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Thymectomy in Children
DOI: http://dx.doi.org/10.5772/intechopen.114888
131
.. Thoracoscopic approach (VATS thymectomy)
It is an approach that has been used since the 1990s, first in adults and then
in children (Figures and ). Its effectiveness is comparable to the transsternal
approach and is more advantageous in terms of morbidity, which can be summarized
as short hospital stay, less blood loss, good cosmetic appearance, and decreased need
for analgesics. With appropriate patient selection, the risk of major vessel injuries and
conversion to open surgery is low [15, 36].
As an alternative to conventional left-sided thoracoscopic approach, thymectomy
with subxiphoid access, whether single port or not, or a right-sided approach in
cases where the thymic lesion was almost totally located on the right side has been
reported [37].
Single lung ventilation with or without using a double lumen endotracheal tube
may assist in good exposure to the thymus. Although VATS thymectomy can be
applied from either side, the right-side approach is more popular in adults and vice
Figure 4.
Thoracoscopic approach to a case of MG unresponsive to maximal medical treatment. Opening of anterior
mediastinal window (A), and exploration and initial dissection of left lobe of thymus (B) (from the author’s own
archive).
Figure 5.
Post-thoracoscopic appearance of port sites (A) and thymectomy material with a mass in the left lobe after
thoracoscopic excision (B) (from the author’s own archive).
Pediatric Surgical Procedures – An Updated Guide – Volume I
132
versa in children. Venous connections with the inferior vena cava and brachiocephalic
trunks can be better defined in right-side approach. The left side approach is more
suitable to reach the fat tissue in the aorta-pulmonary window. If there is a significant
hypertrophy or a large mass in one lobe, the ipsilateral approach should be preferred.
However, if the opposite side cannot be safely explored and dissected from the
ipsilateral side, a simultaneous bilateral approach can be applied. It is also possible
to perform extended thymectomy from both sides with VATS and, if necessary, by
adding a cervical approach.
We performed five thoracoscopic thymectomies in the last 15years in which three
of them suffered from intractable non-thymomatous MG, whereas two patients had
thymoma associated with MG. The patients’ ages ranged between 11 and 16. There
was no intra- or post-operative complication except contralateral pneumothorax in
one patient. Thymectomy completely (n=2) or remarkably (n=2) resolved neurological symptoms in four but the last patient with thymoma associated with MG
showed no improvement.
• As for the technical details of the procedure, the patient is positioned at
30–45° from horizontal plane with the ipsilateral right arm raised above the
head [8, 34].
• In simple VATS thymectomy, three ports are generally used, one of which is for
the camera.
• CO2 insufflation is set to a pressure of 6–8mmHg [8, 39].
• After opening an anterior mediastinal window via incision of parietal pleura,
starting the procedure from lateral border of the thymus provides a better and
safer dissection.
• The dissection is carried from inferior to superior, elevating the gland and
ultimately exposing the vasculature. Maximal care must be taken to protect the
phrenic nerve during the lateral dissection.
• The thymic branches of the internal thoracic artery originate cephalic and lateral
to the gland, while the thymic vein branches drain posteriorly to the innominate
vein. Blood vessels should be isolated and divided between clips or cauterized
with an energy device.
• Next, the thymus is bluntly dissected from its contralateral pleural attachments
taking care to avoid injury to the contralateral phrenic nerve.
• Once the entire gland has been dissected free, the thymus is inserted into a specimen bag to remove it free of seeding.
• In cases where the procedure proceeds smoothly, the routine use of a drain or
chest tube may not be necessary [8].
• In order to better control postoperative analgesia, an intercostal nerve block is
applied after the surgery is completed.
Thymectomy in Children
DOI: http://dx.doi.org/10.5772/intechopen.114888
133
• Extubation is generally uneventful and the patient is then taken to the recovery
room for observation. Patients are usually discharged home 48–72h after the
surgery.
