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P S Babyn

Publications and source records attributed to P S Babyn.

At least 19 recordsLinked to original sources

Reliability and construct validity of the compatible MRI scoring system for evaluation of haemophilic knees and ankles of haemophilic children. Expert MRI working group of the international prophylaxis study group.

We tested the reliability and construct validity of the Compatible magnetic resonance imaging (MRI) scale for the evaluation of haemophilic knees and ankles and compared the diagnostic performance of MRI and plain film radiographs. Sagittal and coronal gradient-echo 1.5-T MR images of knees (n=22) and ankles (n=23) were obtained from boys (age range 4-16 years; mean 11 years) in two centres (Toronto, n=26; Europe, n=19). The MR images were independently read by four blinded radiologists on two occasions. Number of previous joint bleedings and laboratory level of severity of haemophilia were the reference standards for imaging assessment. Both components of the MRI scale demonstrated high inter- and intrareader intraclass correlation coefficients (progressive (P) scale, 0.91 and 0.94; additive (A) scale, 0.81 and 0.92 respectively). The correlation between the osteochondral domain of the MRI scale and patient's age was moderate. Otherwise, correlations between A- and P-scales and clinical laboratory measurements were weak. The areas under the curve (AUCs) used for discrimination of disease severity were similar for the A- and P-scales (AUCs used for mild disease, A-scale, 0.72+/-0.07; P-scale, 0.69+/-0.08; P=0.23; AUCs for severe disease, A-scale, 0.93+/-0.05; P-scale, 0.87+/-0.08; P=0.05). No differences were noted between the AUCs of the MRI and radiographic scales used for discrimination of late osteoarticular changes; MRI scales performed better for discrimination of early changes. In conclusion, both MRI scales demonstrated excellent reliability, poor convergent validity, and moderate and excellent validity for discrimination of mild and severe diseases respectively. Compared with radiographic scores, the MRI scales performed better for discrimination of early osteoarticular changes.

Adolescent↗

Reliability of progressive and additive MRI scoring systems for evaluation of haemophilic arthropathy in children: expert MRI Working Group of the International Prophylaxis Study Group.

Effective treatment of haemophilic arthropathy requires a detailed evaluation of joint integrity. Methodological assessment of magnetic resonance imaging (MRI) scores are needed to assure reproducibility of measurements when comparing results of clinical trials conducted in different centres. We compared the reliability of two MRI scoring systems for assessment of haemophilic arthropathy: one progressive system that displays the most severe change and one additive system that depicts osteochondral and soft tissue-related changes. A total of 47 1.5 T MRI examinations of knees (n = 21) and ankles (n = 26) of 42 haemophilic boys, age range, 22 months to 18 years, performed at different centres (Toronto, n = 20, Europe, n = 12 and Denver, n = 15) were independently reviewed by four radiologists at two occasions. Twenty-two examinations were from children <9 years and 25 from children >/=9. Sagittal and coronal gradient-echo (MPGR, 3D FLASH with fat saturation, GRASS) images were obtained. The MRI examinations of the ankle and knee studies presented with osteochondral abnormalities in 38.5% and 23.8% of the cases respectively. The two scoring systems demonstrated an excellent inter-reader [progressive, 0.88; additive (A, e, s and h components), 0.86] and intra-reader [progressive, 0.92; additive (A, e, s and h components), 0.93] reliability using intraclass correlation coefficients (ICCs). Although ICCs were slightly higher for knees when compared with ankles, and for older children when compared with younger children, all values fell within excellent inter- and intra-reader reliability categories. The two MRI scoring systems demonstrated a comparable reliability. This result constitutes the basis for further development of a combined MRI scoring system for assessment of haemophilic arthropathy, which incorporates progressive and additive components.

Adolescent↗

Physical therapy and imaging outcome measures in a haemophilia population treated with factor prophylaxis: current status and future directions.

