FIG 1 • Wrist and forearm anatomy in radial longitudinal
deficiency (type III is shown). The fibrous anlage, a
remnant of the radius, tethers the wrist radially. The
radial wrist extensors and flexors fuse into a
“dorsoradial muscle mass.” The radial carpal bones are
absent as is the thumb.
FIG 2 • Bayne and Klug classification of RLD. A. Radial
deviation and palmar subluxation are worse with
increasing hypoplasia of the radius. B. Representative
radiographs of RLD types I to IV.
■ Modifications to the original classification system includes introduction of
three additional types:
■ Type N—Thumb hypoplasia with normal radius
■ Type 0—Absent or hypoplastic carpal bones with normal radius
■ Type V—Humeral abnormalities
PATHOGENESIS
■ RLD occurs in 1 in 30 000 to 1 in 100 000 live births. Bilateral and unilateral
cases occur in equal frequency.
■ The cause of RLD is unknown. Environmental and genetic factors are likely
responsible for developmental anomalies in the apical ectodermal ridge (AER)
between the 4th and 7th weeks of gestation. Animal models with radial
deficiencies can be created by ablation of the radial section of the AER.
■ RLD is considered to be a malformation, based on the Oberg, Manske, and
Tonkin classification of 2010.1
NATURAL HISTORY
■ If untreated, radial deviation usually worsens and the wrist develops stiffness.
■ Radial deviation usually recurs to some extent even after surgical correction
unless the ulnocarpal joint has been fused.
■ Although the forearm does grow, it usually grows slower than the normal
forearm and ends up about 60% of normal length.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ The infant’s shoulder, elbow, and wrist should be examined.
■ The degree of wrist deviation and the capacity for passive correction should be
noted and recorded.
■ The status of the thumb and the radial fingers should be noted, especially in
terms of intrinsic muscle development, passive motion of the joints, flexion
and extension creases, and the presence of contractures.
■ RLD is frequently associated with other conditions (70%–90%) and the
likelihood increases with severity.2 Bilateral involvement increases the
likelihood of associated conditions by over 30%.
■ Although a long list of possible conditions can occur with RLD, the most
common are the VACTERL association, Holt-Oram syndrome, craniofacial
microsomia, and Fanconi anemia.
IMAGING
■ Plain radiographs delineate the degree of involvement and help with
classification.
■ The hand-forearm angle (HFA) and the hand-forearm position (HFP) should
be measured on the anterioposterior radiograph (FIG 3).
■ HFA is the angle between the longitudinal axes of the long finger and the
distal ulna.
■ HFP is the shortest distance between a line drawn through the longitudinal
axis of the distal ulna and the proximal pole of the fifth metacarpal.
■ These measures can be followed over time and growth to assess long-term
success of surgical correction vs nonoperative treatment.
DIFFERENTIAL DIAGNOSIS
■ Thrombocytopenia-absent radius (TAR) syndrome is a genotypically distinct
entity, although the treatment is similar.
■ Patients have hypomegakaryocytic thrombopenia with a completely absent
radius. In contrast to RLD with absent radius (type IV), patients with TAR
have thumbs.
■ TAR is an autosomal-recessive condition linked to a defect in chromosome
1q21.1.
■ Clinical findings include bruising, hemorrhage, and cardiac and renal
anomalies.
■ The thumb is biphalangeal and contains varying degrees of skeletal and thenar
muscle hypoplasia.
FIG 3 • Measuring hand-forearm angle (HFA) and hand-
forearm position (HFP) on the plain radiograph.
NONOPERATIVE MANAGEMENT
■ For most patients, nonoperative management with splinting and stretching is
instituted early in infancy.
■ This type of treatment maybe a long-term solution for those with mild RLD
(types 0, I, and II) or a short-term “bridge” solution in preparation for
subsequent surgery (types III and IV).
■ The family performs stretching about 8 to 10 times per day by applying
longitudinal traction and ulnar deviation.
■ A form-fitting radial gutter splint is then used to keep the wrist in the
corrected position.
■ Early on, as the family strives for a neutral wrist, the splint will need to be
worn at all times and will need frequent revisions. Once the wrist is passively
correctable to neutral, the splint can be weaned to night use only.
■ Splinting is also a critical postoperative adjunct to keep the wrist in neutral; as
the natural history of this condition is progressive radial deviation of the wrist
with growth.
■ Treatment may not be indicated at all in the following:
■ Adults who have adapted well
■ Patients who cannot tolerate stretching and splinting (eg, develop hygiene
problems such as rash)
■ Patients in whom operative treatment poses unnecessary medical risk
■ Patients whose function will worsen with a straight wrist (eg, patients with
stiff elbows who may be unable to wipe or feed themselves with the hand).
SURGICAL MANAGEMENT
■ Types 0, I, and II RLD are usually treated with stretching and splinting. In rare
instances where these measures are ineffective, surgical management can
include tendon transfers and/or distraction lengthening.
■ Types III and IV RLD can be treated with stretching and splinting first, as a
“bridge” intervention, to bring the wrist into neutral prior to formal repair.
However, this intervention alone is usually not sufficient to allow the carpus to
translate beyond the distal ulna.
■ In most cases, preoperative soft tissue distraction is necessary to achieve
satisfactory positioning.2 This procedure involves application of a (usually)
uniplanar distractor without an osteotomy. Once slow distraction (1 mm/d)
moves the carpus beyond the distal ulna, formal centralization or radialization
can be performed (FIG 4).
■ In early reports, centralization involved resection of the distal ulna, but this led
to early growth arrest. Thereafter, carpal resection was introduced (carving a
notch in the carpus to accommodate the distal ulna), which helped with
alignment and avoided growth arrest, but led to stiffness and recurrence.
■ To remedy these issues, Buck-Gramcko introduced radialization, which
involves:
■ Stabilization of the radial carpal column onto the distal ulna without carpal
resection.
■ Transfer of the dorsoradial muscle mass ulnarly to counteract the deforming
forces and provide long-lasting correction.
■ There is debate about what constitutes centralization vs radialization.
■ Traditionally, centralization involves carving of a carpal notch for insertion of
the ulnar head and alignment of the base of the third metacarpal with the
central carpus and the distal ulna (FIG 5A).
■ In contrast, radialization involves alignment of the second metacarpal with
the radial carpus without carpal resection (FIG 5B).
■ There continues to be disagreement about which of these options is best. Some
argue that stabilization of the wrist (radialization) without a carpal notch does
not provide a stable equilibrium, whereas others prefer to preserve motion and
avoid shortening.
■ Nevertheless, most surgeons agree that for the operation to be effective:
■ The fibrous anlage and dense soft tissues should be released or excised.
FIG 4 • The process of soft tissue distraction is
demonstrated. The deficient forearm reaches about
60% of the length of a normal one.
■ The dorsoradial muscle mass should be transferred to the ulnar wrist.
■ Alignment should be maintained with a pin that is kept in for several months,
if not longer.
■ Microvascular free toe joint transfer involves transfer of vascularized osseous
structural support to stabilize the radial column of the wrist. Soft tissue
distraction is usually necessary to align the wrist and create a “space” into
which the toe will be placed. The metatarsal and proximal phalanx of the
second toe are transferred with their growth centers and attached to the distal
ulna.
■ Although technically demanding, the operation provides the advantage of
avoiding injury to the distal ulnar physis and preservation of wrist motion.
■ The time to perform corrective surgery is about 1 year of age, barring small
patient size and/or associated comorbidities.
