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34 Rehabilitation in Congenital Hand and Forearm Defects: Rehabilitation of the Child’s Hand—General…
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Image 34.26 Scar
ated nger encouraging an harmonious
movement.
• On reaching the eighth after surgery week in
Image 34.27 Ulnar polydactyly
the case there remained an extension rigidity a
dynamic splint for the exion of the interphalangeal joints can be packaged.
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34.9.3 Ulnar Polydactyly
Ulnar or post-axial polydactyly is characterized
by the presence of a supernumerary portion that
may be a small oating appendage or a complete
normal nger formed on the ulnar side of the
hand. Surgical treatment of choice consists in the
removal of the supernumerary segment with variable techniques based on the presentation. The
rehabilitative treatment in ulnar polydactyly has
as a short-term objective the protection of the
operated structures while in the long term it is
necessary to work on the recovery of the global
mobility of the hand, in particular of the ulnar
segments (Images 34.27 and 34.28).
Below we describe the rehabilitation phases.
34.9.3.1 0–4 Weeks
• Immobilization with a compressive dressing
and mobilization of the free segments.
34.9.3.2 4–6/8 Weeks
• Scar treatment with silicon patches and any
splint to open the space between the ngers
Image 34.28 Picture post ulnar polydactyly surgery
and maintain longitudinal axis of the nger
(Image 34.29).
• Long ngers mobilization, in particular of the
ulnar segments.
• Game activities that stimulate the child to use
the hand with particular attention to the integration of the last two ngers in the grips of
large objects and general dexterity.
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Image 34.29 Removable splint in ulnar polydactyly
• If necessary a syndactyly can be used to avoid
the exclusion and stimulate the integration of
the nger in grip movements.
E. M. Mancon et al.
34.10 Arthrogryposis
Arthrogryposis is a malformation present at birth.
The stiffened joint is the major problem for
this little patient.
It always affects the upper arm and the pathol-
ogy could be distal or proximal; this malformation could affect also the spine and the legs.
The diffuse stiffness and the strength reduc-
tion prevent both the movement and the functionality of the arms. Usually the stiffness is bilateral
(Image 34.30).
There are rarely problems related to the devel-
opment and the sensibility.
The conservative treatment should start as
soon as possible and it should be carried on during his entire childhood.
Unfortunately some of the patients arrive in
our unit at school age.
The origin of this pathology is uncertain and
the manifestation is different depending on the
case.
The typical stiffness of the upper arm is:
• Shoulder: adducted and intrarotated
• Elbow: extended and stiffened
• Wrist: exed and deviated ulnarly
Image 34.30 Bilateral stiffness of the upper limb
• Fingers: extended, deviated ulnarly, and
camptodactilous
• Thumb: exed adducted and in palm (Images
34.31–34.33)
The objective of the conservative treatment is
to achieve the best mobility of the stiffened articulation and improve PROM and AROM of the
shoulder, elbow, wrist, and ngers to maximize
the grip that is important for touching and exploring the environment to optimize the grip.
It is important to recuperate these specic
joints:
– The shoulder controls the arm orientation.
– The elbow is responsible for feeding, touch-
ing, and personal care.
– The wrist is responsible for stabilizing the arm
during the grasp.
– The ngers are responsible for touching and
playing.
– The thumb is responsible for the ne pinch.
The mobilization in the correct position is the
rst thing to do, and then we prepare the splint to
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Images 34.31–34.33 Hand and wrist position
maintain the achieved position. The little patient
wears the splint in the night and during the day; it
depends on the age and the conditions.
Play is the best tool for the reeducation.
With the active and passive mobilizations we
obtain the best position for the arm. The mobilization consists of passive stretching and pressing
the baby to play for cognitive development.
The splint is prepared as soon as possible,
sometimes as early as 20 days after birth; it can
be as small as a pen (Image 34.34a–c).
It is made by thermoplastic material modeled
with warm water. The splint substitutes the old
serial cast.
At the start we do manipulation and stretching
and then using the splint we maintain the ROM.
The splint also increases the ROM.
It is removable and it must be controlled fre-
quently because of the baby development.
At the beginning the baby must always wear
the splint and it should be removed only for the
bath.
Then when the ROM is achieved it is possible
to wear it only during the night.
The splints are also useful for playing or eating without problem to keep the toy or the spoon
(Image 34.35).
But if we also have the problem with the wrist
and the ngers in exion and deviation, it is possible to prepare a splint for the wrist and nger to
be joined together.
