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Congenital Chest Wall Congenital Chest Wall Disorders: A Radiological Disorders: A Radiological
AnalysisAnalysis
Alyssa Courtney, Harvard Medical School, Alyssa Courtney, Harvard Medical School, University of Queensland.University of Queensland.
Gillian Lieberman, MD.Gillian Lieberman, MD.
September 2010Alyssa Courtney, Year IVGillian Lieberman, MD
Presentation OutlinePresentation Outline
Chest Wall Anatomy ReviewChest Wall Anatomy Review
Types of Chest Wall Disorders in Types of Chest Wall Disorders in ChildrenChildren
Imaging ModalitiesImaging Modalities
Congenital Osseous AbnormalitiesCongenital Osseous Abnormalities
Congenital Soft Tissue AbnormalitiesCongenital Soft Tissue Abnormalities
Final PatientFinal Patient
22
Alyssa Courtney, Year IVGillian Lieberman, MD
Chest Wall Anatomy ReviewChest Wall Anatomy Review
33
Alyssa Courtney, Year IVGillian Lieberman, MD
Anatomy: Thoracic SkeletonAnatomy: Thoracic Skeleton
ScapulaScapula
AcromionAcromion processprocess
CoracoidCoracoid processprocess
ClaviclesClavicles
SternumSternum
ManubriumManubrium
AngleAngle
BodyBody
XiphoidXiphoid processprocess
RibsRibs44From Anatomy TV. http://www.anatomy.tv. Accessed 9th September 2010.
Alyssa Courtney, Year IVGillian Lieberman, MD
Anatomy: Thoracic Anatomy: Thoracic Muscles, Posterior Muscles, Posterior Chest WallChest Wall
Internal Internal intercostalsintercostals
SubcostalsSubcostals
55
Alyssa Courtney, Year IVGillian Lieberman, MD
From Anatomy TV. http://www.anatomy.tv. Accessed 9th September 2010.
Anatomy: Thoracic Muscles, Anatomy: Thoracic Muscles, Anterior Chest WallAnterior Chest Wall
SubscapularisSubscapularis
PectoralisPectoralis minorminor
External External intercostals intercostals
IntercostalIntercostal fasciafascia
Underlying musclesUnderlying muscles
Internal and innermost Internal and innermost intercostalsintercostals
TransversusTransversus thoracisthoracis
66
Alyssa Courtney, Year IVGillian Lieberman, MD
From Anatomy TV. http://www.anatomy.tv. Accessed 9th September 2010.
Anatomy: Thoracic Anatomy: Thoracic Muscles Muscles –– PectoralisPectoralis MajorMajor
PectoralisPectoralis majormajor
77
Alyssa Courtney, Year IVGillian Lieberman, MD
From Anatomy TV. http://www.anatomy.tv. Accessed 9th September 2010.
Anatomy: Thoracic Anatomy: Thoracic Vessels and NervesVessels and Nerves
Superior, Superior, internal internal and and laterallateral thoracic thoracic arteries and arteries and veinsveins
IntercostalIntercostal vessels and vessels and nervesnerves 88
Alyssa Courtney, Year IVGillian Lieberman, MD
From Anatomy TV. http://www.anatomy.tv. Accessed 9th September 2010.
Types of Chest Wall Disorders Types of Chest Wall Disorders in Childrenin Children
99
Alyssa Courtney, Year IVGillian Lieberman, MD
Chest Wall Disorders in Children: Chest Wall Disorders in Children: Osseous AbnormalitiesOsseous Abnormalities
Congenital
Pectus excavatum
Pectus carinatum
Tilting of the sternum
Sternal fusion, rib, and scapula abnormalities
Benign
Osteochondroma
Enchondroma
Fibrous Dysplasia
Infection
Langerhans Cell Histiocytosis
Benign Masses
Mesenchymal hamartoma
Malignant Masses
Ewing’s sarcoma
Osteosarcoma
1010Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Alyssa Courtney, Year IVGillian Lieberman, MD
Chest Wall Disorders in Children: Chest Wall Disorders in Children: Soft Tissue AbnormalitiesSoft 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
Neuroblastoma1111
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Imaging ModalitiesImaging Modalities
1212
Alyssa Courtney, Year IVGillian Lieberman, MD
Imaging Modalities: RadiographyImaging Modalities: Radiography
Primary screening modality forPrimary screening modality for
Symptomatic or palpable chest wall Symptomatic or palpable chest wall processesprocesses
May provide a definitive diagnosis of May provide a definitive diagnosis of benign osseous lesionsbenign osseous lesions
Can be useful in preliminary assessment Can be useful in preliminary assessment of suspected malignant osseous lesionof suspected malignant osseous lesion
1313
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Imaging Modalities: Use of Imaging Modalities: Use of Computed Tomography (CT)Computed Tomography (CT)
Useful for Useful for
Further evaluation if Further evaluation if ––
Normal radiographsNormal radiographs
Inconclusive radiographsInconclusive radiographs
Defining lesion extentDefining lesion extent
Determining nature of a disorderDetermining nature of a disorder
Narrowing the range of differentialsNarrowing the range of differentials
But concerns remain for radiation dose and But concerns remain for radiation dose and possible carcinogenic effectspossible carcinogenic effects
1414
