CHAPTER
110
Thoracic Insufficiency Syndrome
INTRODUCTION
The goals of traditional surgical treatment of spinal deformity,
unchanged since the time of Paul Harrington, focus on correc-
tion of the coronal and sagittal plane deformity by internal skel-
etal instrumentation linked with fusion of the spine. Until
recently, this treatment maxim was universally applied to both
adolescents and infants, without regard to the inhibition of
growth of the spine in younger patients and the possible indi-
rect negative effect on growth of the underlying lungs and pul-
monary function long term. Other factors affecting pulmonary
function in the young child with complex spinal deformity,
such as congenital flail chest from rib absence or lung constric-
tion by fused ribs and scoliosis, were obviously not addressed by
just spinal instrumentation. Campbell et al. in 20032 proposed
a more comprehensive approach to these special young patients
in which their spinal disease was considered only a part of a
global thoracic deformity that impacted volume available for the
function and growth of the lungs as well as the thoracic biome-
chanical expansion capabilities that provide pulmonary func-
tion. They described these patients as having thoracic insufficiency
syndrome (TIS), which was defined as the inability of the thorax to
support normal respiration or lung growth.
The diagnosis of TIS can be based either on a thoracic bio-
mechanical deficiency degrading respiratory performance or
growth inhibition of the thorax from deformity. Either may
results in lethal restrictive lung disease, both short term with
the former and often long term with the latter. Normal respira-
tion depends directly on the presence of normal volume of the
thorax and the ability to change that volume.2 Expansion of the
lungs through inspiration is accomplished primarily by the dia-
phragmatic contraction, providing 80% of vital capacity in
adults,16 with the remaining vital capacity due to lung expan-
sion by anterior-lateral expansion of the chest wall through the
intercostal muscles. The specific biomechanical division of dia-
phragm/rib cage expansion of the lungs to generate vital capac-
ity in children remains unknown.
The most important component of TIS is loss of thoracic
volume from deformity and its effect on respiration. To better
define the types of thoracic volume problems in early spine and
chest wall deformity, so that surgical strategies for their correc-
tion could be standardized, Campbell and Smith9 proposed a
classification of thoracic malformation into distinct volume deple-
tion deformities (VDDs) of the thorax (Table 110.1; Fig. 110.1).
In type I VDD, absent ribs, and scoliosis, the concave lung is
not only small, but the surrounding chest wall is absent, so that
rib cage expansion of the lung during respiration is not possible.
LWBK836_Ch110_p1179-1193.indd 1179
Robert M. Campbell Jr
While the diaphragm is intact, the loss of chest wall integrity
means that when the diaphragm contracts, the lung may just pro-
lapse deeper into the chest without effective expansion.
Substantial chest wall defects are commonly lethal early in life.
Treatment should restore stability and volume to the affected
hemithorax and correct spinal deformity in a growth-sparing
manner.
In type II VDD, fused ribs, and scoliosis, the mechanism of
thoracic disability is similar, but the stable fused chest wall does
allow the diaphragm to function in lung expansion. TIS is pres-
ent since respiration is not normal: there is decreased volume
of the fused rib hemithorax and the chest wall cannot expand
to aid the diaphragm in expansion of the underlying lung. The
other component of TIS, growth inhibition, is also present
since the extensively fused chest wall constricts the growth of
the underlying lung. The three-dimensional thoracic deformity
can be addressed by lengthening and enlarging the constricted
hemithorax by a vertical expandable prosthetic titanium rib
(VEPTR) opening wedge thoracostomy (Fig. 110.2), indirectly
correcting the scoliosis without fusion, so that thoracic spinal
growth can continue to contribute to thoracic volume and lung
growth. Surgical restoration of transverse symmetry of both the
concave and convex hemithorax may provide improved area
and configuration of the base of each hemidiaphragm, which
may improve diaphragm kinetics with its important contribu-
tion to vital capacity, but this assumption awaits further studies.
No known surgical treatment can restore degraded chest wall
movement to a thorax with fused ribs and scoliosis, since the
intercostal muscles are absent, but control or correction of
chest and spine deformity may preserve, or increase, the chest
wall motion of the unaffected contralateral convex hemithorax
and its contribution to vital capacity.
In type IIIa VDD, the JarchoLevin chest wall dystrophies,
the chest wall is rigid and the volume problem is due to global
shortening of the entire thorax from congenital spine defor-
mity, often resulting in lethal early onset restrictive lung dis-
ease. Treatment should lengthen the thorax symmetrically to
restore volume.
In type IIIb VDD, most commonly seen in Jeunes asphyxiat-
ing thoracic dystrophy, the often lethal volume depletion is due
to an abnormally narrow, rigid thorax associated with rib dys-
plasia. Treatment should widen the thorax symmetrically to
restore volume. A transverse plane narrowing of the thorax also
may occur in severe cases of early onset scoliosis from wind-
swept deformity of the chest: the spine rotates into convex
hemithorax with extreme loss of volume and lordosis also folds
shut the concave hemithorax with additional volume losses.
1179
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 1180 Section IX Dysplastic and Congenital Deformities
Volume Depletion
TABLE 110.1 Deformities (VDDs) of the
Thorax9
Unilateral thoracic volume depletion deformity
Type I Absent ribs and scoliosis
Type II Fused ribs and scoliosis
Global thoracic volume depletion deformity
Type IIIa JarchoLevin syndrome
Type IIIb Jeune syndrome, early
onset scoliosis
Treatment should correct spinal deformity and stabilize or
reverse chest deformity.
The surgical treatment of TIS should restore volume, sym-
metry, and biomechanical function of the diseased thorax with-
out growth inhibition. This ideal procedure currently does not
exist, but a new class of procedures, collectively grouped under
the term expansion thoracoplasty, can address the VDDs of
TIS by acute thoracic reconstruction, correcting both spine
and chest wall deformity without inhibiting growth of the spine
or the rib cage.
These procedures are made possible by the VEPTR, a chest
wall prosthesis/ribspine distractor (Fig. 110.3A,B) made by
Synthes Spine Co. of West Chester, PA, recently approved by
the FDA as a Humanitarian Use Device (HUD). Unlike spinal
surgery, in which the instrumentation drives the spinal defor-
mity correction, in VEPTR surgery the acute thoracic recon-
struction drives the spinal deformity correction, providing
increased concave hemithoracic volume with indirect correc-
tion of the scoliosis, with the VEPTR instrumentation added
only after surgical thoracic reconstruction is complete, in order
to internally brace both the thoracic and spinal correction.
III a
II
LWBK836_Ch110_p1179-1193.indd 1180
The devices are expanded periodically afterward just to
keep up with growth of the child, not to gain further improve-
ment. So, its important to hit the home run with the initial
implant surgery.
This chapter will describe the use of VEPTR in treatment of
type II VDD of the thorax, congenital fused ribs, and scoliosis,
through an expansion thoracoplasty called a VEPTR opening
wedge thoracostomy.4,5
VEPTR opening wedge thoracostomy for type II
VDD of the thorax, fused ribs, and congenital
scoliosis
Indications for VEPTR treatment of congenital scoliosis and
fused ribs are as follows:
Three or more fused ribs on the concave side of the curve
Greater than 10% reduction in space available for lung (the
ratio of the radiographic height of the concave lung com-
pared to the convex lung)
Progressive thoracic congenital scoliosis in a patient age 6
months up to skeletal maturity
Presence of TIS.
Contraindications
Inadequate soft tissue coverage for the devices
A body weight less than 25% normal for age
Rigid kyphosis greater than 50
Absent diaphragm function
Inability of the patient to tolerate repetitive surgeries
Absence of proximal ribs for VEPTR attachment.
Contraindications can be addressed. Low body weight with
thin soft tissue can provoke skin slough over the devices, so
preoperative weight gain can be achieved by diet supplementa-
tion such as Ensure, appetite stimulation by the medication
III b
Figure 110.1. Volume depletion
deformities of the thorax.
8/26/11 5:44:34 PM
 Chapter 110 Thoracic Insufficiency Syndrome 1181

