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Upper Extremity > Congenital Hand > Flashcards

Congenital Hand Flashcards

1
Q

List the top 3 most common congenital hand anomalies

A
  1. Polydactyly
  2. Syndactyly
  3. Camptodactyly
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2
Q

what is the LEAST common ray pair involved in syndactyly? why?

A
  • thumb-index
  • first to separative via apoptosis
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3
Q

in patients w non-syndromic isolated syndactyly, when do you perform release?

A
  • 6-9 mos: border digits, complex (skeletal involvement) or complicated
  • up to 2 years: central digits, simple
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4
Q

what is your differential of a child of 6 months with a flexed thumb?

A
  • delayed maturation of radial/extensor neuromuscular unit
  • trigger thumb
  • clasped thumb
  • camptodactyly of thumb
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5
Q

what syndromes are associated w longitudinal radial deficiency?

A
  • Fanconi anemia
  • Thrombocytopenia absent radius (TAR) Syndrome
  • VATER (vertebral, anal atresia / imperforate anus, TE fistula, esophageal atresia, renal)
    • or VACTERL (vertebra, anal atresia, cardiac, TE fistula, esophageal atresia, renal, lower limb abn)
  • Holt-Oram (associated cardian anom)
  • Duanne radial ray syndrome
  • HFM
  • Nager Syndrome
  • Thalidomide syndrome
  • various chromosomal aberrations
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6
Q

what is Bower’s criteria?

A
  • criteria that indicates temporal age-related involution of hemangioma
  • 50% by 5 yrs, 70% by 7 yrs, 90% by 9 years
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7
Q

DEFINE THE FOLLOWING TERMS:

  • AMELIA
  • PHOCOMELIA
  • ACROSYNDACTYLY
  • CAMPTODACTYLY
  • CLINODACTYLY
  • BRACHYMETACARPIA
  • SYMBRACHYDACTYLY
A
  • Amelia: congenital absence of limb(s)
  • Phocomedial: upper extremity longitudinal intersegmental deficiency
  • Acrosyndactyly: distal lateral fusion between adjacent digits (skin/soft tissue+/- bone) with proximal separation
  • Camptodactyly: congenital (or adolescent) volar flexion deformity of PIPJ, typically D4.5
  • Clinodactyly: congenital radial flexion deformity in radial-ulnar plane of PIPJ, typically D5
  • Brachymetacarpia: congenital short metacarpal
  • Symbrachydactyly: short, hypoplastic digits with proximal soft tissue fusion
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8
Q

Summarize temporal embryologic development of the upper extremity

A
  • development occurs during critical period of rapid embryo growth from 4 to 8 weeks
  • @ 4 wks, limb bud develops as an invagination of mesoderm into ventrolateral wall ectoderm
  • Weeks 5-6: flat, paddle-shaped hand-plate develops
  • Weeks 6-8: finger separation occurs in steps: 5 mesenchymal condensations, digits webbed, separation complete - via fragmentation of AER and apoptosis
  • Week 8: embryogenesis complete, skeletal structures are cartilage
  • Week > 8: enlargement, maturation, differentiation of existing structures
  • Month 4-5: sweat gland and nail growth and development
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9
Q

List the 3 spatial axis pattern formation

A
  • Proximodorsal
  • Radioulnar
  • Dorsoventral
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10
Q

describe spatial patterning or formation of the upper extremity during embryogenesis

A
  • Proximo-distal
    • Signalling centre is apical ectodermal ridge - a condensation of ectoderm overlying the limb bud
      • It’s function is to program proximo-distal growth, and digit separation (via interdigital apoptosis)
    • Also involved is the progress zone, an underlying layer of mesenchymal cells
      • It’s function is to program lateral/distal growth
    • How it work:
      • AER releases FGF2, FGF4, FGF8 as signalling molecules
      • Amount of time spent in the progress zone determines differentiation - for example cells that leave PZ from earliest to latest are destined to be: shoulder, humerus/arm, forearm, hand
  • Radioulnar
    • Signalling centre is Zone of Polarizing Activity - mesenchymal cells along the posterior (ULNAR) ridge of the limb
    • Overall is responsible for positioning ulnar characteristics
    • How it works: ZPA secretes sonic hedgehog in varying concentrations - high concentrations promote dorsal/ULNAR differentiation and low concentrations promote volar/RADIAL differentiation
  • Dorsoventral
    • signalling centre in dorsal ectoderm, releases Wingless type (Wnt) gene signaling
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11
Q

what is the first and last carpal bone to ossify?

