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BSPR 2000 Abstracts

Title:   MRI HIP IN PAEDIATRIC RHEUMATIC DISORDERS           

 Authors:     Williamson R, Bose S, Machado C, Owens C, Hall C and Murray K

 Hospital      Great Ormond Street Hospital, Great Ormond Street,

         London WC1, United Kingdom

 

Abstract:

 

Aim:                        To describe the abnormalities seen on MRI performed for hip disease in paediatric rheumatic disorders and relate them to clinical evaluation.

 

Method:            A retrospective review of MRI examinations performed on a 1.5T Siemens machine in conjunction with information from a comprehensive casenote review.  Readers of each set of data were blinded to the data from the other set.  31 patients, with 37 MRI examinations were included in the study.

 

Results:            Rheumatic Disorders investigated included JIA (17 cases including 6 with Spondyloarthropathy), Connective Tissue Disorders (5 cases) and other disorders (9 cases).  MRI in JIA showed synovial thickening in 23/28 hips examined plus effusions (17/28), bony erosion (17/28) and avascular necrosis (8/28).  Patients with spondyloarthropathy either showed features typical of JIA or effusions with no evidence of synovial disease (4/14 hips).  Connective tissue disorders were associated with mild abnormality.  A variety of non-rheumatic disorders demonstrated either normal MRI or effusion with no synovial disease.

 

Conclusions:            Synovial thickening and enhancement is a strong indicator of JIA and can assess disease activity.  Effusions without synovial disease are seen in some cases of spondyloarthropathy but are a non-specific finding.  Patterns of disease suggest a future primary diagnostic role for MRI


THE ROLE OF MUSKULOSKELETAL ULTRASOUND AT DIAGNOSIS AND FOLLOW UP IN PATIENTS WITH JUVENILE DERMATOMYOSITIS

 

Dr C M Owens,  Dr S Bose, Dr K Murray, Dr C Machado

 

Great Ormond Street Hospital for Children NHS Trust

Great Ormond Street, London WC1N 3JH, England

 

OBJECTIVE:  To assess the utility of ultrasound of the thigh musculature at diagnosis and follow up in juvenile dermatomyositis 9JDM).  To correlate these findings with MRI and clinical/pathological indicators of disease activity, including response to disease modifying drugs.

 

METHOD:  6 subjects underwent high resolution muscle ultrasound using a Acusom Sequoia system (15LB high frequency probe) at diagnosis, 4/6 patients underwent follow up ultrasound scans (mean time interval between scans – 6 months).  All patients were scanned on the same day as their MRI of the thigh muscles.  Results of the US scans were correlated with clinical biochemical and (if available) histological markers of disease activity, and serial scans correlated with disease activity.  US scans were correlated well with MRI markers of disease activity.

 

RESULTS:  All patients had abnormal US scans at diagnosis, with the majority (5/6) showing diffuse symmetrical hyperechogenicity affecting mainly the adductors, vasti and rectus femoris muscles.  Marked hyperaemia of these muscle groups was present with colour Doppler imaging.  All patients showed marked resolution in both hyperechogenicity and hyperperfusion of musculature but 2/6 showed considerable wasting and fatty infiltration following therapy.  1 patient had calcinosis on follow up.  Ultrasound findings correlated well with MRI markers of disease activity.

 

CONCLUSION:  Muskuloskeletal US is a safe, accurate, inexpensive means of diagnosing and monitoring.  JDM, obviating the need for sedation.


MRI IN JUVENILE DERMATOMYOSITIS – CLINICO-RADIOLOGICAL CORRELATION

 

S Bose, R Williamson, K Murray, C Machado, CM Owens

Department of Radiology

Great Ormond Street Hospital for Children NHS Trust

Great Ormond Street, London WC1N 3JH

 

OBJECTIVE:  To observe the spectrum of MR abnormalities in JDM and correlate the findings with clinical and pathological indicators of disease including response to disease modifying therapy.

