Daisuke Kobayashi

Musculoskeletal conditions of acute leukemia and malignant lymphoma in children.

We retrospectively reviewed 16 patients who presented to hospitals with orthopaedic complaints. Twelve patients experienced initial symptoms in the extremities and four patients had back pain. The leukocyte count was elevated in one patient, decreased in two patients, and normal in 13 patients. On radiographic examination, osteopenia was observed in 10 patients, osteolytic lesions were observed in five, and pathologic fractures were observed in five. Because the initial presentation of patients with leukemia often involves the musculoskeletal system, orthopaedists need to recognize the symptoms of this disease to avoid misdiagnosis and to expedite the initiation of appropriate potentially lifesaving treatment.

Acetabular development in the contralateral hip in patients with unilateral developmental dysplasia of the hip.

BACKGROUNDnAdult patients may present with acetabular dysplasia without a history of developmental dysplasia of the hip. The purpose of the present study was to clarify the development of primary acetabular dysplasia in patients under the age of eighteen years by evaluating the contralateral hip in those with unilateral developmental dysplasia of the hip.nnnMETHODSnRadiographs of the contralateral hip of eighty-eight patients with unilateral developmental dysplasia of the hip were reviewed retrospectively. The center-edge angle was measured at the age of eighteen years. The primary acetabular dysplasia group included hips with a center-edge angle of <20 degrees , and the normal group included hips with an angle of > or =20 degrees . The acetabular index at the age of three years, the center-edge angle between the ages of three and eighteen years, and the acetabular angle of Sharp between the ages of six and eighteen years were measured.nnnRESULTSnAccording to our classification system, twelve hips (13.6%) were assigned to the primary acetabular dysplasia group. At the age of three years, there were no significant differences between the two groups radiographically. A significant difference in the center-edge angle between the two groups was seen at each evaluation period after the age of six years. However, twenty-two patients in the normal group had poor acetabular coverage and three patients in the primary acetabular dysplasia group had good acetabular coverage at the age of nine years. After the age of nine years, improvements in the center-edge angle and the acetabular angle of Sharp were noted in the normal group, whereas no acetabular growth was seen in the primary acetabular dysplasia group. There was no patient with a center-edge angle of <15 degrees at the age of twelve years in the normal group.nnnCONCLUSIONSnAfter the age of six years, a difference in acetabular growth develops between patients with primary acetabular dysplasia and those with normal hips. However a final prognosis for acetabular development appears to be difficult to determine until the age of twelve years.

Standardizing radiation dose reporting in the pediatric cardiac catheterization laboratory-a multicenter study by the CCISC (Congenital Cardiovascular Interventional Study Consortium).

We examine normalized air Kerma area product (PKA) by body weight (PKA/BW) as a reference value of radiation dose and benchmark PKA/BW in pediatric laboratories using a multicenter registry database.

The Pavlik harness in the treatment of developmentally dislocated hips: results of Japanese multicenter studies in 1994 and 2008

BackgroundIt has already been more than 50xa0years since the Pavlik harness was introduced in Japan, and today the Pavlik harness is widely recognized as the standard initial treatment modality for developmental dysplasia of the hip. We performed a multicenter nationwide questionnaire study concerning the results of Pavlik harness treatment twice in 1994 and 2008.MethodsIn 1994 and in 2008, we sent questionnaires to 12 institutes in Japan specializing mainly in pediatric orthopedics. We compare the results of these two studies and discuss differences in reduction rates, incidence of avascular necrosis in the femoral epiphysis and the percentage of joints with acceptable morphology (Severin grade Ixa0+xa0II/total) at skeletal maturity. We statistically assessed these results to see whether there were changes in the treatment outcomes over this 14-year period.ResultsReduction of the dislocated hips was obtained by the Pavlik harness in 80.2xa0% (1990/2481 hips; 1994) and 81.9xa0% (1248/1523 hips; 2008). The incidences of avascular necrosis of the proximal femoral epiphysis in the dysplastic hips were 14.3xa0% (119/835 hips; 1994) and 11.5xa0% (76/663 hips; 2008). The type of avascular necrosis in hips from the 2008 study was determined according to the classification of Kalamchi and MacEwen: 24/69 hips (34.8xa0%) were classified as group I; 20/69 hips (29.0xa0%) as group II; 11/69 hips (15.9xa0%) as group Ill; 14/69 hips (20.3xa0%) as group IV. The percentages of hips with acceptable outcomes at skeletal maturity discerned from Severin X-ray changes (grade Ixa0+xa0II/total) were 72.3xa0% (604/835 hips; 1994) and 77.7xa0% (488/628 hips; 2008).ConclusionReduction rates and the incidence of avascular necrosis in 2008 were statistically similar to the results in 1994. The rate of acceptable outcome (Severin grade Ixa0+xa0II/total) in 2008 was statistically higher than that of 1994.