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Imaging Modalities: CTImaging Modalities: CT
Can use singleCan use single--detector or detector or multidetectormultidetector CTCT
Maximize spatial resolution by using Maximize spatial resolution by using smallest possible field of viewsmallest possible field of view
If infectious or If infectious or neoplasticneoplastic processes processes considered, use a nonconsidered, use a non--ionic intravenous ionic intravenous contrast materialcontrast material
1515
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Imaging Modalities: Use of Magnetic Imaging Modalities: Use of Magnetic Resonance Imaging (MRI)Resonance Imaging (MRI)
Superior contrast and spatial resolution Superior contrast and spatial resolution without ionizing radiation or iodinated without ionizing radiation or iodinated contrastcontrast
Limited in smaller children due to the Limited in smaller children due to the relatively long duration of examination relatively long duration of examination resulting in resulting in --
Possible sedation Possible sedation
Respiratory artifact from breathingRespiratory artifact from breathing1616
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Imaging Modalities: MRIImaging Modalities: MRI
Often reserved for Often reserved for --
ProblemProblem--solvingsolving
Evaluation of vascular anomaliesEvaluation of vascular anomalies
Optimal results if Optimal results if --
Use smallest field of view possibleUse smallest field of view possible
MinimiseMinimise patient motionpatient motion
MinimiseMinimise scan timescan time
Intravenous contrast is used in most casesIntravenous contrast is used in most cases
1717
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Imaging Modalities: MRI Protocols Imaging Modalities: MRI Protocols
Soft tissue pathology Soft tissue pathology ––
MultiplanarMultiplanar T1T1--weighted turbo spin echo and a weighted turbo spin echo and a fatfat--suppression sequencesuppression sequence
If If neoplasticneoplastic, infectious or vascular suspicions , infectious or vascular suspicions use a three dimensional gradient echo T1use a three dimensional gradient echo T1-- weighted imaging with fat suppressionweighted imaging with fat suppression
Bone pathology Bone pathology ––
include include multiplanarmultiplanar spin echo T1spin echo T1-- and T2and T2-- weighted sequences for assessment of marrow weighted sequences for assessment of marrow signalsignal
1818
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Imaging Modalities: MRI Imaging Modalities: MRI PostprocessingPostprocessing
PostprocessingPostprocessing techniques are performed techniques are performed to further define and characterize the to further define and characterize the pathologypathology
SubtractionSubtraction
MultiplanarMultiplanar reconstructionreconstruction
MaximalMaximal--intensity projectionsintensity projections
1919
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Imaging Modalities: Use of Imaging Modalities: Use of UltrasoundUltrasound
Evaluation of palpable, superficial, softEvaluation of palpable, superficial, soft-- tissue chest wall pathologytissue chest wall pathology
Useful in children as Useful in children as ––
RiskRisk--freefree
NonNon--invasiveinvasive
Fast examination timeFast examination time
2020
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Imaging Modalities: Details of Imaging Modalities: Details of UltrasoundUltrasound
Use a highUse a high--frequency linear transducer to frequency linear transducer to
Determine if lesion presentDetermine if lesion present
Determine if cystic or solidDetermine if cystic or solid
Use color Doppler ultrasound and spectral Use color Doppler ultrasound and spectral tracings for information about vascular tracings for information about vascular flow in flow in ––
Vascular malformationsVascular malformations
HemangiomasHemangiomas2121
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Congenital Osseous Congenital Osseous AbnormalitiesAbnormalities
2222
Alyssa Courtney, Year IVGillian Lieberman, MD
Congenital Osseous Congenital Osseous AbnormalitiesAbnormalities
1.1. PectusPectus ExcavatumExcavatum2.2. PectusPectus CarinatumCarinatum3.3. Tilting of the SternumTilting of the Sternum4.4. SternalSternal Fusion AbnormalitiesFusion Abnormalities5.5. Rib AbnormalitiesRib Abnormalities6.6. Scapula AbnormalitiesScapula Abnormalities
2323
Alyssa Courtney, Year IVGillian Lieberman, MD
PectusPectus ExcavatumExcavatum
AA deformity of the chest wall characterized deformity of the chest wall characterized by a by a sternalsternal depression typically beginning depression typically beginning over the middle of the over the middle of the manubriummanubrium and and progressing inward through to the progressing inward through to the xiphoidxiphoid process.process.
Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.2424
Alyssa Courtney, Year IVGillian Lieberman, MD
PectusPectus ExcavatumExcavatum: Incidence: Incidence
1:4001:400--1000 live births1000 live births
M > FM > F
90% of anterior chest wall disorders90% of anterior chest wall disorders
Usually sporadic but increased familial Usually sporadic but increased familial incidenceincidence
Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.2525
Alyssa Courtney, Year IVGillian Lieberman, MD
PectusPectus ExcavatumExcavatum: Pathophysiology: Pathophysiology
Several hypotheses Several hypotheses --
Abnormal cartilage developmentAbnormal cartilage development
Underlying pulmonary conditions Underlying pulmonary conditions
egeg. repaired congenital diaphragmatic hernia, spinal . repaired congenital diaphragmatic hernia, spinal muscular atrophy type 1, muscular atrophy type 1, subglotticsubglottic stenosisstenosis, and , and bronchopulmonarybronchopulmonary dysplasiadysplasia
2626
Alyssa Courtney, Year IVGillian Lieberman, MD
Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.
PectusPectus ExcavatumExcavatum: Associations : Associations and/or Differential Diagnosesand/or Differential Diagnoses
Scoliosis (15%)Scoliosis (15%)
Mitral valve Mitral valve prolapseprolapse
Congenital heart diseaseCongenital heart disease
CardiorespiratoryCardiorespiratory compromisecompromise
Connective tissue disorders Connective tissue disorders ––
MarfanMarfan’’ss syndrome, Ehlers syndrome, Ehlers DanlosDanlos syndrome, and syndrome, and osteogenesisosteogenesis imperfectaimperfecta
Neuromuscular disease Neuromuscular disease
egeg. spinal muscular atrophy. spinal muscular atrophy
Other genetic conditions Other genetic conditions ––
Noonan syndrome, Turner syndrome, and multiple endocrine Noonan syndrome, Turner syndrome, and multiple endocrine neoplasianeoplasia type 2btype 2b
2727
Alyssa Courtney, Year IVGillian Lieberman, MD
Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.Schwartzstein RM. Diseases of the chest wall. Up to Date. May 2010. Accessed 11th September 2010.
PectusPectus ExcavatumExcavatum: Symptoms: Symptoms
ExertionalExertional intolerance intolerance -- 82% 82% of several hundred of several hundred pediatric patients with pediatric patients with pectuspectus excavatumexcavatum
Chest pain Chest pain –– 68%68%
Poor endurance Poor endurance –– 67%67%
Shortness of breath Shortness of breath –– 42%42%
Cosmetic concerns Cosmetic concerns ––
68% of females68% of females
40% of males40% of males
Usually subsides by 20 years of ageUsually subsides by 20 years of age
2828
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.
PectusPectus ExcavatumExcavatum: Evaluation: Evaluation
Physical Exam Physical Exam –– sternalsternal depression, thoracic depression, thoracic abnormalities, musculoskeletal examination abnormalities, musculoskeletal examination respiratory function, and cardiovascular examinationrespiratory function, and cardiovascular examination
Exercise testingExercise testing
Imaging Imaging –– detect severity detect severity and associated scoliosisand associated scoliosis
2929
From Mayer, OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th September 2010.
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.
Index Patient: Index Patient: PectusPectus ExcavatumExcavatum PA ViewPA View
Heart deviated to leftHeart deviated to left
Prominence of Prominence of vessels right of vessels right of midline midline –– obscuring obscuring right heart borderright heart border
Ribs slope Ribs slope downwards more downwards more than normalthan normal
EventrationEventration of right of right diaphragm diaphragm (not a usual (not a usual feature of feature of pectuspectus excavatumexcavatum))PACS, BIDMC
3030Grissom LE. Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.
Alyssa Courtney, Year IVGillian Lieberman, MD
Index Patient: Index Patient: PectusPectus ExcavatumExcavatum PA PA View FindingsView Findings
Heart deviated to leftHeart deviated to left
Prominence of Prominence of vessels right of vessels right of midline midline –– obscuring obscuring right heart borderright heart border
Ribs slope Ribs slope downwards more downwards more than normalthan normal
EventrationEventration of right of right diaphragm diaphragm (not a usual (not a usual feature of feature of pectuspectus excavatumexcavatum))PACS, BIDMC
3131Grissom LE. Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.
Alyssa Courtney, Year IVGillian Lieberman, MD
Index Patient: Index Patient: PectusPectus
ExcavatumExcavatum Lateral ViewLateral View
Deep Deep depression of depression of the sternumthe sternum
BIDMC, PACS 3232
PACS, BIDMC
Alyssa Courtney, Year IVGillian Lieberman, MD
Grissom LE. Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.
PectusPectus ExcavatumExcavatum: : Haller Index Haller Index
Also known as Also known as ‘‘PectusPectus Severity IndexSeverity Index’’
Ratio of the transverse Ratio of the transverse diameter of the thorax (A) to diameter of the thorax (A) to the AP diameter at the the AP diameter at the deepest part of the deepest part of the pectuspectus (B)(B)
Evaluates for surgical repairEvaluates for surgical repair
Surgery usually required with Surgery usually required with indices > 3.25indices > 3.25
3333
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.