A B

Figure 110.2. (A) The fused

hemithorax is sectioned transversely
at the center of the thoracic con-
striction in a VEPTR opening wedge
thoracostomy. (B) The sections of
hemithorax are wedged apart, like
wedging a cast. (C) The VEPTRs
are added to hold the correction.
(Reprinted with permission from
The Journal of Bone and Joint Sur-
gery, Inc; Campbell, RM Jr, Smith
MD, Mayes TC, et al. The effect of
opening wedge thoracostomy on
thoracic insufficiency syndrome asso-
ciated with fused ribs and congenital
scoliosis. J Bone Joint Surg Am 2004; C
86-A(8):16591674.

Periactin, or nasal tube feedings, or G-tube feedings. Significant PREOPERATIVE PLANNING

kyphosis may be reduced by halo traction. The recently
released VEPTR II (Fig. 110.3C) has the capability of variable
rod extension proximally with multiple rib attachment points,
and may provide better control of kyphosis in patients with
TIS. Absence of proximal ribs for VEPTR device attachment
can be corrected by the clavicle augmentation/first rib proce-
dure. A rib autograft is taken from the contralateral side. The
clavicle on the side of the proximal rib deficiency is osteoto-
mized lengthwise, bringing the anterior half under the bra-
chial plexus as a vascularized pedicle, interposing the rib
autograft between the clavicle pedicle and the transverse pro-
cess of T-2. Presence of a history of prior spine fusion, involv-
ing a significant portion of the thoracic spine, is not a con-
traindication, but these patients do not appear to respond as
favorably to VEPTR treatment compared to those with virgin
spines. Rigid curves within the fusion mass do not correct, spi-
nal growth is marginal, and pulmonary outcome tends to be
unfavorable.4
The history should include past respiratory problems and epi-
sodes of pneumonia, the need for oxygen, CPAP, or ventilator
support during episodes of pneumonia, sleep disturbances,
which may suggest early cor pulmonale, and birth histories,
such as prematurity with sequelae of bronchopulmonary dys-
plasia, history of diaphragmatic hernia, tracheobronchial mala-
cia, or other causes of intrinsic lung disease, need to be
documented. Clinical respiratory insufficiency should be
graded by the Assisted Ventilation Rating (AVR)9:
0 No assistance, on room air
1 Supplemental oxygen required
2 Nighttime ventilation/CPAP
3 Part-time ventilation/CPAP
4 Full-time ventilation.
A history of past spine surgery, noting age at operation and
extent of fusion, should also be determined. Congenital renal

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 1182 Section IX Dysplastic and Congenital Deformities
A B
Figure 110.3. (A) Exploded view of the standard rib-to-rib
VEPTR. The rib cradle is superior, the central rib sleeve is seen, and the
inferior rib cradle is below. (B) Exploded view of the standard rib to
spine hybrid VEPTR. The rib cradle is superior, the central rib sleeve is
seen, and the inferior rib cradle is replaced by a 6 mm spinal rod.
(C) The VEPTR II implant. Both the inferior and superior ends are 6
mm titanium alloy rods that can be cut to length, and superior/inferior
rib cradles and inferior hook in hybrid constructs are threaded onto the
rods and secured by a locking nut mechanism (arrows). In kyphosis, the
superior rod can be left long and bent downward to reach the kyphotic
rib cage, and clusters of rib cradles can be threaded onto the rod to
attach to multiple levels of the rib cage to distribute load. The expand-
able portion of the VEPTR II starts at the apex of the kyphosis and
extends down to the inferior endplate of T-12. At implantation, the
superior rod is contoured to only gently pull upward on the kyphotic
thorax. At subsequent lengthening procedures, the device is both
lengthened at the expandable portion site, and a separate limited expo-
sure is made over the superior rod to gently extend it further with in-situ
benders to help further correct kyphosis. (C, Courtesy of Synthes.)
LWBK836_Ch110_p1179-1193.indd 1182
abnormalities should be ruled out by prior ultrasound or MRI,
and spinal cord abnormalities ruled out by MRI. The incidence
of spinal cord abnormalities in patients with fused ribs and sco-
liosis can be as high as 43%.7
The physical examination should include measurement of
the resting respiratory rate, and this is compared to normative
values to determine if there is occult respiratory insufficiency.
Fingers are examined for clubbing, a sign of chronic respira-
tory insufficiency. Standard spinal evaluation for curve flexibil-
ity, head decompensation, truncal decompensation, and trunk
rotation is included. Thoracic function through respiratory
chest wall expansion is tested by the thumb excursion test,2 in
which the examiners hands are placed around the base of the
thorax with the thumbs posteriorly pointing upward at equal
distances from the spine. With respiration, the thumbs move
away from the spine symmetrically because of the anterior lat-
eral motion of the chest wall (Fig. 110.4). Greater than 1 cm
excursion of each thumb away from the spine during inspira-
tion is graded as 3, and this is normal, 0.5 cm to 1 cm excur-
sion is graded 2, motion up to 0.5 cm is graded as 1, and
complete absence of motion is graded 0. Each hemithorax is
graded separately. The concave fused rib hemithorax often has
a 0 thumb excursion test, and if there is significant rib hump
deformity of the convex hemithorax, it will also be stiff, and
have a 0 TET. Absence of chest wall motion is abnormal respi-
ration, one sign of TIS.
Preoperative radiographs should include anteroposterior
(AP) and lateral of the spine, including the rib cage and the
pelvis. Cobb angle, head and truncal decompensation, and
space available for lung are determined. Space available for
lung, the radiographic height of the concave lung (measured
from the dome of the hemidiaphragm to the center of the most
proximal rib ipsilateral) divided by the height of the convex
lung, is a valuable measure of thoracic deformity and probably
reflects adverse changes in lung function better than the Cobb
angle. Bowen et al.1 in a study of patients fused early for scolio-
sis found that space available for lung (SAL) correlated with
L R
Thumb Excursion Test
Figure 110.4. Thumb excursion test. (Reprinted with permission
from The Journal of Bone and Joint Surgery, Inc, Campbell RM Jr,
Smith MD, Mayes TC, et al. The characteristics of thoracic insuffi-
ciency syndrome associated with fused ribs and scoliosis. J Bone Joint
Surg Am 2003;85:399408.)
8/26/11 5:44:34 PM
 Chapter 110 Thoracic Insufficiency Syndrome 1183

