Hand and Wrist Surgery - part 9 pptx

Inthe index finger, the medullary canal of the proximal phalanx requires the rectan-gular reaming to be performed in an orientation of slight rotation such that thedorsal ulnar corner is

Rheumatoid Arthritis:

Metacarpophalangeal Joint Reconstruction Arthroplasty

R John Naranja, Jr and Kevin D Plancher

History and Clinical Presentation

A 53-year-old right hand dominant woman was originally diagnosed with toid arthritis 14 years ago Medical management of her disease process has includedchronic oral prednisone Her primary complaint includes pain and deformity of herhands and decreased function secondary to lack of mobility and strength

rheuma-Physical Examination

The patient has ~25 degrees of ulnar drift at the index through small finger carpophalangeal (MP) joints An extension deficit at the MP measures ~50 degrees(Fig 73–1) The arc of motion of the digits at the MP joint is 20 degrees Key pinchstrength is 30 N and grip strength is 70 N at position II using the Jamar dynamo-meter There is relative preservation of joint motion at the proximal interphalangeal(PIP) joint and wrist articulations without significant collapse or deformity

dif-462

PEARLS

• Meticulous detail is necessary

when reaming the proximal

phalanx of the little finger

when there is poor bone stock

and especially in patients with

juvenile rheumatoid arthritis

• When silicone synovitis and loss

of bone stock occur, a

resec-tion arthroplasty should be

considered

PITFALLS

• Inadequate soft tissue

balanc-ing and poor hand therapy

can lead to recurrence of the

deformities and subluxation of

of The Indiana Hand Center and Gary Schnitz.)

Differential Diagnosis

Degenerative joint diseaseRheumatoid arthritisSeptic joints

MP joint predictably addresses these processes and maintains function better thanarthrodesis or simple resectional arthroplasty in patients with adequate bone stock,soft tissue coverage, and absence of preexisting infection

R H E U M A T O I D A R T H R I T I S : M E T A C A R P O P H A L A N G E A L J O I N T R E C O N S T R U C T I O N A R T H R O P L A S T Y

Figure 73–2 Posteroanterior (PA) radiograph of metacarpopha- langeal (MP) subluxation and joint destruction.

Metacarpophalangeal destruction and deformity are often accompanied by PIPjoint deformity and wrist collapse Timing of surgical interventions as well as consid-eration of other joint involvement (e.g., hip and knee involvement/shoulder and el-bow involvement) requiring further load bearing on the diseased upper extremityfollowing surgery must be noted With associated wrist involvement, reconstruction

of the wrist followed by the MP joint is appropriate The exception is in the face of anextensor tendon rupture with decreased passive MP range of motion Metacarpopha-langeal arthroplasty should be performed first to gain passive motion in associationwith the extensor tendon repairs at the level of the wrist

Surgical Management

A transverse or longitudinal skin incision is created dorsally over the region of themetacarpal necks The dorsal veins are preserved and the extensor hoods to each digitare exposed with care to protect the neurovascular structures radially and ulnarly.The extensor tendon is identified and is usually subluxed ulnarly with attenuation ofthe extensor tendon at its radial aspect (Fig 73–3) The extensor hood is longitudi-nally incised ulnar to the common extensor tendon in the middle and ring fingers,and between the common extensor and the ulnar capsular ligament of each digit As-sociated capsular tissue is released distally to adequately allow the base of the proxi-mal phalanx to be dislocated dorsal to the metacarpal The radial collateral ligament

of each digit is preserved if possible With the MP joint placed into flexion, themetacarpal neck is exposed subperiosteally and transversely osteotomized using a mi-croair saw (Fig 73–4) Care is taken to leave part of the metaphyseal flare for support

of the prosthesis Proliferative synovium along with the transected metacarpal head iscompletely removed (Fig 73–5) Attention is then directed toward the base of theproximal phalanx where all cartilaginous surfaces are removed in addition to anymarginal osteophytes The volar plate is identified and released from its attachment

at the base of the proximal phalanx In all but the index finger it is then completelyresected In the index finger it will be used in conjunction with the radial collateralligament for later capsular reconstruction Release and resection of the volar plate al-low further identification of the underlying flexor sheath An incision is made intothe flexor sheath with subsequent delivery of the flexor tendon into the wound using

a blunt hook Hypertrophic tenosynovium is then removed Evaluation of ate tendon excursion following the tenosynovectomy will determine whether flexortendon exploration on the palmar aspect of the hand over the A1 and A2 pulleys is

necessary This is typically done using a palmar/digital zigzag-type of incision withcare to preserve the A1 and A2 pulleys in the index and long fingers to avoid furtherulnar subluxation of these tendons The ulnar intrinsic tendon in all but the indexfinger is also delivered into the wound and incised at its myotendinous junction typ-ically at the level of the MP joint In the index finger, preservation of the ulnar in-trinsic tendon is recommended to help maintain supination in this digit for pinchingfunction This pinching function is further reinforced by reconstructing the radialcollateral ligament of the index and long finger by using the collateral ligaments andcapsule The ulnar collateral ligament, which has previously been divided at its inser-tion at the proximal phalanx, is now further mobilized by dissection proximally onthe metacarpal, with care taken to maintain its attachment Similarly, the previouslypreserved radial collateral ligament is dissected as a unit with the overlying capsulefrom its distal insertion These capsular tissues may be tagged and retracted for laterreconstruction using a 2–0 Dacron suture.