Alyssa Courtney, Year IVGillian Lieberman, MD
PectusPectus ExcavatumExcavatum: Cardiac : Cardiac DistortionDistortion
Significant cardiac Significant cardiac distortion represented distortion represented at the at the xiphoidxiphoid process process as the as the ––
Cardiac compression Cardiac compression index (H/index (H/MM))
Cardiac asymmetry Cardiac asymmetry index (index (PP//MM))
3434From Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th
September 2010.
Alyssa Courtney, Year IVGillian Lieberman, MD
PectusPectus ExcavatumExcavatum: CT: CT
Axial Axial noncontrastnoncontrast CT image of CT image of the chest the chest demonstrating demonstrating rotation and rotation and marked marked depression of depression of the sternumthe sternum
3535
12 year old girl with pectus excavatum
Alyssa Courtney, Year IVGillian Lieberman, MD
From Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
PectusPectus ExcavatumExcavatum: Treatment: Treatment
MonitoringMonitoring
Psychological support Psychological support –– if appropriateif appropriate
SternalSternal suctionsuction
SternalSternal magnetmagnet
Prosthetic insertsProsthetic inserts
Physical therapyPhysical therapy
Surgical correction Surgical correction –– moderate to severe moderate to severe deformitiesdeformities
3636Mayer OH. Pectus excavatum: Treatment. Up to Date. May 2010. Accessed on September 11th 2010.
Alyssa Courtney, Year IVGillian Lieberman, MD
Congenital Osseous Congenital Osseous AbnormalitiesAbnormalities
1.1. PectusPectus ExcavatumExcavatum2.2. PectusPectus CarinatumCarinatum3.3. Tilting of the SternumTilting of the Sternum4.4. SternalSternal Fusion AbnormalitiesFusion Abnormalities5.5. Rib AbnormalitiesRib Abnormalities6.6. Scapula AbnormalitiesScapula Abnormalities
3737
Alyssa Courtney, Year IVGillian Lieberman, MD
PectusPectus CarinatumCarinatumProtrusion deformity of the anterior chest wall.Protrusion deformity of the anterior chest wall.Types:Types:ChondrogladiolarChondrogladiolar prominenceprominence
Middle and lower portions of sternum prominent and arch forwardMiddle and lower portions of sternum prominent and arch forward
Most commonMost common
ChondromanubrialChondromanubrial prominenceprominence
Upper portion of sternum Upper portion of sternum anteriorlyanteriorly prominent, body of sternum prominent, body of sternum depressed depressed posteriorlyposteriorly, and a final anterior deflection of distal , and a final anterior deflection of distal sternumsternum
ZZ--shape in lateral viewshape in lateral view
Less commonLess common3838
Nuchtern JG. Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed 11th September 2010.
Alyssa Courtney, Year IVGillian Lieberman, MD
PectusPectus CarinatumCarinatum: Incidence and : Incidence and PathophysiologyPathophysiology
Incidence:Incidence:
1 in 1500 live births1 in 1500 live births
Male 4:1 FemaleMale 4:1 Female
Pathophysiology:Pathophysiology: Same as Same as PectusPectus ExcavatumExcavatum
3939
Alyssa Courtney, Year IVGillian Lieberman, MD
Nuchtern JG. Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed 11th September 2010.
PectusPectus CarinatumCarinatum: Associations and/or : Associations and/or Differential DiagnosesDifferential Diagnoses
Musculoskeletal abnormalities Musculoskeletal abnormalities
egeg scoliosisscoliosis
Connective tissue disorders Connective tissue disorders
egeg MarfanMarfan syndrome and syndrome and osteogenesisosteogenesis imperfectaimperfecta
Other genetic conditions Other genetic conditions
egeg Noonan syndrome, Noonan syndrome, cardiofaciocutaneouscardiofaciocutaneous syndrome, Poland syndrome, Coffinsyndrome, Poland syndrome, Coffin--Lowry syndrome, Lowry syndrome, and and MorquioMorquio diseasedisease
4040
Alyssa Courtney, Year IVGillian Lieberman, MD
Nuchtern JG. Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed 11th September 2010.
PectusPectus CarinatumCarinatum: Symptoms: Symptoms
Symptoms worsen during puberty Symptoms worsen during puberty --Cosmetic concernsCosmetic concernsRarely (and lacking objective evidence) Rarely (and lacking objective evidence) --Exercise limitationExercise limitationExertionalExertional dyspnoeadyspnoea
4141
Alyssa Courtney, Year IVGillian Lieberman, MD
Nuchtern JG. Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed 11th September 2010.
PectusPectus CarinatumCarinatum: Evaluation: Evaluation
Physical Exam Physical Exam –– sternalsternal protrusion, thoracic protrusion, thoracic abnormalities, musculoskeletal examination, abnormalities, musculoskeletal examination, respiratory function, cardiovascular respiratory function, cardiovascular examinationexamination
Exercise testing Exercise testing –– if appropriateif appropriate ImagingImaging –– detect severity, associated detect severity, associated
scoliosisscoliosis
4242
Alyssa Courtney, Year IVGillian Lieberman, MD
Nuchtern JG. Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed 11th September 2010.
