Transcript CHEST WALL DEFORMITIES - TNAAP: Tennessee Chapter of …

Carlos A. Angel, MD
CHEST WALL DEFORMITIES
 Pectus excavatum
 Pectus carinatum
 Poland syndrome
 Sternal defects
 Rare lesions:
Thoracic ectopia cordis
Jeune asphyxiating thoracic dystrophy
PECTUS EXCAVATUM
 Most common anterior chest wall deformity
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(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.
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)
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
Associated Abnormalities
 704 patients
Scoliosis
kyphosis
Myopathy
Poland syndrome
Marfan syndrome
Pierre Robin syndrome
Prune belly syndrome
Neurofibromatosis
Cerebral palsy
Tuberous sclerosis
CDH
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Shamberger RC, Welch KJ,: Surgical repair of pectus excavatum. J Pediatr Surg 1998; 23:615-622
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
PECTUS EXCAVATUM
 Work-up
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CT of the chest
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Pulmonary Function Tests
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Echocardiogram
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Type and crossmatch PRBC’s
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
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 <0.001) in VC, TLC and maximal breathing capacity
 Lise and Buhlmann
Pre and pop lung volumes in 12 adults (3-11 y after operation)
Absolute lung volume only improved in patients with interval increase in height
Work capacity increased in 9/10 patients
Pop decrease in heart rate at a given power output
Some of the improvement may have resulted from increased cardiac stroke volume
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% predicted function pre-operatively had improved function
Those with >75% had worsening function, this was in contrast with subjective
reports of improvement in symptoms
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
PULMONARY FUNCTION
Minimally Invasive Repair Studies:
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Borowitz et.al.
10 pts
Normal pulmonary function pre and pop
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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
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Lawson et.al.
408 pectus excavatum patients
45 PFT’s 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
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
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
CARDIAC FUNCTION
CARDIAC FUNCTION
CARDIAC FUNCTION
 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
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%
BODY IMAGE
 Large percentage of patients are self-concious about
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their chests
Even suicide attempts have been reported
Not an inconsequential problem
Psychometric assessments in more than 300 childrenMarked 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
INDICATIONS
 Progressive symptoms
 Restrictive disease, decreased work production or
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oxygen uptake as demonstrated by PFT’s
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
TIMING
 Can be performed in younger children with severe
exercise tolerance
 Best deferred until after the pubertal growth spur
RAVITCH PROCEDURE
RAVITCH PROCEDURE
 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
NUSS PROCEDURE
COMPLICATIONS
Early:
 Wound infection
 Pneumothorax
 Hemothorax
 Pneumonia
 Pericarditis
 Pleural effussion
Late:
 Bar infection
 Bar displacement
 Nickel allergy
 Recurrence
(1%)
(4%)
(0.6%)
(0.5%)
(0.4%)
(0.3%)
(0.5%, only 0.2% required removal)
(1% -5.7%)
(3%)
 Repairs performed in children <4yo result in impaired growth of the ribs
resulting in a band-like narrowing of the chest the chest
POST-OPERATIVE PERIOD
 5-7 days in the hospital
 Peri-operative antibiotics
 Pain management ( epidural PCA, IV acetaminophen,
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ketoralac)
DVT prophylaxis
Incentive spirometer
Muscle relaxants (diazepam, methocarbamol)
H2 blockers
BAR REMOVAL
 Bar stays in place a minimum of 2 years
 Bar should be left in longer in younger patients
 Patients evaluated on an annual basis
 Exercise program very important
 Removal is an outpatient surgery procedure under GA
NUSS vs. RAVITCH
 Meta-analysis (JPS May 2010)
 No prospective randomized trials
 9 prospective and retrospective studies
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No significant difference in complication rates
 Rate of reoperation because of bar migration or persistent deformity higher in Nuss
 POP pneumothorax or hemothorax higher in Nuss
 Duration of operation longer in Ravitch
 No difference in length of hospital stay, time to ambulation, or patient satisfaction.
POST-OPERATIVE ACTIVITY
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Deep- breathing 2x day
Bathe or shower after 5 days
No waist bending, twisting or log rolling for the first 4 weeks
No slouching first month
No heavy lifting for 2 months
No contact sports for 3 mo
Other recommendations:
 MedAlert bracelet recommenced
 May have MRI
 Cardiac defibrillation with anterior posterir paddle placement
BAR REMOVAL
 Usually performed after 2 years
 General anesthesia
 Day surgery procedure
 Chest Xray recommended
PECTUS CARINATUM
PECTUS CARINATUM
 Less frequent than pectus excavatum
 M:F 4:1
 No known cause
 Mild deformity at birth worsens as the child grows
 Positive family history in 26%
 History of scoliosis in 15%
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
andincreased incidence of heart disease)
TREATMENT
 Ravitch procedure
 Bracing
BRACING
BRACING
Martinez-Ferro, JPS, 2008
 208 (154 M) pectus carinatum pts
 208 treated with bracing ( 6 yr study)
 Avg. age 12.5 yrs ( 3-18y)
 Mean utilization time 7.2 h/d x 7 mo.
 28 pts abandoned treatment
 112 completed treatment
 88.4 good or excellent results
BRACING
Stephenson, et.al. JPS, 2008
 63 pectus carinatum pts
 17 patients observed (mild defects)
 46 pts bracing program
 8 failures for non-compliance
 10 pts had not completed treatment at time of
publication
 24/28 (85.7%) that completed treatment good or
excellent results
BRACING
Laberge, 2012, JPS
 Survey of Canadian pediatric surgeons
 71% treat PC , 53% low volume practices (<5 pts/yr)
72% use bracing
83% use it for most of their patients
57% outcomes good or excellent
74% felt most or all patients were satisfied
88% agreed or strongly agreed that bracing was the
preferable option