CHEST WALL DEFORMITIES

CHEST WALL DEFORMITIESCarlos A. Angel, MD

CHEST WALL DEFORMITIESPectus excavatumPectus carinatumPoland syndromeSternal defectsRare lesions: Thoracic ectopia cordis Jeune asphyxiating thoracic dystrophy PECTUS EXCAVATUMMost common anterior chest wall deformity (7-38/10,000 births)Positive family history (37%-47%) 3:1 M:F ratioSpontaneous resolution is rareProgression is expected during growth spurtsTall, lanky , poor postureCause unknownCan be acquired after correction of CDH. PRESENTATIONClinical spectrumPosterior angulation of the body of the sternumPosterior angulation of the costal cartilages that meet the sternumIn severe cases posterior angulation of the most anterior portion of the osseous ribsDepression may be assymetric (carinatum/excavatum deformities)Currarino- Silvermann deformity( protrusion of sterno-manubrial joint)

PRESENTATIONMany are asymptomatic Precordial painPain after sustained exercisePalpitation (mitral valve prolapse)Systolic ejection murmur is frequently identifiedShortness of breathDecreased exercise tolerance

Associated Abnormalities704 patients

Scoliosis 107kyphosis 4Myopathy 3Poland syndrome 3Marfan syndrome 2Pierre Robin syndrome 2Prune belly syndrome 2Neurofibromatosis 3Cerebral palsy 4Tuberous sclerosis 1 CDH 1

Shamberger RC, Welch KJ,: Surgical repair of pectus excavatum. J Pediatr Surg 1998; 23:615-622

PECTUS EXCAVATUMSome 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 EXCAVATUMWork-up

CT of the chest

Pulmonary Function Tests

Echocardiogram

Type and crossmatch PRBCs

PULMONARY FUNCTIONCastile 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 FUNCTIONBrown et.al. Respiratory studies before and after surgery Vital Capacity- nl Maximum breathing capacity greater than 50%decreased (9/11 pts), increased 31% after repairOrzaleski and Cook 12 children with severe pectus excavatum deformities Significant decrease (p