Paediatric Orthopaedics😊

NOTE: Different Fanconis

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Fanconi disease/syndrome
• Proximal tubular reabsorption problem → Type 2 RTA
• Glycosuria, aminoaciduria
Fanconi anemia
(Not syndrome)
• Pancytopenia + radial ray
Fanconi Bickel syndrome
• Mutation in GLUT-2  
•
Bickel → Bi → 2 (GLUT 2)

Defect in glucose sensing → ↓ insulin release
• Postprandial Hyperglycemia.
• Fasting Hypoglycemia
• Glycogen accumulation disorder
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VACTERAL
Holt - Oram (ASD + Radial Ray)
TAR (thrombocytopenia + absent radius)
Congenital torticollis → Cock robin position
VACTERAL
Holt - Oram
(ASD + Radial Ray)
TAR (thrombocytopenia + absent radius)
Congenital torticollis →
Cock robin position
Stranger things characters
  • Dustin (Cleido cranial dysplasia)
  • Robin (Cock robin position)
  • Ray (Radial Ray) Hopper (Holt Oram ASD)

Paediatric Orthopaedics

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NOTE:

  • NAI/Child abuse
    • Bucket handle # → Metaphyseal corner #
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  • M/c # in children
    • Distal forearm (radius) bones
  • M/c # during birth
    • Clavicle #
  • Most common elbow fracture:
    • Supracondylar humerus fracture

Characteristics of Paediatric Bones

  • More Water Content:
    • Flexible and resilient to stress.
    • Bends rather than breaks.
  • Thicker Periosteum: Offers greater stability.

Effects of Applying Force to the Bone

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  • Force within physiological limits:
    • Outer surface is convex.
    • Causes distractive/tension force.
  • Force beyond physiological limits:
    • Outer cortex breaks.
    • Results in a Greenstick/Unicortical fracture

Common Paediatric Fractures

  • Most Common Fracture (m/c) in Children:
    • Greenstick fracture.
    • Forearm: Radius > Ulna (more common in radius).
  • Most Common Fracture (m/c) at Birth:
    • Clavicle fracture.
  • Most Common Fracture (m/c) in Child after Fall on Outstretched Hand (FOOSH):
    • Supracondylar humerus fracture.
  • Note: Most common fracture (m/c) in humans overall is a Clavicle fracture.

Osteochondrosis

  • Due to
    • Idiopathic.
      • Most Common Cause (m/c)
    • primary vascular event
    • repetitive trauma.
  • Self-limiting abnormality of bone growth.
  • Affects ossification centers in the epiphysis.
  • Begins in childhood as a necrotic condition.

Specific Osteochondrosis and Affected Bones:

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Disease
Affected Bone/Region
Key Point
Perthes disease
Femoral epiphysis
Most common site
Kohler’s disease
Navicular bone
Kohli de naavu
Keinbock’s disease
Lunate bone
Keen Lunatic
Panner’s disease
Capitulum
Causes elbow pain

Panni → Captain
Osgood-Schlatter’s disease
Tibial tuberosity
Good → shattered → Tibia good bone
Sever’s disease
Calcaneal epiphysis
Severe → calcaneum
Frieberg
2nd Metatarsal
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Avascular necrosis of Lunate → Keinbock
Avascular necrosis of Lunate → Keinbock

Perthes Disease (AKA Coxa plana/Legg-Calve-Perthes disease)

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Bone necrosis f/b remodelling
Bone necrosis f/b remodelling
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  • Etiology: Idiopathic, spontaneous osteonecrosis.
  • Age: 4–9 years
  • Sex: Boys > girls
  • Site: Femoral epiphysis.
  • Risk Factors
    • Low birth weight
    • Passive smoking
    • Positive family history
    • Low socioeconomic status
    • ADHD
  • A/w
    • Thrombophilia, Protein C & S deficiency
  • Pathogenesis
    • Disruption of blood supply to femoral head
    • Involved vessel: Lateral epiphyseal artery
    • Leads to:
      • Osteonecrosis
      • Cessation of bone growth
    • Healing phase:
      • Neovascularization
      • Removal of necrotic bone
      • Loss of bone mass
      • Weakening of femoral head
  • Clinical Features:
    • Pain at the hip.
    • Limping gait after activity.
  • Management:
    • < 8 years
      • Rest allows vascularity to recover → improve symptoms
      • Bisphosphonates
      • Surgery: Proximal femur varus osteotomy
    • > 8 years
      • Pelvic osteotomy

