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A. Landi et al.
internal medicine domain, especially among dermatologists [1–4] or specialists of intensive care
units [5, 6]. From the prevailing literature, it is
clear that this particular topic has been visited
mainly by hand surgeons, plastic surgeons, orthopaedic surgeons and paediatric surgeons [1, 7–
14] or by personnel of general surgery
departments where there is an afference of
paediatric surgery [6] and specialized centres
dedicated to the treatment of congenital malformations of the upper limb, equipped with personnel possessing specic microsurgical expertise
[4, 15–21].
The denition of Volkmann’s syndrome
refers to the rst description by the author of a
14-year- old boy subjected to an excessively
rigid immobilization for a spontaneous effusion
of the knee [22].
The great merit of this pioneer is to have identied cause and effect of the syndrome but not the
underlying pathogenetic process that will only be
elucidated when the knowledge of the microcirculation will allow to dene the key parameters
of function and survival of the muscles contained
in the muscle compartments.
The values of compartmental pressure in the
normotensive adult are nowadays recorded routinely. When the pressure is maintained continuously for 4–6 h beyond 30 mmHg, muscle
survival can be severely compromised. Just as
with the heart muscle, muscle insult can evolve
into scarring (ischaemic retraction) or colliquative necrosis. The two processes often coexist in
the skeletal muscle, and the muscle infarction
remains conned in the central hypoperfused
area, while scarring usually affects the peripheral
muscle tissue. It is also known that in hypotensive patients, the risk of ischaemic lesions is
already present with a compartment pressure of
about 20 mmHg [15].
Volkmann’s syndrome therefore represents
the natural outcome of an unrecognized and
therefore not promptly treated compartment syndrome, in which increased tissue pressure within
a limited space compromises the circulation,
function and potentially the viability of the contents of the affected area and thus of the main
vessels and nerves [1].
As far as vascular lesions are concerned, these
can be linked to a lesion of a main vessel
upstream, both venous and arterial, which,
despite the increased resistance of the epidermis
to ischaemia, lead to massive necrosis and consequent debridement or amputations at various levels of the upper limb.
32.2 Aected Locations
andPathophysiological
andAetiological
Classication
Volkmann’s syndrome is rarely seen in adults and
children today and should be considered an
exceptional event in the perinatal period.
In this chapter, we will focus on the substantial differences between age groups with regard
to aetiological factors, sites of onset, clinical
symptoms and the different uses of complementary investigations for diagnostic purposes.
The entire upper limb in its various muscle
compartments is a potential site of compartment
syndrome, and this can be evidenced in clinical
practice [23, 24]. At the shoulder level, there are
essentially two compartments: scapular and deltoid. In 1932, Comolli was the rst to describe a
clinical picture of the compartment syndrome at
the level of the scapula in association with a fracture [25].
So far in literature, compartment syndrome
has only been described in adults [12].
Furthermore, the area of the deltoid is almost
exclusively the prerogative of adulthood and is
established in patients who are habitually alcoholics and drug addicts who develop pressure
sores in the deltoid area during long periods of
unconsciousness. There is no description of this
in the perinatal period [16] but only in the paediatric age, usually following injections of medication or vaccines in the deltoid area [26].
Even at the level of the upper limb, the
adult’s CS is often associated with natural catastrophic events such as earthquakes and road
accidents that take form in the Crush syndrome
[15, 21, 27].

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There are two main muscle compartments in
the forearm: the anterior and the postero-lateral.
The forearm represents the most frequent site of
CS in association with supracondylar elbow fractures, closed and compound fractures of the forearm [4, 8, 10, 18, 21, 28]. This site also represents
the most frequent location of Volkmann’s syndrome and peri- and neonatal CS [1, 4, 11, 17, 21,
29, 30].
As regard the hand, the compartments are
formed by the interosseous of the thenar and
hypothenar intrinsic muscles. CS can develop in
this location both in the peri- and in the neonatal
phase but especially in the paediatric age. Fingers
can also be considered non-muscular compartments where the boundaries between the skin of
the dorsum and palm are formed by the ligaments
of Grayson and Cleland with the neurovascular
bundles between them. Fingers can therefore be
the site of a CS in burns and high-pressure injection injuries in adults, while in the paediatric
phase, they are the most frequent location of
amniotic bands that can generate venous and
lymphatic stasis up to perfusion defect which
compromises survival [31].