vital capacity, while there was no correlation between FVC and
the Cobb angle. Supine lateral bending radiographs are used
to determine flexibility and extent of hemithorax constriction
on concave side of the curve. If kyphosis is present, a cross table
lateral radiograph with a bolster under the apex of the kyphosis
is taken to determine flexibility of the kyphosis. Cervical spine
series, including flexion/extension laterals, are performed to
document any cervical spine abnormality or instability.
CT scans are performed to assess the three-dimensional
deformity of the thorax, areas of spinal dysraphism, and pres-
ence of intrinsic lung disease such as bronchiectasis, or chronic
atelectasis. The CT scan is performed at 5 mm intervals, unen-
hanced, including cervical spine, chest, and lumbar spine. A
fluoroscopy or dynamic lung MRI is performed to document
normal diaphragm function, and a screening MRI of the spinal
cord is performed to detect any abnormalities such as tethered
cord or syrinx.
Preoperative laboratory should include CBC, sedimentation
rate, and C-reactive protein, electrolytes, creatinine, PT, PTT,
urinalysis, and capillary blood gases. Commonly these patients
have chronic lung disease and will have mild elevation in sedi-
mentation rate and C-reactive protein levels, so baseline values
are important later when clinical postoperative infection is sus-
pected. An echocardiogram is performed to detect early cor
pulmonale.
Our institution, Childrens Hospital of Philadelphia, rou-
tinely uses a tri-specialty evaluation system where the patient, in
addition to orthopedic evaluation, is seen by both a pediatric
general surgeon and a pediatric pulmonologist. General sur-
gery evaluation is important for appreciating associated GI,
renal, cardiac, and congenital lung malformations. Pediatric
pulmonary evaluation is also important in order to have an
understanding of the pulmonary function, including the risk of
progression of respiratory insufficiency and of intrinsic lung
disease that may be complicating the patients TIS. Through a
preoperative face-to-face meeting of the tri-specialty team, a
weighted TIS profile9 for each patient is created, based on each
specialist providing input into the aspects of history, physical
examination, and studies that support the diagnosis of TIS. If
all investigators believe the patient has progressive TIS, and
VEPTR opening wedge thoracostomy is the best treatment of
choice, then a surgical treatment recommendation is made. If
there are any dissenting votes, then an alternative recommen-
dation is made. Figure 110.5 illustrates the work-up of a VEPTR
patient.
SURGICAL TECHNIQUE4,5,6
THE EXPOSURE
Central venous line, arterial line, and Foley catheter are placed.
The patient is placed in a modified lateral decubitus position
with chest tilted slightly anterior. Sometimes a completely prone
position is used if the operative side can be draped low. An axil-
lary roll is used with foam padding underneath the pelvis and
the lower extremities. A soft bolster is also placed under the
apex of the deformity. The patient is stabilized by a 2 inch wide
cloth tape over a hand towel placed on top of the pelvis taped
to each side of the operating room table. Another hand towel
and cloth tape is brought across the lower extremities. The
upper arms are draped out of the field with a pulse oximeter
attached to the upper hand. The shoulders are brought out at
right angle to the axis of the body with the elbows extended 90
with padding between arms and under the elbows (Fig. 110.6A).
A prone position can also be used if draping is brought low on
the incision side. Spinal cord monitoring of both upper and
lower extremities by somatosensory evoked potentials (SSEPs) is
performed and, if practical, transcranial motor evoked poten-
tials (MEPs) are also monitored. Prophylactic antibiotics, IV
Ancef, 30 mg/kg, is given prior to the procedure.
The patient is draped so that the torso is exposed from the
top of the pelvis to the top of the shoulders. A gentle curvilin-
ear incision, forming a long flap, starting proximally at T-1,
halfway between the medial edge of the scapula and the poste-
rior spinous processes of the spine, is carried gradually anteri-
orly along the level of the 10th rib (Fig. 110.6B,C). Emans

Fused Ribs and Congenital Scoliosis

2 concave ribs fused only 3 fused concave ribs

SAL > 90%
Minimal Scoliosis SAL 90%

Either Progressive

Observe Scoliosis

Or, decrease in % nl FVC, if applicable

Age 6 months up to skeletal maturity
Progression of either SAL
or Cobb angle

VEPTR Opening Wedge Thoracostomy

Three or greater hemivertebrae

One or two isolated hemivertebrae
Hemivertebrectomy
Figure 110.5. The flow diagram of the
Hemiarthrodesis/Hemiepiphysiodesis
VEPTR work-up.

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 1184 Section IX Dysplastic and Congenital Deformities

A B

C D

Figure 110.6. (A) Typical draping for the VEPTR procedure. (B) Preoperative demonstration of the
operative incision for this 3-year-old female with congenital scoliosis and fused ribs. (C) Preoperative AP
radiograph of the patient. (D) Preoperative CT scan in another patient showing intrusion of the medial edge
of the scapula into the canal through an area of dysraphism. (continued)

LWBK836_Ch110_p1179-1193.indd 1184 8/26/11 5:44:36 PM

 Chapter 110 Thoracic Insufficiency Syndrome 1185

E F

G H

Figure 110.6. (Continued) (E) Completed exposure. Note the insertion of the posterior and middle sca-
lene muscles (large arrows), and the paraspinal muscles reflected medially with a thin layer of retained soft tis-
sue over the ribs to protect their vascularity (small arrows). The Freer elevator is probing an intercostal space
that will be the site of the superior rib cradle. (F) A channel to be cut by a Kerrison has been marked by cau-
tery (small arrows), put on tension by a bone spreader anteriorly (large arrows), with a no. 4 Penfield (block
arrow) inserted underneath the combined pleura/periosteum to protect the underlying lung. Because the tho-
racic constriction is so proximal in this patient, the superior rib cradle will be attached through the opening
wedge thoracostomy, rather than just above. (G) An AO bone spreader is now inserted into the channel medi-
ally, and the interval gradually widened to accomplish the opening wedge thoracostomy and lengthen the con-
stricted hemithorax. Note the underlying lung. The pleura was too flail to preserve. (H) The bone spreader is
replaced with the Synthes rib spreader, and the thoracostomy is gradually widened more. (continued)