Preoperative Planning
■ Review risks, benefits, and expectations with parents on the day of surgery.
Specifically outline the risks of possible wrist stiffness, poor growth, and
persistent or recurrent deviation.
■ The long-term outcomes and natural history of this condition should be
discussed well before the operative date.
■ Fluoroscopy, drills, and pins will be required for the procedure.
Positioning and Approach
■ The patient is positioned supine with the arm out on a small hand table.
■ Incisions are planned carefully prior to use of the tourniquet.
■ A transverse dorsoulnar approach is usually undertaken. There is usually no
need to extend incisions along the entire dorsal forearm.
FIG 5 • Centralization vs radialization. A. For
centralization, a transverse distal wrist incision is made,
the “radian” nerve identified, and the tight radial fibrous
anlage released. All tethering soft tissues must be
release. A small notch is carved into the carpus to allow
introduction of the ulnar head. The wrist is reduced and
stabilized with a pin aligning the third metacarpal with
the ulnar head. The ECU is advanced. The dorsoradial
muscle mass is transferred to the ulnar wrist extensors.
A corrective osteotomy of the ulna may be necessary if
the bowing is significant. Excess skin on the ulnar side
of the wrist is excised. B. In radialization, the
maneuvers are similar to those for centralization except
that the carpal bones are not excised. Instead, the
carpus is stabilized over the distal ulna and held there
with a pin. The hand is more ulnarly deviated to provide
a better moment arm for the musculature to keep the
wrist in the proper posture. The pin aligns the second
metacarpal with the distal ulna.
T E C H N I Q U E S
■ Preoperative Soft Tissue Distraction
■ Consult manufacturer instructions for application of distractor of choice.
■ The authors prefer a uniplanar distractor as it is easy to apply and manage
and effective at achieving the desired result.
■ If the wrist is not passively correctable to 50 to 60 degrees of radial
deviation (TECH FIG 1A,B), a concomitant releasing procedure may be
necessary to obtain adequate alignment for placement of the distractor.
■ In this case, plan a transverse incision dorsally on the ulnocarpal joint for
exposure of the radial fibrous anlage and tethered soft tissues.
■ Plan small longitudinal incisions over the third metacarpal and the proximal
ulna. Plan the length of these incisions based on the length of the distractor
clamp that will house the pins. Assemble the apparatus and obtain all needed
parts before the tourniquet is inflated.
■ Exsanguinate the upper extremity.
■ If performing a release, make the dorsal wrist incision and expose the fibrous
anlage from the dorsal approach (TECH FIG 1C).
■ Take care to avoid injury to the “radian” nerve and the extensor tendons.
■ Release the tough radial soft tissues (TECH FIG 1D).
■ Incise over the third metacarpal and the proximal ulna. Spread the soft
tissues; retract and protect any longitudinally running vital structures (tendon,
muscle) until down to bone.
■ Insert one pin into the metacarpal and another into the ulna.
■ Arrange the distractor clamps onto the pins and tighten slightly. Apply the
lengthening bar while manually distracting the wrist; secure the clamps onto
the bar.
■ Insert another pin into the metacarpal and one more into the ulna, each into
the second seat of the respective distractor clamp.
■ Loosen the clamps from the bar. Distract the soft tissues as much as possible
and secure the clamps back onto the bar tightly.
Flexor carpi- ulnaris Fibrous anlage Dorsoradial muscle mass Extensor carpi ulanris D
TECH FIG 1 • Preoperative clinical (A) and
radiographic (B) images of 6-month-old girl with type
IV RLD. C. If the wrist is tight and significantly radially
deviated, the fibrous anlage and tough tethering
tissues should be released prior to placing the
distractor. D. Releasing these structures allows proper
alignment of the distractor and makes the process of
distraction smoother. E,F. After soft tissue distraction
and immediately prior to centralization, the deviation
and subluxation of the wrist has been corrected.
■ Close all the wounds and apply a soft dressing.
■ The family can start distraction at 1 mm/d in about 3 to 4 days.
■ Instruct the family on how to perform pin-site care to avoid infection.
Provide written instructions and a timetable or turning the screw, and mark
the distance between the pins so they can see the progress (and avoid
turning the wrong way).
■ It helps if they send weekly pictures via e-mail so the surgeon can monitor
the progress.
■ Once the carpus has translated beyond the distal ulna, leave the distractor on
without distracting for another several weeks.
■ At that point, the patient will be ready for formal centralization or
radialization (TECH FIG 1E,F).
■ Centralization or Radialization
■ If preoperative distraction has been performed, the distractor can be removed
at the time of the centralization or radialization procedure.
■ A transverse dorsoulnar incision is planned over the ulnocarpal joint. Extra
skin should be reserved until the end where the exact amount can be carefully
assessed prior to removal.
■ The authors do not routinely employ local Z-plasty or transposition flaps.
The use of preoperative soft tissue distraction has virtually eliminated any
problems with skin deficiency.
■ Exsanguinate the extremity.
■ Incise the skin; identify and protect any veins that can be saved. Identify the
fibrous anlage, the radian nerve, the dorsoradial muscle mass, the extensor
tendons to the digits, and the extensor carpi ulnaris tendon.
■ The radian nerve is usually radiodorsally translated and located just under
the skin on the radial aspect of the wrist (TECH FIG 2A–C).
■ Release the fibrous anlage and the dorsoradial muscle mass. Release any
other tight investing fascia.
■ Release the extensor carpi ulnaris from its attachment on the fifth digit
metacarpal. This tendon will be advanced to provide more ulnar pull.
■ Incise the wrist capsule transversely.
■ If performing traditional centralization, carve a small notch into the carpus to
accommodate the distal ulna. Otherwise, no carpal resection is needed.
■ Align the third metacarpal (centralization; see FIG 5A) or second
metacarpal (radialization; see FIG 5B) with the distal ulna.
■ A 0.062-in. Kirschner wire can now be passed anterograde through the
wrist entering the base of the chosen metacarpal. The authors prefer to
insert the pin obliquely to avoid the metacarpophalangeal joint. Thus, the
pin would come out of either the first (if second metacarpal) or second (if
third metacarpal) web space.
■ Insert the distal ulna into the carpal slot (centralization) or stabilize the
carpus over the distal ulna (radialization). Drive the K-wire down the ulna in
retrograde fashion.
■ If necessary, perform an opening wedge ulnar osteotomy to correct any
bowing of the ulna and drive the pin further proximally to secure this
osteotomy.
■ Close the wrist capsule securely.
■ Advance the extensor carpi ulnaris and reattach to the base of the fifth digit
metacarpal. Transfer the dorsoradial muscle mass to the extensor carpi ulnaris
tendon to provide additional ulnar moment arm to the wrist.
■ Excise any redundant skin and close using absorbable sutures. Apply
antibiotic-impregnated gauze to all incisions and cover with sterile gauze.
Wrap loosely.
■ Apply a long-arm cast.
TECH FIG 2 • Preoperative clinical (A) and
radiographic (B) images of an 8-month-old boy with
type IV RLD showing significant deviation. His family
declined preoperative soft tissue distraction but did
perform stretching and splinting such that the wrist
could be passively corrected to 20 degrees of radial
deviation. The “radian” nerve is the most superficial
structure on the radial side of the wrist. C. It is
demonstrated here under the green background. A
smaller dorsal sensory branch (sensory radial nerve
equivalent) is looped and visible dorsally (black
arrow). The expected path of the nerve was marked
on the skin preoperatively (white arrow).