Routine infusions of factor VIII to prevent bleeding, known as prophylaxis, and other intensive therapies are being more broadly applied to patients with haemophilia. These therapies differ widely in replacement product usage, cost, frequency of venous access and parental effort. In order to address residual issues relating to recommendations, implementation, and evaluations of prophylaxis therapy in persons with haemophila, a multinational working group was formed and called the International Prophylaxis Study Group (IPSG). The group was comprised of haemophilia treaters actively involved in studies of prophylaxis from North America and Europe. Two expert committees, the Physical Therapy (PT) Working Group and the Magnetic Resonance Imaging (MRI) Working Group were organized to critically assess existing tools for assessment of joint outcome. These two committees independently concluded that the WFH Physical Examination Scale (WFH PE Scale) and the WFH X-ray Scale (WFH XR Scale) were inadequately sensitive to detect early changes in joints. New scales were developed based on suggested modifications of the existing scales and called the Haemophilia Joint Health Score (HJHS) and the International MRI Scales. The new scales were piloted. Concordance was measured by the intra-class correlation coefficient of variation. Reliability of the HJHS was excellent with an inter-observer co-efficient of 0.83 and a test-retest value of 0.89. The MRI study was conducted using both Denver and European scoring approaches; inter-reader reliability using the two approaches was 0.88 and 0.87; test-retest reliability was 0.92 and 0.93. These new PT and MRI scales promise to improve outcome assessment in children on early preventive treatment regimens.

Hemophilia A↗

The arthritis of inflammatory childhood myositis syndromes.

OBJECTIVE: Arthritis has been an associated finding in juvenile dermatomyositis (JDM), but its prevalence, course, and response to therapy has not been well described. We investigated the frequency, course, and clinical and radiographic features in a large cohort of patients with JDM. METHODS: The charts of 94 patients with idiopathic myositis (1984-99) were reviewed: 80 JDM, 3 juvenile polymyositis (JPM), 5 amyopathic JDM, and 6 overlap myositis syndromes. Compiled data included demographics, clinical features, a detailed description of the arthritis, investigations (radiographs, autoantibodies), course, and response to therapy. All radiographs were independently reviewed by a single radiologist. RESULTS: Sixty-one percent (95% CI 50-72%) of patients with JDM had arthritis. The arthritis was reported a median 4.5 mo (range -73.6 to 76.6 mo) after the JDM onset. When compared to patients with no arthritis, the occurrence of arthritis was not significantly related to sex, race, positive antinuclear antibody or rheumatoid factor, calcinosis, nodules, vasculitis, or Raynaud's phenomenon. The initial involvement was pauciarticular in 67% and polyarticular in 33%. In the pauci group, asymptomatic knee effusions were the predominant finding (n = 19, 58%), and in 18 patients may have been the result of steroid therapy. Two patients evolved from a pauci onset to a polyarticular course. All responded to therapy (corticosteroids; 47 were taking other medications) with remission of the arthritis within a median of 2.0 mo (range 0.1-64.5 mo). However, the arthritis recurred in 39% as the corticosteroids were tapered. Four patients with JDM eventually required corticosteroid wrist injections, with resolution of the arthritis. The arthritis was nonerosive in all cases. No patient with JPM had arthritis. Three of 5 patients with amyopathic JDM and 4 of 6 with overlap myositis syndrome had a nonerosive polyarthritis. CONCLUSION: Nonerosive arthritis involving the knees, wrists, elbows, and fingers is a frequent manifestation of JDM and other idiopathic childhood myositis. The arthritis is seen early in the course of JDM and often responds to treatment. However, the arthritis may recur with tapering of corticosteroids despite remission of the JDM. In a significant proportion of JDM cases, arthritis is the major sequela and may warrant further medical therapy or intraarticular corticosteroid injections.

Adolescent↗

Local complications after limb-salvage surgery for pediatric bone tumours: a pictorial essay.

The prognosis for patients with bone sarcoma treated with LSS has improved considerably over the past 2 decades, but this has also lead to an increase in the number of complications requiring treatment. Imaging plays an important role, not only in assessing the primary tumour, but also in identifying postsurgical complications. Plain radiography demonstrates the majority of the complications associated with LSS and remains the mainstay of follow-up imaging. Complications such as fractures are common and warrant frequent plain film follow-up. Imaging with scintigraphy, MRI and CT should be tailored to the patient's clinical history, type of surgery and suspected complications. A baseline postoperative bone scan examination can be helpful for comparisons with subsequent scans for the detection of complications. Sonography should be considered if infection is suspected. Finally, tumour recurrence may be frequent enough to consider more extensive use of MRI.

