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 Progression is expected during
growth spurts Tall, lanky, poor posture Cause unknown Can be
acquired after correction of CDH.
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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)
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PRESENTATION Many are asymptomatic Precordial pain Pain after
sustained exercise Palpitation (mitral valve prolapse) Systolic
ejection murmur is frequently identified Shortness of breath
Decreased exercise tolerance
PECTUS EXCAVATUM Some believe this is a purely cosmetic
condition This contrasts with the clinical impression that many
patients report improved breathing, stamina and exercise tolerance
after repair Despite 6 decades of work, no consensus has been
achieved as to what degree of cardiopulmonary impairment is
present, if any, in patients with depression chest wall
deformities
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PECTUS EXCAVATUM Work-up CT of the chest Pulmonary Function
Tests Echocardiogram Type and crossmatch PRBCs
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PULMONARY FUNCTION Castile et. al., ( 8 pts, 1 carinatum)
MeanTLC 79% of predicted No suggestion of a significant
ventilation-perfusion abnormality With maximum workload oxygen
extraction exceeded predicted values in symptomatic patients
Increases in tidal volume with exercise were uniformly depressed No
postoperative studies performed
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PULMONARY FUNCTION Brown et.al. Respiratory studies before and
after surgery Vital Capacity- nl Maximum breathing capacity greater
than 50%decreased (9/11 pts), increased 31% after repair Orzaleski
and Cook 12 children with severe pectus excavatum deformities
Significant decrease (p
PULMONARY FUNCTION Cahill et.al. 19 children and adolescents (5
carinatum, 14 excavatum) No pre-0p or pop abnormalities seen in
carinatum patients Excavatum patients showed low normal VC,
unchanged by operation Operation changed TLC Significant
improvement after operation in in maximum voluntary ventilation and
exercise tolerance Devereaux et.al. 88 pts with pectus excavatum
1-20yrs after operation (avg 8 yrs) Those with 75% had worsening
function, this was in contrast with subjective reports of
improvement in symptoms
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PULMONARY FUNCTION Wynn et.al. 12 children Decline in TLC after
repair Kaguraoka et.al. 138 pts Temporary decrease in pulmonary
function after surgery Haller et.al. 36 pts pectus excavatum, 10
controls Decreased FVC did not change after repair Improved
exercise tolerance after repair in 66% of patients, likely the
result of improved cardiac function
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PULMONARY FUNCTION Minimally Invasive Repair Studies: Borowitz
et.al. 10 pts Normal pulmonary function pre and pop Sigalet et. al.
11 pts Subjective improvement in exercise tolerance Pulmonary
function significantly reduced at 3mo. Cardiac function enhanced
with increase stroke volume Limitation in exercise had a
cardiovascular rather than a pulmonary cause Lawson et.al. 408
pectus excavatum patients 45 PFTs after Nuss procedure and bar
removal Pre-operative values for FVC, FEV1 and forced expiratory
flow were 13-20% below average Post-operative significant
improvement for al parameters greatest gains by surgery were seen
in patients older than 11 yrs
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PULMONARY FUNCTION CONCLUSIONS: In the last decade, studies of
hundreds of patients with pectus excavatum have demonstrated that
it is associated with an average decrease of pulmonary function of
85% of predicted values ( 80% is 2 SD below the norm). The increase
in function after surgery occurs in patients with normal pulmonary
parenchyma and airways
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CARDIAC FUNCTION Deformity of the heart Sternal imprint of the
anterior R ventricle Displacement of the heart to the L side
Garusi, et.al. Decreased work capacity significantly lower in
sitting than in supine position Stroke volume decreased 40.3% from
supine to sitting position Increased cardiac output is achieved by
increased heart rate, not stroke volume Beiser et. al.- Provided
further evidence that cardiac function is impaired during upright
exercise
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CARDIAC FUNCTION
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Peterson et.al. 13 patients with pectus excavatum (11
symptomatic) Radionuclide angiography Marked decrease in symptoms
during exercise after surgical correction during a regulated
exercise protocol No changes in L ventricular EF Kowaleski et. al
42 pts Echocardiographic evaluation of cardiac function
Statistically significant changes seen in RV indices (systolic,
diastolic and stroke volume) after surgery All limitations in
stroke volume result from R ventricular compression
Slide 23
CARDIAC FUNCTION Echocardiographic studies: Mitral valve
prolapse 18% ( Udoshi et.al., CHKD, Norfolk) 65% ( Saint- Mezard
et.al.) Resolution of prolapse after repair seen in 43-44%
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BODY IMAGE Large percentage of patients are self-concious about
their chests Even suicide attempts have been reported Not an
inconsequential problem Psychometric assessments in more than 300
children- Marked improvement in psychosocial functioning after
repair Severity of deformity did not correlate with the
parents/patients perception of body image concerns Pectus excavatum
is a deformity which worsens during a developmental period in which
body image is crucial
Slide 25
INDICATIONS Progressive symptoms Restrictive disease, decreased
work production or oxygen uptake as demonstrated by PFTs Ct scan
showing cardiac compression or displacement Haller index greater
than 3.25 Pulmonary atelectases Mitral valve prolapse, bundle
branch block Recurrent pectus excavatum after repair
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TIMING Can be performed in younger children with severe
exercise tolerance Best deferred until after the pubertal growth
spur
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RAVITCH PROCEDURE
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Transverse skin incision Mobilization and retraction of
pectoralis and rectus abdominis muscles Excision of deformed
cartilagues leaving the perichondrium intact Fracture of the
sternum (wedge osteotomy) Metal strut for stabilization
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NUSS PROCEDURE
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COMPLICATIONS Early: Wound infection (1%) Pneumothorax (4%)
Hemothorax (0.6%) Pneumonia (0.5%) Pericarditis (0.4%) Pleural
effussion (0.3%) Late: Bar infection (0.5%, only 0.2% required
removal) Bar displacement (1% -5.7%) Nickel allergy (3%) Recurrence
Repairs performed in children