Companion Companion Patient 1: Patient 1:
PectusPectus CarinatumCarinatum AP ViewAP View
Ribs are Ribs are more more horizontalhorizontal
Thorax can be Thorax can be narrowed in narrowed in pectuspectus carinatumcarinatum –– not not shown hereshown here
4343Grissom LE, Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.
PACS, BIDMC
Alyssa Courtney, Year IVGillian Lieberman, MD
Companion Companion Patient 1: Patient 1:
PectusPectus CarinatumCarinatum
Lateral ViewLateral View
Prominently Prominently bowed bowed sternumsternum
Increased Increased AP diameter AP diameter of the chestof the chest
BIDMC, PACS 4444
PACS, BIDMC
Alyssa Courtney, Year IVGillian Lieberman, MD
Grissom LE, Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.
PectusPectus CarinatumCarinatum: Haller Index: Haller Index
CT ImagingCT Imaging
Measure the Haller index (as seen in Measure the Haller index (as seen in pectuspectus excavatumexcavatum) )
Lower the index = worse deformityLower the index = worse deformity
Mean index of 260 subjects with Mean index of 260 subjects with pectuspectus carinatumcarinatum = 1.81= 1.81
BIDMC, PACS 4545Nuchtern JG, Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed September 11th 2010.
Alyssa Courtney, Year IVGillian Lieberman, MD
PectusPectus CarinatumCarinatum: Treatment: Treatment
No interventionNo intervention
Psychological support Psychological support –– if appropriateif appropriate
Bracing Bracing –– for patients with a flexible mild for patients with a flexible mild to moderate deformityto moderate deformity
Surgical correction Surgical correction –– moderate to severe moderate to severe deformitiesdeformities
BIDMC, PACS 4646
Alyssa Courtney, Year IVGillian Lieberman, MD
Nuchtern JG, Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed September 11th 2010.
Congenital Osseous Congenital Osseous AbnormalitiesAbnormalities
1.1. PectusPectus ExcavatumExcavatum2.2. PectusPectus CarinatumCarinatum3.3. Tilting of the SternumTilting of the Sternum4.4. SternalSternal Fusion AbnormalitiesFusion Abnormalities5.5. Rib AbnormalitiesRib Abnormalities6.6. Scapula AbnormalitiesScapula Abnormalities
4747
Alyssa Courtney, Year IVGillian Lieberman, MD
Tilting of the SternumTilting of the Sternum
Deviation of the typical horizontal positioningDeviation of the typical horizontal positioningof the sternum in the transverse axis of theof the sternum in the transverse axis of thebody.body.Imaging:Imaging:
Usually not apparent on radiographsUsually not apparent on radiographs
Secondary lateral displacement of medial Secondary lateral displacement of medial heads of the adjacent clavicles may assist heads of the adjacent clavicles may assist detectiondetection
4848
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Tilting of the Sternum: Tilting of the Sternum: AssociationsAssociations
Anterior Anterior subluxationsubluxation of the adjacent of the adjacent clavicularclavicular headhead
Abnormal convexity of the adjacent rib Abnormal convexity of the adjacent rib resulting in a palpable chest wall bumpresulting in a palpable chest wall bump
4949
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Congenital Osseous Congenital Osseous AbnormalitiesAbnormalities
1.1. PectusPectus ExcavatumExcavatum2.2. PectusPectus CarinatumCarinatum3.3. Tilting of the SternumTilting of the Sternum4.4. SternalSternal Fusion AbnormalitiesFusion Abnormalities5.5. Rib AbnormalitiesRib Abnormalities6.6. Scapula AbnormalitiesScapula Abnormalities
5050
Alyssa Courtney, Year IVGillian Lieberman, MD
SternalSternal Fusion AbnormalitiesFusion AbnormalitiesExample: Axial Example: Axial noncontrastnoncontrast CT of 1 month old boy with a bifid sternum: CT of 1 month old boy with a bifid sternum:
markedmarked separation of the separation of the clavicularclavicular headsheads and and depression of soft tissues depression of soft tissues in the location of the expected upper sternumin the location of the expected upper sternum
May be an May be an isolated isolated abnormality and abnormality and can require can require surgical surgical correction to correction to prevent prevent cardiopulmonary cardiopulmonary compromisecompromise
5151
Alyssa Courtney, Year IVGillian Lieberman, MD
From Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
SternalSternal Fusion Abnormalities: Fusion Abnormalities: AssociationsAssociations
Example: Lateral Chest Radiograph demonstrating Example: Lateral Chest Radiograph demonstrating absence absence of sternum and of sternum and sternalsternal ossification centersossification centers
Severe Severe sternalsternal fusion fusion abnormaliiesabnormaliies are are associated with associated with ––
Congenital heart Congenital heart disease disease egeg ectopiaectopia cordiscordis ((extrathoracicextrathoracic heart)heart)
PentalogyPentalogy of Cantrell of Cantrell (combination of severe (combination of severe sternum, diaphragm, heart sternum, diaphragm, heart and abdominal wall defects)and abdominal wall defects)
5252
Alyssa Courtney, Year IVGillian Lieberman, MD
From Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Congenital Osseous Congenital Osseous AbnormalitiesAbnormalities
1.1. PectusPectus ExcavatumExcavatum2.2. PectusPectus CarinatumCarinatum3.3. Tilting of the SternumTilting of the Sternum4.4. SternalSternal Fusion AbnormalitiesFusion Abnormalities5.5. Rib AbnormalitiesRib Abnormalities6.6. Scapula AbnormalitiesScapula Abnormalities
5353
Alyssa Courtney, Year IVGillian Lieberman, MD
Rib AbnormalitiesRib AbnormalitiesTypes: Types: Agenesis, Agenesis, hypoplasiahypoplasia, and bifid configuration., and bifid configuration.