PEARLS AND PITFALLS
Technique
■ The pins may be left in for a long time; in fact, some surgeons
leave the pins in for a year or longer. Although this may increase
stability, it decreases range of motion and can result in physeal
injury and hardware failure.
■ The “radian” nerve is the most superficial structure on the radial
side of the wrist; the surgeon should be cautious to avoid injury
while dissecting in this area.
Postoperative
■ Cast immobilization time should be limited, only to allow the soft
tissues to heal. These children have short forearms and can easily
escape from their casts.
Therapy
■ It is critical to instruct the parents to move the fingers passively
during preoperative soft tissue distraction. This action prevents
stiffness and contractures.
POSTOPERATIVE CARE
■ Remove the cast after 2 or 3 weeks and apply a thermoplast splint. Range-of-
motion exercises of the digits can be started at this time.
■ Leave the pin in for 8 to 10 weeks.
OUTCOMES
■ Long-term outcomes for the two cases depicted are shown in FIG 6.
■ The main unfavorable outcome seen after centralization or radialization is
recurrence of the deviation. The recurrence appears to be related to the
preoperative degree of deviation and amount of correction achieved
postoperatively.2
■ Rigorous compliance with night splinting may decrease the risk of recurrence
(FIG 7).
■ Despite the leveling and realignment of skeletal support from the remaining
carpus and hand, the muscular imbalance across the wrist remains the
primary cause for recurrent deviation.
■ Despite the risk of recurrence, Kotwal et al.3 showed that surgical treatment of
RLD is sensible. He compared patients who had undergone surgery to those
who had not, showing that surgical treatment of RLD improves appearance and
function, and leads to better performance in daily activities.
■ Although the focal point of treatment is correction of the radial deviation, the
importance of this concern has been questioned. Recent studies have shown
that wrist and digit range of motion as well as grip strength and forearm length
may be better than wrist angulation in predicting functional outcomes in RLD.4
■ Hence, it may be worthwhile to avoid carpal resection, thereby sacrificing
some loss in position of the wrist in favor of better motion.
FIG 6 • A. The case in TECH FIG 1 year after
radialization. B,C. Long-term postoperative images after
centralization for the case in TECH FIG 2. A carpal notch
was required to adequately reduce the wrist.
COMPLICATIONS
■ Infections during soft tissue distraction are frequent and range from minor pin-
tract infections to cellulitis and, rarely, deep bone infection. These problems are
avoided with good pin-site care and close follow-up.
■ Other complications with the distraction include inadequate distraction by the
parents, device malfunction or malalignment requiring repair, and stiffness
and/or contracture of the digits. These problems can be avoided with good
parent education and support as well as passive range-of-motion exercises.
■ Complications of centralization or radialization include wound infections and
hardware problems. Because the pin has to be in place for many weeks, it may
migrate, dislodge, or break.
■ One of the most concerning complications is early growth arrest of the distal
ulna, since it will cause additional shortening of an already short forearm.
■ This issue can be avoided by earlier removal of the pin and minimal
manipulation of the distal ulna during the corrective operation.
■ Physeal damage may also occur with rapid, aggressive distraction in young
children.
FIG 7 • Long-term result after centralization. A. Initial
radiograph showing type IV RLD. After centralization
and 12 weeks of pin fixation (B) and 14 years later (C).
At long-term follow-up, the wrist maintained adequate
alignment. This child was compliant with nightly splint
use. Note the index finger has been pollicized.
REFERENCES
1. Oberg KC, Feenstra JM, Manske PR, Tonkin MA. Developmental biology and
classification of congenital anomalies of the hand and upper extremity. J Hand
Surg [Am]. 2010;35(12):2066-2076.
2. Taghinia AH, Al-Sheikh AA, Upton J. Preoperative soft-tissue distraction for
radial longitudinal deficiency: an analysis of indications and outcomes. Plast
Reconstr Surg. 2007;120(5):1305-1312.
3. Kotwal PP, Varshney MK, Soral A. Comparison of surgical treatment and
nonoperative management for radial longitudinal deficiency. J Hand Surg Eur
Vol. 2012;37(2):161-169.
4. Ekblom AG, Dahlin LB, Rosberg H-E, et al. Hand function in adults with
radial longitudinal deficiency. J Bone Joint Surg Am. 2014;96(14):1178-1184.
70
CHAPTER
Excision of Vascular
Malformation of the Hand
Amir H. Taghinia
DEFINITION
■ Vascular anomalies fall into two main categories: tumors and malformations.
Tumors, such as hemangiomas, exhibit cellular hyperplasia. In contrast,
malformations represent a localized defect in vascular morphogenesis.
■ Vascular malformations can be broadly classified into slow-flow and fast-flow
lesions.
■ Slow-flow lesions include capillary, lymphatic (LM), and venous (VM)
malformations or mixed lesions.
■ Fast-flow lesions include arteriovenous malformations (AVM).
ANATOMY
■ Vascular anomalies can occur anywhere in the hand and upper extremity, and
can involve all components of soft tissue, bone, and joint. When present, they
can alter or distort the normal anatomy.
PATHOGENESIS
■ The exact etiology of vascular anomalies is the subject of intense scientific
investigation.
■ Somatic activating mutations in the phosphatidylinositol-3-kinase
(PIK3CA)/mammalian target of rapamycin (mTOR) pathway appear to
underlie a number of overgrowth phenotypes with vascular anomalies.1
NATURAL HISTORY
■ Hemangiomas have a biphasic growth phase with proliferation early in life and
subsequent involution.
■ Malformations have a propensity to slowly expand throughout life.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ With a few exceptions, hemangiomas are usually absent at birth or present as a
small stain.
■ Patients present with a growing, vascular, high-flow lesion in early infancy.
■ These lesions have a deep red appearance and strong Doppler signals
throughout.
■ Vascular malformations can present any time in life. Although they are likely
present at birth, they may be clinically silent for years. They usually present
with a combination of discoloration, disproportionate growth, swelling, and
pain (FIG 1).
FIG 1 • A,B. AP and lateral views of a growing, painful
mass on the palm of a 7-year-old boy (the example
case throughout).
FIG 2 • T2-weighted (A) and T1-weighted
postgadolinium (B) MRI images of the lesion.
■ LMs may also develop drainage or infections, and AVMs can develop
paradoxical ischemia and ulceration.
■ Most vascular malformations can be correctly diagnosed by physical
examination. A multidisciplinary approach to diagnosis and treatment gives the
best outcomes.
IMAGING
■ Ultrasonography is the best initial radiological examination for these lesions. It
can be done in the clinic room during the first multidisciplinary visit. The type
and extent of the lesion can be determined quickly and easily.
■ For surgical planning purposes, however, more extensive imaging is usually
required. An MRI is the best soft tissue imaging tool for these lesions (FIG 2).
Angiography is helpful for high flow AVMs.
DIFFERENTIAL DIAGNOSIS
■ The myriad of multiple overlapping conditions that include vascular
malformations can be confusing. Although these lesions can be solitary, the
presence of associated conditions is often the rule rather than the exception. For
an extensive list of associated conditions, the reader is referred to other
readings.2
■ Differential diagnosis includes overgrowth conditions, malignancies, and a
variety of other benign conditions. When in doubt, biopsy is recommended.
NONOPERATIVE MANAGEMENT
■ Hemangiomas are often treated conservatively with no intervention or with
medical therapy including steroids and beta-blockers. Surgery has a minimal
role in the treatment of these lesions.