The present classication based on pathophysiological criteria follows Perricone and
Holden’s classication modied by Landi [17] in
Table 32.1. Group I includes lesions of a main
proximal arterial vessel [15], revascularization
syndrome and subtotal occlusion of the venous
circulation (type ID). In type II, CS is the result
of exclusively local multifactorial pathology
(type IIA) or in association with aggravating systemic factors (type IIB). In type III, CS might
spread all over the body and are linked to systemic diseases that can entail acute permeability
of the capillary network, as observed in the Leak
syndrome (Table32.1) [32]. In the peri- and neonatal phase, the etiopathological classication
differs substantially. Group I, similar to the adult,
includes pathologies of the main proximal vessels both on the arterial and venous sides; however, they are caused by completely different
aetiological agents (Table32.2). In group IIA, CS
may occur as a result of a hypothetical compression of the upper limb during the intrauterine
phase of prolonged labour. In group IIB, the possible aggravating factors should be differentiated
into maternal (chickenpox, gestational diabetes,
etc.) or foetal (clotting disorders, dehydration,
etc.). In group III, the systemic factors causing
multiple CS include meningococcal septicaemia
[34] and severe foetal dehydration [6]. Especially
in the infant, the boundaries between CS,
Volkmann’s syndrome and vascular lesions
appear subtle [36].
Table 32.1 Etiological and physiopatological classication of C.S. in adults
Type I Type II Type III
A Major artery lesion proximal to
the ischaemic muscle region
B Late revascularization syndrome Local disease associated
C Isolated lesion of the vascular
peduncular in type I muscle
(with only one vascular pedicle)
D Complete occlusion of the
venous circle (phlegmasia
cerulea dolens)
Compartmental syndrome
due to multifactorial and
exclusively local causes
with systemic factors
(haemophilia, patient in
treatment with anticoagulant
and antiplatelet drugs, blood
tumors: leukemias)
Systemic disease inducing multiple CS:
1. Leak syndrome (acute increased capillary
permeability) [32]
2. Local myotoxicity in various muscular
compartments (heroin overdose,
theophylline or alcohol abuse)
3. Adrenal massive haemorrhage
(Waterhouse-Friderichsen syndrome) [33]

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Table 32.2 Etiological and physiopathological classication of C.S. in the peri- and neonatal period
Type I Type II Type III
A Major artery lesion proximal
to ischaemic muscle region
(phlegmasia alba dolens)
B Complete occlusion of the
venous circulation:
hypercoagulability in the
newborn of diabetic mother
Compartmental syndrome due to
multifactorial but exclusively local
causes (intrauterine compression)
1. Oligohydramnios
2. Amniotic band
3. Prolonged pressure against
sacrum
Local disease associated with
maternal factors or foetal systemic
factors
1. Smallpox
2. Maternal diabetes
3. Mother and fetus coagulation
disorders
Systemic diseases inducing multiple
CS:
1. Dehydration [6]
2. Meningococcal septicaemia
[34]
3. S. epidermidis septicaemia [35]
4. Malignant hyperthermia [5]
A. Landi et al.
For simplication purposes, peri- and neonatal vascular lesions could include all the pathologies for which, once a vascular obstruction has
been diagnosed, an anticoagulant therapy or
microsurgical exploration and repair of the vessels by microvascular technique should be carried out. This group usually includes extensive
skin necrosis requiring serial debridement or
proximal and distal amputations of the upper
limb.
