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5/7/20151 BONE AND JOINT CONGENITAL DISORDERS WRIST DISORDERS.

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Presentation on theme: "5/7/20151 BONE AND JOINT CONGENITAL DISORDERS WRIST DISORDERS."— Presentation transcript:

1 5/7/20151 BONE AND JOINT CONGENITAL DISORDERS WRIST DISORDERS

2 5/7/20152 Congenital anomalies affect 1% to 2% of newborns Congenital anomalies affect 1% to 2% of newborns approximately 10% of those children have upper-extremity abnormalities approximately 10% of those children have upper-extremity abnormalities

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6 6 BONE AND JOINT CONGENITAL DISORDERS SPRENGEL ” S DEFORMITY FROZEN SHOULDER CARPAL TUNNEL SYNDROM TENNIS ELBOW SYNDACTYLYPOLYDACTYLY

7 5/7/20157 BONE AND JOINT CONGENITAL DISORDERS Osteochondral dysplasias Metabolic disorders Syndroms

8 5/7/20158 Osteochondral dysplasias AchondroplasiaHypochondroplasia Diastrophic dysplasia Kniest dysplasia Spondyloepiphyseal dysplasia Metaphyseal chondrodysplasia Dyschondrosteosis

9 5/7/20159 achondroplasia AD pre. =1.3/ % random new mutation (paternal age) Collagen type II chromosome 12 Enchondral ossification Rhizomelic shortness

10 5/7/ achondroplasia

11 5/7/ achondroplasia Limitation in elbow extension Genu varum Ankle valgus Waddling gait Hip flexion contracture Motor retardation Kyphosis in spine

12 5/7/ achondroplasia

13 5/7/ achondroplasia

14 5/7/ achondroplasia Mortality rate is increased because of: Sudden death CNSCV

15 5/7/ Metabolic disorders Mineral phase Rickets Organic phase OiOsteopetrosis Conective tissue syndroms EndocrinopathiesHypothyroidism Gonadal abnormality Fibrous dysplasia

16 5/7/ Osteogenesis imperfecta Collagen I Fragility of bone 21.8/ Short stature, scoliosis Defective dentinogenesis Middle ear deafness Laxity of lig. Blue sclerae & tympanic membrane Prenatal diagnosis by US except In mild forms( I,IV)

17 5/7/ Increase in woven bone does not mature to lamellar bone Osteocyte are increased Trabeculae are thin and poorly arranged Haversian canal does not developed Bone mineral density is decreased on DEXA

18 5/7/ Osteogenesis imperfecta OI congenita OI tarda

19 5/7/ SHORT STATURE BOWING OF LIMBS SCOLIOSIS BLUE SCLERA I,II DENTINOGENESIS IMPERFECTA TREFOIL SHAPED FACE

20 5/7/ Coxa vara Protrusio acetabuli Wormian bones Biconcave vertebra Long bones are thin and osteopenic Concertina appearance in femur Widening of metaphysis, popcorn epiphysis

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23 5/7/ Syndroms NeurofibromatosisArthrogryposis Down syndrome Turner syndrome Noonan syndrome

24 5/7/ Neurofibromatosis (von Recklinghausen's disease) is the most prevalent skeletal dysplasia transmitted by a single gene. It is inherited in an autosomal dominant pattern with variable expression, although the disease is believed to occur due to a new mutation in 50% of the affected individuals. The estimated prevalence is 1 per 1000 live births.

25 5/7/ Neurofibromatosis type I (NF-I) is the more common form and is characterized by peripheral neurofibromas, skeletal involvement, and “café au lait spots.” The genetic locus for NF-I has been localized to chromosome 17q11.2, an area that encodes for the protein neurofibromin. This protein is present in several organ systems and is believed to be a tumor suppressor. Neurofibromatosis type II (NF-II) manifests as central neurofibromas with bilateral acoustic neuromas and usually presents in the third or fourth decade of life. The gene for NF-II has been localized to chromosome 22 and encodes for the protein schwannomin.