■ Most malformations are amenable to interventional radiological treatments,
which involve sclerotherapy and embolization. Certain lesions cannot be
treated this way—notably, microcystic lymphatic malformations—and thus
require surgery.
■ In the hand and upper extremity, the authors advocate against sclerotherapy of
lesions close to neurovascular structures unless surgical resection is deemed to
be too morbid. Sclerotherapy around major upper extremity nerves can cause
numbness, motor deficit, and chronic pain.
SURGICAL MANAGEMENT
■ The indications for surgical intervention are pain, functional compromise
(impending or extant), rapid or persistent growth, ischemia, and ulceration in
lesions that are not suitable for treatment by interventional radiologists.
■ A few key principles are outlined here2:
■ Plan extensively. Outline the extent of the resection and abide by it.
■ Place incisions thoughtfully. Often multiple debulking procedures may be
necessary.
■ Use magnification.
■ Approach the lesion from known to unknown.
■ Avoid vascular compromise. When a critical arterial segment to an extremity
is removed, it should be reconstructed.
■ Avoid intraneural dissection. Dissection within nerves often leaves
neuromas-in-continuity with disruption of distal sensory or motor function.
■ Avoid partial dissections. Removal of an entire involved area is preferred as
it avoids future challenging dissection in a scarred bed.
■ Replace abnormal or ischemic skin; use flaps or skin grafts.
■ Use drains liberally.
■ Amputate if needed. Ischemic, nonfunctional, painful, and parasitic digits or
limbs should be removed.
■ Follow patients long term.
■ The purpose of surgery should be clearly outlined. In large or complex cases,
the goal of surgery is relief of pain and improvement of function. With the
exception of small lesions, total eradication of most lesions is impossible.
These lesions are not malignant, and so a wide excisional approach that
sacrifices normal functioning structures is ill advised.
Preoperative Planning
■ Again discuss risks, benefits, and expectations with parents and patient on the
day of surgery.
Positioning and Approach
■ The patient is positioned supine with the arm out on a hand table.
■ Incisions are planned carefully prior to use of the tourniquet.
■ In infants and toddlers, an Esmarch bandage is used as a tourniquet because it
is more effective at providing a bloodless field in small arms. In older children,
a standard pneumatic tourniquet is used.
T E C H N I Q U E S
■ Excision of Palmar Venous Malformation
■ Mark a palmar incision that incorporates the thenar flexion crease (TECH
FIG 1A).
■ Exposure will be needed beyond the area of malformation to fully expose
all the vital neurovascular structures—away from the lesion.
■ Design the incision so that distal or proximal extension of the wounds will
be possible.
■ Raise skin flaps over the palmar fascia.
■ Venous malformations tend to become much smaller when the limb is
exsanguinated and the tourniquet is inflated.
■ Incise the palmar fascia sharply and explore to find the nerves and vessels
proximal to the lesion.
■ Isolate the arteries and nerves with vessel loops (TECH FIG 1B). Dissect
these structures off the venous malformation.
■ For lesions of the palm, it is easier to dissect the ulnar and radial aspects of
the lesion first. This way, any vital longitudinal structures that traverse the
lesion can be identified, protected, and dissected more easily.
■ Use vessel loops not only for identification but also to place traction on vital
structures so they can be dissected along their native paths.
■ Venous malformations of the digits and distal palm will often arise from the
venae comitantes of the digital arteries and thus run along the arteries. Isolate
the venous malformation completely with the arteries that traverse it on either
side (TECH FIG 1C,D).
■ Avoid sacrificing arteries. Dissection of the venous malformation off the
digital arteries is possible and should be attempted.
■ Perform the dissection with magnification using an operating microscope
and micro instruments (TECH FIG 1E).
■ Use an ultrafine bipolar forceps (on a low setting) to cauterize all of the
branches of the arteries that feed the venous malformation.
■ Release the tourniquet and obtain hemostasis.
■ To avoid bleeding postoperatively, the best strategy is to remove all the
venous malformation.
■ If subtotal excision is too difficult, the second best strategy is to
prophylactically (ie, during the excision procedure) tie or clip the entire
portion of the venous malformation that will be left behind.
■ The least favorable (though not always avoidable) strategy is to sharply
remove some of the venous malformation and leave some behind with
channels wide open. These will bleed a lot and are very hard to control with
the tourniquet down. Bleeding should controlled with a combination of
prolonged direct pressure, tissue sealants, and local clotting enhancers.
■ Use drains liberally. The author prefers the TLS drain system for digital and
palmar lesions. The suction tube can be changed once it is one-third full or
every 4 hours.
■ A soft gauze dressing is applied followed by a well-padded cast. The cast is
bivalved if there is any concern about postoperative bleeding or swelling.
TECH FIG 1 • A. Incision markings. The proximal
incision extends into the thenar flexion crease. This
incision is made proximal to the lesion to identify the
digital nerves and branches of the superficial palmar
arch. A Y-type pattern at the distal extension in the
web is versatile as this incision can be extended into
the digits via midlateral or oblique incisions while
keeping the flaps wide-based for optimal vascularity.
B. The common digital nerve to the second web space
and its branches have been mobilized (yellow loops).
Proximal incision extension was made to find the
superficial palmar arch, which was absent in this
patient. The venous malformation is decompressed
under tourniquet control. C. The venous malformation
has been completely mobilized—the green
background sits underneath it. The ulnar digital artery
to the index finger and the radial digital artery to the
middle finger have been identified and looped. These
vessels emerge from the malformation. The blue loop
is around the common digital artery to the second web
space. D. The common digital artery (arrow) can be
seen emerging from (the deep arch) underneath the
flexor tendon to the index finger and piercing the
venous malformation. E. The venous malformation
can be safely dissected away from the common and
proper digital arteries using the operating microscope
and microinstruments. The vessels are shown after
the lesion has been removed.
PEARLS AND PITFALLS
Diagnosis
■ A multidisciplinary approach is the best way to take care of these
patients, especially in more complicated cases.
Nonoperative
management
■ Avoid sclerotherapy of palmar venous malformations.
Technique
■ Keep meticulous hemostasis during the case. Once important
structures become bloodstained, they can be much more difficult to
identify and dissect.
Technique
■ Use fine instruments, especially in children who have smaller vital
structures in the hand than do adults.
POSTOPERATIVE CARE
■ For VMs, the drain can be removed within 24 hours, once the threat of
bleeding has faded. For LMs, the drain should be in until drainage has abated
significantly. If the fluid collects in the wound, it can coalesce into firm scar
and added bulk.
■ Immobilize with a cast or a splint for 2 to 3 weeks.
■ Discontinue immobilization and start hand therapy.
OUTCOMES
■ Outcomes are variable and highly dependent on the initial degree of
involvement. In patients with joint and or tendon/muscle contractures, surgery
may result in limited improvement.
■ For patients with painful VMs, removal usually results in significant
improvement in symptoms.3
COMPLICATIONS
■ The most common complications are bleeding and skin flap compromise.
■ Bleeding and hematoma can be avoided with meticulous hemostasis and
liberal use of drains and hemostatic agents.
■ Skin flap compromise may be caused by inherent disease (vascular steal) or
attributed to poor planning and execution. Ischemic flaps should be debrided
and replaced with healthy flaps or skin grafts.
■ Recurrence and persistent growth are common problems, especially in
extensive lesions.
■ Distal ischemia is a dreaded complication, especially after treatment of AVMs
when the arterial feeders have to be divided.
■ In these cases, it is wise to have a backup plan to bypass with vein grafts if
needed.