Miscellaneous Paediatric Orthopaedic Pathologies

Klippel Feil Syndrome

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  • Definition: 
    • Segmentation defect of cervical spine.
    • Fused C Spine
  • Clinical Features (Triad):
      1. Short webbed neck.
      1. Low set hairline.
      1. Restriction of neck movement.
  • Associations:
    • Scoliosis (most common).
    • Kyphosis
    • Cervical rib
    • Sprengel shoulder.
    • Genito-urinary anomalies.
    • Ocular/auditory/cardiac defects.
    • Diastematomyelia
      • congenitally split spinal cord

Sprengel's Deformity

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  • Congenital undescended hypoplastic scapula.
  • Impact: Limits shoulder mobility.
  • Associations: 
    • M/c associated with Klippel Feil syndrome.

Slipped Capital Femoral Epiphysis (SCFE)

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  • AKA: Sub-capital neck of femur fracture.
    • A split fracture at the growth plate of the capital femoral epiphysis.
  • Type: Classified as Type I Salter Harris fracture.
  • Mechanism: 
    • Femoral head remains within the acetabulum
      • Neck → slips relative to the head.
  • Left hip >Right hip

Causes of SCFE

  • Most Common: 
    • Idiopathic.
  • Associations with Endocrinopathies:
    • Hypothyroidism (most common).
    • Hypogonadism.
    • Increased growth hormone.

Clinical Features of SCFE

  • Usual Age: Around puberty.
    • Males: 13 to 17 years.
    • Females: 11 to 14 years.
  • Associated with Hypogonadism:
    • High-pitched voice in boys.
    • Gynecomastia in boys.
  • Coxa Vara Presentation:
    • Gradual/sudden pain at the hip.
    • Restricted abduction and internal rotation (earliest sign)
    • Decreased flexion
    • The affected limb is externally rotated.
    • Axis deviation test:
      • Knee-axilla sign positive
      • Knee points to ipsilateral shoulder on hip flexion
      • normally points towards contralateral shoulder
    • Trendelenburg gait.
    • Extension is not much affected
  • Observation: 
    • Externally rotated toe.
  • Risk Factors:
    • Males > females.
    • Increased weight for age in children.

Investigation of SCFE

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  • Investigation of Choice (IOC): 
    • MRI.
  • X-ray Findings:
    • Trethowan’s sign: 
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      • Klein’s line does not intersect with the head of the femur.
    • Klein’s line: 
      • Line drawn at the lateral aspect of the neck of femur
      • Mnemonic: Cleaner Steffi → runaway with Trethowan
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Developmental Dysplasia of the Hip (DDH)

  • Definition: 
    • Idiopathic spontaneous subluxation of the femoral head.

Epidemiology of DDH

  • Female child
  • First-born
  • Faulty intrauterine position
    • Frank breech with extended knees.
  • Family history.
  • Caucasians > Asians.
  • Incidence: 1 in 1000 live births.

Etiology of DDH

  • Most Common: Idiopathic.

Pathology of DDH

  • Developmental anomaly leading to lack of concavity of the acetabulum
    • flat/shallow/concave
  • Result:
    • Femoral head slips out.

Clinical Features of DDH

  1. Short limb: 
      • Asymmetrical thigh
      • gluteal folds.
  1. Vascular sign of Narath: 
    1. Femoral pulsations absent on the affected side
        • due to displaced femoral head,
        • palpation is not possible

Clinical Diagnosis of DDH

Child < 3 months old:

  • Barlow’s test: 
    • Performed to dislocate a dislocatable hip.
    • Mnemonic: Bahar valikkunath
    • BADD (Barlow → adduct)
  • Ortolani’s test: 
    • Performed to reduce a dislocated hip.
    • Mnemonic: ottayil kuthi keettunath
    • Ortolani → abduct
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Child > 3 months old:

  • Allis/Galeazzi sign: 
    • One knee is higher than the other on knee flexion,
    • → leg length discrepancy.
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  • Trendelenburg gait/Waddling gait: 
    • Abnormal gait due to gluteal muscle weakness or hip instability.

