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Flashcards in Congenital Arm Deck (33):
1

What is this?

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Radial Clubhand

 

2

What is radial clubhand epidemiology

Likely related to Sonic the hedgehog gene

Thumb usually deficient as well

Bilateral in 50-72% 

Incidence 1:100,000

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3

What are the associated conditions of radial club hand ?

TFHOVV

TAR- Autosomal recessive condition with Thrombocyopenia and Absent radius

Fanconii's anaemia= autosomal recessive condition with aplastic anaemia- tx bone marrow transplant

Holt-Oram syndrome- autosomal dominant  characterised by cardiac defects

VACTERL sydrome-vertebral anomalies, anal atresia,cardiac anomalies, transeophageal fistula.  renal agensis and limb defects

VATER syndrome- vertebral anomalies, anal atresia, transeophageal fistula, oesphageal atresia, renal agensis

4

What is the classification system of radial club hand

Bayne and KIng (BK)

Type 1 - Deficient distal radius epiphysis

Type 2- Deficient radius and proximal radial epiphyses

Type 3- Present proximally ( partial aplasia)

Type 4- Completely absent ( total aplasia)- most common

 

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5

What do you see on examination in radial club hand?

Deformity of hand with perpendicular relationship between forearm and hand

Absent Thumb

Careful elbow exam!

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6

What Investigations are useful in radial club hand? 

Xrays- show deficient radius and thumb

U& E

Renal Uss

Cardiac Echo

to screen for VACTERL and VATER syndromes

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7

Describe the currents Tx of radial club hand?

Non operative 

Passive stretching- target radial sided structures

Observation- if absent elbow motion/ biceps deficiency- hand abnormality allows for extra reach to mouth in presence of stiff elbow!

 

Operative

Hand centralisation

8

What are the indications, Contraindications and technique for hand centralisation?

Good elbow movement and biceps function

Done at 6-12 months old

Followed by tendon transfers

Contraindications

Older pts with good function

patients with elbow extension contracture rely on radial deviation

proximal terminal condition

 

Technique

Involves resection varing amounts of carpus, shortening ECU, and angular osteotomy of ulna

may done as 2 stage proceedure with distraction ex fix

If thub deformity then combine with thumb reconstruction at 18 months old.

 

9

What is this?

Can you describe the epidmiology?

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Ulna Club hand

A congential upper extremity abnormality charcterised by a deficiency if the ulna and/or the ulna sided carpal structures

Epidemiology

5-10 times LESS common than radial club hand

10

Name some conditions associated with ulna club hand?

Not systemic cf radial club hand but...

PFFD= Proximal Focal femoral deficiency

Fibular deficiency

Sciolosis

Phocomelia- small limbs- Thalidomide

Multiple hand abnormalities- absent ulnar sided digits

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11

What are the symtpoms and signs of ulna club hand?

Limited function

Usually painless

O/E

Shortened Bowed forearm

Decrease in Elbow function

Loss of digits

12

What is the classificaiton of ulna club hand?

BAYNE

Type 0= Deficiencies of carpus and/or hand only

Type 1= UNDERSIZED ulna with both growth centres present

Type 2= PART of ULNA is missing ( typically distal end)

Type 3= ABSENT ULNA

Type 4= Radiohumeral synotosis

Subtype of each classification based on 1st webspace

A= normal 

B= mild deficienct of webspace

C= moderate to severe deficiency in webspace

D= Absent webspace

see link below for pics

http://www.orthobullets.com/hand/6068/ulnar-club-hand

13

What are the tx goals of ulna clubhand?

Tx depends on 

Hand position, thumb function, elbow stability and syndactyly

Thumb condition most important factor for tx

14

What is the tx of ulnar club hand?

Non operative- STRETCHING and SPLINTING in early tx

Operative

Syndactyly release and digital rotation osteotomies done in 12-18 mo old

Radial head resection and creation of 1 bone forearm- in stage 2 to provide stability at expense of forearm rotation, no gd option to restore elbow motion- not until child >6 mo

Osteotomy of synostosis- in stage iv to obtain elbow motion

15

What is congential dislocation of radial head?

How is it differentiated from truama?

Dislocation of the radial head 

Differentiated by trauma by 

occurs Bilateral

Hypoplastic capitellum

Convex radial head

Other congenital anomalies

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16

What is the pathoanatomy of congenital radial head dislocations?

almost always - POSTERIOR dislocation of radial head

Combined with Bowing and Shortening of radius

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17

What are the symptoms of congenital radial head dislocation?

What do you see on examination?

Symptoms: Asymptomatic but LIMITED Elbow ROM

OE

Radial head prominence

Limited elbow ROM especially in EXTENSION and SUPINATION

Usually painless

18

What is the Xray findings in congenital posterior dislocation of radial head?

