Congenital Chest Wall Diseases

Transcription

Alyssa Courtney, Year IV
Gillian Lieberman, MD
September 2010
Congenital Chest Wall
Disorders: A Radiological
Analysis
Alyssa Courtney, Harvard Medical School,
University of Queensland.
Gillian Lieberman, MD.
Presentation Outline
Chest Wall Anatomy Review
 Types of Chest Wall Disorders in
Children
 Imaging Modalities
 Congenital Osseous Abnormalities
 Congenital Soft Tissue Abnormalities
 Final Patient

2
3
Anatomy: Thoracic Skeleton

Scapula




Clavicles
Sternum





Acromion
process
Coracoid
process
Manubrium
Angle
Body
Xiphoid process
Ribs
From Anatomy TV. http://www.anatomy.tv. Accessed 9th September 2010.
4
Anatomy: Thoracic
Muscles, Posterior
Chest Wall

Internal
intercostals

Subcostals
5
Anatomy: Thoracic Muscles,
Anterior Chest Wall

Subscapularis
Pectoralis minor
External
intercostals
Intercostal fascia

Underlying muscles





Internal and innermost
intercostals
Transversus thoracis
From Anatomy TV. http://www.anatomy.tv. Accessed 9th September 2010. 6
Anatomy: Thoracic
Muscles – Pectoralis Major

Pectoralis
major
7
Anatomy: Thoracic
Vessels and Nerves
Superior,
internal
and lateral
thoracic
arteries and
veins
 Intercostal
vessels and
nerves From Anatomy TV. http://www.anatomy.tv. Accessed 9

th
8
September 2010.
Types of Chest Wall Disorders
in Children
9
Chest Wall Disorders in Children:
Osseous Abnormalities

Congenital





Benign



Pectus excavatum
Pectus carinatum
Tilting of the sternum
Sternal fusion, rib, and
scapula abnormalities
Osteochondroma
Enchondroma



Infection
Langerhans Cell
Histiocytosis
Benign Masses


Mesenchymal hamartoma
Malignant Masses


Ewing’s sarcoma
Osteosarcoma
Fibrous Dysplasia
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N
Am. 2005; 43: 355 – 370.
10
Chest Wall Disorders in Children:
Soft Tissue Abnormalities

Congenital



Poland syndrome
Lymphatic and venous
malformations
Benign


Hemangioma
Rarer –






Lipoblastoma
Fibroma
Fibromatosis
Infantile myofibromatosis
Neurofibromas
Schwannomas



Infection
Langerhans Cell
Histiocytosis
Malignant Masses




Primitive neuroectodermal tumor
Rhabdomyosarcoma
Lymphoma
Rarer –




Congenital fibrosarcoma
Malignant peripheral nerve
sheath tumor
Mesenchymal chondrosarcoma
Neuroblastoma
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin
N Am. 2005; 43: 355 – 370.
11
Imaging Modalities
12
Imaging Modalities: Radiography

Primary screening modality for
 Symptomatic
or palpable chest wall
processes
May provide a definitive diagnosis of
benign osseous lesions
 Can be useful in preliminary assessment
of suspected malignant osseous lesion

Am. 2005; 43: 355 – 370.
13
Imaging Modalities: Use of
Computed Tomography (CT)
 Useful
for
 Further


evaluation if –
Normal radiographs
Inconclusive radiographs
 Defining
lesion extent
 Determining nature of a disorder
 Narrowing the range of differentials
 But
concerns remain for radiation dose and
possible carcinogenic effects
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol
Clin N Am. 2005; 43: 355 – 370.
14
Imaging Modalities: CT
Can use single-detector or multidetector CT
 Maximize spatial resolution by using
smallest possible field of view
 If infectious or neoplastic processes
considered, use a non-ionic intravenous
contrast material

Clin N Am. 2005; 43: 355 – 370.
15
Imaging Modalities: Use of Magnetic
Resonance Imaging (MRI)
Superior contrast and spatial resolution
without ionizing radiation or iodinated
contrast
 Limited in smaller children due to the
relatively long duration of examination
resulting in 
 Possible
sedation
 Respiratory artifact from breathing
N Am. 2005; 43: 355 – 370.
16
Imaging Modalities: MRI

Often reserved for  Problem-solving
 Evaluation

of vascular anomalies
Optimal results if  Use
smallest field of view possible
 Minimise patient motion
 Minimise scan time

Intravenous contrast is used in most cases
Clin N Am. 2005; 43: 355 – 370.
17
Imaging Modalities: MRI Protocols

