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c
d
e
Fig. 23.10 (a) Planned soft tissue incision. (b) Soft tis-
sue excised along with neurovascular bundle. (c)
Illustration of how the soft tissue incision should come
together. (d) Flexion osteotomy is used to correct
wound closure and ensuring that the longitudinal
scars remain dorsal to the mid-axial line. Excision
of the distal portion of the nail plate and sterile
matrix is also an option.
The parents should have clear understanding
that these digits will never have the appearance of
the unaffected contralateral digit, that they will
hyperextension of the distal phalanx, and tuft excision
narrows the dorso-palmar thickness of the ngertip. (e)
End result of debulking and sagittal plan correction with
K-wire in place [5]
be stiff, and that they may need multiple surgeries throughout life.
Figure 23.12 shows a typical patient with
macrodactyly of the index and middle ngers.
Note involvement of all the structures with signicant angulation. As stated above, debulking
on only one side of the nger is performed with
ac
ef
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b
d
Fig. 23.11 (a, b) Incision planning for debulking and
coronal plan correction of middle nger with planned
incision illustration. (c) Nail plate to be excised. (d)
Rotation of the soft tissue to create new perionychial fold.
(e) Extensive debulking grossly overgrown digits. (f)
Postoperative image after debulking and angular
correction with pinning [5]
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S. N. Oishi et al.
Fig. 23.12 (a, b) Clinical photograph and radiograph
showing the large ngers with marked angulation. (c) The
debulking procedure. Note the large digital nerve around
the blue rubber band. (d) The appearance of the middle
excision of associated large digital nerve and the
overlying skin supplied by that nerve. Despite
removal of the nerve, these patients usually
retain good sensation to the tip of the nger.
Physeal closure was performed at the same setting as the ngers had achieved the length of the
same sex parents. In addition, because of
involvement of the hand and median nerve, the
nger after debulking with markings for the debulking of
the index nger. Note that the extension extends to the
carpal tunnel to allow debulking in the hand and carpal
tunnel release
incision was extended proximally to the carpal
tunnel to allow hand debulking and carpal tunnel release.
Because of the generalized overgrowth and
stiffness present, amputation is a good option in
some cases. This is especially true in situations
where the large digits are syndactylized
(Fig.23.13).
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a b
279
Fig. 23.13 (a, b) Syndactyly of macrodactylus digits. (c) Note the large digital nerves present in these digits. (d) Good
function noted after primary ray amputation of the index and middle ngers
Even if successful syndactyly reconstruction
is performed, patients will bypass the two stiff,
large digits and grasp between the thumb and
ring/little ngers. Long-term primary amputation
is a much better option.
A patient may present with an index nger
macrodactyly in which the nger is very stiff
with very little motion or have a nonfunctional
nger after attempted debulking, as shown in
Fig.23.14. Because the nger is very large and
stiff, the child will bypass it. Ray amputation is
an excellent option in these cases and can improve
overall hand function.
In cases where the thumb is extremely large
without much function, it would be tempting to
perform a microvascular toe to thumb transfer.
The issue has always been whether the transplanted digit would eventually be affected with
the same overgrowth. Recently, there have been
several isolated case reports describing successful transfer without enlargement, but certainly
long-term follow-up is required before recommending this as standard treatment [7].
Macrodactyly patients need to be observed
closely for carpal tunnel syndrome since a lipobromatous hamartomas of the median nerve is
frequently present (Fig.23.15) [8].
Because this is present at a very young age, it
is doubtful that complaints of paresthesias will be
voiced and waiting until the presence of ngertip
dryness or thenar wasting is not indicated.
Clinical manifestations may be frequent biting
of the ngers or shaking the hands. Carpal tunnel
release should be done through generous incisions that will provide coverage of the released
nerve. Step-cutting of the transverse carpal liga-
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S. N. Oishi et al.
c
Fig. 23.14 (a) Large stiff macrodactylus index nger at 14 months of age. (b, c) Result after ray amputation of the
index nger
Fig. 23.15 (a, b) Patient with macrodactyly involving the median distribution of the hand. Note the large lipobroma-
tous hamartoma of the median nerve
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ment to facilitate a loose repair will help prevent
prolapse of the enlarge nerve. It is tempting to
either debulk or shorten the nerve, but this should
not be done, nor should a biopsy be taken, as
these manipulations are not helpful and may
result in difcult pain issues.