 

METHOD:  MRI scans of the proximal lower limbs in 15 children with symptomatic JDM were performed on a Siemen’s 1.5 T magnet.

6/15 children underwent T2W axial scans.

9/15 children had additional axial T1W and STIR sequences.

Serial scans performed in 3 patients were correlated with disease progression.  The images were retrospectively reviewed by 2 radiologists for the presence and extent of changes together with clinical, biochemical and where available, histological markers of disease activity at the time of the scan.

 

RESULTS:  Increased signal in muscle groups was seen on T2W or STIR images in 12/15 patients.

Patterns of disease fell into 4 main groups comprising;

  • Diffuse muscle involvement
  • Predominantly circumferential muscle disease
  • Focal central signal change
  • Fatty atrophy

 

A single patient showed fatty atrophy of subcutaneous tissue and muscle with signal void secondary to calcinosis.  Intramuscular oedema on MR correlated with the presence of raised serum inflammatory markers.

 

CONCLUSIONS:  MRI is a useful tool in the assessment of patients with JDM

There is a spectrum of findings both in the distribution and pattern of signal change.  MR findings correlate with clinical parameters of disease activity.


SMALL BOWEL TRANSPLANTATION RADIOLOGICAL REVIEW

 

Authors:  S Parsonage, K Bradshaw and K Johnson

 

Birmingham Children’s Hospital

 

INTRODUCTION:  Birmingham Children’s Hospital is the only Paediatric centre performing liver and small bowel transplantation in the U.K.  Radiological follow up and the early detection of complications in these patients is vital to their management and long term outcome.  We have reviewed the post transplantation radiology of these patients to determine which image modality is best for detection of complications.  We have also documented the workload pressures placed upon the radiology services because of this new innovative procedure.

 

METHOD:  A retrospective review of all the radiology of the 12 patients who have undergone liver and small bowel transplantation.  Age range 7 months 1 week to 7 years 1 month (mean 25 months 2 weeks).  423 separate radiological investigations were performed post transplantation, on these 12 patients.  Correlation was made of the radiological findings with clinical state of the patient.  The complication detection rate for each imaging modality was analysed.

 

RESULTS:  The 423 investigations were, 114 ultrasounds, 212 chest x-rays, 12 CT scans, 15 contrast studies, 61 other plain x-rays, 9 intervention procedures.  The common complications were, loculated intraabdominal collections, bowel perforation, prolonged ileus, chest consolidation/collapse, post transplant lymphoproliferative.

 

CONCLUSION:  Patients undergoing small bowel transplantation require a significant amount of radiological input.   Post operative chest complications and small bowel ileus occur in all patients.  Ultrasound is able to monitor gut vasculaturity reasonable well, but CT is the best method for detecting perforation and  lymphoproliferative disease.  Radiology departments must be aware of the implications of new services instituted by other specialities.  Total number of patients does not equate with the volume of radiological investigations.

 

           


Title:     A 5 Year Review of the Management of Intussusception at Our Lady’s Hospital for Sick Children, Crumlin, from 1994 to 1999.

 

Authors:  Brannigan M., Kelleher J. Hayes R, Blade NS, Phelan EM.

 Department:  Radiology

 Institution:  Our Lady’s Hospital for Sick Children, Crumlin, Dublin

 Address for correspondence:  as above

 Fax 01 409 6969    Telephone 01 409 6454   e-mail:  [email protected]

 

Background:  Intussusception is the most common surgical emergency in childhood.  Therapeutic enema, particularly gas enema, is now accepted as the treatment of coice in the majority of patients and has become the method of choice at Our Lady’s Hospital, Crumlin.

 

Materials and Methods:  199 patients who presented with 229 consecutive episodes of intussusception over a 5 year period, were reviewed.  There were 123 male, 76 femal patients, age range 4 weeks – 15 years.