Soft tissue pathology –
 Multiplanar
T1-weighted turbo spin echo and a
fat-suppression sequence
 If neoplastic, infectious or vascular suspicions
use a three dimensional gradient echo T1weighted imaging with fat suppression

Bone pathology –
 include
multiplanar spin echo T1- and T2weighted sequences for assessment of marrow
signal
N Am. 2005; 43: 355 – 370.
18
Imaging Modalities: MRI
Postprocessing

Postprocessing techniques are performed
to further define and characterize the
pathology
 Subtraction
 Multiplanar
reconstruction
 Maximal-intensity projections
N Am. 2005; 43: 355 – 370.
19
Imaging Modalities: Use of
Ultrasound
Evaluation of palpable, superficial, softtissue chest wall pathology
 Useful in children as –

 Risk-free
 Non-invasive
 Fast
examination time
N Am. 2005; 43: 355 – 370.
20
Imaging Modalities: Details of
Ultrasound

Use a high-frequency linear transducer to
 Determine
if lesion present
 Determine if cystic or solid

Use color Doppler ultrasound and spectral
tracings for information about vascular
flow in –
 Vascular
malformations
 Hemangiomas
N Am. 2005; 43: 355 – 370.
21
Congenital Osseous
Abnormalities
22
Congenital Osseous
Abnormalities
1.
2.
3.
4.
5.
6.
Pectus Excavatum
Pectus Carinatum
Tilting of the Sternum
Sternal Fusion Abnormalities
Rib Abnormalities
Scapula Abnormalities
23
Pectus Excavatum
A deformity of the chest wall characterized
by a sternal depression typically beginning
over the middle of the manubrium and
progressing inward through to the xiphoid
process.
Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.
24
Pectus Excavatum: Incidence
1:400-1000 live births
M>F
 90% of anterior chest wall disorders
 Usually sporadic but increased familial
incidence

September 2010.
25
Pectus Excavatum: Pathophysiology
Several hypotheses  Abnormal
cartilage development
 Underlying pulmonary conditions
 eg.
repaired congenital diaphragmatic hernia, spinal
muscular atrophy type 1, subglottic stenosis, and
bronchopulmonary dysplasia
September 2010.
26
Pectus Excavatum: Associations
and/or Differential Diagnoses





Scoliosis (15%)
Mitral valve prolapse
Congenital heart disease
Cardiorespiratory compromise
Connective tissue disorders –


Neuromuscular disease


Marfan’s syndrome, Ehlers Danlos syndrome, and osteogenesis imperfecta
eg. spinal muscular atrophy
Other genetic conditions –

Noonan syndrome, Turner syndrome, and multiple endocrine neoplasia
type 2b
September 2010.
Schwartzstein RM. Diseases of the chest wall. Up to Date. May 2010. Accessed 11th September 2010.
27
Pectus Excavatum: Symptoms
 Exertional
intolerance - 82% of several hundred
pediatric patients with pectus excavatum
 Chest
pain – 68%
 Poor endurance – 67%
 Shortness of breath – 42%
 Cosmetic concerns –
 68%
of females
 40% of males
 Usually subsides by 20 years of age
Am. 2005; 43: 355 – 370.
September 2010.
28
Pectus Excavatum: Evaluation
 Physical
Exam – sternal depression, thoracic
abnormalities, musculoskeletal examination
respiratory function, and cardiovascular examination
 Exercise testing
 Imaging – detect severity
and associated scoliosis
From Mayer, OH. Pectus excavatum: Etiology and
evaluation. Up to Date. May 2010. Accessed 11th September
2010.
Am. 2005; 43: 355 – 370.
29
September 2010.
Index Patient:
Pectus Excavatum
PA View




PACS, BIDMC
Heart deviated to left
Prominence of
vessels right of
midline – obscuring
right heart border
Ribs slope
downwards more
than normal
Eventration of right
diaphragm (not a usual
feature of pectus
excavatum)
Grissom LE. Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in
Roentgenology. 1998; Vol XXXIII (No. 2):199-208.
30
Index Patient: Pectus
Excavatum PA
View Findings




PACS, BIDMC
Heart deviated to left
Prominence of
vessels right of
midline – obscuring
right heart border
Ribs slope
downwards more
than normal
Eventration of right
diaphragm (not a usual
feature of pectus
excavatum)
31
Index Patient:
Pectus
Excavatum
Lateral View

Deep
depression of
the sternum
PACS, BIDMC
BIDMC, PACS
32
Pectus Excavatum:
Haller Index