Adolescent↗

Traumatic diaphragmatic injuries in infants and children: imaging findings.

OBJECTIVES: Traumatic diaphragmatic injuries (DI) in infants and children are uncommon and are often associated with multiple severe injuries. Delayed presentation can be life threatening due to organ herniation and strangulation. We present the imaging findings in a relatively large population of children who experienced this rare injury. METHODS: Medical records of all patients admitted to our Trauma Service from 1977 to 1998 with DI were retrospectively reviewed recording imaging, clinical and surgical or autopsy findings. RESULTS: Of sixteen patients with DI (7 females, 9 males; age 3 weeks to 15 years), 14 suffered from blunt trauma secondary to high-energy impact, and 2 from penetrating injuries. Unilateral DI occurred equally on each side, with one bilateral injury. Associated injuries, present in 81%, included severe head injuries, visceral, mesenteric and vascular injuries and multiple fractures. Six patients died from multiple organ failure (3), head injury (2), and shock (1). Findings in the initial chest X-ray suggested the diagnosis in 13 (81%) of 16 injuries, and CT demonstrated irregularity and thickening of the diaphragm in 4 out of 7. CONCLUSIONS: Plain film findings suggested the diagnosis in most; CT and MR were useful adjuncts. High index of suspicion and awareness of the mechanism of injury can lead to prompt diagnosis, early repair, and decreased morbidity and mortality.

Adolescent↗

The value of routine follow-up imaging in pediatric blunt liver trauma.

PURPOSE: To evaluate the utility of routine follow-up computed tomography (CT) and/or ultrasound (US) scans in children with blunt hepatic trauma initially managed non-operatively. MATERIALS AND METHODS: Review of the records of 66 children with proven blunt liver injury on initial CT scan, who were initially managed non-operatively during the period January 1991 to December 1996. Follow-up CT and US studies were analyzed and correlated with clinical outcome. RESULTS: Of the 66 children, 30 were not followed with any imaging study, 26 were followed with US only, 7 with CT only and 3 with US and CT. Disappearance of the liver lesion(s) was seen in 25 patients (range: 6 days - 14 months) and decrease in size was noted in 10. In one patient, who developed abdominal and right shoulder pain 10 days after presentation with subsequent hemoglobin drop, CT showed contrast medium extravasation into a hepatic hematoma from portal vein injury that required surgery. CONCLUSION: Our series suggests that in asymptomatic patients, US and CT follow-up studies do not provide the additional information needed for patient management. Therefore, we believe that in asymptomatic children with blunt hepatic trauma who are clinically stable, routine follow-up imaging studies are of very limited value.

Child↗

MRI appearances of hip abnormalities in mucolipidosis, type III.

Mucolipidosis type III (ML-III) is a lysosomal storage disease often presenting with joint involvement. We report the MRI appearance of the hips in two siblings with ML-III showing abnormal signal intensity within the hips with increased synovial thickness. Although the etiology is uncertain this may reflect a fibrous response to ML-III.

Adult↗

What have we learned about traumatic diaphragmatic hernias in children?

BACKGROUND/PURPOSE: Diaphragmatic injuries have been reported to be a predictor of serious associated injuries in trauma and a marker of severity. The aim of this retrospective study was to identify pitfalls in the diagnosis and treatment of these injuries in children. METHODS: Data were collected from all patients admitted to the trauma service with traumatic diaphragmatic hernias for the period of January 1977 to August 1998. The authors evaluated 15 cases of traumatic diaphragmatic rupture (6 girls and 9 boys). RESULTS: Mean age was 7.5 years (range, 3 weeks to 15 years). Thirteen patients suffered from blunt trauma, and 2 patients suffered from penetrating trauma. The right and left hemidiaphragms were injured equally (7 patients each), with 1 additional patient suffering from bilateral injuries. All but 1 patient had laparotomies for trauma (n = 14). The diagnosis was made preoperatively in 8 patients (53%) with just a chest radiograph. Computed tomography (CT) scan, magnetic resonance imaging (MRI), and oral contrast studies were used as ancillary tests to diagnose traumatic diaphragmatic rupture. There were 3 missed injuries. Associated injuries included liver lacerations (47%), pelvic fractures (47%), major vessels tear (40%), bowel perforations (33%), long bone fractures (20%), renal lacerations (20%), splenic lacerations (13%), and closed head injuries (13%). The mean hospital stay was 20 days (range, 7 to 60 days). Complications were observed most commonly in those patients with multiple injuries and included postoperative ileus (40%), pneumonia (30%), pancreatitis (20%), wound infection (20%), intestinal obstruction (20%), cholestasis (10%), and renal failure (6%). Five deaths (33%) were caused by hemorrhagic shock, respiratory failure, coagulopathy, and refractory acidosis. CONCLUSIONS: Traumatic diaphragmatic hernias usually are associated with serious injuries in children. It is important to combine a high index of suspicion with radiological diagnostic tests in patients at risk. Palpation and/or visualization of both diaphragms at laparotomy is extremely important in detecting these injuries when they are not suspected preoperatively.