 

Results:  190 enemas were attempted, 12 using barium and 178 using gas.  One hundred and thirty eight enemas were successful (73%). Fifty two patients in whom radiological reduction was unsuccessful underwent surger.  Thirteen required resection of devitialised bowel and in 44 patients the intussusceptum was manually reduced.  Nine patients were judged unsuitable for enema and went directly to surgery giving an overall success rate for enema of 69% for all patients with intussusception.

 

Conclusion:  Therapeutic enema using gas is the method of choice for reduction of intussusception.  It is a fast and safe method of reduction and has a high probability of success.

 


99m-Tc, HAS Albumin Scinitgraphy

An Investigation of Protein Losing Enteropathy

 

Authors:  M Sultan, M Toy, S Chapman and K Johnson

 

Department of Radiology, Birmingham Children’s Hospital

 

Protein losing enteropathy is a serious disabling condition.   Identification of protein loss into the gut is important to confirm the diagnosis and to localise any protein loss to a section of gut that may be amendable to surgical resection.  The use of labelled albumin is a relatively new investigation for this disorder.  We present our experience in 11 studies.

 

Materials and Methods

10 patients (11 studies) were investigated for protein losing enteropathy.  Patients ranged from 2 years to 15 years (mean 7.5 years) with an equal number of boys and girls.  The results of the 99m-Tc, HAS studies were compared with histological findings, clinical outcome and alternative investigations.

 

Results

Ten of the 11 studies were positive for albumin loss into the gut.  Six of the studies localised protein loss to the small or large bowel and in 4 studies there was diffuse loss in the GI tract.  In the one negative study there was good correlation with other investigations.  In the 10 positive studies, 6 showed good correlation with alternative investigation techniques, and in 2 of these, localisation of the protein loss enabled surgical intervention and cure.  In the 4 other cases where there was discrepancy in the results of the albumin studies and some other investigations, clinical follow-up has confirmed protein loss in 2 of these patients.  In 2 patients there is still no definite diagnosis at 1 year follow-up.  Histological proof of protein losing enteropathy due to intestinal lymphangactesia has been obtained in 6 positive albumin scans.

 

Conclusion

99m-Tc, HAS is useful in the evaluation of protein losing enteropathy.  It allows localisation of protein loss in some cases, making them amenable to surgical treatment.  The technique appears to be as sensitive as other investigations and it is safe and reliable and can be used in children.



Acute hip pain in childhood – are two views necessary?

Authors – Argent J, Clarke N, Fairhurst J, Naadi C, Redfern A.

 

Aim  -  to determine whether both antero-posterior and frog lateral views of the hips are required in the initial assessment of children presenting through Accident & Emegency with hip pain.

 

Method  -  AP only, frog lateral only and AP and frog lateral views were retrospectively reviewed by Consultant Paediatric Radiologists, Trainee Specialist Radiology Registrars and a Specialist Orthopaedic Registrar.  Sensitivity and specificity for the various combinations of views was determined for various categories of observers and for the various combinations of views.

 

Results  -  results for various patient age groups will be presented and recommendations made with respect to the view(s) required.

 


The Role of “Babe” in the Comparison of Image Quality and Dose.

 

D. Cianter, A>D> Cotterill, M. Firzgerald A. Pettett, J.V. Cook, G. Beluffi, S.M. Pablot, N. Perlmutter and K. Schneider.

Radiological Protection Centre, St. George’s Hospital, (QMH for Children, Epsom and St. Helier Trust, IRCCS Policlinico S. Mateo (Pavia) Hospital Univ. des Enfants Reine Fabiola (Brussells) and Klinikum Immenstadt der Munich Children’s Hospitals).

 

Introduction

Achieving diagnostic image quality at the minimum achievable dose is the goal of any imaging department.  Factors that affect quality and dose can be divided into 2 main areas, those that are due to positioning and those that are due to physical factors.

 

A study was undertaken to:

1.  assess which factors contributed most to the concept of diagnostic quality

2.  compare objective and subjective analysis of these factors.

3.  examine how these factors influenced dose.