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E. M. Mancon et al.
a bc
Image 34.34 (a–c) Example of splint
Image 34.35 Functional splint for the wrist
Sometimes it could be advisable to make a
dynamic splint to maintain the thumb open for
the activities.
To maintain the correct position of the wrist
the thumb and the nger are a great problem for a
baby with arthrogryposis.
We use also the k-tape for the thumb for the
best outcome. Taping provides immediate sensomotor feedback regarding functional abilities.
The k-tape is elastic, latex-free, very thin, and
stretches in the longitudinal plane.
It has been suggested to provide proprioceptive input.
Pressing the baby to play is the rst means
available to stimulate and mobilize.
It is important to remember that the activities
are selected depending on the age and the development of the baby.
In conclusion we must remember that the conservative treatment should start as soon as possible and be “aggressive”; we have to monitor the
splint very closely in the rst 2–3 years based on
a tailor-made strategy and involving the parents.
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34.11 Amniotic Band Syndrome
(ABS)
Amniotic Band Syndrome (ABS) can involve the
upper limbs at different levels: forearm, arm,
hand, or ngers.
Some cases require immediate surgery; in fact
a very strict band can compromise blood circulation and the tissues survival. Little patients can
undergo surgery very few hours after birth if necessary to save the whole upper limb or a single
segment.
After surgery, rehabilitation protocol is started
as soon as possible. Surgery could require an
immobilization period to ensure a correct scar
healing. The immobilization’s length is decided
by the surgeon, and it depends on the structures
involved. If necessary, a static thermoplastic
splint is made on the bandage to keep the arm and
ngers in the right position.
Sometimes surgery can be necessary but needs
to be delayed because of other medical needs of
the baby; in this case a preoperative period of
rehabilitation can be suggested to evaluate the
baby’s motricity, to stimulate motorial abilities,
and to stretch soft tissues where necessary.
Each case must be analyzed both by the surgeon and therapist in order to study the better
treatment for the baby. Evaluation is important to
nd the structures involved or limited by ABS:
vessels, bones, tendons, muscles, and nerves.
Parents will be taught by the therapist to do rehabilitation sessions at home: parents and caregivers collaboration is always fundamental to
achieve better results.
34.11.1 Postoperative
Rehabilitation
34.11.1.1 Splint
Thermoplastic splint is tailor-made on the child’s
hand. It must be done respecting the structures
involved in surgery and putting the body segments involved into a safe position. If necessary
it can be unremovable in the rst healing period
and after that a removable one is done by the
therapist. Parents will be taught to put it on and
off during the night and the day.
34.11.1.2 Active Mobilization
Adequate stimulation, based on patient’s age, is
important to improve active motricity and abilities to pick up objects and explore the surrounding environment. Taping helps to improve active
motion: it can be used to stimulate or reduce
muscles activation or to stabilize segments in
order to have a better and correct active motion.
34.11.1.3 Passive Mobilization
Compatible with surgery and time of healing,
passive mobilization is important to improve
joints’ range of motion (ROM). During mobilization the little patient should not feel any pain; this
kind of mobilization prepares structures and
joints to a better active motion.
34.11.1.4 Scar Treatment
As soon as the scar healing is complete, it is
important to massage it in order to have soft scar
tissue. Scars need compression made by silicon
sheets or elastomer put under a splint worn during the night or sleeping time of the baby.
Sensitive stimulation is important to gain a good
sensibility in the area of the scar.
34.11.1.5 Edema Treatment
After surgery and eventually immobilization,
edema could be present and could limit active
and passive motion. Scars or the remaining part
of the amniotic band can make it difcult to
resolve it but through massage, bandage, and taping the scar can become elastic enough to allow
all the movements needed.
34.11.2 Preoperative Treatment
34.11.2.1 Splint
After an adequate evaluation, a removable thermoplastic splint can be done to stretch soft tissues and
to put in a safe and correct position the segments
involved in the ABS.Splint is usually worn during
the night or the sleeping time of the baby.
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34.11.2.2 Passive Mobilization
Stretching soft tissues and mobilizing joints
involved is extremely important to have a better
active motion, like important to arriving at the
surgery time in the better condition as possible, in
order to have an easier postoperative
rehabilitation.
34.11.2.3 Active Mobilization
As every kind of rehabilitation, stimulation must
be adequate at child’s age in order to maintain
and improve active abilities. Taping can help
muscles activation and can be useful to improve
active motion and complete recovery.