NOTE

  • Bony ankylosis → TB Spine
  • Fibrous ankylosis → TB knee/hip

Triangles

  • Babcock Triangle → TB hip
  • Fairbank triangle → Congenital coxa vara (Trendelenberg gait/Waddling gait)
  • Ward triangle → Osteoporosis

Phemister’s triad: → TB HIP

  • Periarticular osteopenia
  • Erosions
  • Reduced joint space

Investigations for DDH

  • Screening IOC: 
    • < 6 months
      • Ultrasonography (USG).
      • Graf classfication → vowels dont stick together
        • Alpha angle: ↓↓
        • Beta angle: ↑↑
        • Ultrasonography
          • To screen for DDH (developmental dysplasia of hip)
          • To look for joint effusion
    • > 6 months
      • X-ray.
  • Confirmation Test: 
    • X-ray.
    • MRI is an additional investigation if needed.

X-ray Lines for Diagnosis:

  • Hilgenreiner’s line (H): 
    • A line passing through the centers of both triradiate cartilages.
    • Epiphysis N in Inferomedial quadrant
    • If elsewhere → abn
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  • Perkins line (P): 
    • A line perpendicular to Hilgenreiner’s line,
    • passing through the lateral edge of the acetabulum.
      • Normal: 
        • Head of femur lies in the inner, lower quadrant.
      • DDH: 
        • Head of femur displaces to the upper, outer quadrant.

Treatment for DDH

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Age Group
Management
0–6 months
Pavlik harness,
von Rosen splint
6–18 months
Spica

Reduction
(open/closed) via
Smith-Peterson approach
18–36 months
Femoral osteotomy
> 3 years
VDOR + Pelvic osteotomy
(Salter, Pemberton)
> 10 years
Total hip replacement after skeletal maturity

Bilateral Knee Deformity

Cause
B/L Genu Varum
(Bow Legs)
B/L Genu Valgus
(Knock-Knees)
Wind Swept Deformity
m/c in children
Rickets > Idiopathic
Idiopathic > Rickets
Rickets
m/c in adults
Osteoarthritis > Rheumatoid arthritis
Rheumatoid arthritis > Osteoarthritis
Rheumatoid arthritis
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Mnemonic: Kaatadichapo kunjnugal rocket (ricket children) pole poi, Adult Room adachitt (RA)

Congenital Talipes Equinus Varus (CTEV)

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  • Most Common (m/c) Anomaly of Foot: 
    • Clubfoot.

Epidemiology of CTEV

  • 50% cases are bilateral.
  • Males > Females.
  • Common in first-born children.
  • Associated with breech presentation and oligohydramnios
    • (not typically twin pregnancy).

Causes of CTEV

  • Most Common: Idiopathic.
  • Spina bifida.
  • Arthrogryposis multiplex congenita.
  • Polio doesn't cause

Pathology of CTEV

  • C A V E Mnemonic (referring to the deformities):
    • Cavus: Exaggeration of medial longitudinal arch.
    • Adduction: At talonavicular/mid tarsal joint.
    • Varus: At talocalcaneal/subtalar joint.
    • Equinus: At ankle joint (plantar flexion).
  • Internal rotation of tibia (a component but not part of CAVE).

Investigations for CTEV

  • X-ray: Kite’s angle (Talocalcaneal angle).
    • notion image
    • Normal: 20 to 40 degrees.
    • Clubfoot: Decreased angle.
  • Screening: 
    • Dorsiflexion test:
      • If dorsiflexion causes the tip of the toe to touch the shin,
      • it indicates good flexibility and is normal
      • If doesnt touch skin → CTEV

Treatment for CTEV

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Topic
Key Facts
• Most accepted method
•
gold standard worldwide
• >95% success if started early
Ponseti method
Age to start Ponseti
As early as possible – ideally within first week of life
Sequence of correction
C-A-V-E
1. Cavus
2.
Adductus
3.
Varus
4.
Equinus (last)
Key manipulation
Abduction of forefoot +
counter-pressure on lateral head of talus