REFERENCES
1. Kurek KC, Luks VL, Ayturk UM, et al. Somatic mosaic activating mutations
in PIK3CA cause CLOVES syndrome. Am J Hum Genet. 2012;90(6):1108-
1115.
2. Upton J, Taghinia A. Special considerations in vascular anomalies: operative
management of upper extremity lesions. Clin Plast Surg. 2011;38(1):143-151.
3. Upton J, Coombs CJ, Mulliken JB, et al. Vascular malformations of the upper
limb: a review of 270 patients. J Hand Surg [Am]. 1999;24:1019-1035.
71
CHAPTER
Section XIV: Tumors
Lumps and Bumps of the
Hand and Wrist
Paymon Rahgozar and Kevin C. Chung
DEFINITION
■ Ganglion cysts
■ Ganglion cysts are the most common soft tissue tumor of the hand.
■ Though not a true cyst because the lack of an epithelial lining, ganglion cysts
are mucin-filled lesions with collagen-lined walls, usually attached to an
underlying joint capsule, tendon, or tendon sheath.1
■ Ganglion cysts are a frequent cause of hand and wrist pain. Sixty to seventy
percent of ganglion cysts appear in the dorsal wrist, whereas 18% to 20% of
ganglions can be found in the volar wrist.1
■ Mucous cysts
■ Mucous cysts are primarily found in the dorsal distal interphalangeal (DIP)
joint and have been referred to as mucous cysts of the finger, mucoid cysts,
mucinous pseudocyst, myxomatous cutaneous cyst, synovial cyst,
periarticular fibroma, periungual ganglion, epidermal cyst, nail cyst, dorsal
cyst, digital myxoid cyst, and cutaneous myxoid cyst.2
■ The lesions may be associated with osteoarthritis of the joint (degenerative
mucous cyst).
■ Giant cell tumors
■ Giant cell tumors are benign, slow-growing tumors of the tendon sheath, and
have also been referred to as fibrous xanthoma, pigmented villonodular
tenosynovitis, or localized nodular synovitis.
■ After ganglion cysts, giant cell tumors are the second most common tumor of
the hand.
ANATOMY
■ Ganglion cysts
■ Ganglion cysts consist of a solitary or multilobulated cyst sac filled with a
clear jellylike mucinous fluid. They are composed of hyaluronic acid,
glucosamine, albumin, and globulin.1
■ The lesion originates from an underlying joint or tendon sheath and maintains
communication to the cyst sac via a tortuous stalk (FIG 1).
■ Dorsal ganglion cysts typically originate at the scapholunate ligament,
whereas volar ganglion cysts often originate from the radiocarpal or
scaphotrapeziotrapezoidal joint, flexor carpi radialis tendon, or A1 pulley.3
■ Volar wrist ganglions may be intertwined with branches of the radial artery,
complicating surgical dissection.
FIG 1 • A. Communication of a dorsal ganglion through
the joint capsule to the underlying scapholunate
ligament. B. In cases where the ganglion cyst is not
directly overlying the scapholunate ligament, it is still
connected via a long pedicle. C. A multicystic ganglion
with intra-articular cysts.
FIG 2 • A. Volar ganglion cyst in situ. B. Giant cell tumor
of the tendon sheath removed from the volar surface of
the long finger.
■ Grossly, the lesions are smooth, well circumscribed, white, and translucent
(FIG 2A).
■ Microscopically, the cysts have dense fibrous connective tissue without any
true cellular lining. The outer surface is irregular and fibrillated, composed of
disorganized collagen fibers.4
■ Mucous cysts
■ Mucous cysts typically originate at the DIP joint with its stalk passing
between the extensor insertion and the collateral ligament.
■ The microscopic appearance is similar to ganglion cysts.
■ Giant cell tumors
■ Giant cell tumors are yellow- to brown-colored rubbery lesions that may
present with joint involvement or bone erosion (FIG 2B).
■ The tumors are typically found on the volar surfaces of the radial three digits
of the hand, though dorsal involvement is also common.5
■ A type I tumor is a single round or multilobulated tumor entirely surrounded
by one pseudocapsule. Type II tumors consist of two or more distinct tumors
not joined or surrounded by a pseudocapsule.6
■ Histologically, giant cell tumors contain multinucleated giant cells,
histiocytes, and hemosiderin deposits.
PATHOGENESIS
■ There are multiple theories for the cause of ganglions and mucous cysts. It had
been thought that synovial herniation resulted in mucoid degeneration.
However, the lack of a cellular lining argues against this theory.
■ Another theory is that a stress-induced stretching of capsular and ligamentous
joint structures stimulates increased mucin production. The mucin collects in
the form of capsular ducts and lakes that eventually coalesce into a main cyst.7
■ The pathogenesis of giant cell tumors is not completely understood. The most
widely accepted hypothesis is that of an inflammatory process leading to a
reactive or regenerative hyperplasia.8
NATURAL HISTORY
■ Ganglion cysts
■ Ganglion cysts typically arise spontaneously and are most frequently found
in women between the second and fourth decades of life.
■ Though a history of trauma is rare, local injury may make the patient aware
of the presence of the tumor.
■ The size of ganglion cysts may fluctuate with use of the extremity, enlarging
with increased activity and shrinking with rest.
■ Ganglion cysts may rupture or disappear without treatment. However, there is
no known marker to reliably predict which cysts will resolve spontaneously
and which will persist.
■ Spontaneous resolution is more commonly seen in the pediatric population.9
■ Mucous cysts
■ Mucous cysts primarily occur between the fifth and seventh decades in the
DIP joint of the index and long fingers.
■ The lesions are typically associated with osteoarthritis of the joint.
■ An open or draining cyst can become infected leading to septic arthritis of
the underlying DIP joint.1
■ Giant cell tumors
■ A giant cell tumor begins as a single nodule but may become multinodular as
it enlarges over time.
■ Malignant transformation has not been reported.10
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Ganglion cysts
■ Patients often present with an asymptomatic 1- to 2-cm well-circumscribed,
slightly mobile, fluid-filled mass.
■ Patients typically report that the mass has been present for months to years
with fluctuations in size.
■ Dorsal wrist ganglions most often present with the mass overlying the dorsal
scapholunate interval (FIG 3A).
■ Volar wrist ganglions commonly appear with a mass between the extensor
tendons and the flexor carpi radialis (FIG 3B).
■ Volar retinacular cysts involve the tendon sheath overlying the A1 or A2
pulleys and may appear fluctuant or firm with palpation. Though typically
asymptomatic, patients may complain of discomfort with forceful grip.
Although on examination these lesions may be slightly mobile, they do not
typically glide with tendon movement.
■ Patients often seek medical attention for ganglion cysts due to aesthetic
concerns.
■ Pain or sensory symptoms secondary to ulnar or median nerve compression
can occur. The pain is typically a dull ache and is most pronounced with
active hand use.
■ Weakness of grip may be a complaint, particularly with dorsal wrist
ganglions.
FIG 3 • A. Clinical appearance of dorsal ganglion cyst.
B. Clinical appearance of volar ganglion cyst.
■ The fluid within ganglion cysts will transilluminate light, whereas other solid
lesions will not.
■ Frequently, there is no history of antecedent trauma. However, if there is a
dorsal ganglion in the presence of trauma, the exam should include a
complete evaluation of carpal stability.
■ Mucous cysts
■ Mucous cysts are commonly found over the DIP joint of the long or ring
finger as a painless mass lateral to midline on either the dorsal radial or ulnar
side of the joint with extension into the eponychial fold.
