Long-term skin scarring and orthopaedic sequelae in survivors of meningococcal septic shock.

OBJECTIVE: To assess the incidence of skin scarring and orthopaedic sequelae (amputation, limb-length discrepancy) in patients who survived meningococcal septic shock (MSS) in childhood and to determine the severity and predictors of these sequelae. METHODS: 179 consecutive patients (170 of whom were eligible) with septic shock and purpura requiring intensive care between 1988 and 2001 in Rotterdam, the Netherlands were invited to visit a follow-up clinic 4-16 years after paediatric intensive care unit (PICU) discharge. RESULTS: 58 (48%) of 120 follow-up patients (median follow-up interval 10 years; median age at follow-up 14.5 years) had skin scarring due to purpura. This varied from barely visible to extremely disfiguring scars. Ten patients (8%) had undergone amputation(s) of extremities, ranging from one toe to both legs and one arm. Seven patients (6%) had lower limb-length discrepancy, in most cases together with angular deformity, requiring one or more late surgical intervention(s). Patients with scars or orthopaedic sequelae had significantly higher severity of illness scores, determined by the Pediatric Risk of Mortality score, Vasopressor score and Disseminated Intravascular Coagulation score. Gender or Neisseria meningitidis serogroup had no significant influence on the presence of scars or orthopaedic sequelae. Patients with lower limb-length discrepancy were significantly younger at the time of PICU admission. CONCLUSIONS: The incidence of long-term skin scarring and orthopaedic sequelae was high (48% and 14%, respectively) in patients who survived MSS in childhood. The severity of these sequelae varied from mild to severe. Patients with scars or orthopaedic sequelae had significantly higher severity of illness scores.

Congenital talipes equinovarus: comparison of outcome between a prenatal diagnosis and a diagnosis after delivery.

OBJECTIVES: To establish the impact on outcome of prenatally versus postnatally detected talipes equinovarus (TEV). METHODS: The prenatal group was represented by pregnancies with sonographically detected TEV of which 18 were isolated and 39 were complex. The postnatal group contained 64 infants with an isolated and 10 infants with a complex TEV detected at birth. Treatment consisted of redressement followed by surgical postero-lateral or postero-medial release at the University Paediatric Orthopaedic Centre. The postnatal isolated TEV group underwent redressement treatment at the University Centre (subset A, n = 39) or at a regional general hospital (subset B, n = 25). RESULTS: For isolated TEV, statistically significant difference existed for the surgical procedure (p < 0.001), age at surgery (p < 0.01) and admission time (p < 0.001) between the prenatal and postnatal subset B and between the postnatal subsets A and B. For the complex TEV, no significant difference was found for these variables between the six surviving infants of the prenatal group and the postnatal group. CONCLUSION: Prenatal detection of isolated TEV results in earlier and less complicated postnatal surgery and a shorter admission time, provided treatment is arranged at a paediatric orthopaedic centre. After prenatal detection of a complex TEV, survival is low and determined by associated anomalies.

[Idiopathic scoliosis in adolescents; an inventory into the possibilities of studying the efficacy of screening and treatment]. / Idiopathische scoliose bij adolescenten; inventarisatie van mogelijkheden van onderzoek naar de effectiviteit van screening en behandeling.

