Phẫu Thuật Tạo Hình Grabb & Smith’s (Bản Cập Nhật – Kevin C. Chung)

Acknowledgments
The work was supported by a Midcareer Investigator Award in Patient-Oriented Research (2 K24-
AR053120-06) to Kevin C. Chung. The content is solely the responsibility of the authors and does not
necessarily represent the official views of the National Institutes of Health.
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References
Ellis, H. Richard von Volkmann: Volkmann’s ischaemic contracture. J Perioper Pract. 2012;22(10):338-339.
von Volkmann, R. Ischaemic muscle paralyses and contractures. 1881. Clin Orthop Relat Res. 2007;456:20-21.
Hildebrand, O. Die Lehre von den ischamische Muskellahmungen und Kontrakturen. Samml Klin Vortrage. 1906;122:559-584.
Bardenheuer, L. Die ischamische Kontraktur und Gangran als Folge der Arterien-verletzung. Leuthold’s Gedenkschr. 1906;2:87-131.
Jepson, PN. Ischaemic contracture: experimental study. Ann Surg. 1926;84(6):785-795.
Griffiths, D. Volkmann’s ischaemic contracture. Br J Surg. 1940;28:239-260.
Bunnell, S, Doherty, EW, Curtis, RM. Ischemic contracture, local, in the hand. Plast Reconstr Surg (1946). 1948;3(4):424-433.
Matsen, FA III. Compartmental syndrome: an unified concept. Clin Orthop Relat Res. 1975;(113):8-14.This is a classic review of the pathogenesis,
pressure factors, time factors, physical findings, and treatment of compartment syndrome.
Zuchelli, D, Divaris, N, McCormack, JE, et al. Extremity compartment syndrome following blunt trauma: a level I trauma center’s 5-year
experience. J Surg Res. 2017;217:131-136.
Thomas, N, Cone, B. Acute compartment syndrome in the upper arm. Am J Emerg Med. 2017;35(3):525.
McQueen, MM, Gaston, P, Court-Brown, CM. Acute compartment syndrome: who is at risk. J Bone Joint Surg Br. 2000;82B(2):200-203.
Branco, BC, Inaba, K, Barmparas, G, et al. Incidence and predictors for the need for fasciotomy after extremity trauma: a 10-year review in a
mature level I trauma centre. Injury. 2011;42(10):1157-1163.
Grottkau, BE, Epps, HR, Di Scala, C. Compartment syndrome in children and adolescents. J Pediatr Surg. 2005;40(4):678-682.
Duckworth, AD, Mitchell, SE, Molyneux, SG, et al. Acute compartment syndrome of the forearm. J Bone Joint Surg Am. 2012;94(10):e63.The
authors retrospectively studied 90 patients who accepted fasciotomy for an acute forearm compartment syndrome from 1988 to 2010. Injury
mechanism, presenting symptoms, time to fasciotomy, methods of wound closure, and long-term complications associated with fasciotomy were
documented with a mean 11-month follow-up period. Younger patients were more likely to require split-thickness skin grafting for wound closure. The
patients who accepted fasciotomy more than 6 hours after presentation were at a higher risk for development of complications.
Velmahos, GC, Toutouzas, KG. Vascular trauma and compartment syndromes. Surg Clin North Am. 2002;82(1):125-141.
Whitesides, TE, Heckman, MM. Acute compartment syndrome: update on diagnosis and treatment. J Am Acad Orthop Surg. 1996;4(4):209-218.
Malinoski, DJ, Slater, MS, Mullins, RJ. Crush injury and rhabdomyolysis. Crit Care Clin. 2004;20(1):171-192.
Ugalde, V, Rosen, BS. Ischemic peripheral neuropathy. Phys Med Rehabil Clin North Am. 2001;12(2):365-380.
Bentley, G, Jeffreys, TE. The crush syndrome in coal miners. J Bone Joint Surg Br. 1968;50B(3):588-594.
Battaglia, TC, Armstrong, DG, Schwend, RM. Factors affecting forearm compartment pressures in children with supracondylar fractures of
the humerus. J Pediatr Orthop. 2002;22(4):431-439.
Cambon-Binder, A, Jehanno, P, Tribout, L, et al. Pulseless supracondylar humeral fractures in children: vascular complications in a ten year
series. Int Orthop. 2018;42(4):891-899.
Dente, CJ, Feliciano, DV, Rozycki, GS, et al. A review of upper extremity fasciotomies in a level I trauma center. Am Surg. 2004;70(12):1088-
1093.
Morin, RJ, Swan, KG, Tan, V. Acute forearm compartment syndrome secondary to local arterial injury after penetrating trauma. J Trauma.
2009;66(4):989-993.
Suzuki, T, Moirmura, N, Kawai, K, Sugiyama, M. Arterial injury associated with acute compartment syndrome of the thigh following blunt
trauma. Injury. 2005;36(1):151-159.
Kalyani, BS, Fisher, BE, Roberts, CS, Giannoudis, PV. Compartment syndrome of the forearm: a systematic review. J Hand Surg.
2011;36(3):535-543.Twelve articles with 84 patients were included in this systematic review to discuss the etiology, diagnosis, treatment, fasciotomy
wound management, complications, and outcome of forearm compartment syndrome. Skin grafting was required to close the fasciotomy wound in 61%
cases, and the most common complication was neurological deficit.
Addison, PD, Lannon, D, Neligan, PC. Compartment syndrome after closure of the anterolateral thigh flap donor site: a report of two cases.
Ann Plast Surg. 2008;60(6):635-638.
Chinn, M, Colella, MR. Prehospital dextrose extravasation causing forearm compartment syndrome: a case report. Prehosp Emerg Care.
2017;21(1):79-82.
Funk, L, Grover, D, de Silva, H. Compartment syndrome of the hand following intra-arterial injection of heroin. J Hand Surg. 1999;24(3):366-
367.
Jue, J, Karam, JA, Mejia, A, Shroff, A. Compartment syndrome of the hand: a rare sequela of transradial cardiac catheterization. Tex Heart Inst
J. 2017;44(1):73-76.
Elmorsy, A, Nutt, J, Taylor, N, Kirk-Bayley, J, Hughes, S. Iatrogenic forearm compartment syndrome. J Intens Care Soc. 2017;18(1):63-65.
Leversedge, FJ, Moore, TJ, Peterson, BC, Seiler, JG III. Compartment syndrome of the upper extremity. J Hand Surg. 2011;36(3):544-559.
Oak, NR, Abrams, RA. Compartment syndrome of the hand. Orthop Clin North Am. 2016;47(3):609-616.
Willhoite, DR, Moll, JH. Early recognition and treatment of impending Volkmann’s ischemia in the lower extremity. Arch Surg.
1970;100(1):11-16.
Dente, CJ, Wyrzykowski, AD, Feliciano, DV. Fasciotomy. Curr Probl Surg. 2009;46(10):779-839.
Whitesides, TE Jr, Haney, TC, Harada, H, et al. A simple method for tissue pressure determination. Arch Surg. 1975;110(11):1311-1313.
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Kistler, JM, Ilyas, AM, Thoder, JJ. Forearm compartment syndrome: evaluation and management. Hand Clin. 2018;34(1):53-60.
Kanj, WW, Gunderson, MA, Carrigan, RB, Sankar, WN. Acute compartment syndrome of the upper extremity in children: diagnosis,
management, and outcomes. J Child Orthop. 2013;7(3):225-233.The article was to retrospectively study 23 pediatric patients with acute
compartment syndrome of the upper arm. Most patients developed compartment syndrome due to fracture. Pain and swelling were commonly seen in
the patients. Seventeen patients (74 %) had no loss of function or sensation, and 5 (22%) obtained minor permanent change at the latest follow-up visit.
Reichman, EF. Compartment syndrome of the hand: a little thought about diagnosis. Case Rep Emerg Med. 2016;2016:2907067.
Lipschitz, AH, Lifchez, SD. Measurement of compartment pressures in the hand and forearm. J Hand Surg. 2010;35(11):1893-1894.
Matsen, FA III, Winquist, RA, Krugmire, RB Jr. Diagnosis and management of compartmental syndromes. J Bone Joint Surg Am.
1980;62A(2):286-291.
Mubarak, SJ, Hargens, AR, Owen, CA, et al. The wick catheter technique for measurement of intramuscular pressure: a new research and
clinical tool. J Bone Joint Surg Am. 1976;58A(7):1016-1020.
Boody, AR, Wongworawat, MD. Accuracy in the measurement of compartment pressures: a comparison of three commonly used devices. J
Bone Joint Surg Am. 2005;87A(11):2415-2422.
Hammerberg, EM, Whitesides, TE Jr, Seiler, JG III. The reliability of measurement of tissue pressure in compartment syndrome. J Orthop
Trauma. 2012;26(1):24-31.
Zandi, H, Bell, S. Results of compartment decompression in chronic forearm compartment syndrome: six case presentations. Br J Sports Med.