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 1186 Section IX Dysplastic and Congenital Deformities

I J

K L

Figure 110.6. (Continued) (I) The Eiffel Tower VEPTR construct, rib to pelvis, with a DunnMcCarthy
hook over the iliac crest, threaded through a transverse incision in the apophysis, just lateral to the SI joint.
Useful when lumber posterior elements are lacking, and when there is pelvic obliquity. Much more mechani-
cal advantage is present compared to a similar pedicle screw at the same level, approximately 2.4 times.
(J) Complete correction of a lateral thoracic contracture with correction of pelvic obliquity by Harrington
outrigger distraction. Note the diaphragm visible through the one of the opening wedge thoracostomies. The
rest of the hybrid can now be inserted. (K) Final construct, with a medial hybrid VEPTR and rib-to-rib lat-
eral load sharing VEPTR. (L) Postoperative AP radiograph. Note not only correction of Cobb angle but
also improvement in space available for lung and clinical balance.

LWBK836_Ch110_p1179-1193.indd 1186 8/26/11 5:44:45 PM


(personal communication) emphasizes that having this rela-
tively straight long flap aids in closure of the incision after
opening wedge thoracostomy.
Utilizing cautery, the latissimus dorsi, trapezius, and rhom-
boid muscles are transected in line with the skin incision. If
there is midthoracic spine dysraphism and the medial edge of
the scapula lies within the spinal canal on preoperative CT
scan (Fig. 110.6D), the approach must be altered to avoid spi-
nal cord injury. In this situation, the scapula should be
retracted posteriorly with a rake to pull it out of the spinal
canal, and the rhomboid muscles sectioned directly off the
scapula, away from the area of dysraphism. Next, the scapula is
elevated by blunt dissection proximally and the common inser-
tion of the middle and posterior scalene muscle into the sec-
ond rib is identified. This is an important landmark since the
neurovascular bundle is immediately anterior and should be
protected. An anterior flap is then developed by cautery up to
the costochondral junction. To complete the exposure, the
paraspinous muscles are reflected by cautery from lateral to
medial until the tips of the transverse processes are palpable
(Fig. 110.6E). Dissection continues no further medially because
exposure of the spine will provoke inadvertent fusion. Care
should be taken to leave a thin layer of soft tissue overlying the
ribs to avoid damage to the periosteum because of the risk of
devascularization.
IMPLANTATION OF THE
SUPERIOR RIB CRADLE
The next step is implantation of the superior rib cradle of the
VEPTR at the uppermost portion of the thoracic constriction.
At least 1 cm of rib should be encircled by the superior rib
cradle. If the rib chosen is too slender, then two ribs are encir-
cled with an extended cradle cap added to the construct in
order to encircle it. A 1 cm incision is made by cautery in the
middle of the intercostal muscle, immediately beneath the rib
of attachment. Next, a Freer elevator is then inserted, pushing
through the intercostal muscle to the lower edge of the rib,
stripping the combined pleura/periosteum layer off from the
rib anteriorly. A second portal for the cradle is placed by cau-
tery above the rib of attachment. A second Freer is inserted in
this portal, pointing distally, to strip off the periosteum of the
rib anteriorly, and the two Freers should touch in the chop-
stick maneuver, to confirm that a continuous soft tissue tunnel
has been made underneath the rib. The VEPTR trial instru-
ment is then inserted into the incisions to enlarge them supe-
riorly and inferiorly.
The rib cradle cap next is inserted by forceps into the
superior portal, facing laterally, to avoid the great vessels and
the esophagus, then turned distally. Next, the superior rib
cradle is then inserted into the inferior portal, mated with the
cradle cap, and attached with a cradle cap lock. The superior
cradle is gently distracted by forceps superiorly to test for
instability. If unstable, the superior cradle can be moved
another level distally to a stronger rib for attachment. Avoid
placing the superior cradle above the second rib because this
endangers the brachial plexus. Do not place the superior cra-
dle above the rigid curve in a proximal segment of flexible
spine because the distraction power of the VEPTR will only
induce a proximal compensatory curve, and not correct the
primary rigid curve. Superior cradle insertion is similar when
the attachment rib has fibrous adhesions instead of intercos-
LWBK836_Ch110_p1179-1193.indd 1187
Chapter 110 Thoracic Insufficiency Syndrome 1187
tal muscles linking it to the ribs above and below. When the
superior cradle needs to be placed within a mass of fused ribs,
however, then the inferior portal for the superior cradle is
created by a bone burr, creating a slot of 5 mm by 1.5 mm,
and a 5 mm superior portal is cut by burr for placement of
the cradle cap.
THE OPENING WEDGE THORACOSTOMY
The object of the opening wedge thoracostomy to provide a
cleavage point for lengthening of the constricted hemithorax.
Much like wedging a cast, the thorax is sectioned transversely
on the concave side, and the osteotomy distracted apart to
straighten the thorax, lengthening the concave rib cage,
with indirect correction of the scoliosis. The chest wall con-
striction, often proximal to the apex of the scoliosis, includes
not only the mass of fused ribs but also any adjacent constricted
chest wall identified by persistent rib intercostal space narrow-
ing seen on the bending films. When the constriction is pri-
marily a fused rib mass of 3 to 4 ribs, a single opening wedge
thoracostomy will usually be adequate. Use an extraperiosteal
soft tissue sparing technique when performing the opening
wedge thoracostomies, avoiding the stripping of rib perios-
teum because of the subsequent risk of devascularization of
the rib.
The thoracostomy interval, following the groove between
the two central fused ribs, is marked by cautery posteriorly.
Anteriorly the fused ribs usually have separated apart, so the
thoracostomy is easily begun at the costochondral junction,
extending posteriorly through cautery lysis of muscle or fibrous
adhesions in line with the marked interval. The underlying
pleura is protected by elevating the muscle/or fibrous tissue
with an underlying clamp. When the junction of fused ribs is
reached, a no. 4 Penfield elevator is inserted under the rib
mass, pointing posteriorly along the groove, to strip away the
pleura/periosteum layer. With the lung protected by the
Penfield, an interval is cut along the groove in the fused ribs by
a Kerrison Rongeur for a distance of 2 cm (Fig. 110.6F). The
Penfield elevator is inserted further and the thoracostomy con-
tinued posteriorly until the tips of the transverse processes are
reached. An AO bone spreader is then inserted into the inter-
val at the posterior axillary line to widen the thoracostomy
(Fig. 110.6G).
Along the line of the thoracostomy, medial to the transverse
processes, there is usually fibrous tissue, and this is carefully
lysed by a Freer elevator with care not to violate the spinal
canal. If bone is present medial to the transverse process in
the line of the thoracostomy, this is carefully removed by sub-
periosteal stripping with a Freer elevator and then resected
under direct vision by Rongeur. Care must be taken not to sac-
rifice any anomalous segmental vessels that may penetrate the
fused rib mass. To avoid entering the spinal canal, the final
5 mm of fused rib bone is disarticulated from the vertebral
column by carefully pulling it free laterally with a curved
curette. Bone wax is placed over any bleeding surfaces. The
thoracostomy interval is further widened with the bone spread-