32.3 Aetiological Factors
inAtypical Upper Limb CS:
Dierences Between
theNewborn andtheAdult
Perinatal CS should certainly be included among
atypical CS both for their incidence and pattern
of occurrence [1, 11, 18, 21, 24, 28]. According
to Bae [18] in fact, out of 33 patients, 26 (76%) of
the child’s CS occur in the setting of fractures,
most frequently in the upper limb. On the other
hand, we consider radial head fractures to be rare
and atypical as well as aspiration of the upper
limb during the emptying of a pool, the primary
tumour pathology such as leukaemia, promyelocytic leukaemia where the surgical treatment of
CS is absolutely contraindicated and the extravasation of anti-rejection drugs in organ transplants
in the paediatric age group (Fig.32.11). An atypical aetiology is also represented by the intraosseous infusion in infant, a technique for
administration of uid to a child who is in a state
of haemodynamic collapse and for whom the
access through conventional means has not been
successful. This method is used in the child from
three months to three years. The best sites for
intraosseous infusion are the distal metaphysis of
the femur and the proximal and distal metaphysis
of the tibia. Displacement of the needle might be
responsible of a CS at the lower limb. Therefore,
in this group, the upper limb is usually spared
[37].
32.4 Upper Limb Intrauterine
Compartment Syndrome
32.4.1 General Aspects
Perinatal CS appears to be restricted to the upper
limb [7] and it is included predominantly within
groups IIA, B and III in our classication
(Table32.2).
In the subgroup IIB (Table32.2), the predisposing factors can be found both on the maternal
and on the foetal sides [29]. On the maternal side,
there is often an association with chickenpox
infection, gestational diabetes or pre-existing
insulin dependence. There is a risk of partial
thrombosis during childbirth or immediately
before, due to placental rupture and related insufciency. A pre-eclamptic condition also plays a
negative role in this.
Simultaneous dehydration or infection can play
a decisive role on the foetal side. Coagulation disorders of the foetus represent an aggravating sys-

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temic factor in type IIB compartment syndromes
(Table32.2), but they are more frequently associated with neonatal gangrene of the upper limb.
Among the loco-regional factors contributing
to the development of a CS of the upper limb, we
must consider the oligohydramnios which can
cause the compression of the limb, in particular
the right one, against the sacrum or the maternal
lumbar spine [8, 29, 38]. The foetus is in fact
most vulnerable to compression after the 37th
week of gestation, when the amniotic uid begins
to decrease [9]. The amniotic bands, especially at
the nger level and in the presence of a swollen
and sub-cyanotic segment, represent the only rare
cases that require, when promptly diagnosed,
decompression in the rst hours of life [31, 39].
32.4.2 Clinical Symptoms
Acute CS of the upper limb should be diagnosed
before it evolves into established Volkmann’s
syndrome. However, this scenario occurs very
rarely, and so far, there are only two cases in
literature where fasciotomy was performed
urgently soon after birth [8, 40] followed by a
complete recovery. Only in the case where the
pathology of amniotic bands belongs to group
IIA of our classication (Table 32.2), the preconditions of an emergency decompression are
met. This evidence can also be found in other
animal species where a case of amniotic band
syndrome (ABS), caused by a stenosis from the
maternal fur, in the front leg of a baby rabbit has
been observed. We proceeded with a circumferential removal of the causal agent and the skin
band, as suggested by Buck-Gramcko and
described by Habenicht [39]. The removal of the
band was done in full respect of the periosteal
and peritendinous vascularization with complete restoration of the normal vascularization
of the paw (Fig.32.1).
In the clinical setting, we were able to perform
a similar operation only in one case 2 h after the
c
Fig. 32.1 (a) Amniotic band syndrome caused by the constriction of the maternal fur in a baby rabbit. (b) The constric-
tion ring has been removed. (c) Closure of the gap by multiple Z plasties

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a b
A. Landi et al.
Fig. 32.2 (a) Amniotic band syndrome associated with
swelling and vascular impairment of the middle nger
associated with intrauterine amputation of the ring nger.
(b) Decompression of the nger 2 h after birth by removal
birth of a premature baby who presented a digital
amniotic band and who was treated according to
the described technique (Fig.32.2) [39].
of the constriction and skin closure. (c) Return of normal
vascular feeding of the nger. (d) Long-term follow-up
showing a normal aspect and good function of the middle
nger
present in association with caesarean sections`,
and the peripheral pulses appear even in the event
of a signicant oedema [41, 42]. Of the 11 cases
reported by Cham [43], the most frequent clinical
picture corresponds to the adult with deformity
32.4.3 Stabilized Peri- andNeonatal
Volkmann’s Syndrome
of the hand in exion and ulnar deviation.