 

Methods

711 AP CXR’s of 5 baby pigs were taken.  The pigs were of varying size and age, were anaesthetised, ventilated and kept in a consistent position while the radiographs were taken in a variety of conditions (kV, screen film speed, copper filtrations and use of a grid) which could be used in normal practice.  The mAs were varied to ensure a constant optical density (1+/- 0.2).

A test object made of aluminium, lead and soft tissue equivalent material was also exposed under identical conditions.

 

The images of the 5 pigs were analysed by 5 experienced paediatric radiologists:  using criteria similar to those produced by the CEC.  Images of the test object were digitised and analysed by a computer to produce a variety of signal to noise ratios.  The objective quality of the test object’s image was compared with the subjective quality of the pig images as assessed by the radiologists.

 

A dose measurement was obtained for each radiograph.

 

Results

Use of the modified CEC criteria resulted in an acceptable correlation between the radiologists with an average coefficient of 0.3-0.7 for the various anatomical regions.  The best correlation was for a general assessment of contrast, bony structures, trachea and major bronchi.  The correlation between the these object and the radiologists was as good as between pairs of radiologists.

 

The radiologists were only able to group the radiographs into 5 main categories, although the objective analysis was able to distinguish between 36 different various in quality.  A wide range of doses was recorded for those radiographs deemed to be acceptable by the radiologists.

 

Conclusion

Positioning any density are very important factors in producing acceptable quality and if these 2 factors are optimised, a very low dose radiograph can be diagnostic.

Good radiographic technique and practice was therefore to be stressed.

 

Subjective analysis by use of the CEC criteria compares well with objective analysis.

 

2 pig radiographs (of low and high dose) will be brought to the meeting and a prize will be given to the delegate who guesses most closely the difference in dose between the 2 images.

 

References

1.     The European Commission, European guidelines on quality for diagnostic radiographic images in paediatrics. EUR16261, EN CEC, Luxembourg (1996)

 

2.     Verdun, F.R. Bochud, F, Depeuringe, C, Desponds, L, Grecescu M, Hesssler, C, Raimondi, S and Valley, J-F Subjective and Objective evaluation of chest imaging system.  Radiation Protection Dosimentry 49 (1/3) 91-4, (1993).

 

3.     Wagner, R.F. and Sandrik, J.M.  An introduction to digital noise analysis.  The Physics of Medical Imaging.  Ed A.G. Haus (Amer. Assoc. Phys. Med ) pp 524-546 (1979).

 

4.     Loo,L-n, Doi Dand Metz, C.E.  A comparison of physical image quality indices and observer performance in the radiographic detection of nylon beads.  Phy.Med. Biol 29 (7) 837-56 (1984).

 

5.     Stender, H.St. and Stieve, F.E., Image Quality-physical and diagnostic parameters.  The radiologists viewpoint Br.J.Radiology, Supplement 18,1-8 (1985).

 

Publications

 

6.     Guidelines on Best Practice in the X-ray Imaging of Children.  Available from Queen Mary’s Hospital for Children, Epsom and St Helier NHS Trust, Wrythe Lane, Carshalton, SM5 1AA.  Tel. 0208 2963010.  Fax 0208 2963013.

 

7.     European Guidelines on Quality Criteria for Diagnostic Radiographic Images in Paediatrics.

 

8.     Radiation Protection 116. Guidelines on education and training in radiation protection for medical exposures.

 

9.     Guidelines on Patient Dose to Promote the Optimisation of Protection for Diagnostic Medical Exposures. 9NRPB).  Available form Stationary Office publications Centre, PO Box 276, London SW8 5DT.

 

10.  Making the Best Use of a Department of Clinical Radiology – Gidelines for Doctors.  Available from the Guidelines Secretary, The Royal College of Radiologists, 38 Portland Place, London.  WIN 4JQ  Fax 0207 323 3100..


 

PARENT HELD RADIATION RECORD BOOK  A FEASIBILITY STUDY

 

J. Fairhurst, M Scriven, F Seymour, P Clewer, P Jackson, L Leavesley

 

Wessex Department of Paediatric Radiology

Department of Medical Physics and Bio-engineering

 

On behalf of the Department of Health we are continuing an evaluation of the potential for recording radiation dose in a record book maintained by the parents.

