Questions
1. Which of the following fascial structures, when diseased, is involved in the creation of a spiral
cord?
a. Grayson ligament
b. Cleland ligament
c. Oblique retinacular ligament
d. Retrovascular cord
e. A1 pulley
2. Which type of collagen is found in greater quantities in Dupuytren tissue as compared to normal
fascia?
a. Type I
b. Type II
c. Type III
d. Type IV
e. Type V
3. Which muscle is involved in the ulnar spiral cord of the small finger, sometimes known as the
isolated digital cord?
a. Adductor pollicis
b. Abductor pollicis longus
c. Abductor pollicis brevis
d. Abductor digiti quinti
e. Extensor digiti minimi
4. Which of the following disease characteristics has not been associated with increased risk of
recurrence and/or extension of disease following treatment of Dupuytren contracture?
a. Male gender
b. Cigarette smoking
c. Ectopic disease
d. Onset before age 50
e. Family history of Dupuytren disease
5. Which of the following has been established as a disadvantage of the use of CCH for the treatment
of Dupuytren contracture, compared to other treatment options?
a. Allergic hypersensitivity
b. Cost of the medication
c. Poor wound healing
d. Unacceptable rate of recurrence
e. Increased risk of bacterial infection
1. Answer: a. The structures that contribute to the spiral cord include the pretendinous band, spiral
band, lateral digital sheet, and Grayson ligament. The Cleland ligaments are not typically involved in
Dupuytren contracture. The oblique retinacular ligament is a component of the extensor mechanism
and is not involved in Dupuytren disease. The retrovascular cord, when present, tends to cause DIP
contractures and is not involved in the spiral cord or in displacement of the neurovascular bundle.
The A1 pulley is not a typical site of attachment of Dupuytren cords, but is rather the site of
triggering in cases of trigger digit.
2. Answer: c. Type III collagen is increased in Dupuytren tissue above the levels present in normal
fascia. Specifically, the ratio of type III to type I collagen is increased, which appears to be directly
mebooksfree.net
related to the increased fibroblast density present in this disorder. Of note, the enzyme CCH
(collagenase Clostridium histolyticum) preferentially targets collagen types I and III, but has less effect
on collagen type IV. This is important because type IV collagen is a major structural component of
the connective tissues surrounding arteries, veins, and nerves.
3. Answer: d. When present, the ulnar spiral cord of the small finger often originates from the fascia
and/or insertional tendon of the abductor digiti quinti muscle, causing PIP and sometimes DIP
contractures of the small finger. The adductor pollicis, as well as the abductor pollicis longus and
brevis, all attach to the thumb ray, and are not involved in disease of the small finger. The extensor
digiti minimi tendon inserts onto the dorsum of the small finger as part of the extrinsic tendon
contribution to the extensor mechanism. The extensor mechanism is not involved in Dupuytren
contracture.
4. Answer: b. Although cigarette smoking has been associated with increased prevalence of
Dupuytren disease, it has not been linked to Dupuytren diathesis, which describes the tendency for
patients to experience recurrence and/or extension of disease after treatment. Each of the other
factors listed earlier has been identified as part of the Dupuytren diathesis.
5. Answer: b. Allergic reactions to CCH have been extraordinarily rare in the case series reported so
far. Despite the intent of the enzyme, skin tears sustained during manipulation generally heal within
a few weeks; delays in wound healing have not been a problem in the reported series. The rate of
recurrence after CCH treatment is comparable—and maybe somewhat better than—needle
aponeurotomy. There has been no increased risk of infection after CCH treatment. The main barrier
to widespread adoption of CCH treatment is cost: the medication still costs thousands of dollars per
treatment, which is a serious barrier to worldwide implementation of this procedure.
mebooksfree.net
References
Elliot, D. The early history of contracture of the palmar fascia. Part 1: the origin of the disease: the curse of the MacCrimmons: the hand of
benediction : Cline’s contracture.
J Hand Surg Br. 1988;13:246-253.
Belusa, L, Selzer, AM, Partecke, BD. Description of dupuytren disease by the Basel physician and anatomist Felix Plater in 1614. Handchir
Mikrochir Plast Chir. 1995;27:272-275.
Elliot, D. The early history of contracture of the palmar fascia. Part 2: the revolution in Paris: Guillaume Dupuytren : Dupuytren’s disease. J
Hand Surg Br. 1988;13:371-378.
Osterman, AL, Murray, PM, Pianta, TJ. Cline’s contracture: Dupuytren was a thief—a history of surgery for Dupuytren’s contracture. In:
Eaton, CE , Seegenschmiedt, MH , Bayat, A , et al, eds. Dupuytren’s Disease and Related Hyperproliferative Disorders: Principles, Research,
and Clinical Perspectives. Heidelberg, Germany: Springer; 2012:195-206.
Ross, DC. Epidemiology of Dupuytren’s disease. Hand Clin. 1999;15:53-62, vi.
Lanting, R, Broekstra, DC, Werker, PM, van den Heuvel, ER. A systematic review and meta-analysis on the prevalence of Dupuytren disease
in the general population of Western countries. Plast Reconstr Surg. 2014;133:593-603.
Dibenedetti, DB, Nguyen, D, Zografos, L, et al. Prevalence, incidence, and treatments of Dupuytren’s disease in the United States: results
from a population-based study. Hand (NY). 2011;6:149-158.
Hindocha, S, McGrouther, DA, Bayat, A. Epidemiological evaluation of Dupuytren’s disease incidence and prevalence rates in relation to
etiology. Hand (NY). 2009;4:256-269.
Thurston, AJ. Dupuytren’s disease. J Bone Jt Surg Br. 2003;85B:469-477.
Foucher, G, Lequeux, C, Medina, J, Garcia, RN, Nagel, D. A congenital hand deformity: Dupuytren’s disease. J Hand Surg Am. 2001;26:515-
517.
Urban, M, Feldberg, L, Janssen, A, Elliot, D. Dupuytren’s disease in children. J Hand Surg Br. 1996;21:112-116.
Larsen, S, Krogsgaard, DG, Aagaard Larsen, L, et al. Genetic and environmental influences in Dupuytren’s disease: a study of 30,330 Danish
twin pairs. J Hand Surg Eur Vol. 2015;40:171-176.Twin studies are an elegant way to try to differentiate the influence of genetics versus
environment. In this large twin study on Dupuytren disease in the Danish population, the authors found increased concordance in monozygotic twins
compared to dizygotic twins, meaning genetic factors must play a major role. Statistically, they concluded that the heritability of DD is approximately
80%.
Dolmans, GH, Werker, PM, Hennies, HC, et al. Wnt signaling and Dupuytren’s disease. N Engl J Med. 2011;365:307-317.
Lee, LC, Zhang, AY, Chong, AK, et al. Expression of a novel gene, MafB, in Dupuytren’s disease. J Hand Surg Am. 2006;31:211-218.
Gudmundsson, KG, Arngrimsson, R, Sigfusson, N, et al. Epidemiology of Dupuytren’s disease: clinical, serological, and social assessment:
the Reykjavik study. J Clin Epidemiol. 2000;53:291-296.
Early, PF. Population studies in Dupuytren’s contracture. J Bone Jt Surg Br. 1962;44B:602-613.
Burge, P, Hoy, G, Regan, P, Milne, R. Smoking, alcohol and the risk of Dupuytren’s contracture. J Bone Jt Surg Br. 1997;79B:206-210.
Noble, J, Arafa, M, Royle, SG, et al. The association between alcohol, hepatic pathology and Dupuytren’s disease. J Hand Surg Br. 1992;17:71-
74.
Critchley, EM, Vakil, SD, Hayward, HW, Owen, VM. Dupuytren’s disease in epilepsy: result of prolonged administration of anticonvulsants.
J Neurol Neurosurg Psychiatry. 1976;39:498-503.
Arafa, M, Steingold, RF, Noble, J. The incidence of Dupuytren’s disease in patients with rheumatoid arthritis. J Hand Surg Br. 1984;9:165-166.
Gabbiani, G, Majno, G. Dupuytren’s contracture: fibroblast contraction? An ultrastructural study. Am J Pathol. 1972;66:131-146.
Badalamente, MA, Sampson, SP, Hurst, LC, et al. The role of transforming growth factor beta in Dupuytren’s disease. J Hand Surg Am.
1996;21:210-215.
Luck, JV. Dupuytren’s contracture; a new concept of the pathogenesis correlated with surgical management. J Bone Jt Surg Am. 1959;41A:635-
664.
Murrell, GA, Francis, MJ, Bromley, L. The collagen changes of Dupuytren’s contracture. J Hand Surg Br. 1991;16:263-266.
Leibovic, SJ. Normal and pathologic anatomy of Dupuytren disease. Hand Clin. 2018;34:315-329.Written by a knowledgeable expert in the field,
this detailed description of the normal and pathologic fascial structures of the hand is truly a comprehensive review. Excellent illustrations make the
anatomy easy to understand for both the medical student and the seasoned surgeon.
Hettiaratchy, S, Tonkin, MA, Edmunds, IA. Spiralling of the neurovascular bundle in Dupuytren’s disease. J Hand Surg Eur Vol. 2010;35:103-
108.
Hindocha, S. Risk factors, disease associations, and Dupuytren diathesis. Hand Clin. 2018;34:307-314.
Abe, Y, Rokkaku, T, Ofuchi, S, et al. An objective method to evaluate the risk of recurrence and extension of Dupuytren’s disease. J Hand Surg
Br. 2004;29:427-430.
Hindocha, S, Stanley, JK, Watson, S, Bayat, A. Dupuytren’s diathesis revisited: evaluation of prognostic indicators for risk of disease
recurrence. J Hand Surg Am. 2006;31:1626-1634.Knowing a patient’s risk factors for recurrence and extension of disease is important for shared
decision-making and patient-centered care. This “revisit” of the Dupuytren diathesis is a helpful review of the factors that are statistically associated
with poorer outcomes (recurrence and extension) after treatment.