Also known as ‘Pectus
Severity Index’
Ratio of the transverse
diameter of the thorax (A) to
the AP diameter at the
deepest part of the pectus (B)
Evaluates for surgical repair
Surgery usually required with
indices > 3.25
From Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.
Grissom LE, Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology.
1998; Vol XXXIII (No. 2):199-208.
Haller JA. Kramer SS. Lietman SA. Use of CT scans in selection of patients for pectus excavatum
surgery: a preliminary report. J Pediatr Surg.1987; 10: 904-906.
33
Pectus Excavatum: Cardiac
Distortion

Significant cardiac
distortion represented
at the xiphoid process
as the –


Cardiac compression
index (H/M)
Cardiac asymmetry
index (P/M)
From Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.
34
Pectus Excavatum: CT
12 year old girl with pectus excavatum

Axial
noncontrast
CT image of
the chest
demonstrating
rotation and
marked
depression of
the sternum
From Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol
Clin N Am. 2005; 43: 355 – 370.
35
Pectus Excavatum: Treatment
 Monitoring
 Psychological
support – if appropriate
 Sternal suction
 Sternal magnet
 Prosthetic inserts
 Physical therapy
 Surgical correction – moderate to severe
deformities
36
Mayer OH. Pectus excavatum: Treatment. Up to Date. May 2010. Accessed on September
11th
2010.
Congenital Osseous
Abnormalities
1.
2.
3.
4.
5.
6.
Pectus Excavatum
Pectus Carinatum
Rib Abnormalities
37
Pectus Carinatum
Protrusion deformity of the anterior chest wall.
Types:
Chondrogladiolar prominence


Middle and lower portions of sternum prominent and arch forward
Most common
Chondromanubrial prominence



Upper portion of sternum anteriorly prominent, body of sternum
depressed posteriorly, and a final anterior deflection of distal
sternum
Z-shape in lateral view
Less common
Nuchtern JG. Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed 11th September 2010.
38
Pectus Carinatum: Incidence and
Pathophysiology
Incidence:


1 in 1500 live births
Male 4:1 Female
Pathophysiology: Same as Pectus Excavatum
39
Pectus Carinatum: Associations and/or
Differential Diagnoses
 Musculoskeletal
 eg
scoliosis
 Connective
 eg
abnormalities
tissue disorders
Marfan syndrome and osteogenesis imperfecta
 Other
genetic conditions
 eg
Noonan syndrome, cardiofaciocutaneous
syndrome, Poland syndrome, Coffin-Lowry syndrome,
and Morquio disease
40
Pectus Carinatum: Symptoms
Symptoms worsen during puberty  Cosmetic
concerns
 Rarely (and lacking objective evidence)  Exercise
limitation
 Exertional dyspnoea
41
Pectus Carinatum: Evaluation
 Physical
Exam – sternal protrusion, thoracic
abnormalities, musculoskeletal examination,
respiratory function, cardiovascular
examination
 Exercise
testing – if appropriate
 Imaging – detect severity, associated
scoliosis
42
Companion
Patient 1:
Pectus
Carinatum
AP View

Ribs are
more
horizontal

Thorax can be
narrowed in pectus
carinatum – not
shown here
PACS, BIDMC
Grissom LE, Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology.
1998; Vol XXXIII (No. 2):199-208.
43
Companion
Patient 1:
Pectus
Carinatum
Lateral View


Prominently
bowed
sternum
Increased
AP diameter
of the chest
PACS, BIDMC
BIDMC,
Grissom LE, Harcke HT. Thoracic Deformities and the
Growing PACS
Lung. Seminars in Roentgenology.
1998; Vol XXXIII (No. 2):199-208.
44
Pectus Carinatum: Haller Index
CT Imaging
 Measure the Haller index (as seen in
pectus excavatum)
 Lower
the index = worse deformity
 Mean
index of 260 subjects with pectus
carinatum = 1.81
BIDMC, PACS
Nuchtern JG, Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed September
45
11th
2010.
Pectus Carinatum: Treatment
No intervention
 Psychological support – if appropriate
 Bracing – for patients with a flexible mild
to moderate deformity
 Surgical correction – moderate to severe
deformities

BIDMC, PACS
Nuchtern JG, Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed September
46
11th
2010.
Congenital Osseous
Abnormalities
1.
2.
3.
4.
5.
6.
Pectus Excavatum
Pectus Carinatum
Rib Abnormalities
47
Deviation of the typical horizontal positioning
of the sternum in the transverse axis of the
body.
Imaging:
 Usually
not apparent on radiographs
 Secondary lateral displacement of medial
heads of the adjacent clavicles may assist
detection
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N
Am. 2005; 43: 355 – 370.
48
Tilting of the Sternum:
Associations
 Anterior
subluxation of the adjacent
clavicular head
 Abnormal convexity of the adjacent rib
resulting in a palpable chest wall bump
Am. 2005; 43: 355 – 370.
49
Congenital Osseous
Abnormalities
1.
2.
3.
4.
5.
6.
Pectus Excavatum
Pectus Carinatum
Rib Abnormalities
50
Example: Axial noncontrast CT of 1 month old boy with a bifid sternum:
marked separation of the clavicular heads and depression of soft tissues
in the location of the expected upper sternum