With regard to the small digit MP joint, the abductor digits minimi tendon is lated and sectioned with care to protect the ulnar neurovascular structures Theproximal end of the abductor digiti minimi tendon is then attached to the flexordigiti minimi tendon immediately volar in position to the abductor digiti minimi.The intramedullary canal of the metacarpal and proximal phalanx is thenreamed in a rectangular fashion centrally in the canal using a combination of

curette, broach, and microair drill with a smooth leader point bur (Fig 73–6) Inthe index finger, the medullary canal of the proximal phalanx requires the rectan-gular reaming to be performed in an orientation of slight rotation such that thedorsal ulnar corner is more dorsal than the dorsal radial corner, and the radialpalmar corner is volar to the ulnar palmar corner of the rectangle when viewedaxially This will favor slight supination to help the index finger function insupination In the small finger proximal phalanx, the reverse is true and the rec-tangular reaming should place the dorsal radial corner more dorsal than thedorsal ulnar corner and the ulnar palmar corner is volar to the radial palmar cor-ner This will favor the slight pronation required for the small digit Care must

be taken to avoid perforation of the cortex Rough edges are flattened carefullyand trial implants are placed for sizing Evaluation of proper fit should includeplacing the MP joint into full extension with no demonstrable impingement orinstability Tension may be further modified with soft tissue releases or bony re-section The largest possible implant should be used with good fit of the steminto the canal and appropriate apposition of the midsection with bone (Fig.73–7) With good bone stock, a grommet may also be used and press-fit into theintramedullary canals with the midsection of the implant resting on the grom-met Following placement of the implant, residual laxity of the index and occa-sionally the long finger joint can be addressed by imbricating the radial collateral

ap-to prepare the canal (Illustration courtesy of The Indiana Hand Center and Gary Schnitz.)

ligament and capsule over the dorsal aspect of the base of the proximal phalanx

at the dorsoradial border through a 1-mm drill hole (Fig 73–8) Proximal tachment to the metacarpal may also be necessary If the radial collateral liga-ment has become too attenuated for the reconstruction, a proximally based flapusing the radial half of the volar plate may be incorporated and similarly sutured

reat-to the dorsoradial aspect of the proximal phalanx The distally released ulnar lateral ligament is then sutured to the ulnar edge of the radial collateral ligamentand capsule The extensor hood is repaired using 4–0 Dexon sutures and invert-ing the knots The radial portion of the extensor hood is reefed to centralize theextensor tendon with care to bury the knots In the index and long fingers, over-correction of the extensor tendons radially assists in avoiding a pronation force

col-In some cases, if the extensor tendon does not have adequate longitudinal sion, it requires imbrication The juncturae tendinum are repaired and the skin isclosed with interrupted 5–0 nylon sutures, followed by application of a volumi-nous hand-conforming dressing

ten-R H E U M A T O I D A ten-R T H ten-R I T I S : M E T A C A ten-R P O P H A L A N G E A L J O I N T ten-R E C O N S T ten-R U C T I O N A ten-R T H ten-R O P L A S T Y

Figure 73–7 Implant is placed in the canal with the best fit and appropriate position with bone (Illustra- tion courtesy of The Indiana Hand Center and Gary Schnitz.)

Figure 73–8 Implant in place with imbrication of the radial/collateral liga- ment added for stability (Illustration courtesy of The Indiana Hand Center and Gary Schnitz.)

Dressings are changed 2 days postoperatively and the patient is fitted for a dynamicextension splint to be worn during the day and a resting splint to be used at night Anoccupational therapist is helpful in maintaining proper alignment of the digits duringactive and active-assisted range of motion A passive range-of-motion goal of 0 to 70degrees in the ring and small finger and 0 to 45 degrees in the index and long finger isachievable by approximately 2 weeks Further aggressive range of motion, articularly inthe index and long fingers, is not necessary to promote stability and durability of theimplant in these digits Care must be taken to avoid contracture in other joints, specif-ically the PIP, elbow, and shoulder This can be avoided by dorsal extension splints ofthe PIP to be used during exercise, and concomitant range-of-motion exercises directed

at the elbow and shoulder Therapy is required for a minimum of 3 months

Complications

Rheumatoid patients with a history of being treated with steroids are at risk for the velopment of infection, wound healing difficulties, and complications related to poorbone stock such as fractures Recurrence of deformity with subluxation of the joint orextensor tendons can usually be traced back to insufficient soft tissue balancing and/orinadequate postoperative bracing and therapy Further complications related to im-plant wear can result in silicone particles inciting an inflammatory response and sub-sequent erosive changes Salvage procedures for complications related to MP jointarthroplasty typically involve revision capsuloligamentous reconstruction in cases of in-stability, or implant replacement in the event of implant failure or fracture With moreserious complications such as deep infection or bony erosion and loss of bone stock sec-ondary to silicone synovitis, simple resection arthroplasty is a reasonable option

de-Acknowledgments

This chapter is dedicated to James W Strickland who is always willing to share hiswisdom through years of experience

Suggested Readings

Beckenbaugh RD, Dobyns JH, Linscheid RL, Bryan RS Review and analysis of

silicone-rubber metacarpophalangeal implants J Bone Joint Surg [Am] 1976;58A: 483–487.

Bieber EJ, Weiland AJ, Volenec-Dowling S Silicone-rubber implant arthroplasty

of the metacarpophalangeal joints for rheumatoid arthritis J Bone Joint Surg [Am]

1986;68A:206–209

Kirschenbaum D, Schneider LH, Adams DC, Cody RP Arthroplasty of the pophalangeal joints with use of silicone-rubber implants in patients who have rheuma-

metacar-toid arthritis: long-term results J Bone Joint Surg [Am] 1993;75A:3–12.