2005;39(9):e35.
Abe, Y, Fujii, K. Chronic compartment syndrome of the mobile wad: a case report. J Hand Surg Asian Pac Vol. 2017;22(4):516-518.
Chidgey, LK, Szabo, RM, Kolack, B. Effects of elevation on nerve function in an acute upper extremity nerve compression model. J Orthop
Res. 1989;7(6):783-791.
Ouellette, EA, Kelly, R. Compartment syndromes of the hand. J Bone Joint Surg Am. 1996;78A(10):1515-1522.The retrospective study reported the
outcomes of 17 patients after fasciotomy for hand compartment syndrome. Thirteen patients regained normal functions of the hand, but 4 patients
whose compartment syndrome was obtunded by a serious illness or injury or anesthesia had poor results.
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C H A P T E R 9 0
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Common Congenital Hand Anomalies
Paymon Rahgozar and Kevin C. Chung
Key Points
Congenital hand anomalies can be classified into seven 
 categories based on the type of embryologic
failure.
Patients often have associated syndromes or systemic abnormalities that must be considered during
the initial patient evaluation.
Ideally, reconstruction is completed by school age to optimize hand function and growth, as well as
to minimize self-conscious feelings around peers.
The goals of syndactyly reconstruction include creation of an anatomically normal webspace, tension
free closure of soft tissue, and return of function to the fingers.
Ulnar polydactyly is common in African Americans and is frequently treated with an in-office
ligation procedure.
Patients with camptodactyly or clinodactyly frequently present with mild deformities and no
functional deficits.
Trigger thumb often presents with a palpable Notta nodule proximal to the A1 pulley.
If severe enough, constriction ring syndrome can lead to critical lymphedema or compartment
syndrome, requiring emergent release of the constricting bands.
Pollicization is the preferred treatment for thumb hypoplasia that lacks a stable carpometacarpal
joint.
Congenital hand anomalies are quite common with an incidence of 1 in 500 to 1900 live births. 1,2
Syndactyly, polydactyly, and camptodactyly are the most frequently encountered disorders.
Approximately 60% of cases occur spontaneously, 20% are inherited, and 20% are secondary to an
environmental cause. 3 Although inheritance patterns may be either autosomal dominant or recessive,
congenital hand anomalies also present as part of a sequence or an association. A single in utero insult, such
as a vascular thrombosis, may initiate a sequence of events with a series of subsequent anomalies. Poland
syndrome, for example, occurs after a unilateral insult during embryogenesis, leading to a sequence of
events resulting in a chest deformity and ipsilateral symbrachydactyly. On the other hand, an association,
such as VACTERL association (vertebral abnormalities, anal atresia, cardiac defects, tracheoesophageal
fistula, renal agenesis, limb anomalies) seen in patients with radial dysplasia, involves a series of
nonrandom abnormalities that are not part of a syndrome nor the result from one specific event.
Management of the patient with a congenital hand anomaly requires many considerations. The surgeon
may be one of the first practitioners to evaluate the patient, and the tendency will be to focus on the
obvious defect. However, given the multitude of syndromes, sequences, and associations involved with
hand anomalies, the surgeon must perform a comprehensive examination to rule out and appropriately
treat any life-threatening conditions.
Unlike an adult, a child is not able to effectively communicate or follow instructions during an
examination. Often, the most valuable historians are the parents, who observe the child regularly and can
report on abnormal posturing or functional limitations. The 
 in-office exam should focus on observing
the child moving the extremity spontaneously, testing of the passive range of motion, and comparing the
affected extremity with the contralateral side. The absence of flexion creases is suggestive of lack of
motion, which may be due to a joint, soft tissue, or neurovascular cause. Plain films are an invaluable tool
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to determine the osseous anatomy as well as the presence of any abnormalities such as a triphalangeal
thumb, 
 complex syndactyly, or bracketed epiphysis.
The timing of intervention requires thoughtful planning. The hands double in length in the first 2 years of
life. A digit that tethers another digit (syndactyly) will result in marked deformity during this rapid
growth spurt and will require early release. 3 The cortical control for placing the limb in space and for
strong grasping is developed by 1 year. Prehensile grasp and pinch between the thumb and fingers
continue to become refined up to 3 years of age. Surgery is performed at an early age when the function
of parts of the hand is to be altered by transposition (pollicization) to minimize the degree of cortical
relearning required.
The major goals of surgery are to orient the hand in space and provide adequate prehension. The minimal
prerequisites for a functional hand include the presence of a thumb, two ulnar-sided digits, and a wide
thumb webspace. Therefore, in cases where the child has acceptable hand function, as in many cases of
cleft hand, surgery may not be indicated despite an abnormal appearance. 3 Operative intervention may
also be contraindicated in an older patient who has already adopted alternative hand patterns for
functional use of the extremity. Altering the hand position would require relearning how to use the hand
entirely. 3 To minimize potential feelings of self-consciousness among peers, the goal should be to
complete the reconstruction by school age, particularly if multiple procedures are necessary.
Classification of congenital hand anomalies not only requires a thorough understanding of normal
anatomy, but also knowledge of embryology and development. To provide a unified system for the
classification of congenital hand anomalies, the American Society for Surgery of the Hand (ASSH) and the
International Federation of Societies for Surgery of the Hand (IFSSH) have adopted a system that groups
anomalies into seven categories, based on the type of presumed embryologic failure 4 :
Type I: Failure of formation of parts (e.g., radial dysplasia, phocomelia)
Type II: Failure of differentiation (separation) of parts (e.g., syndactyly, camptodactyly, clinodactyly,
trigger digits)
Type III: Duplication (e.g., polydactyly)
Type IV: Overgrowth (e.g., macrodactyly)
Type V: Undergrowth (e.g., hypoplastic thumb)
Type VI: Congenital Constriction Band Syndrome
Type VII: Generalized skeletal abnormalities (e.g., dwarfism)
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Embryology
Upper extremity development takes place between the fifth and eighth weeks of intrauterine growth. It
begins with the outgrowth of the arm bud at approximately 30 days gestation, and by 37 days gestation
the hand plate is well developed. 5,6 Concentration gradients of a variety of growth factors influence
growth in three spatial axes: proximodistal, anteroposterior (radial-ulnar), and dorsoventral.
Proximodistal limb growth is controlled by a thickened ridge of ectodermal tissue known as the apical
ectodermal ridge (AER). It is the last axis to develop and its function is dependent on the secretion of
several fibroblastic growth factors that control the AER differentiation and growth. 6,7 Experimental
removal of the AER leads to a truncated limb whereas implantation at another site leads to an ectopic
limb. 6,8 The delineation of digital rays and finger separation occurs through apoptosis of specific portions
of the AER between 47 and 53 days. 3,6 Aberrations in this process can lead to polydactyly or syndactyly. 3
The zone of polarizing activity (ZPA) is responsible for anteroposterior or radio/ulnar growth and is
driven by the expression of sonic hedgehog protein. It is the first axis to be established with its orientation
predetermined before the start of limb bud growth. 6 Alterations in the ZPA can lead to the development
of a mirror hand. 7,8 Experimental transplantation of the ZPA to the radial side of a developing limb will
result in mirror duplication of the ulnar hand, or ulnar dimelia. 6 Finally, the dorsal ectoderm controls the
dorsal/volar characteristics of the limb and is driven by the expression of wingless-type mouse mammary
tumor virus integration site family member 7a (Wnt-7a). Disturbances of this axis of development may
lead to palmar duplication syndrome. 7
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Syndactyly
The term syndactyly is derived from Greek: syn = together and dactylos = digit. It occurs because of a
failure of differentiation during embryogenesis with an incidence of 1 in every 2000 to 3000 births. Males
are affected twice as often as females. 8,9 Although most cases are isolated occurrences, 10% to 40% of
cases are familial, inherited in an autosomal dominant pattern with variable expressivity, incomplete
penetrance, and less severe phenotypes compared to autosomal recessive cases. 3,10 The majority of
inherited cases are associated with one of more than 300 distinct syndromic anomalies or sequences, such
as Apert syndrome or Poland sequence.
Syndactyly may be classified into complete (extending through the fingertips with nail involvement),
incomplete (webbing only partially along the length of the digit), simple (soft tissue involvement only),
complex (bony or joint fusion), or complicated (multiple involved tissues including bones, joints, tendons,
muscles, or neurovascular bundles). Complicated syndactyly is often seen in association with other
conditions or syndromes such as polydactyly, constriction rings, toe webbing, brachydactyly, spinal
deformities, heart disorders, or Apert Syndrome. 5 In cases of complete syndactyly, patients have a shared
fingernail between the digits, known as synonychia. The most common webspace affected in
nonsyndromic syndactyly is the middle/ring finger webspace (57% of cases) followed by the ring/little
finger webspace (27% of cases). 9,11 In syndromic cases, the thumb/index finger and index/middle finger
webspaces are more commonly involved. 9,11
The timing of surgical intervention is determined by the length discrepancy of the involved digits. For
example, there is little length discrepancy between the long finger and ring finger. Therefore, there is less
concern for digital tethering of the digits with growth and surgery may be delayed until approximately
12 months of age. Ideally, the release is timed so that it is done early enough to permit normal growth but
late enough to avoid postoperative complications. Surgery before 1 year of age is associated with higher
rates of scar contracture and potential anesthetic complications.
