1. CHEST WALL DEFORMITIES
Carlos A. Angel, MD

3. CHEST WALL DEFORMITIES
Pectus excavatum
Pectus carinatum
Poland syndrome
Sternal defects
Rare lesions:
Thoracic ectopia cordis
Jeune asphyxiating thoracic dystrophy

4. 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.

5. 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)

6. 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

9. Associated Abnormalities
704 patients
Scoliosis
kyphosis
Myopathy
Poland syndrome
Marfan syndrome
Pierre Robin syndrome 2
Prune belly syndrome 2
Neurofibromatosis
Cerebral palsy
Tuberous sclerosis
CDH
Shamberger RC, Welch KJ,: Surgical repair of pectus excavatum. J Pediatr Surg 1998; 23:

10. 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

11. PECTUS EXCAVATUM Work-up CT of the chest Pulmonary Function Tests
Echocardiogram
Type and crossmatch PRBC’s

13. 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

14. 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

15. 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

16. 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

17. 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 PFT’s after Nuss procedure and bar removal
Pre-operative values for FVC, FEV1 and forced expiratory flow were % below average
Post-operative significant improvement for al parameters
greatest gains by surgery were seen in patients older than 11 yrs

18. 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

19. 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

20. CARDIAC FUNCTION

21. CARDIAC FUNCTION

22. 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

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%

24. 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

25. INDICATIONS 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 atelectases
Mitral valve prolapse, bundle branch block
Recurrent pectus excavatum after repair

26. TIMING
Can be performed in younger children with severe exercise tolerance
Best deferred until after the pubertal growth spur

27. RAVITCH PROCEDURE

28. 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

29. NUSS PROCEDURE

38. 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 <4yo result in impaired growth of the ribs resulting in a band-like narrowing of the chest the chest

39. POST-OPERATIVE PERIOD
5-7 days in the hospital
Peri-operative antibiotics
Pain management ( epidural PCA, IV acetaminophen, ketoralac)
DVT prophylaxis
Incentive spirometer
Muscle relaxants (diazepam, methocarbamol)
H2 blockers

40. 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

45. NUSS vs. RAVITCH Meta-analysis (JPS May 2010)
No prospective randomized trials
9 prospective and retrospective studies
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.

46. POST-OPERATIVE ACTIVITY
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

47. BAR REMOVAL Usually performed after 2 years General anesthesia
Day surgery procedure
Chest Xray recommended

48. PECTUS CARINATUM

49. 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%

50. 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)

51. TREATMENT
Ravitch procedure
Bracing

52. BRACING

54. 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

55. 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

56. 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