CONGENITAL TALIPES EQUINOVARUS (CTEV)
Why are you saying that the deformity is congenital?
- Deformity since birth
- Bilateral presentation
- Male (usually).
What is the meaning of talipes?
What are the components of CTEV?
- Hindfoot-equinus
- Midfoot varus
- Forefoot adduction and supination
- Internal tibial torsion
How do you know equinus is present?
Hindfoot equinus confirmed by:
- Small highly placed heel, facing towards sky
- Equinus crease at heel
- Tight TA (tendo achilles tendon).
How do you confirm midfoot varus?
- Varus crease at medial border of foot.
- Small, less prominent medial malleoli and large more prominent lateral malleoli.
- Small, concave medial border and large convex lateral border.
How do you confirm forefoot adduction?
By medial deviation of all the toes.
What investigation do you suggest in CTEV child?
ANGLE MEASUREMENT
- Talocalcaneal angle:
- AP view: 35–40—normal
- Lateral view: 30–35—normalIn CTEV—0° or both axis are parallel to each other.
- Talo 1st metatarsal:
- Normal—AP (AP view)—15–20°
- CTEV—5°–10°
Lateral view—talotibial—normal 70–75° (in CTEV—decrease).
Tibiocalcaneal—normal—75–85° (CTEV—decrease).
- Photography (foot print in stamp pad)
- Clinical photography
How will you treat a case of CTEV?
Conservative:
- Manipulation + strapping (by bandage)—started immediately after birth.
- Denis Brown splint.
- Corrective POP cast above knee at 60° knee flexion.↓Continue till 3 months of age—80% gets corrected by this method.
Surgery: After 3 months, if not corrected by conservative method:
- Tenotomy.
- Soft-tissue release—PMSTR (posteromedial soft tissue release).
In PMSTR following structures are usually cut and released:
- Capsular release
- Ligament release:Spring (talocalcaneonavicular)Incision at plantar fascia.
- Tendon release:
- Tendoachilles (z-plasty)
- Tibialis posterior
- Flexor hallucis longus
- Flexor digitorum longus.
Master knot of Henry—tight tendinous fascia structure at the insertion of tibialis.
Posterior at the base of navicular.
If the deformity is not getting corrected by PMSTR—extensile release can be done.
Extensile release: PMSTR and release of following structures:
- Lateral capsule of ankle joint + calcaneocuboid joint capsule.
- Fibular collateral ligament.
If deformity more than 2 years (continue till 4 years):
- Calcaneal wedge osteotomy
- Dillwyn Evan's procedure
- Tarsectomy
- Metatarsectomy.
After 4 years: Extra-articular arthrodesis (Grice green arthrodesis)—continue till 10 years.
If deformity: > 10 years—Triple arthrodesis
Management of CTEV at any age:
- JESS fixator
- Ilizarov's external fixator (ring fixator).
CONGENITAL VERTICAL TALUS
How do you diagnose a case of congenital vertical talus?
Investigation:
Talocalcaneal angle measurement in X-rays.
Management by:
- Corrective foot brace
- Corrective surgery—osteotomy.
DEVELOPMENTAL DYSPLASIA HIP (DDH)
DDH at left hip.
What is the clinical features of DDH?
Clinical features: In child, dislocated femoral head on the post aspect of hip joint.
- Immobility of affected limb
- May be shortening present
- Muscle wasting of gluteal and quadriceps muscle
- Deformity of the affected limb (flexion, adduction)↓Confirmed by—Barlow's test and Ortolani's test.
How will you investigate a case of DDH?
Birth history
Clinical features:
Investigation proper:
- X-ray pelvis with both hip (AP view) and affected hip + thigh (lateral view).
- MRI of both hip.
- To differentiate any other clinical condition—CRP, routine blood test is required.
How will you do treatment?
Conservative—abduction pillow/brace (Milwalky/Pavlik harness brace)— up to the age of 6 months of child.
If no correction—operative management by corrective derotation osteotomy.
OSTEOGENESIS IMPERFECTA
What is the incidence?
It is one of the commonest heritable bone disorders (1 in 20000).
What are the commonest feature?
- Osteopenia
- Proneness to fracture
- Laxity of ligaments
- Blue sclera
- Crumbling teeth.
What is the clinical features?
Type I (Mild)
- Majority develop fractures a year or two later
- Deep blue sclera
- General joint laxity.
Type II:
- Severe lethal—still born infant
- Respiratory difficulties.
Type III:
- Diagnosed at birth or more than 6 years
- Kyphoscoliosis
- Triangular face, blue grey sclera
- Wormian bones in skull.
Type IV:
- Short stature
- Scoliosis
- Pale blue sclera
- Hearing affected.
How will you manage a case osteogenesis imperfecta?
