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Congenital Abnormalities/Deformities of the Lower Limb

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<strong>Congenital</strong><br />

<strong>Abnormalities</strong>/<strong>Deformities</strong> <strong>of</strong> <strong>the</strong><br />

<strong>Lower</strong> <strong>Limb</strong><br />

Gordon F Watt. FCPodMed<br />

Lecturer in Podopaediatrics<br />

Glasgow Caledonian University


Overview<br />

• Genetic Factors<br />

• Environmental Factors<br />

• Mixture <strong>of</strong> both<br />

• 1 in 50 born with severe malformation<br />

• More common in premature babies and possibly<br />

mo<strong>the</strong>rs with diabetes<br />

• Genetic counselling<br />

• Total correction may not be possible – residual<br />

deformity and handicap – ongoing<br />

podiatric/orthopaedic management


International Classification<br />

• Failure <strong>of</strong> formation <strong>of</strong> parts<br />

• Failure <strong>of</strong> differentiation <strong>of</strong> parts<br />

• Duplication<br />

• Undergrowth<br />

• Overgrowth<br />

• <strong>Congenital</strong> constriction band syndrome<br />

• Generalised skeletal abnormalities


Failure <strong>of</strong> Formation <strong>of</strong> Parts


Failure <strong>of</strong> Differentiation <strong>of</strong> Parts


Duplication


Undergrowth


Overgrowth


<strong>Congenital</strong> Constriction Band<br />

Syndrome (1)


Generalised Skeletal <strong>Abnormalities</strong><br />

• Achondroplasia<br />

• Turners Syndrome<br />

• Apert’s syndrome (Acrocephalosyndactyly,<br />

craniosynostosis)


Achondroplasia


Turner’s Syndrome


Apert’s syndrome<br />

(Acrocephalosyndactyly,<br />

craniosynostosis) (1)


Apert’s syndrome<br />

(Acrocephalosyndactyly,<br />

craniosynostosis) (2)


Apert’s syndrome<br />

(Acrocephalosyndactyly,<br />

craniosynostosis) (3)


Common <strong>Congenital</strong> Conditions (1)<br />

• Hammer toe<br />

• Mallet toe<br />

• Hallux – 3 phalanges<br />

• Fifth toe – 2 phalanges<br />

• <strong>Congenital</strong> curly or varus toe (curly toe<br />

syndrome)<br />

• Digiti quinti minimi varus (congenital<br />

overlying fifth toe)


Hammer Toe


Mallet Toe


Claw Toe


Curly Toe Syndrome


Management


Digiti Quinti Minimi Varus<br />

(<strong>Congenital</strong> Overlying Fifth Toe)


<strong>Congenital</strong> Curly or Varus Toe<br />

(Curly Toe Syndrome)


Common <strong>Congenital</strong> Conditions (2)<br />

• <strong>Congenital</strong> hallux varus<br />

• Primary<br />

• 2 nd type<br />

• 3 rd • 3 type rd type<br />

• <strong>Congenital</strong> Hallux Abducto Abducto-Valgus Valgus<br />

• Polydactyly<br />

• Pre-axial Pre axial<br />

• Post Post-axial axial<br />

• Central


<strong>Congenital</strong> Hallux Varus


<strong>Congenital</strong> Hallux Varus<br />

• Primary – No o<strong>the</strong>r associated congenital<br />

abnormalities. Supernummery digit on<br />

medial side <strong>of</strong> foot undergoes<br />

developmental arrest. Becomes tight<br />

fibrous or cartlaginous band and<br />

progressively pulls <strong>the</strong> toe to <strong>the</strong> midline.


<strong>Congenital</strong> Hallux Varus<br />

• 2nd Type – Associated with o<strong>the</strong>r deformities <strong>of</strong><br />

<strong>the</strong> forefoot. Hallux varus with primarily<br />

adductedfirst metatarsaland hallux varus with<br />

congenital marked shortening and broadening <strong>of</strong><br />

<strong>the</strong> first metatarsal.<br />

• 3 rd<br />

rd Type – Associated with developmental<br />

afflictions <strong>of</strong> <strong>the</strong> skeleton. e.g. diastrophic<br />

dwarfism.


<strong>Congenital</strong> Hallux Varus


<strong>Congenital</strong> Hallux Abducto Abducto-Valgus Valgus


Polydactyly<br />

• Pre-axial Pre axial – on side <strong>of</strong> hallux<br />

• Post Post-axial axial – on side <strong>of</strong> 5 th toe (most<br />

common)<br />

• Central – duplication <strong>of</strong> one <strong>of</strong> <strong>the</strong> middle<br />

toes


Polydactyly<br />

• Duplication may be <strong>of</strong> <strong>the</strong> distal phalanx, <strong>the</strong><br />

