This document discusses various chest wall deformities and conditions that affect the structure and function of the chest wall. It covers scoliosis, pectus excavatum, ankylosing spondylitis, flail chest, thoracoplasty, pectus carinatum, Poland Syndrome, and chest wall tumors. For each condition, it describes the presentation, pathophysiology, clinical features, treatment options, and relevant anatomy and imaging findings.
3. Scoliosis
• Scoliosis is defined as a
lateral curvature of the
spine and is associated
with rotation of the spine
and viscera adjacent to it.
• It may be (but is usually
not) accompanied by
kyphosis.
7. • Thoracic scoliosis to the right- most common
presentation.
• More prevalent- Girls.
• Higher & Increase angle of curvature- worse
prognosis.
8. Cobb’s angle
• Lines are drawn parallel
to the upper border of
the upper vertebral body
and to the lower border
of the lowest vertebra of
the structural curve.
• Perpendiculars are then
erected from these lines
to cross each other, the
angle between the
perpendiculars being the
angle of curvature or
Cobb’s angle
9. Pathology
• Total lung volume is reduced and the lungs
differ in size, with distortion of lobar shapes
due to the deformity.
• Alveolar size may vary, alveolar numbers per
acinus may correspond to that developmental
stage.
• Vascular medial hypertrophy- Cor pulmonale
10. Physiology
• Lung volumes are reduced, with a restrictive
pattern.
• VC, TLC, MVV, functional residual capacity
(FRC) and peak expiratory flow (PEF)- reduced.
• The higher the curve in the dorsal spine, the
more severe its effect on function.
• FEV1/FVC ratio is normal unless obstructive
disease such as asthma supervenes.
• Dlco is reduced
11. • Lung compliance in scoliosis is commonly low-
inability to take a deep breath.
• Exercise tolerance is limited.
• Patients with severe scoliosis (with an angle
≥100°), severe desaturation may occur during
sleep with episodes of central or obstructive
apnoea and hypopnoea.
• In severe disease- increasing hypoxia- Pulmonary
artery hypertension.
• Co2 retention- diminished ventilatory responses.
12. Clinical features
• Mild scoliosis to have respiratory symptoms-
unusual.
• Daytime somnolence may indicate the presence
of nocturnal desaturation.
• Exertional dyspnoea- due to the restriction.
• PHT and RVH often occurs in the fourth decade.
• Death most commonly occurs as a result of
respiratory failure or cardiac disease.
• Respiratory tract infection.
13. Treatment- Surgery
Surgical intervention- may ameliorate the angle of
curvature and halt the progression of scoliosis &
halt the progression of scoliosis.
• Surgical fusion of spine- role in children with
congenital spinal deformities or in children with
KS secondary to neurological defects.
• Non-fusion approach includes implantation of
growth-friendly rods such as expandable spinal
rods, titanium rib implants, or remotely
distractible magnetically controlled rods.
14.
15. Medical treatment
• Long-term domiciliary oxygen therapy-
respiratory failure,
• Assisted ventilation- PPV (nasal mask or full-face
mask),
• Life-threatening hypoventilation include tank
respirators or cuirass shells,
• Less satisfactory for the patient, but equally
effective, is nocturnal ventilation via a permanent
tracheostomy,
• Diuretic therapy- RVH,
16. Medical treatment
• Bronchodilator therapy,
• Smoking cessation,
• Maintenance of body weight within a desirable
range, engaging in frequent physical activity to
improve exercise capacity,
• Immunization against influenza and
pneumococci,
• Prompt treatment of respiratory infections,
• Psychological support for those with diminished
self-esteem.
17. Pectus Excavatum
• Pectus excavatum (funnel chest), refers to a
congenital chest wall deformity characterized by
excessive depression of the sternum and the
adjacent ribs producing a caved-in appearance to
the rib cage.
• 1st and 2nd ribs, manubrium are in normal
position.
• Asymmetrical depression may be present and the
right is often more depressed than the left.