Hurst, LC, Badalamente, MA. Nonoperative treatment of Dupuytren’s disease. Hand Clin. 1999;15:97-107.
mebooksfree.net
Ball, C, Izadi, D, Verjee, LS, et al. Systematic review of non-surgical treatments for early dupuytren’s disease. BMC Musculoskel Disord.
2016;17:345.
Kadhum, M, Smock, E, Khan, A, Fleming, A. Radiotherapy in Dupuytren’s disease: a systematic review of the evidence. J Hand Surg Eur Vol.
2017;42:689-692.Although radiotherapy is not a common choice for Dupuytren treatment in the United States, it is still something that patients will
ask about, as it is often mentioned on informational websites, which patients seek out. This systematic review is a great summary of the data, and
reminds us that the studies that have shown some mild benefit were hampered by significant sources of bias.
Elzinga, KE, Morhart, MJ. Needle aponeurotomy for Dupuytren disease. Hand Clin. 2018;34:331-344.
McMillan, C, Binhammer, P. Steroid injection and needle aponeurotomy for Dupuytren contracture: a randomized, controlled study. J Hand
Surg Am. 2012;37:1307-1312.
Badalamente, MA, Hurst, LC. Development of collagenase treatment for Dupuytren disease. Hand Clin. 2018;34:345-349.
Mickelson, DT, Noland, SS, Watt, AJ, et al. Prospective randomized controlled trial comparing 1- versus 7-day manipulation following
collagenase injection for dupuytren contracture. J Hand Surg Am. 2014;39:1933-1941, e1.
Weinzweig, N, Culver, JE, Fleegler, EJ. Severe contractures of the proximal interphalangeal joint in Dupuytren’s disease: combined
fasciectomy with capsuloligamentous release versus fasciectomy alone. Plast Reconstr Surg. 1996;97:560-566.
McCash, CR. The open palm technique in Dupuytren’s contracture. Br J Plast Surg. 1964;17:271-280.
Hueston, JT. ‘Firebreak’ grafts in Dupuytren’s contracture. Aust NZ J Surg. 1984;54:277-281.
Ketchum, LD, Hixson, FP. Dermofasciectomy and full-thickness grafts in the treatment of Dupuytren’s contracture. J Hand Surg Am.
1987;12:659-664.
Ullah, AS, Dias, JJ, Bhowal, B. Does a ‘firebreak’ full-thickness skin graft prevent recurrence after surgery for Dupuytren’s contracture?: a
prospective, randomised trial. J Bone Jt Surg Br. 2009;91B:374-378.
Werker, PMN, Degreef, I. Alternative and adjunctive treatments for Dupuytren disease. Hand Clin. 2018;34:367-375.
Craft, RO, Smith, AA, Coakley, B, et al. Preliminary soft-tissue distraction versus checkrein ligament release after fasciectomy in the
treatment of Dupuytren proximal interphalangeal joint contractures. Plast Reconstr Surg. 2011;128:1107-1113.
Kan, HJ, Selles, RW, van Nieuwenhoven, CA, et al. Percutaneous aponeurotomy and lipofilling (PALF) versus limited fasciectomy in
patients with primary Dupuytren’s contracture: a prospective, randomized, controlled trial. Plast Reconstr Surg. 2016;137:1800-1812.
Roush, TF, Stern, PJ. Results following surgery for recurrent Dupuytren’s disease.
J Hand Surg Am. 2000;25:291-296.This study is an honest,
conscientious report of what surgeons can expect when operating on recurrent Dupuytren disease. Drs. Roush and Stern found that 68% of patients
had some disturbance of sensation at final evaluation, and a surprising 11% of fingers had no sensibility. These sobering statistics can help us as we
counsel patients about reoperation in DD.
Felici, N, Marcoccio, I, Giunta, R, et al. Dupuytren contracture recurrence project: reaching consensus on a definition of recurrence. Handchir
Mikrochir Plast Chir. 2014;46:350-354.
Beaudreuil, J, Allard, A, Zerkak, D, et al. Unite Rhumatologique des Affections de la Main (URAM) scale: development and validation of a
tool to assess Dupuytren’s disease-specific disability. Arthritis Care Res (Hoboken). 2011;63:1448-1455.
van Rijssen, AL, Gerbrandy, FS, Ter Linden, H, et al. A comparison of the direct outcomes of percutaneous needle fasciotomy and limited
fasciectomy for Dupuytren’s disease: a 6-week follow-up study. J Hand Surg Am. 2006;31:717-725.
van Rijssen, AL, ter Linden, H, Werker, PM. Five-year results of a randomized clinical trial on treatment in Dupuytren’s disease:
percutaneous needle fasciotomy versus limited fasciectomy. Plast Reconstr Surg. 2012;129:469-477.This randomized trial represents some of the
best available outcomes data on the procedures we use to treat DD. It helped confirm the superiority of fasciectomy over needle aponeurotomy with
regard to recurrence, but also highlighted the increased disability in the short-term for the more invasive procedure.
Stromberg, J, Ibsen-Sorensen, A, Friden, J. Comparison of treatment outcome after collagenase and needle fasciotomy for Dupuytren
contracture: a randomized, single-blinded, clinical trial with a 1-year follow-up. J Hand Surg Am. 2016;41:873-880.
Scherman, P, Jenmalm, P, Dahlin, LB. One-year results of needle fasciotomy and collagenase injection in treatment of Dupuytren’s
contracture: a two-centre prospective randomized clinical trial. J Hand Surg Eur Vol. 2016;41:577-582.
Scherman, P, Jenmalm, P, Dahlin, LB. Three-year recurrence of Dupuytren’s contracture after needle fasciotomy and collagenase injection: a
two-centre randomized controlled trial. J Hand Surg Eur Vol. 2018;43(8):836-840.This multicenter randomized controlled trial aims to answer one
of the biggest questions in Dupuytren treatment: which minimally invasive procedure is best? As it turns out, the outcomes are very similar with
regard to contracture correction (or lack thereof) between groups, but the collagenase group had more pain associated with treatment.
Haase, SC, Chung, KC. Bringing it all together: a practical approach to the treatment of dupuytren disease. Hand Clin. 2018;34:427-436.
mebooksfree.net
C H A P T E R 8 6
mebooksfree.net
Hand Tumors
Mark E. Puhaindran
Key Points
Soft tissue sarcomas in the hand present early and are frequently mistaken for benign tumors. This
has to be taken into account when assessing tumors of the hand.
Magnetic resonance imaging should be performed for suspicious tumors, followed by an incision or
needle biopsy before definitive treatment is decided.
Epidermal inclusion cysts in the fingertip can mimic infective or malignant conditions—a definitive
diagnosis should be made before ablative surgery is performed.
Most ganglions can be managed conservatively. However, surgical management of mucous cysts of
the distal interphalangeal joint should be considered due to the risk of rupture and subsequent
infection and septic arthritis.
The hand is a compact structure, with nerves, vessels, muscles and tendons, bone and joints lying close
together. Any tumor that arises there would quickly involve adjacent structures. It is also very visible, and
most tumors are detected when they are small. Although the majority of tumors that arise in the hand
and wrist are benign, malignant tumors will also be encountered occasionally. 1 These malignant tumors
may not have the typical features of malignancy like a large size or overlying skin changes, and this may
lead to a wrong diagnosis and management. 2 Finally, the intricate anatomy and complex functions of the
hand and wrist have significant implications on definitive resection and reconstruction. The aim of this
chapter is to highlight the clinical assessment of hand masses, the role and limits of different imaging
modalities, and principles of management of specific tumors that commonly affect the hand and wrist.
mebooksfree.net
Clinical Assessment
The following points should be covered in the patient history:
Age
Onset and rate of progression of mass
Associated symptoms (pain, numbness and/or weakness)
Risk factors
In addition, a detailed medical and family history as well as information regarding occupational,
recreational, travel, and sexual activity is useful when considering the various differential diagnoses.
Other factors that may affect the functional outcome after surgery including the hand dominance,
occupation, recreational activities, and general functional status should also be documented.
Tumors arising from the hand and wrist usually present as a painless mass. Skin and subcutaneous
masses are often noticed early, particularly over the dorsum where the skin is thin and pliable, and may
become painful if located over a bony prominence or if interfering with grip and pinch. Subungual
tumors can cause pain, nail deformities, or bleeding. Deeper masses associated with nerves can produce
sensory symptoms (e.g., numbness, paresthesia, and pain) or motor symptoms (e.g., weakness, muscle
atrophy). Tumors that arise around joints can cause joint stiffness, and masses arising around the flexor
sheaths can cause triggering. Finally, osseous tumors may present with acute pain and swelling following
pathologic fractures.
During the physical examination, it is important to document the location of the mass, as well as the size,
shape, overlying skin changes, temperature, consistency, adherence to adjacent tissues, and whether the
mass is tender on palpation. Vascular lesions may be compressible and have an audible bruit and
palpable thrill. Transillumination is an invaluable clinical sign when assessing for ganglions. A Tinel test
is useful to evaluate masses that arise from, or are located adjacent to nerves. Tumors that arise from
tendons or tendon sheaths are usually mobile sideways and move proximally and distally with tendon
excursion. The passive and active range of joint motion should be assessed and documented. A vascular
examination with Allen’s test is essential, especially if the mass is near the radial, ulnar, or digital arteries.
For lesions involving the nail complex, abnormal nail pigmentation and deformity (e.g., ridging, loss of
adherence) should be looked for. Finally, examine the axilla and epitrochlear regions for
lymphadenopathy.