Does Salter Innominate Osteotomy Predispose the Patient to Acetabular Retroversion in Adulthood

BackgroundSalter innominate osteotomy has been identified as an effective additional surgery for the dysplastic hip. However, because in this procedure, the distal segment of the pelvis is displaced laterally and anteriorly, it may predispose the patient to acetabular retroversion. The degree to which this may be the case, however, remains incompletely characterized.Questions/purposesWe asked, in a group of pediatric patients with acetabular dysplasia who underwent Salter osteotomy, whether the operated hip developed (1) acetabular retroversion compared with contralateral unaffected hips; (2) radiographic evidence of osteoarthritis; or (3) worse functional scores. (4) In addition, we asked whether femoral head deformity resulting from aseptic necrosis was a risk factor for acetabular retroversion.MethodsBetween 1971 and 2001, we performed 213 Salter innominate osteotomies for unilateral pediatric dysplasia, of which 99 hips (47%) in 99 patients were available for review at a mean of 16 years after surgery (range, 12–25 years). Average patient age at surgery was 4 years (range, 2–9 years) and the average age at the most recent followup was 21 years (range, 18–29 years). Acetabular retroversion was diagnosed based on the presence of a positive crossover sign and prominence of the ischial spine sign at the final visit. The center-edge angle, acetabular angle of Sharp, and acetabular index were measured at preoperative and final visits. Contralateral unaffected hips were used as controls, and statistical comparison was made in each patient. Clinical findings, including Harris hip score (HHS) and the anterior impingement sign, were recorded at the final visit.ResultsPatients were no more likely to have a positive crossover sign in the surgically treated hips (20 of 99 hips [20%]) than in the contralateral control hips (17 of 99 hips [17%]; pxa0=xa00584). In addition, the percentage of positive prominence of the ischial spine sign was not different between treated hips (22 of 99 hips [22%]) and contralateral hips (18 of 99 hips [18%]; pxa0=xa00.256). Hips that had a positive crossover or prominence of the ischial spine sign in the operated hips were likely also to have a positive crossover sign or prominence of the ischial spine sign in the unaffected hips (16 of 20 hips [80%] crossover sign, 17 of 22 hips [77%] prominence of the ischial spine sign). At the final visit, five hips (5%) showed osteoarthritic change; one of the five hips (20%) showed positive crossover and prominence of the ischial spine signs, and the remaining four hips showed negative crossover and prominence of the ischial spine signs. There was no significant difference in HHS between the crossover-positive and crossover-negative patient groups nor in the prominence of the ischial spine-positive and prominence of the ischial spine-negative patient groups (crossover sign, pxa0=xa00.68; prominence of the ischial spine sign, pxa0=xa00.54). Hips with femoral head deformity (25 of 99 hips [25%]) were more likely to have acetabular retroversion compared with hips without femoral-head deformity (crossover sign, pxa0=xa00.029, prominence of the ischial spine sign, pxa0=xa00.013).ConclusionsOur results suggest that Salter innominate osteotomy does not consistently cause acetabular retroversion in adulthood. We propose that retroversion of the acetabulum is a result of intrinsic development of the pelvis in each patient. A longer-term followup study is needed to determine whether retroverted acetabulum after Slater innominate osteotomy is a true risk factor for early osteoarthritis. Femoral head deformity is a risk factor for subsequent acetabular retroversion.Level of EvidenceLevel III, therapeutic study.