Developmental anatomic variations can present Developmental anatomic variations can present as asymptomatic palpable chest wall massesas asymptomatic palpable chest wall massesExamples Examples ––
Prominent convexity of anterior rib or costal Prominent convexity of anterior rib or costal cartilagecartilage
Prominence of Prominence of costochondralcostochondral junctionjunction
Small Small parachondralparachondral nodules of unknown originnodules of unknown origin
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Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Congenital Osseous Congenital Osseous AbnormalitiesAbnormalities
1.1. PectusPectus ExcavatumExcavatum2.2. PectusPectus CarinatumCarinatum3.3. Tilting of the SternumTilting of the Sternum4.4. SternalSternal Fusion AbnormalitiesFusion Abnormalities5.5. Rib AbnormalitiesRib Abnormalities6.6. Scapula AbnormalitiesScapula Abnormalities
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Scapula Abnormalities: Scapula Abnormalities: SprengelSprengel’’ss DeformityDeformity
Failure of descent of the scapula.Failure of descent of the scapula.
Most notable scapula deformityMost notable scapula deformity
Sometimes the scapula is tethered to the spine Sometimes the scapula is tethered to the spine by an by an osteocartilaginousosteocartilaginous connection called the connection called the omohyoidomohyoid bone bone
Can cause neck stiffness and restrict Can cause neck stiffness and restrict abduction of the armabduction of the arm
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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 Am. 2005; 43: 355 – 370.Thacker MM. Sprengel Deformity. eMedicine. July 2009. Accessed on September 11th 2010.
Scapula Abnormalities: Scapula Abnormalities: SprengelSprengel’’ss Deformity, Associations and TreatmentDeformity, Associations and Treatment
Associated with Associated with
KlippelKlippel--FeilFeil syndromesyndrome
Osseous abnormalitiesOsseous abnormalities
Spinal cord abnormalitiesSpinal cord abnormalities
Treatment:Treatment: Physiotherapy or surgeryPhysiotherapy or surgery
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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 Am. 2005; 43: 355 – 370.Thacker MM. Sprengel Deformity. eMedicine. July 2009. Accessed on September 11th 2010.
Congenital Soft Tissue Congenital Soft Tissue AbnormalitiesAbnormalities
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Congenital Soft Tissue Congenital Soft Tissue AbnormalitiesAbnormalities
1.1. Poland SyndromePoland Syndrome2.2. Lymphatic MalformationsLymphatic Malformations3.3. Venous MalformationsVenous Malformations
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Poland SyndromePoland SyndromeRare congenital malformation Rare congenital malformation of chest wall with of chest wall with hypoplasiahypoplasia ororaplasiaaplasia of the of the pectoralispectoralis majormajormuscle and adjacentmuscle and adjacentcartilaginous, osseous, and softcartilaginous, osseous, and softtissue structures.tissue structures.Clinical asymmetry of the chestClinical asymmetry of the chest
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Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Schwartzstein RM. Diseases of the chest wall. Up to Date. May 2010. Accessed 11th September 2010.
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).
Poland Syndrome: Pathophysiology Poland Syndrome: Pathophysiology and Incidence and Incidence
Pathophysiology: Pathophysiology:
Unknown Unknown
Hypothesized to occur as a Hypothesized to occur as a result of result of ipsilateralipsilateral subclaviansubclavian artery disruptionartery disruption
Incidence: Incidence:
1/30 000 live births1/30 000 live births
Usually unilateralUsually unilateral
Males > FemalesMales > Females
Right > LeftRight > Left6161
Alyssa Courtney, Year IVGillian Lieberman, MD
Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Schwartzstein RM. Diseases of the chest wall. Up to Date. May 2010. Accessed 11th September 2010.
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).
Poland Syndrome: Chest RadiographsPoland Syndrome: Chest Radiographs
The The hypoplasiahypoplasia of chest wall soft tissues of chest wall soft tissues results in relative lunacy of affected results in relative lunacy of affected hemithoraxhemithorax
Differential diagnoses from radiograph:Differential diagnoses from radiograph:
Pulmonary entities that cause air trappingPulmonary entities that cause air trapping
Congenital lobar emphysemaCongenital lobar emphysema
Obstruction from a foreign bodyObstruction from a foreign body
SwyerSwyer--James syndrome James syndrome
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Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Schwartzstein RM. Diseases of the chest wall. Up to Date. May 2010. Accessed 11th September 2010.
