Hanan Fathy

Pediatric Nephrology Unit

University of Alexandria
 A boy frightened of going to bed

 8 year old boy presented to his local hospital with a three

week history of worsening respiratory symptoms.

 He was previously fit and well, had not been febrile, and
his only medical history was a recent visit to his general
practitioner because he “found it hard to catch his breath
at night.”

 He was becoming increasingly scared of going to bed at

night and his mother was also concerned about some
bumps that she could feel on his scalp while stroking his
head in bed.
 Chest radiograph.

James C et al. BMJ 2009;339:bmj.b2787

©2009 by British Medical Journal Publishing Group

 The decision was made to drain in theatre under
general anaesthetic what was assumed to be a
right sided pleural effusion.

 Upon induction, the patient became apnoeic and

was difficult to ventilate.

 He then became asystolic and cardiopulmonary

resuscitation was commenced. Emergency
bronchoscopy was required to establish an airway
and cardiac output was restored after 20 minutes
of cardiopulmonary resuscitation.
 Blood tests were undertaken and his
initial blood results were as follows:
 Haemoglobin 117 g/l (115-155)
 White blood cell count 6.35×109/l (6.0-18.0)
 Platelet count 125×109/l (150-450)
 Lactate dehydrogenase 3406 U/l (432-700)
 Uric acid 1170 μmol/l (135-320)
 Urea 8.9 mmol/l (2.5-6.0)
 Creatinine 95 μmol/l (35-80)
 Calcium 2.12 mmol/l (2.19-2.66)
 Magnesium 0.83 mmol/l (0.7-0.95)
 Phosphate 4.34 mmol/l (1.1-1.75)
 Albumin 25 g/l (37-56)
 Alkaline phosphatase 50 U/l (200-495)
 C reactive protein 80 mg/l (<10)
 Computed tomogram of the chest at the level of the great vessels.

James C et al. BMJ 2009;339:bmj.b2787

©2009 by British Medical Journal Publishing Group

 REMEMBER
 Children presenting with large superior anterior mediastinal masses
represent a medical emergency.

 Such children are at extremely high risk of tracheal compression

leading to respiratory or circulatory compromise.

 To minimize the risk of cardiopulmonary arrest, initiating basic

airway management—such as sitting the patient upright and
avoiding causing unnecessary distress to the patient—is critically
important.

 If such patients do lose their airway, turning them to the prone

position might be life saving as the weight of the mass is lifted off
the airway, making intubation and ventilation possible.
 Be sure to know….

 Respiratory symptoms occur in 40% to 60% of

children with mediastinal mass.

 High incidence of cardio -respiratory arrest with

sedation / general anesthetics.
 Some facts about mediastinal masses
 Most mediastinal tumors may be asymptomatic
(incidental finding) or associated with vague
complaints.

 The likelihood of malignancy depends on mass

location, patient's age, and the presence of
symptoms
 Some facts about mediastinal masses
 Malignant masses are found in the anterior, middle, and posterior
mediastinum in approximately 60%, 30%, and 15% of cases,
respectively.

 Symptoms are present in 80% to 90% of patients with malignant

mediastinal tumors at presentation, compared to 46% of patients
with benign masses.

 Approximately 10% to 15% of patients with myasthenia gravis

have a thymoma.

 Neurogenic tumors are commonly observed in children.

 Lymphoma, chronic lymphocytic leukemia, thymomas, and

thyroid tumors tend to occur in adults.
The diagnosis for different mediastinal
masses is done step by step
The evaluation of the clinical aspects
The diagnostic imaging (standard chest radiography,
ultrasonography, echocardiography, CT scan, MRI,
and scintigraphy).
Laboratory data, and serum bio markers.
Endoscopic examinations (mediastinoscopy,
thoracoscopy, esophagoscopy, and
tracheobronchoscopy),
And lastly, biopsy.
 History and Physical
Examination
 Age of the patient.
 In children, neurogenic tumors of the posterior
mediastinum are common. Lymphoma is the second
most common mediastinal tumor, usually located in
the anterior or middle compartment, and thymoma is
rare .