There are many differential diagnoses for hand and wrist masses, and these include nonneoplastic
pathologies such as infection (e.g., osteomyelitis, septic arthritis), trauma, autoimmune conditions (e.g.,
rheumatoid arthritis), and metabolic problems (e.g., gout, calcinosis, and Brown tumors) (Figure 86.1). A
detailed history and physical examination as well as selective laboratory and radiologic investigations are
required to exclude these possibilities.
mebooksfree.net
FIGURE 86.1 Solitary gouty tophus arising from the third MCPJ that was initially thought to be a soft tissue tumor.
mebooksfree.net
Investigative Methods
Imaging studies, starting with plain radiographs, are main modality for evaluating hand tumors. For soft
tissue tumors, soft tissue calcification, bone scalloping, and erosion should be looked for. The following
must be assessed for osseous lesions: the bone affected, location within the bone (diaphyseal,
metaphyseal, epiphyseal), matrix of the tumor, margins, periosteal reaction, fracture, and presence of a
soft tissue component. 3
Because of the complex skeletal anatomy of the carpus and distal radioulnar joint, CT scans, which
provide better resolution and three-dimensional bone anatomy, are frequently needed to augment X-rays
to properly assess bony changes.
Ultrasound is a low-cost and readily available modality that can help to assess the nature of the mass
(solid versus cystic), the vascularity, and the relative location of the mass. It even has a higher resolution
than magnetic resonance imaging (MRI) scans. However, it is operator dependent and is limited in its
ability to evaluate more complex masses, and it is difficult to differentiate between benign and malignant
masses on ultrasound alone.
MRI remains the main modality for imaging complex soft tissues masses. It plays an important role in
characterization of tumor activity, with heterogeneous contrast enhancement as well as perilesional
edema suggesting a locally aggressive tumor. In addition, MRI can be used to assess response to adjuvant
therapy and look for local recurrence in a surgical bed. MRI can also delineate soft tissue compartments
and relationship of the mass to neurovascular structures, 4 which is critical when planning surgical
excision of the mass, especially in the hand.
Nuclear medicine scans are increasingly used in the evaluation of tumors. Bone scans are used to assess
bone activity in the lesion, and can also help to assess for other sites of skeletal involvement. However,
they are not specific and cannot differentiate between infection, trauma, and neoplasms. New
radionuclides have been introduced in an effort to increase the diagnostic accuracy, with different
radionuclides providing different information. For example, 18-FDG (18-flurodeoxyglucose), the most
commonly used radionuclide for positron emission tomography scans, will be taken up preferentially by
metabolically active tissue such as the brain, as well as a majority of aggressive tumors. This will reflect
the metabolic activity of the primary tumour, and help in the detection of metabolically active distant
metastases. When nuclear medicine scans are coupled with anatomical imaging such as CT and MRI
scans, their utility is further enhanced. 18-FDG positron emission tomography scans can also help to
monitor the response to neoadjuvant treatment such as chemotherapy, and can be used as a tool to help
prognosticate patients. 5 However, some tumors, such as myxoid liposarcomas, may not be metabolic and
have low 18-FDG uptake, which will limit the utility of these scans.
mebooksfree.net
Differentiating Benign and Malignant Tumors
Patients with soft tissue sarcomas classically present with a mass that has been enlarging over weeks to
mass. They often have a deep intramuscular location, firm consistency, attachment to surrounding
structures, and overlying skin changes with prominent superficial vessels. 6 Many are large, with a size of
more than 5 cm. In the hand, however, a majority (77%) of patients with soft tissue sarcomas present with
tumors less than 5 cm in greatest dimension. Hence, size should not be the only criterion to rule out
malignancy. 2
Some of the imaging criteria used to differentiate benign from malignant soft tissue tumors include the
size, depth of the lesion, and internal signal intensity. 4 However, again because a majority of malignant
tumors in the hand are detected when they are small, size is not a useful criterion on imaging. Overall, it
may be better to treat all indeterminate lesions as potentially malignant, and then an appropriate biopsy
done to determine the diagnosis before definitive treatment is initiated (Figure 86.2).
FIGURE 86.2 Fungating lesion arising from the middle finger with aggressive features. However, it was shown to be a
pyogenic granuloma on biopsy.
mebooksfree.net
Principles of Biopsy for Suspected Malignant Tumors
The standard for diagnosis is biopsy. A good biopsy helps to provide the diagnosis, and does not
compromise the definitive treatment. Conversely, a poorly executed biopsy can have disastrous
consequences, with either a wrong diagnosis, compromised definitive treatment, or both. 7 When
performing a surgical biopsy for a tumor, the following factors need to be considered:
The presumptive diagnosis
The definitive treatment plan (limb salvage versus amputation)
Who will perform the definitive treatment, and where should it be performed (and should the
patient be referred there for the biopsy)
Whether
additional
support
services
(e.g.,
frozen
section,
specialized
pathology,
immunohistochemistry, and molecular diagnostics) are required and available
Core needle versus open incisional versus excisional biopsy
When performing an open incisional biopsy, the following steps should be observed:
1. Gravity exsanguination
2. Longitudinal skin incision
3. Minimize flaps
4. Avoid tumor spillage
5. Obtain a representative sample (verified with frozen section when necessary)
6. Avoid creating stress risers for bone lesions by creating ovoid bone windows
7. Meticulous hemostasis
8. Drain placement in line with incision
9. Sutures close to edges
10. Bulky, fitting dressings
11. Elevation for edema control
12. Early rehabilitation
13. Splint the affected extremity if required with protected weight bearing for bone biopsies
There are additional considerations when performing a biopsy of the hand, including placement of
incisions in relation to skin creases, tendon sheaths, and neurovascular structures. The choice of biopsy
incision has to consider the eventual plan for tumor resection, and may differ greatly from traditional
operative approaches such as Bruner incisions. Because of this, it is imperative that a surgeon with
experience in hand surgery and management of musculoskeletal tumors be involved in the planning and
execution of biopsy of hand and wrist tumors. Ideally, the surgeon doing the biopsy should also be the
surgeon who will carry out the definitive surgical treatment of the tumor.
mebooksfree.net
Management
Benign Tumors
Marginal excision is sufficient for almost all benign soft tissue tumors. This is performed immediately
around the capsule of the tumor, at the interphase with normal tissue. Wide excision, which involves
excision of the tumor through a margin of normal tissue, may be required for some benign but locally
aggressive tumors such as desmoid tumors.
Intralesional curettage and excision is performed for most benign bone tumors such as enchondromas, in
an attempt to preserve as much bone as possible. For locally aggressive bone tumors such as giant cell
tumors, local adjuvant treatment using techniques such
as cryosurgery or phenol are added to reduce
local recurrence rates.
mebooksfree.net
Malignant Tumors
The main objective of surgical treatment for malignant tumors is to completely excise the tumor with
negative gross and microscopic margins. For sarcomas, there is no universally agreed margin, but in
general, 1 cm of soft tissue, or an appropriate anatomic layer such as fascia, is accepted. 8-10 Closer
margins can be accepted to preserve critical neurovascular structures that are not involved by the tumor.
In the hand, the margins are invariably narrow due to the closeness of critical structures.
Preoperative radiotherapy and/or chemotherapy can be considered for selected large and/or high-grade
tumors, to downstage them and enable effective surgical resection. 11 However, there is a risk of tumor
progression during neoadjuvant treatment, and higher wound infection rates are seen in patients that
have undergone preoperative radiotherapy.
Postoperative radiotherapy should be considered for patients following resections with close margins or
microscopically positive margins. 12,13 Postoperative chemotherapy can also be considered for selected
patients with large, high-grade tumors. 14 The decisions on the type of treatment, and the timing that it is
administered, should be individualized and made by a multidisciplinary tumor board.
The biopsy track should be excised en bloc together with the main tumor during the definitive resection.
Dissection should be done through normal tissue that is not contaminated by tumor. Critical vessels and
nerves that are not grossly involved by tumor can be preserved by dissecting through the adventitia or
epineurium. Radical resection (resection of the entire anatomic compartment) is no longer routinely
required for most patients. At times, amputation may still be required to achieve local tumor control, and
should be considered when complete resection of the tumor would result in a limb that is nonfunctional
and will be a liability to the patient.
Surgical clips can be used to mark the extent of resection to facilitate subsequent radiotherapy planning.
Surgical drains should be inserted close to, and preferably in line with the surgical incision.
The surgeon and the pathologist should review the resection specimen, and mark the margins together,
identifying the areas of concern. If a gross positive margin is identified, repeat resection to negative
margins should be strongly considered. When a microscopic positive is identified on histological
assessment, a repeat resection is advised. However, microscopic positive margins can be accepted in
situations where further resection will result in severe morbidity (e.g., when sacrifice of major vessels or
nerves is required).
Hand preservation is possible for most patients with soft tissue sarcomas of the hand, even those with
relatively large tumors. 15 At times, partial hand amputations—digit disarticulations or modified ray
amputations—may be required. 16
The final functional outcome depends on the amount of native tissue that can be preserved—the more
extensive the resection, the poorer the functional outcome expected. Despite this, the priority of surgery is
to remove the tumor, and this objective cannot be compromised in an effort to preserve limb function.
mebooksfree.net
Reconstruction Following Tumor Resection
There is no fixed formula for functional reconstruction of the hand following tumor resection; these
decisions have to be made on a case-by-case basis, tailoring treatment to the patient’s functional
requirements, goals, and expectations. For example, a well-performed ray amputation can result in a
more functional hand than a reconstructed digit that is painful, poorly padded, and stiff.
Before planning any reconstruction, it is important to understand the functional components of the hand:
The radial component comprises the thumb, index, and middle fingers. These are involved in precision
tasks such as key pinch, pulp pinch, and tripod (chuck) grip. Important elements include a stable
thumb metacarpophalangeal (MCP) joint, mobile trapeziometacarpal joint, and one or more
opposing posts (index and middle fingers) that are sensate and of adequate length. Well-functioning
thenar, first dorsal interosseous, and adductor pollicis muscles are also crucial.
The ulnar component includes the ring and little fingers, and is principally involved in power grip.
This requires painless flexion of the MCP and interphalangeal (IP) joints, intact long flexors, and
mobile carpometacarpal joints for the hand to cup around an object.
The transverse component of the hand is needed for activities that require reach as well as pronation
and supination. This requires a wide span, provided by the border digits (thumb and little finger),
stable MCP joints, a good thumb-index webspace, intact interosseous muscles, and a transverse arch
maintained by the underlying palmar fascia.