23.5 Conclusion
While macrodactyly can be associated with syndromes such as neurobromatosis and Ollier and
Klippel-Trenaunay syndromes, many cases are
postzygotic mutations involving upregulation of
PIK3CA and can be components of PROS. Close
monitoring of these patients is warranted as rapid
growth can be seen at an early age, with this subset of patients being candidates for mTOR inhibitor treatment. Surgical intervention is warranted
in some patients, but stiff ngers will be expected
despite aggressive debulking procedures. The
goal for these patients is to achieve the most aesthetically pleasing nger(s) with minimal morbidity and good overall hand function. Parents
must be counseled about realistic goals and
expectations early on to assist them in making
reasonable decisions for their child.
References
1. Rios JJ, Paria N, Burns DK, Israel BA, Cornelia R,
Wise CA, et al. Somatic gain-of-function mutations
in PIK3CA in patients with macrodactyly. Hum Mol
Genet. 2013;22(3):444–51. Epub 2012/10/27.
2. Keppler-Noreuil KM, Rios JJ, Parker VE, Semple
RK, Lindhurst MJ, Sapp JC, et al. PIK3CA-related
overgrowth spectrum (PROS): diagnostic and testing
eligibility criteria, differential diagnosis, and evalua-
tion. Am J Med Genet A. 2015;167A(2):287–95. Epub
2015/01/06.
3. Ben-Bassat M, Casper J, Kaplan I, Laron Z.Congenital
macrodactyly. A case report with a three-year follow-
up. J Bone Joint Surg Br. 1966;48(2):359–64. Epub
1966/05/01.
4. Hardwicke J, Khan MA, Richards H, Warner RM,
Lester R. Macrodactyly - options and outcomes.
J Hand Surg Eur Vol. 2013;38(3):297–303. Epub
2012/06/28.
5. Gluck JS, Ezaki M. Surgical treatment of macro-
dactyly. J Hand Surg. 2015;40(7):1461–8. Epub
2015/06/08.
6. Li J, Kim SG, Blenis J. Rapamycin: one drug,
many effects. Cell Metab. 2014;19(3):373–9. Epub
2014/02/11.
7. Cavadas PC, Thione A. Treatment of hand macro-
dactyly with resection and toe transfers. J Hand Surg.
2018;43(4):388.e1–6. Epub 2017/09/21.
8. Amadio PC, Reiman HM, Dobyns JH.Lipobromatous
hamartoma of nerve. J Hand Surg. 1988;13(1):67–75.
Epub 1988/01/01.
Palliative Surgery inObstetrical
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Brachial Plexus Palsy
FilippoM.Senes, NunzioCatena,
andChiaraArrigoni
24
Abstract
The chance of recovering functional defects of
obstetrical palsy through nerve surgical procedureshas not reduced the role of palliative or
secondary surgery, also called “functional surgery.” Although nerve injury at birth is not a
progressive lesion, growth changes of the
upper limb and skeletal adaptations can signicantly inuence recovery, especially during adolescence, when body size changesand
creates an imbalance thatcan impairalready
achieved functions. In particular, skeletal
deformities can decrease jointrange of motion
as much as muscle imbalance due to nerve
palsy. Secondary surgery would aim to achieve
some essential functions of the upper limb (a
satisfactory shoulder motion in external and
internal rotation, a signicant active exionextension of the elbow, good wrist control,
F. M. Senes (*)
Department of Hand Surgery and Rehabilitation,
San Giuseppe Multimedica Hospital, Milan
University, Milano, Italy
e-mail: lipposenes@fastwebnet.it
N. Catena
UOSD Microchirurgia Ricostruttiva e Chirurgia
della Mano, IRCCS Istituto Giannina Gaslini,
Genova, Italy
C. Arrigoni
Scuola di Specializzazione in Ortopedia e
Traumatologia, Università degli Studi di Torino,
Torino, Italy
and an opposable thumb with adequate hand
grasp). Depending on patient’sage, there are
denite steps to perform surgery. In the arti-
cle, indications to correct deformities induced
by sequelae of obstetrical palsy of the upper
arm are reported.