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29 5/7/ Plexiform neurofibromas are large nerve tumors that are locally invasive, feel like a “bag of worms,” and have the potential for malignant transformation. Plexiform neurofibromas are large nerve tumors that are locally invasive, feel like a “bag of worms,” and have the potential for malignant transformation. Skeletal lesions: Skeletal lesions: Scoliosis is most common Scoliosis is most common Hypertrophy or hemiatrophy due to neurofibromatosis can be seen Hypertrophy or hemiatrophy due to neurofibromatosis can be seen Congenital pseudarthrosis of the tibia and forearm may be present. Congenital pseudarthrosis of the tibia and forearm may be present. Protrusio acetabuli Protrusio acetabuli

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34 5/7/ CONGENITAL ELEVATION OF THE SCAPULA (SPRENGEL'S DEFORMITY) Results from a failure of the normal caudal migration of the scapula during the fetal period of development. Results from a failure of the normal caudal migration of the scapula during the fetal period of development. Scapula with this malformation is usually hypoplastic with decreased vertical length and increased horizontal width-to-height ratio, which is 2 to 10 cm more cephalad than normal Scapula with this malformation is usually hypoplastic with decreased vertical length and increased horizontal width-to-height ratio, which is 2 to 10 cm more cephalad than normal

35 5/7/ ROTATION ROTATION The inferior pole is rotated medially with the glenoid displaced inferiorly. The inferior pole is rotated medially with the glenoid displaced inferiorly. The periscapular muscles may be hypoplastic or absent, causing scapular winging. The periscapular muscles may be hypoplastic or absent, causing scapular winging. Right = left Right = left bilateral involvement = 10% to 30% bilateral involvement = 10% to 30%

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37 5/7/ Assossiated anomalies Scoliosis Klippel-feil syndrom Rib cage CDHHandFootRenal

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41 5/7/201541polydactyly

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47 5/7/ syndactyly

48 5/7/ Apart & Poland syndromes

49 5/7/ RADIAL HEAD DISLOCATION

50 5/7/ Transvers failure of formation

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52 5/7/ Longitudinal failure of formation Radial club hand Ulnar club hand

53 5/7/ Radial club hand

54 5/7/ Radius deficiency 1:30,000 nearly always associated with thumb and carpal deficiencies and frequently associated with other upper extremity anomalies, anomalies of other organ systems, and syndromes VACTERL association (not inheritable; it may be accompanied by Vertebral, Anal, Cardiac, Tracheo- Esophageal, Renal or Radial, and Lung anomalies) Holt-Oram syndrome (autosomal dominant inheritance of cardiac septal defects associated with upper limb anomalies) TAR syndrome (autosomal dominant or recessive inheritance of completely absent radius with a near- normal thumb and thrombocytopenia).

55 5/7/ Radius deficiency is usually bilateral, although the two sides are frequently asymmetric; when the condition is unilateral, it is more common on the right. The radial wrist extensors and extrinsic thumb motors are usually absent or aberrant. The radial nerve is usually absent below the elbow, and the median nerve is always present and often the most prominent structure on the radial side of the wrist. The radial artery is usually absent.

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59 5/7/ treatment Children with type 0, 1, or mild type 2 radial deficiency usually require stretching and splinting surgical treatment.

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62 5/7/ Thumb Hypoplasia

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64 5/7/ BONE AND JOINT CONGENITAL DISORDERS SPRENGEL ” S DEFORMITY FROZEN SHOULDER CARPAL TUNNEL SYNDROME TENNIS ELBOW SYNDACTYLYPOLYDACTYLY

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66 5/7/ cts At the level of the wrist, the median nerve lies within the confined space of the carpal tunnel along with the long flexor tendons to the fingers and thumb. The tunnel is bounded on three sides by the bones of the carpus, which make up the floor of the canal, and on the fourth by the transverse carpal ligament (TCL), which forms the roof. Compressive neuropathy of the median nerve within the carpal tunnel may result from any space-occupying lesion under the TCL. A frequent cause is flexor tenosynovitis; other causes are fractures and dislocations of the floor of the canal and distal radius, and other space-occupying lesions such as tumors and ganglia. These space-occupying lesions increase the volume of the contents of the noncompliant carpal tunnel, raising the pressure on its contents, which include the median nerve. In many cases, there are no particular identifiable causes even though the nerve is clearly compressed. Although many of these cases are attributed to “ nonspecific synovitis, ”

67 5/7/ Compressive neuropathy of the median nerve within the carpal tunnel may result from any space-occupying lesion under the TCL. A frequent cause is flexor tenosynovitis; other causes are fractures and dislocations of the floor of the canal and distal radius, and other space-occupying lesions such as tumors and ganglia. These space-occupying lesions increase the volume of the contents of the noncompliant carpal tunnel, raising the pressure on its contents, which include the median nerve. In many cases, there are no particular identifiable causes even though the nerve is clearly compressed. Although many of these cases are attributed to “ nonspecific synovitis, ”

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69 5/7/ Complain = aching or burning pain along the median nerve distribution and of numbness and tingling in the median-nerve-innervated digits during night and early morning as well as during activities. (Numbness may extend into the ulnar digits in some patients.) These symptoms are aggravated by elevation, repetitive activities, and prolonged flexion positioning of the wrist. Radiation of symptoms proximal to the wrist is not unusual. Complaints of the hand feeling fat, clumsiness in manipulation, and dropping items are also frequent. The incidence is greater in women than in men, although the difference is decreasing. In the past, postmenopausal women were the most common patients; commonly associated diagnoses were rheumatoid arthritis and distal radius malunion. Recently, a large, younger group of patients with essentially equal distribution of women and men has emerged. In this group the carpal tunnel disease has been labeled idiopathic.

