Congenital deformities

1. Congenital deformities Presenters: Dr.A.Prakash , Dr. C. Arun Prasath . Institution: Annamalai University . Date:12.2.2013.

2. Congenital talipes equino varus ( ctev )

3. ctev Vague term used to define a number of abnormalities in the foot.

4. Ctev -etiology The true etiology of congenital clubfoot is unknown. Idiopathic Mechanical-intra uterine pressure Ischeamia of calf muscles genetic Secondary Paralytic disorders Arthrogryposis multiplex congenita .

5. Ctev-patho anatomy Bones Smaller Talus faces downwards Calcaneum small Joints Ankle- equinus Subtalar -inversion Mid tarsal-Forefoot adduction and cavus Muscles and tendons Posteriorly - tendo achilles . Medially- TP, FDL, FHL Capsules and ligaments Capsules of ankle and sub talar joint, Medially- Talo navicular ligament, spring ligament, deltoid ligament Plantar fascia and ligaments Interosseous ligaments.

6. Ctev -clinical features Detected at birth Brought during early infancy Brought during late infancy and early childhood Brought during late childhood Examination Normally-foot dorsiflexed to touch the chin of tibia Foot is smaller, in equinus , varus and adduction. Heel is small Deep skin creases on the back and medial side of foot Bony prominences and callosities on the lateral side of foot Outer side of the foot is convex.

7. Ctev -clinical features

8. Ctev -diagnosis Xrays - reduced talo calcaneal angle. Normal-more than 35 degrees.

9. Ctev -treatment Non-operative Manipulation Manipulation and corrective plaster Operative Postero -medial soft tissue release(TURCO’S)-<3 years Limited soft tissue release Tendon transfers Dwyer’s osteotomy Dilwyn -Ewan’s procedure- 4 to 8 years Wedge tarsectomy - 8 to 11 years Triple arthrodesis - >11 years Illizarov’s technique- recurrent.

10. Ctev-maintanance Ctev splints Denis-Brown splint CTEV shoes

11. Spina bifida

12. Spina Bifida A congenital disorder in which the two halves of the posterior vertebral arch fail to fuse at one or more levels.

13. Spina Bifida-types Occulta Mildest form Midline defect near the lamina Cystica More severe form Contents of the canal prolapse Meningocele Myelomeningocele -open, closed. hydrocephalus

14. Spina Bifida-clinical features Spina bifida occulta Usually nothing Midline dimple, tuft of hair, pigmented navus Neurological symptoms-enuresis, incontinence, weakness in the lower limbs Spina bifida cystica Saccular lesion in the lumbar region Hydrocephalous Equinovarus or calcaneo valgus of feet Recurvatum of knee Hip dislocation LMN type paralysis Loss of sphincter control

15. Spina Bifida-clinical features

16. Spina Bifida-clinical features

17. Spina Bifida- diagnosis Xray CT MRI

18. Spina Bifida-treatment Wound care Poper closure of defect Hydrocephalous- ventriculo -peritoneal shunt, if necessary for 5 to 6 years. Physiotherapy and splinting

19. Congenital dislocation of hip (CDH)

20. CDh Spontaneous disloation of the hip occuring before, during or shortly after birth.

21. CDh-aetiology Not well understood Hereditary predisposed to joint laxity Hormone induced joint laxity Breech presentation Hereditary faulty development of acetabulum .

22. CDh -pathology Femoral head is dislocated upwards- small capital epiphysis Femoral neck- anteverted Acetabulum -shallow Ligamentum teres -hypertrophied. Fibrocartilaginous labrum- folded limbus . Streched capsule Muscles-shortened

23. CDh -clinical features At birth-from pediatrician Early child hood-asymmetry of groin creases, limitation of hip movements, click Older children-peculiar gait.

24. CDh -clinical features

25. CDh -diagnosis Barlow’s test Ortolani’s test Galeazzi’s sign Telescopy test Trendelenburg’s test Xray -shallow acetabulum , break in shenton’s line, small head Ultrasound

26. CDh -diagnosis

27. CDh -treatment Aim-reduce by closed means. Methods of reduction Closed manipulation Traction followed by closed manipulation Open reduction Maintenance of reduction Plaster cast Splints-Von Rosen’s splint Acetabular procedures Salter’s osteotomy Chiari’s pelvic displacement osteotomy Pemberton’s pericapsular osteotomy

28. Sprengel’s shoulder congenital elevation of scapula

29. Sprengel’s shoulder Failure of descent of the scapula, which is developmentally a cervial appendage.

30. Sprengel’s shoulder-clinical features May be noticed at birth Shoulder on the affected side is elevated Smaller shoulder Occasionally both sides are affected( klippel feil syndrome) Shorter neck-associated with kyphosis or scoliosis Abduction and elevation restricted Xray -scapula may be elevated, associated vertebral anomalies.

31. Sprengel’s shoulder-clinical features

32. Sprengel’s shoulder-treatment Mild- left alone Children younger than 6 years-scapula an be repositioned by release of the muscles, exision of the supraspinous portion of the scapula.

33. Madelung deformity Radial club hand

34. Madelung deformity Defective growth of the distal radial epiphysis resulting in a deformity due to a comparative overgrowth of the ulna. Traumatic and congenital

35. Madelung deformity-clinical features Lower end of radius curves forwards and ventrally Wrist is dislocated Lower end of ulna projects out May be isolated or a part of generalised dysplasia Deformity increases until growth ceases

36. Madelung deformity-clinical features

37. Madelung deformity-treatment Exision of the lower end of ulna Exision of the damaged physis

38. Radio ulnar synostosis

39. Radio ulnar synostosis Synostosis -abnormal fusion of bones

40. Radio ulnar synostosis -clinical features Postero lateral dislocation of radial head Complete loss of supination and pronation

41. Radio ulnar synostosis -treatment Exision of the bony bridge- unsuccessful. Change the resting position of the limb to supination by tendon transfers.

42. Congenital pseudarthrosis of tibia

43. Pseudarthrosis of tibia A birth defect in the lower end of tibia in children, where a fracture fails to unite.

44. Pseudarthrosis of tibia-presentation Usually diagnosed in early infany Child may be born with a fractured tibia or attenuated later in life. Leg is bowed anteriorly Xray Gap, marked thinning , sometimes fibula is also afffected

45. Pseudarthrosis of tibia-presentation

46. Pseudarthrosis of tibia-presentation

47. Pseudarthrosis of tibia-presentation

48. Pseudarthrosis of tibia-treatment Simple immobilisation -fails ORIF with bone grafting-succeeds occasionally Excision of the affected segment and slowly closing the gap.