Appendix 1 A UK questionnaire auditof imaging protocols for suspected skeletal dysplasia
Objective
To determine current practice regarding imaging protocols for skeletal dysplasias among members of the British Society of Paediatric Radiologists (BSPR) with a view to proposing standardisation if required.
Skeletal dysplasia group (SDG) members noted a wide range of protocols being used for imaging suspected dysplasias and proposed formulation of standardised guidelines. However, it was believed to be important to audit current practice first to identify the published guidelines in use, for which purpose a questionnaire survey was created.
Aims
The aims of the questionnaire were to establish:
- The number of skeletal surveys performed for suspected dysplasia per year at individual hospitals.
- Who reports the skeletal surveys and how frequently these are referred for external expert opinion.
-The prevalence of written protocols.
-The source of written protocols, where these are in place.
-Current practice regarding use of babygrams, cervical spine imagingand imaging family members.
Method
An Internet-based questionnaire was generated using the Survey Monkey online tool and sent by e-mail in September 2013 to all 250 members of the British Society of Paediatric Radiology (BSPR). The membership primarily composes practicing paediatric radiologists, and radiologists with a special interest in paediatrics within the UK and Ireland. The survey deadline was one month after the initial e-mail.
Results
There were 55 respondents (22%) from 49 hospitals. Duplicate responses from the same hospital were excluded.
Demographics
Of the responding hospitals, 47% (23/49) were district general hospitals, 33% (16/49) were specialist children’s hospitals, 14% (7/49) were university teaching hospitals and 6% (3/49) were tertiary referral centres.
Fifty-five percent of responding hospitals image fewer than 10 cases per year of suspected skeletal dysplasia.
Forty percent of responding hospitals (20/49) do not have a dedicated paediatric radiologist but all have a general radiologist with a specialist interest in paediatrics.
Reporting policies
In 10% of responding hospitals (5/49), skeletal surveys are routinely referred for reporting at another centre. In 20% of centres (10/49) a general radiologist reports the surveys. In the remainder, either paediatric radiologists or a combination of paediatric and general radiologists report the surveys.
Survey protocols
Seventy-one percent of hospitals (35/49) have a written protocol for skeletal dysplasia surveys.
The protocol source was variable. 24% of all responding hospitals (12/49) use a published protocol. The remainder use protocols created locally (18%) or from an unknown source (28%).Ten hospitals (20%) commented that the specific views obtained were usually tailored to the suspected diagnosis, and therefore the views obtained in each case were different with no routine dysplasia protocol in place.
Some hospitals also use condition-specific protocols, e.g., imaging the cervical spine in all cases of suspected mucopolysaccharidosis.
Follow-up imaging
Six percent of centres (3/49) routinely perform a follow-up survey of abnormal sites. Where centres stated they perform follow-up imaging (7/49), either routinely or in selected cases, all stated they waited at least a year to reimage.
Cervical spine imaging
In cases where odontoid hypoplasia/aplasia is suspected, 24% of centres (12/49) stated that they routinely perform flexion/extension views. Where a cervical spine abnormality is detected, 22% of centres (11/49) routinely perform follow-up MRI.
Babygrams
Seventy-three percent of centres (36/49) never perform babygrams on live infants, reserving them for postmortem imaging only.
Of the hospitals performing babygrams, 54% (7/13) perform a single view only with 38% (5/13) performing separate skull view and 15% (2/13) performing separate views of the hands/feet.
Imaging family members
None of the responding centres has protocols for imaging siblings and other family members although these may be undertaken following specific request from a clinician.
Appendix 2Audit of radiographs referred for tertiary opinion in cases of suspected skeletal dysplasia
Method
Skeletal survey images transferred by Image Exchange Portal (IEP) to Great Ormond Street Hospital during a 2-year period were reviewed. Any surveys performed for suspected physical abuse or oncological indications (such as Langerhans cell histiocytosis) were excluded, leaving only surveys performed for suspected dysplasia. The views available at the time of reporting were recorded, along with clinical indication and reported findings. When analysing results, dedicated separate views were not considered essential if the views provided were sufficient for diagnosis, i.e. a single thorax, abdomen and pelvic view would be counted as including chest, pelvis and AP thoracolumbar spine. A right-hand view or hand included with an arm view would be counted as adequate if the left hand was not imaged.
Results
One-hundred-eleven patients were identified. Six were excluded from the study: Five were adult patients and one was a follow-up survey.
One-hundred-five patients, referred from 44 institutions, were included in the study.
Median age was 2 years 4 months.
Indications
A range of indications – the most common being short stature (21%).
Evidence of dysplasia
In 39% of the examinations, there was definite evidence of skeletal dysplasia. In 16%, there was a possible dysplasia.
The most common diagnosis was osteogenesis imperfecta (10 possible cases = 8%).
The next most common were dysostosis multiplex/mucopolysaccaridosis (4 cases = 4%) and achondroplasia (3 cases = 3 %)
Number of exposures
The number of separate exposures acquired for each survey ranged from 3 to 23 (median: 10).
Omitted views
With reference to the suggested skeletal survey protocol suggested by Offiah and Hall in Paediatric Radiology (2003)[2], 61% of surveys were considered incomplete. The most commonly omitted views were:
Body part / Frequency of omissionAP skull / 35%
AP spine / 30%
Hand / 17%
Lateral spine / 17%
Chest / 15%
Lateral skull / 12%
Pelvis / 9%
Lower limb / 6%
Upper limb / 3%
Additional views
There were also additional views obtained for many patients – 49% included dedicated dorsal-palmar views of the feet (51 patients), which are not considered necessary as part of the standard routine skeletal survey due to the wide variation in normal appearances.
Definite or possible dysplasia was identified in 26 cases (51%). After review of these 26 diagnoses, the authors concluded that the foot views were non-contributory in all cases.
In one of the 105 cases, further foot views and a chest radiograph were suggested as they were not included on initial survey. Our differential diagnosis was probable hyperparathyroidism/neonatal rickets or I cell disease.
One further case was diagnosed as split hand/foot malformation with long bone deficiency 1, FATCO. This diagnosis was made without dedicated dorsal-plantar foot radiographs.