A
  • Capitate - 3 to 6 mos
  • Pisiform - 6 - 8 years
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12
Q

What are general treatment goals when considering a congenital upper extremity defect of the hand?

A
  • power grasp
  • precision pinch
  • maintaining growth potential
  • cosmesis
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13
Q

List the IFSSH / Swanson classification of congenital upper extremity anomalies

A
  1. failure of formation
  2. failure of differentiation
  3. duplication
  4. overgrowth
  5. undergrowth
  6. constriction band
  7. generalized skeletal abnormality not otherwise specified
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14
Q

List examples of Swanson / IFSSH type 1:

A
  • failure of formation
    • transverse arrest
    • longitudinal arrest
      • pre-axial / radial longitudinal deficiency
      • axial / central longitudinal deficiency (cleft hand)
      • post-axial / ulnar longitudinal deficiency
      • longitudinal intersegmental deficiency (phocomelia)
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15
Q

list examples of swanson / IFSSH type 2:

A
  • failure of differentiation
    • syndactyly
    • camptodactyly
    • clinodactyly
    • flexed thumb (trigger, clasped)
    • arthrogryposis
      *
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16
Q

list examples of swanson / IFSSH type 3

A
  • duplication / polydactyly
    • pre-axial / radial
    • axial / central
    • post-axial / ulnar
    • ulnar dimelia
    • triphalangism (some considered to be failure of differentiation)
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17
Q

WHAT FEATURES ARE ASSOCIATED WITH:

  • Fanconi anemia
  • VACTERYL
  • Holt-Oram
  • Duanne Radial Ray syndrome
A
  • Fanconi anemia: pancytopenia, ASD, various pre-axial abn
  • VACTERL:
    • vertebral, anal atresia, cardiac, TE fistula, esophageal atresia, renal, lower limb
  • Holt-Oram: septal defect + radial longitdinal def
  • Duane radial ray: ocular & radia/pre-axial
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18
Q

what derivatives of lateral plate mesoderm and what are derivatives of somatic mesoderm?

A
  • lateral plate: bone, cartilage tendon
  • somatic: muscle, vessels, nerves
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19
Q

What upper extremity findings are typically present (abnormal) in longitudinal radial deficiency?

A
  • Thumb: absent or hypoplastic
  • Wrist: radially deviated, flexed, pronated –> UNSTABLE
  • Forearm: radius hypoplastic or absent, ulna short + bowed
  • Elbow: STABLE, maybe stiff, hypoplasia of distal humerus is common
  • D5: normal or near normal appearance and function
  • Fibrous anlage: pre-axial bones/muscles/tendons coalesce, resulting in stiffness
  • Bones:
    • Radius: hypoplastic, partially absent, totally absent
    • Ulna: normal vs. short +/- bowed
    • Trapezium, scaphoid: absent > hypoplastic
    • Thumb Phalanges +/- absent
    • radial digits hyplastic / absent
  • Muscles
    • often absent: BR, EIP, ECRL, ECRB, thumb intrinsic/extrinsic, pronator/supinator
    • other radial sided muscles may be absent/hypoplastic
  • Nerves:
    • Radial nerve and MCN often absent
  • Vessels
    • Radial artery often absent
  • Soft tissue deficiency variable
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20
Q

What are treatment goals for radial longitudinal deficiency

A
  • Correct radial deviation
  • Balance wrist on forearm
  • Promote wrist and finger motion
  • promote growht of forearm
  • REconstruct thumb
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21
Q