Syndactyly of the border digits (little/ring fingers, thumb/index fingers) results in a length discrepancy
leading to growth and functional disturbances from asymmetric growth, flexion contractures, and
rotation deformities. In these cases, surgical release should be considered earlier, specifically between 3
and 6 months of age. 9,12 Our preference is around 6 months when the infant is bigger and the anesthetic
risk is minimal. Similarly, for complicated syndactyly, earlier intervention may be beneficial. With
bilateral involvement, both hands should be corrected simultaneously in nonambulatory patients
younger than 12 to 14 months. 9
To avoid neurovascular compromise when multiple fingers are involved, staging of the releases is
recommended, releasing one side of a digit at a time. Priority is given to the border digits, particularly the
thumb and the little finger. Sequential surgery can be performed within 3 months of each other. It is ideal
to complete the reconstruction before 24 months of age, when the patterns of function of the digits are
established. 9
The goals of syndactyly release include the following 9 :
creation of an anatomically similar webspace,
tension-free closure of soft tissue, and
return of function to the fingers.
Normal function of the fingers requires proper webspace location and depth. The webspace has a dorsal
to volar inclination of 40 to 45 degrees and it is reconstructed with a dorsal skin flap. 8 Because the
circumference of the separated fingers is approximately 22% to 30% greater than the circumference of the
conjoined digits, release often results in a skin deficit requiring skin grafting (Figure 90.1). 8,12 This can be
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demonstrated to the parents by measuring the circumference of a parent’s ring and small fingers
separately and comparing it to the measurement of the circumference when the fingers are held together.
Full thickness skin grafts are preferable to minimize contracture but should be avoided in the webspaces
and overlying PIP joints. Instead, these areas should be covered with carefully designed flaps. Skin grafts
should be taken from the groin and not from the wrist or the forearm because these are exposed areas and
the scars are aesthetically objectionable.

FIGURE 90.1 Diagram demonstrating the skin deficiency in syndactyly.
A dorsal rectangular or trapezoidal flap is designed approximately two-thirds the distance between the
metacarpal heads and the PIP joints. As originally described by Cronin, dorsal and volar digital flaps are
then designed in a zig-zag pattern to minimize scar contracture (Figure 90.2). 13 The dorsal zig-zag
incision begins at the apex of the dorsal rectangular flap and extends to the midline of the PIP joint, then
across to the neighboring middle phalanx, and back across to the midline of the DIP joint. The volar flap
is designed as a mirror image with the proximal midline vertical incision extending to the level of the
desired webspace, where a horizontal incision is made completing a T for inset of the dorsal webspace
flap. 14 The triangular flaps are transposed and sutured in place ensuring flap coverage of the PIP joints.
To reconstruct the nail folds, adequate skin and pulp are required. Transposition of pulp flaps described
by Buck-Gramcko is elevated to create the paronychial folds (Figure 90.3). 15
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FIGURE 90.2 A. Skin markings for syndactyly release. A dorsal trapezoidal flap is designed two-thirds the distance between
the metacarpal heads and the proximal interphalangeal joints to reconstruct the webspace. Zig-zag dorsal and volar flaps are
utilized to separate the fingers. B. Dorsal release of the fingers. C. Volar release of the fingers. The volar flaps are drawn as
mirror images of the dorsal flaps. Note that the flaps are designed so that they may cover the interphalangeal joints to minimize
cicatricial contracture. D. Suturing of the flaps with an inset of full-thickness skin grafts.
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FIGURE 90.3 As described by Buck-Gramcko, synonychia, or fusion of the nails, is reconstructed by designing pulp flaps to
create the paronychial folds.
(Reproduced with permission from Bae, D. Release of simple sundactyly. In: Hunt, TRIII , ed. Operative Techniques in Hand, Wrist, and Elbow Surgery.
Philadelphia, PA: Wolters Kluwer; 2016:1371. Figure 153-2.)
Postoperative dressings are important to a successful outcome. When skin grafts are used, compression
and immobilization are the rule. In young children, the dressing is reinforced with an above-the-elbow
cast with the elbow flexed at 90 degrees to prevent the cast from slipping down the arm. The cast is
typically removed at 2 to 3 weeks and light dressings can be applied until all wounds are fully closed.
Potential complications include graft loss or web creep (distal migration of the webspace) (Figure 90.4).
Overcompensation of the webspace by positioning it more proximally may help minimize postoperative
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web creep deformities. 9
FIGURE 90.4 Web creep. Distal migration of the webspace may occur as the child grows. Positioning of the webspace more
proximally at the time of syndactyly release may help limit the degree of web creep.
In an effort to minimize or avoid the need for a skin graft, some surgeons advocate recruiting excess
dorsal tissue with the design of a dorsal pentagonal flap. This requires more proximal dorsal hand
incisions leading to a more conspicuous scar. 9,16 Defatting of the digits prior to flap closure is another
method to minimize skin grafts. However, this must be done judiciously to avoid neurovascular injury,
venous congestion, or a withered appearance. Younger patients (3–6 months) have the most digital fat
amenable to defatting. 9
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Apert Syndrome: Acrocephalosyndactyly
Apert syndrome is an autosomal dominant congenital malformation that presents with craniosynostosis,
midfacial malformations, and complex extremity malformations. It is associated with disturbance of the
cell surface receptor for fibroblastic growth factor. 17 
 Apert syndrome represents the severe end of the
syndactyly spectrum with involvement of bones, joints, tendons, and nerves. It can be classified into three
types. Type 1 deformity, known as obstetrician’s or spade hand, is characterized by a narrow first
webspace and syndactyly of the four fingers without thumb involvement. This is the most common and
least severe form. Patients with a type 2 deformity, also referred to as mitten or spoon hand, have
syndactyly of all four fingers with a simple syndactyly of the thumb to the index ray. Finally, those with a
type 3 deformity, also known as rosebud or hoof hand, have a tight osseous or cartilaginous fusion of the
thumb, index, long, and ring fingers with conjoined nail plates. The little finger is often spared from an
osseous union but is still joined to the other digits by a complete simple syndactyly. 17 Separation of the
digits follows the principles of syndactyly reconstruction, requiring staged procedures starting at an early
age. The staging principle is to separate the thumb and little finger bilaterally in the first stage, typically
around the age of one when the child starts to use the thumb to provide a tripod pinch. Then in stage 2
separation of one of the central digits is done to give the child a four-digit hand. Because of skin shortage,
it is not necessary for reasonable function to create a five-digit hand.
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Symbrachydactyly
Symbrachydactyly is caused by a disruption in embryonic formation and differentiation resulting in a
hand with shortened or webbed digits, digital nubbins, or absent digits. It occurs in 0.6 per 10,000 live
births, is more common in males, and is often unilateral, affecting the left extremity in two-thirds of cases.
18 When associated with Poland syndrome, it more commonly involves the right extremity and patients
also present with an absent or hypoplastic pectoralis muscle. 19 Although symbrachydactyly may appear
similar to amniotic band syndrome, the two can easily be distinguished from one another. Hands with
symbrachydactyly often have small nubbins with fingernails, whereas with constriction bands the short
digits occur as a result of intrauterine amputation and will lack nails. 18 Furthermore, patients with
amniotic band syndrome will not only have a visible band on the hand but also on other locations of the
body.
Surgical intervention is not always entirely straightforward and varies from patient to patient. It may
require simple removal of nubbins to complex reconstruction with a microvascular toe transfer. Soft
tissue reconstructions such as webspace releases must be done thoughtfully. Often the digits are
hypoplastic and unstable, and separation may prove to be a detriment to function. The goal of
reconstruction should be to improve pinch, grip, and single hand function. Reconstruction of one digit
can provide a stable post to press a button. The presence of two digits permits pinch, and if one digit is
positioned as a thumb it can provide opposition and improved prehension. A three-digit hand will
further advance function by making a three-point pinch possible. 18
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Polydactyly
Polydactyly, or the presence of an extra digit, occurs as a result of a duplication during development. It is
one of the most common congenital hand anomalies and can be found on either the radial hand (preaxial)
with duplication of the thumb, the ulnar hand (postaxial), or the central hand with involvement of the
three central rays.