 

The study commenced in 1997, logging the individual radiation doses of 500 neonates born and transferred to the neonatal unit at the Princess Anne Hospital, Southampton, with the aim of recording and subsequently analysing the radiation doses received during the first five years of life.

 

Parents of the neonates were consented and issued with a radiation record book.  The Book was structured to record the essential information from diagnostic procedures (x-ray or nuclear medicine) which would enable the estimation of radiation dose obtained from any hospital the child might subsequently attend.  An important aspect of the study is to test compliance of parents to present the radiation record book to the hospital at each and every investigation involving radiation.  In order to assess the ability to remember the existence and retrieve the record book as and when required, parents were requested to return the logbook to the Radiology Department at six-monthly intervals over a period of five years.  The 500th book was issued in April 1999 and the data has been analysed, firstly in September 1999 and more recently, September 2000.  Although 500 books were issued there are only 413 infants registered on the study.  This discrepancy comprises a mortality of 35 infants and 54 babies who for many reasons were not recruited onto the study.

 

Whilst the study has several more years to run, the early results would indicate that there is poor parental compliance.


 

Ultrasound features of the dysfunctional paediatric bladder with Urodynamic correlation

 

Aim:  Bladder dysfunction in children is an extremely important, but often difficult diagnosis to make.  The clinical history may be suggestive, but is often misleading.  Urodynamics is the accepted gold standard for diagnosing dysfunction, but it is an invasive investigation not suitable for screening.  We have looked at detailed bladder ultrasound as a non-invasive first line study to identify those children with urinary symptoms most likely to benefit from formal urodynamics.

 

Method:  Data was collected retrospectively on 73 children, 49 boys and 24 girls (age range 4-14 years), who were referred to our unit for urodynamic examination between 1993 and 1997.  All the children were symptomatic.  Bladder ultrasound scans performed at the time were then compared with their urodynamic findings.  A second asymptomatic group of 40 normal children underwent bladder ultrasound examination alone prospectively, to serve as a control.

 

Results:  Urodynamics revealed 9 (11%) children with normal studies, and 65 (89%) with abnormalities (44 with detrusor instability, 13 with bladder hypotomia, and 8 with dyssermergic bladders).  Abnormal bladder ultrasound findings included thickened bladder wall, post-micturition residual urine, trigonal “dimple” and dilated distal ureters.  All children with abnormal urodynamics had at least one of these abnormalities on ultrasound.  The mean bladder wall thickness in the urodynamically abnormal group with 6.15mm, versus 4.75mm in the normal group, and 2mm is the control group.  A widened trigonal impression or “dimple (>3mm) was seen in 83% of children with abnormal urodynamics compared with only 38% of those with normal urodynamic findings, and in none of the controls.  A significant post-micturition volume (>50ml) was present in 34% of those with urodynamic abnormality compared to only 1 child in the normal group.  Similarly, ureteric dilation (>4mm) was identified in 61% of the abnormal group, but in only 1 in the normal group.  None of the children in the control group had abnormal ultrasounds.

 

Conclusion:  Abnormalities of the bladder trigone have not previously been described in children with bladder dysfunction.  Our study found this to be a useful sign, with sensitivity of 84.6% and specificity of 50% for predicting urodynamic abnormality.  The finding of a significantly thickened bladder wall in the symptomatic group correlates well with other reports in the literature.  We were unable to show a significant difference in wall thickness between the urodynamically normal and abnormal groups, but this may have been a problem of sample size.  This study once again confirms urodynamics as the gold standard in the diagnosis of the dysfunctional paediatric bladder.  While ultrasound can in no way be viewed as a replacement, it has an important role to play in identifying those children most at risk.  It may also have a role to play in the monitoring of children being treated with this difficult condition.