However, when the eschar is located at the
lateral-external surface, the position at birth is in
The typical manifestation of Volkmann’s syndrome at birth is characterized by a sufciently
stereotyped clinical picture [41]. The presence of
a cutaneous eschar (sentinel lesion) is almost a
constant [8, 29, 42] and could coincide with the
site of a greater intrauterine compression or
depend on an underlying thrombo-embolic event
(Fig. 32.3). Oedema is often widespread in the
forearm and in the hand and it is associated with
sub-cyanotic phenomena. The eschar as such can
be traced back to the intrauterine phase and not to
perinatal adverse factors. The eschar may also be
wrist extension, and it is often associated with
stiffness of the MPJ (metacarpal-phalangeal
joint). The retraction of the muscles of the latero-
external compartment is therefore at the basis of
the deformity in extension of the wrist which is
not passively correctable [43]. The wrist retrac-
tion in extension is also reported in 2 out of 4 of
the cases of Rombouts [9] and can also be seen in
our clinical experience of 15 cases where in 7
cases we found ischaemic retraction of the mus-
cles of the dorsal compartment, in some cases
associated with stiffness of the MPJ [11].

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g
Fig. 32.3 (a, b) Skin defect after removal of the sentinel
lesion performed one week after birth. The defect was
covered shortly with a skin graft. (c) Early neurolysis and
supercial dislocation of the median and ulnar nerve three
months after birth. Tenotomy of the wrist exors and of
FPL. (d) Latissimus dorsi free muscle transfer performed
at the age of four years. The Ilizarov apparatus was imple-
mented to overcome the irreducible wrist exor contrac-
ture. (e–g) Follow-up at 23 years: obvious limb
discrepancy but good elbow exion. No impairment on
DLA and bimanual activities

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A. Landi et al.
The skin lesion is to be considered pathognomonic of a prenatal Volkmann’s syndrome, especially since it is present in 20 out of 24 cases
reported by Ragland [8] who denes it as “the
sentinel lesion”, and is conrmed by many other
studies [4, 6, 15–17, 21].
32.4.4 Clinical Picture
The association of the skin lesion and the underlying compartment syndrome is often missed and
fasciotomy in emergency rarely performed. The
possible cause of the condition might be related
to an associated intrinsic pathology. The relationship between the skin lesion, the underlying muscles ischaemia and the late effects on angular
deformity and physeal closure of the radius distal
to the lesion in the proximal forearm might be
related to the concept of the angiosome and the
arterial vascular supply of the muscles [8]. The
overlying areas of the skin and underlying physis
of the radius might benet the same vascular supply [44]. In one of our cases, where the proximal
Volkmann’s forearm contracture was associated
with a congenital constriction ring at the wrist
level, we recorded a contracture of the medial
epicondyle muscles leading to a exion contracture of 30° of the elbow. The underlying premature physeal closure both of the proximal and
distal radius represents a unique feature in this
pathology.
In conclusion, the substantial difference
between paediatric and adult Volkmann’s syndromes lies in the almost constant presence of the
sentinel skin lesion at the forearm level.
32.4.5 Clinical Symptoms
Only in the paediatric age group it is easy to discriminate the clinical symptoms of CS to those of
the adult. In a retrospective report of 33 children
with diagnosis of CS of the Boston Children’s
Hospital [45], it appeared that the traditional 5 P
symptoms (pressure increased in the compartment, pain with stretch, paresis or anaesthesia,
paresis or paralysis, pulses intact) [24] was unreliable in children [7, 18]. The AA recommended
that the children at risk of CS should be monitored with the 3 As (increasing analgesic requirement, anxiety and agitation) [7, 21].
The muscle involvement has been taken into
account and we classied our patients into four
clinical groups as suggested by Ragland [8].
Group I that included patients who had skin
involvement without compartment ischaemia or
muscle dysfunction but late evidence of compromised skeletal development of the distal radius
on a vascular basis (none of our patients).
Group II comprised seven patients of our
series who had a skin lesion with late extrinsic
contracture only of the muscles of the dorsolateral compartment.