Reconstruction after tumor reconstruction should follow this general approach 17,18 :
1. Skeletal reconstruction, with or without selective arthrodesis to establish a base for soft tissue
support
2. Vascular reconstruction to reestablish distal perfusion, either through direct repair or through vein
grafting
3. Soft tissue coverage with skin grafts and/or flaps that will closely replicate the tissue that has been
removed. It needs to be durable and, when needed, able to withstand adjuvant treatment such as
radiotherapy
4. Functional reconstruction to restore motor and sensory function through tendon transfers, nerve
transfers, and free functioning muscle transfers. Functional reconstruction can be delayed until all
other treatment has been completed.
mebooksfree.net
Specific Considerations for Common Hand Tumors
Ganglions
Ganglions are the most common soft tissue tumors seen in the hand—they are mucin-filled cysts and are
not true neoplasms. Ganglions can occur at any age, though they are more commonly seen between the
second to fourth decade. They can arise from any joint within the hand and wrist, as well as from tendon
sheaths. The most commonly encountered are dorsal wrist ganglions (∼70%), followed by volar wrist
ganglions (∼20%), then ganglions arising from the flexor tendon sheaths and mucous cysts arising from
the distal interphalangeal joints.
The walls of ganglions are formed by compressed collagen fibers and lined by flattened cells, and the
main constituents of the fluid include hyaluronic acid, glucosamine, albumin, and globulin. 19
The exact cause of ganglions is not known—the most commonly accepted theory is that a small injury
allows the mucin to dissect through the joint capsule and ligament, producing the cyst. The mucin is then
prevented from returning to the joint because of the presence of a one-way valve. 20,21
The diagnosis of a ganglion can be established on careful clinical evaluation—a cystic, occasionally
multilobulated, mass that transilluminate (Figure 86.3). X-rays are helpful in evaluating underlying joint
changes. MRIs can confirm the diagnosis, as well as identify occult ganglions that may be symptomatic
but not clinically detectable.
FIGURE 86.3 Patient with a dorsal wrist ganglion that transilluminates brilliantly.
There are many treatment options for ganglions—simple observation should be considered because up to
50% resolve without treatment, 22 with the rate of spontaneous resolution even higher in children. 23 Other
options include aspiration and needle rupture, though this is associated with a high recurrence rate. 24
Various substances such as steroids 25 or hyaluronidase 26 have been injected following aspiration of the
ganglion in an attempt to reduce the recurrence rate, albeit with limited success. Injection of sclerosants
should be avoided due to the risk of articular cartilage damage. 27
Excision of ganglions can be done using open or arthroscopic techniques. Open surgery traces the stalk of
the ganglion down to the joint capsule and excises it together with a cuff of capsule. Recurrence rates
between 0.6% and 40% have been reported following open surgery. 21,28 Arthroscopic resection, though
technically more difficult, has the advantage of better visualization of the joint and a better surgical scar,
with similar recurrence rates to open surgery. 29
mebooksfree.net
Giant Cell Tumors of Tendon Sheath
Giant cell tumors of tendon sheath are known by various terms, including pigmented villonodular
synovitis (PVNS), fibrous xanthoma, and localized nodular synovitis. They are the second most common
soft tissue tumors seen in the hand after ganglions (Figure 86.4). Despite the name, these tumors do not
always arise from the tendon sheath or contain giant cells. 30 The tumors are firm and nodular, and
generally are nontender. They can cause pressure erosions of the underlying bone or rarely, actual bone
invasion. These features can be detected on plain radiographs. 31,32
FIGURE 86.4 Patient with PVNS of the thumb that was associated with the ulnar digital nerve. Marginal excision of the mass
was performed.
Marginal excision is the treatment of choice, with recurrence rates of 9% to 27% 30,33 reported. Factors
associated with local recurrence include the presence of degenerative joint disease, involvement of the
distal interphalangeal joint, radiographic evidence of bone pressure erosion, as well as involvement of the
flexor or extensor tendons, or joint capsule. The cause of local recurrence is thought to be due to satellite
nodules that are not completely excised during surgery. Malignant transformation of giant cell tumors of
tendon sheath has not been reported, though a malignant variant has been described. 34
mebooksfree.net
Epidermal Inclusion Cyst
Epidermal inclusion cysts or epidermal cysts are thought to result from the traumatic implantation of
epithelial cells into the soft tissue, or even into bone. 35,36 Over a period of months to years, a painless
lump forms as these cells grow. Epidermal cysts are frequently seen over the fingertips (Figure 86.5A)
and at times even in the stumps of amputated fingers (Figure 86.5B).
FIGURE 86.5 A. Epidermal cyst of the fingertip. B. Epidermal cyst in the stump of an amputated finger.
Patients most frequently present with a painless mass that has been growing slowly over months to years.
Occasionally, the mass may be painful and erythematous. Often, the patient may not recall a prior history
of trauma. On examination, cysts in the soft tissue will be firm, well circumscribed, and slightly mobile.
Those that involve the bone will be fixed; and they may have a lytic appearance on X-ray. Because of this,
epidermal cysts can be mistaken for an infectious or malignant process. 37 Hence, patients with lytic
lesions involving the distal phalanx should undergo biopsy to confirm the diagnosis before any definitive
surgical treatment is performed. Epidermal inclusion cysts can be treated with a marginal excision with a
minimal risk of local recurrence.
mebooksfree.net
Schwannoma
Schwannomas (also known as neurilemomas) are nerve tumors that are commonly seen in the upper
extremity. 38-40 These benign tumors arise from the Schwann cells. They are slow-growing, well-
circumscribed masses that often lie eccentrically within a peripheral nerve (Figure 86.6).
FIGURE 86.6 Schwannoma arising from the digital nerve of a finger.
Patients often present with a painless mass, though they can occasionally present with radiating pain that
occurs when the lesion is traumatized, or even neurological deficits. Examination will reveal a firm mass
that is often mobile transversely (together with the nerve) and not longitudinally. MRI is the most useful
investigation in delineating the lesion, and to identifying the nerve of origin. However, it is often difficult
to differentiate a schwannoma from other nerve tumors such as neurofibromas, or even malignant
peripheral nerve sheath tumors on MRI alone. 41
It has been described that schwannomas can be easily dissected and shelled out from the nerve fibers.
Magnification should be used to separate the nerve fascicles from the tumor mass. Even then, there are
tumors that cannot be separated from the nerve fascicles even with the aid of a microscope. Postoperative
loss of function has been reported in 2.5% to 30% of patients, 38,42,43 and patients need to be counseled on
this possibility before surgery. Local recurrence following tumor excision is rare, and there have been
cases of malignant transformation reported. 44
mebooksfree.net
Enchondroma
Enchondromas are the most common primary bone tumor seen in the hand. They are thought to develop
from residual nests of cartilage originally arising from physis. 45 In the hand, enchondromas are most
frequently seen in the proximal phalanges, followed by metacarpals and middle phalanges. The small
finger ray is the most commonly involved, whereas carpal involvement is rare. 46 They are usually
solitary, and are found in the diaphysis of the bone, though larger lesions can occupy almost the entire
bone. Multiple enchondromas can be seen in patients with Ollier disease and Maffucci syndrome, where
they are also associated with multiple soft tissue hemangiomas. Grossly, enchondromas have a lobulated
appearance with a gray-white color. Microscopically, well-differentiated hyaline cartilage is seen
surrounded by lamellar bone, with variable calcification of the matrix.
Patients are often asymptomatic, and present with pain and swelling due to a pathological fracture.
Digital deformity can be seen in patients with multiple enchondromatosis. The diagnosis can usually be
made with plain radiographs, which will show a lytic diaphyseal lesion, with lobulated margins and
endosteal scalloping; the matrix is clear with a chondroid calcification pattern. 45
Increased pain and swelling may be a sign of malignant change, which is more frequently seen in patients
with Ollier disease (25%) and Maffucci syndrome (100%). Features suggestive of malignant change
include cortical destruction, loss of matrix mineralization, periosteal reaction, and a soft tissue mass.
Incidental lesions can be observed, with counseling with regards to the risk of pathological fracture.
Surgical treatment of enchondromas generally involves intralesional curettage and burring of the cavity
with or without local adjuvant treatment such as phenol or cryotherapy. Following removal of the tumor,
the techniques for reconstruction of the bone defect that have been described include leaving it alone,
cement, bone substitute, allograft, and autograft (Figure 86.7). For patients who present following
pathological fractures, there is the option of early definitive treatment with intralesional curettage, bone
grafting and internal fixation, or staged treatment with closed treatment of the fracture followed by
delayed surgery once fracture union has occurred. 47 In the event of an atypical clinical presentation, or if
there are suspicious radiographic findings, a biopsy should be considered before definitive treatment.
FIGURE 86.7 Patient with enchondroma of the distal phalanx causing nail plate deformity. X-ray showed a lytic bone tumor
with expansion and thinning of the cortex. Curettage of the lesion was performed, and the defect was packed with autologous
bone graft.
mebooksfree.net
Soft Tissue Sarcomas
Sarcomas originate from the embryonic mesodermal layer, and there are more than 50 histological
subtypes that have been described. The classification systems used continually evolve along with
pathology diagnostic techniques (immunohistochemistry and molecular studies).