distal and middle phalanx or whole toe.<br />

• The metatarsal may be partially or totally<br />

duplicated.<br />

• Duplicated digits may share a common<br />

metatarsal.<br />

• May be accompanied by syndactyly and/or<br />

macrodactyly/microdactyly.<br />

• May occur alone or be associated with<br />

supernummery digits on <strong>the</strong> hands


Polydactyly


Polydactyly


Polydactyly With <strong>Congenital</strong><br />

Overlying 5 th Toe


Common <strong>Congenital</strong> Conditions (3)<br />

• Syndactyly<br />

• Oligodactyly<br />

• Divergent or Convergent toes<br />

• Macrodactyly<br />

• Microdactyly


Syndactyly<br />

• Usually 2/3 – may be partial or total.<br />

• May be associated with curly mallet or<br />

hammer toe.<br />

• May affecy any or all toes to a lesser or<br />

greater extent.<br />

• Treatment not usually required.<br />

Occasional exception being 1/2


Syndactyly


Syndactyly


Syndactyly


Syndactyly


Syndactyly


Oligodactyly & Macrodactyly


Macrodactyly & Microdactyly


Macrodactyly & Microdactyly


Macrodactyly & Microdactyly


Common <strong>Congenital</strong> Conditions (4)<br />

• <strong>Congenital</strong> constriction band syndrome


<strong>Congenital</strong> constriction band<br />

syndrome<br />

• May result in congenital loss <strong>of</strong> parts.<br />

• Floating strands <strong>of</strong> amnion wrap around<br />

affected part. Early foetal life –<br />

amputation. Later foetal life – deep or<br />

shallow concentric bands.<br />

• Deep bands affect venous and lymphatic<br />

drainage causing distal portion to enlarge.


<strong>Congenital</strong> Constriction Band<br />

Syndrome (1)


<strong>Congenital</strong> Constriction Band<br />

Syndrome (2)


Common <strong>Congenital</strong> Conditions (5)<br />

• Lobster claw foot/cleft foot/partial<br />

adactyly


Lobster claw foot/cleft foot/partial<br />

adactyly<br />

• Variably missing middle toes and<br />

metatarsals with overloading <strong>of</strong> o<strong>the</strong>r<br />

plantar structures and alteration to gait.


Common <strong>Congenital</strong> Conditions (6)<br />

• <strong>Congenital</strong> vertical talus<br />

• <strong>Congenital</strong> pes cavus<br />

• Club Foot<br />

• Talipes equino equino-varus varus<br />

• Talipes calcaneo calcaneo-valgus valgus<br />

• Talipes calcaneo calcaneo-varus varus<br />

• Talipes equino valgus<br />

• <strong>Congenital</strong> metatarsus adductus


<strong>Congenital</strong> Vertical Talus – Rocker<br />

Bottom Foot


<strong>Congenital</strong> Vertical Talus – Rocker<br />

Bottom Foot


<strong>Congenital</strong> Pes Cavus<br />

• Retracted toes.<br />

• Increased angulation <strong>of</strong> metatarsals.<br />

• Backward tilting <strong>of</strong> calcaneum.<br />

• “Humping” or hog’s back tarsus,<br />

• Trigger hallux.<br />

• Tight and shortened plantar fascia and<br />

tendo tendo-achilles. achilles.


<strong>Congenital</strong> Pes Cavus


Club Foot<br />

• Loose term used to describe any<br />

abnormality in <strong>the</strong> shape <strong>of</strong> <strong>the</strong> foot.<br />

• Latin synonym for club foot is talipes and<br />

<strong>the</strong> descriptive nomenclature for a club<br />

foot combines this term with <strong>the</strong> latin<br />

description <strong>of</strong> <strong>the</strong> deformity.


Talipes - Nomenclature


Club Foot<br />

• If <strong>the</strong> foot is inverted and adducted at <strong>the</strong> mid-<br />

tarsal joint so that it cannot be fully everted <strong>the</strong><br />

deformity is classed as varus. The opposite is<br />

true <strong>of</strong> valgus.<br />

• If <strong>the</strong> foot is fixed in a position <strong>of</strong> plantar flexion<br />

and cannot be fully dorsi dorsi-flexed flexed <strong>the</strong> deformity is<br />

described as equinus. The opposite is describes<br />

as calcaneus.


Talipes


Talipes Equino Equino-Varus Varus


Talipes Equino Equino-Varus Varus<br />

• Incidence – 2-4 4 times in every 1000 births.<br />

Males affected twice as <strong>of</strong>ten as females. In half<br />

<strong>of</strong> affected children both feet are deformed.<br />

• Unless very slight <strong>the</strong> diagnosis is obvious with<br />

<strong>the</strong> heel being drawn up, <strong>the</strong> foot inverted and<br />

<strong>the</strong> hindfoot adducted.<br />

• In a small minority <strong>the</strong> deformity is postural and<br />

easily corrected by manipulation to neutral and<br />

beyond.<br />

• The majority have rigidly deformed feet.


