TÀI LIỆU HỘI NGHỊ KỸ THUẬT ĐIỆN QUANG VÀ Y HỌC HẠT NHÂN VIỆT NAM LẦN 7 (2019) Nguồn: https://hinhanhykhoa.com/

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TECHNIQUE IN PEDIATRIC BRAINTECHNIQUE IN PEDIATRIC BRAIN
MSUD-MAPLE SYRUP URINE DISEASE
SIEMENS AMIRA 1.5T
NGUYEN DINH PHUONG
DIAGNOSTIC IMAGING DEPARTMENT- NATIONAL CHILDREN’S HOSPITAL
OVERVIEW
- MSUD- Maple Syrup Urine Disease, also known as branched-chain ketoaciduria, is a
recessive genetic disease on autosome, metabolic disorder of branched chain amino
id BCAA O th ld MSUD t i 1/185000 i f t I Vi t N MSUD t did ’tacid-BCAA. On the world, MSUD rate is 1/185000 infant. In Viet Nam, MSUD rate didn’t
record.
- The classic presentation occurs in the neonatal period with developmental delay, failure
to thrive, feeding difficulties, lethargy, irritation, and maple syrup odor in the cerumen and
urine, and can lead to irreversible neurological complications, cerebral edema, coma,
respiratory failure, and death if left untreated. If diagnosed and applied a special diet,
clinical outcomes are generally good in patients where treatment is initiated early.
- MRI with routine sequences, Diffusion (Resolve) and MRS is very important to
detection MSUD early.

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CONTENT
- A classic MSUD case was diagnosed by Amino Acid Quantification
Testing at Vietnam National Children’s Hospital.
- Evaluated injury brain in acute and development phase by Siemens
Amira 1.5T. With routine sequences T2SE, T2FLAIR, T1SE, Diffusion
(Resolve) and Spectroscopy sequence (MRS).( ) ( )
CLINICAL CASE
Hi t f th l- History of mother: normal
- Infant: female, 2900gr, full-term. fifth day: baby don't breastfeed, difficult
to breath, confusion, test cetone urine (++++) a province Pediatrics
Hospital. At National Pediatric Hospital, baby needed dialysis and
follow MSUD. 01 months after apply treatment, patient is stable.
- MRI scan brain at Diagnostic Imaging Department- Vietnam National
Children's Hospital.
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INDICATIONS AND CONTRA-INDICATIONS
1. Indications:
- Maple syrup urine disease (MSUD)
2. Contraindications:
- Any electronic, magnetic equipments or implants (For example:
pacemaker implants inside brain IAC hearing aids )pacemaker, implants inside brain, IAC, hearing aids…).
- Any magnetic tools.
PREPARATION
3. Patients
- Patient's family must to sign consent form before
come in scan room.
- Explain examination for patient's family.
4. Equipments
Si A i 1 T- Siemens Amira 1.5T.
- Vital signs monitor.
- Coil Head/Neck 16.

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POSITIONING
5. Patient position
- Supine.
- Head is at coil center, use the cushion to reduce motions.
- Fill patient's information, select protocol.
SEQUENCES
1. Localiser
2. T2_SE_Axial
3. T1_SE_Sagittal
4. T2_Dark fluid_Coronal(FLAIR)
5. Diffusion(resolve) Axial5. Diffusion(resolve)_Axial
6. Spectro (MRS)

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SEQUENCES AND PARAMETERS
1. T2_SE_ Axial_4mm
TR
(ms)
TE
(ms)
FOV
(mm)
Slices Slice
(mm)
Matrix
6030 86 150 24 4 320x320
SEQUENCES AND PARAMETERS
2. T1_SE_Sagittal_4mm
TR
(ms)
TE
(ms)
FOV
(mm)
Slices Slice
(mm)
Matrix
500 7.7 150 24 4 256x256

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SEQUENCES AND PARAMETERS
3. T2_FLAIR_Coronal.
TR
(ms)
TE
(ms)
TI
(ms)
FOV
(mm)
Slices Slice
(mm)
Matrix
9000 92 2500 150 24 4 256x256
SEQUENCES AND PARAMETERS
4. Diffusion(Resolve)
TR
(ms)
TE
(ms)
FOV
(mm)
Slices
Slice
(mm)
Matrix B-Valuce
4200 67 150 24 4 150x150 0/1000

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5. SPECTRO-(MRS)
PARAMETERS AND IMAGES
- Single Voxel Sequences (SVS) TE=30ms and 135ms.
- Position ROI at injured areas: Posterior limb of internal capsule,
lentiform nucleus.
- Use Sat band to avoid noise signal around ROI.
6. SVS TE 30 7. SVS TE 135

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Before treatment
After treatment
Before treatment
After treatment

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Before treatment
After treatment
Before treatment
After treatment

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Before treatment
After treatment
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DISCUSSION
- Pediatric MRI is a complicated method because patient uncooperative,
need to sedate or anaesthetize through scan time. Brain of pediatric
patients is smaller than adult.
- Patient's body is small and don't have optimize coil.
- The role of Radiology Technologist is very important, we must to adjust
parameters: FOV, Matrix,... that accord with patients and get highparameters: FOV, Matrix,... that accord with patients and get high
quality images.
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DISCUSSION
- Advanced DWI (Resolve) is very important to detect early MSUD- Advanced DWI (Resolve) is very important to detect early MSUD,
because Advanced DWI (Resolve) give high resolution, easier to see
small lesion, decrease artifact and correct distortion, result to higher
quality images than routine DWI.
- Evaluate development or regression of disease based on restricted
diffusion or not.
DISCUSSION
- MRI with special sequences and MR Spectroscopy is important to detect
early MSUD help pediatricians to select optimize treatment MRS appliedearly MSUD, help pediatricians to select optimize treatment. MRS applied
before dialysis to diagnostic; evaluate lactate peak, NAA/Cr ratio, BCAA
peaks.
- In classic MSUD and clearly lesion cases, we use SVS 135 and SVS 30
sequences for MR Spectroscopy. With lesion is not clear, we should to use
CSI sequencesCSI sequences.
- MRS evaluate metabolism peaks, include: Lactat at 1.3 ppm, NAA at
2.0ppm, Cr at 3.0ppm, BCAA include Leucine, Valine, Isoleucine at 0.9 ppm.

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RECOMMENDATION
- With infant, we should to use T1 Flair sequence to suppress fluid better
and make a difference between white matter and gray matter clearly.
- Indicate MRI examination for infant with sign of emergency and
suffocation is very necessary for screening MSUD.
- Apply quiet sequences.
- Scan time about 30 minutes.
REFERENCES:
1. https://mrimaster.com/PLAN%20PED%20BRAIN.html
2. https://radiopaedia.org/articles/maple-syrup-urine-disease
3. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3775146/
4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3680892/4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3680892/
C t t i f tiContact information :
1. M.D.Ph.D Tran Phan Ninh - Head of Department - Vietnam National Childrent’s Hospital
2. M.D Le Hong Nhung - Vietnam National Childrent’s Hospital
3. B.Sc. Nguyen Tuan Dung - Vietnam National Childrent’s Hospital
4. B.Sc. Nguyen Dinh Phuong - email: phuongnguyen.nig@gmail.com. Phone: 0904523231
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