ers to ensure the superior hemithorax segment is completely
mobilized down to the spine. If there is greater than 4 fused
ribs constricting the hemithorax or the ribs below the fused
rib mass are closely adherent with fibrous tissue, then a second
or even third thoracostomy will needed to correct the defor-
mity. At least 2 rib thickness should be present in each section
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 1188 Section IX Dysplastic and Congenital Deformities
of the chest osteotomized apart by the opening wedge
thoracostomies.
Next, a Kidner is used to gently take down pleura proximally
and distally underneath the osteotomized ribs to mobilize it.
Once the thoracostomy interval is widened a distance of several
centimeters, the AO bone spreader is replaced with the Synthes
rib distractor, and the thoracostomy is slowly widened to its
maximum extent (Fig. 110.6H). With successful lengthening of
the constricted hemithorax, the oblique proximal ribs will
begin to assume a more horizontal position and the superior
rib cradle, tilted medially at initial insertion, will begin to line
up with the longitudinal axis of the body.
IMPLANTATION OF A HYBRID VEPTR
When patients are older than age 18 months, usually the lum-
bar spinal canal is adequate enough to accommodate a laminar
hook, so a hybrid VEPTR from proximal ribs to lumbar spine
can be used for maximum thoracic correction. The most
common choice of the lumbar level is either L2 or L3. It is
important to place the hybrid lumbar extension hook in the
lumbar spine at least two levels distal to any junctional kyphosis
seen on the lateral weight bearing X-ray to prevent accentuat-
ing the kyphosis.
With the Synthes rib spreader left in place to continue to
lengthen the constricted chest wall through the thoracostomy,
attention is turned to the lumbar spine where the inferior end
of the VEPTR lumbar hybrid extension is to be attached. A
paraspinous skin incision, 5 cm long, is made 1 cm lateral to
the midline at the level of the proximal lumbar spine. A skin
flap is elevated medially to expose the midline of the spine.
Cautery is used to longitudinally section the apophysis of the
two posterior spinous processes at the correct interspace, and
a Cobb elevator used to strip the spine laterally. Use a large
Cobb elevator in stripping the paraspinal muscles to minimize
the risk of violating the canal during exposure because occult
dysraphism is common in extensive congenital scoliosis. It is
important not to violate the cortex of the lamina of attach-
ment because this weakens its ability to withstand the distrac-
tion forces. The ligamentum flavum is then resected and the
laminar hook inserted. If the interspace is too small for the
hook, then a superior laminotomy of the interspace is per-
formed. Gelfoam is placed over the exposed dura. A bone
block of autograft, usually from rib resection, is then placed
from the superior lamina to the top of the hook, anchoring it
with a single level fusion. To hold the hook in place until the
bone block fuses, a no. 1 Prolene suture is wrapped around the
shank of the hook and underneath the posterior spinous pro-
cess at that level.
If there is scoliosis extending into the lumbar spine, or if
there is considerable pelvic obliquity, then the hybrid can
extend down to the iliac crest with attachment by a S-hook.
This is termed an Eiffel Tower construct and the S-hook
attachment to mid-iliac crest is termed iliac crest pedestal
fixation (Fig. 110.6I). This construct is also a powerful means
to address pelvic obliquity.8 An efficient way to slowly correct
pelvic obliquity is to first stretch the interval between the proxi-
mal ribs and the iliac crest by a Harrington outrigger (Fig.
110.6J) before placing the hybrid rib to crest VEPTR. With
the superior rib cradle in place, then the S-hook is then
implanted. Then the peg of the outrigger is placed within a
temporary rib sleeve attached to a rib cradle temporarily placed
LWBK836_Ch110_p1179-1193.indd 1188
under a rib of the chest wall lateral to the superior rib cradle
already in place. This strategy allows gradual gentle distraction
of the complete lateral thoracic contracture without excessive
force on the implanted VEPTR load points.
The outrigger is distracted by hand rotation of the knurled
nut until there is excessive reactive pressure. Distraction is tem-
porarily halted for 3 minutes to allow the viscoelastic tissues of
the chest to relax, then the cycle is repeated. As the tilted pelvis
gradually shifts underneath the trunk, reactive pressure increases,
and the rod wrench is then used on the outrigger to continue
distraction, but only a few millimeters at a time, allowing a full
3 minutes for the pressure to subside. When there is almost
instantaneous increase in large reactive pressure on turning the
outrigger nut, then maximum correction has been achieved.
With the outrigger in place, the VEPTR hybrid is engaged into
the implanted medial rib cradle, and then threaded into the
domino attached to the S-hook. The VEPTR is distracted and the
outrigger removed. This strategy can also be used with a rib to
spine VEPTR hybrid, but care must be taken not to plow the
spinal hook distally with excessive force, and the outrigger must
be removed before the implant can be placed.
Once the lateral thoracic contracture is corrected, and the
superior cradle and distal attachment of the hybrid rib-to-
spine VEPTR are implanted, the exposure is ready for com-
plete hybrid implantation. The size of hybrid lumbar exten-
sion rib sleeve needed is determined by measuring from the
bottom of the rib of attachment encircled by the superior rib
cradle down to the endplate of T12. This can usually be esti-
mated by palpating the 12th rib clinically. The distance in cen-
timeters should correspond to the number inscribed on the
rib sleeve and the hybrid lumbar extension. The hybrid device
is assembled and locked with a distraction lock. To estimate
the proper length, the device is then placed into the field
with the rib sleeve engaged into the implanted superior cra-
dle proximally and the spinal rod marked by a skin marker
approximately 1.5 cm below the bottom of the spinal hook.
The hybrid is removed from the field and the rod cut smoothly
by a rod cutter. Avoid using a bolt cutter because the resulting
sharp edges may cut through the overlying soft tissues. The
end of the rod is bent into slight lordosis and valgus by a
French bender so that the rod will line up with the axis of the
spine after implantation and conform to the lordosis of the
lumbar spine. A subfascial canal is created for safe passage of
the sized lumbar hybrid extension. A long Kelley clamp is
threaded from the proximal incision, through the paraspinal
muscles, into the distal incision, with care taken not to violate
the chest wall and the pericardium. A no. 20 chest tube is
then attached to the clamp, and the tube pulled upward into
the proximal incision. The end of the rod of the hybrid is
then placed into the chest tube, and the device carefully
guided through the muscle by the chest tube into the distal
incision. The tube is removed, and the rod threaded into the
hook, and then upward into the superior cradle. A distraction
lock engages the superior cradle to the rib sleeve. To perform
the initial tensioning of the device, a Synthes C-ring is
attached to the rod just above the hook, and a VEPTR distrac-
tor used to distract the hybrid proximally from the hook
through the C ring. The hook is then tightened onto the rod.
The Synthes rib distractor is then removed from the thoracos-
tomy. If there is adequate distraction from the hybrid device,
then the opening wedge thoracostomy should maintain its
open position.
8/26/11 5:44:48 PM
 Chapter 110 Thoracic Insufficiency Syndrome 1189