Indications: Thymic hyperplasia, thymic cysts, non-thymomatous MG, and non-
invasive thymoma.
Contraindications: Invasion of large vessels, in particular, contraindicates for
minimally invasive thymectomy and requires an open procedure. Additionally, if the
patient is thought to be unable to tolerate one-lung ventilation, resection via sternotomy or transcervical approach is indicated [3].
Advantages: Nice cosmetic appearance (less scar), rapid healing, faster improvement of lung function, reduction of surgical trauma, short hospitalization, decreased
postoperative pain, and no complications originated from breastbone.
Disadvantages: Limited surgical manipulation, transient intercostal neuralgia,
and limited exposure of contralateral side during unilateral thoracoscopy.
.. Robotic thymectomy
The first robotic thymectomy was reported by Yoshino et al. for a small thymoma
in 2001and followed by Rea et al. and Ashton et al., both described the first case
series of robotic thymectomy for MG in 2003 (Figure ) [40–42]. To date, many case
series and several meta-analyses were published, mostly addressing the treatment of
adult thymic disorders. In their meta-analysis, Xu et al. suggested that robotic videoassisted thoracoscopic surgery can be performed on both sides; however, left-side
approach had significantly lower complications, regarding atrial fibrillation, open
conversion, and air leaks [43]. Another meta-analysis found robotic thymectomy
to be advantageous over conventional thoracoscopic VATS in terms of short-term
outcomes such as shorter duration of drainage, less total drainage, and a lower rate of
conversion [44]. The recurrence rate was comparable between the two techniques. In
recent years, case series of children who underwent thymectomy with robotic VATS
have also been published [3, 19].
Figure 6.
General perspective of console, operating table, and robotic arms in robotic thymectomy. Sites of ports and robotic
arms in left-sided robotic thymectomy (A). Sites of ports and robotic arms in subxiphoid approach for robotic
thymectomy (B).
Pediatric Surgical Procedures – An Updated Guide – Volume I
134
• Patient position and port entry sites are similar to thoracoscopic procedure.
Alternatively, robot-assisted thymectomy with subxiphoid access or a right-sided
approach in cases where the thymic lesion was almost totally located on the right
side can be used.
• In left-sided robotic thymectomy technique, which is a most common approach,
a camera port for the 3-D camera (12mm) is introduced and followed by
other two or three working ports are positioned on the midaxillary or anterior
axillary lines under its guidance. Forth trocar may be also used as an optional
table-site assistant port. The arms of the robotic system are then connected to
the ports [45].
• Whether left or right-side approach is used, following exploration of the
anterior mediastinum and chest cavity, the dissection begins at a safe window
between sternum and phrenic nerve or the level of cardiophrenic angle and
moves upwards. The procedure then follows the basic principles of thoracoscopic
thymectomy.
• Briefly, thymus and/or, if necessary, the entire mediastinal tissue is fully isolated
from the anterior border of the phrenic nerve, pericardium, mammarian, and
innominate vessels with great care.
• The connecting vessels or thymic veins/arteries are identified, coagulated/
clipped, and divided.
• After removal of the specimen and hemostasis, a chest tube is usually placed (the
anterior port site is preferred), the lung is fully inflated, and the other incisions
are closed.
• Intercostal nerve block is applied to better control postoperative analgesia after
the surgery is completed.
Indications: Similar to thoracoscopic thymectomy.
Contraindications: Similar to thoracoscopic thymectomy.
Advantages: Compared with traditional open approach surgery, robotic thymec-
tomy has better cosmetic effect, faster improvement of lung function, reduction of
surgical trauma and length of stay, and no complications originated from breastbone.
Disadvantages: Long set-up procedure, high cost (expensive equipment), and
limited number of robotic centers.