Keywords
Obstetrical brachial plexus palsy · Brachial
plexus palsy sequelae · Shoulder motion
limitation · Elbow motion limitation
24.1 Introduction
The chances of recovering from functional
defects of obstetrical brachial plexus palsy
(OBPP) through both early and latenerve repair
have not reduced the importance of palliative or
secondary surgery, also dened as functional surgery. It is well known that a severe functional
impairment stems from the primary nerve lesion.
Nerve palsy is consistently followed by muscular
palsy and imbalance, both altering bone growth
and triggering joint incongruence. Although
nerve injury at birth is not a progressive lesion,
growth changes of the upper limb and subsequentskeletal adaptations can signicantly inuence therecovery, especially during adolescence,
when body size grows fast and creates an imbalance that impairs already achieved functions. In
particular, skeletal deformities contribute to
© Springer Nature Switzerland AG 2023
G. Pajardi (ed.), Pediatric Hand Surgery, https://doi.org/10.1007/978-3-031-30984-7_24
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F. M. Senes et al.
decrease joint range of motion as much as
themuscle imbalance due to nerve palsy [1].
A basic distinction between patients suffering
from upper root involvement and those with partially recovered complete plexus involvementis
mandatory: the weak muscle strength and reduced
hand sensation indeed signicantly impair the
outcome of this latter group, even for shoulder
function.
The success of palliative surgery depends on
the extension of the palsy, which affects single
segments of the upper arm. Multilevel
malfunctioning of the upper arm, due to different
root involvements, inuences the outcome and
worsens the motion of the upper arm. Indications
for surgery mainly concern upper limb defects
due to upper and intermediate lesions of the brachial plexus, whereas total involvement of the
brachial plexus often discourages palliative surgery or reduces options for treatment.
Moreover, dealing with obstetrical brachial
plexus palsy, we face a complex nerve lesion,
totally different from peripheral nerve lesions in
which there are sufcient muscular groups to
compensate for defects. This is particularly true
for upper arm distal defects, when the palsy
involves several areas of the forearm and hand.
Secondary surgery should aim to achieve
some basic functions, namely, a satisfactory
shoulder motion in external and internal rotation,
a signicant active elbow function allowing exion and extension movements, good wrist control, and an opposable thumb with adequate hand
grasp. Nevertheless, hand motion recovery might
be less predictable, because it is strongly inuenced by the lack of sensation. Unfortunately,
these conditions are difcult to achieve as a
whole, particularly when hand involvement is so
severe as to impair every surgical effort.
According to patients’ age, there are denite
steps to perform surgery.
Some surgical procedures in precise moments
of growth are suitable for correcting deformities,
whereas the same procedures performed in an
inappropriate period might be ineffective or
could even damage the child. Surgeons must be
aware of this basic point and consider it before
operating on patients.
Regarding surgical approaches, it is very
important to avoid trying to reach an idealisticfunction of the limb,if this could determine a
decrease of motion in others, particularly when
advantages might be minimal.
From this standpoint, the therapeutic approach
should aim to correct deformities and maximallyincrease therecovery. The nal goal is not
complete elimination of defects butthe achievement of thehighest degree of recovery.
24.2 Clinical Features
andTherapeutic Options
Based on our experience and the literature, we
would like to describe thechancesof improving
the upper arm motion through surgical procedures, keeping in mind the age of the child.
Although some functional limitations are easily
detected, multiple involvements of musculoskeletal areas have to be considered, especially
because deformities inuence each other.
Since the shoulder and elbow are more commonly involved by OBBP, we will describe in
detail both districtsthat are more suitable for surgical procedures. However, some considerations
about distal segments wil bereported at the end
of the chapter.
24.2.1 Shoulder
In the natural history of neonatal brachial plexus
palsy, the shoulder is constantly involved
because of the injury of C5–C6 roots, which
mainly results in a lack of abduction and external
rotation. The characteristic aspect of the shoulder in the internal rotation is mainly due to muscular imbalance caused by nerve palsy; however,
24 Palliative Surgery inObstetrical Brachial Plexus Palsy
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joint stiffness very often occurs in the rst
months after birth, futher impairing motion.