Radial head Posterior to Capitellum

Radial head large and convex

Radius is short and bowed

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19

Describe the tx of congenital radial head dislocation?

Non operative- Obeserve- 1st line

Operative- Radial head resection 

Done in adulthood if pt has significant pain, restricted motion and cosmetic concerns of elbow

outcome of resection= reduce pain, may improve some elbow rom

20

What is Madelung's deformity?

A congential DYSCHONDROSIS of the DISTAL radial PHYSIS that ->

Partial deficency of growth of volar and ulnar aspect of  distal radial physis

Excessive Radial inclination & Volar tilt

Ulnar carpal IMPACTION

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21

What is the Epidemiology of Magelung's deformity?

Occurs predominantly in ADOLESCENT Females

Females X4 cf males

Common in gymnasts

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22

What is the pathophysiology of Madelung's deformity?

Disruption of the ULNAR VOLAR PHYSIS of DISTAL RADIUS

thought to be due to repetitive trauma/ dysplastic arrest

One hypothesis - tethering of Vickers ligament

VL- a fibrous band running from distal radius to lunate on volar surface of the wrist

Another is a pathologically thick radiolunotriquetral ligament

23

What is the genetics of Madelung's deformity?

X LINKED DOMINANT Disorder

24

Name any associated conditions of Madelung's deformity?

Leri- Weill dyschondrosteosis

suspect if bilateral, fhx, short stature

A rare genetic disorer 

Mutation in SHOX gene- (short statute homeobox- containing gene)

Anatomically at tip of sex chromosome

-> Mesomelic dwarfism ( short stature)

Madelung's deformity of the arm

 

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25

What are the symptoms and signs of Madelung's deformity?

Symptoms

Asymptomatic until adolescent then

Symptoms of ULNA IMPACTION

Median Nerve irriation

O/E

Leads to Radial and Volar displacement of hand

Ulna becomes dorsally prominent

Restricted forearm rotation- Supination and extension

 

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26

Can you describe the investigations useful in Madelung's deformity and what is seen?

Xrays- Proximal Synostosis

Overgrowth of volar, ulnar corner of radius

Increase radial inclination and volar tilt

MRI: concern re thicknened Vicker's ligament- see thickening from dista radius to lunate

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27

What are the Tx options in Madelung's deformity?

Non operative- Observations only if asymptomatic

Physiolysis- resection of  ulnar zone in distal radial physis and replace by fat so loosening Vicker's ligament= for decreased rom /tightness in wrist

Restricted activity- atheletes w repeitive wrist impaction- cessation of weight bearing activities until pain ceases

Operative

Corrective osteotomy +/- Distal ulnar resection for pain, cosmetic deformity, functional limitations

28

Describe the technique of corrective osteotomy + distal ulna resection in Magelund's deformity?

Goals - restore mechanics to distal radius

Approach- volar approach to distal radius

Technique-

Darrach's - excison of distal ulna but risk of ulnar translatio of wrist.

Osteotomy distal radius ( closing wedge ) and shortening of ulna, conservation of DRUJ.- Salon et al 2000

staged in some with ex fix to avoid NV stretching

dome osteotomy allows coronal and sagittal correction  

Sauve- Kapanje- if carpus subluxes off radius 

29

What is congential radial ulnar synostosis?

An abnormal bony connection between the radius and ulna

As radius and ulna divide form distal to proximal at 7th wk of development

Synostosis is usually Proximal half

30

What is the Epidemiology and genetics of congenital radial ulnar synostosis?

Bilateral in 60% cases

FHX- Autosomal Dominant but can be sporadic

pts often have DUPLICATION in Sex Chromosome

31

What are the signs of congential radial ulnar synostosis

Children present 3-5 yrs

No pronation or supination

FIXED in varying degrees of pronation (50% pts have>50 degrees of pronation)

Pic below girl trying to supinate hands note that forerams are stil palmar

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32

What investigations are useful and what do you see in congenital radial ulnar synostosis?

xray- Ap and lateral of forearm and elbow

see proximal synostosis

Radius is heavy and bowed

Chromosome analysis identify duplication in sex chromosome

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33

What are the Tx of congenital radial ulnar synostosis?

Non operative- Observation- preferred if defomity is unilateral- most cases this is tx

 

Operative: Osteotomy with fusion: in rare severe pronation >60 degrees

 aim to obtain function of pronation

if unilateral set pronation 10-20 degrees

if bilateral - dominant arm pronation 10-20 degrees, other supination 20-35 degrees

 

technique - use percutanoeus pins to aid fusion

preform at 5 yrs of age

resection and interposition proceedures fail of proximal radial - ulnar joint  will reossify