. Postoperative care and follow-up
Early postoperative care should be continued in collaboration with anesthesiologist,
intensivist, and pediatricians. Extubation is generally uneventful, and the patient is
then taken to the recovery room for observation. To evaluate lung expansion, chest
tube position, and diaphragm, a postoperative chest x-ray should be taken in the
recovery unit. Patients who undergo thoracoscopic/robotic thymectomy are usually
discharged home 48–72h after the surgery. Hospitalization is longer in the transsternal approach (5 to 7days).
Thymectomy in Children
DOI: http://dx.doi.org/10.5772/intechopen.114888
135
For transsternal thymectomy, sternal precautions such as avoiding heavy lifting
and excessive shoulder movement should be maintained for at least 6 months [35].
Pain can be controlled with analgesic infusion or patient-controlled anesthesia and
switched to oral medications once the patient tolerates the diet. After less invasive approaches, the need for analgesics is significantly reduced. Chest tubes are
removed when the drainage is low, contents are not hemorrhagic, and air leakage
has stopped, usually on 1st or 2nd postoperative day following minimally invasive
surgery [35].
In patients with MG, the patient’s respiratory condition and ventilatory support
require great attention in the early postoperative period. Respiratory complications
can be minimized with careful preoperative preparation. As long as they are hospitalized, all MG and thymoma patients should be followed dynamically by a multidisciplinary team, including pediatric neurologists/oncologists as well as pediatric
surgeons.
After discharge from the hospital, close follow-up should be continued in a multidisciplinary manner within the framework of a standardized protocol (
Table ).
. Immunological changes and prognosis after thymectomy
After 1 year of age, T cell production in the thymus begins to decrease rapidly
and reaches a minimal level, similar to that in healthy adults. After neonatal thymectomy, CD4+ and CD8+ T lymphocyte rates and numbers decrease significantly
compared to those who underwent thymectomy at later ages [12]. This situation, in
which there is a change in T cell composition at a very early period similar to that in
adults, is defined as “premature immunosenescence”. However, there is not enough
data to show that this has a negative impact on the health of the infant. Broek et al.
suggest that 5–10years after neonatal thymectomy, redevelopment of thymic
components with adaptive mechanisms in the peripheral lymphoid tissue and an
increase in T cell numbers indicate that the thymic tissue has regenerative capacity
[5]. In a systematic review, it has been reported that early thymectomy, either partial
or complete, may be associated with a reduction in many T cell subpopulations
and TCR diversity, and these alterations may persist during long-term follow-up
[13]. Alternative solutions should be studied, either in the operative technique with
partial preservation of the thymus or through the autograft of fragments of the
gland [12].
Despite these opposing views which are suggestive for laboratory based immunological impacts, according to our current knowledge, thymectomy has not been
found to have a significant effect on susceptibility to infection, response to childhood
vaccines, and the presence of organ-specific autoantibodies [19].
Mortality after thymectomy in children is negligible, and morbidity is minimal. It
is difficult to determine the long-term effects of thymectomy in adults and even more
difficult in children. This is due to heterogeneity in patient groups, differences in
indications, and non-standardization of surgical treatment methods. Although there
are pediatric cases in large series, there are no large case series consisting entirely of
children. The long-term effects of thymectomy in patients with MG are also variable.
Most cases begin to improve within 1year, and some of them go into permanent
remission but the remaining part of thymectomized patients still needs maximal
medical care [21]. Christison-Lagay et al. reported that, as a result of long-term
follow-up (average time: 38.5months), the rate of improvement in symptoms was
Pediatric Surgical Procedures – An Updated Guide – Volume I
136
Routine follow-up protocol*
Benign Conditions Malign Conditions
Follo w-Up
First month Chest x ray
Sixth month Chest x ray
First year Chest CT
Secondyear Chest x ray
Third year No routine follow-up
Fourth year No routine follow-up
Fifth year No routine follow-up
After fifth
year
*Our institutional follow-up protocol (Akdeniz University School of Medicine, Department of Pediatric Surgery).
Thymic hyperplasia
(not associated with
MG), thymic cyst,
thymolipoma, ectopic
thymic tissue, etc.