Millender LH, Nalebuff EA Metacarpophalangeal joint arthroplasty utilizing the

silicone rubber prosthesis Orthop Clin North Am 1973;4:349–371.

Nalebuff EA Surgical treatment of finger deformities in the rheumatoid hand Surg

Clin North Am 1969;49:833–846.

Swanson AB Flexible implant arthroplasty for arthritic finger joints Rationale,

technique, and results of treatment J Bone Joint Surg [Am] 1972;54A:435–455.

468

Section XII

Congenital Anomalies

of the Hand

Short Below-Elbow Amputation

Robert T Ciocco and Scott H Kozin

Transverse Deficiency of the Digits

Scott H Kozin

Hypoplastic Digits and Hands

Scott H Kozin

Reconstruction of a Type II Hypoplastic Thumb

Allan E Peljovich and Peter M Waters

C Failure of Differentiation

Congenital Radioulnar Synostosis

Allan E Peljovich and Peter M Waters

D Duplication of Parts Polydactyly and Bifid Thumb

Kevin D Plancher

E Overgrowth Macrodactyly

Ann E Van Heest and James House

74 Short Below-Elbow Amputation

Roberta T Ciocco and Scott H Kozin

History and Clinical Presentation

A 4-month-old girl presented with congenital deficiency of the right upper tremity (Fig 74–1) She was born 2 weeks early by cesarean section secondary tolow amniotic fluid The child presented with no other medical or developmentalproblems There was no family history of congenital anomalies, including her twosisters

ex-Physical Examination

The patient was a healthy young infant with partial amputation of the right upperextremity The shoulder and elbow had full active and passive range of motion Theproximal third of the forearm was present with the remaining extremity absent.The residual stump was well padded and there were several small nubbins at theend The other extremities were normal and no amniotic bands were appreciated

S H O R T B E L O W - E L B O W A M P U T A T I O N

PEARLS

• Transverse deficiencies of the

upper extremity occur most

common just below the elbow

• Transverse deficiencies are

usually unilateral, sporadic in

occurrence, and rarely

associ-ated with other anomalies.

• Early fitting (before 2 years

of age) increases the

accep-tance rate of upper limb

prosthetics

PITFALLS

• Surgery usually is not necessary

for short, below-the-elbow

deficiencies

• Limb lengthening of forearm

transverse deficiencies is

usu-ally not indicated.

Figure 74–1 (A) A 4-month-old child with congenital anomaly of the right upper ity (B) Normal-appearing shoulder, elbow, and proximal forearm and absence of remaining extremity.

extrem-Diagnostic Imaging

Radiographs of the right upper extremity demonstrated a proximal forearm with ashortened and dysplastic radius and ulna (Fig 74–2) There was no evidence of os-seous development of the remaining forearm, wrist, or hand structures

Differential Diagnosis

Amniotic band syndromeCongenital transverse deficiencyRadial deficiency

Intrauterine necrosis of the right forearmAmniotic band or constriction band syndrome is a result of entrapment of de-veloping embryonal tissue by the fetal lining This can manifest as amputation

of a part and most commonly affects the digits (hands or toes) The diagnosis

of constriction band requires the presence of a constriction band either in theinvolved extremity or elsewhere Radial clubhand is a longitudinal deficiency ofthe preaxial border of the upper extremity The radius can be partially or com-pletely absent and the wrist is positioned in radial deviation The ulna is present,although shortened compared with normal Radial clubhand is often associated

472

Figure 74–2 Radiograph of right arm with absence of the distal two-thirds forearm, wrist, and digits.

with visceral abnormalities (e.g., cardiac or genitourinary) Intrauterine necrosis

of the forearm is a rare abnormality characterized by varying degrees of necrosis

of the upper extremity evident at birth The clinical manifestations range fromminor areas of skin and muscle damage to mummification of the entire forearm.The etiology is either direct pressure on the affected limb or thromboembolicdisease

Diagnosis

Congenital Transverse Deficiency

Congenital transverse deficiency is a failure of formation and defined according

to the last remaining bone segment A short below the elbow type amputation isthe most common transverse deficiency of the upper extremity The stump is usu-ally well cushioned, and rudimentary nubbins or dimpling can be found on the end.These anomalies are usually unilateral, sporadic in occurrence, and rarely associatedwith other anomalies

Nonsurgical Management

Initially, the most important intervention is to provide the family with accurateinformation and resources to help parents emotionally cope with their child’sdisability Most often the initial prosthetic intervention is to fit the child with ei-ther a passive hand or spring-loaded terminal device prosthesis at the age whenindependent sitting is achieved, usually around 7 to 9 months of age (Fig 74–3).There are some facilities that begin fitting early, at 3 to 4 months of age Studieshave shown that early fitting (before 2 years of age) increases the acceptance rate

of upper limb prosthetics It is equally important to involve the family as much

as possible in the decision-making process when considering prosthetics Familyacceptance and support of the device are probably the other crucial variables thatpositively influence prosthetic acceptance At ~15 months to 2 years of age,the child is usually transitioned to some type of body-powered prosthesis This isthe time developmentally that the child can begin to follow directions and can

S H O R T B E L O W - E L B O W A M P U T A T I O N

Figure 74–3 A variety of passive hand prostheses used when independent sitting is achieved.