Preaxial polydactyly, or thumb duplication, has an incidence of 0.08 per 1000 live births (Figure 90.5A). 3
Although most cases occur sporadically, patients who present with a triphalangeal thumb exhibit an
autosomal dominant inheritance pattern. 20 Thumb duplication is classified using the Flatt classification
system, which sequentially describes the duplication from distal to proximal (Figure 90.6). 21 Even
numbers represent complete phalangeal duplications and odd numbers describe incomplete duplications:
type I deformity has a bifid distal phalanx; type II has a duplication at the level of the interphalangeal
joint; type III is a bifid proximal phalanx; type IV (most common) describes duplication at the level of the
MCP joint; type V is a bifid metacarpal; type VI describes duplication at the level of the carpometacarpal
joint; and type VII involves a triphalangeal thumb.
FIGURE 90.5 A. Preaxial polydactyly. Note that the radial thumb is more hypoplastic than the ulnar duplicate. B. Postaxial
polydactyly.
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FIGURE 90.6 Flatt classification system of thumb duplication.
(Reproduced with permission from Wassel, HD. The results of surgery for polydactyly of the thumb. A review. Clin Orthop Relat Res. 1969;64:175-193. Figure
4-2.)
Surgery for preaxial polydactyly is generally performed between 9 and 15 months of age, prior to the
development of pinch grasp or progressive deviation of the duplicated thumbs. Reconstructive options
include total ablation of one thumb, sharing of equal halves (Bilhaut-Cloquet procedure), or
reconstruction using composite parts from each of the duplicated digits. 3 It must be recognized that none
of the anatomic components of either duplicates is normal. 20 There may be varying degrees of
hypoplasia, including abnormalities in osseous anatomy, tendon anatomy, or joint stability. Although
both the ulnar and radial thumb demonstrate a degree of hypoplasia, the ulnar thumb is usually larger.
The ulnar duplicate is preferentially preserved, not only because of its larger size, but also to preserve the
native ulnar collateral ligament, which is essential to providing stability during thumb to index pinch. To
maintain varus MCP joint stability, the radial collateral ligament of the ablated thumb is preserved and
secured to the radial aspect of the retained ulnar thumb. Furthermore, the flexor and extensor tendons
may be split with eccentric insertions, or may demonstrate fusion on the radial side of the thumb known
as a pollex abductus deformity. The surgeon must carefully inspect the anatomy and realign or reposition
tendons as necessary to ensure optimal function.
Postaxial polydactyly can present as a small skin nubbin on the ulnar aspect of the hand or as a fully
formed digit (see Figure 90.5B). This is the most common type of polydactyly and is frequently found in
African Americans with an incidence of 1 in 143 African American births compared to 1 in 1339
Caucasian births. 20 In African Americans, it has an autosomal dominant inheritance pattern with
incomplete penetrance. However, in Caucasians it is more frequently associated with a syndrome and
should prompt a genetics consultation.
Postaxial polydactyly can be classified into two main groups: type A describes a fully developed
duplicate digit that articulates with the fifth or, at times, sixth metacarpal and type B represents a
rudimentary poorly developed ulnar duplication with a thin, narrow stalk. Type B deformities require
simple ligation of the skin bridge, which can be performed with surgical resection, or suture ligation. To
avoid anesthesia, many prefer an in-office procedure that involves simple ligation of the base of the stalk
with a vascular clip. We do not 
 advocate suture ligation because it will leave a stump that eventually
requires excision. A vascular clip can be placed at the base of the digit to leave no residual nubbin. This
occludes the neurovascular supply to the duplicated digit leading to dry gangrene and autoamputation.
Similar to duplicate thumb reconstruction, type A deformities require surgical reconstruction with
consideration given to maintaining a stable, functional small finger with preservation of the ulnar
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collateral ligament and the adductor digiti minimi. 20
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Camptodactyly
Camptodactyly is a nontraumatic irreducible flexion contracture of the proximal interphalangeal joint,
most commonly involving the ring or small fingers. The term camptodactyly means bent finger in Greek. 22
It can be divided into simple and complex types. The simple type only has a flexion deformity of the PIP
joint, whereas the complex type may have other associated deformities such as syndactyly or
clinodactyly. Although hyperextension of the DIP or MCP joints may occur in camptodactyly, flexion
contracture of these joints would instead suggest a post-traumatic cause. 22
Camptodactyly occurs in less than 1% of the population and typically presents in a bimodal pattern
during the periods of most rapid growth: infancy and puberty. 23,24 The early type is often present at birth,
involves the little finger, and affects boys and girls equally. The delayed type, which is more common,
presents during adolescence, and affects girls more often than boys. 25 Although most cases occur
sporadically, camptodactyly can be transmitted in an autosomal dominant fashion, or can be associated
with Trisomy 13, oculodentodigital, orofacial digital, Aarskog, and cerebrohepatorenal syndromes. 25
The etiology of camptodactyly has been the subject of much debate. Because of its presentation during
periods of rapid growth, some believe that an inability of palmar tissues to grow and lengthen in
proportion to skeletal growth causes abnormalities in the skin, soft tissue, or pericapsular tissue resulting
in tethering of the finger. 22,25 Most commonly there is an anomalous or absent flexor digitorum
superficialis or an anomalous lumbrical muscle. 23,26 Other potential causes include extensor mechanism
laxity and anomalies of the interosseous muscles or the transverse or oblique retinacular ligaments. 22,23,27
Patients may present with a loss of function that interferes with activities of daily living or with
extracurricular activities such as inability to participate in sports, play musical instruments, or hold a
brush. 23 However, because the involved ulnar digits are primarily involved in flexion and grasp, patients
with mild camptodactyly (<30 degrees) have minimal impediment of hand function and can tolerate it
quite well. 24 To differentiate between an intrinsic joint problem and extrinsic flexor tendon involvement,
the examiner should assess the degree of flexion deformity with the MCP joint flexed and extended. 27 If
the contracture improves with an MCP joint flexion, the tethering effect is most likely related to an
extrinsic flexor tendon (i.e., flexor digitorum superficialis). More advanced cases may also present with an
extensor lag from central slip laxity or a boutonniere deformity.
Camptodactyly can often be treated effectively with nonoperative management. PIP joint stretching
exercises have been successful to improve contractures particularly in patients less than 3 years old. 28
Dynamic and static support splints as well as serial finger casting can be employed with gradual
adjustments made as the finger contracture improves. 22,27 However, in patients with flexion contractures
of 30 degrees or greater, impaired function, or unacceptable aesthetics, surgical treatment may be
indicated if the deformity persists or progresses despite a 6 to 12 month trial of nonoperative therapy. 22–24
During surgical release, one needs to avoid neurovascular injury from overaggressive extension of the
contracted digit, or joint instability from overzealous release of the stabilizing joint structures. 24 Nearly
all patients will at least require: (1) the release of skin pterygium with local transposition flaps or Z-
plasties and possible coverage with full thickness skin grafts; (2) release of the fascia and subcutaneous
tissues; and (3) tenotomy of the flexor digitorum superficialis tendon at the level of the Camper chiasm.
23,29 If a contracture persists despite the above maneuvers, a sliding volar plate release may also be
necessary. In patients with a severe PIP joint extensor lag, an intrinsic transfer using the released FDS
tendon can be performed. Additionally, a Fowler distal extensor tenotomy may be needed to treat a
hyperextended DIP joint. Although in general, surgical intervention improves the degree of contracture,
the amount of improvement is typically modest and prone to recurrence. 22,24,25,28
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Clinodactyly
Clinodactyly is a congenital radio-ulnar deviation of the fingers. Although all bones and all rays of the
hand may be affected, bilateral small finger involvement with radial deviation of the middle phalanges is
most common (Figure 90.7A). It occurs either sporadically or is inherited in an autosomal dominant
pattern with variable expressivity and incomplete penetrance. 30 Clinodactyly can be found with other
conditions of the hand and foot including polydactyly, cleft hand or foot, and mild tibial hemimelia. 31 It
may also be seen in Down syndrome, Rubinstein-Taybi syndrome, Apert syndrome, and oculodental
dysplasia. 32
FIGURE 90.7 A. Bilateral small finger clinodactyly. B. Bracketed epiphysis with deviation of finger toward the epiphysis.
Patients often have an abnormal trapezoidal or triangular shaped middle phalanx referred to as a delta
phalanx, or a longitudinally bracketed epiphysis. This C-shaped epiphysis is characterized by an
irregularly shaped physis, or growth plate, that spans the axial length of the phalanx. Therefore,
longitudinal growth is unilaterally restricted, leading to a progressive deviation of the digit toward the
bracketed epiphysis (Figure 90.7B). 33 Although the exact etiology is not known, it is thought to result
from incomplete development of primary ossification centers. 31
Primary complaints with clinodactyly are either aesthetic or functional, such as difficulty with playing
musical instruments. Treatment for clinodactyly is typically considered when there is more than
20 degrees of deviation. 32,33 Options for surgical treatment include a physiolysis or a corrective
osteotomy. In a physiolysis the bracketed epiphysis is resected and tissue such as a fat graft is interposed
to prevent recurrence. 30 This method does not immediately repair the deformity, but as the child grows,
there is an increase in longitudinal growth and a decrease in angular deformity. This method is preferred
in younger patients where an osteotomy of small bones may be technically more challenging and there is
a longer propensity for growth and correction of the deformity. 32
A corrective osteotomy is an alternative for patients who are older and have bone fragments that are large
enough to permit accurate surgical manipulation. Although osteotomy options include closing wedge,
opening wedge, and a reverse wedge osteotomy, the opening wedge osteotomy is preferred to maintain
the finger length. 32 Osteotomy can also be used to treat older children who present after closure of the
bracket epiphysis, or as a subsequent procedure after physiolysis, to lengthen or correct recurrent or
persistent angulation. 31
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Radial Column (Preaxial) Abnormalities
Pediatric patients may present with a trigger thumb or a clasped thumb. These two entities can often be
confused with one another, and at times patients may be referred by a pediatrician with an incorrect
diagnosis of a fracture or dislocation.