70 5/7/ Examination includes sensory, provocative, sudomotor, and strength testing

71 5/7/ the most consistent and reliable way to evaluate sensibility in nerve compression is to use threshold testing (Semmes– Weinstein monofilaments, vibrometry, and 256 cps vibration testing).

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73 5/7/ Provocative tests compress or percuss nerve to elicit the median numbness and paresthesias in the distribution of… Provocative tests compress or percuss nerve to elicit the median numbness and paresthesias in the distribution of…

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75 5/7/ The mild group consists of patients with intermittent symptoms that have been present less than 1 year, who have normal two-point discrimination, no thenar weakness or atrophy, no denervation potentials on EMG, and mildly elevated NCV. With conservative treatment and steroid injection, 40% will be free of symptoms at 12 months.

76 5/7/ The severe group consists of those with profound, persistent symptoms that have been present longer than 1 year, thenar weakness or atrophy, and marked abnormalities on electrodiagnostic studies. Patients in the severe group fail to respond adequately to conservative therapy and should receive operative treatment, which may include tendon transfers concurrent with carpal tunnel release.

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79 5/7/ In the moderate group, conservative treatment shows findings and gives results intermediate between those of the mild and severe groups. The presence of underlying disorders or advanced age in any of these patients diminishes the response to conservative care.

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89 De Quervain syndrome washerwoman's sprain, radial styloid tenosynovitis, de Quervain disease, de Quervain's tenosynovitis, de Quervain's stenosing tenosynovitis, mother's wrist, or mommy thumb) 5/7/201589

90 history Swiss surgeon Fritz de Quervain Swiss surgeon Fritz de Quervain SwisssurgeonFritz de Quervain SwisssurgeonFritz de Quervain1895 de Quervain's thyroiditis de Quervain's thyroiditis 5/7/201590

91 Pathology. extensor pollicis brevis extensor pollicis brevis abductor pollicis longus abductor pollicis longus 5/7/201591

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93 Cause The cause of de Quervain's disease is not known. idiopathicidiopathic. idiopathic overuse injuries overuse injuries 5/7/201593

94 Symptoms Symptoms are pain, tenderness, and swelling over the thumb side of the wrist, and difficulty gripping Finkelstein's test Finkelstein's test 5/7/201594

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97 OsteoarthritisOsteoarthritis of the first carpo-metacarpal joint Osteoarthritis Intersection syndromeIntersection syndrome—pain will be more towards the middle of the back of the forearm and about 2–3 inches below the wrist Intersection syndrome Wartenberg's syndrome Wartenberg's syndrome 5/7/201597

98 treatment 5/7/201598

99 Kienbock's disease It is named for Dr. Robert Kienböck, a radiologist in Vienna, Austria who described osteomalacia of the lunate in Robert KienböckradiologistVienna, AustriaosteomalaciaRobert KienböckradiologistVienna, Austriaosteomalacia 5/7/201599

100 Kienbock's disease fewer than 200,000 people in the U.S. population. 5/7/

101 It is breakdown of the lunate bone lunate bonelunate bone is another name for avascular necrosis [ is another name for avascular necrosis [avascular necrosisavascular necrosis (death and fracture of bone tissue due to interruption of blood supply) with fragmentation and collapse of the lunate. 5/7/

102 Predisposing factors a number of factors predisposing a person to Kienbock's. a number of factors predisposing a person to Kienbock's.Trauma Ulnar variance about one-third of sufferers report the condition in their non-dominant hand. 5/7/

103 Classification Type I Type I Normal radiograph (possible lunate fracture). Type II Type II Sclerosis of the lunate without collapse. Type III Type III Lunate is completely dead. Type IV Type IV Changes up to and including fragmentation, with superimposed arthritic change. 5/7/

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105 Treatment Revascularization Radial shortening Proximal Row Carpectomy Titanium or silicon implant Total wrist fusion 5/7/

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