utilize cause-and-effect reasoning necessary for prosthetic training For the short,below-the-elbow transverse deficiency, a supracondylar socket, figure-of-9 har-ness, and either a voluntary closing or voluntary opening terminal device is theprosthesis of choice (Fig 74–4) (Table 74–1) There are some proponents ofgoing directly to a myoelectric single site (cookie crusher) system at this time.This single-site system allows control over opening by contraction of the controlmuscle, and closing is achieved automatically upon relaxation of the muscle.However, our philosophy is to wait until the child is 3 to 4 years of age beforeintroducing myoelectrics The benefit of waiting is that it allows the child andfamily to gain experience with a conventional prosthesis, which may be the pre-ferred prosthesis for certain activities in adolescence and adulthood In addi-tion, a conventional prosthesis provides more proprioceptive feedback and is lesslikely to accidentally pinch the user or other children At this time, it is also rec-ommended that the child begin receiving formal prosthetic training on an outpa-tient basis, regardless of the type of prosthesis prescribed.

At 5 years of age, the child is considered for a dual-site myoelectric hand (Fig.74–5) By this age, most children are able to isolate the muscle contractions neces-sary to operate this type of system The benefit of the dual-site hand is that it allowsthe child full control over both grasp and release of objects, whereas the single-site cookie crusher allows only control over opening Formal therapy for prosthetictraining is recommended at the time of prosthetic change to ensure the child gainsthe full benefit of the prosthesis During the child’s developmental years, prostheticmonitoring is necessary as the child’s needs and expectations change It is important

474

Figure 74–4 A supracondylar socket, figure-of-9 harness, and voluntary closing terminal device body-powered prosthesis used at

~15 months to 2 years of age.

Table 74–1 Prosthesis Choices for Below-the-Elbow Transverse Deficiency Type of TD Description Age Range Pros Cons

TD, terminal device; CAPP, child amputee prosthetic project; ADEPT, anatomically designed and engineered polymer technology.

Passive light-weight mitt orfoam-filled hand usuallyattached to self-suspendingsocket and can be a fisted oropen hand

A terminal device attached to asocket that does not have anytype of cable

Traditional voluntary openingTD; uses rubber bands forclosing tension; controlled by

a cable that is activated by thecontralateral shoulderVoluntary closing TD that wasdesigned to give more control

of grasp force; has shape thatallows easy grasp of cylindri-cal objects; same as ADEPTbut modified to resemblehand

Voluntary opening TD that wasdesigned for toddlers andpreschool children; shapedlike a lobster claw; closingforce achieved by springmechanism

Cable-driven hand can be eithervoluntary opening or closingand has a cosmetic glove;

currently research in progress

to develop a more efficientpediatric mechanical hand

Single-site or dual-site system;

battery-operated motorizedhand that is activated bymuscle contraction; grasp isachieved with thumb and firsttwo fingers

Various options for sports orspecial hobbies, e.g., guitarpick, golf, fishing, baseball

3–12 mos

12–24 mos

15 mos andolder

3 yrs andolder

15 mos to

5 years

3 yrs andolder

18 mos to

5 years forsingle site;

5 yrs andolder fordual site

When plicable

ap-Very cosmetic; helps toprovide balance andestablish wearingtolerance; gives childbetter weight-bearingsurface for crawlingAllows passive grasp usu-ally by parent placingobjects into TDProvides good vision ofobjects to be grasped;

easy to operate; goodfor picking up smallobjects

Provides good vision ofobjects to be grasped;

gives more ceptive feedback touser; more controlover grasp force; morecosmetic than hook;

proprio-life touch is designed

to look like a handHas good gripping sur-face; some parents feel

it is more cosmeticthan hook; easy tooperate; consideredsafer than hookCosmetic

Cosmetic; no harness orcable; strong efficientgrasp

Provides option for cialized tasks; quickdisconnect makesinterchanging easy

spe-Functionally very limiting

Less cosmetic

Less cosmetic; limited ing force, objects tend toslip out

hold-Sometimes more difficult

to train and use; propercabling crucial to efficientoperation

Vision of small objects ismore occluded

Force required to open orclose hand is significantlygreater, therefore moredifficult to operate; vision

of object to be grasped isusually partially or com-pletely occluded; cosmeticgloves are not durableCosmetic gloves are notdurable; slightly increasedweight; batteries need to

be charged; not mended for use aroundsand, dirt, or water; par-tial visual obstruction;sometimes makes noisewhen activatedCould be costly; slightlyinconvenient to have tointerchange units

recom-to consider all of the following facrecom-tors along the way and recom-to help the family make adecision that best fits the child’s overall situation:

1 Family values

2 Child’s lifestyle and activity preferences

3 Financial resources

4 Physical findings (i.e., skin condition, muscle function, limb shape, and length)

5 Prosthetic history (wearing time, care, and maintenance)

6 Social emotional status

7 Developmental skillsHaving a team of professionals, which includes a physician, prosthetist, occupa-tional or physical therapist, social worker, and nurse, can help the child and familysort out many of these issues and make informed decisions

irritate the residual stump Excision of the bone or removal of the nubbins can viate this problem.

alle-The use of limb lengthening for transverse deficiencies is controversial At thislevel, an increase in limb length would not eliminate the use of a prosthesis, and theexpected gains do not outweigh the procedural risks

The Krukenberg procedure converts the forearm into a pincher apparatus by ing the radius and ulna to function independently This procedure is reserved for bi-lateral amputee patients with long forearms and would not be appropriate for thispatient In addition, this operation is usually recommended in the sight-deficient pa-tient or in impoverished countries where prosthetic use is not practicable

allow-The advent of microsurgical transfer of parts has added an additional option totreat the congenitally deficient limb The microvascular transfer of a toe from thefoot to the residual limb is feasible if the deficiency occurred at the wrist level How-ever, a transfer to the elbow area would not be beneficial with regards to function

Suggested Readings

Armstrong AP, Page RE Intrauterine vascular deficiency of the upper limb J Hand

Surg [Br] 1997;22B:607–611.