Adolescent↗

The association between Turner's syndrome and juvenile rheumatoid arthritis.

BACKGROUND: Turner's syndrome (TS) is a chromosomal disease frequently associated with autoimmune conditions including thyroid disease, inflammatory bowel disease, and diabetes. Recent reports have described an association with juvenile rheumatoid arthritis (JRA) and psoriatic arthritis. We describe three additional cases of TS associated with JRA. OBJECTIVE: The objective of this report is to describe the radiographic and clinical features of TS associated with JRA to heighten awareness of this association and alert radiologists to recognize the superimposition of radiographic bony changes of chronic joint disease and the bone changes of TS. Patients and methods. Clinical history and radiographic images of three girls with TS and arthritis were reviewed. The radiographic findings typical of TS and juvenile arthritis are described. RESULTS: Of about 65 patients at our center with Turner's syndrome 3 had JRA (as described in this report), supporting the association between TS and JRA. All our patients who met American College of Rheumatology Classification criteria for JRA had radiographic and clinical findings consistent with both their JRA and Turner's syndrome. CONCLUSION: We believe that it is important to consider the diagnosis of Turner's syndrome in girls with JRA, recognizing that characteristic radiographic findings such as metacarpal shortening are usually present. Conversely, suspicion of an underlying inflammatory arthritis is warranted in search for radiological findings consistent with JRA in girls with TS and joint symptoms.

Adolescent↗

Delayed hemorrhage after nonoperative management of blunt hepatic trauma in children: a rare but significant event.

PURPOSE: Nonoperative management of blunt hepatic injury (BHI) has become widely accepted in hemodynamically stable children without ongoing transfusion requirements. However, late hemorrhage, especially after discharge from the hospital can be devastating. The authors report the occurrence of serious late hemorrhage and the sentinel signs and symptoms in children at risk for this complication. METHODS: Nonoperative management of hemodynamically stable children included computed tomography (CT) evaluation on admission and hospitalization with bed rest for 7 days, regardless of injury grade. Activity was restricted for 3 months after discharge. Hepatic injuries were classified according to grade, amount of hemoperitoneum, and periportal hypoattenuation. RESULTS: Over 5 years, nonoperative management was successful in 74 of 75 children. One child returned to the hospital 3 days after discharge with recurrent hemorrhage necessitating surgical control. Review of the CT findings demonstrated that he was the only child with severe liver injury in all four classifications. A second child, initially treated at an outside hospital, presented 10 days after injury with ongoing bleeding and died despite surgical intervention. Only the two children with delayed bleeding had persistent right abdominal and shoulder discomfort in the week after BHI. CONCLUSIONS: Our findings support nonoperative management of BHI. However, late hemorrhage heralded by persistence of right abdominal and shoulder pain may occur in children with severe hepatic trauma and high injury severity scores in multiple classifications.

Adolescent↗

Nonoperative management of pancreatic injuries in children.