Poland Syndrome: TreatmentPoland Syndrome: Treatment
Surgical correction if severe chest wall Surgical correction if severe chest wall deformitiesdeformities
CT scanning or MR imaging is useful to CT scanning or MR imaging is useful to determine the extent of the deformity for determine the extent of the deformity for presurgicalpresurgical planningplanning
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Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Schwartzstein RM. Diseases of the chest wall. Up to Date. May 2010. Accessed 11th September 2010.
Congenital Soft Tissue Congenital Soft Tissue AbnormalitiesAbnormalities
1.1. Poland SyndromePoland Syndrome2.2. Lymphatic MalformationsLymphatic Malformations3.3. Venous MalformationsVenous Malformations
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Lymphatic MalformationsLymphatic MalformationsIncreased number of dilated lymphatic channelsIncreased number of dilated lymphatic channelslined by endotheliumlined by endothelium
MicrocysticMicrocystic, , macrocysticmacrocystic, or combined, or combined
Most common in: Most common in: AxillaAxilla, chest, , chest, cervicofacialcervicofacial regionregion
In the chest: In the chest:
focal or diffuse masses confined to the focal or diffuse masses confined to the subcutaneuossubcutaneuos tissues ortissues or
involve the spine and/or involve the spine and/or mediastinummediastinum
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Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
From Children’s Hospital Boston. Lymphatic Malformation. Accessed 14th September 2010.
Lymphatic Malformations: MRI (1)Lymphatic Malformations: MRI (1) 2 year old boy with left lateral chest wall lymphatic
malformations
Axial (C+) T2Axial (C+) T2-- weighted MRI weighted MRI demonstrating a demonstrating a multiloculatedmultiloculated highhigh--signalsignal-- intensity left lateral intensity left lateral chest wall mass chest wall mass with with internal internal septationsseptations
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Alyssa Courtney, Year IVGillian Lieberman, MD
From Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Lymphatic Malformations: MRI (2)Lymphatic Malformations: MRI (2) 2 year old boy with left lateral chest wall lymphatic malformations
Axial fatAxial fat--saturated, T1saturated, T1-- weighted weighted postcontrastpostcontrast MRI MRI –– low signal low signal intensity of the cystic intensity of the cystic component component and and enhancement of walls enhancement of walls and and septationsseptations
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From Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Congenital Soft Tissue Congenital Soft Tissue AbnormalitiesAbnormalities
1.1. Poland SyndromePoland Syndrome2.2. Lymphatic MalformationsLymphatic Malformations3.3. Venous MalformationsVenous Malformations
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Venous MalformationsVenous MalformationsIsolated or multiple dilated, tortuous, thinIsolated or multiple dilated, tortuous, thin--walled (lack of smooth muscle) venous walled (lack of smooth muscle) venous structuresstructures
Grow in proportion to child growthGrow in proportion to child growth
Focal abnormalities through to diffuse Focal abnormalities through to diffuse involvement of the deeper soft tissues and involvement of the deeper soft tissues and bonebone
Affects chest wall less than lymphatic Affects chest wall less than lymphatic abnormalitiesabnormalities
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Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Venous Malformations: ImagingVenous Malformations: Imaging
Ultrasound Ultrasound –– hypoechoichypoechoic, , isoechoicisoechoic, or , or hyperechoichyperechoicPhlebolithsPhleboliths may also be identifiedmay also be identifiedColor Doppler spectral tracings demonstrate Color Doppler spectral tracings demonstrate
either loweither low--flow venous patterns or no flowflow venous patterns or no flowMRI MRI -- evaluates extent of involvement evaluates extent of involvement
and characterization of flow and characterization of flow
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Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Venous Malformations: Venous Malformations: TimeTime--resolved MR Angiography (1)resolved MR Angiography (1)
Axial (C+) MR STIR Axial (C+) MR STIR image with a image with a cluster cluster of of serpiginousserpiginous highhigh-- signal structuressignal structures
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10 year old boy who has left posterolateral chest wall venous malformations
Alyssa Courtney, Year IVGillian Lieberman, MD
From Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Venous Malformations: Venous Malformations: TimeTime--resolved MR Angiography (2)resolved MR Angiography (2)
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Axial Axial postcontrastpostcontrast T1T1-- weighted MRI weighted MRI -- late late venousvenous--phase phase enhancement of enhancement of abnormal vascular abnormal vascular structuresstructures
10 year old boy who has left posterolateral chest wall venous malformations
Alyssa Courtney, Year IVGillian Lieberman, MD
From Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Venous Malformations: Venous Malformations: Treatment Treatment
ObservationObservation
SclerotherapySclerotherapy
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Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Final PatientFinal Patient
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Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.