Fulcrum of correction: Talar head

First cast → corrects cavus
• by supinating forefoot
Number of casts
Usually 6 weekly casts
Degree of abduction
60–70° (critical to prevent relapse)
Percutaneous tenotomy
Done in 90–95% cases when only equinus remains
under LA → OPD → at ~6–10 weeks
Bracing (most important!)
Steenbeek Foot Abduction Brace (FAB)
- Full time (23 h/day) for 3 months
- Then
night + nap time till 4–5 years

Relapse rate without brace >80%
Surgery indications
- Late presentation (>2–3 yrs)
-
Resistant/recurrent after proper Ponseti
-
Syndromic CTEV
Historical operation
PMSTR (Postero-Medial Soft Tissue Release)
Worst complication of PMSTR
Overcorrection → valgus flat foot
Best prognostic factor
Early start + parental compliance with bracing
Dobb’s method
New dynamic method (ongoing trials), not standard yet

Initial: 

  • Manipulation of foot to correct deformity,
  • followed by POP cast
    • serial casting for 8-9 weeks

Ponseti Method:

  • Fulcrum of correction: Talar head
  • Order of Manipulation (CAVE Mnemonic):
    • Cavus.
    • Adduction.
    • Varus.
    • Equinus.
  • Method: 
    • Start as early as possible
    • Apply POP cast (above knee) for 21 weeks
    • then remove and manipulate again.
Status
Age group
Management
Deformity corrected
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Till 1 year
• Dennis Brown splint
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1 – 5 years
• CTEV shoes (day)
• Dennis Brown splint (night)
Not corrected / untreated
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1 – 3 years
• Posteromedial soft tissue release (PMSTR)
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3 – 5 years
• Dillwyn Evans procedure
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5 – 8 years
• Dwyer osteotomy
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8 – 10 years
• Wedge tarsectomy
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> 10 years
• Triple arthrodesis, External fixators

Maintenance:

Before child starts walking (<1 years): 

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  • Dennis Brown splint (used at night/rest periods).
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    • Till the baby learns to walk,
    • Mechanism : As baby moves the leg, deformity gets corrected,

Once child starts walking (>1 year):

  • Day: CTEV shoe.
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  • Night: Dennis Brown splint.

CTEV Shoes (for correcting residual deformities):

  • Straight inner border: To correct adduction.
  • Outer raise: To correct inversion & varus.
  • Absence of heel: To correct equinus.
  • Should be worn at least 3 hrs/day

Triple Arthrodesis:

  • Indication: Presentation at >10 years of age.
  • Procedure: Joint fusion surgery.
  • Most important joint: Talonavicular joint.

Joshi’s External Stabilization System (JESS):

  • Used for CTEV resistant to treatment.

Achondroplasia

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  • Most Common Cause (m/c) of: 
    • Disproportionate dwarfism.
  • Cause: 
    • FGFR3 gene mutation
    • Chromosome 4 (Autosomal dominant or sporadic).
  • Pathology:
    • Endochondral ossification defect
      • affects growth plate development
    • Intramembranous ossification is normal
      • skull and clavicle formation are unaffected by this process

Clinical Features of Achondroplasia

Feature
Description
Champagne Glass Pelvis
Characteristic X-ray appearance
Proximal limb shortening
Rhizomelic shortening – upper arm/thigh disproportionately short
Brachydactyly
Short, stubby fingers
Frontal Bossing
Prominent forehead
Starfish Hand
All fingers appear of same length due to short middle phalanx
Trident Hand
Wide gap between middle and ring fingers
Bullet Nose Vertebrae
X-ray:
Bullet-shaped vertebral bodies
due to
decreased height
Saddle Nose
Flattened nasal bridge
Trunk
Normal in length
Intelligence & Sexual Development
Normal IQ and sexual development

Note: Differential Diagnosis for Limping Child (DDx)

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  • Chevron’s sign
    • Femur length = Tibia length
    • Dont confuse with scurvy/rickets
    • Rhizomelic dwarfism
      • Rhizomelic shortening of femur
      • Metaphyseal dyplasia
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Other signs

  • Trident/ Starfish hand:
    • Increased gap between middle and ring fingers.
  • Champagne glass pelvis
  • Tombstone appearance of iliac blades
  • Flat acetabulum
  • Mnemonic: Achondroplasia → Tyrion lannister → carries a trident → drinks champagne → acetabulum got flat due to repeated sex → died in tombstone