PURPOSE: The safety and efficacy of nonoperative management of pancreatic contusions and transections was examined by reviewing the case histories of 35 consecutive children with pancreatic injuries treated over the past 10 years. METHODS/RESULTS: Surgical exploration was performed for the management of associated injuries only. The diagnosis of pancreatic trauma was suspected in children with abdominal pain, tenderness, elevated serum amylase levels and findings consistent with pancreatic injury on abdominal ultrasound scan or computerized tomography (CT) examination. After children were diagnosed with pancreatic injury, enteral feedings were withheld and total parenteral nutrition administered until abdominal pain resolved and serum amylase levels and radiographic findings improved. Twenty-three children received diagnosis within 24 hours of injury, and in 12, the diagnosis was delayed 2 to 14 days. Hyperamylasemia was found in 27 of 35 children. Twenty-eight children sustaining pancreatic injuries were treated nonoperatively. Abdominal imaging in these children demonstrated pancreatic contusion in 14, transection in 11, and pseudocyst in three. Enteral feeding resumed an average of 15 days after injury. The average hospital stay was 21 days. Pseudocysts formed in 10 children (2 of 14 with contusion; 5 of 11 with transection; three children presented late, and the type of pancreatic injury could not be determined), whose average hospital stay was 25 days. All pseudocysts were successfully managed nonoperatively, although percutaneous aspiration or drainage was required in six children. Children underwent follow-up for an average of 10 months after injury (range, 1 to 144 months). Abdominal pain and radiological abnormalities resolved in all children before discharge from the clinic. CONCLUSIONS: Nonoperative management of pancreatic contusion and transection diagnosed radiologically is effective and safe. Pseudocysts may form after pancreatic injury, and if large or symptomatic, can be managed successfully by percutaneous drainage.

Abdominal Injuries↗

Normal bone marrow: signal characteristics and fatty conversion.

Understanding the dynamic MR appearance of normal bone marrow during childhood is essential. This article reviews normal bone marrow structure and development, especially the process of fatty conversion, laying the cornerstone for accurate interpretation of marrow MR imaging.

Adipose Tissue↗

Diagnosis and management of duodenal injuries in children.

Traumatic duodenal perforations in children pose a diagnostic and therapeutic challenge. To identify specific diagnostic criteria and define an optimal therapeutic protocol, we reviewed all duodenal injuries treated at our institution in the past 10 years. There were 14 hematomas and 13 perforations. The diagnosis was confirmed by computed tomography (CT), ultrasound scan (US), upper gastrointestinal contrast studies (UGI), or at laparotomy. The clinical findings and CT findings of the two groups were compared. Children with suspected duodenal hematomas were treated expectantly, and children with duodenal perforations were treated surgically. Twenty-five associated injuries (10 pancreatic) occurred in 19 children. Children with perforations had higher injury severity scores (ISS) (25 v 9), but the two groups could not be differentiated based on presenting signs, symptoms, or laboratory findings. CT findings of retroperitoneal air or contrast were seen in 9 of 9 perforations and in 0 of 10 hematomas. CT findings of intraabdominal or retroperitoneal fluid, mesenteric enhancement, and thickened duodenal wall did not differentiate the two groups. Duodenojejunostomy was performed in one patient, and primary repair was performed in 11 children who had perforation. In five children, duodenostomy tube drainage with feeding jejunostomy or gastrojejunostomy were added. Complications occurred in three of four children in the first 5 years of the study and in two of nine children in the last 5 years. The decreased morbidity rate correlated with reduced time to definitive therapy (28 v 7.8 hours). Duodenal fistulae resulted in three of seven children treated without duodenostomy tube drainage and zero of five treated with drainage. Enteral feeds resumed faster (average, 12 v 27 days) if repair of perforation was combined with feeding jejunostomy or pyloric exclusion and gastrojejunostomy. Children with duodenal hematoma resumed eating an average of 16 days after injury. Only one child required surgery for persistent obstruction. The findings of retroperitoneal air and contrast extravasation on CT accurately distinguish duodenal perforation from hematoma. Conservative management of hematoma is safe and effective. Primary repair of perforation with duodenal drainage results in fewer postoperative complications, and gastrojejunostomy or feeding jejunostomy shorten the time to resumption of feeds.

Child↗

Changes in gastric emptying in early postnatal life.