Outpatient exam. in
pediatric surgery
Outpatient exam. in
pediatric surgery
Outpatient exam. in
pediatric surgery
Outpatient exam. in
pediatric surgery
Check-up when there is
a clinical complaint
Check-up when there is
a clinical complaint
Check-up when there is
a clinical complaint
No routine follow-up
Check-up when there is
a clinical complaint
Thymic hyperplasia
associated with MG,
non-thymomatous
MG
Chest CT
Outpatient exam. in
pediatric neurology
Outpatient exam. in
pediatric surgery
Outpatient exam. in
pediatric neurology
Outpatient exam. in
pediatric surgery
Chest CT
Outpatient exam. in
pediatric neurology
Outpatient exam. in
pediatric surgery
Outpatient exam. in
pediatric neurology
Outpatient exam. in
pediatric surgery
Outpatient exam. in
pediatric neurology
Outpatient exam. in
pediatric surgery
Outpatient exam. in
pediatric neurology
Outpatient exam. in
pediatric surgery
Outpatient exam. in
pediatric neurology
Outpatient exam. in
pediatric surgery
Outpatient exam. in
pediatric neurology
Outpatient exam. in
pediatric surgery
Thymoma and other epithelial
tumors
Chest CT
Outpatient exam. in pediatric surgery
Outpatient exam. in pediatric neurology
Outpatient exam. in pediatric oncology
Chest CT
Outpatient exam. in pediatric surgery
Outpatient exam. in pediatric neurology
Outpatient exam. in pediatric oncology
Chest CT
Outpatient exam. in pediatric surgery
Outpatient exam. in pediatric neurology
pediatric oncology
Chest CT
Outpatient exam. in pediatric surgery
Outpatient exam. in pediatric neurology
Outpatient exam. in pediatric oncology
Chest CT
Outpatient exam. in pediatric neurology
Outpatient exam. in pediatric oncology
Chest CT
Outpatient exam. in pediatric neurology
Outpatient exam. in pediatric oncology
Chest CT
Outpatient exam. in pediatric neurology
Outpatient exam. in pediatric oncology
Lifelong monitoring
Outpatient exam. in pediatric neurology
Outpatient exam. in pediatric oncology
Table 3.
Follow-up protocol of pediatric patients who underwent thymectomy.
75%, and the rate of asymptomatic patients was 47% in their juvenile MG series of
15 cases [46]. Tracy et al. found that thymectomy is an effective treatment in 62%
of children with MG, and remission is complete in 31% [47]. In a large series with
an average follow-up period of nearly 10years, 34 thymectomized patients were
examined, and it was suggested that thymectomy made a significant contribution to
relieving generalized symptoms [22].
Thymectomy in Children
DOI: http://dx.doi.org/10.5772/intechopen.114888
. Conclusion
137
Aside from the fact that surgical diseases of the thymus such as intractable thymic
hyperplasia, generalized myasthenia gravis, symptomatic ectopic thymic cysts/tissues, and thymoma are rare in childhood, the indication, timing, and technique of
thymectomy are also controversial in a remarkable part of them. In this respect, it is
important to evaluate such cases with a multidisciplinary approach, and the surgeon
must be experienced in both open and thoracoscopic thymectomy techniques.
Thoracoscopic thymectomy is recommended in patients with thymic hyperplasia
and MG resistant to conservative approach or in early stage thymoma. Excision is the
first option in symptomatic ectopic thymic cysts/tissues, and transsternal extended/
maximal thymectomy is the firs
Incidental thymectomy is a routine procedure in most of open-heart surgery for
congenital heart diseases.
Postoperative morbidity is significantly lower in thoracoscopic thymectomy
then in open thymectomy techniques. Mortality due to thymectomy is very low and
comparable between thoracoscopic and open techniques.
t option in cases of the advanced stage thymoma.
Pediatric Surgical Procedures – An Updated Guide – Volume I
138
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