IMPLANTATION OF THE SECOND VEPTR: the rib sleeve are first removed, a longer rib sleeve is added
RIB-TO-RIB CONSTRUCT with a matching hybrid lumbar extension bridging to the prox-
imal lumbar spine where a separate exposure is made, and then
A second rib-to-rib construct VEPTR device should be added
the hybrid is tensioned. A second rib-to-rib VEPTR may be
laterally to assist the hybrid device in deformity correction and
added, if necessary.
decrease load on the medial rib cradle. The second superior
cradle usually is attached around the ribs encircled by the
hybrid device medially. The inferior cradle site chosen for CLOSURE
attachment should be a stable, sizeable rib, no lower than the To aid closure so that there is minimal tension on the suture
tenth rib, and the rib should be relatively transverse in orienta- line, the skin and muscle flaps are sequentially grasped with dry
tion. The span of chest wall bridged by the VEPTR should be as laparotomy sponges, and stretched vigorously toward each
long as possible so device expansion potential is maximum. other. Once stretched, the soft tissue flaps should overlap eas-
The portals for the inferior cradle site are prepared in the same ily. Two deep drains, nos. 7 and 10 Jackson Pratt, are added. A
fashion as the superior cradle site. The length of the rib-to-rib chest tube is needed only if there is significant pleural rent or
VEPTR should be based on the corrected length of the con- tear in the lung visceral pleura. Small rents do not require
stricted hemithorax, so, after the superior cradle is implanted, repair, but if an extensive pleura tear occurs, then this is
the thoracostomy interval is again opened by the Synthes dis- repaired with a patch of Surgisis, a bioabsorbable membrane,
tractor to maximum correction. Next, the distance from the attached by an absorbable interrupted suture20 (Fig. 110.7).
inferior edge of rib of rib cradle attachment superiorly to the Subcutaneous pain catheters for local anesthetic are helpful in
superior edge of the inferior rib of attachment is measured. controlling postoperative pain and are placed near implanted
The distance in centimeters will correspond to the number devices.
inscribed on the rib sleeve and inferior cradle of the rib-to-rib The musculocutaneous flap is first approximated along the
VEPTR needed. The inferior cradle is threaded into the rib apex with several interrupted figure of eight sutures of no. 0
sleeve, and the combined rib sleeve/inferior rib cradle is ready Vicryl through the deep muscles. Care should be taken to mon-
for implantation. itor both pulse oximeter and the SSEP and MEPS tracings of
Both the standard superior rib cradle and the inferior rib the upper extremities to detect signs of acute thoracic outlet
cradle of the rib-to-rib VEPTR are in neutral position, but if the syndrome. This can occur during closure because the fused
inferior osseous rib of attachment is oriented differently in the anomalous ribs are distracted superiorly into a brachial plexus
transverse plane from the superior rib due to rib obliquity, then that may be congenitally shortened.7 If there are any signs of
the VEPTR may not fit well around the inferior rib. A 30 brachial plexus depression, especially a decrease in the ulnar
angled right-handed or left-handed inferior VEPTR cradle may nerve tracings, or loss of pulse oximeter reading, the closure is
be used to better fit the inferior cradle around the rib in such a relaxed slightly, and usually monitoring signs improve. If
situation.
The Synthes rib distractor is removed. The inferior cradle
cap first inserted, facing laterally, into the distal portal of the
inferior rib cradle site, then turned superiorly to be in posi-
tion to mate with the inferior rib cradle. If the inferior rib of
attachment is somewhat oblique in orientation, then an
extended cradle will be needed to span the extra distance
needed to surround the rib. The inferior rib cradle is next
placed in the superior portal of the inferior cradle site and
locked to the end cradle cap with a cradle cap lock. The prox-
imal end of the rib sleeve is engaged into the superior cradle
at an angle, and slowly levered into place. A distraction lock is
placed proximally. The rib-to-rib VEPTR is distracted 0.5 cm
to tension the construct, and then locked with a distal distrac-
tion lock.
The lateral device is distracted, then the medial hybrid
device is distracted again to balance the distraction force for
final medial construct tensioning. The hook is tightened, and
the distraction C ring removed (Fig. 110.6 K,L).
The ideal age for VEPTR intervention from a pulmonary
viewpoint is age 6 months to 2 years, but VEPTR hybrid place-
ment may not be practical for young children, age 18 months
or younger because there may not be adequate room in the
lumbar spine spinal canal for a laminar hook. In these chil-
dren, a medial rib-to-rib construct can be used instead to stabi-
lize the thoracostomy. Correction with the rib-to-rib VEPTR
construct may not be as great as with a VEPTR hybrid, but it can
keep the thoracostomy open until the child reaches age 2 years, Figure 110.7. Surgisis membrane (thin), over a pleural rent at the
then, if desired, greater correction can be obtained by replac- base of the lung where herniation is most likely. Defects more proxi-
ing it with a hybrid. To accomplish this, the inferior cradle and mally less than 4 cm do not need a membrane patch.