OBJECTIVE: To collect information for the purpose of establishing starting points and possibilities for a cost-effectiveness analysis of screening for adolescent idiopathic scoliosis (AIS). DESIGN: Interviews, literature review, questionnaires, an estimation of costs and discussions with experts and involved parties. METHOD: Following an initial interview with 16 orthopaedic surgeons and school doctors a literature study into the efficacy of treatment was carried out regarding the years 1989-1999. The variation in current practice was delineated by means of a questionnaire sent to all 51 municipal health services in the Netherlands. The costs of screening and treatment were estimated on the basis of health insurance premiums and a municipal health service cost model. All of the results were presented to five methodological experts and finally the study results and the recommendations of the five methodological experts were evaluated during a meeting of persons especially invited for this purpose. RESULTS: Screening for AIS was established to realise early diagnosis and treatment with a brace, so as to reduce unsatisfactory cosmetic outcomes and the need for surgery. Screening was performed using the bending test and was performed in 40/48 (83%) of the participating municipal health services. The overall costs of screening and treatment amount to 6 million euros per year. There was no convincing evidence that the screening programme was sufficiently sensitive and bracing sufficiently effective. Neither was there proof of the opposite. The following was recommended: obtain reliable data by carrying out a randomised controlled trial on the effectiveness of treating AIS with bracing in an early stage; carry out a case-control study combined with a retrospective patient follow-up study to evaluate the current screening practice; draw up a national standard for the screening of postural disorders in youth healthcare to ensure effective practice.

Recurrent digital fibroma, focal dermal hypoplasia, and limb malformations.

Recurrent digital fibroma of infancy generally is considered a sporadic tumor of childhood. We describe the case of a mother with recurrent digital fibroma at a young age who gave birth to a daughter with focal dermal hypoplasia, coloboma of the iris and eyelids, anal atresia, and extensive limb malformations. When the infant was 3 months old, fibromas started to appear at the fingertips. The cases of three additional patients are described, with a similar combination of multiple digital fibromas, pigmented marks on the temporal region, and limb malformations. One of these patients has consanguineous parents. The clinical findings overlap partially with Gorlin-Goltz syndrome, which has been renamed by some authors "microphthalmia with linear skin defects" (MLS). Since the skin signs are clearly different, however-more like those of Setleis syndrome ("forceps mark" temporal dysplasia)-the patients described here seem to have a new combination of congenital malformations. Deletion of distal Xp, known to occur in some MLS patients, was not detected using cosmids in fluorescence in situ hybridization. This pattern of digital fibroma with congenital malformations seems to represent a new syndrome.

Doppler sonography of the anterior ascending cervical arteries of the hip: evaluation of healthy and painful hips in children.

OBJECTIVE: We evaluated the Doppler sonography of small feeding arteries to the femoral head in children. SUBJECTS AND METHODS: In a prospective study of 224 hips in 112 patients (mean age, 5 years 11 months), the anterior ascending cervical arteries of the hip were identified with color Doppler sonography. Subsequently, we measured the resistive index (RI) with pulsed Doppler sonography. RESULTS: In 61% (137/224) of hips, a Doppler signal could be obtained. In asymptomatic hips (n = 64), the mean RI was 0.58. In symptomatic hips, the definitive diagnoses and mean values of RI included transient synovitis (n = 31) and 0.92, Perthes' disease (n = 9) and 0.67, and miscellaneous (n = 5) and 0.68. In 28 symptomatic hips, no definite diagnosis could be determined and the complaints spontaneously disappeared during follow-up (mean RI, 0.57). We found no statistically significant difference in the RI of symptomatic versus asymptomatic hips, except in patients with transient synovitis (p < 0.001). In 11 hips with transient synovitis that were reexamined after 4-6 weeks, the RI returned to normal (0.57). The RI in symptomatic hips showed a positive correlation with the amount of effusion (r = 0.69, p < 0.001). In symptomatic and asymptomatic hips, we found no correlation with age (p = 0.9 and 0.1, respectively). CONCLUSION: The deep capsular vessels of the hip joint can be evaluated on Doppler sonography in more than 60% of hips. Also, the RI is age independent and correlates with the amount of effusion.

Prenatal ultrasonographic diagnosis of posteromedial bowing of the leg: two case reports.

Congenital posteromedial bowing of the leg was prenatally detected in two pregnancies, at 20 and 31 weeks of gestation. Posteromedial bowing is a rare anomaly of unknown etiology. The prenatal course, monitored by ultrasonography, and the postnatal clinical and radiographic outcomes are discussed and show a complex differential diagnosis. The initial postnatal therapy is conservative. Leg length discrepancy can eventually be treated by lengthening or epiphysiodesis on the contralateral side.