Bora FW Jr, Nicholson JT, Cheema HM Radial meromelia The deformity and its

treatment J Bone Joint Surg [Am] 1970;52A:966–979.

Brenner C Electronic limbs for infants and pre-school children J Prosthet Orthotics

1991;4:184–190

Clark S, Patton J Occupational therapy for the limb deficient child Clin Orthop

1980;148:93–100

Crone N A comparison of myo-electric and standard prostheses—a case study of a

pre-school aged congenital amputee Can J Occup Ther 1986;53:217–222.

Gover A, McIvor J Upper limb deficiencies in infants and young children Inf Young

Children 1992;5:57–71.

Hubbard S, Kutz I, Naumann S Myoelectric prostheses for the limb-deficient

child Phys Med Rehabil Clin North Am 1991;2:847–865.

Kozin SH, Thoder JJ Congenital anomalies of the upper extremity In: Baratz ME,

Watson AD, Imbriglia JE, eds Orthopaedic Surgery: The Essentials New York: Thieme;

1999:657–673

Marquardt EG A holistic approach to rehabilitation for the limb deficient child

Arch Phys Med Rehabil 1983;64:237–242.

Nathan P, Trung N The Krukenberg operation: a modified technique avoiding skin

grafts J Hand Surg [Am] 1977;2:127–130.

Scotland TR, Galaway HR A long term review of children with congenital and

ac-quired upper limb deficiency J Bone Joint Surg [Br] 1983;65B:346–349.

Vilkki SK Advances in microsurgical reconstruction of the congenitally adactylous

hand Clin Orthop 1995;314:45–58.

S H O R T B E L O W - E L B O W A M P U T A T I O N

75 Transverse Deficiency of the Digits

Scott H Kozin

History and Clinical Presentation

A 10-month-old boy presented with a congenital anomaly of the right hand (Fig 75–1)

He is the product of a full-term normal vaginal delivery There were no complicationsduring pregnancy or delivery The child uses the right hand for bimanual activity and isotherwise healthy Developmental milestones have been reached at the appropriate time

He is an only child, and there is no family history of congenital anomalies

Physical Examination

A healthy-appearing infant with an isolated anomaly of the right upper extremity.The right shoulder, elbow, forearm, and wrist appear normal and equal in size whencompared with the left side The right hand is abnormal with absence of the thumb,

478

PEARLS

• Transverse deficiencies of the

digits are most common at the

phalangeal level.

• Deficient hands with intact

metacarpophalangeal joints

and small proximal phalanges

are very adept at function

• Nonvascularized toe

phalan-geal transfer requires

extra-periosteal dissection

PITFALLS

• Nonvascularized toe

phalan-geal transfer does not result in

prolonged growth

• Distraction osteogenesis

re-quires frequent monitoring to

avoid potential complications

• Toe-to-hand transfer is difficult

in adactyly and may require

multiple toe transfers

Figure 75–1 A old with congenital anomaly

10-month-of the right hand.

index, long, ring, and small digits (Fig 75–2) There are palpable metacarpals, but

no phalanges appreciated There is excellent skin coverage and skin tubes are presentwhere the phalanges would be expected No amniotic bands or chest wall abnormal-ities were noted

Diagnostic Imaging

Radiograph of the right hand demonstrated five metacarpals with absent phalanges(Fig 75–3) A comparison radiograph of the left hand revealed five metacarpals andnormal phalanges

Differential Diagnosis

BrachydactylyPoland syndromeTransverse deficiency of the digitsConstriction band syndromeBrachydactyly refers to a short hypoplastic digit that has the normal complement

of bones The index and small fingers are most commonly affected The shorteningmost commonly involves the middle phalanx, which is the last phalanx to ossify.Poland syndrome combines hand hypoplasia with chest wall abnormalities and isusually sporadic in occurrence The most common hand anomaly is symbrachy-dactyly (syndactyly combined with brachydactyly), and the typical chest abnormal-ity is ipsilateral absence of the sternocostal portion of the pectoralis major muscle.Constriction band syndrome is a result of entrapment of developing embryonal tis-sue by fetal lining Mild band formation can cause simple constriction rings that areprimarily a cosmetic deformity, whereas severe bands can cause autoamputation ofthe digits As in transverse deficiency of the digits, the thumb is often spared Thediagnosis of constriction band requires the presence of a constriction band either inthe hand or elsewhere

Diagnosis

Transverse Deficiency of the Digits

Transverse deficiency of the digits is most common at the phalangeal level withcomplete absence of the phalanges (aphalangia) or presence of only the proximalphalanx bases There may or may not be nail remnant and vestigial tendons, andrudimentary neurovascular structures The central digits are most commonly defi-cient and the thumb is often spared The incidence of transverse deficiencies in theupper extremity is ~1.5 in 10,000 births, which includes transverse deficiencies atthe digits level