■ Mucous cysts may make the overlying dermis thin, causing rupture of the
skin and drainage.
■ Longitudinal nail grooving or Heberden nodes with DIP joint osteoarthritis
can be present in mucous cysts.
■ Giant cell tumor
■ Giant cell tumors present as a painless, firm, nodular, nontender, slow-
growing subcutaneous nodule.
■ Patients may present with sensory deficits secondary to nerve compression.
■ It typically presents in females in the fourth to sixth decade and is often
found on the volar surfaces of the hand, though dorsal involvement is not
infrequent. Most often, it is found in the radial three digits and the DIP joint.
■ The lesion is firmer than a ganglion cyst and will not transilluminate light.
IMAGING
■ Radiographs are required if the patient complains of pain or there is a history
of trauma.
■ Plain films are useful in identifying intraosseous ganglion cysts.
■ An arthrogram can demonstrate communication between the cyst and the joint
space.
■ MRI is used to identify an occult ganglion cyst or to localize the site of origin
for preoperative planning.
■ Mucous cysts often have radiographic osteoarthritic changes in the joint with
Heberden nodes
■ On plain radiographs, giant cell tumors may demonstrate erosion of bone
secondary to mass effect. Although bony erosion may indicate a higher risk of
recurrence, bony indentation does not.6
■ Ultrasonography can aid in detecting giant cell tumor satellite lesions.11
DIFFERENTIAL DIAGNOSIS
■ Ganglion and mucous cysts
■ Lipoma
■ Synovial cyst
■ Epidermal inclusion cyst or sebaceous cyst
■ Giant cell tumor of tendon sheath
■ Giant cell tumor
■ Tendon sheath fibroma
■ Synovial hemangioma
■ Tophaceous gout
■ Periosteal chondroma
NONOPERATIVE MANAGEMENT
■ Ganglion cysts can be treated nonsurgically with rest, splinting, oral
analgesics, or aspiration. The injection of lidocaine, hyaluronidase, or steroids
can also be employed during aspiration.
■ Aspiration treatments yield recurrence rates as high as 59%.12
■ Volar retinacular cysts that are not associated with a stenosing tenosynovitis
have a better response to aspiration/injection, and surgical excision can often
be avoided.
■ Traumatic rupture with a book (ie, Bible) is only of historical significance.
■ Aspiration of nondraining mucous cysts may be performed, but repeat
aspiration is not advised to avoid joint infection.
■ There is no role for nonoperative management for giant cell tumors.
SURGICAL MANAGEMENT
■ For ganglion and mucous cysts, excision is indicated for symptomatic patients
and for those who have failed conservative measures.
■ Draining mucous cysts should be excised because of the risk of infection,
which can ultimately lead to septic arthritis.
■ Surgery is indicated for giant cell tumors for appearance, loss of function, or
neuropathy.
■ A marginal excision with identification and excision of satellite lesions is
necessary.
Preoperative Planning
■ Appropriate preoperative imaging should be performed to identify the extent
of the lesion, presence of any satellite lesions, or involvement of any vital
structures.
■ In cases of volar wrist ganglions, an intact palmar arch must be confirmed with
a preoperative Allen test.
■ Patients with giant cell tumors must be informed of the high recurrence rates.
Positioning
■ The patient is positioned supine on the operating table, and the extremity is
positioned on a hand table.
■ The extremity is prepped and draped circumferentially.
■ The appropriate anesthesia is administered (general, regional block, digital
block) and an appropriate tourniquet is applied (upper arm or digit).
■ The location of the lesion dictates the technique and the approach chosen.
T E C H N I Q U E S
■ Dorsal Wrist Ganglion
■ Exsanguinate the extremity and apply an upper arm tourniquet.
■ Make a 2-cm transverse incision centered over the dome of the ganglion
(TECH FIG 1A).
■ Dissect the subcutaneous tissues, taking care to identify and protect the
dorsal radial and ulnar sensory nerve branches.
■ Identify the extensor pollicis longus (EPL) and extensor digitorum
communis (EDC) tendons and retract them. Use a self-retaining retractor for
exposure. The ganglion is typically in the interval between the third and
fourth extensor compartments (TECH FIG 1B).
■ Free the cyst circumferentially using blunt dissection. If possible, avoid
rupturing the capsule, so that it is easier to trace the ganglion to the stalk,
which is attached to the dorsal retinaculum (TECH FIG 1C,D).
■ Identify the stalk connecting the main cyst to the joint. It usually can be
found on the dorsal aspect of the scapholunate interosseous membrane just
proximal to the dorsal scapholunate ligament.
■ Tangentially excise the ganglion with the capsular attachments, without
disrupting the scapholunate ligament. A mucin-filled duct piercing the fibers
of the scapholunate ligament connecting the joint with the cyst can be
identified (TECH FIG 1E). One should not cut into the scapholunate
ligament.
■ If the ganglion is large, it should first be decompressed. With a no. 15 blade,
excise the base of the stalk with a rim of the dorsal wrist capsule.
■ To minimize recurrence, cauterize the site of origin of the cyst and the edge
of the wrist capsule with bipolar cautery.
■ Release the tourniquet, confirm hemostasis, and close the wound in multiple
layers. The skin is closed with a nonabsorbable monofilament suture.
■ Capsular closure should be avoided to prevent joint stiffness. Place the wrist
in slight flexion postoperatively.
■ Place the hand in a bulky dressing with a neutral palmar splint. Remove the
dressing and sutures in 7 to 10 days.
TECH FIG 1 • A. Typical transverse incision made
over the scapholunate ligament. B. The dorsal
ganglion cyst is most frequently found between the
extensor pollicis longus (EPL) and extensor digitorum
communis (EDC) tendons. C. The incision into the
capsule begins on the radial surface of the cyst and
continues circumferentially. D. A circumferential rim of
capsule is taken with the cyst and continued in the
volar direction. E. The typical relation of the mucin
duct with the scapholunate ligament. It is important to
include this in the resection to minimize future
recurrence.
■ Volar Wrist Ganglion
■ Perform a preoperative Allen test to evaluate for radial dominant circulation.
■ Make an incision centered over the cyst. This incision is most commonly in
the interval between the first extensor compartment and the flexor carpi
radialis (FCR) tendon (TECH FIG 2A).
■ Create skin flaps and place a self-retaining retractor for exposure. Incise the
underlying forearm fascia longitudinally.
■ While taking care to protect the branches of the lateral antebrachial
cutaneous nerve and the radial artery, identify the capsule and trace the
pedicle to the volar joint capsule (often the radiocarpal or the scaphotrapezial
ligament) (TECH FIG 2B).
■ If extension into the carpal canal is necessary, avoid injury to the palmar
cutaneous branch of the median nerve by not straying ulnar to the FCR
tendon.
■ In a fashion similar to the dorsal wrist ganglion, open the joint and excise all
the capsular attachments along with the cyst (TECH FIG 2C).
■ If the cyst is adherent to the radial artery, a small cuff may be left behind to
avoid arterial injury. It is particularly important to avoid dissecting the
ganglion away from the radial artery because a shared wall may be indistinct
and injuring the radial artery is likely. Therefore, one should decompress the
ganglion and leave the cyst wall attached to the artery. Then trace the
ganglion to the wrist joint for resection and cauterization of the stalk.
■ After excision of the cyst, digitally compress the surrounding tissue to rule
out additional mucin-filled pockets requiring excision.
■ Release the tourniquet, obtain hemostasis, and close the wound with a
nonabsorbable monofilament suture. Avoid capsular closure.
