Upload
dinhque
View
220
Download
4
Embed Size (px)
Citation preview
8/10/2015
1
Treatment of Radial and Ulnar
Deficiency
Joshua M. Abzug, MD
Director, Pediatric Orthopaedics
Deputy Surgeon-in-Chief, University of Maryland Children’s Hospital
Longitudinal Deficiency
Named for missing bone
Radial
Ulnar
Intercalary
Radial Longitudinal Deficiency
“Radial club hand” a misnomer
Not just deficiency of radius
Humerus and ulna frequently small as well
Carpus and digits may be hypoplastic or missing
50% are bilateral
Seen with syndromes
Holt-Oram
TAR
Fanconi’s anemia
VACTERL
8/10/2015
2
Radial Deficiency
Fanconi anemia Chromosomal Challenge Test
Chromosomes are fragile and
recombine
Onset of anemia: 8 yrs; diagnosis
made before onset of hematologic
manifestations in only 30%
Can be cured by bone marrow
transplant if dx in time
Radial Deficiency
Thrombocytopenia – Absent Radius (TAR) CBC• Severe thrombocytopenia in first year
• Thumb is present
Radial Deficiency
Holt Oram Echocardiogram• Cardiac abnormalities usually septal defects
8/10/2015
3
Radial Deficiency
VACTERL Association Skeletal survey, renal U/S, echo• Vertebral, anal, cardiac, TE fistula, renal and radial anomalies, LE
anomalies
Radial Deficiency
Classification of radius deficiency
1: slightly short radius (distal)
2: hypoplastic radius (both ends)
3: partial absence (distal)
4: total absence of radius
Radial Dysplasia
Decision Making
Prognosis of related conditions
Timing related to associated conditions
Severity of deformity
Assess child’s ability to get hand to
mouth (elbow motion, radial
deviation, wrist flexion, etc.)
Expectations of parents
Age and adaptation of patient
8/10/2015
4
Radial dysplasia - Treatment
Stretching
Splinting
Soft tissue distraction
Surgical rebalancing
Centralization/Radialization/Ulnarization
Ulnocarpal arthrodesis
Soft tissue procedures
Ulnar lengthening
Radial lengthening
Free-tissue transfer
2nd MTP joint transfer
Nonoperative Treatment
Kotwal et al. JHS Eur 2012
Retrospective review – 446 patients (137 nonop/309
operative – centralization or radialization)
Surgical patients
Improved appearance
Improved function
Improved alignment
Improved range of motion
Improved strength
Radialization/Centralization/Ulnarization
Does well if performed following soft tissue distraction
- Goldfarb et al. JHS 2006
- Nanchahal and Tonkin JHS Br 1996
- Kanojia et al. JHS Eur 2008
Recurrence
Damore et al.
Correction from 83° to 25° but 6.5 years later 63°
8/10/2015
5
Ulnocarpal arthrodesis
Pike et al.
Good salvage procedure following recurrence of deformity
after a centralization
Indications
- Radial angulation > 45°
- Inability to actively extend wrist to within 25° of neutral
- Both
Ulnar Lengthening
Typically performed with a circular frame
- Correct radial deviation simultaneously
Farr et al.
6 patients – initial gains not maintained
25° preop 23° 4 years postop
Major complications – Ulnar fracture; Insufficient rejenerate
Peterson et al.
9 children all had at least 1 pin tract infection
Yoshida et al.
1st Lengthening 59% 89% but the regressed to 70%
2nd lengthening 102% regressed to 83% and growth markedly
decreased
Radial Lengthening
(with simultaneous soft tissue distraction)
Matsuno et al.
4 cases Bayne and Klug type II/III
Several lengthenings needed to correct recurring
discrepancy
Only 2/4 had acceptable function and appearance despite
multiple procedures
8/10/2015
6
Vascularized 2nd MTP joint transfer
Vikki et al.
24 patients with 11 years of followup
Complications present in >50%
5 failures
6 cases of joint subluxation
Additional patients needed subsequent osteotomies or joint
transfer procedures
My Preference
Assess ability to get hand to face
Relative contraindication if radial deviation and wrist
flexion required
Soft tissue distraction followed by centralization at 12-18
months of age
Most improvement with lowest complication rate
1 ½ year-old male with left type IV radial deficiency
8/10/2015
7
Step 1
Now What?
8/10/2015
8
Step 2- Centralization
Elliptical dermodesis incision
Isolate Tendons
Open Capsule
8/10/2015
9
Identify Ulnar Head
Ulnar Osteotomy
Antegrade 0.062” Wire
8/10/2015
10
K-wire Joystick
Be The Ball!
