relationship between the age at presentation of the SCFE and the age at diagnosis of the malignancy; there was a negative linear relationship between the age at presentation of the SCFE and the amount of radiation therapy.

Growth arrest resulting from unicameral bone cyst.

Growth arrest complicating unicameral bone cyst of the proximal humerus is reported in five patients. These patients represented 10% of 51 consecutive patients treated by the senior author at our hospital from 1988 through 1995. The youngest patient at first clinical presentation was aged 6 years, and the oldest was 14 years 9 months. The follow-up ranged from 35 to 69 months (average, 57.2). The youngest patient at final follow-up was 9 years 1 month, and the oldest was 20 years 5 months. Pathologic fracture was the common clinical presentation in all patients. Growth arrest was diagnosed by limb-length discrepancies, as well as radiographic evidence of premature closure of the physis and deformity of the upper humerus. Treatment was either aspiration of the cyst and local injection of corticosteroids or curettage and bone grafting. Growth arrest was documented, both clinically and radiographically, before the surgery in the two cases treated by curettage and grafting. The origin of growth arrest resulting from unicameral bone cyst remains uncertain. Direct iatrogenic damage to the physis was not a likely cause of growth arrest in this series. Growth arrest as a complication of unicameral bone cyst of the proximal humerus is more common than is generally appreciated (10%).

A review of Morquio syndrome.

Morquio syndrome is one of the mucopolysaccharidoses. Glycosaminoglycans accumulate within the cells, leading to many systemic alterations. Alterations in connective tissue and cartilage ground substance result in abnormal formation and growth of the skeletal system. The major orthopedic manifestations include shortening of the trunk and limbs, spinal curvature, odontoid hypoplasia with upper cervical instability, and lower-limb alignment problems. The management of the orthopedic manifestations of this syndrome requires a broader knowledge of the genetic, metabolic, and systemic effects of the metabolic alterations.

Measurement of acetabular index intraobserver and interobserver variation.

Pelvic radiographs of 25 children aged 6 months to 2 years had the acetabular index measured 3 times by each of five pediatric orthopaedists. Interobserver measurements were found to vary +/-3.0 degrees, whereas the intraobserver variation was +/-3.6 degrees. This error reflects only measurement error and does not consider error introduced with different positioning of the pelvis.

Fibrous dysplasia.

Fibrous dysplasia provides the orthopedic surgeon with tremendous reconstructive challenges. The manifestations of the condition are very diverse and often involve organ systems not routinely cared for by the orthopedic surgeon. We present a concise review of the radiography, histology, clinical manifestations, and complications of this condition, with the main focus on the non-orthopedic management challenges.

Function of the upper extremities in hereditary multiple exostoses.

The radiographic anatomy, functional status, and impairment ratings of twenty-eight patients (fifty-six extremities) who had hereditary multiple exostoses were evaluated. The patient (or the parent) also subjectively graded the function of each extremity with use of a standard rating-assessment tool. Degenerative joint disease was evident in three (5 per cent) of the fifty-six extremities at the time of follow-up, when the patients were an average of twenty-one years old. With use of the hand test of Jebsen et al., the average score was in the forty-seventh percentile for the dominant extremity and in the twenty-eighth percentile for the non-dominant extremity. Loss of pronation and supination increased with increasing age. Dislocation of the radial head was significantly associated with negative ulnar variance (p=0.008) and with the impairment rating (p=0.001), but not with the subjective score or with the performance on the hand test of Jebsen et al. So-called whole-person impairment ratings ranged from 0 to 17 per cent (average, 5 per cent). It has been our experience that deformities of the upper extremity in patients who have hereditary multiple exostoses are well tolerated and lead to little loss of function as measured both subjectively and objectively.

A 13-month-old boy with progressive genu valgum.

The following case illustrates the roentgenographic and clinical findings of a condition of interest to the orthopedic surgeon. Initial history, physical findings, and roentgenographic examinations are indicated below. The final clinical and differential diagnoses are presented on the following pages.

Orthopaedic manifestations in de Barsy syndrome.

de Barsy syndrome is a rare, genetically transmitted condition characterized by severe cutis laxa, joint hypermobility, growth retardation, mental retardation, and characteristic facies. Affected individuals have many orthopaedic manifestations, including developmental dysplasia of the hip, scoliosis, multiple joint dislocations and subluxations, and congenital vertical talus. The management of the orthopaedic manifestations of this syndrome is presented in two typical cases.

Closure of the physis after pinning of slipped capital femoral epiphysis.

