Download - Congenital anamolies upper limb - Dr.KK
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CONGENITAL ANAMOLIES OF UPPER LIMB
DR. KK
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DEFINITION
• Deformities since birth called congenital anamolies
• Anamolies of hand, forearm, arm, shoulder…
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Incidence
each year about 4 out of every 10,000 babies will have upper limb anamolies
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EMBRYOLOGY
• Onset of development of arm bud - 27 days 4th week
• Well-developed arm bud - 28-30 days• Elongation of arm bud - 34-36 days
5th week • Formation of hand paddle - 34-38
days• Onset of finger separation - 38-40
days• Full separation of fingers - 50-52
days 7th week
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EMBRYOLOGY
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International Federation of Societies for Surgery of the Hand
(IFSSH - 1983)I. Failure of formation of parts (arrest of
development)
A. Transverse arrest (common levels are upper third of forearm, wrist, metacarpal, phalangeal)
B. Longitudinal arrest (including phocomelia, radial/ulnar club hands, typical cleft hand, atypical cleft hand otherwise referred to as part of the spectrum of symbrachydactyly)
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International Federation of Societies for Surgery of the Hand
(IFSSH - 1983)
II. Failure of differentiation of partsA. Soft tissue involvementB. Skeletal involvementC. Congenital tumorous conditions radio-ulnar synostosis, symphalangism (stiff PIP joints with short
phalanges), camptodactyly, arthrogryposis, syndactyly.
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International Federation of Societies for Surgery of the Hand
(IFSSH - 1983)
III. Duplication
IV. Overgrowth
V. Undergrowth (thumb hypoplasia, Madelung's deformity (abnormal distal radial growth)
VI. Congenital constriction band syndrome
VII. Generalised skeletal abnormalities.
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Diagnosis before birth
Antenatal diagnosis by ultrasound is increasingly possible, particularly in cases of aplasia.
This will prepare the parents for the deficiency before birth.
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CLUB HAND
• Club hand deformites are classified into two main categories radial and ulnar.
• Radial club hand• Ulnar club hand
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RADIAL CLUB HAND
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RADIAL CLUB HAND
• Radial club hand includes a wide spectrum of disorders that encompass absent thumb, thumb hypoplasia, thin first metacarpal and absent radius.
• Radial club hand is frequently syndromatic
• 1733
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RADIAL CLUB HAND
Well-developed arm bud - 28-30 days males : females (3:2) 1:55000 to 1:100000 live births
maternal drug exposure, compression of the uterus, vascular injury, Apical Ectodermal Ridge (AER)
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RADIAL CLUB HAND
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RADIAL CLUB HAND
Type I: Short distal radius - mildest type,
Type II: Hypoplastic radius, Type III: Partial absence of radius,Type IV: Total absence of radius - most
common variant.
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RADIAL CLUB HAND
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RADIAL CLUB HAND
Syndromes associated
Holt-Oram,
TAR syndrome ,thrombocytopenia pancytopenia of Fanconi's
anaemia
VATER syndromevertebralAnalTracheo-oesohagealRadial club hand
.
DeLange, Daune, Ives Houston,Roberts, Rothmund Thompson Shokeir syndromes. Aase syndrome
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RADIAL CLUB HAND
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RADIAL CLUB HAND
TREATMENT :
slow distraction by external fixationhand can be aligned on the forearmpreserve some wrist movementindex finger is pollicised.
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Ulnar club hand
• Ulnar club hand is much less frequent than radial club hand
• ranges from mild deviation of hand on the ulnar side of forearm to complete absence of ulna.
• ulnar club hand is usually isolated anomaly
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TREATMENT
Exerciselimb lengtheningOsteotomyRadializationSplintWrist centralization
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Madelung deformity
A growth disturbance in the volar-ulnar distal radial physis
volar and ulnar tilted distal radial articular surface,
volar translation of the hand and wrist,
a dorsally prominent distal ulna.
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Madelung deformity
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Madelung deformity
adolescent females pain, decreased range of motion, and
deformity. genetic etiology and is associated with
mesomelic dwarfism and a mutation on the X chromosome.
Surgery addressing the deforming bony and ligamentous lesions, correcting the abnormal position of the radial articular surface, and equalizing the longitudinal levels of the distal radius and ulna
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Madelung deformity
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Apert's syndrome
craniofacial anomalies with mitten hands.
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Apert's syndrome
• Apert syndrome is a form of acrocephalosyndactyly, a congenital disorder characterized by malformations of the skull, face, hands and feet.
• It is classified as a branchial arch syndrome, affecting the first branchial (or pharyngeal) arch, the precursor of the maxilla and mandible
• Disturbances in the development of the branchial arches in fetal development create lasting and widespread effects.
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Apert's syndrome
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Apert's syndrome
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CLEFT HAND
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CLEFT HAND
• Typical cleft hand is usually bilateral, often affecting the feet as well
• AD
• The deficiency is maximal on the ulnar size, leaving in severe cases only a hypoplastic thumb.
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CLEFT HAND
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Syndromes
Cornelia de Lange’s syndromeOculodigital complexOrodigital complexOtodigital complex (Wildervank syndrome)Silver-Russell syndromeElectrodactyly-ectodermal dysplasia-clefting syndrome
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RING CONSTRICTIONS
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RING CONSTRICTIONS
These are released by cutting large Z-plasties, which often have to be repeated as the finger grows.
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Polydactyly
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THUMB HYPOPLASIA AND APLASIA
• no trapezium or trapezio-metacarpal joint.
• Hypoplastic thumbs can be stabilised and given more movement by tendon transfers.
• The operation of choice is to pollicise the index finger
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THANKZ
"The only disability in life is a bad attitude.“
Scott Hamilton
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CONGENITAL ANAMOLIES OF UPPER LIMB
…….. Known is a drop….…………………..unknown is an ocean………..
Dr. Kalaivanan Kanniyan Assistant Professor – OrthopaedicsArthroplasty and Adult Reconstruction UnitSMCH, Saveetha University, chennai, Tamil
Nadu, India.