Trigger thumb, also historically called congenital trigger thumb, has an estimated incidence of 3.3
children per 1000 live births by the first year of life. 34 The term trigger is a misnomer because it is
uncommon to see snapping as in adult trigger finger. Instead, children typically present with a fixed
flexion contracture of the IP joint averaging 25 to 35 degrees with inability or difficulty with active or
passive thumb extension. 35,36 Furthermore, congenital trigger thumb is also a misnomer because studies of
newborns have failed to show its presence at birth, suggesting an acquired etiology. 34,37 The average age
of presentation is approximately 24 months. 35,36 It is thought to occur as a result of an enlarged flexor
pollicis longus tendon proximal to a tight A1 pulley, leading to an area of thickening or a palpable Notta
nodule. 35 The nodule obstructs the tendon’s ability to glide through the A1 pulley maintaining it in a
fixed position with a flexed posture.
Initial treatment involves a period of observation to assess for spontaneous resolution of symptoms. Baek
et al. prospectively followed 87 trigger thumbs and found that within 5 years, approximately 75% had
symptomatic improvement without residual deformity or compensatory hyperextension of the MCP
joint. 36 Splinting has not been shown to be of any consistent benefit, and parents may find it difficult to
keep the child from removing the splint. Candidates for surgery are those who fail to improve despite a
period of observation, the exact timing of which is often surgeon dependent. Treatment is performed with
a surgical release of the A1 pulley through an incision over the proximal flexion crease. Care should be
taken to avoid the neurovascular bundles, which are found in a more central location on the volar surface
of the thumb as compared to the fingers. The surgeon must demonstrate full excursion of the thumb
intraoperatively to confirm adequate release.
In contrast to trigger thumb, congenital clasped thumb deformity presents with contracture at the MCP
joint. Clasped thumb can be divided into two subtypes: supple and complex. In the supple subtype, the
extensor mechanism is hypoplastic or absent, and the contracture can be corrected with passive
extension. Initial treatment involves a trial of splinting but may ultimately require tendon transfer if
splinting fails. Patients with the complex subtype frequently have bilateral involvement and present with
additional pathology such as joint or first webspace contractures, or abnormalities of the collateral
ligaments and thenar musculature. This results in uncorrectable MCP flexion and thumb adduction
contractures. Associated abnormalities such as arthrogryposis, digitotalar dysmorphism, or Freeman-
Sheldon syndrome may also be present. 38 Treatment for the complex subtype requires reconstruction of
the lax ligaments and appropriate correction of fixed contractures through a combination of Z-plasties for
first web contractures, thenar and adductor muscle releases, flap coverage of the volar skin deficit, and
flexor pollicis longus tendon lengthening. 38,39
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Constriction Ring Syndrome
Constriction ring syndrome, also known as amniotic band syndrome or constriction band syndrome, is a
condition that results in distal limb malformation or deformation, lymphedema, acrosyndactyly, or
amputation (Figure 90.8). The incidence has been described to range from 1/1200 to 1/15,000 live births
with no known autosomal inheritance pattern or genetic predilection. 40 Factors that have been found to
be associated with constriction ring syndrome include prematurity (<37 weeks), low birth weight
(<2500 g), maternal drug exposure, and maternal illness or trauma during pregnancy. 40,41 Patients present
with characteristic constriction rings, and if severe enough, may present with neurovascular impairment
from direct compression or from compartment syndrome. In cases of acrosyndactyly, digits that were at
one point separated undergo refusion at the site of the band, resulting in an epidermal lined sinus tract
that communicates from the dorsal side to the volar side.
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FIGURE 90.8 Patient with constriction ring syndrome. Note the shortened digits and the characteristic bands on the index and
ring fingers.
The cause of constriction ring syndrome has long been debated. The intrinsic theory proposes that a
teratogenic insult, viral infection, or vascular disruption results in an inherent defect during
embryogenesis affecting mesodermal tissue, leading to the characteristic rings. The extrinsic theory
suggests that an external band entangles, constricts, or amputates the limbs. Torpin theorized that early
amniotic rupture followed by a period of oligohydramnios with torn cords of amnion results in extrinsic
compression of the fetal appendages and the characteristic deformities. 42 Despite the debating theories,
most contemporary authors believe the extrinsic theory to be more accurate, citing that the asymmetric
clinical presentation of the limbs, the presence of a straight line across adjacent digits, and the occurrence
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of acrosyndactyly (refusion of previously separated digits) are better explained in an extrinsic cause. 40
The goal of surgical treatment is to optimize function and appearance. The timing of surgery is
dependent on the severity of the constriction band. Shallow bands may require no operative treatment
other than for aesthetic purposes. However, more severe constriction bands that result in severe distal
lymphedema, cyanosis, or circulatory compromise may lead to ischemia or compartment syndrome and
should be treated urgently after birth. 40 In less acute cases, the treatment involves one or two stage
releases beginning at around 3 months of age. Release is performed with a series of Z-plasties or W-
plasties and excision of the bands. Given potential concerns of vascular interruption of the distal segment,
some authors advocate a two-stage release where approximately 50% of the band is released at the first
stage, followed by an interval of 6 to 12 weeks to reestablish cutaneous circulation across the scar before
subsequent final release. To address the contour deformity that results from the subcutaneous deficiency
under the constriction band, Upton and Tan published a technique describing separate advancement of
subcutaneous adipose flaps to add bulk to the area underlying the constriction band. The skin flaps are
then transposed as separate thin Z-plasty flaps resulting in superior aesthetic outcomes. 43
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Radial Longitudinal Deficiency
Radial longitudinal deficiency (RLD), or radial club hand, is a 
 result of a congenital failure of formation
of the radial column. The severity of presentation can vary from mild hypoplasia to complete absence of
the radius, carpal bones, and radial digits. The radial 
 musculotendinous units may be absent or
hypoplastic and often form a fibrous tether at the wrist, contributing to the angled position of the hand. 44
It has an incidence of approximately 1 in 5400 live births. 2 RLD occurs because of an insult to the apical
ectodermal ridge during the fourth to seventh weeks of intrauterine development. 45,46 The condition
gained widespread attention in the 1960s with the thalidomide induced epidemic of phocomelia. 45
Approximately one-third of patients have an associated syndrome such as Holt-Oram, Fanconi anemia,
TAR (thrombocytopenia-absent radius), or VACTERL (vertebral abnormalities, anal atresia, cardiac
defects, tracheoesophageal fistula, renal agenesis, limb anomalies). Furthermore, two-thirds of patients
have an associated medical or musculoskeletal anomaly ranging from congenital heart malformations,
blood dyscrasias, and renal or gastrointestinal dysfunction. 45 Often the limb abnormality may be the only
outward manifestation of the underlying syndrome, necessitating the surgeon to perform an appropriate
work-up including a renal ultrasound, echocardiogram, and complete blood count. 46 The Bayne and
Klug classification divides the radial dysplasias into four types, based on the radiographic appearance 47 :
Type I: short distal radius,
Type II: hypoplastic radius,
Type III: partial absence of the radius, and
Type IV: total absence of the radius.
This classification system was further modified by James to include the presence of thumb hypoplasia,
carpal anomalies, and radioulnar synostosis. 48
Treatment for radial longitudinal deficiency initially involves passive stretching and serial splinting. In
severe cases, some advocate distraction of the tight radial tissues with an external fixator device for at
least 6-8 weeks before surgical reconstruction, a procedure we advocate. The goals of surgical treatment
include the following 45 :
straightening of the radial bowing of the forearm,
correction of radial and volar subluxation of the carpus,
improvement of the limb length to optimize function,
improvement of aesthetic appearance, and
treatment of the thumb hypoplasia.