Pinning Carpus & Ulnar Osteotomy
8/10/2015
11
Capsular Repair, Centralize Tendons
ECU Imbrication
Final Product
8/10/2015
12
Thumb Hypoplasia
Part of spectrum of Radial longitudinal deficiency
Thumb Hypoplasia (Radial Deficiency)
Blauth Classification of thumb hypoplasia
1: mild generalized hypoplasia
2: thenar hypoplasia, webspace tight, MP joint unstable
3: same as type 2 with skeletal anomalies
• 3A: stable CMC joint
-------------------------------------------------------------------
• 3B: unstable CMC joint
4: pouce flottant
5: total absence of thumb
Reconstruction
Pollicization
Thumb Hypoplasia:
Treatment
I. +/-Opponensplasty
II. + Web & MCP UCL
reconstruction
IIIa. + Extrinsic tendon reconstruction
- - - - - - - - - - - - - - - - - - - - - - - - - -
IIIb. Pollicization
IV. Pollicization
V. Pollicization
8/10/2015
13
Ulnar Deficiency
Much less common than radial deficiency - 1/10th
Fewer associations with syndromes
Associated with other limb malformations
Fibular hemimelia, PFFD, scoliosis
Bilateral in 25%
Classification varies
No hematologic work up needed but must check spine and LE
Ulnar Deficiency-
Classification(Bayne)
Type I - Small ulna - both physes present
Type II - Partial absence of ulna
Type III - Complete absence of ulna
Type IV - Radiohumeral synostosis
Ulnar Deficiency
Type I - Small ulna -
both physes present
8/10/2015
14
Ulnar Deficiency
Type II - III Partial to
complete absence
of ulna
Ulnar Deficiency
Type IV
Radiohumeral
synostosis
Ulnar Deficiency –
Treatment
? Anlage excision
Osteotomy to correct bow
Hand reconstruction!
8/10/2015
15
Forearm Treatment Necessary?
Ulnar Dysplasia (Manske)
Condition of thumb determines majority of surgical treatment
8/10/2015
16
Thanks!!!
8/5/2015
1
Treatment of Syndactyly
Roger Cornwall, M.D.
Division of Orthopaedic Surgery
Cincinnati Children’s Hospital
Disclosures
• No financial disclosures
Background
• 2-3 per 10,000 live births
• Slight male predominance
• 50% bilateral
• Sporadic, autosomal dominant, syndromic
• Maternal smoking
8/5/2015
2
Classification
• Partial vs complete
• Simple vs complex
Complicated
• Synpolydactyly
Complicated
• Acrocephalosyndactyly
8/5/2015
3
Complicated
• Amniotic band syndrome
Complicated
• Cleft hands
Complicated
• Superdigits
8/5/2015
4
Timing of Treatment
• Separate border rays early
• 3rd webspace syndactyly – no hurry
The Math of Treatment
The Math of Treatment
Waters & Bae: Pediatric Hand and Upper Limb Surgery: A Practical Guide, 2012
8/5/2015
5
Graftless Techniques
• Local tissue advancement
• Not quite closing
• Defatting
• Stretching
• Tissue expansion
Graftless Techniques
• Local tissue advancement
• Not quite closing
• Defatting
• Stretching
• Tissue expansion
Skin Grafts
8/5/2015
6
Skin Grafts
Skin Grafts
Skin Grafts
8/5/2015
7
Many Techniques
Waters & Bae, 2012
Drawing the Incision
Waters & Bae, 2012
Drawing the Incision
Waters & Bae, 2012
8/5/2015
8
Volar Commissure
Waters & Bae, 2012
Volar Commissure
Lateral Nail Folds
• Buck-Gramcko flaps
8/5/2015
9
Graft Donor Sites
• (Groin)
– Hair at puberty, hyperpigmentation
• Volar wrist
– Park et al, 1999
• Foreskin
– Oates and Gosain, 1997
• Antecubital fossa
– Benatar, 2004
Antecubital Fossa
No Grafts in the Commissure
8/5/2015
10
Post-op Protocol
• Type of immobilization
– Cast
– Tape cast
– Boxing glove dressing
• Length of immobilization
– 3 weeks
– Weekly dressing changes?
Complications
• Vascular compromise
– Flaps too tight
– Remove sutures
– Add more graft
• Web creep
• Instability/deformity
Outcomes
• Barabas and Pickford, JHS(E) 2014
– 144 patients, 5 year follow-up
– 7 graft failures, 4% web creep
• Vekris et al, Tech Hand UE Surg 2010
– 131 patients, 11 year follow-up
– Worse results in border digits, delayed
surgery, triangular commissure flaps
• No long-term studies for graftless
techniques
8/5/2015
11
Grafts vs. Graftless
• Doug Hutchinson, MD
• Bilateral syndactyly patients
– One side with grafts
– One side without grafts
• Parents, therapists preferred
the grafts
Best of Both Worlds?
