• Diagnosis
• Fracture history
• Medication use (steroid, anticonvulsant, and bisphosphonate)
• Status of hips (dislocation, past surgery)
• Presence of nonremovable orthopedic or internal hardware and location (plates, screws, rods, baclofen pump, button, tracheostomy)
• Tubes (ventriculoperitoneal shunt, feeding tube, portacath)
• Scoliosis
• Use of contrast material for medical tests in the past 7 days may affect total body and lumbar spine values and require rescheduling
Positioning and Artifacts
It can be difficult if not impossible to scan the recommended body sites of whole body and PA lumbar spine in children who have joint contractures or limited mobility, dislocated hips, severe scoliosis, compression fractures, and limited range of motion, as can be seen in children with CP, muscular dystrophy (MD), certain genetic syndromes and conditions, and OI (Fig. 9.1). Movement disorders, lack of understanding due to limited cognitive abilities, and the inability to lie in a required position can pose additional challenges to obtaining usable scans. Lying flat on the back for the duration of the scan time, for instance, as is required for the total body scan may not be possible for some patients who have severe gastroesophageal reflux or respiratory compromise in that position. Severe scoliosis, kyphosis and/or lordosis can make appropriate positioning impossible for technically valid lumbar spine measurements.
Fig. 9.1
Total body DXA in a 17-year-old male with CP. Head motion, contractures, severe scoliosis, and dislocated right hip are evident. Total body less head values unaffected by head movement
The presence of internal metallic devices or hardware causes a fictitious increase in BMD results. Metallic artifacts commonly seen in children with disabilities can include any combination of rods, screws, plates, clips, and baclofen pumps (Fig. 9.2a, b). Other artifacts that can affect BMD include movement, positioning aids (wedges, pillows, sandbags), and tubes (IV’s, button, ventriculoperitoneal shunt, feeding tube, portacath). When performing serial scans on patients with nonremovable artifacts, careful attention should be paid to the appearance and position of the artifact which would affect the ability to compare serial studies and interpret change in BMD over time. If the artifacts are external (e.g. a lead running from the patient to a machine), the position of the artifact should be replicated to ensure better reproducibility.
Fig. 9.2
Metallic artifacts frequently encountered when measuring BMD in children with neuromuscular disabilities. (a) Total body DXA in 11-year-old female with OI: note spinal fusion and fixation of left forearm and lower extremities. Note increased density in distal femurs and proximal tibias due to bisphosphonate treatment. (b) Lumbar scoliosis and indwelling baclofen pump in lower right corner of scan on lumbar spine DXA in 18 year old male with CP. Scan successfully accomplished avoiding overlap of metallic pump with edge of vertebral bodies
It is common to encounter a number of complicating factors in children with disabilities that can compromise scan quality or negate the value of the scan. Some DXA body sites are more prone to be problematic, such as the whole body. Other body sites, while obtainable, may give information of limited clinical value. Positioning concerns and the presence o f artifact must be considered in scan site selection.
Body Sites to Measure
Recommend body sites to measure by DXA in children are addressed in Chap. 5, and include the total body (less head) and PA lumbar spine. Other body sites such as the lateral distal femur and forearm may also be useful sites to measure by DXA in specific circumstances [22]. Alternative sites are sometimes the only sites that can be measured and for some populations may provide the most clinically relevant information about BMD in these children. This section describes the circumstances commonly encountered while measuring children with special needs at the common and alternative pediatric DXA body sites (Table 9.2). Site-specific influences noted in Table 9.2 do not always mean that a measure from that site should be disregarded; rather, it should be accounted for in interpretation. It is important to obtain scans on as many body sites as feasible during the initial exam, because a body site may become of limited value over time as fractures occur or metal rods or hardware are inserted. Subregion analysis of a body site may allow the continued use of that site over time.