Companion Companion Patient 2Patient 2
WhereWhere’’s the s the congenital congenital chest wall chest wall abnormality?abnormality?
Please turn to the next Please turn to the next page to reveal thepage to reveal theabnormalityabnormality
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Companion Companion Patient 2: Patient 2: Relevant Relevant FindingFinding
RibAbnormality
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Companion Companion Patient 2: Patient 2: Additional Additional Findings?Findings?
Can you detect the additional findingson this radiograph?
Please turn to the nextpage to reveal thefindings
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Companion Companion Patient 2: Patient 2: Additional Additional FindingsFindings
PneumomediastinumTranstracheal oxygenCathetorSubcutaneous neck EmphysemaMulti-focal linear atelectasis in bilateral mid and lower lungs
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SummarySummary
Chest Wall Anatomy ReviewChest Wall Anatomy Review
Types of Chest Wall Disorders in Types of Chest Wall Disorders in ChildrenChildren
Imaging Modalities: Imaging Modalities:
Chest radiography or ultrasound then Chest radiography or ultrasound then MRI or CT if requiredMRI or CT if required
Congenital Osseous AbnormalitiesCongenital Osseous Abnormalities
Congenital Soft Tissue AbnormalitiesCongenital Soft Tissue Abnormalities
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References (1)References (1)1. Anatomy TV. http://www.anatomy.tv. Accessed 11th September 2010.2. 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.
3. Children’s Hospital Boston. Lymphatic Malformation. http://www.childrenshospital.org/az/Site1256/mainpageS1256P0.html. Accessed 14th
September 2010.4. Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children.
Radiol Clin N Am. 2005; 43: 355 – 370.5. Grissom LE. Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in
Roentgenology. 1998; Vol XXXIII (No. 2):199-208.6. Habib M. Mahajan S. Kuchey GA. Gupta D. Sharma S. Poland Syndrome, a rare
entity. The Internet Journal of Orthopedic Surgery. 2009; 12 (1). http://www.ispub.com/journal/the_internet_journal_of_orthopedic_surgery/volume_12 _number_1_3/article/poland_syndrome_a_rare_entity.html. Accessed 14th September 2010.
7. 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.
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References (2)References (2)8. 8. Mayer OH. Mayer OH. PectusPectus excavatumexcavatum: Etiology and evaluation. : Etiology and evaluation. Up to DateUp to Date. May 2010. . May 2010.
http://http://www.uptodate.com/online/content/topic.do?topicKeywww.uptodate.com/online/content/topic.do?topicKey=pedipulm/21013&selec=pedipulm/21013&selec tedTitle=5%7E20&source=tedTitle=5%7E20&source=search_resultsearch_result. Accessed 11. Accessed 11thth September 2010.September 2010.
9. 9. Mayer OH. Mayer OH. PectusPectus excavatumexcavatum: Treatment. : Treatment. Up to DateUp to Date. May 2010. . May 2010. http://http://www.uptodate.com/online/content/topic.do?topicKeywww.uptodate.com/online/content/topic.do?topicKey=pedipulm/21914&selec=pedipulm/21914&selec tedTitle=4%7E20&source=tedTitle=4%7E20&source=search_resultsearch_result. Accessed on September 11. Accessed on September 11thth 2010.2010.
10.10. NuchternNuchtern JG. Mayer OH. JG. Mayer OH. PectusPectus CarinatumCarinatum. . Up to DateUp to Date. May 2010. . May 2010. http://http://www.uptodate.com/online/content/topic.do?topicKeywww.uptodate.com/online/content/topic.do?topicKey=pedipulm/11080&selec=pedipulm/11080&selec tedTitle=3%7E20&source=tedTitle=3%7E20&source=search_resultsearch_result. Accessed 11. Accessed 11thth September 2010.September 2010.
11.11. PACS, Beth Israel Deaconess Medical CenterPACS, Beth Israel Deaconess Medical Center12. 12. SchwartzsteinSchwartzstein RM. Diseases of the chest wall. RM. Diseases of the chest wall. Up to DateUp to Date. May 2010. . May 2010.
http://http://www.uptodate.com/online/content/topic.do?topicKeywww.uptodate.com/online/content/topic.do?topicKey=int_lung/15716&select=int_lung/15716&select edTitle=1%7E20&source=edTitle=1%7E20&source=search_resultsearch_result. Accessed 11. Accessed 11thth September 2010.September 2010.
13. 13. Thacker MM. Thacker MM. SprengelSprengel DeformityDeformity. . eMedicineeMedicine. July 2009. . July 2009. http://emedicine.medscape.com/article/1242896http://emedicine.medscape.com/article/1242896--overviewoverview. Accessed on . Accessed on September 11September 11thth 2010.2010.
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AcknowledgementsAcknowledgements
Larry Larry BarbarasBarbaras
Gillian Lieberman, MDGillian Lieberman, MD
Emily HansonEmily Hanson
Pauline Bishop, MDPauline Bishop, MD
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