Pediatric Congenital Forearm and Elbow
-
Upload
jeffrey-wint -
Category
Health & Medicine
-
view
62 -
download
5
Transcript of Pediatric Congenital Forearm and Elbow
Congenital Problems of the Forearm and ElbowJeffrey C. Wint, M.D.
The Hand Center of Western Massachusetts
CONGENITAL PROBLEMS OF THE FOREARM AND ELBOWJeffrey C. Wint, M.D.
@[email protected] 413 733 2204
SourcesMorrey, The Elbow and its
DisordersBuck-Gramcko, Congenital
Malformations of the Hand and Forearm
Peimer, Surgery of the Hand and Uper Extremity
EmbryologySynovial joint forms from 7 -10
weeks in uteroEnchondral ossification
◦begins in radius and ulna at 7 weeks in elbow region
◦olecranon and coronoid at 12 weeks◦radial tuberosity at 14 weeks
Physes begin at 14 weeks
Postnatal DevelopmentCRMTOL /CRITOE ossification
centers appear◦Capitellum 1- 2 years◦Radial head 3- 4◦Medial or Inner epicondyle 5 - 6◦Trochlea 7- 8◦Olecranon 9-10◦Lateral or External epicondyle 11- 12
CRITOE
Amputations
Transverse failure of formation 1/20,000 live births below elbow 1/270,000 live births above elbow
Constriction band 1/15,000 (intrauterine deformation)
Associated with systemic anomalies
Embryology- Limb Patterning
• Limb growth= 3 axis system– Proximodistal– Anteroposterior• radial/ ulnar• pre- axial/ post axial– Dorsoventral
Proximodistal AxisAER• Ectoderm over limb bud• Promotes cell proliferation w/o
differentiation• AER key for morphogenesis,
limb elongation• Absence= aplasia Scanning electron micrograph of a 4-week human embryo (5mm), with 34 pairs of somites. Toward the lower left, the right arm bud protrudes from the body. (From Jirásek JE: Atlas of human prenatal morphogenesis, Amsterdam, 1983, Martinus Nijhoff.)
Proximodistal AxisFibroblast growth factor• FGF-2, 4, 8• FGF 10- Transverse arrest• HOX genes• Absent HOX= absent distal
elements• BMPs
Embryology• Loss of AER limb truncation ortransverse deficiencies
AmputationsBelow elbow
Congenital transverse deficiency is defined according to the last remaining bone segment
◦Functional arc of elbow◦Most common transverse deficiency ◦Rarely associated with other anomalies◦Normal biceps and triceps
Radial head dislocation Radioulnar synostosis
Amputations/BEAPassive limb, sitting (6 months)Active terminal ( 2 years)
◦Cable and harness (body powered) 34%
◦Myoelectic 44%, 30%
Compliance and prehension use limited prior to age 8
Kruger LM, Fishman S, Myoelectric and body powered prosthesis JPO 13:68, 1993
BEA
Amputations/BEASurgery
Z plasty for constriction bands Kruckenberg
BILATERAL BLIND AMPUTEE developing countries
◦Transplantation
Amputation/BEA
Kruckenberg procedure
Burkhard Heim 1925 –2001German theoretical physicist
Kruckenberg
Kruckenberg
stump over 10 cm long from the tip of the olecranon
no elbow contracture,
good psychological preparation and acceptance.
J Bone Joint Surg Br. 1991 May;73(3):385-8.The Krukenberg hand.Garst RJ.
TransplantNot for pediatric patients
◦About 50 adult patients worldwide◦US 1999 first case
Recent publicity
Phildelphia child hand transplant
Radioulnar synostosis
Radioulnar synostosisCongenital Synostosis of Radius
and Ulna Sandifort 1793 first anatomic
description
Congenital radioulnar synostosisUpper limb bud 25-28 daysEnd of growth and differentiation at 48 -
50 daysElbow first discernable at 34 days
intrauterine development forearm is in a position midway between neutral and full pronation … thus failure of proximal RU joint differentiation typically leaves the forearm forever in its fetal position
Congenital radioulnar synostosisetiology unknowngenetic basis for some cases
◦20% of their patients, Cleary and Omer found a genetic basis for an autosomal dominant form (with variable penetrance)
positive FH has been reported Leary JE, Omer GE. Congenital proximal
radio-ulnar synostosis. Natural history and functional assessment. J Bone Joint Surg Am. Apr 1985;67(4):539-45
Congenital radioulnar synostosisacropolysyndactlyly
( Carpenter's syndrome)acrocephalosyndactyly ( Apert's)Arthrogryposismandibulofacial dysostosisnondysfunctional sex
chromosomal abnormalities ie Klinefelter's syndrome
Congenital radioulnar synostosis3/2 male
functional complaints are variable◦ Pain is usually not a presenting symptom until the
teenage years, when progressive and symptomatic radial head subluxation may be noted
Often undiscovered◦ Age 6 average with range age 6 months -22years
Leary JE, Omer GE. Congenital proximal radio-ulnar synostosis. Natural history and functional assessment. J Bone Joint Surg Am. Apr 1985;67(4):539-45.