Are there any useful non-operative interventions for radial longitudinal deficiency? Explain

A
  • non-op management is crucial to success of treatment
  • can be only treatment for less severe deformities
  • must be executed such that the radial deviation can be passively corrected prior to surgical intervention
  • splinting, serial casting, passive stretching
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22
Q

List some indications and contraindications to operative interventions in radial longitudinal deficiency

A
  • Indications
    • appearance
    • to achieve wrist stability
    • to promote greater finger movement (esp extensor)
  • Contraindications
    • medically unfit (ex: severe syndromic manifestations)
    • unable to passively correct radial deviation pre-op
    • bilateral invovlement w elbow stiffness
    • (older) patient that have adapted functionally
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23
Q

what are potential operative interventions that can be employed for severe radial longitudinal deviation?

A
  • treatment has 3 components after pre-operative physical therapy
  1. stabilization of the wrist
    1. centralization - ulna aligned to 3rd MC
    2. radialization - ulna aligned to 2nd MC (replaces radius)
    3. wrist arthrodesis
  2. Tendon transfers
    1. If ECRL/FCR are present but less functional - transfer to ECU
    2. ECU transfer / advancement - to restore wrist extension centrally or on 5th MC
    3. FCU transferred to extensors
  3. Soft tissue reconstruction (severe soft tissue contraction/deficiency w/ severe cases)
    1. Local or regional well vascularized flaps to cover radial wrist deficiency
  4. Considerations
    1. ulnar closing wedge osteotomy to address bow
    2. pollicization 6 mos after wrist stabilizing procedure
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24
Q

what is a characteristic defining feature between radial longitudinal deficiency and ulnar longitudinal deficiency

A
  • in radial deficiency, wrist is unstable but elbow is stable
  • in ulnar deficiency, wrist is stable and elbow is unstable.
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25
Q

what are associations w ulnar longitudinal deficiency?

A
  • 50% other MSK abnormalities
    • PFFD - proximal femoral focal deficiency; fibula def; club foot
    • spine abn (spina bifida)
    • c/l phocomelia
  • other syndromes: Cornelia de lange, schnizel syndrome - both have developmental delay
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26
Q

what clinical features are usually present or can be present w ulnar longitudinal deficiency?

A
  • forearm: short, residual radius is bowed radially
    • ulna can be hypoplastic, partial absent, absent
  • elbow: can be UNSTABLE, flxn contracture, ltd ROM
  • wrist: misisng ulnar carpus, ulnar deviation when severe
  • hand:
    • missing digits (ulnar MC or phalanges)
    • syndactyly
    • thumb abn including hypoplasia and contracture of 1st web
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27
Q

describe a treatment approach to ulnar longitudinal deficiency

A
  • similar philosphy to radial longitudinal deficiency - goal is to stabilize/centralize the wrist and hand, permit functional hand motion
  • non-operative treatment is mandatory - need PT, splints, serial casting to neutralize hand position
    • may be all that is needed
    • pre-requisite to surgical intervensions
  • operative interventions are limited
    • hand: release of any syndactyly including 1st web deepening, thumb recon (position, reconstruction)
    • forearm: correct radial bowing w closing wedge osteotomy; humoral osteotomy; consider radiohumeral fusion in severe cases
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28
Q

what is the transmission of cleft hand?

A
  • AD w strong variable penetrance; severity increases w subsequent generation; mutation on 7q
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29
Q

what are common associations w cleft hand?

list 1 uncommon association w cleft hand

A
  • common: bilaterality, uni/bilateral foot involvement, finger/toe syndactyly, CL/P
  • uncommon: cornealia de lange syndrome, ectrodactyly-ectodermal dysplasia clefting syndroem
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30
Q

in upper extremity embryogenesis, where is the abnormality thought to arise in cleft hand?