Nonsurgical Management

The initial treatment for transverse deficiencies of the digits is nonoperative Theparents are often devastated and require support and education Parents often feelguilty about this anomaly and have lots of questions A team that consists of aphysician, therapist, and social worker often provides sufficient information forthe family We attempt to explain to the parents that transverse deficiencies areusually sporadic in occurrence and not associated with other syndromes Thisdefect most likely affected the limb bud covering (apical ectodermal ridge) earlyduring pregnancy (between 4 and 8 weeks of gestation) We also stress that theparents attempt to treat the child as normal as possible, as the child does notknow he or she is different In addition, the child will develop many adaptive ma-neuvers to achieve a variety of activities These manipulations are necessary forindependent function and are part of normal development in the child with atransverse deficiency

480

Surgical Management

Hands with transverse deficiencies through the carpus and absent metacarpals arenot amenable to surgical correction The remaining carpus allows wrist range of mo-tion, and the extremity is used primarily as a helper for bimanual activity Prostheticfitting is an option in these children, although the acceptance rate is low because

of the adequate limb length, intact sensation, and available wrist motion Adaptiveequipment can be used to supplement hand function for a particular task that cannot

be accomplished (e.g., tying shoes, playing musical instruments)

Deficient hands with short proximal phalanges and intact metacarpophalangealjoints have good potential for function, as the shortened digits can be used for crudegrasp and have normal sensibility These hands have the potential for surgical recon-struction aimed at improving length There are multiple methods to increase digi-tal length including web space deepening, bone grafting, on-top plasty, distractionosteogenesis, or toe-to-hand transfer Web deepening is indicated when there is

a combination of syndactyly and digital deficiencies The commissure should beplaced as proximal as possible to achieve a relative lengthening of the separated dig-its Intercalary bone grafting and on-top plasty are most applicable during firstweb space reconstruction (see Case 79, Hypoplastic Digits and Hands) An indexmetacarpal can be excised during first web space reconstruction and used as bonegraft to increase length of the thumb or long finger In addition, an index proximalphalanx can be harvested as a neurovascular flap and transferred to the tip of thethumb to augment length and function (Fig 75–4)

Distraction osteogenesis can be applied to these hands, but there is little ance for error The small size of the phalanges and technicalities of external fixationapplication are important preoperative considerations Distraction lengthening isusually reserved until the bone is large enough to accommodate the fixator deviceand the child old enough to comprehend the external fixator The distraction is per-formed slowly at 1 mm per day with careful pin and soft tissue monitoring Therapy

toler-is required to prevent contracture formation, and weekly radiographs are necessary

to assess callus formation and consolidation Distraction osteogenesis can doublethe lengthened segment, but the regenerate bone is prone to fracture, and the exter-nal fixator must remain in place for three times the duration of the lengthening toallow for bony consolidation

Transverse deficiency without phalanges is more difficult to manage surgically asthe options for treatment diminish Lengthening of the metacarpals may provide a

T R A N S V E R S E D E F I C I E N C Y O F T H E D I G I T S

Figure 75–4 First web space reconstruction and neuro- vascular harvest of an index proximal phalanx in prepara- tion for thumb on-top plasty.

pinch post in hands with an adequate thumb (Fig 75–5) However, metacarpallengthening does not result in motion and is contraindicated when there are otherdigits that possess good motion In these cases, a long stiff finger hinders hand func-tion because it interferes with the mobile digits Nonvascularized toe phalangealtransfer is an option in hands without phalanges (aphalangia) and can use any ofthe toes as a donor The donor phalanx is harvested in an extraperiosteal fashion(Fig 75–6) The phalanx is removed along with the metacarpophalangeal collateral

ligaments and a portion of the volar plate The toe phalanx is placed within the softtissue sleeve of the recipient finger An adequate skin tube must be present to pre-vent disruption of blood supply to the skin and subsequent tip necrosis The collat-eral ligaments are sutured to the metacarpal and any vestigial tendons are sutured tothe transferred phalanx The transferred phalanx and the donor toe are pinned with

a 0.035- or 0.045-inch longitudinal Kirschner wire (K wire) Both the lower andupper extremities are placed in a cast with the pins covered The foot is placed in awalking cast and the pin removed between 3 and 4 weeks after surgery The trans-ferred phalanx K wire is maintained for 10 to 12 weeks time This technique addsadditional bone and creates a metacarpophalangeal joint However, the ensuinggrowth of the transferred phalanx is limited with mild growth only for a few years.Toe-to-hand transfer is another option for the transverse deficiency without pha-langes A portion of a metacarpal ray must be available to accept the transferreddigit and multiple toe transfers may be necessary in the adactylous hand The desir-able time for toe transfers is between 2 and 4 years of age Family counseling is en-couraged, and discussion with treated patients helpful This procedure has severaladvantages but is not without inherent risks The microsurgical transfer of a toesupplies not only added digital length but also additional functional joints Thetransferred toe has the added benefit of continued growth and the possibility of nor-mal sensation The disadvantages include the technical demands of the procedureand the potential loss of the transferred toe

Suggested Readings

Flatt AE The Care of Congenital Hand Anomalies, 2nd ed St Louis: Quality Medical

Publishing; 1994:149–179

Goldberg NH, Watson HK Composite toe (phalanx and epiphysis) transfers in the

reconstruction of the aphalangic hand J Hand Surg [Am] 1982;7:454–459.

Ireland DC, Takayama N, Flatt AE Poland’s syndrome A review of forty-three cases

J Bone Joint Surg [Am] 1976;58A:52–58.

James MA, Durkin RC Nonvascularized toe proximal phalanx transfers in the

treat-ment of aphalangia Hand Clin 1998;14:1–15.