Table 9.2
Conditions encountered when scanning children with disabilities, effect on DXA scan, and body site affected
Condition encountered | Problem created with scan | DXA site affected |
---|---|---|
Excessive movement | Movement artifact | All; least likely to affect LDF |
Contractures | Inability to place correct regions of interest | TB; H |
Scoliosis | Inability to place correct regions of interest | TB; LS |
Metallic implant | Artifact—metal adds density | All; implants least likely in LDF |
Feeding tube | Artifact—adds density or area | TB; likely LS |
Baclofen pump | Artifact—metal adds density | TB; possibly LS |
High mus cle tone | Positioning problems; unable to lie flat | TB; H; rarely LDF |
Startle reflex | Movement artifact when table and/or arm moves | All; most likely TB |
Obesity | Patient exceeds width of table; excessive tissue thickness | TB; possibly LS |
Cognitive delays | Patient unable to understand instructions | All; most likely TB |
Dislocated hip | Atypical anatomy invalidates landmarks for valid analysis | H; possibly LDF |
Recommended DXA Sites for Children
Lumbar Spine
Many patients with disabilities can cooperate for lumbar spine scan acquisition due to a short scan time (<30 s) and a stable position. The position required is comfortable for most because while patients are still on their back, their hips and knees are in flexion and the knees are supported on a foam block which has the net effect of creating more trunk stability and less stress on contracted muscles with limited range of motion. Gastroesophgeal reflux and respiratory concerns may still be present, though they are typically not a concern due to the very short scan time. However, scoliosis, the presence of abdominal artifacts (tubes, shunts, pumps), and spinal compression fractures, and atypical skeletal morphology, as seen in the skeletal dysplasias, all have an effect on the scan analysis and must be considered for scan interpretation. Spinal fusion instrumentation to stabilize scoliosis may be present in the lumbar region and preclude measurement of some or all of the vertebral elements. Orthopedic hardware will artificially elevate BMD results.
Total Body
Total body is usually the most problematic body site to measure by DXA in children with special needs for several reasons. The total body scan takes the longest to perform, running 1.5–55 min depending on the scanner used and size of the patient; holding still for the time required for scan acquisition can be challenging for young children, those with limited cognitive ability, and for patients with positioning limitations. Because of the longer scan time, this body site is most susceptible to movement artifact. A valid total body DXA scan requires the patient to lie flat on the scan table, which can be impossible for children with contractures. For many children with disabilities, lying supine creates discomfort and may be contraindicated due to gastroesopageal reflux or respiratory compromise that may be exacerbated by the position. Some children with special needs, particularly those with sensorimotor issues or high startle reflexes (as seen in spasticity) feel unstable on their back and fear that they may fall off the edge of the table. Indwelling artifacts from metallic hardware or tubes are frequently present somewhere in the body and because the entire body is being scanned, all nonremovable medical equipment in or on the patient will be evident on the scan and will affect results by elev ating BMD values.
Alternative DXA Sites
Forearm
The forearm DXA scan is useful for children who exceed the weight limit of the table or who have metallic or other artifacts in body parts measured at other scan sites. In a group of 90 children with repeated forearm fractures, forearm DXA results (BMD and BMC) were found to be significantly reduced [23]. Usefulness of the forearm for monitoring site-specific changes has been demonstrated, and it correlates well to strength indices of peripheral quantitative computed tomography [24]. Compared to total body and spine, the forearm scan in children has the poorest precision [25]. The forearm is a resident scan modality for scan acquisition and analysis on bone densitometry machines. Normative data are available for children, but are limited to the Hologic scanner [26, 27].
The position requires the patient to place their forearm in the center of the scan table, typically leaning from a seated position alongside the table. For small children with special needs, the positioning may involve the child laying supine on the scan table with the arm extended. The hand must be flat on the scan table which can be problematic for children with hand contractures. If arm range of motion is limited due to contractures, extension of the arm in order to reach the proper position on the table may not be possible. For more adult-sized obese patients, like teenage boys with Duchenne Muscular Dystrophy, it may not be possible for the arm to reach the required position on the scan table due to excessive soft tissue around the torso, limited arm reach, and pain.
Lateral Distal Femur
The lateral distal femur (LDF) was first described by Harcke et al. in 1998 and was developed specifically to measure BMD in children for whom the traditional sites were invalid or unobtainable due to the presence of hardware, contractures, scoliosis, or excessive movement [28]. The distal femur was chosen because it is the bone most likely to fracture in non-ambulatory children and therefore is the most clinically relevant site to assess [18, 29]. The lateral body placement allows for a comfortable, side-lying position for scan acquisition and it is well tolerated in children with a variety of disabilities including CP, SB, DMD, OI, spinal muscular atrophy, Rett syndrome, Pelizaeus-Merzacher Syndrome, and Mucopolysacchridosis IV (Morquio A & B) [9, 16, 19, 30–37]. Many times it is the only viable and valid site to measure in children with special needs [4].