Extraabdominal desmoid tumor in a surgical scar of a patient with Sprengel's deformity.

Patients with an extraabdominal desmoid tumor have multiple minor bone abnormalities. The authors describe a rare case of an extraabdominal desmoid tumor that developed in a scar 2 years after surgery for Sprengels deformity of the right shoulder in an 8-year-old girl. The association between the tumor and Sprengels deformity has never been reported previously. Antecedent surgical trauma might play a role in the development of this tumor.

The epidemiology of developmental dysplasia of the hip in Japan: Findings from a nationwide multi-center survey

BACKGROUNDnIt has been reported that the national incidence of developmental dysplasia of the hip (DDH) has decreased in Japan. This is because of prevention activities after birth since around 1970. However, cases of late-diagnosed DDH have still been noted in some childrens hospitals. There has been no recent survey of DDH in Japan. The purpose of this study was to investigate the current epidemiology of DDH using a comprehensive nationwide survey.nnnMETHODSnA questionnaire was sent to orthopedic surgeons in 1987 facilities nationwide, who were asked to complete and return a survey card on each DDH patient treated between April 2011 and March 2013.nnnRESULTSnA total of 783 (39%) facilities completed and returned the card. Of these, 79% reported no cases of DDH-related dislocation over the 2-year period, while the remaining facilities reported 1295 cases. The characteristics of children diagnosed with DDH-related dislocation were as follows: girls (89%), left side involvement (69%), bilateral involvement (4%), positive family history (27%), first-born (56%), and pelvic position at birth (15%). Seasonal variation showed an increase in DDH incidence among those born in the winter. Overall, 199 cases (15%) were diagnosed at >1 year of age, and these included 36 cases diagnosed very late, at >3 years of age. The majority of the 199 cases of late diagnosis had received earlier routine screening at <1 year of age.nnnCONCLUSIONnThe characteristics of the children diagnosed with DDH nationwide were similar to past data from local regions. However, many children were diagnosed late (>1 year of age), particularly in the more populous regions. The findings identify a need for improved early routine screening for DDH in Japan.

A new predictive indicator by arthrography for future acetabular growth following conservative treatment of developmental dysplasia of the hip.

The aim of this study was to find a new predictive indicator for acetabular growth of developmental dysplasia of the hip. Seventy-three hips that were diagnosed with developmental dysplasia of the hip and treated by conservative reduction were included in our study. In 30 hips with center-edge angle⩽10° at age 4, the center-edge of the acetabular limbus angle (CEALA) in the arthrogram was measured. On the basis of the results, CEALA was significantly smaller in the secondary acetabular dysplasia group than in the normal group at maturity. In conclusion, CEALA is a more reliable and accurate predictive indicator for acetabular development than center-edge angle or acetabular index.

Validation and refinement of the catheterization RISk score for pediatrics (CRISP score): An analysis from the congenital cardiac interventional study consortium

To externally validate the CRISP score, and determine if refinements might improve clinical utility.

Cardiac Lymphangioma Encasing Right Coronary Artery in an Infant

Cardiac lymphangioma is a rare primary benign tumor of the heart. We report a 3-year-old with cystic lymphangioma encasing the right coronary artery. Cardiac magnetic resonance imaging confirmed a intra-pericardial heterogeneous mass measuring 2.6xa0× 2.4xa0× 3.9 cm and situated right anterolateral to the ascending aorta and extending into the right atrioventricular groove. Furthermore, the right coronary artery traversed through the center of the mass. Surgical resection, on cardiopulmonary bypass, consisted of excision by skeletonizing the right coronary artery along the length of the mass. The pathology report was consistent with a lymphatic malformation. The postoperative course was uneventful without recurrence at follow-up.