Rib notching

Causes of rib notching

1. RibNot ching Dr. Y. Madhu Madhava Reddy

2. Introduction Rib notching  refers to deformation of the superior or inferior surface of the rib. It can affect single ribs (from trauma or solitary masses e.g. schwannoma ) or can affect multiple ribs. The differential differs according to whether it is the superior or inferior surface that is notched.

3. Differentials Superior rib notching: The superior rib notching can be caused by 1. Abnormal osteoblastic activity osteogenesis imperfecta connective tissue diseases rheumatoid arthritis systemic lupus erythematosus (SLE)

4. Superior rib notching 2. Abnormal osteoclastic activity hyperparathyroidism 3. Miscellaneous neurofibromatosis type 1 restrictive lung disease Poliomyelitis Marfan syndrome

5. Inferior rib notching Aka Roesler sign 1. Enlarged collateral vessels coarctation of the aorta interrupted aortic arch subclavian artery obstruction Takayasu disease Blalock- Taussig shunt : involves only upper 2 rib spaces AVM of the chest wall SVC obstruction with enlarged venous collaterals pulmonary AVM

6. Inferior rib notching 2. Neurogenic tumours schwannoma  (usually single) neurofibromatosis type 1 (rarely can be superior if neurofibroma is very large)

7. Superior and Inferior Rib notching 3. Superior and Inferior Rib notching - hyperparathyroidism

8. Superior rib notching O steogenesis imperfecta : The hallmark feature of osteogenesis imperfecta is that fragile bones that fracture easily. It affects both bone quality and quantity. It is due to abnormality of type 1 collagen, so sclera, cornea, joints, and skin are abnormal. Due to the abnormal osteoblastic activity the superior part of ribs is deformed.

9. Types of OI Type 1: Most common. Sclera are blue, Bony fragility is mild, Stature is reduced, Deafness can occur in adult life. Osteoporosis occur with cortical thinning. Long Bones are bowed and gracile .

10. OI

11. Types of OI Type 2: Gross demineralisation of long bones with thin cortices. Numerous healing or healed rib fractures are seen. Type 2A: Long bones are bowed, short and broad. Numerous fractures are seen. Ribs are broad with continuous beading . Type 2B: Long bones as in case of type 2A, but ribs show less or no beading.

12. Types of OI Type 2C : the long bones are thinned, show numerous fractures and ribs are too thin and beaded. Type 3 : over all bones are demineralised , Vertebral compression is seen, kyphoscoliosis may present. Sutures may be wide and wormian bones persists. Associated with dentinogenesis imperfecta .

13. Types of OI Type 4: the ribs are so soft and thin that the down ward pull of the intercostal muscles make their posterior portion convex downward.

14. OI

15. OI

16. ZEBRA LINES IN BISPHOSPHONATE THERAPHY

17. Rheumatoid Arthritis It is a chronic systemic inflammatory disease which affects many organs, but predominantly attacks the synovial tissues and joints . Proximal joints are affected first, with symmetrical involvement of joints.

18. Rheumatoid Arthritis Diagnosis is based on a combination of clinical, radiographic and serological criteria . Morning stiffness lasting at least 1 hour before maximal improvement Soft tissue swelling of 3 or more joints observed by a physician Swelling of the proximal interphalangeal , metacarpophalangeal , or wrist joints Symmetric swelling Rheumatoid nodules The presence of rheumatoid factor; and Radiographic erosions and/or periarticular osteopenia in hand and/or wrist joints.

19. Rheumatoid Arthritis The erosions are typical being localized defects in the superior aspect of the upper ribs towards their posterior ends. These erosions occur symmetrically and involve each particular rib at a constant distance from the costo verterbral joint.

20. RA

21. RA Clavicular erosion

22. SLE It is a complex autoimmune disease with multisystem involvement.  Diagnosis is made using the following criteria: Malar rash, discoid rash, photo sensitivity, pleuritis / pericarditis etc. SLE affects bone formation and bone strength there by causing the rib notching.

23. Marfan syndrome Marfan syndrome  is a multi system hereditary connective tissue disease with a high penetrance and variable expression. Pathology:- Results from a defect in fibrillin 1 (FBN1) gene located in chromosome 15 which is responsible for cross linking collagen. 