• Landi et al, JHS(E) 2014
– Hyaluronic acid matrix skin graft substitute
– 22 patients, 2 year follow-up
Take Home Points
• Understand potential complexity
– Bony abnormalities, associated problems
• Use meticulous surgical technique
– Careful incision planning
– Not just opening and closing
– Don’t get fancy unless an expert
• Know the pitfalls
– When in doubt, use more graft
8/5/2015
12
Thanks
8/7/2015
1
Treatment of Polydactyly
Dan A. Zlotolow, MDShriners Hospital for Children
Philadelphia
Disclosures
• Elsevier
– Royalties
• Osteomed
– Royalties and Consulting
• McGinley Orthopaedic Innovations
– Shareholder
• Arthrex
– Royalties and Consulting
Polydactyly
• Aberrant segmentation
• “Pre-axial,” radial or Thumb polydactyly
• “Post-axial,” or ulnar polydactyly
8/7/2015
2
Polydactyly• Fragmentation or duplication of AER when hand paddle is undergoing apoptosis leads to digital duplication
• Bmp4 deficiency leads to postaxial polydactyly by delayed induction and maturation of the AER that results in expanded SHH signaling
• AER persists longer in the Bmp4 deficient limb buds, exposing the forming digits to prolonged Fgf8 signaling
Introduction
• Most common congenital difference of the thumb
– 1/1,000-10,000
– More common among caucasian children
– Males>Females
– Typically sporadic
– Triphalangeal thumbs may have other anomalies
Wassel Classification
8/7/2015
3
Modified Classification
Zuidam et al. J Hand Surg Am (2008) vol. 33 (3) pp. 373-7
Anatomy
• More of a split than a duplication
– Both thumbs smaller
– Share components• Flexor tendon
• Flexor sheath
• Extensor tendon
• Collateral ligaments
• Thenar muscles
• Nerves\arteries
Anatomic Variations
• Pollex abductus– Connection between EPL and
FPL – Present 20% of the time– Causes abduction of MP joint
and diminished IP joint flexion crease
• Eccentric Tendon Insertions• Neurovascular structures not
typical
Watt and Chung, 2009
Tay et al. J Am Acad Orthop Surg (2006) vol. 14 (6) pp. 354-66
8/7/2015
4
Surgical Options
• Merge 2 into 1
– Bilhaut-Cloquet
– Modifications
• Remove the smaller thumb (radial)
– Transfer components from one thumb to the other
Horii, et al 2009
Bilhaut-Cloquet
Surgical Goals
• 1 thumb from 2
• Passive joint motion at IP/MP/CMC
• Central flexor tendon with pulleys
• Central extensor mechanism
• Thenar muscles and adductor
• Joint stability
• Neurovascular supply
• No lumps/bumps
Complications
• Instability• Joint stiffness• Inadequate size• Growth arrest• Nail ridging (Bilhaut etc.)• Rotational deformity• Longitudinal Deviation
• Clinodactyly• Z (zigzag) deformity
8/7/2015
5
Modified Bilhaut-Cloquet
Baek et al. J Bone Joint Surg Am (2008) Suppl 2 Pt 1 pp. 74-86
Modified Bilhaut-Cloquet
Baek et al. J Bone Joint Surg Am (2008) Suppl 2 Pt 1 pp. 74-86
Modified Bilhaut-Cloquet
Baek et al. J Bone Joint Surg Am (2008) Suppl 2 Pt 1 pp. 74-86
8/7/2015
6
Modified Bilhaut-Cloquet
Baek et al. J Bone Joint Surg Am (2008) Suppl 2 Pt 1 pp. 74-86
Divergent/Convergent
Abid et al. Orthop Traumatol Surg Res (2010) vol. 96 (5) pp. 521-4
Ablation/Reconstruction
8/7/2015
7
Ablation/Reconstruction
Ablation/Reconstruction
Ablation/Reconstruction
8/7/2015
8
Ablation/Reconstruction
Ablation/Reconstruction
Ablation/Reconstruction
8/7/2015
9
Ablation/Reconstruction
Ablation/Reconstruction
Ablation/Reconstruction
8/7/2015
10
Closing wedge osteotomy
Chew et al. J Hand Surg Eur Vol (2010) vol. 35 (8) pp. 669-75
CM
CM
Extensor Tendon Neurovascular Bundle
8/7/2015
11
CM
Flexor Tendon (eccentric insertion)
CMAPB Insertion
Subperiosteal Elevation
CM
APB insertion lifted with RCL Insertion
8/7/2015
12
CM
Two-Facet MC Head
CM
Abductor Reinserted
CM
8/7/2015
13
JL
JL