18. Pectus excavatum
• Pectus excavatum is present at birth or within
the 1st year of life in most patients.
• It may worsen at adolescent.
• Believed to result from an abnormal growth of
cartilage around the sternum due to a defect
in collagen formation ( more prevalant in
Marfan’s)
• It may be associated with scoliosis, asthma.
19. Pectus Excavatum
• Most common anterior chest wall deformity
(7-38/10,000 births)
• Positive family history (37%-47%)
• 3:1 M:F ratio
• Spontaneous resolution is rare
• Tall, lanky, poor posture
• Causes unknown
• Can be acquired after correction of CDH.
21. PRESENTATION
• Clinical spectrum
• Posterior angulation of the body of the sternum
• Posterior angulation of the costal cartilages that
meet the sternum
• In severe cases posterior angulation of the most
anterior portion of the osseous ribs
• Depression may be assymetric
(carinatum/excavatum deformities)
• Currarino- Silvermann deformity
( protrusion of sterno-manubrial joint)
22. PRESENTATION
• Many are asymptomatic- cosmetic condition
• Precordial pain
• Pain after sustained exercise
• Palpitation (mitral valve prolapse)
• Systolic ejection murmur is frequently
identified
• Shortness of breath
• Decreased exercise tolerance
23. Haller index
• The ratio of the
transverse to AP
diameters of the rib
cage at the level of
the deepest sternal
depression.
• Normal individuals
this ratio is about 2.5.
• Ratio greater than
3.25 - significant
sternal depression.
24. Pathophysiology
• Restriction on PFT
• Haller index of 5 or higher can be associated
with mild reductions in TLC and VC
• Obstructive defects are infrequent
• CPET is usually normal in individuals with
pectus excavatum.
25. Treatment
• Surgery is most often performed for cosmetic
reasons.
• Optimal timing for repair is around 10 to 14
years of age or at a younger age if there is
significant cardiopulmonary compromise.
26. Indications for Surgery
• Progressive symptoms
• Restrictive disease, decreased work production or
oxygen uptake as demonstrated by PFT’s
• Ct scan showing cardiac compression or
displacement
• Haller index greater than 3.25
• Pulmonary atelectasis
• Mitral valve prolapse, bundle branch block
• Recurrent pectus excavatum after repair
29. Ravitch procedure
• open-resection method
• Resection of costal cartilage and a sternal
osteotomy, with or without fixation of the
sternum by external or internal supports.
• Suited for individuals with combined pectus
excavatum and carinatum, marked
asymmetry, or extensive defects involving the
upper ribs.
30.
31.
32.
33. Nuss procedure
• minimally invasive alternative
• placing a curved metal bar under the sternum
at the point of deepest depression through
small incisions made on each side of the rib
cage.
• bar is generally left in place for 2 to 4 years,
resulting in permanent chest wall remodeling.
39. ANKYLOSING SPONDYLITIS
• AS is a chronic multisystem disease characterized by
inflammation of the spine, sacroiliac and peripheral
joints as well as involvement of extra-articular organs
including the lungs and the heart.
• Spinal mobility is reduced and there is considerable
limitation of rib cage expansion.
• Men > women, 16:1
• Etiology is unclear
• AS have the HLA-B27 antigen
40. Clinical Presentation
• Late adolescence or early adulthood- back
pain or morning stiffness due to involvement
of sacroiliac joints.
• AS have involvement of a peripheral joint or
non-granulomatous anterior uveitis.
• Aortitis and dilatation of the aortic root
41. • Chest pain due to inflammation of
manubriosternal junction and/or the
sternoclavicular joints with inability to fully
expand the chest on inspiration.
• Chronic back pain may cause sleep
fragmentation.
• Rib cage expansion less than 2.5 cm should
raise the possibility of AS in young patients.
42. fusion of anterior and
posterior elements- Bamboo
sign
Inflammatory lesions of the
manubriosternal joint
Sacro-iliac fusion
43. Pathophysiology
• Limited rib cage expansion is the
pathophysiological hallmark of AS.