May be an
isolated
abnormality and
can require
surgical
correction to
prevent
cardiopulmonary
compromise
Clin N Am. 2005; 43: 355 – 370.
51
Sternal Fusion Abnormalities:
Associations
Example: Lateral Chest Radiograph demonstrating absence
of sternum and sternal ossification centers
Severe sternal fusion
abnormaliies are
associated with –


Congenital heart
disease eg ectopia
cordis (extrathoracic heart)
Pentalogy of Cantrell
(combination of severe
sternum, diaphragm, heart
and abdominal wall defects)
Clin N Am. 2005; 43: 355 – 370.
52
Congenital Osseous
Abnormalities
1.
2.
3.
4.
5.
6.
Pectus Excavatum
Pectus Carinatum
Rib Abnormalities
53
Rib Abnormalities
Types:
Agenesis, hypoplasia, and bifid configuration.

Developmental anatomic variations can present
as asymptomatic palpable chest wall masses
Examples –
 Prominent convexity of anterior rib or costal
cartilage
 Prominence of costochondral junction
 Small parachondral nodules of unknown origin
54
Am. 2005; 43: 355 – 370.
Congenital Osseous
Abnormalities
1.
2.
3.
4.
5.
6.
Pectus Excavatum
Pectus Carinatum
Rib Abnormalities
55
Scapula Abnormalities: Sprengel’s
Deformity
Failure of descent of the scapula.
 Most
notable scapula deformity
 Sometimes the scapula is tethered to the spine
by an osteocartilaginous connection called the
omohyoid bone
 Can cause neck stiffness and restrict
abduction of the arm
Brett-Fleegler M. Evaluation of neck stiffness in children. Up to Date. August 2009. Accessed on
September 11th 2010.
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N 56
Am. 2005; 43: 355 – 370.
Thacker MM. Sprengel Deformity. eMedicine. July 2009. Accessed on September 11th 2010.
Scapula Abnormalities: Sprengel’s
Deformity, Associations and Treatment
Associated with
 Klippel-Feil
syndrome
 Osseous abnormalities
 Spinal cord abnormalities
Treatment: Physiotherapy or surgery
Brett-Fleegler M. Evaluation of neck stiffness in children. Up to Date. August 2009. Accessed on
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N 57
Am. 2005; 43: 355 – 370.
Thacker MM. Sprengel Deformity. eMedicine. July 2009. Accessed on September 11th 2010.
Congenital Soft Tissue
Abnormalities
58
Abnormalities
1.
2.
3.
Poland Syndrome
Lymphatic Malformations
Venous Malformations
59
Poland Syndrome
Rare congenital malformation
of chest wall with hypoplasia or
aplasia of the pectoralis major
muscle and adjacent
cartilaginous, osseous, and soft
tissue structures.
Clinical asymmetry of the chest
From Habib M. Mahajan S. Kuchey GA.
Gupta D. Sharma S. Poland Syndrome, a
rare entity. The Internet Journal of
Orthopedic Surgery. 2009; 12 (1).
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 60
2005; 43: 355 – 370.
Poland Syndrome: Pathophysiology
and Incidence
Pathophysiology:
 Unknown
 Hypothesized to occur as a
result of ipsilateral subclavian
artery disruption
Incidence:
 1/30 000 live births
 Usually unilateral
 Males > Females
 Right > Left
From Habib M. Mahajan S. Kuchey GA.
Gupta D. Sharma S. Poland Syndrome, a
rare entity. The Internet Journal of
Orthopedic Surgery. 2009; 12 (1).
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 61
2005; 43: 355 – 370.
Poland Syndrome: Chest Radiographs
The hypoplasia of chest wall soft tissues
results in relative lunacy of affected
hemithorax
Differential diagnoses from radiograph:
 Pulmonary entities that cause air trapping

 Congenital
lobar emphysema
 Obstruction from a foreign body
 Swyer-James syndrome
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am.62
2005; 43: 355 – 370.
Poland Syndrome: Treatment