The deformity is corrected by centralizing the carpal bones over the ulna and rebalancing the
musculotendinous structures to alter the deforming forces on the wrist. Although centralization is
commonly performed, it has been shown to result in recurrence of angulation and in limb shortening. 44
All cases of radial longitudinal deficiency have an associated thumb hypoplasia, though thumb
hypoplasia may exist without an abnormality of the radius. 48 Blauth developed a classification for thumb
hypoplasia based on the size of the thumb, the thenar musculature, first webspace contracture, and
stability of the carpometacarpal joint 49,50 :
Type I: minor thumb hypoplasia,
Type II: first webspace narrowing, hypoplastic or absent thenar intrinsic muscles, MCP joint
instability,
Type III: first webspace narrowing, hypoplastic or absent thenar intrinsic muscles, MCP joint
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instability, extrinsic tendon abnormalities, skeletal abnormalities:
Type IIIA: hypoplastic metacarpal, stable CMC joint and
Type IIIB: partial metacarpal aplasia, unstable CMC joint,
Type IV: floating thumb (pouce flottant), and
Type V: absent thumb
Surgical treatment of thumb hypoplasia is most dependent on the presence and stability of the thumb
carpometacarpal joint (CMC). In milder cases (Blauth types II and IIIA), reconstruction is focused on
widening of the first webspace, an opponensplasty procedure, and metacarpophalangeal joint
stabilization. However, in more severe cases that lack a stable CMC joint (Blauth types IIIB, IV, or V), the
thumb, if present, cannot be effectively reconstructed. Treatment is best performed with thumb ablation
and reconstruction with index finger pollicization, transferring the index finger to the thumb position
(Figure 90.9). The principles include skeletal shortening of the index metacarpal shaft, rotation and
stabilization of the index finger, and rebalancing of the musculotendinous units by transfer of the first
dorsal and volar intrinsic muscles for abductor and adductor function. 46,51 Pollicization provides a
pleasing aesthetic reconstruction of the thumb and successfully restores opposition and grasp functions.
FIGURE 90.9 A. Preoperative image of patient with Blauth type IV thumb hypoplasia. B. Postoperative image after
pollicization of the index finger.
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Cleft Hand (Central Longitudinal Deficiency)
Cleft hand or central longitudinal deficiency is characterized by varying degrees of growth suppression
of the bones and soft tissue of the central hand, most frequently involving the central rays. The defect
does not extend proximal to the wrist but may have associated carpal coalition or radioulnar synostosis. 44
It is frequently associated with central polydactyly or osseous syndactyly. Although the cleft itself causes
little functional limitation, the main deficit is secondary to a narrowed thumb-index webspace. Many
classification schemes exist, but arguably the most useful one that can help guide surgical treatment is
that proposed by Manske and Halikis that characterizes the degree of first webspace narrowing. 52
Surgical reconstruction is performed to improve functional limitations of the hand that may be related to
a severe flexion contracture of one or more digits, a malpositioned index finger ray, or syndactyly
involving the thumb. Because deformities may worsen with growth, patients are treated by 1 year of age
with completion of any additional reconstructions by school age (5–6 years). Surgical repair involves: (1)
preservation of a stable and mobile thumb; (2) transposition of the index ray to the ulnar side of the cleft;
(3) creation of a wide and functional first webspace; (4) correction of any index finger malrotation or
deviation; (5) preservation of an adductor pollicis muscle; and (6) creation of a satisfactory aesthetic
appearance. 53
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Conclusion
The evaluation and treatment of a patient with a congenital hand anomaly can be both challenging and
rewarding. The overall goal is to restore form and function, and this is typically achieved by the time the
child is of school age. To be able to appropriately reconstruct the hand, the surgeon must not only have
mastery of normal anatomy, but also must grasp the intricacies of the defect, which may involve bones,
joints, tendons, muscles, and neurovascular structures. The potential to impact an individual for an entire
lifetime makes treatment of congenital hand anomalies arguably one of the most fulfilling of all surgeries.
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Questions
1. A 2-year-old patient presents to the clinic with a left thumb duplication. Based on the Flatt
classification system, which is the most common type of duplication?
a. Type I
b. Type II
c. Type III
d. Type IV
e. Type V
f. Type VI
g. Type VII
2. A 14-year-old girl presents with progressive worsening of flexion deformities of the bilateral ring
finger proximal interphalangeal joints. Which of the following diagnosis does she most likely have?
a. Clinodactyly
b. Constriction ring syndrome
c. Camptodactyly
d. Syndactyly
3. A 15-month-old girl is brought in by her mother who is concerned that she cannot fully extend the
thumb. On examination, the thumb is in 20 degrees of flexion at the interphalangeal joint and cannot
but passively corrected. What is the most likely diagnosis?
a. Triphalangeal thumb
b. Trigger thumb
c. Clinodactyly
d. Camptodactyly
e. Syndactyly
4. A 4-month-old boy presents with syndactyly of all webspaces and decision is made to release his
syndactyly in a staged fashion. Which of the following webspaces should be released at the first
stage?
a. 1st and 4th
b. 1st and 3rd
c. 2nd and 4th
d. 2nd and 3rd
e. 3rd and 4th
5. A 6-month-old girl presents to the office with syndactyly of the left middle and ring fingers. The
fusion involves the entire length of the digits including the nails. An X-ray shows separate middle
and ring finger phalanges. How is this syndactyly classified?
a. Complex, incomplete
b. Complex, complete
c. Simple, incomplete
d. Simple, complete
e. Complicated
1. Answer: d. The type IV deformity describes duplication at the level of the metacarpophalangeal
joint and occurs 43% of the time. The next most common is type VII (20%), followed by type II (15%),
type III (6%), type V (10%), and types I and VI (6%).
2. Answer: c. Camptodactyly describes an irreducible flexion contracture of the proximal
interphalangeal joints and most commonly involves the ring or small fingers. Patients frequently
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present either shortly after birth or during adolescence during periods of rapid growth spurts.
Clinodactyly is most commonly found in the small finger and describes deviation of the digit in the
radio/ulnar plane. Constriction ring syndrome presents with characteristic rings and often shortened
digits. Syndactyly presents at birth and describes fusion of the digits involving soft tissue or bone.
3. Answer: b. Unlike adults, children who present with trigger thumb do not have triggering of the
digit, but instead the thumb is maintained in a flexed position. Often there is a palpable Notta nodule
proximal to the A1 pulley. A triphalangeal thumb may be found in patients with thumb duplication.
Clinodactyly is most commonly found in the small fingers, and presents with radio-ulnar deviation
of the digits. Camptodactyly describes flexion contractures of the PIP joints and is typically found in
the ring or small fingers. Syndactyly describes fusion of the fingers and the digits typically are not
held in a flexed position.
4. Answer: a. When syndactyly involves multiple webspaces, a thoughtful plan must be developed
for its reconstruction. Border digits (thumb and small finger) should be released at an earlier age,
typically 4 to 6 months, to minimize any growth disturbances. Furthermore, to avoid potential
neurovascular compromise, both sides of a digit should not be released in the same operation. In this
patient, the border digits should be released first (1st and 4th webspaces). The remainder of the
webspaces can be released in subsequent operations.
5. Answer: d. Complex syndactyly describes bony fusion of the digits whereas simple syndactyly
only has soft tissue involvement. When the syndactyly occurs along the entire length of the digits,
including the nail bed, it is classified as complete. Incomplete syndactyly occurs when there is only
partial fusion of the fingers. Complicated syndactyly is often seen with other syndromes, such as
Apert syndrome, and may involve multiple structures including bones, joints, tendons, muscles, or
neurovascular structures.
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ACKNOWLEDGMENTS
The work was supported by a Midcareer Investigator Award in Patient-Oriented Research (2 K24-
AR053120-06) to Kevin C. Chung. The content is solely the responsibility of the authors and does not
necessarily represent the official views of the National Institutes of Health.
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Medina, JA, Lorea, P, Elliot, D, Foucher, G. Correction of clinodactyly by early physiolysis: 6-year results. J Hand Surg Am. 2016;41(6):e123-
127.
Kikuchi, N, Ogino, T. Incidence and development of trigger thumb in children. 
 J Hand Surg Am. 2006;31(4):541-543.
Slakely, JB, Hennrikus, WL. Acquired thumb flexion contracture in children: congenital trigger thumb. J Bone Jt Surg Br. 1996;78B:481-483.
Baek, GH, Lee, HJ. The natural history of pediatric trigger thumb: a study with a minimum of five years follow-up. Clin Orthop Surg.
2011;3(2):157-159.The study progressively followed 87 thumbs in 67 pediatric patients with trigger thumb, who had no treatment. At a mean follow-up
of 87 months, 76% of patients had complete resolution. The median time from initial visit to resolution was 49 months. The authors concluded that
most patients with trigger thumb can safely be observed for a period of time, effectively delaying or avoiding an operation.
Rodgers, WB, Waters, PM. Incidence of trigger digits in newborns. J Hand Surg Am. 1994;19(3):364-368.
Hisham Abdel, G, El-Naggar, A, Hegazy, M, et al. Characteristics of patients with congenital clasped thumb: a prospective study of 40
patients with the results of treatment. J Child Orthop. 2007;1(5):313-322.
Serbest, S, Tosun, HB, Tiftikci, U, et al. Congenital clasped thumb that is forgetten a syndrome in clinical practice: a case report. Medicine
(Balt). 2015;94(38):e1630.