Atrophy of the quadriceps muscle in children with a painful hip.

The objective of this study was to determine the degree of muscle wasting of various components of the quadriceps muscle in children with a painful hip. Between January 1994 and September 1997, 327 consecutive children with a unilateral painful hip and/or limping were evaluated prospectively with ultrasonography. Quadriceps thickness was measured on both sides. Moreover, muscle thickness was measured in 59 control subjects. The patients were divided into eight groups; transient synovitis (n = 134), Perthes' disease (n = 35), slipped capital femoral epiphysis (n = 5), osteomyelitis (n = 4), aspecific synovitis (n = 5), rheumatoid arthritis (n = 3) and miscellaneous (n = 16). In 125 patients, no sonographic and radiological abnormalities were found and during follow-up the symptoms disappeared ('no pathology' group). Ipsilateral muscle wasting was present in all patient groups, whereas the control subjects showed no significant difference in muscle thickness between legs. The degree of muscle wasting was compared between transient synovitis, the 'no pathology' group, Perthes' disease and control subjects. For both quadriceps and vastus intermedius muscles, there was a significant difference between these groups, except between control subjects and the 'no pathology' group. For the rectus femoris muscle, there was a significant difference between these groups, except between transient synovitis and 'no pathology'. Muscle wasting showed a positive correlation with duration of symptoms and pre-existing muscle mass. In conclusion, different diseases show different degrees of muscle wasting, and there are different patterns of muscle wasting of various components of the quadriceps femoris muscle.

Anterior joint capsule of the normal hip and in children with transient synovitis: US study with anatomic and histologic correlation.

PURPOSE: To study the anatomic components of the anterior joint capsule of the normal hip and in children with transient synovitis. MATERIALS AND METHODS: Six cadaveric specimens were imaged with ultrasonography (US) with special attention to the anterior joint capsule. Subsequently, two specimens were analyzed histologically. These anatomic findings were correlated with the US findings in 58 healthy children and 105 children with unilateral transient synovitis. RESULTS: The anterior joint capsule comprises an anterior and posterior layer, mainly composed of fibrous tissue, lined by only a minute synovial membrane. Both fibrous layers were identified separately at US in 98 of 116 (84%) hips of healthy subjects and in all hips with transient synovitis. Overall, the anterior layer was thicker than the posterior layer. In transient synovitis compared with normal hips, no significant thickening of both layers was present (P = .24 and .57 for the anterior and posterior layers, respectively). Normal variants include plicae, local thickening of the capsule, and pseudodiverticula. CONCLUSION: Increased thickness of the anterior joint capsule in transient synovitis is caused entirely by effusion. There is no US evidence for additional capsule swelling or synovial hypertrophy.

US of the painful hip in childhood: diagnostic value of cartilage thickening and muscle atrophy in the detection of Perthes disease.

PURPOSE: To evaluate the combined use of several ultrasonographic (US) criteria in the detection of Perthes disease. MATERIALS AND METHODS: In a prospective study, 144 consecutive children with a painful hip underwent US. The thicknesses of the (a) anterior recess of the joint capsule, (b) cartilage of the femoral head, and (c) quadriceps muscle were assessed. Sixty-eight children with no symptoms, the control group, were also examined. RESULTS: The final diagnosis was transient synovitis (n = 58), Perthes disease (n = 21), slipped capital femoral epiphysis (SCFE; n = 5), or miscellaneous (n = 6). Fifty-four patients had no US or radiographic abnormalities, and symptoms disappeared during follow-up. The anterior recess in patients with transient synovitis was significantly wider than that in the other patients and control subjects (P < .001). Patients with Perthes disease showed significant cartilage thickening in the symptomatic hip compared with the other patients and control subjects (P < .001). Patients with Perthes disease and patients with SCFE showed significant atrophy of the ipsilateral quadriceps muscle compared with all other groups (P < .001). The combined use of these US criteria for the diagnosis of Perthes disease resulted in a positive predictive value of 95%, a negative predictive value of 95%, a sensitivity of 71%, and a specificity of 99%. CONCLUSION: The combination of several US criteria increases the diagnostic value of US of the painful hip in patients with Perthes disease.