Group III comprised ve patients of our series
who had a skin lesion associated with either a
complete exor or extensor compartment involvement [17].
Group IV included the residual patients where
all compartments were involved and associated
with distal tissue loss of ngers and the hand.
We have three cases of this kind but we believe
that they should be included in the subheading of
vascular lesion.
32.4.6 Complementary
Investigations intheAdult
To complete the clinical diagnosis, the role of an
objective examination plays a crucial aspect,
especially in the case of complex diagnostics, in
patients with acute disorders of the state of
consciousness.
In acute cases, where the time element has an
essential role, the measurement of intracompartmental pressure is in fact the gold standard, given
the need to make a timely diagnosis or carry out
continuous monitoring.
The main model used by us is the “split” catheter consisting of a hollow tube, the terminal of
which has a series of ssurations, connectable to
a small pocket-sized detection unit with a silicon
transducer (STIC Catheter) or the Camino

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Catheter; this contains a local detection microsensor that must be connected to an external control unit equipped with a monitor [45, 46] that
enables evaluation of the variations of the PC
over time [47].
CT in established Volkmann’s contracture in
forearm muscles was introduced in 1989 [48] and
enabled identication of the muscle infarct which
corresponds to a sharp area of hypodensity (10,2
Hounseld Unit (HU) compared to 50–60 HU in
contralateral normal muscles). The extent of the
muscle damage within the muscle compartment
and involvement of different compartments might
positively inuence the choice of the most appropriate surgical treatment. CT as MRI imaging has
so far been utilized exclusively in adults [48, 49].
32.4.7 Complementary
Investigations intheChild
Compartmental pressure in children has been
measured by some authors with the same technique used in adults [15]. Normal resting compartment pressure in children has been described
in the lower limb and reported to be higher (13.3
mmHg and 16.6 mmHg) compared with adults
(5.2 mmHg and 9.7 mmHg). Generally intracompartmental pressure over 30 mmHg [16] or 40
mmHg, which corresponds quite accurately to
the infant’s diastolic blood pressure, should be
considered as a threshold for fasciotomy. In any
case, apprehension, poor cooperation and difculties with communication in the conscious
child [28] complicate the evaluation by any needle device. We never resorted to it as the majority
of our cases presented as an already established
Volkmann’s contracture.
Near-infrared spectroscopy has been introduced as a noninvasive technique to overcome
this problem [7]. Based on the same principle,
FRIR (Fourier transform infrared spectrophotometer), a smartphone thermal imaging which
consents the detection of perforator for free aps
and monitoring even at a physical distance from
the operator, has been used in recent years [19,
20, 50, 51].
X-rays are mandatory as the radiological ndings
are similar in these children: forearm bone shortening and widening of the distal epiphysis [10].
Ultrasound sonography remains the most
straightforward method for a rapid assessment of
the extent of residual muscle mass in the various
compartments.
In conjunction with the evaluation of compartmental pressure or the use of near-infrared spectroscopy, the echo-Doppler for the study of the
main and deep arterial and venous circulation,
especially when the distal pulses are not detected
during the clinical examination, is mandatory.
Angiography and MR angiography represent
techniques used in malformative and iatropathic
pathologies of main vessels [15, 43].
EMG studies are not easily tolerated by the
paediatric population and are not routinely
administered. Surprisingly in two cases of the
established perinatal Volkmann’s contracture in
children with the age of six and eight years, SEPs
were comparable to the values recorded in the
unaffected site in spite of the overt damage of the
median and ulnar nerve observed at surgery
(unpublished data).
32.5 Surgical Treatment
inPerinatal Volkmann’s
Contracture
32.5.1 General Aspects
The overall experience of the Institute of
Orthopedics of University of Modena for the
period 1999–2013 was of 175 operated cases of
Volkmann’s contracture, including the upper and
lower limbs (Table32.3). The incidence of perinatal Volkmann’s contracture has been of 8%,
and this signicant value might be related to the
presence of a dedicated congenital deformities of
outpatient clinic.
The complexity of cases and difcult family
context sometimes leads parents to adopt an attitude of abstention when faced with a proposal of
multiple complex surgeries from the medical
team.