Kay SP, Wiberg M Toe to hand transfer in children Part 1: technical aspects J Hand

Surg [Br] 1996;21B:723–734.

Kay SP, Wiberg M, Bellow M, Webb F Toe to hand transfer in children Part 2:

Functional and psychological aspects J Hand Surg [Br] 1996;21B:735–745.

Kozin SH, Thoder JJ Congenital anomalies of the upper extremity In: Baratz ME,

Watson AD, Imbriglia JE, eds Orthopaedic Surgery: The Essentials New York: Thieme;

Vilkki SK Advances in microsurgical reconstruction of the congenitally adactylous

hand Clin Orthop 1995;314:45–58.

T R A N S V E R S E D E F I C I E N C Y O F T H E D I G I T S

Alexander D Mih

History and Clinical Presentation

A 4-month-old girl was seen for evaluation of right upper extremity deformity Shewas the product of a full-term delivery to a 30-year-old mother The mother recalls

no specific problems with pregnancy or medication requirement There was no ily history of orthopaedic problems The patient’s history included a known ventric-ular septal defect

fam-Physical Examination

The patient’s right upper extremity was remarkable for an elbow with a fixed tracture at 20 degrees, a shortened right forearm compared with the uninvolved op-posite side, severe radial deviation at the level of the wrist, and absent thumb

con-No passive motion of the elbow was present The wrist was passively correctable

to neutral in the radioulnar plane as well as to 10 degrees extension in the posterior plane

• Partial carpal bone excision

to achieve wrist reduction is

preferable to excessive

pres-sure on the distal ulnar physis

In some cases the lunate may

require complete excision

Complete radial-side soft

tis-sue release is necessary for

reduction The radial-side

Z-plasty may be required in

cases of inadequate skin, with

redundant skin excised from

the ulnar side of the wrist

PITFALLS

• Although some authors have

not recommended wrist

recon-struction in these patients, it

is possible to achieve elbow

flexion through triceps

length-ening and capsulectomy Pins

should be cut off beneath the

skin to allow more prolonged

retention, and ulnar osteotomy

may be required in cases of

find-Radial longitudinal deficiency is seen in ~1 in 50,000 births This condition is seenmore often in males than females and is bilateral in almost 50% of the cases Classifi-cation of this deformity is as follows: type I, short distal radius; type II, hypoplastic ra-dius; type III, partial absence of radius; type IV, complete absence of radius The type

IV deficiency is the most common Radial longitudinal deficiency occurs frequently

in association with other malformations involving the cardiac, gastrointestinal, monary, genitourinary, neurologic, and skeletal systems Syndromes that involve ra-dial longitudinal deficiency include Fanconi’s anemia, the thrombocytopenia absentradius syndrome, the Holt-Oram syndrome, and the VATER association (vertebraldefects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia)

pul-In this disorder, the preaxial muscles along the radial aspect of the forearm areseverely affected or absent A common flexor/extensor carpi radialis may be foundand provides a significant deforming force Although the ulnar artery is present, theradial artery is usually absent The radial nerve is also absent with an anomalousbranch of the median nerve often found to be a tethering structure when surgery isperformed

Surgical Management

At age 6 months the patient underwent surgery for correction of the elbow ture as well as wrist deformity With the patient supine on the operating table and thearm on a short-arm board, a general anesthetic was administered A sterile tourniquetwas utilized with elevation to 200 mm Hg

contrac-The elbow was approached through a straight midline incision directly over thetriceps The ulnar nerve was identified and retracted The triceps was Z lengthenedusing a step-cut technique and a posterior elbow capsulectomy was performed Thisallowed passive motion of the elbow to 120 degrees of flexion The triceps was thenreapproximated using nonabsorbable sutures

The wrist was approached through a gently curving incision, which allowed posure of the distal ulna A capsulotomy was performed to allow reduction of thecarpal bones over the end of the ulna under direct vision Radical radial soft tissuerelease was required The nonossified lunate could be reduced on the end of the ulnawithout pressure A conjoined flexor and extensor carpi radialis tendon was identi-fied and was detached from its insertion into the radial soft tissues and transferred

ex-to the ulnar side of the wrist, where it was sewn inex-to the extensor carpi ulnaris don A single 2.0-mm Steinmann pin was advanced retrograde through the end ofthe ulna and out the ulnar shaft It was then advanced antegrade into the indexmetacarpal to achieve radialization The Steinmann pin was cut off and advanced be-neath the skin but left prominent to allow for removal at a future date (Fig 76–2)

ten-Postoperative Management

The patient was placed in a long-arm cast with the elbow at 120 degrees of flexion.The cast was extended the entire length of the forearm to include the hand Immo-

Figure 76–2 (A) Postradialization anteroposterior radiograph (B) Lateral postradialization radiograph.

Alternative Methods of Management

Treatment of radial longitudinal deficiency may require multiple procedures In thecase presented, elbow release was combined with corrective surgery at the wrist andpollicization was performed 1 year later Centralization is performed by aligning thethird metacarpal axis with the long axis of the ulna and utilizing internal fixation forstability In the example given, radialization was performed and may allow a moreaggressive correction of the wrist deformity In more severe cases or in patients pre-senting at a later age, a partial carpectomy may be required to allow reduction of thecarpus onto the end of the ulna In all cases, excessive pressure must be avoided atthe level of the distal ulna to prevent growth arrest

Some authors have advocated the use of soft tissue stretching techniques usingexternal fixators prior to correction with either radialization or centralization Thiscan be of great benefit in allowing easier correction

Although recent reports have shown the promise of vascularized epiphyseal plateand metatarsophalangeal joint transfer, the long-term growth potential of this trans-ferred tissue is not known

Further reconstructive procedures such as pollicization are deferred until at least

1 year of age and may be combined with internal fixation removal

Suggested Readings

Bayne LG, Klug MS Long-term review of the surgical treatment of radial

deficien-cies J Hand Surg [Am] 1987;12A:169–179.