Soft tissue sarcomas of the hand were initially thought to have a poorer prognosis than tumors arising
elsewhere in the extremities. 48 More recent publications suggest that the reverse is possibly true, with
patients with hand sarcomas appearing to have a better prognosis. 2,49 Part of the reason could be that
patients with hand tumors present earlier with small tumors. The balance between the need for complete
excision of the tumor, with the desire to preserve maximum function in the affected limb, is even more
challenging in the narrow confines of the hand. Continuing improvements in imaging techniques such as
MRI allow better imaging and delineation of the tumor, and greatly assist surgical planning. This then
results in better surgery that preserves more native tissue, while achieving the required negative resection
margins, improving the overall outcomes for patients.
mebooksfree.net
Questions
1. A 30-year-old man presents with painless swelling of the right index fingertip. X-ray shows a lytic
lesion involving the distal phalanx. MRI shows an indeterminate soft tissue mass scalloping of the
bone. What is the next step of management?
a. Ray amputation of the finger
b. Disarticulation of the finger at the distal interphalangeal joint
c. Staging CT scan of the thorax
d. Incision biopsy
2. A 70-year-old patient presents with a firm swelling in the thenar muscles of the left hand. It has
been progressively increasing in size over the past 3 months. Clinically, the mass is less than 5 cm in
size and is nontender. What is the next step of management?
a. MRI
b. CT scan
c. Incision biopsy
d. Excision biopsy
3. A 30-year-old man presents with pain and deformity of the right index finger after minor trauma.
X-ray shows a lytic lesion in the proximal phalanx, consistent with an enchondroma, with a
pathological fracture. What is the next step of management?
a. Bone scan to evaluate for multiple enchondromatosis
b. Immediate internal fixation of the finger for stability
c. Splinting of the fracture, with staged open biopsy, curettage, and bone grafting of the lesion
d. Ray amputation of the finger
1. Answer: d. Epidermal inclusion cysts are benign tumors that occur frequently in the distal
phalanx. They can cause bone erosion and mimic malignant or infective conditions. Hence, a biopsy
is advised so that a definitive diagnosis can be made before an ablative procedure is done.
2. Answer: a. MRI is the best imaging modality to evaluate soft tissue tumors, and should be
performed before any surgery. Patients with soft tissue sarcomas in the hand often present early,
when the tumor is less than 5 cm in size, hence there should be a lower threshold for requesting for
imaging studies.
3. Answer: c. Most enchondromas are solitary, and routine bone scan is not required. If fixation of
the fracture is to be performed, it should also be accompanied by curettage of the enchondroma.
Because these lesions are benign and have a local recurrence rate, ablative procedures such as
amputations are generally not required.
mebooksfree.net
References
Schultz, RJ, Kearns, RJ. Tumors in the hand. J Hand Surg Am. 1983;8(5 pt 2):803-806.This article provides an overview on the types of hand tumors
encountered in a hand surgery practice, This article provides an overview on the types of hand tumors encountered in a hand surgery practice.
Ganglions are the most common, followed by PVNS. Malignant tumors are rarely encountered.
Puhaindran, ME, Rohde, RS, Chou, J, et al. Clinical outcomes for patients with soft tissue sarcoma of the hand. Cancer. 2011;117(1):175-
179.This is the largest published series on soft tissue sarcomas of the hand. It showed that the majority patients with soft tissue sarcomas of the hand
present early (<5 cm) and are misdiagnosed (large number of excision biopsies). Despite this, overall survival is good.
Miller, TT. Bone tumors and tumorlike conditions: analysis with conventional radiography. Radiology. 2008;246(3):662-674.
Wu, JS, Hochman, MG. Soft-tissue tumors and tumorlike lesions: a systematic imaging approach. Radiology. 2009;253(2):297-316.
Kubo, T, Furuta, T, Johan, MP, Ochi, M. Prognostic significance of (18)F-FDG PET at diagnosis in patients with soft tissue sarcoma and bone
sarcoma; systematic review and meta-analysis. Eur J Cancer. 2016;58:104-111.
Myhre-Jensen, O. A Consecutive 7-year series of 1331 benign soft tissue tumours. clinicopathologic data: comparison with sarcomas. Acta
Orthop Scand. 1981;52(3):287-293.
Mankin, HJ, Mankin, CJ, Simon, MA. The hazards of the biopsy, revisited. Members of the musculoskeletal tumor society. J Bone Joint Surg
Am. 1996;78A:656-663.Second article on the hazards of biopsy published by members of the Musculoskeletal Tumor Society. Unfortunately, the first
article did not have its desired impact, and a large number of the patients reviewed in this article still had poorly performed biopsies that compromised
outcomes.
ESMO/European Sarcoma Network Working Group. Soft tissue and visceral sarcomas: ESMO clinical practice guidelines for diagnosis,
treatment and follow-up. Ann Oncol 2012;23(suppl 7)vii92-vii99.
Grimer, RJ, Athanasou, N, Gerrand, C, et al. UK guidelines for the management of bone sarcomas. Sarcoma. 2010;2010:317462.
von Mehren, M, Randall, RL, Benjamin, RS, et al. Soft tissue sarcoma, version 2.2014. J Natl Compr Canc Netw. 2014;12:473-483.
O’Sullivan, B, Davis, AM, Turcotte, R, et al. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised
trial. Lancet. 2002;359(9325):2235-2241.
Pisters, PW, Harrison, LB, Leung, DH, et al. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue
sarcoma. J Clin Oncol. 1996;14(3):859-868.
Yang, JC, Chang, AE, Baker, AR, et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft
tissue sarcomas of the extremity. J Clin Oncol. 1998;16(1):197-203.
Pervaiz, N, Colterjohn, N, Farrokhyar, F, et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for
localized resectable soft-tissue sarcoma. Cancer. 2008;113(3):573-581.
Puhaindran, ME, Steensma, MR, Athanasian, EA. Partial hand preservation for large soft tissue sarcomas of the hand. J Hand Surg Am.
2010;35(2):291-295.
Puhaindran, ME, Healey, JH, Athanasian, EA. Single ray amputation for tumors of the hand. Clin Orthop Relat Res. 2010;468(5):1390-1395.
Talbot, SG, Mehrara, BJ, Disa, JJ, et al. Soft-tissue coverage of the hand following sarcoma resection. Plast Reconstr Surg. 2008;121(2):534-543.
Mehrara, BJ, Abood, AA, Disa, JJ, et al. Thumb reconstruction following resection for malignant tumors. Plast Reconstr Surg. 2008;121(4):1279-
1287.
Loder, RT, Robinson, JH, Jackson, WT, Allen, DJ. A surface ultrastructure study of ganglia and digital mucous cysts. J Hand Surg Am.
1988;13:758-762.
Jayson, MI, Dixon, AS. Valvular mechanism in juxta-articular cysts. Ann Rheum Dis. 1970;29:415-420.
Angelides, AC, Wallace, PF. The dorsal ganglion of the wrist: its pathogenesis, gross and microscopic anatomy, and surgical treatment. J
Hand Surg Am. 1976;1:228-235.
McEvedy, BV. The simple ganglion. Lancet. 1954;1:135.Classic article on ganglions, reviewing the natural history, anatomy, pathogenesis, and options
for treatment.
Rosson, JW, Walker, G. The natural history of ganglia in children. J Bone Joint Surg Br. 1989;71B:707.
Stephen, AB, Lyons, AR, Davis, TR. A prospective study of two conservative treatments for ganglia of the wrist. J Hand Surg Br. 1999;24;104-
105.
Varley, GW, Needoff, M, Davis, TR, Clay, NR. Conservative management of wrist ganglia: aspiration versus steroid infiltration. J Hand Surg
Br. 1997;22:636-637.
Paul, AS, Sochart, DH. Improving the results of ganglion aspiration by the use of hyaluronidase. J Hand Surg Br. 1997;22:219-221.
Mackie, IG, Howard, CB, Wilkins, P. The dangers of sclerotherapy in the treatment of ganglia. J Hand Surg Br. 1984;9:181.
Thornburg, LE. Ganglions of the hand and wrist. J Am Acad Orthop Surg. 1999;7:231-238.
Rizzo, M, Berger, RA, Steinmann, SP, Bishop, AT. Arthroscopic resection in the management of dorsal wrist ganglions: results with a
minimum 2-year follow-up period. J Hand Surg. 2004;29A:59-62.
Moore, JR, Weiland, AJ, Curtis, RM. Localized nodular tenosynovitis: experience with 115 cases. J Hand Surg Am. 1984;9(3):412-417.Broad
overview on the presentation and management for PVNS of the hand.
Karasick, D, Karasick, S. Giant cell tumor of tendon sheath: spectrum of radiologic findings. Skeletal Radiol. 1992;21(4):219-224.
Uriburu, IJ, Levy, VD. Intraosseous growth of giant cell tumors of the tendon sheath (localized nodular tenosynovitis) of the digits: report of
mebooksfree.net
15 cases. J Hand Surg Am. 1998;23(4):732-736.
Williams, J, Hodari, A, Janevski, P, Siddiqui, A. Recurrence of giant cell tumors in the hand: a prospective study. J Hand Surg Am.
2010;35(3):451-456.
Bliss, BO, Reed, RJ. Large cell sarcomas of tendon sheath: malignant giant cell tumors of tendon sheath. Am J Clin Pathol. 1968;49(6):776-781.
Carroll, RE. Epidermoid (epithelial) cyst of the hand skeleton. Am J Surg. 1953;85(3):327-334.Classic article on epidermal cysts of the hand. The
discussion at the end of this paper between the author and authors of other articles in the journal (not included) is also interesting, particularly because
it highlights that they had come across inappropriate management for epidermal cysts of the hand in their respective practices.
Lincoski, CJ, Bush, DC, Millon, SJ. Epidermoid cysts in the hand. J Hand Surg Eur Vol. 2009;34(6):792-796.
Pagnano, MW, Athanasian, EA, Bishop, AT, Rock, MG. Intraosseous epidermoid cyst in a metacarpal mimicking malignancy. Orthopedics.
1997;20(8):719-721.
Kim, DH, Murovic, JA, Tiel, RL, et al. A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University
Health Sciences Center.
J Neurosurg. 2005;102(2):246-255.
Phalen, GS. Neurilemmomas of the forearm and hand. Clin Orthop Relat Res. 1976;(114):219-222.
Strickland, JW, Steichen, JB. Nerve tumors of the hand and forearm. J Hand Surg Am. 1977;2(4):285-291.Classic article that reviews the nerve
tumors that are encountered in the hand, and discusses the options of management.
Stull, MA, Moser, RP Jr, Kransdorf, MJ, et al. Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 1991;20(1):9-
14.
Knight, DM, Birch, R, Pringle, J. Benign solitary schwannomas: a review of 234 cases. J Bone Joint Surg Br. 2007;89B:382-387.