A
  • apical ectodermal ridge
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31
Q

describe associated ipsilateral upper extremity findings in cleft hand

A
  • proximal to hand, no deficiency of vessels, nerves, muscles, tendons or bones
  • hand
    • phalanges: longitudinal bracketed epiphysis, triphalangeal thumb, double phlanges, syndactyly
    • MC: absent, double, bifid, transverse
32
Q

cleft hand and symbrachydactyly are thought to be in a spectrum. compare and contrast

A
  • CLINICAL
    • CLEFT: V-shaped deficiency often w absent 3rd MC, variable degree of defiency of 3rd ray but 5TH ray always present, digit supression is more radial sided, no finger nubbins, syndactyly present (esp first web), carpal bones usually normal, 1-4 limbs involved (classic is all 4)
    • SYMBRACHY: U-shaped deficiency, involves 2,3,4 absent and D1/5 ALWAYS present, supression is more ulnar sided, finger nubbins present, syndactyly absent, carpal bone hypoplasia, classically unilateral u/e
  • GENETIC/EPIDEM
    • CLEFT: insufficiency of AER; M>F, R>L; AD (incomplete penetrance); 1:90,000
    • SYMBRACHY: failure of formation of bone; no sex or side predelection; sporadic, 1:40,000
33
Q

what are the treatment goals for cleft hand?

A
  • reconstruct a functional hand with good pinch and grasp
  • cosmetic result
34
Q

what operative treatments can be offered to treat cleft hand?

A
  • none: in a mild deformity or a patient with a functional hand
  • early syndactyly release of border digits (esp D1/D2 - z-plasty or other web-deepening procedures to facilitate growth of thumb ray)
  • early removal of any transverse bones
  • other syndactyly release as necessary
  • Snow-Littler technique to reduce the cleft and reconstruct an opposing thumb
    • close cleft by transposing index / D2 ray onto residual D3 metacarpal
    • design volarly based flap to transpose to residual D1 web, to provide cover and deepen
    • reconstruct transverse MC ligaments using A1 pulley flaps
  • if thumb is hypoplastic or absent, then appropriate procedures: opponensplasty, pollicization, great toe free transfer
35
Q

discuss treatment options for symbrachydactyly

A

pgoals is to create a functional grast

  • consider creating or deepending the central cleft using web-deepening procedures
  • discard severely hypoplastic and non-functional nubbins
  • consider non-vascularized (or vascularized) toe proximal phalanx transfer to increase length of functional nubbins
  • consider transfer (akin to pollicization) of a functional digit to a more functional position
  • consider free toe transfer (often 2nd toe, perhaps multiple toes)
    • ensure pre-op CTA to investigate vessels/recipients
  • distraction osteogenesis
36
Q

how do you differentiate symbrachydactyly from transverse arrest?

A
  • symbrachydactuly
    • U shaped cleft
    • presence of nail plates on nubbins (indicates presence of distal phalanges)
  • transverse arrest
    • no cleft
    • no nail plates
    • digits/nubbins all arrest at same level
37
Q

what is a differential for carpal tunnel syndrome in pediatric age group?

A
  • congenital:
    • persistent median artery
    • anomolous muscle (FDS, FPL)
    • mucopolysaccharidosis
  • trauma
    • unrecognozed trauma like perilunate injuries
38
Q

what is arthrogryposis?

what swanson class does it fall under?

A
  • arthrogryphosis is a non-progressive congenital disorder of stiff joints, often bilaterally symmetric, with resulting severe limitations in range of motion
  • thought to be a swanson 2, failure of differenitation
39
Q

what are the classic upper extremity clinical characteristics of arthrogryphosis?

A
  • shoulder muscle absent and internal rotation
  • elbow extended
  • wrist flexed and ulnar deviiation
  • fingers flexed, esp at MCPJ
  • thumb flexed and adducted
  • skin is waxy, no creases
  • limbs are thin
40
Q

discuss operative management of arthrogryphosis

A
  • operative management is rarely employed
  • for patients w localized disease, can consider operations to address specific limitation
  • for patients with bilateral and diffuse upper extremity involvement, ensure one arm remains extended positioning (self-care) and one arm/elbow is flexed posture (feeding, to mouth)
  • procedures can be osteotomies for repositioning/neutralization, release of contractures (prom) to tendon transfers (arom)
41
Q

what is congenital trigger thumb?

what is the swanson classification?

what is the characteristic finding on physical exam?

what is the treatment approach - non-operative and operative?

