Chest Wall Deformity

Scoliosis
Pectus Excavatum
Ankylosing Spondylitis
Flail chest
Thoracoplasty
Pectus carinatum
Poland Syndrome
Chest wall tumors

1. CHEST WALL DEFORMITIES Dr Kamal Bharathi. S

2. Chest Wall Deformities Scoliosis Pectus Excavatum Ankylosing Spondylitis Flail chest Thoracoplasty Pectus carinatum Poland Syndrome Chest wall tumors

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.

4. Etiology classification of scoliosis a) Non-structural Postural compensatory Sciatic Inflammatory Hysterical b) Structural Idiopathic Adolescent kyphosis c) Osteopathic Congenital scoliosis Klippel–Feil syndrome Spondylolisthesis d) Myopathic Duchenne muscular dystrophy Faciohumeroscapular dystrophy

5. Etiology classification of scoliosis e) Neuropathic Poliomyelitis Cerebral palsy Syringomyelia Neurofibromatosis Friedreich’s ataxia f) Hereditary syndromes 1. Dominant Marfan’s syndrome Ehlers–Danlos syndrome 2. Recessive Homocystinuria Morquio’s syndrome 3. Sex-linked Turner’s syndrome

6. Etiology classification of scoliosis g) Miscellaneous Long-standing unilateral lung fibrosis Burns Irradiation Thecoperitoneal shunts Empyema Thoracoplasty Hiatus hernia

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. D lco 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 . N on-fusion approach includes implantation of growth-friendly rods such as expandable spinal rods, titanium rib implants, or remotely distractible magnetically controlled rods .

14. 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,

15. 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.

16. 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.

17. 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.

18. 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.

19. Associated Abnormalities 704 patients Scoliosis 107 kyphosis 4 Myopathy 3 Poland syndrome 3 Marfan syndrome 2 Pierre Robin syndrome 2 Prune belly syndrome 2 Neurofibromatosis 3 Cerebral palsy 4 Tuberous sclerosis 1 CDH 1 Shamberger RC, Welch KJ,: Surgical repair of pectus excavatum . J Pediatr Surg 1998; 23:615-622

20. 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 )

21. 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

22. Haller index The ratio of the transverse to AP diameters of the rib cage at the level of the deepest sternal depression. N ormal individuals this ratio is about 2.5 . R atio greater than 3.25 - significant sternal depression.

23. Pathophysiology R estriction on PFT Haller index of 5 or higher can be associated with mild reductions in TLC and VC O bstructive defects are infrequent CPET is usually normal in individuals with pectus excavatum .

24. Treatment S urgery is most often performed for cosmetic reasons. O ptimal timing for repair is around 10 to 14 years of age or at a younger age if there is significant cardiopulmonary compromise.

25. 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

26. Surgery Su bcutaneous fulfilment of the funnel. Ravitch procedure. Nuss procedure. Non surgical treatment.

27. Subcutaneous fullfillment of the funnel Silicone solid implant

28. Ravitch procedure open-resection method R esection of costal cartilage and a sternal osteotomy, with or without fixation of the sternum by external or internal supports . S uited for individuals with combined pectus excavatum and carinatum , marked asymmetry, or extensive defects involving the upper ribs.

29. 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 .

30. Before and After Surgery

31. Non surgical treatment .

32. 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 . S pinal mobility is reduced and there is considerable limitation of rib cage expansion . M en > women, 16:1 E tiology is unclear AS have the HLA-B27 antigen

33. 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

34. 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.

35.  fusion of anterior and posterior elements- Bamboo sign  Inflammatory lesions of the manubriosternal joint Sacro -iliac fusion

36. 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.

37. Only mild reductions in VC or TLC PaO2 is either in the normal range or mildly reduced . Exercise capacity may be mildly decreased

38. Pleuropulmonary Abnormalities Upper lobe fibrobullous disease manifest as interstitial infiltrates, fibrosis with honeycombing, or cavitation. The pathogenesis of upper lobe fibrobullous disease is unknown.

39. Treatment pain relief, physical therapy, and measures to maintain posture TNF antagonist- effect on pain control, fatigue, spinal flexibility, and quality of life. S creened for latent TB and receive prophylactic treatment with isoniazid before starting treatment.

40. 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).

41. 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 .

42. 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.

43. Expiration P leural 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.

44. Pathogenesis of respiratory failure

45. Treatment Objective of treatment: 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) M echanical 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

46. Thoracoplasty Pre- antituberculous chemotherapy era, thoracoplasty was the standard surgical approach to control PTB. R ib removal, Rib fracture , P hrenic nerve resection, Lung compression by filling the pleural space with foreign material (i.e., ping pong balls )

47. Clinical Presentation Dyspnea , S evere restrictive dysfunction, C hronic hypercapnic respiratory failure The severity of the restrictive defect was often comparable to KS.

48. Thoracoplasty is indicated for treatment of bronchopleural fistulae that have failed to close following decortication or F or treatment of persistent empyema in which decortication is not feasible or has failed to eradicate the infection

49. Treatment Supplemental oxygen, A ntibiotics for respiratory infections, N oninvasive ventilatory support.

50. 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%

51. 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)

52. Treatment: Ravitch procedure Bracing

53. 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.

54. Poland's Syndrome

55. 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

56. Bone Malignant osteochondroma chondroma fibrous dysplasia Desmoid tumor Liposarcoma Soft tissue Bone Rhabdomyosarcoma Chondrosarcoma Osteosarcoma Ewing’s sarcoma Most Common Benign

57. Primary bone tumor Benign Osteochondroma Chondroma Fibrous Dysplasia Eosinophilic Granuloma Malignant Chondrosarcoma Ewing’s Sarcoma Osteosarcoma Solitary Plasmacytoma

58. Primary soft tissue tumor Benign Hemangioma Neu r ofib r oma Lipoma Fibroma Malignant Desmoid Soft tissue sarcoma

59. 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

60. Chond r oma 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

61. 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

62. 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

63. 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%

64. 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%

65. 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%

66. 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%

67. 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%

68. 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: chest wall is the only site of disease loco - regional disease is controlled 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

69. Thank You…!!!