[One hundred years of orthopedics in the Netherlands. VIII. Pediatric orthopedics]. / Honderd jaar orthopedie in Nederland. VIII. Kinderorthopedie.

Orthopaedic disorders in children differ in type from those in adults: most frequent are congenital anomalies and disorders of growth and development. The special nature and relative rarity of these conditions justify the separate development of this branch of the discipline. Fractures almost always heal normally after closed reduction and immobilization in a plaster cast; fractures close to epiphyseal discs and in joints require special attention. Slipping of the upper femoral epiphysis necessitates surgical fixation of the epiphysis. Benign bone tumours occur relatively often and mostly require no surgical intervention. The prognosis of solid malignant bone tumours has improved since the introduction of (neo)adjuvant chemotherapy and limb-sparing surgery. In case of difference in leg length, the length of both legs is predicted with the aid of roentgenological measurements. Inhibition of the growth of the longer leg gives rise to fewer complications than lengthening of the short leg. The essence of the treatment of growth disorders due to abnormal ossification of the cartilage is to monitor the natural repair process and to intervene if permanent malformation threatens.

The straight line graph in limb length inequality. A new design based on 182 Dutch children.

Moseley's Straight Line Graph (M-SLG), which is based on growth data obtained in the 1940s and 1950s, is helpful for the timing of physiodesis. We investigated whether current growth data could improve this graph. We estimated growth curves based on recent data on 182 Dutch children, collected between 1979 and 1994, using repeated measure analysis of variance. In both boys and girls, the mean femur and tibia length had increased, when compared to the data collected by Anderson et al. (1964). Based on our growth data, a new straight line graph (Rotterdam Straight Line Graph; R-SLG) was created. Its value was assessed by comparing the difference between the predicted length of the short (i.e., not operated) limb at maturity with the final limb length. In a group of 34 children who underwent physiodesis up to 10 years ago, the R-SLG gave better prediction of limb length at maturity than the M-SLG did in 22 of 34 cases and equal results were obtained in 5 cases. We conclude that our updated SLG can improve the prediction of final limb length and thus also the timing of physiodesis.

Measurement of limb length inequality. Comparison of clinical methods with orthoradiography in 190 children.

We studied the agreement in measuring limb length inequality with orthoradiograms and clinical methods. In 190 children attending our Limb Length Clinic for the first time, 95% of the measurements with wooden boards was within -1.4 and +1.6 cm of the results of the orthoradiograms. A tape measure had significantly less agreement. The predictive value of a localization of the main limb length inequality above the knee, as found with a tape measure, was 64% and for a localization of the main limb length inequality below the knee 75%. A Wooden Board Reliability Graph is presented, which can be helpful in the decision to perform orthoradiographic measurements of limb length inequality in, e.g., evaluation of impairment.

[Osteomyelitis as a complication following BCG vaccination]. / Osteomyelitis als complicatie na BCG-vaccinatie.

Osteomyelitis of the proximal femur was diagnosed in a 5-month-old girl after BCG vaccination at the age of 2 weeks. The diagnosis was confirmed by biochemical and molecular techniques (DNA fingerprinting). The girl responded well to antituberculous treatment.

Treatment of chronic post-traumatic dislocation of the radial head in children.