• This limitation results from fusion of the
costovertebral and sternoclavicular joints and
possibly leads to intercostal muscle atrophy.
• Reduction in chest wall and total respiratory
system compliance but lung compliance is
generally normal.
44. • Only mild reductions in VC or TLC
• PaO2 is either in the normal range or mildly
reduced.
• Exercise capacity may be mildly decreased
45. Pleuropulmonary Abnormalities
• Upper lobe fibrobullous
disease
• manifest as interstitial
infiltrates, fibrosis with
honeycombing, or
cavitation.
• The pathogenesis of
upper lobe fibrobullous
disease is unknown.
46. Treatment
• pain relief, physical therapy, and measures to
maintain posture
• TNF antagonist- effect on pain control, fatigue,
spinal flexibility, and quality of life.
• Screened for latent TB and receive
prophylactic treatment with isoniazid before
starting treatment.
47. Flail chest
• double fractures of three or more adjacent ribs or
the combination of sternal and rib fractures are
required to produce a flail segment of the rib
cage and lead to respiratory failure.
Causes:
• trauma related to automobile accidents or falls
• aggressive cardiopulmonary resuscitation
• pathological rib fractures (i.e., multiple myeloma,
other metastases).
48. • Disruption of rib cage integrity in flail chest
renders motion of the flail segment entirely
dependent on pleural pressure changes during
breathing.
• The most common anatomical location for flail
chest is the lateral rib cage.
• VC and FRC can be reduced to as much as 50% of
predicted.
• With surgical stabilization , VC usually returns to
normal range in about 3 months.
49.
50. Inspiration
• Pleural pressure becomes
sub-atmospheric, which is
inflationary to the lung and
deflationary to the rib
cage.
• Consequently, the flail
segment moves inward
rather than outward during
inspiration.
51. Expiration
• Pleural pressure becomes
more positive and the flail
segment moves outward.
• This paradoxical motion of
the flail segment is
augmented by conditions
that load the respiratory
system thereby amplifying
pleural pressure swings.
53. Treatment
Objective of treatment:
a) Pain control:
• enhance cough efficiency
• use of oral or intravenous narcotics, intercostal nerve
blocks, or epidural anesthesia.
b) Minimize atelectasis:
• frequent tracheal bronchial toilet
c) Mechanical ventilation:
• low impedance modes- CPAP- associated with less chest
wall distortion during inspiration.
• Reduce morbidity and length of hospitalization.
c) Surgical fixation of the flail chest
54. Thoracoplasty
Pre-antituberculous chemotherapy era,
thoracoplasty was the standard surgical
approach to control PTB.
• Rib removal,
• Rib fracture,
• Phrenic nerve resection,
• Lung compression by filling the pleural space
with foreign material (i.e., ping pong balls)
55.
56. Clinical Presentation
• Dyspnea,
• Severe restrictive dysfunction,
• Chronic hypercapnic respiratory failure
• The severity of the restrictive defect was often
comparable to KS.
57. • Thoracoplasty is indicated for treatment of
bronchopleural fistulae that have failed to
close following decortication or
• For treatment of persistent empyema in which
decortication is not feasible or has failed to
eradicate the infection
59. Pectus carinatum or pigeon chest
• sternum is prominent, forming an anterior ridge
like the keel of a ship with the ribs falling away
steeply on either side.
• associated with other congenital anomalies,
especially cardiac lesions and coarctation of the
aorta.
• Less frequent than pectus excavatum
• M:F 4:1
• Cause: unknown
• Positive family history in 26%
60. PECTUS CARINATUM
Presentation:
• Symmetric or asymetric protrusion of the
sternum
• Associated lateral depression of the ribs
• Pain in the area
• Some patients experience exercise limitation
• Rotation of the sternum is often seen
• Chondromanubrial protrusion (rare), results in a
comma-shaped sternum ( these children have
and increased incidence of heart disease)
62. Poland’s syndrome
• Poland’s syndrome consists of syndactyly plus
ipsilateral absence of the pectoralis major
muscle.