Talipes Equino Equino-Varus Varus – Aetiology<br />

(1)<br />

• “It has been suggested that raised intra intra-uterine uterine pressure<br />

forces <strong>the</strong> lower limbs limbs <strong>of</strong> <strong>the</strong> <strong>the</strong> foetus against <strong>the</strong> walls <strong>of</strong><br />

<strong>the</strong> uterus so as to mould <strong>the</strong> feet into <strong>the</strong> position <strong>of</strong><br />

deformity. The presence <strong>of</strong> lesions <strong>of</strong> <strong>the</strong> skin over <strong>the</strong><br />

convex aspect <strong>of</strong> <strong>the</strong> foot in talipes equino equino-varus equino equino-varus varus (TEV)<br />

which very much resemble healed pressure sores, lends<br />

support to this belief. It has alternatively been suggested<br />

that <strong>the</strong> primary disturbance is in <strong>the</strong> muscles <strong>of</strong> <strong>the</strong> calf<br />

where some form <strong>of</strong> contracture possibly ischaemicin<br />

nature, has been visualised as drawing <strong>the</strong> foot into a<br />

position <strong>of</strong> deformity


Talipes Equino Equino-Varus Varus – Aetiology<br />

(2)<br />

• Nei<strong>the</strong>r <strong>of</strong> <strong>the</strong>se postulated aetiological<br />

mechanisms have received widspread support<br />

and <strong>the</strong> most commonly held view isthat <strong>the</strong><br />

primary disturbance is a developmental defect <strong>of</strong><br />

<strong>the</strong> s<strong>of</strong>t tissues <strong>of</strong> <strong>the</strong> leg affecting particularly<br />

<strong>the</strong> ligaments on <strong>the</strong> concave side <strong>of</strong> <strong>the</strong> curve,<br />

or possibly, in <strong>the</strong> case <strong>of</strong> TEV, <strong>the</strong> development<br />

<strong>of</strong> <strong>the</strong> neck <strong>of</strong> <strong>the</strong> talus. <strong>Congenital</strong> club foot<br />

may be paralytic and secondary to<br />

myelodysplasia.”<br />

Bailey Bailey and and Love’s Love’s Short Short Practice Practice <strong>of</strong> <strong>of</strong> surgery surgery


Talipes Equino Equino-Varus Varus -<br />

Management<br />

• Conservative – Correction obtained and maintained by Dennis<br />

Brown splint. The splint is progressively bent to hold a greater<br />

element <strong>of</strong> correction over 77-14<br />

14 days until an over over-corrected corrected<br />

position is reached and <strong>the</strong> child is sent home in splints. Continued<br />

with manipulation over over-correction correction and reapplication <strong>of</strong> splints every<br />

2 weeks until child starts to stand at end <strong>of</strong> first year.<br />

• Alternative strategies – strappings and <strong>the</strong> use <strong>of</strong> PoP.<br />

• Once walking – splints discarded. Appropriate footwear with outer<br />

raise on heel and continued manipulation with night splints.<br />

• Management gradually abandoned with progression to normal<br />

footwear.<br />

• However, <strong>the</strong> limb may be shorter, <strong>the</strong> foot smaller and stiffer than<br />

usual and <strong>the</strong> lower tibial region may look wasted.


Talipes Equino Equino-Varus Varus -<br />

Management<br />

• Surgical – S<strong>of</strong>t tissue release to <strong>the</strong> medial and<br />

posterior aspects <strong>of</strong> <strong>the</strong> foot and ankle. The<br />

ligaments on <strong>the</strong> medial side <strong>of</strong> <strong>the</strong> ankle, talo-<br />

navicular and navicular navicular-cunieform navicular navicular-cunieform cunieform joints are<br />

divided, The tendond <strong>of</strong> Tib. Ant., Tib. Post.,<br />

FHL & FDL are divided and elongated by Z-<br />

plasty.<br />

• In severe cases bony correction will be<br />

necessary.


Talipes Equino Equino-Varus Varus


Talipes Equino Equino-Varus Varus


Talipes Equino Equino-varus varus -<br />

Management


Talipes Equino Equino-varus varus -<br />

Management


Talipes Equino Equino-Varus Varus


Talipes Calcaneo Calcaneo-Valgus Valgus


Talipes Calcaneo Calcaneo-Valgus Valgus<br />

• The opposite deformity to that <strong>of</strong> TEV.<br />

• Excellent prognosis being treated by daily<br />

manipulation.


Metatarsus Adductus


Metatarsus Adductus


Nail Conditions<br />

• Anonychia<br />

• Onychauxis/onychgryphosis<br />

• Onychocryptosis<br />

• Involution<br />

• Macro Macro-onychia onychia<br />

• Micro Micro-onychia onychia<br />

• Claw Claw-like like 5 th toe nails<br />

• Additional/Accessory nails


Anonychia


Onychauxis/Onychgryphosis


Onychauxis/Onychgryphosis


Onychocryptosis – In In-Growing Growing Toe<br />

Nail


Onychocryptosis - In In-Growing Growing Toe<br />

Nail


Involution


The End

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