A
  • congenital trigger thumb is a congenital flexion deformity of the IPJ of the thumb, presenting at birth or shortly after birth
  • swanson class 2: failure of differentiation
  • characteristic findings is palpable “Notta’s node” at base of thumb / proximal to A1 pulley
    • on physical exam, triggering may not be observed but rather flexion deformity (or more rarely in extension)
  • there is no non-operative management but, operations are not offered until after a perior of observation, because a certain proportion resolve over time
    • 30% resolve by 1 yr
    • 12% resolve w/i 6 mos of presentation
  • therefore, offer operation in the following circumstances:
    • always when presenting after age 3, or when patient reaches age 3
    • always after 1 yr of age when presenting at birth
    • always after 6 mos observation periord when presenting between 6 - 30 mos
42
Q

how do you differentiate between clasped thumb and trigger thumb?

A
  • trigger
    • flexion at IPJ
    • (Notta’s node)
  • clasped thumb
    • flexion at MCPJ
    • almost all bilateral
    • extension lag at IPJ
43
Q

how do you approach operative treatment for clasped thumb?

A
  • treatment is determined by severity of findings
  • pre-operative PT/SPLINTING always mandatory
  • if supple clasped thumb with deficient extension only: then PT, EIP to EPB transfer (usually this is an isolated EPB deficiency)
  • if there is deficient extension with flexion contracture (not supple), then PT, joint contracture release, FPL lengthening, consider MCPJ arthrodesis (older) or tendon transfer (EIP to EPB or EPL)
  • if there is hypoplasia of the thumb with more extensive extensor musculotendinous deficits, then deepen webspace, consider thumb MC rotational osteotomy, multiple tendon transfers: EIP to EPB/EPL and PL to APB (vs. MCPJ arthrodesis)
44
Q

what is the suspected etiology of syndactyly?

what is its swanson classification?

A
  • failure of: digital patterning, apoptosis, AER regression
  • swanson 2, failure of differentiation
45
Q

what is the order of frequency of webspace involvement for isolated vs syndromic syndactyly

A
  • long-ring
  • ring-small
  • index-long
  • thumb-index

same order for both isolated and syndromic, but in syndromic there is proportionally more 1st web involvement

46
Q

List the primary tenets of syndactyly release (6 marks)

A
  • complete release of all soft tissue and skeletal structure
  • complete preservation of vascular supply and innervation
  • creation of a deep and wide webspace, using local flaps inset tension-free
  • use of synchronous dorsal and volar local flaps
  • use of full thickness skin grafts for resurfacing of remaining defects
  • development of appropriate finger tips

(from grabbe and smith)

47
Q

discuss timing for syndactyly release

discuss sequence of release

A
  • early release @ ~ 6 mos:
    • border digits - due to length discrepency and potential to impair growth
    • complex or complicated syndactyly
  • later release @ ~ 12-18 mos:
    • simple syndactyly of non-border digits
  • sequence when multiple digits involved (ex: Apert syndrome)
    • thumb-index & ring-long
    • index-long & ring-small
48
Q

how is syndactyly classified?

A
  • complete (to tip) vs. incomplete
  • simple (skin, sc only) vs. complex (skin, sc, musculotendinous, skeletal, synonychia)
  • complicated - acrosyndactyly, extra bones, synostosis
49
Q

describe your surgical plan / steps for a complete simple syndactyly release between ring and long