Kawamura, K, Chung, KC. Constriction band syndrome. Hand Clin. 2009;25(2):257-264.
Foulkes, G, Reinker, K. Congenital constriction band syndrome: a seventy year experience. J Pediatr Orthop. 1994;14(2):242-248.
Torpin, R, Faulkner, A. Intrauterine amputation with the missing member found in the fetal membranes. JAMA. 1966;198(2):185-
187.Postulated a series of events that has led to the extrinsic theory of constriction ring syndrome. An early amniotic rupture causes oligohydramnios
and a string of chorion to encircle the extremity causing compression and growth suppression or physiologic amputation.
Upton, J, Tan, C. Correction of constriction rings. J Hand Surg Am. 1991;16(5):947-953.
Manske, PR, Goldfarb, CA. Congenital failure of formation of the upper limb. Hand Clin. 2009;25(2):157-170.
Colen, DL, Lin, IC, Levin, LS, Chang, B. Radial longitudinal deficiency: recent developments, controversies, and an evidence-based guide to
treatment. J Hand Surg Am. 2017;42(7):546-563.
Maschke, SD, Seitz, W, Lawton, J. Radial longitudinal deficiency. J Am Acad Orthop Surg. 2007;15(1):41-52.
Bayne, LG, Klug, MS. Long-term review of the surgical treatment of radial deficiencies. J Hand Surg. 1987;12(2):169-179.
James, MA, McCarroll, HR, Manske, PR. The spectrum of radial longitudinal deficiency: a modified classification. J Hand Surg Am.
1999;24(6):1145-1155.
Blauth, W. Der hypoplastische Daumen. Arch Orthop Unfallchir. 1967;62(3):225-246.
Manske, PR, McCarroll, HR, James, M. Type III-A hypoplastic thumb. J Hand Surg Am. 1995;20(2):246-253.
Buck-Gramcko, D. Pollicization of the index finger: method and results in aplasia and hypoplasia of the thumb. J Bone Jt Surg Am.
1971;53(8):1605-1617.
Manske, PR, Halikis, MN. Surgical classification of central deficiency according to the thumb web. J Hand Surg Am. 1995;20(4):687-697.
Upton, J, Taghinia, AH. Correction of the typical cleft hand. J Hand Surg Am. 2010;35(3):480-485.
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C H A P T E R 9 1
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Upper Limb Amputations and Prosthetics
Kate E. Elzinga and Kevin C. Chung
Key Points
Upper extremity amputations range from amputations of the soft tissue of the distal phalanx to
shoulder disarticulation. As the injury becomes more proximal, there is greater patient morbidity
and mortality.
Durable, supple, sensate soft tissue coverage is important for all residual limbs. The level of
amputation is determined based on the level of injury, the condition of the preserved tissues, the
patient’s overall health and goals, and the plan for prosthetic wear.
Multidisciplinary care is essential after limb amputation. Collaborative care by plastic and
orthopedic surgeons, physiatrists, physical and occupational therapists, nurses, social workers,
prosthetists, engineers, and other professionals can optimize the function of an individual with
amputation and minimize pain. The goal is to permit patients to return to work and recreational
activities, albeit using modified techniques.
Targeted muscle reinnervation (TMR) and regenerative peripheral nerve interfaces (RPNIs) are
innovative techniques for neuroma management.
Myoelectric prostheses permit movement of multiple degrees of freedom and, with targeted
reinnervation, simultaneous movement of multiple joints. Cost and the limb-prosthetic interface are
ongoing challenges that limit prosthetic use.
Targeted muscle and sensory reinnervation techniques permit intuitive prosthesis control with the
potential for sensory feedback.
Many patients ultimately abandon the prosthesis owing to lack of comfort and/or lack of
functionality.
Upper limb amputations are common, most often affecting the thumb and fingers. More proximal
amputations, through the forearm, elbow, and upper arm, are less common but markedly more limiting
in function. Most upper limb amputations are caused by trauma. With more proximal traumatic
amputations, replantation success rates are lower because of the increased amount of skeletal muscle
requiring revascularization and the longer regeneration distances for repaired nerves. Goals of upper
extremity reconstruction include the following:
Motion
Length
Sensation
Durability
Appearance
Pain-free, prevention of symptomatic neuromas
Prevention of joint contractures
Eradication of disease
Early prosthetic fitting, when appropriate
Early return to work and recreational activities
After an injury, the primary goal is to maximize upper extremity function, with either limb salvage and
reconstruction or amputation and prosthesis fitting. Other indications for amputation include
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malignancy, infection, vascular disease, high-pressure injection injury, burns, frostbite, and peripheral
nerve injury. Following a burn, frostbite, thrombotic, or embolic injury, limb amputation is often delayed
until tissue demarcation is complete in an effort to maintain functional length.
Patients with amputations are best managed by a multidisciplinary team that includes plastic and
orthopedic surgeons, physical medicine and rehabilitation physicians, prosthetists, engineers,
occupational and physical therapists, psychiatrists, psychologists, nurses, and social workers. The
physical, mental, and emotional needs of the patient are addressed. The patient is taught how to perform
their activities of daily living using their remaining limbs, various aids, and prostheses when appropriate.
Patient-specific management is a core component of care after amputation. Each patient’s occupation and
avocations are explored, their activities of daily living are observed, and their functional and esthetic
goals are discussed. Despite similar amputation levels, patients with similar injuries can have vastly
different desires regarding prostheses. For example, for a middle finger amputation through the midshaft
of the proximal phalanx, some patients adapt to the function and appearance of the short proximal
phalanx, others choose to wear a cosmetic prosthesis to maintain a five-fingered hand, and some undergo
a ray amputation with or without the transposition of the index finger. If a patient is unsure of the
treatment they would like to pursue, maintenance of tissue is recommended; further surgery can be
performed later if the patient desires.
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Amputation Versus Limb Salvage
Upper limb salvage has superior outcomes compared with lower limb salvage. 1 The main functions of
the upper extremity are grip and prehension. This is typically achieved better with a salvaged upper
extremity than an upper limb prosthesis. 2 The injured extremity maintains some sensory feedback, is
more durable and convenient than a prosthesis, and requires less maintenance. Graham et al. determined
that replanted upper extremities result in superior functional results compared to amputation and
prostheses using the Carroll test. 3 Otto et al. reported that replantation is preferred with the advantages
of sensation and psychological well-being compared to a prosthesis. 4
For most upper extremity injuries, arm length preservation is the goal. Free tissue transfers can be
performed to provide coverage over exposed bone, tendons, and neurovascular structures. Flaps permit
stable and durable residual limb coverage for prosthetic fitting. Skin grafts are generally inadequate.
In contrast, the main functions of the lower extremity are weight-bearing and ambulation and can be well
restored with a prosthesis. More than 15% of patients with salvaged lower extremities request elective
amputation, but this is very rare in those with upper extremity salvage. 5 The upper extremity can tolerate
shortening procedures to improve fracture, musculotendinous unit, nerve, and vessel repairs. Limb-
length discrepancy is not well tolerated in the lower extremity. Most upper extremity amputations are
caused by trauma, whereas most lower limb amputations are attributed to peripheral vascular disease.
For multitrauma patients, the “life before limb” principle applies. Damage control surgery principles are
used to optimize the patient’s chance of survival. Intracranial, intrathoracic, and intraabdominal injuries
require emergent management. Extremity injuries are treated secondarily. Multiple surgical teams work
in a coordinated fashion to address the patients’ injuries, and then the patient is brought to the intensive
care unit for monitoring and correction of hypothermia, coagulopathy, and acidosis.
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Amputation Level
Preservation of upper extremity length is generally advocated, as long as stable soft tissue coverage can
be achieved, the limb will be pain free, and the preserved muscles and nerves are functional. However, in
some cases, the principle of length preservation is not applied. For example, a ray amputation can give
better results than a partial finger amputation.
In non–life-threatening cases, management by a multidisciplinary amputation team permits optimal
patient evaluation, education, and formulation of treatment recommendations. Amputation level and
aptness for prosthetic wear are determined based on the patient’s personal goals and lifestyle.
If surgery is indicated, the incisions for an amputation are planned in advance to ensure that the limb will
be well padded with smooth and durable soft tissue to permit comfortable prosthetic wear (Figure 91.1).
For the fingers, hand, and forearm, the thicker volar skin is preferred for coverage compared with the
thinner dorsal skin. Sensory-innervated regional and free flaps are helpful adjuncts to provide sensate
soft tissue coverage over exposed critical structures when preserving limb length.
FIGURE 91.1 A. A transradial amputation was performed 5 years after a distal injury to the patient’s median, ulnar, and radial
nerves that had resulted in a nonfunctional hand. The patient found that the hand was in the way and often sustained traumatic
injuries due to the lack of protective sensation. A fishmouth incision was used. A myodesis was performed. B. Fasciocutaneous
skin flaps were elevated and closed over the residual limb to provide durable, supply soft tissue coverage, resulting in a good
esthetic and functional result.