Congenital radioulnar synostosistetralogy of fallotVSD hypoplasia of first and second ribs
and pectoral musculaturemicrocephalyhydrocephalusencephaloceleMRdevelopmental delayhemiplegia
Congenital radioulnar synostosis
Associated hand anomaliesthumb aplasia or hypoplasiapolydactylysyndactylycongenital constriction ring
Congenital radioulnar synostosis
Wilkie 2 types of congenital synostosis,
based on the proximal radioulnar junction
Type 1 complete synostosis with radius and ulna fused proximally
Type 2 partial union associated with radial head dislocation and distal to the physis..Wilkie DP. Congenital radio-ulnar synostosis. Br J Surg. 1914;1:366-75.
Congenital radioulnar synostosis
Congenital radioulnar synostosis
Cleary and Omer four radiographic types:
I fibrous union with a normally-located radial head
II, osseous synostosis with a normal radius
III, osseous synostosis with posterior dislocation of a hypoplastic radial head
IV, a short osseous synostosis with an anterior dislocation of the radial head.
Congenital radioulnar synostosis
Cleary and Omer
However, we noted no relationship between any of these patterns and function.We concluded that operative treatment of congenital radio-ulnar synostosis is rarely indicated, that less emphasis should be placed on the single factor of the position of the forearm
Congenital radioulnar synostosis
Congenital radioulnar synostosiscarpal bone rotatory hypermobility of
up to 45 deg is present but carpal instability does not become a problem
40% fixed pronation < 30 degrees40% > 60 20% between 30 and 60 degrees
<30 degrees of fixed pronation usually do not need surgery
Congenital radioulnar synostosis compensatory rotation around the wrist and functional
results after rotation osteotomy, 40 cases mean pronation of the ankylosed forearm in those who
complained of disabilities in daily life was 60.7° and without complaints was 21.2°.
In almost all cases with total ankylosis, the forearm had compensatory movement around the wrist, the mean arc being from 76.3° of pronation to 42.9° of supination.
13 limbs in 11 patients treated by transverse rotational osteotomies through the fusion mass have followed up for over two years. The functional results after surgery were satisfactory in all patients. Rotational osteotomy of the forearm is a useful and reliable treatment for congenital radio-ulnar synostosis.T. Ogino1 and K. HikinoCongenital radio-ulnar
synostosis: Compensatory rotation around the wrist and rotation osteotomy Journal of the British Society for Surgery of the HandVolume 12, Issue 2, June 1987, Pages 173-178
Congenital radioulnar synostosis if surgery is to be done it is to improve position alone and is best
done prior to school age with a derotational osteotomy through the proximal synostosis
De-rotation of up to 45 degrees can be done at once but if greater, best in more than one sitting ◦ watch out for vascular compromise and go back ie ease off rotation if you
have to.
unilateral involvment, optimum final position is 20 deg of pronation some authors believe nondom should be 20 supination… but that is
awkward bilateral place the dominant arm in 20 pronation and tailor the non
dom in a kid place in neutral in an adult the nondominant arm MAY be tailored to the specific task…tricky though don’t try to overthink
Congenital radioulnar synostosis
Alternatives7 patients associated radial head dislocationsaverage age 8 all maleFree vascularized Fascio-Fat graftsRadial osteotomy
The Journal of Bone and Joint Surgery 80:1186-92 (1998)© 1998Mobilization of a Congenital Proximal Radioulnar Synostosis with Use of a Free Vascularized Fascio-Fat Graft*FUMINORI KANAYA, M.D. and KUNIO IBARAKI, M.D., OKINAWA JAPAN
Radioulnar synostoses
Radioulnar synostosisElbow slight flexion deformityForearm may also be shortRotational hypermobility at wristPronation in forearm fixed at:
◦40% less than 30 degrees◦40% more than 60 degress
Radioulnar synostosisPronation dictates functional loss
◦May not be noticed at young age in unilateral with little pronation contracture (avg. age dx at 2.5 years )
◦Difficulty using spoon, pencil or holding small object
◦Difficulty dressing◦Backhanded use of bottle or toy◦Sports difficulty
Radioulnar synostosisNonoperative
◦Less than 60 degrees◦Unilateral◦Compensatory intercarpal and
radiocarpal supination◦Kids do OK, parents and teachers
can be concerned
Radioulnar synostosisOperative
◦Resection, interposition, etc POOR◦Derotational osteotomy for those
fixed in greater than 60 degrees of pronation. Distal to coronoid Pin fixation Compartrment syndrome reported up to
36%
Radioulnar synostosis
If second pin used allow forquick removal if signs of vascular problem develops
Radioulnar synostosisEmbryionic common cartilaginous
analage separates into radius and ulna ( for a time the radius/ulna share common perichondrium)
Pronation predominatesUsually isolated eventMale/ female 3/280% bilateralAssociated syndromes
Radioulnar synostosisclassification
Original Type 1: proximal or true
radioulnar synostosis
radius and ulna are smoothly fused at their proximal borders for a variable distance.