Surgical correction if severe chest wall
deformities
CT scanning or MR imaging is useful to
determine the extent of the deformity for
presurgical planning
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am.63
2005; 43: 355 – 370.
Abnormalities
1.
2.
3.
Poland Syndrome
64
Increased number of dilated lymphatic channels
lined by endothelium
 Microcystic, macrocystic, or combined
 Most common in:
Axilla, chest, cervicofacial region
 In the chest:


focal or diffuse masses confined to the
subcutaneuos tissues or
involve the spine and/or mediastinum From Children’s Hospital Boston.
Lymphatic Malformation. Accessed
14th September 2010.
Am. 2005; 43: 355 – 370.
65
Lymphatic Malformations: MRI (1)
2 year old boy with left lateral chest wall lymphatic
malformations

Axial (C+) T2weighted MRI
demonstrating a
multiloculated
high-signalintensity left lateral
chest wall mass
with internal
septations
Clin N Am. 2005; 43: 355 – 370.
66
Lymphatic Malformations: MRI (2)
2 year old boy with left lateral chest wall lymphatic malformations

Axial fat-saturated, T1weighted postcontrast
MRI – low signal
intensity of the cystic
component and
enhancement of walls
and septations
Clin N Am. 2005; 43: 355 – 370.
67
Abnormalities
1.
2.
3.
Poland Syndrome
68
Isolated or multiple dilated, tortuous, thinwalled (lack of smooth muscle) venous
structures
 Grow in proportion to child growth
 Focal abnormalities through to diffuse
involvement of the deeper soft tissues and
bone
 Affects chest wall less than lymphatic
abnormalities
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin
N Am. 2005; 43: 355 – 370.
69
Venous Malformations: Imaging
 Ultrasound
– hypoechoic, isoechoic, or
hyperechoic
 Phleboliths
may also be identified
 Color Doppler spectral tracings demonstrate
either low-flow venous patterns or no flow
 MRI
- evaluates extent of involvement
and characterization of flow
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin
N Am. 2005; 43: 355 – 370.
70
Venous Malformations:
Time-resolved MR Angiography (1)
10 year old boy who has left posterolateral chest wall venous
malformations
Axial (C+) MR STIR
image with a cluster
of serpiginous highsignal structures
From Fefferman NR, Pinkney LP. Imaging Evaluation of
Chest Wall Disorders in Children. Radiol Clin N Am.
2005; 43: 355 – 370.
71
Time-resolved MR Angiography (2)
10 year old boy who has left posterolateral chest wall venous
malformations
Axial postcontrast T1weighted MRI - late
venous-phase
enhancement of
abnormal vascular
structures
From Fefferman NR, Pinkney LP. Imaging Evaluation of
Chest Wall Disorders in Children. Radiol Clin N Am.
2005; 43: 355 – 370.
72
Treatment
Observation
 Sclerotherapy

Am. 2005; 43: 355 – 370.
73
Final Patient
Am. 2005; 43: 355 – 370.
74
Companion
Patient 2

Where’s the
congenital
chest wall
abnormality?
Please turn to the next
page to reveal the
abnormality
PACS, BIDMC
75
Companion
Patient 2:
Relevant
Finding
Rib
Abnormality
PACS, BIDMC
76
Companion
Patient 2:
Additional
Findings?
Can you detect the
additional findings
on this radiograph?
Please turn to the next
page to reveal the
findings
PACS, BIDMC
77
Companion
Patient 2:
Additional
Findings
Pneumomediastinum
Transtracheal oxygen
Cathetor
Subcutaneous neck
Emphysema
Multi-focal linear
atelectasis in bilateral
mid and lower lungs
PACS, BIDMC
78
Summary
 Types of Chest Wall Disorders in
Children
 Imaging Modalities:

 Chest
radiography or ultrasound then
MRI or CT if required
Congenital Osseous Abnormalities
 Congenital Soft Tissue Abnormalities

79
References (1)
1.
2.
3.
4.
5.
6.
7.
Anatomy TV. http://www.anatomy.tv. Accessed 11th September 2010.
Brett-Fleegler M. Evaluation of neck stiffness in children. Up to Date. August 2009.
http://www.uptodate.com/online/content/topic.do?topicKey=ped_symp/10542&selecte
dTitle=2%7E150&source=search_result . Accessed on September 11th 2010.
Children’s Hospital Boston. Lymphatic Malformation.
http://www.childrenshospital.org/az/Site1256/mainpageS1256P0.html. Accessed 14th
September 2010.
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Acknowledgements
Larry Barbaras
 Gillian Lieberman, MD
 Emily Hanson
 Pauline Bishop, MD

82

Congenital Chest Wall Diseases

Transcription

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