LWBK836_Ch110_p1179-1193.indd 1189 8/26/11 5:44:48 PM

 1190 Section IX Dysplastic and Congenital Deformities
continued alterations in pulse oximeter and/or spinal cord
monitoring are encountered, even with relaxation of the clo-
sure, it may be necessary to resect the anterior-lateral portion
of the 1st and 2nd ribs, lateral to the devices, in order to provide
clearance for the brachial plexus.
After the apex is secured, the proximal and distal muscle
layers are closed with running suture of no. 0 Vicryl, with skin
closure by no. 4-0 Monocryl suture. Steri-strips are then placed
over the wound, and a bulky dressing is applied. Surgical poly-
urethane foam is then placed over the dressings to further pad
the incisions. AP and lateral radiographs are taken in the OR to
check position of devices, verify correction, check for pneu-
mothorax, and confirm proper position of the endotracheal
tube above the carina.
POSTOPERATIVE MANAGEMENT
Patients are usually left intubated 24 to 72 hours. VEPTR tho-
racic reconstruction acutely alters pulmonary function mechan-
ics to a much greater extent than a standard thoracotomy, so
immediate extubation is often not well tolerated. The hemat-
ocrit is checked daily for 3 days. Although blood loss usually
averages 50 ccs,4 continual oozing underneath the large flaps
result in a 50% risk for postoperative transfusion. Generally, a
hematocrit of 30% or greater is optimal for oxygen-carrying
capacity for these patients. Fluid management should be on the
restrictive side to prevent acute pulmonary edema.
Once weaned off the ventilator, the patient can be trans-
ferred to the surgical ward. Jackson Pratt drains are removed
when their individual drainage decreases to 20 cc, or less, over
a 24-hour period. Chest tubes are removed once their drainage
equals 1 cc per kilogram of patient weight over 24 hours.
Vigorous pulmonary toilet, including percussion, is needed
postoperatively. The patients are mobilized as soon as possible. No
bracing is used because of the potential chest constrictive effects.
LWBK836_Ch110_p1179-1193.indd 1190
In some rare instances the patient may go into respiratory
distress after drains and chest tubes are removed, so consider a
chest X-ray to look for reaccumulation of the pleural effusion
with compression of the lung. If present, temporary chest tube
drainage will be helpful by placement of an anterior pig-tail
chest tube.
VEPTR OUTPATIENT EXPANSION
The VEPTR devices are first expanded 6 months after the
implantation in outpatient surgery under general anesthe-
sia. No spinal cord monitoring is necessary, unless changes
were encountered in the initial implantation. Positioning is
similar to the implantation procedure. The distraction locks
of the devices are exposed through 3 cm incisions, either in
the thoracotomy incision, if it is adjacent to a distraction
lock, or through a new incision paralleling the device if the
distraction lock is distal from a previous incision. The dis-
traction locks are removed, the devices expanded by the
expansion forceps (Fig. 110.8) until a maximum reactive
force is encountered, and then new distraction locks are
placed. It is important to maintain thick muscle flaps over
the devices, by meticulous soft tissue technique, in order to
minimize the risk of skin slough. If the distraction lock is
exposed through the thoracotomy incision, a Freer elevator
is inserted proximally along the top of the device and used
to elevate the overlying muscle. Cautery is inserted into the
soft tissue tunnel created by the Freer elevator, and is used
to release the muscle deeply on each side of the device so
that a thick muscle flap is mobilized with the free edge at
the skin incision. The same approach is used distally. The
mobilized muscle flaps are closed without tension over the
locks when device expansion is complete. When the skin
incision parallels the device, the muscle incision is made by
cautery along the side of the device at the distraction lock site
of the rib sleeve, then the cautery is turned sideways to
release the muscle flap off the device. The full-thickness
Figure 110.8. Outpatient expan-
sion of VEPTR through a 3 cm inci-
sion. The procedure commonly takes
approximately 30 minutes. (Reprinted
with permission from The Journal of
Bone and Joint Surgery, Inc, Campbell
RM Jr, Smith MD, Mayes TC, et al. The
effect of opening wedge thoracostomy
on thoracic insufficiency syndrome
associated with fused ribs and congeni-
tal scoliosis. J Bone Joint Surg Am
2004;86-A(8):16591674.
8/26/11 5:44:49 PM

A B
Figure 110.9. (A) Limited exposure of an asymptomatic superior migration of a rib cradle. Probing of the
cradle confirmed that there was no osseous rib within the cradle. (B) Curved curettes are used to sculpt the
reformed ribs into a size and shape that would fit into the cradle for reattachment.
muscle flap is reflected by a Freer elevator, the expansion
procedure performed, and the full-thickness flap brought
back over the device for closure.
Postoperatively AP and lateral standing spine X-rays are per-
formed and the patients are usually able to be discharged
within 24 hours of surgery. All devices are expanded on sched-
ule every 6 months. Pulmonary function tests are performed
yearly once the patients are old enough to cooperate.
VEPTR REPLACEMENT PROCEDURE
Once the devices have been maximally expanded, then a
replacement procedure is performed; replacing the rib sleeve
and the inferior cradle/hybrid lumbar extension portion of the
devices while retaining the superior cradles/spinal hooks/
DunnMcCarthy pelvis hooks. Spinal cord monitoring is neces-
sary. Device replacement can usually be accomplished through
limited incisions over the caudal end over the hook, midpor-
tion of the hybrid devices, and a third small incision over the
superior cradle, while in the rib-to-rib VEPTRs the upper por-
tion and the distal portion of the device are accessed.
COMPLICATIONS
The most common complication is the slow asymptomatic
superior migration of the superior rib cradle through the rib of
attachment. Several months after implantation, the rib of
attachment undergoes hypertrophy, and, in most cases, the rib
cradle is filled with new bone around the rib. Some of the new
bone can also form below the cradle, so the device appears to
be migrating into the hypertrophied rib, but it actually remains
in its original position. Complete migration through the rib of
attachment, however, may gradually occur, an average of 3 years
after implantation, but this is also usually asymptomatic, often
seen fortuitously on scheduled predevice expansion X-rays.
This is treated by accessing the superior cradle at time of expan-
sion surgery through a limited incision of the proximal portion
LWBK836_Ch110_p1179-1193.indd 1191
Chapter 110 Thoracic Insufficiency Syndrome 1191
of the thoracotomy incision, and reimplanting it into the rib of
attachment that is usually reformed. Curved curettes are
extremely useful for shaving the hypertrophied rib down to
acceptable size for reimplantation of the rib cradle (Fig. 110.9).
The second most common complication is skin slough or infec-
tion. This is treated with debridement without removal of
devices and irrigation with dilute Betadine irrigation (Fig.
110.10). The skin edges are loosely approximated with Prolene
sutures, leaving a 5 mm gap in the wound, allowing it to close
by secondary intention. The patient is maintained on 4 to
6 weeks of IV antibiotics with culture results determining the
specific antibiotic. Sometimes it is helpful to add a wound Vac
overlying the device to promote healing.
Recurrent infections require removal of the rib sleeve and
the lumbar hybrid extension or the inferior rib cradle, and the
patient is maintained on 6 weeks of antibiotics. When sedimen-
tation rate/C-reactive protein have normalized, and the wound
is healed, then reinsertion of the device can be considered.
Skin slough is treated by debridement and mobilization of
flaps. In patients with long-standing VEPTR devices, dense soft
tissue scarring sometimes occurs over devices and recurrent
skin slough becomes a problem. For these patients, soft tissue
expanders are placed laterally to mobilize skin, the scar
resected, and then the new skin is transferred posteriorly over
the devices with the assistance of a plastic surgeon (Table 110.2;
Fig. 110.11).
THE FUTURE TREATMENT
OF THORACIC INSUFFICIENCY
SYNDROME
VEPTR opening wedge thoracostomy for congenital scoliosis
and fused ribs represents the first attempt at logical treatment
of TIS. It addresses the VDD component of TIS and allows rib
cage and spine growth, but it cannot restore motion to the
muscle deficient fused chest wall. Much remains to be learned
about the natural history of TIS and not only how VEPTR
8/26/11 5:44:49 PM
 1192 Section IX Dysplastic and Congenital Deformities