OBJECTIVE: To study the pattern of gastric emptying in very premature infants and to determine whether there are changes with postnatal age and the ability to tolerate feedings. METHODS: Sequential ultrasound measurements of the gastric antral cross-sectional area were obtained in 32 infants (mean gestational age, 26 +/- 1 weeks) before and after feeding for 2 hours. Studies were carried out after initiation of feedings, when full feedings were received, and at 32 weeks. Infants classified as feeding intolerant (n = 9) were also studied when feedings were restarted. Gastric emptying was assessed by the time taken for antral cross-sectional area to reach maximal value and to decrease to half the maximal increment (half-antral clearance). RESULTS: Delayed antral distention was observed at the time of the initial study in both feeding-tolerant (8 of 23) and feeding-intolerant (8 of 9) infants; however, there were significant differences in times for maximal antral distention (p < 0.002) and half-antral clearance (p < 0.006) between the feeding-tolerant and feeding-intolerant infants. By the time of full feedings, the feeding-intolerant infants showed immediate gastric emptying but still had a longer half-antral clearance time (p < 0.01). By 32 weeks, all infants had immediate antral distention and a more mature curvilinear pattern of gastric emptying. CONCLUSIONS: Knowledge of these different patterns of gastric emptying in very premature infants may lead to the development of more rational feeding strategies.

Aorta, Abdominal↗

Imaging gastrointestinal perforation in pediatric blunt abdominal trauma.

OBJECTIVE: To assess the role of imaging, in particular CT, in the early detection of GI perforation. SUBJECTS AND METHODS: In a 10-year period, 43 patients with surgically confirmed GI perforation were identified from hospital records; 22 of these had preoperative CT evaluation. Medical records and radiology were retrospectively reviewed and CT studies were particularly assessed for extraluminal air, free intraperitoneal fluid, bowel wall thickening, bowel wall enhancement, and bowel dilatation. During the study period an additional 12 trauma patients were identified who had CT studies demonstrating the above findings, but who had hypovolemic shock bowel or nondisrupting bowel injury without perforation evident. RESULTS: Extraluminal air was demonstrated in 47 % of the imaged perforations. There was one false-positive extraluminal air. Perforation was confirmed in patients who had all five of the above CT findings, but this was the case for only 18 % of patients with perforation. One or more of the five specified CT findings were present in all CT studies reviewed. No false-negative CT study was performed in the study period. CONCLUSION: Separating nondisrupting bowel injury from perforation is diagnostically difficult; however, CT remains a good modality for assessing GI perforation in pediatric blunt trauma, but it cannot replace diligent and repeated clinical evaluation of all potential perforation victims.

Abdominal Injuries↗

Discoid meniscus in children: magnetic resonance imaging characteristics.

OBJECTIVE: To describe the magnetic resonance imaging (MRI) appearance and associated abnormalities of discoid menisci in children. PATIENTS AND METHODS: The MRI findings for a total of 30 knees (in 20 children ranging in age from 4.5 to 16 [mean 9] years) in which discoid meniscus had been diagnosed were reviewed and correlated with clinical symptoms and arthroscopy findings. RESULTS: The symptoms, bilateral in 5 patients and unilateral in 15, included pain in 15 knees, snapping or clunking in 8, reduced mobility in 8, locking in 2 and "giving-way" in 2; there were flexion contractures in 2 patients. Five patients had unilateral symptoms but bilateral discoid menisci. The transverse diameter of the meniscus ranged from 18 to 37 mm and the depth from 14 to 36 mm. The lateral meniscal height was significantly greater than the medial meniscal height (p = 0.0001). On T1-weighted and T2-weighted images the meniscal signal was increased heterogeneously in 11 knees, in a central, horizontal, linear fashion in 9, diffusely in 6 and in a partly linear, partly diffuse fashion in 3. Thirteen joint effusions, 10 lateral meniscal tears and 4 meniscal cysts were identified. Eight arthroscopy procedures were performed in six of the children (for seven knees). Discoid meniscus was confirmed in six knees (with one initial false-negative result), tears were confirmed in four, and an additional tear was identified in one; in addition, one meniscus was characterized as Wrisberg type. CONCLUSIONS: Discoid meniscus commonly occurs bilaterally. High intrameniscal signal is found, especially in symptomatic patients. The size criteria for diagnosing this condition in children are similar to those for adults.

Adolescent↗