 

 

A Review of Histiocytosis in the Irish Paediatric Population

 

Authors:            Brannigan M, Phelan E., Hayes R,

 Department      Radiology

 Presenter:            Margot Brannigan

 Institution:      Our Lady’s Hospital for Sick Children, Crumlin, Dublin

email:  [email protected]

 

Histiocytosis involves the proliferation of histiocytic cells and the infiltration of these cells in to normal tissues.  It consists of a spectrum of disorders which differs widely in its presentation.  Lesions may develop in any tissue, but bone, skin and lymph nodes are the most commonly affected.  More than 75% of patients develop skeletal lesions.

 

55 patients with newly diagnosed, biopsy proven histiocytosis were seen at Our Lady’s Hospital for sick Children, Crumlin between April 1980 and June 2000.  There were 37 male, 18 female patients, age range 3 weeks – 15 years (median 3 years).

 

We have reviewed the disease pattern, treatment and survival of these patients.  Thirty seven of our patients had multisystem disease and received combination chemotherapy 18 patients had unisystem disease, predominantly bone lesions, which were treated with surgery, irradiation or steroids.

 

Conclusion:  The clinical presentation of histiocytosis is very variable.  It can mimic a wide variety of metabolic, infectious and neoplastic diseases.  While it frequently behaves in a benign manner it can be a very aggressive disease and very resistant to current treatment regimens.

 

 

 

TITLE:  Magnetic Resonance Imaging of Symptomatic Vascular Anomalies

 Author:   Moji Balogun and Philip John

 Institution:  Radiology Department, Birmingham Children’s Hospital, Birmingham, UK

 

Background:  Most vascular anomalies or birthmarks can be differentiated clinically into haemangiomas and vascular malformations, however some lesions have ambiguous appearances and may require further radiological evaluation.  The purpose of this paper is to present our recent experience imaging these lesions on high field strength MR.

 

Patients and methods:  74 consecutive infants and children (age range 1 day – 17 years, mean age 7.7 years) presented over a 2.5 year period with symptomatic vascular birthmarks.  A retrospective study was made on 59/74 children undergoing MR (55 patients images on 1.5 Tesla and 4 patients on 1 Tesla systems)  using the following pulse sequences:  T1 SE (pre and post contrast), T1 GE and T2 FSE (including fat saturation) 3D GE contrast enhanced MR venography was carried out in those with low flow malformations.

 

Results:  In the 36/59 patients with vascular birthmarks classfield on clinical criteria MR confirmed the clinical diagnosis in 29 patients (10 venous, 11 lymphatic, 3 mixed venous and lymphatic malformations, 5 haemangiomas) and reclassified the birthmark in 7 patients (6 venous malformations, 1 haemangioma), MR provided a more precise assessment of lesion sub types in 12 patients with low flow vascular malformations (1 venous, 4 lymphatic, 3 mixed venolymphatic malformations) and in 1 patient with a haemangioma variant.  In the 23/59 patients where the clinical diagnosis was unclear, MR established the diagnosis in 21 (15 venous malformations, 2 lymphatic malformations and 4 haemangiomas) and was unable to adequately classify the birthmarks in 2 patients.

 

Conclusions:  MR imaging is useful in evaluation symptomatic vascular birthmarks.  MR establishes the diagnosis in the majority (91%) of clinically ambiguous lesions.  In children with clinically diagnosed vascular birthmarks, MR confirms the diagnosis in 80%, provided further information of low flow vascular malformations in 33% and can lead to a change in diagnosis of the birthmark in 20%.




SPONASTRIME DYSPLASIA:  EXPANSION OF THE INFANTILE PHENOTYPE

AC Mbamali, M Lees, RM Winter, CM Hall

Departments of Radiology and Clinical Genetics

Hospital for Sick Children,  Great Ormond Street, London, WC1N 3JH

 

 The term for this relatively new dysplasia was coined by Fanconi et al in 1983;  it is an acronym derived from spondylar and nasal abnormalities with striations of the metaphyses of the long bones.  Fewer than 20 cases have been published in the literature, of which at least 3 are not believed to have the characteristic radiological findings of sponastrime dysplasia.  We expand the infant phenotype by presenting the clinical and radiological findings in a Caucasian female from birth to age 4.5 years.  Only one other infant patient has previously been published.  On the basis of these two cases, we conclude that characteristic radiological findings are present early on, which may allow the diagnosis of sponastrime dysplasia to be made in infancy.