A
  • timing at 12-18 mos
  • markings
    • dorsal flap: height 2/3 from mcp to pip; width: mid-axis of digits
    • synchronoous dorsal and volar zig-zag flaps - best aesthetics w largest dorsal flap on ulnar digit
  • separation of skin, sc and preservation of individual NV bundles following markings
    • need to release conjoined clelands ligament dorsal to NV bundle
    • separation of skeletal structures
  • separation of tip and nail plate (excise any conjoined plate), design interdigitating tip flaps following Buck-Gramco
  • creation of a deep and wide webspace using dorsal flap inset tension-free
  • template and precise FTSG to resurface remaining defect - always required
  • Bolster dressing and splint
50
Q

what are some contraindications to release of syndactyly

A
  • medically unfit
  • severe developmental delay or condition that precludes rehab/post-op care
  • functionally adjusted to syndactyly w/ lack of concern
  • lack of individual stable skeletal elements to support separation
  • superdigit: double MC supporting a single large digit
  • vascular of soft tissue insufficiency
51
Q

what are complciations of syndactyly release?

A
  • early:
    • partial/total flap/skin graft loss, dehiscence, infection, finger ischemia, tip necrosis, bleeding
  • late
    • recurrence, web creep, scar, failure to adjust to separation (cortical repatterning)
52
Q

what are the most commonly involved webs, and the most commonly functional joints in Apert hand?

A
  • Complete 2nd and 3rd web +/- incomplete/complete 1st and 4th web
  • functional joints are MCPJ and D5 PIPJ
  • other IPJ usually have symphangalism
53
Q

what is the classification for Apert hand?

A
  1. obstetrician/spade
    1. incomplete simple 1st / 4th web
    2. complete 2nd, 3rd web, variable symphangalism IPJs (except D5)
  2. mitten/spoon
    1. complete simple 1st / 4th web
    2. complete 2nd 3rd web +/- skeletal abn +/- synonychia
  3. rosebud/hoof
    1. complete complex 1st web, still usually complete simple 4th web
    2. very abnormal skeleton, synonychia, tight cupping central
54
Q

what is a treatment approach to apert hand?

A
  • consideration to surgical release of synonychia in advance as a infection prophylaxis measure
  • if 1st web is complete, then 1st web / 3rd web first
  • 2nd web / 4th web second
  • if 1st web incomplete, may consider D1 MC osteotomy, 2nd/4th web (border digit) release then deepen 1st web w/ 3rd web release 2nd stage
55
Q

what is camptodactyly

A
  • congenital flexion deformity of PIPJ, typically D5, typically associated w/ MCPJ hyperextension
56
Q

what are xray findings seen w camptodactyly

A
  • lateral xray
  • flat dorsal head PIPJ
  • exaggerated recess of PIPJ neck
  • narrow PIPJ joint space
  • beak of volar base of MP
57
Q

discuss treatment considerations and options for camptodactyly

A
  • considerations: will not progress once growth stops
  • non-operative management first
    • patients w flexion < 30’ do not need an operation
  • operative intervention may not help, may make problems worse
  • consider operative intervention when
    • rapid progression
    • failure conservative
    • flexion > 90’s
  • Surgical treatment options include
    • lumbrical: release, transfer
    • FDS: tenolysis, lengthening, transfer (to lateral band), release
    • address skin contracture if present: z-plasty
    • most reliable option may be to arthrodese PIPJ in a more favourable functional position
58
Q

what is symphalangism

A
  • congenital stiffness of any finger joint, most commonly the PIPJ, most commonly ulnar digits
59
Q

what physical exam finding corresponds w symphalangism

A

absent creases

60
Q

discuss treatment considerations for symphalangism

A
  • finger is ankylosed in extension, need to consider what is a more functional position
  • consider arthrodesis in slight flexion
  • in adults, consider interpositional arthroplasty (ie use VP)
    *
61
Q

why is there deviation in the radio-ulnar plane in clinodactyly?

A
  • there is an abnormal longitudinally bracketed epiphysis, which causes abnormal growth shape of the phalanx, often called a “delta phalanx”
62
Q

what are treatment considerations for clinodactyly?

A
  • if not functional impairment, no intervention required (often not required < 40’ angulation)
  • if functional impairment (> 40’), patient desire for cosmesis then consider surgery at skeletal maturity
    • osteotomy: closing, opening, rotational, reversed wedge
63
Q

list syndromes associated w ulnar polydactyly. what type of polydactyly is more commonly syndromic?