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A. Landi et al.
Table 32.3 1998–2013 upper and lower limb Volkmann’s
syndrome
Site Aetiology Cases
Upper limb Volkmann’s syndrome
Proximal humerus Fracture + axillary
artery lesion
Distal humerus Supracondylar 15
Elbow Fracture 3
Forearm Fracture 19
Forearm Crush syndrome 9
Forearm Radio ulnar synostosis
(osteotomy)
Forearm Perinatal Volkmann 15
Forearm Others (iatropathic) 8
Hand (intrinsic
muscles)
Leak syndrome 2
WaterhouseFriderichsen
syndrome
Lower limb Volkmann’s syndrome
Clinica Ortopedica,
Modena
Hesperia Hospital,
Modena
Miscellaneous (mainly
posttraumatic)
Total 90
Total 85
Total cases 175
4
2
12
1
71
14
The natural history of the syndrome has not
been reported in literature and has been monitored and illustrated in detail as in the case of our
series (Fig. 32.4) [17]. In the adolescent phase,
this patient encountered alternating problems of
social integration, eventually entering a community and maintaining a pertinent use of the dysmetric limb in its remaining potential.
The priority of treatment is represented by
removal of the skin eschar of variable dimensions,
which in itself might herald underlying CS, which
can be solved (Fig.32.3) by simple escharotomy.
The exposed skin areas must then be resurfaced
by simple skin grafts. The second priority is represented by the need to displace nerves of the
forearm, median ulnar and radial, out of the
scarred bed, so they should be supercialized
when the skin problem is solved (Figs.32.3 and
32.5), considering that the necrotic and therefore
removable muscle is already easily recognizable
after three weeks [11].
32.5.2 Surgical Techniques
The surgical techniques are adopted according to
the well-established grade of severity of the syndrome [21].
The substantial difference between newborn
and adult is that newborn severity of involvement
can vary signicantly, ranging from no muscle
ischaemia to complete exor and extensor compartment loss. In the newborn if acute (fresh),
extensive skin lesions are noted over the exor
and/or extensor surfaces; emergent fasciotomy is
recommended, especially if distal cyanosis is
present. Timing is critical as the compartments
must be released before ischaemia and muscle
death occurs. If the skin lesions appear to be
older (i.e. partially healing), waiting to explore
and debride the involved muscle and performing
neurolysis are warranted. When the infant is stabilized, formal exploration and debridement of
the dead muscle and neurolysis are warranted to
prevent xed contracture. Importantly, if the FDP
(exor digitorum profundus)/ FPL (exor pollicis longus) muscles are debrided, the stumps of
the tendons should be secured in the forearm to
the radius to prevent retraction into the carpal
tunnel, which makes secondary reconstruction
much more difcult. Also, the anterior interosseous nerve should be tagged for easier identication at the time of reconstructive surgery. In these
severe cases, free functional muscle transfer can
restore reasonable function for the patient. In the
adult and paediatric population, the main involvement is located at the anterior surface, where the
following treatment guidelines are usually suggested, based on the classication of the extent of
damage according to Tsuge [21]:
• Mild localized type extensor compartment,
scar tissue removal and planning for palliative
surgery
• Mild localized type deep exor compartment:
muscle sliding operation
• Moderate type deep and supercial exor
compartment + neurological decit: exor
sliding with neurolysis median and ulnar
nerve

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Fig. 32.4 (a) Severe outcome of a deep amniotic band
and at the forearm. (b) Coping strategies by holding
objects between the trunk and the affected limb (c) X-rays
show discontinuity of the radius and ulna caused by the
amniotic band. (d) Possibility to support the body at the
• Severe type supercial and deep exor compartments + extensor compartment: single
[52] or double free muscle transfer [13, 14]
site of pseudarthrosis of the forearm. (e) Follow-up at 18
years. The adopting parents have not consented to stabilize of the forearm bones early in life. (f) Patient is bimanual in virtue of the normal function of the elbow joint and
in spite of the signicant limb discrepancy
In the newborn, normally the extensor muscles of the wrist are spared and scarring is limited
to the EDC (extensor digitorum communis). We
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