■ Place a bulky dressing. Remove sutures approximately 7 to 10 days after
surgery.
TECH FIG 2 • A. A longitudinal incision spanning from
the proximal portion of the cyst to its distal extent. If
additional distal exposure is necessary, the incision
may be carried out into the carpal tunnel. B.
Identification of the cyst pedicle toward the volar joint
capsule. C. Excision of ganglion with all the capsular
attachments. (Arrow indicates the FCR tendon.)
■ Mucous Cyst
■ Apply a finger tourniquet after obtaining digital block anesthesia.
■ If the overlying skin is of good quality, make a gentle curving transverse
incision centered over the DIP joint.
■ If there is involved overlying skin, make a longitudinal elliptical incision
including the overlying skin to be excised (TECH FIG 3A).
■ Mobilize the cyst and trace it to the joint capsule, excising it with the capsule
and any involved skin (TECH FIG 3B).
■ Often, it will be necessary to excise all the soft tissue between the collateral
ligament and the extensor tendon, leaving the DIP joint exposed.
■ It is imperative to avoid injury to the germinal nail matrix, the insertion of
the extensor tendon, and the collateral ligament.
TECH FIG 3 • A. An elliptical incision is used to
include the overlying skin when it is involved with the
mucous cyst. B. The cyst is identified and traced down
to the joint capsule. C. Closure of defect with
nonabsorbable monofilament suture. Sutures are
typically removed 7 to 10 days after surgery.
■ With the extensor tendon retracted dorsally, explore the opposite side and
excise any occult cysts or thickened synovial tissue.
■ Excise osteophytes and hypertrophic synovial tissue with a rongeur to
minimize recurrence.
■ Release tourniquet and achieve hemostasis.
■ Close the defect primarily with nonabsorbable monofilament suture (TECH
FIG 3C).
■ If the skin defect is excessive, a long rotational flap or full-thickness skin
graft may be necessary. No excess tension should be placed on the closure.
■ Place the distal joint in a splint, and remove sutures in 7 to 10 days.
■ Giant Cell Tumor
■ Perform initial exposure using an appropriate incision based on the anatomic
location of the mass, and isolate the neurovascular bundle proximal and distal
to the mass.
■ After identifying the pseudocapsule, use a Freer elevator to bluntly dissect
the surrounding tissue from the lesion (TECH FIG 4A).
■ Excise the tumor in total with a margin of normal tissue. If necessary, a
portion of the tendon sheath may be excised and the area cauterized with
bipolar electrocautery.1
■ Meticulously examine the local area and excise any satellite lesions to
minimize recurrence (TECH FIG 4B).
■ If there is joint involvement, perform a capsulotomy and inspect the joint,
debriding any pigmented soft tissue. If there is bony erosion, local curettage
is necessary. If joint involvement is extensive, arthrodesis may be necessary
to ensure complete excision.
■ If the extensor tendon is involved, a portion may need to be excised. In rare
cases, a tendon reconstruction can be performed if necessary.
■ Close primarily and place hand in a bulky dressing.
TECH FIG 4 • A. The giant cell tumor is dissected with a
margin of normal tissue after identifying the
neurovascular bundle proximal and distal to the mass.
B. The local area must be closely inspected to identify
and excise any satellite lesions. Failure to do so will
inevitably lead to a high likelihood of recurrence.
PEARLS AND PITFALLS
Ganglion
cyst
■ Adequate excision requires removal of a small cuff of the joint capsule.
■ Do not cut into the scapholunate ligament to avoid inadvertent injury.
■ Cauterize the edge of the capsule before releasing the tourniquet. It will
be very difficult to adequately cauterize it subsequently.
■ Do not repair the capsule to avoid joint stiffness.
■ Always identify the deep and superficial branches of the radial artery
when excising volar wrist ganglion cysts. It may be adherent to the
artery.
Mucous
cyst
■ Adequate excision requires debridement of joint osteophytes and any
occult cysts.
■ Great care must be taken to avoid nail bed injury even if a small amount
of cyst must be left behind.
■ Avoid injury to extensor tendon or collateral ligament to prevent
postoperative extensor lag or DIP joint instability.
■ Evaluate both sides of the extensor tendon and the entire DIP joint to
rule out occult cysts.
■ Do not close skin flaps under excess tension.
Giant
cell
tumors
■ Satellite lesions must be identified and excised to lower the likelihood
of recurrence.
■ Perform capsulotomy if there is joint involvement to adequately resect
all involved tissue.
■ Take care to identify and protect the neurovascular bundles because the
lesion can displace or envelop the neurovascular structures
POSTOPERATIVE CARE
■ For ganglion and mucous cyst excisions, the hand is placed in a neutral wrist
splint. Early hand elevation and early finger motion are encouraged. The splint
and sutures are removed at 7 to 10 days postoperatively, and range-of-motion
exercises begin at this time.
■ After excision of a giant cell tumor, range-of-motion exercises should be
started immediately. Sutures can be removed after 7 to 10 days.
OUTCOMES
■ Ganglion cysts: The recurrence rates following open surgical excision is
21%.12
■ Mucous cysts: Recurrence rates are as low as 2% with complete osteophyte
excision at the time of surgery.2
■ Giant cell tumors: Recurrence rates range from 5% to 50%.1
■ Factors predictive of high recurrence include incomplete excision, bony
invasion, type II tumors, cellularity and mitotic activity on histology, and
absence of nm23 gene expression on immunohistochemistry.6,10
COMPLICATIONS
■ Ganglion cysts: The most common complications include wound
complications (painful or hypertrophic scar, infection, neurapraxia) or early
recurrence.
■ Recurrence is secondary to incomplete excision or a missed occult cyst.
■ Pain may persist postoperatively despite complete resection.
■ Stiffness can occur as a result of capsular closure or delayed postoperative
movement.
■ Mucous cysts: Excisions can result in extensor lag, joint stiffness, DIP joint
deformity, nail plate deformity, pain, or infection.
■ Giant cell tumors: In addition to recurrence, postoperative complications may
include transient numbness or infection.
REFERENCES
1. Nahra ME, Bucchieri JS. Ganglion cysts and other tumor related conditions of
the hand and wrist. Hand Clin. 2004;20(3):249-260.
2. Kasdan ML, Stallings SP, Leis VM, Wolens D. Outcome of surgically treated
mucous cysts of the hand. J Hand Surg [Am]. 1994;19(3):504-507.
3. Henderson MM, Neumeister MW, Bueno R Jr. Hand tumors: I. Skin and soft-
tissue tumors of the hand. Plast Reconstr Surg. 2014;133(2):
154e-164e.
4. Loder RT, Robinson JH, Thomas Jackson W, Allen DJ. A surface
ultrastructure study of ganglia and digital mucous cysts. J Hand Surg [Am].
1988;13(5):758-762.
5. Briet JP, Becker SJ, Oosterhoff TC, Ring D. Giant cell tumor of tendon
sheath. Arch Bone Jt Surg. 2015;3(1):19-21.
6. Al-Qattan MM. Giant cell tumours of tendon sheath: classification and
recurrence rate. J Hand Surg Br. 2001;26(1):72-75.
7. 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(3):228-235.
8. Fotiadis E, Papadopoulos A, Svarnas T, et al. Giant cell tumour of tendon
sheath of the digits: a systematic review. Hand. 2011;6(3):244-249.
9. Wang AA, Hutchinson DT. Longitudinal observation of pediatric hand and
wrist ganglia. J Hand Surg [Am]. 2001;26(4):599-602.