Type 2: radioulnar synostosis
with congenital dislocation of the head of the radius in which the fusion is just distal to the proximal radial epiphysis.
Modified (Cleary et al., "Congenital Proximal Radio-Ulnar Synostosis." JBJS, 67-A:4, 1985.)
Type I: synostosis does not involve bone, associated with reduced radial head.
Type II: visible osseous synostosis, associated with normal reduced radial head.
Type III: visible osseous synostosis with a hypoplastic and posteriorly dislocated radial head.
Type IV: short osseous synostosis with an anteriorly dislocated mushroom shaped radial head.
Congenital Pseudarthrosis of the forearmLike tibiaRareOne or 2 bonesNeurofibromatosis
Does not “fade away” risk of malignant transformation still exists even after benign report
Elbow synostosisRAREHumerus with both radius and
ulna◦Primary failure of cavitation◦Absence of intrauterine elbow
motion◦Ulnar club hand◦Phocomelia variants
Cartilaginous synostosis
Bony ankylosis
Cartilaginous synostosis ulnar club hand
Phocomelia
Elbow synostosisOperative treatment
◦Synostosis excision and interposition are not likely to work
◦Osteotomy indicated for poor positioning of the hand in space
Radiohumeral synostosis
Complete Ulnar HemimeliaRadiohumeral synostosis and other
anomalies, associated with tridactyly and elbow malrotation
Abdulkadir AY, Adigun IA. Ulnar Hemimelia with Oilgodactyly: Report of Two Cases. Radiology Case Reports. [Online] 2009;4:240.
Dislocated Radial HeadIsolatedAssociated with other congenital
anomaliesAssociated musculoskelatal
anomalies
Dislocated Radial HeadMaroteux-LamyArthrogryposisCornelia de
Lange’sCraniofacial
dystosisDyschondroosteo
sisEhlers- DanlosKlinefelter’sLarsen’s
Mesomelic dysplasia
Multiple osteochondromatosis
Nai-patella syndrome
Rubenstein-TaybiSilver
Dislocated Radial HeadCongenital hip
dislocationClubfeetBrachydactylyClinodactylyTibial fibular
synostosis
Radial or ulnar club hand
Madelung’sFamilial
osteochondromatosis
Dislocated Radial HeadUsually posterior or
posterolateralUnilateral anterior can be
confused with acute cases
Dislocated Radial HeadPresent late after birth
◦Limited elbow extension◦Posterolateral prominence◦Activity pain◦Mld cubitus valgus
Dislocated Radial HeadNon operativeOperative
◦excision
Dislocated radial head
Dislocated radial head
Pterygium cubitale
Pterygium CubitaleWeb across antecubital fossaLoss of elbow extension
◦Involvement of all anatomic structures
◦Similar ro arthrogryposis◦AR and AD inheritance◦Associated anomalies
Nonoperative treatment
Pterygium cubitale
Pterygium cubitale
Psuedarthrosis forearm
Congenital Psuedarthrosis ForearmRare46 cases in literature
◦Ulna 20, Radius 15, both 11Neurofibromatosis
Pseudarthrosis forearm
Ulnar Dimelia
Ulnar Dimelia
Ulnar DimeliaTwo ulna no radiusRare 60 cases reportedUnilateralTwo ulnohumeral jointsElbow and forearm motion 50%
reduced
Ulnar DimeliaSurgery
◦Excise one olecranon Preaxial ( radial) Restores some elbow and forearm motion
Treat hand later
Ulnar Dimelia
Phocomelia“seal limb”Intercalary deficiencyVariableElbow may be dysplastic or
totally absentShoulder girdle often good1960’s Thalidomide
Phocomelia
PhocomeliaSurgery rarely indicatedProsthesis not helpful in many
cases but if it is to be worn, save do not take off fingers
Lengthening has been described◦Clavicular transpostion◦Fibular allograft
Thank you
Thank you