A study was conducted to determine what effect pinning in situ for slipped capital femoral epiphysis (SCFE) had on the timing of subsequent closure of the growth plate. Eighty-nine patients with a SCFE were reviewed. Twenty-six patients with a unilateral SCFE were treated with closed pinning in situ; no manipulative reductions were performed. The timing of physeal closure was assessed using postoperative radiographs of both pinned and non-pinned sides. The average time until physeal closure was 12.0 months for the pinned physis and 22.2 months for the non-pinned physis. In two cases, the physes closed simultaneously, and in no patient did the non-pinned physis close earlier than the pinned physis. The pinned physes closed an average of 10.2 months (range: 0 to 22) earlier than the unpinned physes. No difference was noted in the rates of closure with different types of internal fixation. This study supports the theory that pinning SCFE in situ causes early closure of the physis.

Reflex sympathetic dystrophy in children: an orthopedic perspective.

To design diagnostic criteria for reflex sympathetic dystrophy (RSD) and to initiate a prospective treatment protocol, we reviewed our experience with 49 episodes of RSD in 36 children. There were 24 females and 12 males; mean age at diagnosis was 13.4 years (range: 8 to 19); mean time from pain onset to correct diagnosis was 9.2 months (range: 1 to 53). Lower extremity involvement predominated. Pain was "severe" in 61%, and skin color changes, swelling, hyperesthesia, abnormal skin temperatures, muscle weakness, and decreased range of motion were all present in at least 75% of cases. Osteopenia was observed in 15 of 38 radiographs; of 24 bone scans, 7 were normal, 11 showed increased uptake, and 6 demonstrated decreased uptake. Of the 23 children who had psychological evaluations, 83% revealed some type of significant emotional dysfunction. Analgesic and antiinflammatory medications were not helpful, nor were local injections or regional blockades effective. An inpatient diagnostic and rehabilitation program for treating chronic pain, including orthopedics, rheumatology, psychology, and twice-daily physical therapy was most likely to lead to resumption of age-appropriate activities. Despite extensive physiological testing, physician, parent, and/or patient reluctance to accept absence of a primary organic disease was common. We present diagnostic criteria for pediatric RSD.

Chronic recurrent multifocal osteomyelitis.

Chronic recurrent multifocal osteomyelitis is a rare entity that is easily confused with pyogenic osteomyelitis. Three cases that illustrate the common modes of presentation and roentgenographic findings are presented. The current orthopaedic literature is reviewed, and management guidelines are discussed.

Acute arthritis of the hip in a child infected with the Lyme spirochete.

Acute Lyme arthritis may mimic acute pyogenic arthritis. Although the arthritis associated with infection with the spirochete, Borrelia burgdorferi, is more commonly seen in the chronic stage (Stage III) of the disease, occasionally it may present as the initial clinical manifestation. A five-year-old girl with acute arthritis of the hip is reported to discuss classification and management of arthritis associated with Lyme disease.

Computed tomography for early evaluation of developmental dysplasia of the hip.

Computed tomography (CT) provides an axial image of the dysplastic hip. We reviewed 130 CT scans performed after closed reduction for developmental dysplasia of the hip (DDH) in 42 patients (52 hips). We evaluated methods of measuring CT scans and found no described measurement that was predictive of development of avascular necrosis (AVN) or persistent acetabular dysplasia. The CT scan provides an excellent image of the infant hip when the child is in a spica cast and is useful to confirm an adequate reduction. No patient in this series lost reduction after 2 weeks.

Reflex sympathetic dystrophy in children. A physical therapy approach.

Children with reflex sympathetic dystrophy (RSD) almost always receive physical therapy as part of a multidisciplinary approach, but there is controversy about the efficacy of many alternative modalities. In a retrospective chart review of 24 females and 12 males with 49 episodes of RSD (mean age at onset, 13.4 years), the average time to correct diagnosis was 9.4 months (median, 4.2 months; range, 1-53 months). Sixteen ankles, 12 knees, eight wrists, two hips, and two shoulders were involved. Psychological assessments revealed significant abnormalities in 25 (83%) of 30 children evaluated. Thirty-four (94%) of 36 children received physical therapy including a wide variety of nonstandardized approaches. Children with one to two episodes of RSD averaged 4.0 physical therapy modalities; unresolved cases had 8.9 modalities attempted. Time from the first RSD episode to resolution averaged 9.0 months in 69% of children. Incorrect diagnoses prolonged many initial episodes; following correct diagnosis, symptom resolution occurred in 3.1 months. Recurrences are common, and 25% of children still exhibited RSD symptoms at last follow-up.