The LDF scan modality is not available on any DXA scanner, so the scan is acquired in the forearm mode. Because the scan modality does not exist on any DXA system, specialized training on scan acquisition and scan analysis is required; acquisition and analysis techniques vary depending on manufacturer. Several pediatric centers in the United States and internationally have been trained and regularly utilize the LDF DXA scan. For scan analysis, the DXA technologist manually creates three subregions of interest for the LDF using a standardized approach that was designed to accommodate changes in bone due to growth. In addition, a correction factor is applied to adjust for technological system upgrades that can affect the aspect ratio of the scan image [38]. The three regions of interest represent three types of bone: Region 1, adjacent to the growth plate is the metaphyseal region, comprised primarily of trabecular bone; Region 2 is the metadiaphyseal region, and is a balanced aggregate of cortical and trabecular bone; and Region 3 is the diaphyseal region, comprised primarily of cortical bone (Fig. 9.3). Because Region 1 is primarily cancellous (trabecular) bone, it is the most sensitive area for noting bone turnover (gain or loss). The growth plate, where endochondral bone formation occurs, is highly affected by bisphosphonates [30].
Fig. 9.3
LDF DXA analysis protocol requires placement of 3 subregions of interest: Region 1 (distal metaphysic) is primarily trabecular bone ; Region 2 (metadiaphyseal) is mixed cortical and trabecular bone; and Region 3 (diaphyseal) is mainly cortical bone. Note movement at the top of the scan beyond regions of interest: patient was able to hold still long enough for successful scan acquisition
The position requires the patient to lie on their side for the leg that is being measured. Positioning aids (foam blocks and a sand bag) are frequently used to elevate and support the leg that is not being measured. Bilateral measurements are recommended because there are side-to-side difference in non-ambulatory children and a high likelihood that one femur may fracture sometime during childhood [39, 40]. Lower extremity fracture is the most common site of fracture in non-ambulatory children with CP and within the lower extremities, the majority of are distal femur fractures [29, 41–43]. Most patients tolerate the position very well, as it is similar to a side-sleeping position, and when properly positioned, very stable and less susceptible to movement than other DXA sites (Fig. 9.4). However, some patients with dislocated hips may have difficulty lying on their side. Scan acquisition time is 20–40 s. Normative data are available for children, but are limited to the Hologic scanner [44].
Fig. 9.4
The side-lying position required for LDF DXA acquisition is comfortable for children with disabilities. The leg being measured (closest to the table) is positioned with femoral shaft following the long axis of the table. LDF lateral distal femur, DXA dual energy X-ray absorptiometry
More information about the LDF can be obtained at www.lateraldistalfemur.org.
Strategies for Scanning Children with Special Needs: Measurement and Analysis
When acquiring a DXA on a child with special needs, it is usually helpful to have the family or care providers stay in the room to help to reassure the child and assist with moving and positioning the child. They also usually know best how to help their child relax. If the child has a movement disorder , extra hands may be needed to help to hold the child during the scan. Prior to scanning, the DXA technologist should inform the child and care providers about what will happen during the exam, and how the machine moves and sounds during the scan.
Patients can present with chronic pain, dislocated hips, and the inability to move or control extremities. Large body habitus and knee contractures pose a challenge in positioning children with special needs—some are unable to fit within the parameters of the scanning table. If the patient exceeds the scanning width of the table, as can occur with large patients with Duchenne’s Muscular Dystrophy (DMD) or in patients with knee flexion contractures, an approach called “offset scanning” should be used [45]. In offset scanning, the patient is positioned so the axis of the body is offset from the center axis of the scan table, thereby allowing one side of the patient to lie beyond the width of the scan table and ensuring that one side of the patient is completely scanned. If the patient has knee and hip contractures that will not allow them to straighten their legs for the total body measurement except in a wind-swept or frog-legged position they can lie flat on the table; these leg positions can still yield valid total body scans. Similarly, if the child is not able to lie or keep their arms by their sides for the total body, care must be taken to ensure that the arms and hands remain flat on the table in whatever position is comfortable; care must be taken to keep the hands out the head region of interest for valid total body less head results.
Experienced bone densitometry technologists who are accustomed to acquiring images on children with special needs are familiar with ways to decrease anxiety for children and accompanying care providers. General tips for obtaining scans on children with disabilities are listed in Table 9.3. It is imperative that positioning is consistent at every body site measured to create valid, reproducible serial scans (described in Chap. 5).