Park, MJ, Seo, KN, Kang, HJ. Neurological deficit after surgical enucleation of schwannomas of the upper limb. J Bone Joint Surg Br.
2009;91B:1482-1486.
Das Gupta, TK, Brasfield, RD. Solitary malignant schwannoma. Ann Surg. 1970;171(3):419-428.
O’Connor, MI, Bancroft, LW. Benign and malignant cartilage tumors of the hand. Hand Clin. 2004;20(3):317-323, vi.This article provides an
overview of the diagnosis and management for the cartilage tumors that are found in the hand.
Gaulke, R. The distribution of solitary enchondromata at the hand. J Hand Surg Br. 2002;27(5):444-445.
Ablove, RH, Moy, OJ, Peimer, CA, et al. Early versus delayed treatment of enchondroma. Am J Orthop (Belle Mead NJ). 2000;29(10):771-772.
Brien, EW, Terek, RM, Geer, RJ, et al. Treatment of soft-tissue sarcomas of the hand. J Bone Joint Surg Am. 1995;77A:564-571.
Buecker, PJ, Villafuerte, JE, Hornicek, FJ, et al. Improved survival for sarcomas of the wrist and hand. J Hand Surg Am. 2006;31(3):452-455.
mebooksfree.net
C H A P T E R 8 7
mebooksfree.net
Treatment of Vascular Disorders of the Hand
Brian C. Pridgen and James Chang
Key Points
Physical examination with Allen testing and pencil Doppler may be adequate for localization of
obstructive arterial lesions, but angiography is the standard study for vascular imaging.
Hand ischemia in the setting of chronic thrombosis typically is not responsive to medical
management and requires surgical treatment.
Arterial reconstruction requires careful preoperative planning with appropriate vascular imaging to
identify suitable distal targets.
Periarterial sympathectomy is a particularly effective treatment for patients with Raynaud
phenomenon due to scleroderma who have failed treatment with behavioral modification and
medical management.
Following resection or ligation of diseased arterial segments, these defects should be reconstructed
whenever possible with either direct repair if the defect is short enough or interposition vascular
grafts.
Vascular disorders of the hand and associated hand ischemia may result in significant morbidity. Hand
ischemia is caused by a diversity of conditions including local or systemic factors, acute or chronic
processes, and occlusive, vasospastic, traumatic, or anatomic disorders. Proper treatment requires a
detailed understanding of the complex vascular anatomy of the upper extremity, a comprehensive
history and physical examination, appropriate use of diagnostic testing, and knowledge of the various
treatment options for each disorder.
mebooksfree.net
Anatomy
Arterial Anatomy
The primary blood supply to the hand is through the radial and ulnar arteries. The ulnar artery arises
from the brachial artery in the proximal forearm and lies deep to the flexor carpi ulnaris along its course.
Because of the distal muscle belly of the flexor carpi ulnaris, the underlying ulnar artery may be
compressed in some instances and may not be palpable, in contrast to the more superficially located
radial artery at the level of the wrist. Shortly after branching from the brachial artery, the ulnar artery
gives rise to the common interosseous artery, which then forms the anterior and posterior interosseous
arteries. The radial artery also arises from the brachial artery in the proximal forearm. It passes through
the interval between brachioradialis and pronator teres proximally and between brachioradialis and
flexor carpi radialis distally in the forearm. In the distal forearm, the radial artery often anastomoses with
the anterior and posterior interosseous arteries. The interosseous arteries and a persistent median artery
are possible sources of secondary collateral blood supply to the hand in cases of chronic ischemia.
In the hand, the radial and ulnar arteries form the deep and superficial palmar arches. As described in the
classic anatomic study by Coleman and Anson in 1961 1 and in a more recent 2001 anatomic study by
Gellman and colleagues, 2 the palmar arterial anatomy has significant variability.
The superficial palmar arch (Figure 87.1A and B) is formed from the ulnar artery as it crosses the wrist. It
is located deep to the palmar fascia and distal to the deep palmar arch. There is significant variability in
the superficial palmar arch, which can be described as either complete or incomplete depending on the
presence or absence of anastomoses between the dominant ulnar artery contribution to the superficial
palmar arch and the radial contributions to the superficial palmar arch. In Gellman’s anatomic study, a
complete superficial palmar arch was found in 84.4% of specimens and was subdivided into five variants
initiated by the ulnar artery with various contributions from the superficial volar branch of the radial
artery, the median artery, or communicating branches from the deep palmar arch. Branches from the
superficial palmar arch typically include a branch to the deep palmar arch, a proper ulnar digital artery
branch to the small finger, and common digital artery branches to the second, third, and fourth
webspaces.
mebooksfree.net
FIGURE 87.1 Most common variations of the palmar arterial anatomy as found by Gellman and colleagues. A. A complete
superficial palmar arch, as defined by an anastomosis between the vessels constituting it, is most commonly formed by the
dominant ulnar artery inflow and an anastomotic connection to the superficial volar branch of the radial artery. B. An incomplete
superficial palmar arch is most commonly formed by the ulnar artery and the superficial volar branch of radial artery but without
an anastomosis between these two vessels in the palm. C. The deep palmar arch is most commonly formed by the dorsal
branch of the radial artery, which anastomoses with a deep volar branch from the ulnar artery.
The deep palmar arch (Figure 87.1C) is formed from the dorsal branch of the radial artery after it has
passed dorsally deep to the abductor pollicis longus and extensor pollicis brevis tendons and then returns
volarly into the deep palm through the two heads of the first dorsal interosseous. Before returning
volarly, the dorsal radial artery branch typically gives rise to the dorsal carpal rete and the princeps
pollicis artery. The radialis indicis artery may arise either directly from the dorsal radial artery branch or
from the princeps pollicis artery. As the deep palmar arch crosses the palm, it passes deep to the flexor
tendons distal to the distal carpal row. A complete deep palmar arch was found in nearly all patients with
anastomoses to one or both deep volar branches of the ulnar artery in all patients.
mebooksfree.net
Venous Anatomy
Although the arteries of the hand are accompanied by two venae comitantes, the primary venous
drainage of the fingers and hand is through the dorsal veins. These vessels drain into the basilic and
cephalic veins in the forearm.
mebooksfree.net
Evaluation
History
Accurate diagnosis of upper extremity vascular disorders requires a thorough history. Important details
include a history of acute or repetitive trauma, known medical comorbidities including diabetes or
cardiovascular disease, signs of systemic sclerosis or other rheumatologic conditions, drug or tobacco use,
cold intolerance, prior digital ulcerations, and weakness or neurovascular changes. The history may be
supplemented with the use of validated patient questionnaires for initial evaluation and longitudinal
assessment, including the Cold Sensitivity Severity scale developed by McCabe and colleagues. 3
mebooksfree.net
Physical Examination
A thorough examination of the entire upper extremity should evaluate for prior scars or trauma, palpable
masses, color changes including pallor or cyanosis, temperature differences, digital ulcers, and perfusion
of the nail beds. The vascular examination may be supplemented by using a handheld pencil Doppler if
evaluating for obstructive lesions. An Allen test may be used to evaluate for collateral inflow to the hand
through the radial and ulnar artery. Ruengsakulrach and colleagues found the Allen test to be reliable
when compared against Doppler ultrasonography, and when using the Allen test to assess patients for
suitability for radial artery harvest, they had no ischemic complications following harvest of 1657 radial
arteries over a 6-year period. 4 However, the reliability of the Allen test is controversial and has been
questioned in case reports and studies, as reviewed by Brzezinski and colleagues. 5
Capillaroscopy is a technique using high-powered light microscopy to evaluate the morphology and
distribution of the nail bed microvasculature. 6 Visible spectrum evaluation may be augmented with the
use of dynamic fluorescent angiography. Assessment of the nail bed capillaries using this technique is a
useful screening tool to distinguish between patients with primary Raynaud phenomenon (RP) and
secondary RP. These studies typically are performed by rheumatologists and dermatologists to document
distal perfusion and are less commonly used by hand surgeons.
mebooksfree.net
Diagnostic Studies
Vascular imaging often is necessary for diagnosis or surgical planning for vascular disorders of the upper
extremity. 7 Duplex ultrasonography is most useful for providing real-time blood flow evaluation or
larger blood vessels. Computed tomography angiography is a rapid and relatively noninvasive vascular
imaging modality.
8 With recent improvements in image resolution with magnetic resonance
angiography, this may be used when it is desirable to avoid ionizing radiation. However, the standard for
vascular imaging is digital subtraction angiography owing to its superior spatial resolution, particularly
for smaller vessels, and detailed temporal resolution allowing for real-time assessment of vascular inflow
and outflow (Figure 87.2). Angiography also permits concomitant interventional procedures such as
intra-arterial delivery of thrombolytics or thrombectomy. Risks of angiography include inherent risks of
an invasive procedure such as aneurysm or hematoma, nephrotoxicity from higher doses of contrast, and
higher doses of radiation. Surgeons treating vascular disorders of the upper extremity should be
comfortable with proper selection and interpretation of the appropriate vascular imaging studies. 7
mebooksfree.net
FIGURE 87.2 MR angiogram in a patient with scleroderma. Note the poor resolution of the common digital arteries.
mebooksfree.net
Acute Ischemia
Traumatic
Evaluation and management of traumatic injuries to the upper extremity requiring revascularization or
replantation are reviewed in Chapter 83.
mebooksfree.net
Iatrogenic
Iatrogenic ischemia to the hand may result from arterial catheterization or harvest of the radial artery for
either vascular reconstruction or with a radial forearm flap. Thrombosis of the radial and ulnar artery
following catheterization may occur, but it rarely results in hand ischemia. 5,9 Similarly, in appropriately
selected candidates for radial artery harvest, collateral flow and a compensatory increase in flow through
the ulnar artery results in only rarely described cases of hand ischemia. 4
In critically ill patients receiving vasopressors, peripheral vasoconstriction may result in digital ischemia
in up to 2% of patients 10 (Figure 87.3). Treatment typically is limited to local wound care to prevent
progression to wet gangrene followed by eventual amputation of the ischemic digits once the patient has
recovered from their illness. One must, however, rule out concomitant radial artery injury from an
arterial line that may rarely require repair or reconstruction to restore arterial inflow (Figure 87.4).