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Thumb Amputation
Thumb injuries are common and can result in significant morbidity. The thumb accounts for 40% of hand
function; preservation of length is critical. 6 Length and position are more important than motion. The
thumb functions well as a post.
Replantation is preferred but in cases when this fails or is not possible, options for increasing thumb
length include first webspace deepening, metacarpal lengthening using distraction osteogenesis,
osteoplastic reconstruction, pollicization (Figure 91.2), on-top-plasty, and toe transplantation.
FIGURE 91.2 A, B. A pollicization was performed for this patient after a traumatic amputation of his left thumb. A groin flap
was performed for initial wound coverage. C, D. After pollicization, the patient had improvement of his grip and pinch strength,
shown here 6 months postoperatively.
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Digital Amputation
Following amputation of the index, middle, ring, or small finger, the patient’s function is assessed. The
length of the residual digit and the associated joint motion are evaluated. For example, if the amputation
is through the middle phalanx, distal to the insertion of the flexor digitorum superficialis tendon, then
preservation of the digital length is often recommended to maintain motion and strength through the
proximal interphalangeal joint. However, if the amputation is through the middle phalanx, proximal to
the insertion of the flexor digitorum superficialis, preserving the short middle phalanx stump is often a
hindrance because there will be no active flexion through the proximal interphalangeal joint.
Proximal digital amputations through the proximal phalanx and more proximal can result in difficulty in
holding small objects. For improved function and appearance, ray amputation may be preferred. This
narrows the hand width, resulting in 40% less grip strength. 7,8 Digital transposition can be performed; for
example, moving an intact second ray over to the base of the third metacarpal after ring finger ray
amputation, to close the cleft and to improve the esthetic result (Figure 91.3).
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FIGURE 91.3 A. Ray amputation and transposition of the adjacent digit improves the esthetics of the hand. A transverse or a
step-cut osteotomy is performed through the base of the adjacent metacarpal and the digit is mobilized and transposed into the
site of the previous digital amputation. B. Internal fixation is performed at the metacarpal base. The intermetacarpal ligament is
repaired at the distal metacarpal with sutures and reinforced with temporary K-wires if needed.
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Partial Hand Amputation
Toe to thumb transfers can be performed to restore sensation, durability, and function following a partial
hand amputation (Figures 91.4 and 91.5). Prosthesis suspension is difficult at this level without impairing
wrist motion. Prosthetic wear often makes the limb longer than the contralateral uninjured side to fit the
required movable components.
FIGURE 91.4 A, B. Toe transplantation provided a neo-thumb after a traumatic right radial hand amputation. C, D. Four
months postoperatively, the toe transfer allowed the patient to grasp and manipulate objects.
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FIGURE 91.5 The donor site morbidity from a second toe transplant harvest, shown here with the toe harvested at the
metatarsal phalangeal joint, is generally minimal.
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Wrist Disarticulation
The radial and ulnar styloid are contoured to permit prosthetic wear without pressure points following a
wrist disarticulation. The triangular fibrocartilage complex is preserved to maintain distal radioulnar joint
(DRUJ) stability and forearm pronation and supination. At this level, with preservation of the DRUJ,
radioulnar convergence does not occur.
Patients with unilateral amputations who do not want to wear a prosthesis are well served by a wrist
disarticulation. However, if they want to wear a prosthesis, a transradial amputation may be preferred as
this level permits space for prosthetic components and an improved appearance of the prosthesis.
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Transradial Amputation
Transradial amputation is the most common level of upper extremity amputation. Myodesis (muscle
sutured directly to bone, typically using bone tunnels) or myoplasty (muscle to muscle suture repair over
the bone) is performed to provide durable soft tissue coverage over the distal bone, to stabilize the distal
musculature, and to permit strong contraction of the distal musculature for prosthetic control. Proximal
migration of the muscle mass impairs signal detection for a myoelectric prosthesis. Myodesis or a
combined technique are preferred over myoplasty alone as they improve the stability of the amputated
limb and result in less migration of the muscle with contraction, in turn permitting improved signal pick-
up by a myoelectric prosthesis (Figure 91.6). With either myodesis and/or myoplasty, the muscles are
repaired at their physiologic tension.
FIGURE 91.6 A transradial combined myodesis-myoplasty technique. The flexor compartment is elevated volarly and the
extensor compartment is elevated dorsally from the radius and ulna. The muscle groups are secured to the bone, as well as to
each other, distally over the bone to provide durable, stable soft tissue coverage and to create new insertions for the muscles.
(A,C,D from Bickels, J, Gortzak, Y, Kollender, Y, et al. Above-elbow and below-elbow amputations. In: Wiesel, SW , ed. Operative Techniques in Orthopaedic
Surgery. 2nd ed. Vol 2. Philadelphia, PA: Wolters Kluwer; 2016:2266. Figures 15-3B, 15-4B, 15-4D.)
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With the loss of the DRUJ, radioulnar impingement is common. Soft tissue interposition at the time of the
amputation can help prevent this complication. Depending on the level of the amputation, the pronator
quadratus or an extensor and a flexor tendon can be interposed. 5
Maintenance of two-thirds of the forearm length preserves pronation and supination. Amputations 10 cm
or more proximal to the wrist permit increased prosthetic options; myoelectric prostheses can be
considered. 9 Maintenance of a minimum of 5 cm of the ulna preserves elbow function and adequate
fitting of a prosthesis. Patients with transradial amputations can wear harness-suspended body-powered
prostheses or myoelectric prostheses suspended at their humeral condyles or using suction sleeves.
Patients with transradial amputations have greater function than those with transhumeral amputations.
Pedicled and free tissue transfers should be performed to preserve the elbow and proximal forearm.
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Elbow Disarticulation, Distal Transhumeral Amputation
For patients with unilateral amputations who do not want to wear a prosthesis, preserving the elbow
permits for greater reach of the amputated arm to assist the uninjured arm in moving and stabilizing
objects.
For patients who choose to wear a body-powered prosthesis, the elbow condyles permit suspension of
the prosthesis. For those choosing a myoelectric prosthesis with a myoelectric elbow, a transhumeral
amputation 5 to 6 cm above the olecranon can be performed. 10 Alternatively, a humerus shortening
osteotomy can be performed to preserve the humeral condyles while elevating the elbow so that the
elbow joint of the prosthesis will be at a similar level to that of the contralateral arm. 11
For distal transhumeral amputations, a Marquardt angulation osteotomy at an angle of 70° to 110° at the
distal humerus can be used to provide a lever arm for suspension when the epicondyles have been
removed and for rotational control of the prosthesis (Figure 91.7). 12
FIGURE 91.7 A distal humerus Marquardt angulation osteotomy can be performed to facilitate prosthesis suspension and
rotational control when the humerus epicondyles have been removed in a distal transhumeral amputation.
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Proximal Transhumeral Amputation
Transhumeral amputations that preserve 5 to 7 cm of the proximal humerus are preferred compared with
more proximal amputations to facilitate prosthetic suspension, proper maintenance of the function of the
deltoid, and an acceptable shoulder contour. 5 More proximal amputations can be optimized for
prosthetic fitting by maintaining length using a pedicled latissimus dorsi flap if the soft tissues are
deficient or a free fibula flap if the humerus is deficient. Distraction lengthening using the Iliazarov
technique is an alternative way of lengthening the residual limb for improved prosthetic fitting.
In a nonfunctional upper extremity, such as that following a panplexus preganglionic brachial plexus
injury, transhumeral amputation at 25%-30% of humeral length is recommended by Dumanian and
Kuiken to remove the weight of the arm while maintaining the shoulder and upper arm contour for
clothing wear. 10
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Shoulder Disarticulation
Traumatic avulsion of the arm at the shoulder is a life-threatening injury, often presenting with associated
thoracic and abdominal injuries due to significant trauma. Urgent surgical intervention is required to
identify and stop bleeding from the axillary artery and vein. Blood transfusions are frequently required.
Replantation is not attempted; these patients are hemodynamically unstable.
Once vascular control is obtained and the patient is stabilized, typically in a second operation, TMR can
be performed using the terminal ends of the brachial plexus for both neuroma prevention and control of a
myoelectric prosthesis. Preservation of the scapula and clavicle improves the shoulder contour and the
appearance when the patient wears clothes. Flap coverage is often required to close the soft tissue defect
at the disarticulation site, using regional or free flaps.
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Pediatric Amputations
Preservation of length and physes is important. 9 Pediatric patients have greater healing ability than most
adults. Tighter and more tenuous wound closure can be tolerated. Regional or free flaps are valuable
adjuncts to maintain skeletal length.
Pediatric patients have a higher incidence of bony overgrowth than adults. Yearly follow-up is
recommended with radiographs. Transhumeral amputations require revisions surgery for bony
overgrowth most frequently, in up to 12% of pediatric patients. 13
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