10. Grover R, Grobbelaar AO, Richman PI, Smith PJ. Measurement of invasive
potential provides an accurate prognostic marker for giant cell tumour of
tendon sheath. J Hand Surg Br. 1998;23(6):728-731.
11. Suresh SS, Zaki H. Giant cell tumor of tendon sheath: case series and review
of literature. J Hand Microsurg. 2010;2:1-5.
12. Head L, Gencarelli JR, Allen M, Boyd KU. Wrist ganglion treatment:
systematic review and meta-analysis. J Hand Surg [Am]. 2015;40(3):546-
553.e8.
72
CHAPTERExcision of Glomus Tumors
Paymon Rahgozar and Kevin C. Chung
DEFINITION
■ Glomus tumors are benign hamartomas of the glomus body typically found in
the reticular layer of the dermis in the distal phalanx or the subungual region.
■ Glomus tumors account for 1% to 5% of all hand tumors.1
ANATOMY
■ The glomus body normally regulates blood flow in the reticular dermis in
response to local temperature changes, functioning as an arteriovenous shunt.
■ Glomus bodies are found throughout the body but are most numerous in the
fingertips and the nail bed.
■ Subungual involvement is found in 25% to 65% of cases, typically seen as
discoloration or deformation of the nail plate.2
■ Histologically, glomus cells, smooth muscles, blood vessels, and
nonmyelinated nerve fibers are found within the glomus tumor. There are three
distinct histologic types that can occur with the possibility of having multiple
histologic tumors simultaneously.3
■ Type I: mucoid hyaline type
■ Type II: solid type (classic glomus tumor)
■ Type III: angiomatous type
■ The solitary glomus tumor is a well-encapsulated lesion with numerous small
lumina, most commonly found in the subungual region.
■ Multiple tumors, on the other hand, are not encapsulated and rarely
subungual.
PATHOGENESIS AND NATURAL HISTORY
■ Glomus tumors occur as a result of hyperplasia of the neuromyoarterial
glomus body.3
■ They can be solitary or multiple. Solitary tumors often present with chronic
extremity pain, frequently leading to initial misdiagnosis and inappropriate
treatment.1
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Glomus tumors typically present in women 30 to 50 years old.
■ Solitary glomus tumors have been associated with a classic triad of
paroxysmal pain, pinpoint tenderness, and cold intolerance. The lesions can
present with nail plate ridges, a pulp nodule, and/or bluish discoloration of the
nail bed.4
■ Often patients present with years of pain before definitive diagnosis. The
tumor is often misdiagnosed because of its rarity, lack of the triad symptoms in
the early course of the tumor, and several differential diagnoses.1,5
■ Multiple glomus tumors tend to present earlier in life than solitary glomus
tumors, and are usually asymptomatic.
■ Diagnostic tests include the following:
■ Love’s pin test—severe pain reproduced with direct pressure
■ Hildreth’s test—relief of pain with tourniquet application followed by
subsequent return of pain with tourniquet release
■ Cold sensitivity test—immersion in an ice bath or application of a refrigerant
spray elicits pain.
IMAGING
■ If there is doubt about the diagnosis or the specific location of the tumor, MRI
is helpful in identifying glomus tumors as small as 2 mm in the fingertip.
■ There will be high signal intensity on T2 images with strong enhancement
following gadolinium infusion on T1 images.
■ The appearance of a high signal central dot surrounded by a zone of less
signal intensity is characteristic3,5,6 (FIG 1).
■ In long-standing glomus tumors, a plain x-ray may show pressure atrophy of
the distal phalanx.1
■ Doppler ultrasonography can identify low signals within a hypoechogenic
mass as small as 2 mm.6
DIFFERENTIAL DIAGNOSIS
■ Leiomyoma
■ Eccrine spiradenoma
■ Cavernous hemangioma
■ Sarcoma
■ Neuroma
■ Chronic paronychia
■ Calcinosis
■ Ganglion/mucous cyst
■ Epithelial inclusion cyst
NONOPERATIVE MANAGEMENT
■ Although excision is the only known cure for glomus tumors, nonsurgical
treatment with prostaglandin inhibition, sclerotherapy, laser, or radiation have
been described with mixed results.5,6
FIG 1 • MRI of glomus tumor of the ring finger. Note the
high signal intensity of the tumor.
FIG 3 • A. The tumor is palpated and marked for
excision. This corresponds to the area of most
tenderness on examination. B. The volar digital glomus
tumor was resected through a zigzag incision.
SURGICAL MANAGEMENT
■ A bloodless field is necessary during excision to delineate the tumor from
surrounding tissues. Poor visualization increases the likelihood of inadequate
resection and future recurrence (FIG 2).
■ Subungual tumors require elevation of the nail plate and excision of the mass
from the nail bed, followed by nail bed repair.
■ If no gross tumor is found at exploration, remove a wedge of tissue from the
area of maximum patient discomfort.5
■ If the tumors have invaded the underlying bone, perform a partial bone
excision or curettage.1
Preoperative Planning
■ Preoperative imaging, if necessary, should be performed to identify the extent
of the lesion and the involvement of any local vital structures.
Positioning
■ The patient is positioned supine on the operating table, and the extremity is
placed on a hand table.
■ The extremity is prepped and draped circumferentially.
■ The appropriate anesthesia is administered (general, regional block, digital
block), and a tourniquet is applied (upper arm or digit). Typically, a digital
nerve block is sufficient.
FIG 2 • A clean bloodless field permits easy
visualization of the glomus tumor and the surrounding
normal tissue.
Approach
■ The location of the lesion dictates the approach. Subungual tumors are excised
through either a transungual or a midaxial incision.
■ Although the transungual approach provides the best visualization, it can lead
to nail bed injury and postoperative nail deformity leading to an unsatisfactory
cosmetic result. Careful excision and precise placement of sutures to repair
the nail bed are essential.
■ The midaxial incision does not risk injury to the nail bed, but a flap needs to
be developed under the periosteum, making the approach more challenging.
Also, there is a risk of digital nerve injury.
■ Mark the specific location of the tumor based on palpation or the area of most
tenderness (FIG 3A).
■ Volar digital tumors are resected with a zigzag incision (FIG 3B).
T E C H N I Q U E S
■ Transungual Approach
■ Identify the area of most tenderness or discomfort. This is particularly
important in lesions that are not grossly evident on examination (TECH FIG
1A).
■ Inject digital anesthesia and apply a tourniquet. Limit exsanguination to
elevation of the extremity because the tumors lose color in a bloodless field,
making it more difficult to distinguish tumor from normal surrounding
tissue.5
■ Elevate the nail plate (TECH FIG 1B).
■ Fold the nail over or remove it completely for adequate exposure if
necessary (TECH FIG 1C).
■ If additional exposure is necessary, make incisions over corners of the nail
fold in an oblique angle to avoid contracture (TECH FIG 1D).
■ With a no. 15 blade, make a longitudinal incision into the nail matrix
directly over the tumor. Dissect the tumor from the surrounding nail
matrix.3
■ Free the lesion circumferentially to the level of the phalanx and excise it
completely by leaving a defect in the nail bed (TECH FIG 1E).
■ Curette the portion of the phalanx involved with the lesion.
■ Close the nail bed defect with 6-0 plain gut suture meticulously by
reapproximating the edges of the incision to minimize nail deformity (TECH
FIG 1F).
■ Replace the nail into the eponychial fold to act as a protective stent for the
nail bed.
■ Close the corner incisions primarily.
■ Place a bulky dressing.