FIGURE 87.3 Patient who had been on high-dose vasopressors who developed tissue necrosis from hand ischemia with a
clear line of demarcation.
mebooksfree.net
FIGURE 87.4 A. ICU patient on vasopressors with signs of hand ischemia, including cyanotic fingertips and poor turgor. B.
Return of color and turgor without any surgical intervention after warming the patient and removing the radial artery catheter.
Creation of an arteriovenous fistula for hemodialysis access may result in decreased blood flow to the
hand, but only rarely does it cause ischemic changes in the hand because of a steal phenomenon. In one
series, the incidence of hand ischemia was 4.3% after placement of an arteriovenous graft and 1.8% after
creation of an arteriovenous fistula. 11 A steal phenomenon was noted within 1 week in eight of the fifteen
observed cases and within 1 month in twelve of the fifteen cases. Ligation of the arteriovenous access will
reverse the underlying steal phenomenon but leaves the patient without arteriovenous access for
hemodialysis. Various treatments have been described for steal phenomenon, but in a recent large
retrospective review from a single institution, distal revascularization with interval ligation (DRIL) was
noted to have similar outcomes to ligation but with the benefit of preservation of arteriovenous access;
the DRIL procedure had superior outcomes compared with other surgical treatments. 12
mebooksfree.net
Thromboembolism
Common causes of acute arterial occlusion from thromboembolism include embolism from a cardiac
source because of an arrhythmia such as atrial fibrillation, endothelial injury from catheterization or local
trauma, thrombus formation due to an aneurysm, intra-arterial injection of illicit substances, or
hypercoagulable states. 13 Patients may present with pain, pallor, pulselessness, and coolness. Splinter
hemorrhages in the nail beds, fingernail changes, or digital ulcers may be a sign of a more chronic
etiology. Angiography is the study of choice to characterize the level of occlusion, sources of collateral
flow, and possible proximal sources of emboli.
Treatment is directed at the underlying etiology. Proximal thromboembolism often is treated with
embolectomy or surgical bypass as needed. However, more distal obstruction presents additional
challenges owing to the small size of the vessels. Iannuzzi and Higgins reviewed treatment options for
acute arterial thrombosis. 14 For the treatment of associated vasospasm, administration of vasodilators has
had mixed results, and they instead recommend the use of local measures including warming and
sympathetic blocks. Heparin and aspirin may be used to prevent progression of additional arterial
thrombosis. In the absence of medical or surgical contraindications, intra-arterial delivery of
thrombolytics such as tissue plasminogen activator often is considered as an initial treatment. However,
there have been mixed results with thrombolysis, and it typically is most effective in cases of acute
ischemia. 13,14 Thrombolysis may be used alone or as an adjunct to surgical revascularization. When
treating patients with thrombolytics, they should be monitored closely for bleeding complications.
Revascularization procedures should be reserved for cases in which there is a clear site of obstruction that
either can be resected and reconstructed or bypassed to a patent distal target. If offered judiciously for
appropriate indications, revascularization often has high rates of success.
mebooksfree.net
Venous Thrombosis
Isolated deep venous thrombosis of the upper extremity is uncommon and rarely results in clinically
significant problems in the upper extremity. However, in patients with total or near-total thrombotic
occlusion of the deep and superficial veins, they may develop ischemic thrombosis, also known as
phlegmasia cerulea dolens. This is more frequently described in the lower extremity but has been
observed in the upper extremity as well. 15 Following diffuse venous thrombosis, these patients can
develop venous hypertension with resulting elevated interstitial pressures. Patients should be promptly
diagnosed and treated before progressing from cyanosis to ischemia to venous gangrene. Described
treatments include anticoagulation, thrombolysis, or thrombectomy. Additionally, signs of compartment
syndrome should prompt consideration for surgical management with fasciotomies.
mebooksfree.net
Chronic Ischemia
Vasospastic Disease
Raynaud phenomenon (RP), first described by Maurice Raynaud in 1862, is characterized by episodic
vasospasm of the digits. Classically, an episode of RP is triggered by cold exposure or emotional stress
and is described as a triphasic progression of color changes from well-demarcated pallor (white) to
cyanosis (blue) to reactive hyperemia (red) upon reperfusion. These episodes are associated with
paresthesias and burning pain.
Owing to disagreement and conflicting diagnostic criteria described in the literature, a recent consensus
panel of experts established a set of criteria for RP 16 (Figure 87.5). This panel agreed that a diagnosis
requires cold sensitivity as a trigger and biphasic color changes from white to blue during an attack.
Notably, although other triggers such as emotional stress have been described, and triphasic color
changes were classically described, neither of these were determined to be necessary for a diagnosis of
RP. In addition to cold sensitivity and biphasic color changes, three of the following seven criteria must
be met:
1. triggers other than cold,
2. bilateral involvement,
3. associated numbness or paresthesias,
4. well-demarcated areas of involvement,
5. photographic evidence of RP supplied by the patient,
6. involvement of areas other than fingers or toes, and
7. presence of the classic triphasic color changes from white to blue to red.
mebooksfree.net
FIGURE 87.5 Three-step approach summarizing consensus criteria for diagnosis of Raynaud phenomenon. If three or more
criteria are met from step 3, the patient has Raynaud phenomenon.
(From Maverakis, E, Patel, F, Kronenberg, DG, et al. International consensus criteria for the diagnosis of Raynaud’s phenomenon. J Autoimmun. 2014;48-
49:60-65, with permission.)
mebooksfree.net
Other diagnostic criteria that had been previously described were not sufficiently agreeable among the
panel members. Among patients who meet diagnostic criteria for RP, additional criteria for diagnosis of
primary RP include normal capillaroscopy, no evidence of secondary causes or connective tissue disease,
and negative or low antinuclear antibody titers.
RP may be subcategorized as either primary (Raynaud disease) if there is no underlying disorder or
secondary (Raynaud syndrome) if there is an underlying systemic condition, including rheumatologic
diseases such as systemic sclerosis (scleroderma), systemic lupus erythematosus, dermatomyositis,
Sjögren syndrome, mixed connective tissue disease, or rheumatoid arthritis. 17,18 Other conditions that
have been associated with RP include hematologic disorders, environmental exposures including
mechanical injury from chronic vibrations, other vasospastic disorders such as migraines and Prinzmetal
angina, and certain medications including serotonin agonists, beta-blockers, interferons, and certain
chemotherapy agents. 17,18 Unlike in primary RP which is painful but typically has a benign course,
secondary RP often is associated with significant morbidity including digital ulceration and gangrene.
Owing to regional variations and variable diagnostic criteria for RP, reported prevalence rates range from
1% in men to 30% in women across various studies. 19 A meta-analysis of epidemiology studies limited to
primary RP among the general population demonstrated a prevalence of 1.6% to 7.5% for definite
primary RP; the prevalence of possible primary RP was found to be 4.0% to 12.7%. 20 Risk factors included
female gender, family history in a first-degree relative, smoking, and migraines. In two longitudinal
studies of patients with primary RP who were screened at the onset of the study for associated
underlying conditions, there were no cases of progression to secondary RP, and in many patients, their
primary RP resolved. 21,22 However, other studies have found progression of primary RP to secondary RP
in a small number of patients; the difference in these findings may be explained by careful screening for
underlying conditions at the initial evaluation in the cited studies.
Although the mechanism of RP remains poorly understood, it is thought to be mediated by sympathetic
alpha-adrenergic innervation, which often is cold induced. 23 This vasoconstrictive process may be
exacerbated by endothelial cell dysfunction, including increased endothelin-1 activity and decreased
nitric oxide activity.
Patients with primary RP often can be managed with behavior modifications such as cold avoidance,
smoking cessation, and avoidance of certain medications such as sympathomimetics and migraine
medications, without any additional medical or surgical intervention. 17 Treatment typically is reserved
for patients with refractory primary RP or with secondary RP. A meta-analysis of medical treatments for
RP demonstrated strong evidence supporting the use of calcium channel blockers and iloprost, limited
evidence supporting the use of atorvastatin, and conflicting evidence for the use of ketanserin and
bosentan. 24 Additional treatments evaluated include prazosin, cisaprost, cyclofenil, angiotensin-
converting enzyme inhibitors, antioxidants, and tadalafil, but none were found in this meta-analysis to be
of benefit. A more recent meta-analysis
of randomized controlled trials evaluating phosphodiesterase-5
inhibitors including sildenafil and tadalafil demonstrated modest improvements in symptoms, duration,
and frequency of attacks. 25 However, owing to the limited availability of high-quality clinical trials,
treatment algorithms often are based on expert opinion and clinical experience. 17
Injection of botulinum toxin for treatment of RP was first reported in 2004. 26 Additional work by
Neumeister demonstrated improvements in symptoms and refinements of the injection technique. 27
A
recent prospective, single-blind randomized trial of botulinum toxin B injections in patients with
scleroderma, demonstrated improvements in symptoms, skin temperature recovery after cold immersion,
and digital ulcers, in patients receiving higher dose treatments compared with untreated controls. 28 The
mechanism of botulinum toxin for treatment of RP is not completely understood but is thought to be
related to inhibition of sympathetic-mediated vasoconstriction and inhibition of substance P-mediated
pain signaling. 27
mebooksfree.net
Surgical treatment with periarterial sympathectomy may be indicated in patients who fail medical
management and have persistent digital pain or ulcerations, particularly in patients with systemic
sclerosis (Figure 87.6). In the early 20th century, Leriche described his extensive experience with
performing sympathectomies for a variety of indications, including periarterial sympathectomies for
Raynaud disease and scleroderma. 29 Though more proximal procedures such as cervical or thoracic
sympathectomies may be limited because of high recurrence rates, the digital artery sympathectomy
described by Flatt in 1980 30 continues to be used for treatment of patients with refractory RP. A 2003
systematic review by Kotsis and Chung of patients undergoing digital sympathectomy for digital
ischemia due to RP found that 14% of patients required digital amputation, 18% had ulcer recurrence or
incomplete healing, and 37% experienced a postoperative complication. 31 They noted that their study
was limited by heterogeneity and low quality of the published studies available for review. Subsequent
reports by other groups have shown excellent results with periarterial sympathectomy in appropriately
selected patients 32–35 and possibly decreased rates of ulcer recurrence in patients who undergo concurrent
vascular bypass of occluded arterial segments. 33 Various techniques have been described by these groups
and reviewed by others, 36 but the underlying principles and goals of the operation are to (1) disrupt
sympathetic tone to the palmar and digital vessels by stripping the periarterial adventitia (Figure 87.7)
and dividing the nerve of Henle arising from the ulnar artery, 37 (2) perform distal revascularization if
there is proximal occlusion and there are appropriate targets distally 33 (Figure 87.8), and (3) debride
digital ulcers. However, for a variety of reasons including limited access to hand surgeons because of
referral patterns from rheumatologists and surgeon availability, this operation is still not widely
performed. 38
mebooksfree.net
FIGURE 87.6 A. Angiogram of a patient with scleroderma with decreased flow in the common digital arteries and the proper
digital arteries. B. The same patient undergoing sympathectomy of the radial and ulnar arteries at the wrist and the superficial
arch and common digital arteries in the palm. Note the dilated common digital arteries.