A
  • syndromic ulnar polydactyly more common w well-developed (vs. rudimentary)
  • associations: Laurence - Mood - Beidle, trisomy 13, orofacial digital syndrome
64
Q

what are operative principles of ulnar polydactyly treatment?

A
  • removal of extra digit
  • identification and preservation of proper digital n to normal digit
  • reconstruction/reblancing of soft tissues & skeleton: tendons, collaterals, excessively wide phalanx
65
Q

what is commonly associated w ulnar dimelia when inherited in AD fashion?

A

fibular dimelia

66
Q

what is aberration during embryology that occurs to produce ulnar dimelia?

A
  • abnormality w/ ZPA or interaction between AER and ZPA
67
Q

what is the commonest theory to support the development of isolated digital macrodactyly?

A
  • congenital abnormal innervation to digit
68
Q

List syndromes with macrodactyly as part of syndromic presentation

A
  • vascular malformations
    • Proteus syndrome
    • Klippel-Trenaunay-Weber
    • Maffucci
  • Other dysregulated tissue growth
    • Neurofibromatosis
    • Ollier’s disease
69
Q

how would you manage a patient presenting with single enlarged long finger?

A
  • history - key to rule out syndrome
  • physical - key to rule out other etiology (vascular / lymphatic malformation, NF, syndromes like proteus vs. isolated macrodactyly)
  • imaging - MRI to help rule in/out isolated vs. secondary to other etiology
  • treatment
    • nothing
    • await until growth arrest then assess
    • best options are ray amputation for isolated macrodactyly or terminal shortening; debulking procedures are not often successful; physeal arrest will result in continued circumferential growth.
70
Q

what are treatment considerations for congenital contstriction band syndrome?

A
  • superficial bands without distal edema/lypmhedema or concern for vascular compromise
    • treatment may be for cosmetic purposes
    • can wait until child is older
    • staged hemi-circumferential z-plasty technique
  • deep bands with distal edema/lymphedema, vascular insufficiency, skeletal fusion
    • treatment is urgent/emergent in some cases
    • still employ staged hemi-circumferential z-plasty technique w excision of tight constriction band
    • release acrosyndactyly following principles for syndactyly release, staging release of digits with bilateral involvement
71
Q

what are the theories of development of constriction band syndrome?

A
  • intrinsic theory - older theory
    • intrinsic defect of mesodermal tissue
  • extrinsic theory - more widely accepted theory currently
    • extrinsic effect of constriction of amniotic bands/adhesions formed in utero around the limb
72
Q

what is madelung’s deformity?

A
  • most often it is a sporadic x-linked gene mutation
  • growth disturbance to the volar/ulnar distal radius physis causes characteristic deformity of distal radius (apex dorso-radial angulation) and correpsonding deformity of dorsal subluxation of ulna / volar subluxation of carpus
73
Q

what is vicker’s ligament?

A
  • vicker’s ligament is a pathologic band between the lunate and volar/ulnar distal radius that is thought to be the pathological finding that results in abnormal distal radius physis in Madelung’s deformity
74
Q

what are the clinical findings in Madelung’s deformity?

A
  • distal radius
    • apex dorso-radial angulation
    • excessive radial inclination, volar tilt
  • carpus
    • volar subluxation
  • ulna
    • dorsal subluxation
  • Associated complaints / findings
    • carpal tunnel syndrome / compression of median nerve at carpal tunnel
    • attrition rupture of extensor tendons (EDC)
    • pain
    • limited range of motion
    • ulnar abutment syndrome
75
Q

what are treatment options for Madelung’s deformity?

A
  • corrective surgery at skeletal maturity:
    • DRUJ arthrodesis
    • partial / total radiocarpal arthrodesis
    • Joint leveling procedures: dorsal wedge osteotomy
    • distraction osteogenesis
76
Q
A

Upper Extremity (27 decks)

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