34.12 Epidermolysis Bullosa
E. M. Mancon et al.
Epidermolysis bullosa (EB) is, as we described in
the previous chapter, a rare and disabling genetic
disease that causes boils and lesions on the skin
and in the inner mucosa, symptoms that can
occur spontaneously or because of the slightest
friction. It is also known as “buttery children’s
syndrome,” referring to the fragility of patients in
common with the proverbial of the wings of a
buttery. The tissues involved are those underlying the basal lamina; therefore the healing of the
lesions involves the formation of scars and this is
of particular interest in the treatment of the upper
limb and even more the hand.
The treatment of these young patients will be
at 360° on the various systemic problems, focusing on all aspects of the pathology in the various
localization of onset.
The most affected areas to the formation of
lesions and bubbles are the parts most exposed to
friction, therefore the hands and feet, often ulcerated with loss of skin annexes.
In the rst months and years of life, the
repeated small traumas to the hands and the consequent injuries, followed by the cicatrization
phase, imply the formation of more or less tight
syndactyls, until the hand remains closed in a st
with the long ngers exed and the thumb
adducted (Image 34.36). This position, if maintained for a long time, can cause serious injuries
up to the loss of the nails and sometimes the last
phalanx and almost always marked and signi-
Image 34.36 Hand with EB
cant retraction of the rst web, with notable and
serious repercussions on both the functional and
the morphological levels.
The main goal in these patients is to preserve,
recover, and maintain the functionality as long as
possible to give the patient autonomy in the management of the daily life activities.
The approach on this pathology, that is
extremely disabling and recessive, is for most of
the cases surgical; rehabilitation is a very important tool to optimize and maintain the results that
have been obtained through surgery and can also
delay the relapse that, unfortunately, always
occurs. In the dystrophic-recessive EB the majority of literature agrees about the surgical treatment
that consists in a degloving of the ngers and a
widening and opening of the rst web.
Rehabilitation starts early with a good medication and splint with a good position of ngers
and wrist. Each nger is abducted and extended
as much as possible to guarantee a better healing
and elasticity of the skin.
The dressing will be cleaned up and done if
necessary in the surgery room to optimize the
results but most of all to respect the pain on our
little patients; it is very important to keep the
hand as open as possible until the complete healing (Image 34.37a–c).
bc
ab
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a
Image 34.37 (a–c) The post medication splint
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Image 34.38 (a, b) Splint
When the wounds are completely healed the
splint will be removed and it will be possible to
start with rehabilitation. The mobilization will
be active at rst and playing will be encouraged,
of course in different ways for different ages.
The sessions have to be associated with a tailormade “shell” splint: in this splint the digits are
abducted and the webs are compressed with
paddings.
As we wrote in the introduction making a
pediatric splint requires a great ability and a high
experience and this is for all the children, however we can say that splints for EB are the most
difcult ever. This is because every little pressure
or every small part of the splint without padding
can cause a rubbing on the skin and this means
excoriation and wounds that can take a lot of time
for healing. This problem can also speed up the
relapse. A great attention has to be made on pad-
dings and the choice of materials, often splints
for EB patients need to be covered and protected
also externally to avoid rubbing on the face or on
other parts of the body (Image 34.38a, b).
Every exercise or game has the aim of opening
the hand as much as possible, especially for the
widening of the rst web to promote its
independence.
Because of the skin fragility in those patients
the scar massage and the use of silicon patches
are not suggested, to avoid the occurrence of
hypertrophic scars (Image 34.39).
Physically those patients are extremely limited; however it is very important for the therapist
to remember that cognitively they are perfectly
on time and this is why they need to improve in
daily activity as much as possible; an appropriate
and individual ergonomic proposal is needed and
different for every little patient.
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Image 34.39 Silicon patch with elastomer
34.13 The Rehabilitative Treatment
oftheObstetrician Paralysis
The rehabilitative treatment of the obstetrician
paralysis is a long process that must start with the
diagnosis, i.e., since the birth until the end of the
growth of the little patient.
To adequately deal with this long way, it is
necessary that the therapist knows the anatomy of
the involved structures associated with the
lesions, the timing of the nervous regeneration,
the problems related to the nervous lesion, and
the phases of the development of the child’s
motor and cognitive competences.
Knowledge from all these allows us to elaborate a rehabilitative project whose long-term aims
are streamlining of functional recovery and the
overtaking of all the psychomotor development
phases.