mebooksfree.net
FIGURE 87.7 Patient with scleroderma who underwent sympathectomy 4 weeks earlier on the left hand. Note the improved
color to the fingers (compared with the unoperated right hand), the healing left middle finger ulcer, and the healing incisions.
mebooksfree.net
FIGURE 87.8 This patient with scleroderma had an occluded ulnar artery. She underwent interposition vein graft from the
proximal ulnar artery to the common digital artery.
mebooksfree.net
Aneurysm and Thrombosis
True aneurysms result from dilation of all three layers of the arterial wall (Figure 87.9). False or
pseudoaneurysms lack the intimal layer with endothelial cells and instead involve only the media and
adventitia. Aneurysms often present as a painless, palpable, pulsatile mass that occasionally is associated
with signs of local nerve compression. Ischemia is uncommon unless there is associated thrombosis and
embolism.
FIGURE 87.9 A. Angiogram of a patient with a true aneurysm extending from the ulnar artery into the common digital arteries.
B. The entire aneurysm is dissected free. C. The ulnar artery and the common digital arteries were reconstructed with a Y vein
graft.
Traumatic thrombosis of the ulnar artery was termed hypothenar hammer syndrome by Conn and
colleagues in 1970. 39 It is classically described in patients who use the ulnar aspect of their hand as a
hammer or are subject to repetitive trauma at this site, such as jackhammer operators, although it may
also result from an isolated trauma. As the ulnar artery emerges from the Guyon canal, it is in a relatively
superficial and unprotected position on the superficial aspect of the hamate where it is subject to trauma
from external forces. This can result in formation of a true aneurysm or pseudoaneurysm. Patients are
then at risk for thrombus formation, which can lead to secondary occlusion of the ulnar artery or distal
embolization.
Among patients presenting to subspecialty clinics for evaluation of RP, 1.1% and 1.6% of patients in two
separate studies were found to have hypothenar hammer syndrome upon further evaluation. 40,41 In these
studies, nearly all patients were men, smokers, and had occupational or hobby-related exposures to
repetitive hand trauma. Patients typically present with unilateral vascular insufficiency to the small, ring,
or middle fingers; involvement of the thumb strongly suggests an alternative diagnosis. In some patients,
mebooksfree.net
the small finger is spared because of the proper ulnar digital artery to the small finger arises proximal to
the Guyon canal. Symptoms may include pain, cold intolerance, and numbness or weakness due to local
compression of the ulnar nerve. Examination findings may include a pulsatile mass at the level of the
wrist, subungual splinter hemorrhages, ischemic changes in the fingers, or digital ulceration.
Noninvasive diagnostic testing includes Allen test, handheld pencil Doppler to evaluate for the level of
occlusion of the ulnar artery and digital blood flow, and duplex ultrasound. Angiography is the standard
for diagnosis of hypothenar hammer syndrome and can be used to rule out more proximal embolic
sources. The pathognomonic corkscrew sign, first described by Hammond and colleagues, 42 is an early
sign of chronic damage to the intima and media leading to progressive fibrosis with ectasia. Additional
angiographic findings include occlusion or aneurysm at the level of the hamate and multiple embolic
occlusions of the digital arteries. 43 Angiography also facilitates operative planning by defining the level
of involvement of the ulnar artery, evaluating for thrombotic occlusion and/or aneurysm, assessing
perfusion and anatomy of the palmar arch, and determining inflow through the digital arteries.
Treatment of patients is guided by severity of symptoms and should restore inflow to the hand while
reducing the risk of additional thrombotic or embolic events. Yuen and colleagues outline a
comprehensive treatment algorithm 44 (Figure 87.10). Nonoperative treatment often includes behavioral
modification, smoking cessation, and medical management with vasodilators and antiplatelet agents. In
one series, 83% of patients were treated successfully with medical management alone. 41 For acute
thromboembolic occlusion with associated ischemia, antiplatelet and thrombolytic therapy may be
administered as described previously. If the thrombosis remains unresolved or if an aneurysm is present
that may increase the risk of future thromboembolic events, surgical intervention is indicated. Rates of
surgical intervention for patients presenting with hypothenar hammer syndrome range from 17% to 71%
in two separate studies. 40,41 Surgical options include resection or ligation of the aneurysmal segment.
Even if there is adequate distal flow with backfilling into the distal ulnar artery, the defect should be
reconstructed with either direct repair if the defect is short enough or reconstruction with an
interpositional vein or artery graft (Figure 87.11). Although vein grafts typically are longer, more easily
harvested, and have less associated donor site morbidity, arterial grafts are more physiologic and may
have improved long-term outcomes. A systematic review comparing venous and arterial grafts for upper
extremity vascular reconstruction or bypass demonstrated 100% patency with arterial grafts at an average
follow-up of 21 months compared with 82% patency of venous grafts at 40 months. 45 Arterial graft donor
sites include the descending branch of the lateral femoral circumflex artery, deep inferior epigastric
artery, thoracodorsal artery, or subscapular artery; the contralateral radial artery should not be harvested
because of the high incidence of occlusion or ectasia of the ulnar artery seen in the contralateral
asymptomatic extremity in a study of patients with hypothenar hammer syndrome. 40 Hui-Chou and
McClinton suggest using arterial grafts in younger and more active patient populations. 46 In a systematic
review of patients who underwent nonoperative treatment for hypothenar hammer syndrome, 12% of
patients had a complete response to treatment and 70% had a partial response to treatment; among those
who underwent operative treatment, 43% of patients had a complete response to treatment and another
43% had a partial response to treatment. 43
mebooksfree.net
FIGURE 87.10 Suggested treatment algorithm for management of hypothenar hammer syndrome. *Fibrinolysis (or
thrombolysis) is advocated only for treatment in cases of acute onset of digital ischemia (less than 2 weeks).
(From Yuen, JC, Wright, E, Johnson, LA, Culp, WC. Hypothenar hammer syndrome: an update with algorithms for diagnosis and treatment. Ann Plast Surg.
2011;67(4):429-438
, with permission.)
mebooksfree.net
FIGURE 87.11 A. Angiogram of a patient with hypothenar hammer syndrome and aneurysm of the ulnar artery in the palm. B.
The ulnar artery aneurysm is dissected free. C. Because the ulnar artery has some laxity, a direct end-to-end repair was
performed after resection of the aneurysm.
(From Galvez, MG, Chang, J. Upper extremity arterial reconstruction and revascularization distal to the wrist. In: Dalman, RL , ed. Operative Techniques in
Vascular Surgery. Philadelphia, PA: Wolters Kluwer; 2015, with permission.)
mebooksfree.net
Buerger Disease
Buerger disease, also known as thromboangiitis obliterans, is an inflammatory disease of the small- and
medium-sized arteries of the upper and lower extremities. 47 Tobacco exposure is the primary risk factor,
and it occurs most commonly in young men. Patients typically present with claudication and signs of
limb ischemia including rest pain and ischemic ulcerations. Discontinuation of tobacco use is the only
definitive treatment. Owing to the diffuse involvement of small caliber vessels that would serve as distal
targets, bypass and revascularization procedures typically are not successful.
mebooksfree.net
Vascular Anomalies
A comprehensive review of the diagnosis and management of vascular anomalies is beyond the scope of
this chapter and is reviewed in more detail in Chapter 27. Upton and colleagues reviewed their 28-year
experience in 270 patients with vascular anomalies of the upper extremity. 48 Although their descriptions
of vascular anomalies predate the current naming conventions, they found that venous malformations
comprised 46% of the patients in their series and that slow-flow venous, lymphatic, capillary, or
combined malformations (Figure 87.12) comprised 88% of the patients in their series. They outline
management algorithms and describe surgical principles for excision of slow-flow venous malformations,
including delicate soft-tissue handling, meticulous dissection, ensuring complete excision, avoiding wide
dissection planes resulting in devascularization, staged operations if necessary, and obtaining careful
hemostasis. Complications after excision occurred in 22% of slow-flow lesions and 28% of fast-flow
lesions; the most common complications included seroma/hematoma, amputation, neuroma, and wound
healing complications.
FIGURE 87.12 A. This patient had an arteriovenous malformation of the left wrist and forearm causing pain. B. The
arteriovenous malformation was dissected free under tourniquet control. C. One arterial feeder from the radial artery was found
and ligated.
Glomuvenous malformations (GVMs), previously known as glomus tumors, are a vascular anomaly most
commonly found in the upper extremity. In one large series, all GVMs were located in the distal phalanx
and 59% were in the subungual region. 49 This is due to the high concentration of glomus bodies in this
region, which are neuromyoarterial cells important for peripheral thermoregulation. Patients typically
mebooksfree.net