The Paediatric Upper Limb

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The Paediatric Upper Limb

• Manoj Ramachandran
• Consultant Orthopaedic Surgeon
• Barts and The London NHS Trust

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Objectives

• Embryology
• Sprengels anomaly
• NBP
• Pseudarthrosis of the clavicle
• Radial head dislocation
• Congenital radioulnar synostosis
• Madelungs deformity

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Normal limb development

• Upper limb bud 4 weeks
• Lower limb bud few days later
• Mesoderm covered by thin surface of ectoderm
  limb bud

Zone of polarizing activity (ZPA) directs apical
ectodermal ridge (AER) ZPA activity mediated by
sonic hedgehog (Shh) genes and AER by HOX genes
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Normal limb development

• Growth from proximal to distal in limb bud
• Joint formation by apoptosis
• Visceral formation at similar time
• Complete differentiation by 7 to 8 weeks

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Basic management principles

• Improve function
• Utilize potential of developing brain
• Complete reconstruction if possible before school
  age
• Early surgery for progressive deformities
• Later surgery for procedures requiring
  cooperative rehabilitation

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Classification (IFSSH)

• Failure of formation
• Failure of differentiation (separation)
• Duplication
• Overgrowth
• Undergrowth
• Constriction ring syndrome
• Generalized abnormalities and syndromes

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Classification
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Sprengels anomaly

• Malposition and dyslasia of the scapula
• Scapula forms as part of the limb bud then
  descends to in between T2 and T7
• Associated with e.g.
• Klippel-Feil
• Cervical ribs
• Fused ribs
• Scoliosis

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Subclavian Artery Supply Disruption Sequence
Klippel-Feil
Sprengels
Polands anomaly Syndactyly Sternal head
pectoralis major
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Sprengels anomaly

• 3M1F
• LgtR
• Sporadic or AD
• Abduction limited, shoulder asymmetry, scapula
  higher and hypoplasic
• Chest and C-spine X-rays
• Omovertebral bar from superomedial scapula to
  vertebral spinous processes

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Sprengels anomaly

• Leave alone
• Physiotherapy
• Surgery for cosmesis or restricted function
• Scapular osteotomy and release of musculature
  from the scapula (Green) or their origin
  (Woodward)
• Vertical osteotomy (Wilkinson and Campbell)
• Resection of supraspinous portion
• Major risk - brachial plexus injury

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Neonatal brachial plexopathy

• 0.3-2.5 per 100o live births
• Risk factors
• Large birth weight
• Prolonged second stage of labour
• Shoulder dystocia
• Breech
• Forceps assisted delivery
• Prior NBP

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NBP

• Present with decreased movement in involved limb
• Consider clavicle fracture, humeral fracture,
  septic arthritis, monoplegia and arthrogryposis
• C5 shoulder, C6 elbow flexion, C7 elbow, wrist
  and finger extension, C8/T1 hand grip
• Moro reflex helpful
• 10 associated clavicle fractures

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NBP

• Upper (Erb)
• Most common
• Adducted, internally rotated shoulder, extended
  elbow, good hand function
• Whole
• Flail arm and hand, may have Horners
• Lower (C7-T1)
• Klumpke palsy

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NBP

• Gilbert and Tassin/Narakas classification
• Group 1 C5/6 involvement gt90 fully recover
• Group 2 C5/6/7 involvement 65 fully recover
• Group 3 C5/6/7/8/T1 involved lt50 fully recover
• Group 4 C 5/6/78 T1 involved
• Horner's syndrome None fully recover
• Elbow flexion by 12 weeks (Gilbert Waters)
• Error rate 12

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NBP

• Total plexus
• Horners syndrome
• Failure of return of function by 3 - 6 months
• Poor long term outcome

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NBP

• Physiotherapy
• ?Botox
• Early surgery
• 1-3 months - total Horners
• 4 months - no recovery / EPS
• Procedures
• Neurolysis
• Nerve graft
• Nerve transfer e.g. Oberlin ulnar nerve portion
  to musculocutaneous
• Reimplantation

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NBP

• Late surgery
• e.g. shoulder - internal rotation contracture,
  humeral retroversion and glenoid deficiency
• consider e.g. subscapularis release and
  rotational humeral osteotomies

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Pseudarthrosis of the clavicle

• Congenital failure of ossification of central
  clavicle
• Rare (200 cases)
• Nontender lump not limiting function
• Always on the right (except in dextrocardia)
• Related to subclavian artery vascular anlage
  crossing first rib just below
• Surgery for cosmesis and pain
• Compression plating and grafting

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Radial head dislocation

• Congenital
• Hypolastic capitellum and small dome-shaped
  radial head
• 1/3 have other upper limb anomalies e.g. CRUS
• 1/3 have other skeletal anomalies e.g. DDH, CTEV
• Minimal symptoms
• Surgery rarely indicated
• ?Open reduction and annular ligament
  reconstruction (before age 2)
• Excise radial head after skeletal maturity
• Acquired
• Trauma
• Secondary to abnormal ulna growth

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Congenital radioulnar synostosis
Rare (400 cases) Failure of longitudinal
segmentation during 7th week
Fixed pronation of forearm from neutral to severe
pronation If severe and/or bilateral, affects
activities of daily living Indications for
surgical intervention Definite - gt600
pronation Relative 150 600 pronation
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Radiographic classification
III osseous synostosis/head posteriorly
dislocated
IV osseous synostosis/head anteriorly dislocated
I fibrous synostosis
II osseous synostosis/head located
Cleary, Omer JBJSAm 198567539-45
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Surgery improve rotation
Radical resection with interposition and
stabilization with wires (e.g. Green and
Mital) Cylinder-shaped stainless steel swivel
prosthesis Free vascularized fascio-fat
graft Complicated by recurrence of ankylosis and
soft tissue compromise
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Surgery improve position
Aim for best position of function Ilizarov
method Rotational osteotomies distal to
synostosis
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Madelung deformity

• Anterior-ulnar distal radial bowing and dorsally
  prominent ulna
• Consider trauma, infection, Olliers and
  Leri-Weil dyschondrosteosis
• Aetiology unknown (?SHOX)
• Vickers and Nielsen ligament under PQ from
  ulnovolar radial metaphysis to lunate and TFCC

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Madelung deformity

• Bilateral true neutral PA radiographs
• Treatment
• Leave alone
• Epiphysiolysis
• Distal radial dome osteotomy and release of
  Vickers and Nielsens ligament

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Objectives

• Embryology
• Sprengels anomaly
• NBP
• Pseudarthrosis of the clavicle
• Radial head dislocation
• Congenital radioulnar synostosis
• Madelungs deformity

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Thank you!

• Manoj Ramachandran
• Consultant Orthopaedic Surgeon
• Barts and The London NHS Trust