Treatment of Radial and Ulnar Deficiency · PDF file8/10/2015 1 Treatment of Radial and Ulnar...

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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

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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

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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

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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°

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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

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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

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Step 1

Now What?

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Step 2- Centralization

Elliptical dermodesis incision

Isolate Tendons

Open Capsule

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Identify Ulnar Head

Ulnar Osteotomy

Antegrade 0.062” Wire

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K-wire Joystick

Be The Ball!

Pinning Carpus & Ulnar Osteotomy

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Capsular Repair, Centralize Tendons

ECU Imbrication

Final Product

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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

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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

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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!

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Forearm Treatment Necessary?

Ulnar Dysplasia (Manske)

Condition of thumb determines majority of surgical treatment

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Thanks!!!

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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

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Classification

• Partial vs complete

• Simple vs complex

Complicated

• Synpolydactyly

Complicated

• Acrocephalosyndactyly

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Complicated

• Amniotic band syndrome

Complicated

• Cleft hands

Complicated

• Superdigits

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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

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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

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Skin Grafts

Skin Grafts

Skin Grafts

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Many Techniques

Waters & Bae, 2012

Drawing the Incision

Waters & Bae, 2012

Drawing the Incision

Waters & Bae, 2012

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Volar Commissure

Waters & Bae, 2012

Volar Commissure

Lateral Nail Folds

• Buck-Gramcko flaps

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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

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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

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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

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Thanks

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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

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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

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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

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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

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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

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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

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Ablation/Reconstruction

Ablation/Reconstruction

Ablation/Reconstruction

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Ablation/Reconstruction

Ablation/Reconstruction

Ablation/Reconstruction

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Ablation/Reconstruction

Ablation/Reconstruction

Ablation/Reconstruction

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Closing wedge osteotomy

Chew et al. J Hand Surg Eur Vol (2010) vol. 35 (8) pp. 669-75

CM

CM

Extensor Tendon Neurovascular Bundle

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CM

Flexor Tendon (eccentric insertion)

CMAPB Insertion

Subperiosteal Elevation

CM

APB insertion lifted with RCL Insertion

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CM

Two-Facet MC Head

CM

Abductor Reinserted

CM

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JL

JL

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JL

KN

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KN

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KN

CC

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Type B

Type A

Postaxial Polydactyly

• Well-formed extra ulnar digit

• African descent = other

• Associated anomalies - foot polydactyly

Postaxial Polydactyly Type A

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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

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• Treatment - deletion of digit:

ligature

ligaclip

surgical

Post-axial Polydactyly Type B

My Preference

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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

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• Treatment - individualized:

motion, stability, appearance

Sympolydactyly

Sympolydactyly

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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

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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

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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”

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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

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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