8/7/2015
14
JL
KN
8/7/2015
15
KN
8/7/2015
16
8/7/2015
17
KN
CC
8/7/2015
18
Type B
Type A
Postaxial Polydactyly
• Well-formed extra ulnar digit
• African descent = other
• Associated anomalies - foot polydactyly
Postaxial Polydactyly Type A
8/7/2015
19
Treatment Same as Pre-Axial
Postaxial Polydactyly Type B
• Pedunculated, poorly formed extra digit
• African descent : other :: 10 :1
• Autosomal dominant inheritance
• Isolated finding
Postaxial Polydactyly Type B
8/7/2015
20
• Treatment - deletion of digit:
ligature
ligaclip
surgical
Post-axial Polydactyly Type B
My Preference
8/7/2015
21
Sympolydactyly (Central Polydactyly)• Loss of HOXD 9-13
• Excessive carpals
• Irregular digital elements
• Loss of alternating ID regions
Synpolydactyly
• Autosomal dominant, Variable expression
• Malformation of all involved structures
Sympolydactyly
8/7/2015
22
• Treatment - individualized:
motion, stability, appearance
Sympolydactyly
Sympolydactyly
7/29/2015
1
Joshua A. Ratner MD
The Hand and Upper Extremity Center of
GA
Children's Healthcare of Atlanta at Scottish
Rite
I have NO financial interests to disclose
related to this material.
Classified as an Overgrowth Syndrome
Implicated gene: mosaicism of PI3-K-AKT pathway (proto-oncogene)
Usually follows nerve territory
Two SubtypesStaticProgressive
Goal of surgery
• Function>Aesthetics
7/29/2015
2
Primary goal is to end up with a
digit comparable in size to the
same sex parent’s digit.
Length, circumference and
angular deformity are the criteria
to be considered.
Girth: soft tissue debulking
Length: epiphyseodesis
shortening?
Angulation: osteotomy
Journal of Hand Surgery 2015 40, 1461-1468DOI: (10.1016/j.jhsa.2015.04.017)
Copyright © 2015 American Society for Surgery of the Hand Terms and Conditions
Pulp of digit:
Midline incision to avoid
digital nerve braches
Avoid crossing IP flexion
crease
Consider volar closing
wedge
osteotomy to resolve distal
phalanx recurvatum
7/29/2015
3
Journal of Hand Surgery 2015 40, 1461-1468DOI: (10.1016/j.jhsa.2015.04.017)
Copyright © 2015 American Society for Surgery of the Hand Terms and Conditions
MacrodactylySoft Tissue Debulking
If globally overgrown, use midaxial
incisions, usually gentle zig-zags
Incisions preferred on convex side of
digis
Consider excision of ipsilateral digital
nerve
(much of the dermatome is being
removed)- unless the other side has
had neurectomy
Protect the ipsilateral digital artery
If needed, come back for other side of
digit in 3+ months
Journal of Hand Surgery 2015 40, 1461-1468DOI: (10.1016/j.jhsa.2015.04.017)
Copyright © 2015 American Society for Surgery of the Hand Terms and Conditions
MacrodactylyNarrowing the Nail
“Hemi-digit resection”
7/29/2015
4
Journal of Hand Surgery 2015 40, 1461-1468DOI: (10.1016/j.jhsa.2015.04.017)
Copyright © 2015 American Society for Surgery of the Hand Terms and Conditions
Usually combined with
debulking
Drill bit or k-wire to destroy
physis
Add K-wires to compress
Closing wedge osteotomy
along physis where needed
Young child with a digit that is
already larger than same sex
parent’s digit.
Likely progressive variant
Shortening procedures often
do not have positive
outcomes.
Ray amputation should be
considered
7/29/2015
5
Midaxial incisions except at the pulp
Glabrous skin incisions can keloid
• Support for Methotrexate use
Make sure epiphyseodesis is global
Consider sagittal correction with osteotomy, not just coronal
Consider sacrificing DIP joint to shorten
Use Colorado tip bovie
Leave drains/ loosely spaced sutures
MANAGE EXPECTTIONS
Gluck and Ezaki, Journal of Hand Surgery 2015 40,
1461-1468)
Copyright © 2015 American Society for Surgery of the Hand Terms and Conditions