- Splinting of fracture
- Open reduction and internal fixation with nail or plate
- Mobilization to prevent osteoporosis
- Multiple osteotomies of deformity correction.
SKELETAL DYSPLASIA
What are the different types of skeletal dysplasia?
- Diaphyseal aclasis
- Achondroplasia
- Hypochondroplasia
- Osteopetrosis
- Proximal focal femoral deficiency.
DIAPHYSEAL ACLASIS (HEREDITARY MULTIPLE EXOSTOSIS)
What are the clinical features of diaphyseal aclasis?
- Multiple bony lump over long bone, scapula, pelvis
- Discovering childhood
- Genu varum/valgum or ulnar deviation
- Lump may cause neurogenic symptoms by pressing on nerve.
What is the pathology of diaphyseal aclasis?
It is a congenital disorder with autosomal dominant. Basically there are accumulation of multiple solitary exostosis or osteochondroma.
What is the X-ray finding of diaphyseal aclasis?
Broadening of metaphysis with pedunculated or sessile exostosis.
How do you treat the diaphyseal aclasis?
- Excision of osteochondromatic mass, when they will put pressure on vessel or nerve.
ACHONDROPLASIA (DWARFISM)
What are the clinical features of achondroplasia?
It is autosomal dominant disorder:
- Large skull, prominent forehead.
- Shortening of all limbs (mostly proximal segment).
- Too long trunk.
- Prominent buttock with flexed hip, knee.
- Bow legs or flexion deformity at elbow.
What is the X-ray finding of achondroplasia?
Short tubular bone with wide metaphysic and small pelvis.
How do you treat the achondroplasia?
- Corrective osteotomy for genuvarum or valgum.
- Limb lengthening by ilizarov fixator.
- Excision of mass, when they will put pressure on vessel or nerve.
HYPOCHONDROPLASIA
Write short notes on hypochondroplasia?
- It is a autosomal dominant disorder and mild form of achondroplasia
- Shortness of stature and increased lumbar lordosis is the commonest features
- Other features of achondroplasia may be present
MULTIPLE EPIPHYSEAL DYSPLASIA
Write short note on multiple epiphyseal dysplasia?
Pathology: Autosomal dominant disorder due to abnormal development and ossification of epiphyses.
Clinical features: Newborn or child with multiple deformity:
- Growth retardation
- Joint pain
- Secondary osteoarthritis
- May be severe crippled.
Investigation: X-ray—irregular/abnormal epiphyseal line.
- Delayed appearance of epiphyses.
Treatment:
- Corrective osteotomy for genuvarum or valgum
- Reconstructive surgery for secondary OA
OSTEOPETROSIS (MARBLE BONE DISEASE)
Write short notes on osteopetrosis?
Pathology: Autosomal dominant disorder, benign lesion.
Clinical features: Patient is asymptomatic usually, diagnosis done after fracture occur.
Pathological fracture or nerve compression may occur.
X-ray: Increased density of all bones with wide cortex and narrow medullary canal, with sclerosis.
Treatment: Symptomatic.
PROXIMAL FOCAL FEMORAL DEFICIENCY
Write short notes on osteopetrosis?
In this case, proximal femur is absent or cartilaginous or shortened.
Clinical features: Shortened limb, Coxa vara.
X-ray: Large gap between acetabulum and upper third femur.
Treated by limb lengthening by ilizarov fixator.
CONGENITAL TIBIAL PSEUDARTHROSIS
Write short notes on congenital tibial pseudarthrosis?
- Anterior bowing of tibia in infant/child usually
- May or not affecting fibula
- X-ray: Gap in shaft of tibia, and looking like fracture nonunion
- Biopsy: Histopathology shows neurofibroma or fibrous dysplasia
- Treatment by bone grafting/ilizarov
- Prognosis may not be good, and may lead amputation.
SPINA BIFIDA
It is congenital disorder in which the two halves of the posterior vertebral arch have failed to fuse.12
Describe the pathology of spina bifida?
There are four types:
- Spina bifida cystica:
- Meningocele
- Myelomeningocele.Baby presents with paralysis, associated with hip dislocation, talipes, claw toes.
- Spina bifida occulta:
- A midline dimple in overlying skin
- Tuft of hair
- Splitting of spinal cord
- Pigmented nevus (diastematomyelia).
- Hydrocephalus:
- Distal tethering of cord
- Cause herniation of cerebellum
- Obstruction in CSF circulation
- Ventricles and skull enlarges.
- Neurological dysfunction
What investigation will you do for spina bifida?
- X-ray whole spine (AP/lateral view)
- Muscle charting
- MRI brain/spine
- NCV study.
What treatment will you offer to spina bifida?
- Skin closure at day 1
- Ventriculocaval shunt at 1st week
- Stretch and strap at 1st month
- Orthopedic surgery—according to need
- Urogenital surgery, if needed.