 In both adults and children, approximately 25% of

mediastinal tumors are malignant rather than benign

12
 Airway compression

dyspnoea, stridor, haemoptysis, cough

Oesophageal compression

dysphagia, odynophagia, weight loss

Recurrent laryngeal
nerve compression
hoarse voice

Superior vena cava obstruction

facial swelling, headache
Plethora , collateral veins
Sympathetic ganglion involvement Horner's syndrome
miosis, ptosis, and anhidrosis
 myasthenic pain, palpable mass
Chest wall invasion ptosis, diplopia, dysarthria

Myasthenic symptoms
easy fatigability, drooping eyelid,
Constitutional symptoms double vision, dysarthria
of malignancy

weight loss, night sweats, fever.

fever, pallor, petechiae, hepatosplenomegaly,
abdominal mass.
Variation in the presentation in mediastinal masses
based on the anatomic site with age

Adults Children
Anterior mediastinum 54% 43%
Middle mediastinum 20% 18%
Posterior mediastinum 26% 40%
 Laboratory tests
 Suspected thymoma: CBC and acetylcholine
receptor antibody

 Suspected mediastinal goitre: Thyroid function

tests

 Suspected parathyroid adenoma: Serum calcium,

phosphate, and parathyroid hormone

 Suspected germ cell tumor: Alpha-fetoprotein and

beta-human chorionic gonadotrophin
 Laboratory tests

 Suspected phaeochromocytoma: 24-hour urinary

metanephrines and catecholamines, and plasma
free metanephrines

 Suspected neurogenic tumor: 24-hour urinary

homovanillic acid and vanillylmandelic acid

 Suspected haematological malignancy: CBC and

blood film, flow cytometry, HIV serology.
PNMT, phenylethanolamine N-methyltransferase; COMT,
catechol-O-methyltransferase;
SULT1A3, monoamine-preferring sulfotransferase.
 Radiological tests

 Chest x-ray
 CT scan with intravenous contrast enhancement
 MRI
 Trans-oesophageal echocardiogram
 Barium swallow
 Testicular ultrasound
 Chest X-ray
 A CXR often initiates the evaluation of
mediastinal disorders but is rarely diagnostic
eggshell calcifications, teeth or bones within a
mass, air fluid levels).
 Approach for diagnosis in x-ray
1. Is the mass actually in the mediastinum or is it in
the lung?

2. If in the mediastinum, then in which

compartment?

3. What is the differential diagnosis for the mass?

 Clues to locate mass to mediastinum
 Mediastinal masses are  Masses in the lung
invested by parietal parenchyma typically:
pleura, so will have:
 Are surrounded by air
 Smooth contour  May contain air
 Tapered borders bronchograms
 May be seen bilaterally  Will be on one side only
 The following characteristics indicate that a
lesion originates within the mediastinum:

 Unlike lung lesions, a mediastinal mass will

not contain air bronchograms.

 The margins with the lung will be obtuse.

 Mediastinal lines (azygoesophageal recess,

anterior and posterior junction lines) will be
disrupted.

 There can be associated spinal, costal or sternal

abnormalities.

A lung mass abutts the mediastinal surface and
creates acute angles with the lung, while a
mediastinal mass will sit under the surface
creating obtuse angles with the lung (Figure).
 Which compartment?
Use these signs to help
1. Cervicothoracic sign
2. Thoracoabdominal sign
3. Hilum overlay.
4. Effect on adjacent structures
 Trachea
 Ribs
1. Cervicothoracic sign
See sharp
margin
above clavicle

Click for lateral view

1. Cervicothoracic sign
This
should
help!
1. Cervicothoracic sign

Mass
“disappears”
at clavicle
Thoracoabdominal
sign

Can you
see the
outline of the
mass below
the diaphragm?
Hilum overlay

Hilum can
be seen
through
mass
Thyroid goiter

Trachea is
deviated
to left
 Summary
 Remember the approach:
1. Is the mass actually in the mediastinum or is it in
the lung?
2. If in the mediastinum, then in which
compartment? Use the signs
3. What is the differential diagnosis for the mass?
Use clinical and radiographic clues
 CT scan
 CT helps delineate anatomic location, extent of disease,
tissue invasion, and tissue density.

 CT is occasionally diagnostic and is usually sufficient for

pre-operative evaluation.

 It is useful in imaging associated mediastinal or hilar

lymph nodes, distinguishing mediastinal tumors from
vascular abnormalities, identifying concomitant
parenchymal lung disease, and demonstrating complex or
variant anatomy.
 Fluid containing masses

 Thymic Cyst
 Thymoma
 Teratoma
 Pericardial Cyst
 Foregut Duplication
 Meningocoele
 Neuroenteric Cyst
 Cystic Lymphadenopathy
 Lymphangioma
 Fat containing masses

 Thymolipoma
 Teratoma (Germ cell tumors)
 Esophageal lipoma
 Fat deposition
 Lipoma
 Lipoblastoma
 Liposarcoma
 Extramedullary hematopoiesis
 Magnetic resonance imaging (MRI)
 MRI is superior to CT for imaging nerve plexus
and blood vessels, distinguishing tissue planes and
invasion, and imaging in non-trans axial planes.