A B

Figure 110.10. (A) Infected VEPTR incisions (arrows). (B) Incision and drainage were performed. Little
purulence was encountered and the process was localized, so the device was not removed. A Rongeur (arrow)
is shown being used to remove necrotic tissue in the proximal wound. With dressing changes the wound
healed by secondary intention. Wound Vac treatment can also be used.

affects it, but how other traditional treatments, such as growing
rod, convex fusion procedures, or classic spine fusion, affect
it.21 Reports of small series13,14 suggest early spine fusion is asso-
ciated with adverse pulmonary outcome, and a recent report by
Karol et al.15 notes extensive spine fusion in early life appears
detrimental to pulmonary function by adolescence, especially
proximal fusion, and recommends alternatives to spine fusion
of the young child. They note that it is unclear how much the
very heterogeneous thoracic deformities of their patients con-
tribute to the loss of pulmonary function and how much is due
to the loss of growth of the thoracic spine from fusion surgery.
Severe restrictive lung disease was most common in those fused
patients with a thoracic spine 18 cm or less in length by skeletal
maturity, suggesting a threshold for shortening of the thorax,
probably due to a combination of congenital deformity/fusion
that significantly affects pulmonary function. Ramirez et al.19
reported a natural history model of TIS in older survivors of
spondylothoracic dysplasia whose spines were a literally a single
block vertebrae, averaging 24% normal in height, with a vital
capacity of only 29% normal. These patients may also represent
a human model of early spine fusion. Rib cage growth and vol-
ume may also be adversely affected by spine fusion. Canavese
et al11 have noted in a rabbit model that posterior spine fusion
causes alteration in rib cage growth with both reduction in sag-
ittal diameter and decrease in length of the sternum. The effect
of spine fusion on thoracic growth and function will probably
have to be settled by continued development of an animal
model of TIS.
The rationale in the past for early fusion of congenital curves
was that the unilateral unsegmented bars were incapable of

A B

Figure 110.11. (A) Patient with recurrent skin slough. A soft tissue expanders (arrows) had been placed
inferiorly and expanded over several months to obtain tissue for coverage. The dotted incision line outlines
the area of attenuated skin to be resected. (B) The dense scar was resected, and the inferior soft tissue
expander (arrow) removed, with development of a large proximal soft tissue flap created by the expander for
coverage. The flap was pulled distally to obtain closure without tension.

LWBK836_Ch110_p1179-1193.indd 1192 8/26/11 5:44:51 PM


San Antonio VEPTR
TABLE 110.2
Complications, 198920049
n 201 patients 1412 procedures
Mean procedures/pt 7.02/pt
Mean f/u 6 y
Infection rate/procedure 3.3%
Skin slough% patients 8.5%
Migration index (risk of complete migration/patient/year)
Migrations/pt/y 0.09
Mean time to migration 3.2 y
% patients with migration 27%
Patient device breakage% 6%
growth, but these appear to grow with the addition to the height
and volume of the thorax with VEPTR treatment.3 Emans et al
also confirmed an increase in CT lung scan volumes in VEPTR
treated patients.12 VEPTR opening wedge thoracostomy also
seems to safeguard pulmonary function in the short term.4,18
Other growth sparing treatments, such as the growing rod, have
limited experience with congenital scoliosis, and pulmonary
outcome data is not available in the current literature for these
techniques. VEPTR treatment may be considered for extensive
congenital scoliosis, but should not be used for single-level
hemivertebrae. Limited convex fusion or hemivertebrectomy is
preferable in those cases.
What is the end point of VEPTR treatment? Lung growth by
either alveolar cell multiplication or hypertrophy continues to
the age of skeletal maturity, so continued VEPTR treatment is
recommended until the time of skeletal maturity as assessed by
Risser sign. Definitive posterior spine fusion can be considered
at that time, with removal of hybrid VEPTR devices, but the
more lateral VEPTR devices can be left in the patient if they are
asymptomatic. These do not require any further expansion.
Yearly follow-up post-fusion is advised with radiographs and
pulmonary function testing. The VEPTR II design is now avail-
able, and a self-expanding device is under development.
As was stated earlier in this chapter, the ideal surgical treat-
ment of TIS should restore volume, symmetry, and biomechani-
cal function of the diseased thorax without growth inhibition. It
is doubtful that spine fusion can address any of these concerns,
and without outcomes assessment, such as pulmonary function
testing and CT scan lung volumes, it is unclear if spine surgery
without fusion (i.e., growing rods) can address these concerns.
VEPTR surgery does address the VDD of TIS, has a positive effect
on symmetry, and permits growth of the spine. The effect on the
underlying lungs is assumed positive, but difficult to demonstrate
inpatients. Mehta et al.17 have developed a rabbit model of TIS
by tethering the rib cage early in life and then simulating VEPTR
treatment by performing opening wedge thoracostomy later to
correct the induced VDD. The model shows severe alteration in
lung histology in the disease model by the compression of the
fused chest wall, and partial rescue by the opening wedge thora-
costomy. Further development of this model is planned.
Since the biomechanical performance of the thorax is cur-
rently poorly understood, it is difficult to understand the effect
of VEPTR treatment on this aspect of the thorax, and develop-
ment of advanced assessment tools, such as dynamic MRI of the
lungs, is needed to understand thoracic disease and the effects
of all forms of treatment on the thoracic engine of respiration.
LWBK836_Ch110_p1179-1193.indd 1193
Chapter 110 Thoracic Insufficiency Syndrome 1193
Campbell et al. reported clear patterns of abnormal rib cage
motion and diaphragm excursion in early onset scoliosis and
kyphoscoliosis in a preliminary study of patients with dynamic
lung MRI,10 and additional studies are underway to define the
ratio of hemithorax rib cage/diaphragm expansion of the lungs,
both in the natural history of TIS and the response to treat-
ment, whether by VEPTR, growing rod, or spine fusion.
In the future, there will probably be devices that address all
these concerns of TIS, but, for now, further development of
VDD correction by a growth sparing technique appears to be
the best practical goal.
ACKNOWLEDGMENT
This chapter honors the memory of Melvin D. Smith, pediatric
general surgeon, who made major contributions to the under-
standing of TIS and the development of VEPTR procedures.
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