Shoulder stiffness stems from subscapularis
muscle contraction, which is very frequent, and
from joint cuff and anterior soft tissue retraction;
both conditions modify the shape of humeral
epiphysis because of compressive forces and
cause early loss of congruence of the humeral
head that tends to atten when in contact with
the hypoplastic glenoid cavity [2]. Scapular
winging is a typical feature due to the lack of
congruence of the scapulothoracic girdle
(Fig.24.1). This deformity can be detected either
for internal or external limitation of shoulder
motion, often occurring in some planes of
motion; itdepends on attening of the epiphyseal humeral head and misalignment of the gle-
Fig. 24.1 Scapular winging: the scapular blade rotates
along with the humeral head during the internal rotation
and adduction of the arm showing an evident detachment
of the scapula from the dorsi
noid cavity. Patients and their families
oftencomplain about the aberrant motion of the
shoulder blade, rather than for limitation of
motion [3].
An internally rotated shoulder with limited or
no abduction, scapular winging, minimal or
absent retroposition, and limited internal adduction toward the midline are constant features in
children suffering from upper-intermediate
OBPP sequelae. These patterns varyaccording to
growth changes that occurre in some patients,
namely, hypoplastic growth of the scapulohumeral girdle and upper arm involvementas a
whole, particularly inthe elbow.
In the early 1970s, Mallet assessed shoulder
deformities by drawings of simple gestures scoring different degrees of disability (Fig.24.2).
Later on, Gilbert and Raimondi described an
improved classication through a detailed
description of shoulder limitations, aiming at the
detection of surgical indications. Over the years,
other assessment methods have been presented
with similar purposes [4, 5].
Indications for treatment might differ according to the severity of shoulder impairment that
can address physical therapy alone or surgical
procedures.
At anearly age, Physical therapy is the rst
therapeutic approach, particularly in shouldermotion recovery.
The aim of this chapter is not to discuss physical therapy in detail; however, some indications
must be highlighted such as reduction of muscular imbalance, muscle strength renforcement, and
joint motion increase.
Although physical therapy should be continued during growth, even including some sports,
some clinical features of OBPP sequelae cannot
be treated by physical therapy alone, and very
often surgery is needed.
Swimming has been advocated for ages as the
best solution, but in some conditions as posterior
dislocation of the shoulder, it might worsen
shoulderdislocation.
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F. M. Senes et al.
Fig. 24.2 According to Mallet’s classication, clinical assessment of the shoulder can be explored with simple gestures
to evaluate joint motion
24.2.1.1 Limited External Rotation
Apart from primary nerve surgery during the rst
months of life, which represents the best option
for OBPP without prognostic signs of recovery,
from one to two years of age, limitation of external rotation of the shoulder can be treated through
nerve transfers. When there is a free passive
shoulder motion regardless a complete lack of
activeexternal rotation, isolated transfer of accessory spinal nerve onto suprascapular nerve should
be performed to reinnervate external rotator muscles. However, very often subscapularis muscle
release or coracohumeral ligament section
areneeded to achieve a free passive motion of the
humeral head [6].
Both the procedures act in decompressing the
humeral head, favoring a balance among muscular groups to make up forpalsied external rotator
musclesfunction [7].
In the event of a lack of external rotation combined with soft tissue retraction and joint instability during the rst years of life, many Authors state
that a rebalancing of muscles and shoulder
joint congruence can be attained through tendon
transfers (latissimus dorsi and teres major).
Additionally, an anterior release and open glenohumeral joint reduction should be performed [8–
11]. As previously reported, optionsto achieve a
free shoulder passive motion are subscapularis
muscle release and coracohumeral ligament section. Depending on the surgeon’s preference, anterior release of shoulder joint can be carried out by
either an open or arthroscopic technique [12].
From two to four years of age, subscapularis
muscle sliding or coracohumeral ligament release
might be similarly performed to avoid asymmetrical forces acting on the mostly cartilaginous
humeral head, preventing loss of head sphericity.
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