To reach the aims, all the operations adopted
by the therapist must have as a primary purpose
the prevention of secondary lesions of the musculoskeletal and cutaneous apparatus generated by
the nervous lesion, the promotion of the activation of muscle groups under reinnervation, and
the facilitation of the development of a correct
body scheme that includes the superior limb
involved in the nervous lesion.
In order to optimize the treatment results, it is
necessary to involve the parents who must be
taught about the meaning of their child’s pathology, about its correct management, and espe-
E. M. Mancon et al.
cially about how to carry out the exercises at
home.
The parents must be informed about the
child’s possibilities based on the evolution of the
clinical picture. Only through the awareness of
the clinical picture they will be motivated to
reach specic objectives for each development
phase.
According to the level and the gravity of the
lesion, the functional recovery can be spontaneous or a primary operation might be necessary
after 3 or 4 months of birth if the biceps action is
absent as well as palliative operations to balance
out residual motoric decits at the age of 3 or 4.
In all of these cases a rehabilitation has a primary importance because it allows the prevention
of rigidity development as well as joint deformation, the denervated muscle hyperextension, and
the neglection of involved limb.
For each lesion level, the superior limb tents to
acquire specic faulty attitudes that must be
known to be prevented. In case of high lesions
(C5, C6, C7), there is the risk of adduction rigidity and internal rotation of the humerus, in pronation of the forearm and the rigidity in the exion
of the wrist and the ngers. Therefore in this type
of lesions the passive mobilization must be
focused on the maintenance of the external passive complete rotation, on the elevation and
abduction of the shoulder.
The rigidities of the inter-rotation are very
common and dangerous as they lead to a bone
deformity of the humerus head and they promote
the posterior sub-dislocation of the humerus
head. They are caused by the withdrawing of the
shoulder inner-rotation muscles, the front capsule, and the anterior glenohumeral ligament.
The bone deformations of the humerus head
do not allow the mechanic rotation of the head
itself inside the glenoid cavity of the shoulder
blade, and they make impossible the external passive rotation and as a consequence they frustrate
any possibility of recovery of the active
movement.
The most efcient intervention to lengthen the
inner rotator cuff, above all the subscapular one,
is the one with abducted humerus and 90° bent
elbow. In case of premature rigidities it is recom-
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mended to tailor a night tutor which maintains
the humerus in abduction and extrarotation, in
order to keep the muscles retracted and in extension for many hours.
The second risk in these lesions is the dislocation of the radius head that could happen in case
of operations of forced supine position and it
could provoke, as a consequence, a rigidity in the
exion of the elbow.
In low lesions (C8 T1) the risk of rigidity is set
in the supine position of the forearm and the
deformations of the hand are similar to the ones
provoked by the lesions of median and ulnar.
In total lesions risks and problems are summed
up and they lead to a much more difcult clinical
picture.
The mobilization must be carried out by the
parents at home many times a day; it must be
gentle and complete and it must cover all the
involved zones of the lesion.
During the rst week of birth, it is suggested
to keep the arm at rest through the use of the pin
that maintains the humerus close to the chest and
the elbow in bent position, to facilitate the reabsorption of the post-traumatic, mostly in case of
associated lesions as the collarbone breaking.
At the end of the rst week, the program of
passive mobilization of all the districts of the
superior limb should start; it has to be gently and
delicately carried out, without forcing the joint to
the maximum grade and by making stable the
proximal joint compared to the mobilized one.
During the arrangement of the mobilization
program associated possible lesions have to be
considered; according to them, timing and procedures of the mobilization and the treatment must
be modied. The most frequent lesions are the
breaking of the collarbone and the humerus as well
as the lesion of the phrenic nerve and, as a consequence, the alteration of the breathing pattern
caused by the ipo-validity of a hemidiaphragm.
The parents have to be informed not only on
the procedure of mobilization but also on the
baby’s daily handling, in order to avoid other
traumas and tractions of the arm.
Proper strategies have to be taught for the
baby’s nappy changing moment, for breastfeed-
ing, for the position during sleeping and for the
moment in which the baby is awake.
A very useful but practical example, which
could seem banal, is to dress up the baby starting
from the damaged arm and continuing with the
healthy one, and vice versa to undress him.
The little attention allows the parents to reduce
at the minimum the arm traumas in the baby’s
changing moments.
