Congenital deformities

CONGENITAL DEFORMITIESPresenters: Dr.A.Prakash, Dr. C. Arun Prasath.Institution: Annamalai University. Date:12.2.2013.

CONGENITAL TALIPES EQUINO VARUS(CTEV)

CTEV

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

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.

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.

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.

CTEV-CLINICAL FEATURES

CTEV-DIAGNOSIS

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

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.

CTEV-MAINTANANCE

Ctev splints Denis-Brown splint CTEV shoes

SPINA BIFIDA

SPINA BIFIDA

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

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

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

SPINA BIFIDA-CLINICAL FEATURES

SPINA BIFIDA-CLINICAL FEATURES

SPINA BIFIDA- DIAGNOSIS

Xray CT MRI

SPINA BIFIDA-TREATMENT

Wound care Poper closure of defect Hydrocephalous- ventriculo-peritoneal

shunt, if necessary for 5 to 6 years. Physiotherapy and splinting

CONGENITAL DISLOCATION OF HIP (CDH)

CDH

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

CDH-AETIOLOGY

Not well understood Hereditary predisposed to joint laxity Hormone induced joint laxity Breech presentation Hereditary faulty development of

acetabulum.

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

CDH-CLINICAL FEATURES

At birth-from pediatrician Early child hood-asymmetry of groin

creases, limitation of hip movements, click

Older children-peculiar gait.

CDH-CLINICAL FEATURES

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

CDH-DIAGNOSIS

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

SPRENGEL’S SHOULDERCONGENITAL ELEVATION OF SCAPULA

SPRENGEL’S SHOULDER

Failure of descent of the scapula, which is developmentally a cervial appendage.

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.

SPRENGEL’S SHOULDER-CLINICAL FEATURES

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.

MADELUNG DEFORMITYRADIAL CLUB HAND

MADELUNG DEFORMITY

Defective growth of the distal radial epiphysis resulting in a deformity due to a comparative overgrowth of the ulna.

Traumatic and congenital

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

MADELUNG DEFORMITY-CLINICAL FEATURES

MADELUNG DEFORMITY-TREATMENT

Exision of the lower end of ulna Exision of the damaged physis

RADIO ULNAR SYNOSTOSIS

RADIO ULNAR SYNOSTOSIS

Synostosis-abnormal fusion of bones

RADIO ULNAR SYNOSTOSIS-CLINICAL FEATURES

Postero lateral dislocation of radial head

Complete loss of supination and pronation

RADIO ULNAR SYNOSTOSIS-TREATMENT Exision of the bony bridge-

unsuccessful. Change the resting position of the limb

to supination by tendon transfers.

CONGENITAL PSEUDARTHROSIS OF TIBIA

PSEUDARTHROSIS OF TIBIA

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

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

PSEUDARTHROSIS OF TIBIA-PRESENTATION

PSEUDARTHROSIS OF TIBIA-PRESENTATION

PSEUDARTHROSIS OF TIBIA-PRESENTATION

PSEUDARTHROSIS OF TIBIA-TREATMENT Simple immobilisation-fails ORIF with bone grafting-succeeds

occasionally Excision of the affected segment and

slowly closing the gap.