24. Disease spectrum and associated features Skeletal:- General tall stature osteopaenia joint laxity recurrent dislocations

25. Marfan syndrome Spine / skull High arched palate Atlanto -axial subluxation Dural ectasia Increased interpedicular distance kyphoscoliosis Enlarged sacral foramina Meningocoele pre sacral meningocoele lateral sacral meningocoele Vertebral scalloping

26. Marfan syndrome Cardiovascular:- Arterial dissection Aortic aneurysm Aortic valve regurgitation (AR) Aortic coarctation Aortic sinus dilatation Myxomatous degeneration of the mitral valve Pulmonary arterial dilatation

27. Marfan syndrome Ocular:- Ectopia lentis   lens usually displaced upwards and out Myopia Retinal detachment Megalocornea

28. Hyperparathyroidism It   is the effect of excess parathyroid hormone in the body. Increased levels of parathyroid hormone (PTH) lead to increased osteoclastic activity. The resultant bone resortpion produces cortical thinning ( subperiosteal resorption ) and osteopaenia . This leads to the rib notching on Chest X ray.

29. Hyperparathyroidism Hyperparathyroidism causes both superior and inferior rib notching due to the increased levels of PTH in blood initiating the osteoclastic reaction.

30. Inferior rib notching ( Roesler sign) Enlarged collateral vessels:- Coarctation of the aorta Interrupted aortic arch Subclavian artery obstruction Takayasu disease Blalock- Taussig shunt : involves only upper 2 rib spaces AVM of the chest wall SVC obstruction with enlarged venous collaterals Neurogenic tumours :- Schwannoma  (usually single) Neurofibromatosis type 1  (rarely can be superior if neurofibroma is very large)

31. Coarctation of the aorta Coarctation of the aorta  refers to a narrowing of the aortic lumen. It can be primarily divided into two types Infantile (pre- ductal ) form  Adult ( juxta-ductal , post- ductal or middle aortic) form 

32. Coarctation of the aorta Figure of 3 sign  - contour abnormality of the aorta Inferior rib notching -  Roesler sign Secondary to dilated intercostal collateral vessels Seen only in long standing cases, and therefore not seen in infancy Seen in 70% of cases presenting in older children or adults If unilaterally seen on the left, then this suggests an associated aberrant right subclavian artery arising after the coarctation .

33. Coarctation of the aorta If unilaterally seen on the right, then the origin of the left  subclavian artery is distal to the coarctation . Most often involves 4 th -8 th  ribs Occasionally involves 3 rd  and 9 th  ribs Does not involve 1 st  and 2 nd  ribs (the associated arteries are branches of the costocervical trunk, and thus proximal to coarctation )

34. Coarctation of the aorta Coarctation of the aorta with an aberrant right subclavian artery : Left-sided rib notching is seen. Occurs when the aberrant right subclavian artery arises after the coarctation . Coarctation of the aorta proximal to the left subclavian artery : Right-sided rib notching occurs.

35. Coarctation of the aorta Rib notching in Coarctation of aorta occurs due to dilatation of the posterior intercostal arteries, which act as collateral vessels. Since 1 st and 2 nd posterior intercostal arteries arise from the costocervical trunk of sub clavian artery rather than descending aorta, they do no form a collateral path and hence do no cause rib erosion.

36. COA

37. FIGURE OF 3

38. COA

39. COA

40. Interrupted aortic arch It is an uncommon congenital cardiovascular anomaly where there is a separation between the ascending and descending aorta  . It can either be complete or connected by a remnant fibrous band . An accompanying large ventricular septal defect  (VSD) and or patent ductus arteriosus (PDA) is frequently present. Pathology Faulty embryological development of the aortic arch (thought to occur during the 5 th  to 7 th  week of intra uterine life).

41. Classification:- Type A  : second commonest : interruption occurs distal to the left subclavian arterial origin  Type B  : commonest  (> 50 %) : interruption occurs  between left common carotid arterial and left subclavian origins Type C  : rare : interruption occurs proximal to left common carotid arterial origin Each type is divided into 3 subtypes  : sub-type 1  : normal subclavian artery sub-type 2  : aberrant subclavian artery sub-type 3  : isolated subclavian artery that arises from the ductus arteriosus .

42. Type A  : second commonest : interruption occurs distal to the left subclavian arterial origin  Type B  : commonest  (> 50 %) : interruption occurs  between left common carotid arterial and left subclavian origins Type C  : rare : interruption occurs proximal to left common carotid arterial origin

43. Abberant Right Subclavian Art.

44. Radiographic features Plain film - CXR Plain film features are often non specific   The aortic knuckle may be absent May show cardiomegaly Antenatal ultrasound The right ventricle may appear a lot larger than the left The ascending aorta may also appear more vertical than usual. MRI / MRA Non visualisation of portion of interruption Great vessels may show a "V" configuration on coronal imaging  2

45. IAA

46. Subclavian artery obstruction Takayasu disease Blalock- Taussig shunt : involves only upper 2 rib spaces

47. Takayasu arteritis Takayasu arteritis (TA)  (also known as  idiopathic medial aortopathy   or   pulseless disease ) is a granulomatous large vessel  vasculitis  predominantly affecting the aorta and its major branches. It may also affect the pulmonary arteries.