 MRI is particularly useful when iodinated contrast

is contraindicated, for imaging posterior
mediastinal masses and for assessing tissue,
vascular, or cardiac invasion.
 Thyroid scanning with radioactive iodine can identify and
evaluate masses of suspected thyroid origin.

 Alternative thyroid imaging modalities are thalium-201

imaging, technetium-99m-sestamibi imaging, Positron
emission tomography with 18 fluorodeoxyglucose (FDG-
PET), or scintigraphy with radiolabeled octreotide, a
synthetic analog of somatostatin.

 Technetium-99m-sestamibi imaging is 90–100% sensitive

for identifying parathyroid adenomas in the neck and
mediastinum and may identify parathyroid carcinomas.

 Methionine PET may more accurately localize

parathyroid adenomas.
 Biopsy techniques
 Image-guided percutaneous needle biopsy
 Endoscopes biopsy with or without
ultrasonography
 Mediastinoscopy
 Mediastinotomy/Chamberlain procedure
 Thoracoscopy
 Open biopsy sternotomy or thoracotomy
 Bone marrow biopsy
 Peripheral lymph node biopsy
 Superior vena cava syndrome
 Horner's syndrome
 Tracheal obstruction
 Bronchial obstruction
 Thoracic aortic aneurysm rupture
 Aortic dissection
Suspected condition Test of first choice

Thoracic aortic aneurysm Transoesophageal echo

Non Hodgkin`s lymphoma peripheral lymph node excision biopsy

Metastatic cancer biopsy
Thymoma CT scan of chest
Aortic dissection Transoesophageal echo,
CT scan of chest with contrast
enhancement
Hodgkin`s lymphoma peripheral lymph node excision biopsy

Mediastinal germ cell CT scan of chest, abdomen, and pelvis

tumor seminoma
Suspected condition
Mediastinal germ cell tumor
non seminoma
Thymic carcinoma
Primary tracheal tumors
Neurogenic tumor
Thyroid neoplasia
Mediastinal germ cell tumor
mature teratoma
Small cell carcinoma lung
cancer
Test of first choice
CT scan of chest, abdomen, and pelvis
CT scan of chest, abdomen, and pelvis
CT scan of chest with contrast
enhancement
CT scan of chest with contrast
enhancement
CT scan of neck and chest without
contrast enhancement
CT scan of chest, abdomen, and pelvis
CXR
 Robotic Resection of Pediatric Chest Masses
John Meehan, John Lawrence, Laura Phearman, Paula Francis, and Anthony Sandler
The Children’s Hospital of Iowa
University of Iowa Hospitals and Clinics, Iowa City, IA

Purpose Figure 2-1 Figure 2-2 Figure 2-3 Case 5

Robotic surgery is a new technology which may broaden the variety of minimally A 17 year old girl presented with cough. Work-up revealed the anterior
invasive procedures available for pediatric patients. In addition to providing fine- mediastinal calcified mass with fat shown in figure 5-1. A fine needle biopsy at
motion filters that eliminate physiologic tremor, robotic instruments can articulate to Robot Location an outside facility demonstrated a teratoma. Cart position utilizing 4 ports is
aid in difficult dissections. Thoracic masses are particularly suited for robotic surgery shown in figure 5-2. The mass was densely adherent to the lung. An

E
because the articulating instruments may be capable of circumnavigating around a intraoperative photo shows a calified portion of the mass (figure 5-3). Pathology
rigid mass which may be difficult with standard rigid laparoscopic and thoracoscopic demonstrated a mature teratoma. Follow-up scans have shown no evidence of
equipment. We present our initial results using robotic surgery to resect benign and recurrence 6 months after surgery.
malignant thoracic masses. Figure 5-1 Figure 5-3
Figure 5-2

Methods

E
We performed surgical resections of chest masses in 5 patients using the Da Vinci 3-
arm Surgical Robot (Intuitive Surgical, Sunnyvale, CA). Our version of the Da Vinci Case 3 Robot Location
has one 3-D 12 mm camera arm and two 5 mm instrument arms. In one case, we An 11 year old boy presented with fatigue. After an initial chest x-ray showed a large Instrument
Arms

used a 5 mm 2-D scope. All patients had pre-operative scans showing a mediastinal mass, an MRI demonstrated a right anterior mediastinal (figure 3-1) tumor. His beta- Accessory
mass. Two children had a posterior solid mediastinal mass, 2 had an anterior solid HCG and AFP were normal. He underwent resection using 4 ports as shown in figure Port

mediastinal mass, and one had a posterior mass extending around the right lung 3-2. An intraoperative photograph is shown in figure 3-3 during the dissection of the Camera

hilum with central necrosis. phrenic nerve. In addition to the phrenic nerve, the tumor was adherent to the SVC,
right lung, and the heart. The tumor was resected enbloc. Pathology demonstrated a Patien Age Wt. Diagnosis Trocar Trocar Docking Robot Total Tumor Length Follow-
Case 1 germ cell tumor. He underwent chemotherapy and currently no evidence of recurrence
t (kg) s Placemen
t
Time Time Time Size (cm) of Stay up