 

 

 SPONASTRIME DYSPLASIA:  PRESENTATION IN INFANCY

      Offiah AC, Lees M, Winter R, Hall CM

      Great Ormond Street Hospital

      November 2000

 

INTRODUCTION

      Fanconi et al 1983

 

·      SPOndylar

·      NASAi

·      STRIiated

·      MEtaphyses

 

BACKGROUND

·      Rare: only 12 other reported cases

·      Of these 12, only one in infancy

·      In our case, diagnosis made retrospectively at 5 years of age, but films from birth available.

·      Review of radiological findings from birth to 5 years.

 

CASE REPORT

ANTENATAL?NEONATAL

·      First child

·      Health non-consanguineous parents

·      20 week US-? Achondroplasia

·      CS (breech presentation)

·      Birth weight 2.46Kg (<3rd centile)

·      Birth length 44 cm (<3rd centile)

 

CASE REPORT

INFANCY/CHILDHOOD

·      Midfacial hypoplasia

·      Saddle – shaped nose

·      Significant joint laxity

·      Normal intelligence

·      46XX

 

CASE REPORT

INFANCY/CHILDHOOD

·      Significant rhizomelic shortening

·      Height and weight below 0.4th centile despite GH therapy

·      Progressive kyphoscoliosis

 

RADIOLOGICAL FINDINGS

(Spine – Neonate)

·      Straight

·      Progressive reduction in interpedicular distances from L1 to L5

·      Platyspondyly


RADIOLOGICAL FINDINGS

(Spine – 5 months)

·      Significant platyspondyly

·      Increased height of anterior portions of vertebral bodies

·      Bullet – shaped.

 

RADIOLOGICAL FINDINGS

(Spine – 2 years, 6 months)

·      Less marked platyspondyly

·      More obvious junction between anterior and posterior portions of vertebral bodies.

·      Posterior scalloping

 

RADIOLOGICAL FINDINGS

(Spine – 4 years, 8 months)

·      Significant kyphoscoliosis

 

RADIOLOGICAL FINDINGS

(Hips – 6 months)

·      “Spanner–like” appearance of proximal femora

 

RADIOLOGICAL FINDINGS

(Hips – 13 months)

·      “Spanner–like” appearance still evident.

·      Coxa vara

 

RADIOLOGICAL FINDINGS

(Hips – 3 years, 1 month)           

·      Progressive coax vara

·      “Spanner-like” appearance lost

·      Dislocated left hip.

 

RADIOLOGICAL FINDINGS

(Knees – 2 years 6 months)

·      Mild irregularity of epiphyses

·      Metaphyseal striations

 

RADIOLOGICAL FINDINGS

(Upper limbs – 13 months)

·      Rhizomelic shortening

·      Flared distl humeral metaphyses

 

RADIOLOGICAL FINDINGS

(Upper Limbs – 4 years 8 months)

·      Rhizomelic shortening

·      Metaphyseal flaring less obvious

·      Striated distal radial metaphysic

·      ? humeral focal fibrocartilaginous dysplasia

 

RADIOLOGICAL FINDINGS

(Bone Age at 4 years 8 months)

·      Delayed:  2 years 6 months

       (-2SD)

 

RADIOLOGICAL FINDINGS

(MCCP – 4 years 8 months)

·      Up and down pattern variation

·      Pattern variability index 0.51

(>o.7 – dysmorphogenesis)

·      Brachymetacarpy

(normal Z score range +2 to -2)

 

SPONASTRIME DYSPLASIA

·      Autonormal recessive/germ line mosaicism

·      Characteristic facies and normal intelligence

·      Diagnostic radiology in infancy

·      Suggested nomenclature:

Spondylooemetaphyseal

      Dysplasia (semd) with midface hypoplasia

      and depressed nasal bridge.