• This results in hypoplasia of the ribs on the
affected side and hypertranslucency of the
lung on CXR.
• The etiology is unknown.
64. Chest Wall Tumors
• majority are metastatic or local invasive
• primary chest wall tumor 5% of all thoracic
tumor 1% of all primary tumor
• 40-60% of primary chest wall tumor is
malignancy Rib cage is the most common site
68. Osteochondroma
most common benign bone neoplasm
(50% all benign rib tumors)
painless mass
arise from metaphyseal region of rib and
develops as stalked mass with
cartilaginous cap
male x3 > female
complete surgical resection of treatment of
choice
69. Chondroma
• 15% of all benign rib neoplasms
• 20-30 years, equal both sexes- costochondral
junction
• slowly enlarging a symptomatic mass
• expansile medullary mass causes thinning of
cortex difficult to ddx chondroma and low
grade chondrosarcoma
• all chondroma must managed as malignant
lesion, with wide excision
70. Fibrous Dysplasia
benign, cystic lesions
30% of all benign chest wall tumor
most common present as a solitary mass in lateral of posterior
rib cage
male and female are equally
asymptomatic slow growing mass
multiple lesion can occur in Albright’s syndrome
a trabeculated, expansile lesion with a ground glass center and
thinning of the cortex
conservative
71.
72. Eosinophilic Granuloma
lymphoreticular system disease, not a true bone
tumor
fever, malaise, wright loss, lymphadenopathy,
splenomegaly, leukocytosis, eosinophilia, anemia
expansile lesion with periosteal new bone
formation and uneven destruction of cortex
radiation, chemotherapy and corticosteroid
73.
74. Chondrosarcoma
most common primary chest wall bone neoplasm
60% arises in costochondral arches or sternum
30-40 years old
cause is unknown
slow enlarging mass become painful
lobulated mass arising in medullary portion with
destruction of cortex and mineralization of tumor
matrix(mottled type of calcification)
treatment is complete resection, 5-yr survival of 64%
75.
76. Ewing’s sarcoma
small round cell sarcoma
15% presented at chest wall, 17% all primary chest wall tumor
age >40 years old, male x2> female
painfull, enlarging mass associated with fever, malaise,
leukocytosis, anemia, increase ESR
mottled destruction containing lytic and blastic area, onion skin
appearance
medical treatment, surgical role to biopsy
5-year survival 48%
77.
78. Osteosarcoma
10% of all primary chest wall tumor, poor
prognosis
teenagers, and young adults
rapidly enlarging, painful mass, high ALP
bone destruction with indistinct borders merge
into adjacent normal bone, sunbrust
appearance
chemotherapy with wide
resection 5-year survival 15%
79.
80.
81. Desmoid
• locally invasive tumors
• most common chest wall sarcoma 21%
• adolescence - 40yrs old, male=female
• associated with Gardner’s syndrome
• asymptomatic, paresthesia, hyperesthesia, motor
weakness, progressive neural encasement
• homogenous mass with indistinct border
• treat with wide resection, radiation used in recurrent
• 5-year survival 93%, 5-year recurrent rate 29%
82.
83. Soft tissue sarcoma
one-half of primary malignant chest wall
adult life except rhabdomyosarcoma(child
and young adult <45)
painless mass
wide surgical resection, multimodality
therapy
overall 5-year survival 60%
84.
85. Metastatic disease and Recurrent
breast carcinoma
metastatic disease 20-30% of all chest wall neoplasm
role of surgical resection is controversy
criteria for curative resection:
1. chest wall is the only site of disease
2. loco-regional disease is controlled
3. complete resection with negative margin is possible.
• 5-year survival for chest wall metastasectomy 20%
• 10-12% stage II CA breast recur locally after mastectomy