A 2 year old girl had a mass found on chest x-ray for mild pulmonary symptoms. A CT 15 months after resection. 1 2 years 13. Ganglioneuroblasto 3 10 min 3 min 56 min 69 min 6.5 x 4.2 1 day 18 mo.
scan showed a superior and posterior right mediastinal mass with small calcifications 9 ma x 1.7
2 16 61. Inflammatory Mass 4 10 min 3 min 143 min 156 min 6.0 x 4.0 3 days 15 mo.
(Figure 1-1). The mass was adherent to the azygous vein and superior vena cava
(SVC). She underwent complete resection using 3 ports; a 12 mm camera port and
two 5 mm robot instrument ports (figure 1-2). An intraoperative photo is shown in
Figure 3-1 E
Figure 3-2
Figure 3-3
3
years
11
years
7
40.
0
Germ Cell Tumor 4 14 min 4 min 131 min 146 min
x 2.0
12.0 x
14.0 x 5.2
1 day* 15 mo.

figure 1-3. She went home on post-operative day #1. Pathology demonstrated a 4 4 years 21. Ganglioneuroma 3 18 min 4 min 22 min 44 min 4.0 x 2.0 1 day 13 mo.
5 x 2.5
ganglioneuroblastoma. She did not require any further therapy and she has been 5 17 70. Teratoma 4 8 min 3 min 140 min 151 min 5.6 x 4.7 1 day 6 mo.
disease free for 18 months. years 5 x 2.9

Figure 1-2 Figure 1-3 Avg. 9.8 41. - - 12.5 min 3.4 min 98.4 min 113.2 - 1.4 13.4 mo
Figure 1-1 Robot Location years 5 min days
Robot Location Camera
Phrenic Nerve
* - The patient with the germ cell tumor was ready for discharge on post-op day #1 but required chemotherapy.
Therefore, he was transferred to the oncology service for 2 additional days of inpatient chemotherapy.

Instrument Discussion
E

Arms Case 4 Complete resection of the primary mass was achieved in all patients. No open
A 4 year old boy presented with mild flu-like symptoms. Work up revealed a chest conversions were required. Subjectively, the articulating instruments allowed
Camera
mass unrelated to his complaints (figure 4-1). Complete resection was accomplished dissection of the mass seem superior to standard non articulating minimally
using 3 ports; a 5 mm 2-D robot camera port and two 5 mm instrument ports. invasive instruments. Treacherous areas such as the SVC, azygous vein,
Pathology revealed a ganglioneuroma. pulmonary hilum, phrenic nerve, and heart were dissected with relative ease
Robot Location Figure 4-3 and no complications occurred. Times for trocar placement are comparable to
Figure 4-1
Case 2 our thoracoscopic experience and docking times are relatively insignificant.
A 16 year old athlete had and fevers, fatigue, and cough. She had been on antibiotics
E

with no improvement and eventually developed wheezing and hemoptysis. A CT scan

(figure 2-1) revealed a mediastinal mass with multiple loculated fluid collections and
compromise of the right mainstem bronchus (arrow). Although lymphoma was initially Conclusions
Instrument
suspected, an intraoperative biopsy showed inflammation with no evidence of tumor. Arms
Robotic surgery is a safe and effective method for resecting thoracic masses in
Due to the bronchus compression, we elected to perform a resection. Approximately 10 pediatric patients. Dissection can be facilitated by the articulated robotic
cc’s of purulent material was drained from the mass during the resection. Aerobic, Camera instruments. Considering the rigidity of the chest wall, articulating instruments
anaerobic, and fungal cultures were negative. Special stains for AFB were also may prove to be superior in dissecting in the thorax. Further study of this
negative. Pathological evaluation revealed inflammation and necrosis but a specific technology is warranted as it may increase the variety of procedures which can
etiology was never identified. She went home post-op day #3 and has remained be safely performed using a minimally invasive approach.
asymptomatic. Subsequent CT scans have remained clear.