Finally, we have to teach the importance of
stimulating the involved arm with surfaces of different consistency, put themselves to the side of
the damaged arm, and so on, to facilitate the integration of the arm into the body scheme.
As it is in this very rst phase, the use of positional static tutors to obtain the correct alignment
of the wrist and the hand is suggested. In case of
total lesions it must be produced a tutor in safety
position (extended wrist MF bent IF in position
zero, thumb in opposite position with the opening
of the rst space), and in case of high lesions C5,
C6, and C7 a tutor in extension of the wrist and,
if needed, one tutor to correct the attitude in
extension and pronation of the elbow.
The use of tutors is an integrating part of the
obstetrician paralysis treatment. They can be
static, dynamic, or functional, for the wrist and
the hand or for the elbow, according to the different phases and to the baby’s clinical case.
In addition to the tutors, another tool at disposal is the functional bandage, used to control
the instability of the shoulder blade, to encourage
the shoulder movement, or to correct with attitude in pronation of the forearm.
In addition to the passive mobilization, the
therapist has to stimulate the active recruitment
of the muscular groups in the innervation phase,
through postures, games, and movement that call
and reinforce those muscular groups.
With the child’s birth, games and manual
activities have to be proposed to him and sports
like swimming and dancing are recommended;
they are not only useful as a muscular reinforcement but also as a psychological crossing of the
diversity given by the primary lesion.
The most frequent residual decits in the older
children are, in the high lesions, the complication
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E. M. Mancon et al.
in the active abduction and in the elevation of the
shoulder, the asymmetry in the exion of the
elbow, and the decit in the supine position.
The elevation decit is due to the weakness of
the anterior deltoid and to the hyposthenia of the
external rotators, whose activation allows the
greater tuberosity to turn and pass under the coracoacromial arch.
Therefore in the reinforcement program, the
deltoid and the external rotators of the humerus
have to be reinforced.
Another particular element is the detachment
of the medial border of the scapula during the
movement of abduction of the shoulder.
The scapula mobility must be optimal to allow
the recovery of scapular-humeral rhythm and, in
case of muscular decit that activates the
scapular- humeral one, the movement of the
scapular- thoracic allows a better orientation of
the humerus into the space.
Associated contractions are another problem.
They inhibit the expression of movements functionally valid for the lack of the normal balance
between agonist and antagonist muscles.
Co-contractions and hyperactivity of muscular group that prevents to the ipo-valid antagonist
to express its strongness (for example the hyperactivity of the triceps that causes a decit in the
exion of the elbow) can be treated with the use
of botulinum toxin. This procedure is under
experimentation.
In the obstetrician paralysis treatment the
patient’s global vision must not be forgotten.
With the growth, the imbalances generated by the
asymmetry of the superior limbs must be
supervised.
Sometimes other problems can occur, like the
scoliosis or the lumbar hyperlordosis, which
increases the effort of elevation of the arm.
For the complexity of the pathology and for
the presence of many factors to be evaluated, the
treatment has to be carried out by a multidisciplinary equipe composed of the surgeon, the
physiotherapist, the pediatrician, and the
psychologist.
The role of the psychologist is fundamental to
follow the parents the initial phase and, in the
second period, the baby, who in the growth
become aware of the problem.
34.13.1 Summary
oftheRehabilitative Project
oftheObstetrician Paralysis
0–3 months: aims of physiotherapy the muscularskeletal secondary lesions stimulations sensibility involvement of the damaged limb into the
development of the body scheme stimulation
active muscular recruitment correct placement
and handling.
34.13.1.1 Activation oftheBiceps?
YES: carry on with FKT: aim: progressive stimulation of the active muscular contraction and
development of the psychomotor scheme.
NO: primary surgery intervention:
0–3/4 weeks: xed tutor in damping
4 week: begin with the mobilization in damping
and the placement in damping since week 5:
carry on with the passive mobilization and the
progressive muscular recruitment together
with the recovery due to the distal reinnervation (see paragraph).
34.13.1.2 3–4 Years Old: Sucient
Functionality forADL?
YES: carry on with a focused FKT and sport
NO: possible palliative intervention (see
paragraph)
34.13.1.3 Supervision ofFKT Until
theEnd
oftheDevelopment
Pay attention to the posture, stimulation for the
use in the ADL, sport activity (Images 34.40,
34.41, and 34.42)
34.13.2 Post-surgery Treatment
Surgery for both primary and palliative obstetric
paralysis has to be considered a rehabilitative
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