48. Takayasu arteritis Pathology  There is segmental and patch granulomatous inflammation of the aorta which results in stenosis , thrombosis and aneurysm formation. Half of patients present with an initial systemic illness whereas the other 50% present with late-phase complications. 

49. Takayasu arteritis Two phases of the disease are classically described :- Pre pulseless phase : characterised by nonspecific systemic symptoms Pulseless phase : presents with limb ischaemia or  renovascular hypertension.  

50. Takayasu arteritis The initial systemic illness may include symptoms of malaise, fever, night sweats, weight loss and arthralgia . There is often anaemia with raised inflammatory markers. This phase gradually resolves with initiation of the chronic phase which is characterised by inflammatory and obliterative changes in the aorta and its branches. There are often reduced or absent peripheral pulses, giving rise to it's alternative name of " pulseless disease

51. Takayasu arteritis Subtle notching of the first three ribs may be an expression of sub clavian artery occlusion . Nothing of the lower ribs is usually associated with severe narrowing or occlusion of the upper abdominal aorta with intercostal arteries serving as prominent collateral channels.

52. Location and classification Type I  - classic type involving the solely the aortic arch branches : brachiocephalic trunk, carotid and subclavian arteries  Type II  -    IIa  - involvement of the aorta solely at its ascending portion and/or at the aortic arch +/- branches of the aortic arch  IIb  - involvement of the descending thoracic aorta +/ - ascending or aortic arch + branches  Type III  - involvement of the thoracic and abdominal aorta distal to the arch and its major branches (i.e. descending thoracic aorta + abdominal aorta +/ - renal arteries) Type IV  - sole involvement of the abdominal aorta and/or the renal arteries Type V  - generalised involvement (all aortic segments)

53. Radiographic features Ultrasound :- Homogeneous circumferential thickening of affected vessels (indistinguishable from atherosclerotic plaque) Vascular occlusion +/- dilation Flow velocity elevations beyond stenotic segments

54. CT/MRI:- Wall thickening - acute active phase Wall enhancement - acute active phase Aortic valve disease - stenosis , regurgitation Occlusion of major aortic branches Aneurysmal dilatation of the aorta or its branches.  Pseudoaneurysm formation  Diffuse narrowing distally (i.e. descending and abdominal aorta ) - in late phase

55. Blalock- Taussig shunt Blalock- Taussig shunt: involves only upper 2 rib spaces. A  Blalock- Taussig (BT) shunt  is a palliative procedure performed in patients with  tetralogy of Fallot  (prior to the ability to repair the defect) to increase the pulmonary blood-flow.  The shunt sacrificed the  subclavian artery (with a distal ligation) and the proximal portion is routed downwards to an end to side anastomosis with the ipsilateral branch of the pulmonary artery. 

56. Blalock- Taussig shunt

57. Blalock- Taussig shunt Rib notching is Ipsilateral to the side of shunt. Due to division of all the branches of the first part of the subclavian artery performed during shunt creation.

58. Points to remember Coarctation of aorta proximal to left subclavian artery : Right sided rib notching Coarctation of aorta with an abberant right subclavian artery: left sided rib notching Sub clavian stenosis : ipsilateral to the side of stenosis . Blalock taussig shunt: ipsilateral to the side of shunt.

59. Superior vena caval obstruction Superior vena caval obstruction   can occur from of a wide variety of pathologies.  Superior vena caval  syndrome refers to the clinical syndrome with symptoms that results from obstruction of the superior vena cava.

60. Causes include : - Malignancy, e.g. lung cancer (the most common cause) Central venous catheters Pacemaker wires Fibrosing mediastinitis Due to development of collateral channels to the inferior vena cava via the intercostal veins rib notching occurs in cases of SVC obstruction

61. Neurogenic tumours :- Schwannoma  (usually single) Neurofibromatosis type 1 (rarely can be superior if neurofibroma is very large) They arise from the intercostal neurovascular bundles. They grow large in size causing rib notching.

62. NeuroVascular Bundle

63. Neurogenic tumor

64. NF 1

65. RIBBON RIBS

66. Bilateral rib notching

67. PROGERIA

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