 


TITLE:   Acute Limb Ischaemia in Children

 

AUTHOR:   Philip John

 

INSTITUTION:  Radiology Department, Birmingham Children’s Hospital, Birmingham, UK

 

Background:  Acute limb ischaemia in children is uncommon and most reported cases follow femoral artery catheterisation.  The purpose of this paper is to present our recent 12 month experience and outline our angiographic and thrombolytic practice in such cases.

 

Patients and methods:  A retrospective study covering a 12 month period beginning 08/1999 was carried out on six consecutive patients (age range 4 wk – 13 years) referred to radiology with acute limb ischaemia (lower limb in 4 and upper limb in 2).  Angiography was carried out in 5/6 patients (catheter angiography in 2, catheter and MR angiography in 2, MR angiography in 1) and Doppler ultrasound in 1.  Systemic heparin was given in all, thrombolytics in 4 pt, intra-arterial vasodilators in 3 pt, intravenous prostacycline in 2 pt and oral steroids in 2 pt.

 

Results:  Acute limb ischaemia followed vascular interventions in 5/6 cases;  surgical interventions in 2 (renal transplant and axillary vein graft), Radiological interventions in 2 (femoral artery intervention and hand sclerotherapy) and medical intervention in 1 (umbilical arterial catheter removal).  The cause of acute vessel occlusion was thrombosis (in 2), embolisation (in 2), dissection (in 1) and systemic vasculitis (in 1).  Local thrombolysis was ineffective in 2 pt. and systemic thrombolysis provided some improvement in 2 pt.  Surgery was undertaken in 4 (revascularisation in 3, amputation in 1) and 2 were treated conservatively.

 

Conclusions:  In our series vascular interventional treatments are the commonest cause of acute limb ischaemia.  Once diagnosed early referral and active multidisciplinary management is essential to achieve satisfactory results.

 

 

MRI FEATURES OF EARLY JUVENILE IDIOPATHIC ARTHRITIS

 Authors:  k Johnson, B Wittkop, S. Ehtisham, T Southwood, C Ryder, J Gardner-Medwin, S Chapman, F Haigh.

Birmingham Children’s Hospital

 

Introduction:  Juvenile idiopathic arthritis (JIA) covers a heterogenous group of conditions.  Magnetic Resonance Imaging (MRI) is widely regarded as the most sensitive modality for the investigation of this disease.  To date, MRI in JIA has documented a wide variety of imaging findings but the patient groups studies have included both acute and chronic cases some of whom have received different treatment regimen.  There is at present no published data on the value of MRI and the imaging findings in newly diagnosed (within 9 months), untreated cases of JIA.

 

Method:  We retrospectively reviewed the MR imaging findgs of the knee joint in all newly diagnosed JIOA patients between 1998 and 2000.  These patients had clinical evidence of disease for less than 9 months and had not received any treatment.  The examinations were reviewed independently by two experienced Consultants Radiologists.  14 MRI scans of the knee were reviewed.  All examinations were done pre and post contrast.

 

Results:  The post contrast images significantly improved the detection of pathological abnormalities.  Significant findings were;  lymphadenopathy was (present in over 90% of examinations) and enhancing synovium (present in all patients and seen in the supra patella bursa in all cases).  Other locations of enhancing synovium throughout the rest of the knee varied.  No erosions were seen in any patient.  There was relatively good preservation of articular cartilage in all patients.  There were no specific bone marrow changes demonstrated.

 

Conclusion:  MRI of the knee is very valuable in the investigation of JIA.  The use of i.v. gadolinium in patients suspected of JIA is mandatory.  The site and size of effusions and inflamed synovium are helpful in refining the differential diagnosis of inflammatory activity in patients.  Knowledge of the early features of JIA is useful to monitor disease progression and treatment response.