Seven children with chronic post-traumatic dislocation of the radial head were treated by open reduction and ligament reconstruction by a triceps tendon slip. In the four patients with anterior dislocation, good results were achieved; in the two with anterolateral dislocations bowing of the ulna persisted and subluxation recurred. One patient with an anterior dislocation developed a radio-ulnar synostosis. For anterolateral dislocations, we advise the combining of open reduction and ligament reconstruction with osteotomy of the ulna.

Distal femoral physiolysis in renal osteodystrophy. Successful nonoperative treatment of 3 cases followed for 5 years.

Slippage of 5 distal femoral epiphyses in 3 children with renal osteodystrophy was successfully treated without operation. During optimal medical treatment and plaster immobilization of the knee, remodelling took place by reorientation of the growth plate.

Timing of physiodesis in limb length inequality. The Straight Line Graph applied in 30 patients.

In a prospective study 30 children underwent 33 physiodeses for lower limb length inequality (LLI). Timing of surgery was based on (bi)annual orthoradiographic measurements and skeletal age, and in accordance with Moseley's Straight Line Graph. The mean predicted LLI was 5.2 (3.0-11) cm and the mean LLI at the end of growth was 1.4 (0.0-4.3) cm. In 9 patients final LLI exceeded 1.5 cm, and one of these patients was operated on twice. In total, secondary operations were performed three times. After analysis of the failures it is concluded that the accuracy of the Straight Line Graph is mainly limited by the pattern of skeletal maturation. Recommendations to prevent failures from other causes are given.

[The treatment of congenital clubfoot in The Netherlands; results of a survey among orthopedic surgeons]. / De behandeling van de congenitale klompvoet in Nederland; resultaten van een enquête onder orthopedisch chirurgen.

As the result of an inquiry among Dutch orthopaedic surgeons a consensus is formulated on the treatment of congenital talipes equinovarus in the first year of life. From this, the following general directives emerge: Treatment should start early and consist of repeated redressing followed by immobilisation; In more severe cases often surgical release is necessary, this should be performed preferably between the 4th and 9th months of life, in order to make normal motor development possible.

[Ultrasonography as initial imaging study can replace roentgen studies under certain circumstances in suspected congenital hip dysplasia]. / Echografie kan röntgenonderzoek onder bepaalde voorwaarden vervangen als eerste beeldvormend onderzoek bij vermoeden van congenitale heupdysplasie.

Sonography of the infant hip is increasingly used in the investigation of congenital dysplasia of the hip (CDH). It tends to replace the radiological investigation, which was the common method up till now. In this study sonography and radiological investigation were compared for 255 patient (510 hips), who had been referred to the orthopaedic surgeon on the presumption of CDH. For the sonography the predictive value of a negative finding was 99% (95% CI: 99-100%). The predictive value of a positive finding was 87% (95% CI: 78-95%). Specificity was high: 98% (95% CI: 97-99%); sensitivity lower: 95% (95% CI: 90-100%). Our conclusion is that sonography can replace the radiological investigation if it shows a normal result, for an infant older than 3 months. Additional radiological investigation is necessary if the sonography is abnormal. For infants under 3 months of age the investigation should be repeated at the age of 3 months, at any rate in case of doubt.

[Coxitis fugax. The sonographic and radiologic picture in 65 cases]. / Coxitis fugax. Sonographisches und radiologisches Bild in 65 Fällen.

Sonography is a valuable technique for the detection of hip joint effusion in children with transient synovitis. In a retrospective study of 65 patients distension of the anterior recess was found to be increased by 2 mm or more in all patients investigated. A sonographic follow-up examination was carried out in 30 patients. These patients showed complete regression of hip effusion after 4 weeks. The importance of attention to sonographic changes of the adjacent bony outline and femoral head deformity in connection with other hip diseases is emphasized. In 45 patients in this study (62% of the cases) conventional radiography showed one or more indirect signs such as displacement, blurring or even obliteration of the fatty intermuscular planes or an increased joint space. In uncomplicated cases with clear sonographic and clinical findings a pelvic radiogram is unnecessary.