Buck-Gramcko D Radialization as a new treatment for radial club hand J Hand

Surg [Am] 1985;10A:964–968.

Kessler I Centralization of the radial club hand by gradual distraction J Hand Surg

[Br] 1989;14B:37–42.

Lamb DW Radial club hand A continuing study of sixty-eight patients with one

hundred and seventeen club hands J Bone Joint Surg [Am] 1977;59A:1–13.

Lamb DW, Scott H, Lam WL, Gillespie WJ, Hooper G Operative correction of radial

club hand A long-term follow-up centralization of the hand on the ulna J Hand Surg

1997;22B:533–536

Riordan DC Congenital absence of the radius, a 15-year follow up J Bone Joint

Surg [Am] 1963;45A:1783.

Watson HK, Beebe RD, Cruz NI A centralization procedure for radial club hand

Figure 76–4 (A) Recurrence of radial deviation in patient 2 years postsurgery (B) Flexion deformity recurrence in same patient.

Scott H Kozin

History and Clinical Presentation

A 5-month-old boy presented with congenital anomalies of both upper extremities(Fig 77–1) He is the product of a full-term pregnancy, born by cesarean sectiondelivery There were no complications during pregnancy or delivery The child isotherwise healthy and has attained his early developmental milestones at the appro-priate time There is no family history of congenital anomalies, including two sib-lings without abnormalities

Physical Examination

The patient is a healthy-appearing infant with congenital anomalies of both upper tremities His right extremity is longer than his left Both shoulders had full range ofmotion and strength Both elbows and forearms had no active or passive motion Theright elbow was fixed at 45 degrees of flexion and the forearm rigid in neutral rotation.The left elbow appeared to be hyperextended with the forearm facing backward and

ex-U L N A R D E F I C I E N C Y ( ex-U L N A R C L ex-U B H A N D )

PEARLS

• Ulnar deficiency is associated

with other musculoskeletal

abnormalities.

• Ulnar and radial-sided hand

anomalies can be present in

ulnar deficiency

• Progressive radial bowing may

indicate the presence of an

necessary for function

• Avoid surgery on pterygium

cubitale

Figure 77–2 Radiograph

of both upper extremities Right side has a fused elbow, hypoplastic-appearing ulna, and three-digit hand Left side has fused elbow, small proximal ulna remnant, and three-digit hand.

the hand resting on the flank The left forearm was fixed in 30 degrees of pronationand appeared foreshortened compared with the right

The right wrist had full flexion/extension with apparent absence of the ring andsmall rays and a three-digit hand (thumb, index, and long) (Fig 77–1B) Thesedigits appeared normal and possessed full motion with the ability to grasp andrelease The left wrist had 30 degrees of extension and 80 degrees of flexion Thishand also possessed three digits with apparent absence of the ring and small rays.The remaining digits were hypoplastic with less movement compared with theright, and there was symphalangism of the most ulnar finger proximal interpha-langeal joint Normal sensation and palpable radial and ulnar pulses were present

on both sides

Diagnostic Studies

Radiographs of the upper extremities at age 21⁄2 years indicated anomalies of bothsides (Fig 77–2) The right elbow was fused between the humerus and the radius.The ulna appeared slightly hypoplastic There was absence of the ring and small rayswith normal-appearing thumb, index, and long digits The left elbow was also fusedwith the forearm pointing backward There was absence of the ulna with a smallproximal segment remaining The right wrist demonstrated early ossification of thecarpus, whereas the left carpus showed delayed ossification A three-digit hand waspresent bilaterally

Differential Diagnosis

Ulnar deficiency (ulnar clubhand)Radial deficiency (radial clubhand)Arthrogryposis multiplex congenitaProximal radioulnar synostosisMultiple pterygium syndrome

Radial deficiency is more common and associated with visceral abnormalities(e.g., cardiac or genitourinary), whereas ulnar deficiency is linked with muscu-loskeletal anomalies that may involve the contralateral limb, spine, or lower extrem-ities (e.g., proximal focal femoral deficiencies, congenital amputations, or scoliosis)

In radial deficiency, the radius is often absent and the wrist unstable in severe radialdeviation, whereas in ulnar deficiency the ulna is frequently partially absent and thewrist stable in slight ulnar deviation Arthrogryposis multiplex congenita is a syn-drome of multiple joint contractures that are present at birth and may be diffuse inpresentation or isolated to the upper or lower extremity The skin is often waxy anddevoid of skin creases, and considerable muscle wasting is evident The typical up-per extremity posture is shoulder adduction and internal rotation, elbow extension,forearm pronation, wrist flexion, and ulnar deviation Radioulnar synostosis is atransverse fusion of the proximal radius and ulna that prevents forearm rotation,not flexion/extension The condition may be bilateral, but is not associated withhand deformities Pterygium cubitale is a syndrome of congenital webbing of theneck, axilla, knees, and digits In type III ulnar deficiency there may be pterygiumcubitale, but it is an isolated finding (Fig 77–3)