Table 9.3
General tips for scanning children with disabilities
Tip | Effect |
---|---|
Explain and show how the scanning table/arm moves | Helps the child know what to expect—see how machine moves; hear machine sounds |
Low noise, dim lights | Lessen startle reflex from noises; calming atmosphere to promote relaxation |
Parent/care provider stands beside child at head of table | Assures child that they will not roll or fall off of the machine table. Can provide help holding the child, if needed, for positioning and reassurance |
Use sheet on table | Used as draw sheet to help with positioning |
Have positioning aids handy | Pillows, foam blocks, wedges, sandbags can be useful for positioning, stabilization, and comfort |
Take time; do not rush | Relaxed technologist = relaxed parent/care provider = better chance of relaxed child |
Wait for patient to get used to new position | Tone is higher after repositioning; wait for tone to decrease |
Avoid conversation during scan acquisition | Discourage response by child during scan to minimize movement and excitability. Parent/care provider should sing or calmly talk continuously to child to keep them relaxed and engaged |
Wait until entire exam is complete to celebrate and shower child with praise | Share in great joy when study is complete! Calm, positive encouragement given after each scan during study. Excessive excitement between scans increases tone and child loses focus |
Hands on | Strategically placed hands help stabilize, prevent movement artifact, and relax the child |
Have scan ready to start before posi tioning | Quickly scanning once patient is positioned requires patient to hold position for less time |
During scan analysis , technologists should carefully review bone mapping and ROI placement, particularly if auto-analysis was used, making any necessary corrections. Errors are more apt to occur with auto-analysis when measuring atypical anatomy or with very low bone density. During analysis of whole body scans acquired using the “offset” technique, the values of the side that was completely scanned are then mirrored for the extremities that were not included on the scan. This approach is deemed to be more accurate than ignoring the body parts that were excluded from the scan. The way that this “offsetting” is accomplished varies by machine brand: one manufacturer’s machine allows for replacement of one limb exhibiting large differences in bone density with the BMD values from the contralateral limb and another manufacturer’s software uses a mirroring technique of half of the body scan [45]. The general tips listed on Table 9.3 apply to any body site being measured.
Specific Scanning Tips by Body Site
Following are specific tips, listed by body site, which can be helpful when measuring children with special needs.
Lumbar Spine
The lumbar spine position is generally more comfortable and easily obtained than the total body. This provides an immediate success and lets the child understand the sounds and movement of the machine, which relaxes both child and care-provider and can help for the remainder of the exam. It is important to consider how well the child can control his legs. If muscle control is poor or nonexistent or if the child has uncontrolled movements, the technologist should keep his or her hands on the patient’s legs during the scan and whenever the legs are on the positioning block to ensure patient safety. If the child has a g-tube, move or remove the extension tubing so that it is not overlapping the spine. If a baclofen pump is present and overlaps the edge of a vertebra on the scan, repositioning the child may decrease or eliminate overlap on the spine. Because the scan time is very quick, it can be useful to perform this scan first (Fig. 9.2b).
Total Body
The whole body is generally the most difficult to acquire and the most challenging scan to analyze. Correct ROI placement may not be attainable depending on the positioning limitations of the patient (Fig. 9.1). Attempting to acquire this scan last will allow the child to get used to the sounds and movement of the machine, which can help them relax and decrease muscle tone for better positioning and reduce movement. It will also help the parent or care provider relax, which in turn helps the child to relax. It may not be possible to acquire a total body scan and successfully acquiring other scans first will make success in acquiring the total body less stressful. Assess for attached artifact-producing items and remove as many as possible. For example, some patients with SMA will continuously wear a pulse oximeter. Determine if it is possible to remove it for the scan duration. If it is not possible, minimize the amount of the lead on the table, and ensure that the device itself is not on the scan table.
The technologist should be familiar with the scan acquisition pattern of the machine and use this to determine how they make accommodations with explanation and/or strategic holding of body parts. For instance, some scanners acquire the total body scan in several full-length passes of the table, moving across the table from the patient’s right to left. If the child is able to understand and can answer when asked if they will be able to hold their arm down by their side for a few seconds while the scan arm passes over that area, they may be able to temporarily hold that position, and then once that arm has been scanned, the technologist can carefully move the arm to a more comfortable position as long as it is in the scan field that has already been acquired. Tape can be useful to help the child temporarily hold a position, as long as the technologist has verified that it would be permissible to use tape and that the patient does not have a tape allergy. In order to position appendages flat on the table within the width of the table, and not overlying other body parts, it may be necessary to strategically move appendages during the scan. This requires explanation to the child that you will help them by doing this, their agreement, and the willingness of the technologist to be hands-on. It also requires the technologist to carefully keep an eye on both the scan arm as the scan is being acquired to see where the X-ray beam is relative to the body, and the computer screen to make sure the image is being acquired properly. Explaining to the child what is happening, when they might be touched or moved, and when the machine will move is important. For some children with high muscle tone or a strong startle reflex, the act of touching or moving one body part will cause inv oluntary movement in another. This possibility must be assessed prior to starting the scan.