Life-threatening presentation of mediastinal neoplasms: report on 7 consecutive pediatric patients

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Brief Reports Life-threatening presentation of mediastinal neoplasms: report on 7 consecutive pediatric patients Marco Piastra MD a, * , Antonio Ruggiero MD b , Elena Caresta MD a , Antonio Chiaretti MD a , Silvia Pulitano MD a , Giancarlo Polidori MD a , Riccardo Riccardi MD b a Pediatric Intensive Care Unit, Catholic University Medical School, 00168 Rome, Italy b Pediatric Oncology, Catholic University Medical School, 00168 Rome, Italy Received 20 December 2003; accepted 21 December 2003 Abstract Background: Cases of respiratory failure at presentation in children with anterior mediastinal malignancies can be very challenging for clinicians. Seven consecutive children presenting with superior mediastinal syndrome are reported needing urgent critical care approach. Patients and Methods: Seven children (age range from 0.8 to 14 years; mean, 4.5 years) suffered from critical mediastinal neoplasms. Clinical presentation, laboratory findings, treatment, and outcome are discussed. Setting: a tertiary-care 6-bed medical and surgical pediatric intensive care in a university hospital. Interventions included emergency management, resuscitation and intensive care admission, and diagnostic and therapeutic procedures. Results: All cases showed a respiratory compromise and underwent ventilatory and/or cardiovascular support. Two patients needed renal replacement therapy. Pediatric Intensive Care Unit discharge was achieved in all patients. Conclusions: Critical or extreme presentation of mediastinal neoplasms does not preclude a good clinical outcome: an intensive care approach is essential to allow patient recovery and effective antineoplastic therapy administration. D 2005 Elsevier Inc. All rights reserved. 1. Introduction Superior mediastinal syndrome and superior vena cava syndrome (SVCS) are almost synonymous terms to indicate the compression of vital structures of superior mediastinum: namely, SVCS means a predominant venous compression. This is a rare but serious condition, sometimes manifesting bde novo,Q requiring prompt treatment. In children, malig- nant lymphomas are the second most common cause [1] of SVCS after surgery for congenital heart diseases. The clinical picture at onset may be critical — as in presented cases—and upper airway obstruction, superior vena cava (SVC), cardiac or pulmonary artery compression, and acute pulmonary edema can represent the initial clinical signs in previously apparently healthy children. Up to now, cases of mediastinal neoplasm emergencies have been reported only 0735-6757/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.ajem.2003.12.025 * Corresponding author. Tel.: +39 06 30155203; fax: +39 06 35402286. E-mail address: [email protected] (M. Piastra). American Journal of Emergency Medicine (2005) 23, 76–82 www.elsevier.com/locate/ajem

Transcript of Life-threatening presentation of mediastinal neoplasms: report on 7 consecutive pediatric patients

www.elsevier.com/locate/ajem

Brief Reports

Life-threatening presentation of mediastinal neoplasms:report on 7 consecutive pediatric patients

Marco Piastra MDa,*, Antonio Ruggiero MDb, Elena Caresta MDa, Antonio Chiaretti MDa,Silvia Pulitano MDa, Giancarlo Polidori MDa, Riccardo Riccardi MDb

aPediatric Intensive Care Unit, Catholic University Medical School, 00168 Rome, ItalybPediatric Oncology, Catholic University Medical School, 00168 Rome, Italy

Received 20 December 2003; accepted 21 December 2003

AbstractBackground: Cases of respiratory failure at presentation in children with anterior mediastinal

malignancies can be very challenging for clinicians. Seven consecutive children presenting with

superior mediastinal syndrome are reported needing urgent critical care approach.

Patients and Methods: Seven children (age range from 0.8 to 14 years; mean, 4.5 years) suffered from

critical mediastinal neoplasms. Clinical presentation, laboratory findings, treatment, and outcome are

discussed. Setting: a tertiary-care 6-bed medical and surgical pediatric intensive care in a university

hospital. Interventions included emergency management, resuscitation and intensive care admission,

and diagnostic and therapeutic procedures.

Results: All cases showed a respiratory compromise and underwent ventilatory and/or cardiovascular

support. Two patients needed renal replacement therapy. Pediatric Intensive Care Unit discharge was

achieved in all patients.

Conclusions: Critical or extreme presentation of mediastinal neoplasms does not preclude a good

clinical outcome: an intensive care approach is essential to allow patient recovery and effective

antineoplastic therapy administration.

D 2005 Elsevier Inc. All rights reserved.

1. Introduction

Superior mediastinal syndrome and superior vena cava

syndrome (SVCS) are almost synonymous terms to indicate

the compression of vital structures of superior mediastinum:

namely, SVCS means a predominant venous compression.

0735-6757/$ – see front matter D 2005 Elsevier Inc. All rights reserved.

doi:10.1016/j.ajem.2003.12.025

* Corresponding author. Tel.: +39 06 30155203; fax: +39 06

35402286.

E-mail address: [email protected] (M. Piastra).

This is a rare but serious condition, sometimes manifesting

bde novo,Q requiring prompt treatment. In children, malig-

nant lymphomas are the second most common cause [1] of

SVCS after surgery for congenital heart diseases. The

clinical picture at onset may be critical—as in presented

cases—and upper airway obstruction, superior vena cava

(SVC), cardiac or pulmonary artery compression, and acute

pulmonary edema can represent the initial clinical signs in

previously apparently healthy children. Up to now, cases of

mediastinal neoplasm emergencies have been reported only

American Journal of Emergency Medicine (2005) 23, 76–82

Life-threatening presentation of mediastinal neoplasms: report on 7 consecutive pediatric patients 77

rarely as single-case reports or among general series [1-3];

on the contrary, perioperative morbidity and mortality of

patients with superior mediastinal syndrome/SVCS under-

going surgical procedures have been previously analyzed by

several authors, and therapeutic approach discussed. All

authors agree in defining these children as extremely

unstable and challenging for the caregivers.

2. Illustrative cases

From 1994 to 2002, 7 patients suffering from severe

cardiorespiratory compromise because of the newly diag-

nosed anterior mediastinal mass were admitted to the

Pediatric Intensive Care Unit (PICU) at Catholic University

of Rome (Table 1). Their ages ranged from 0.8 to 14 years

(mean, 4.5 years). The clinical manifestations of SVC

compression included variable degrees of face, neck, and

upper thorax swelling; external jugular and superficial chest

vein distension was present in all patients, sometimes

associated with cyanosis or plethora. Stridor, dyspnea, and

cough were almost always apparent at presentation, together

with increased oxygen need.

1. A 4-year-old boy with signs of impending cardiore-

spiratory failure was referred to our PICU in November

1994 from another hospital. He was diagnosed having a

mediastinal mass 7 days before: the onset of clinical history

was characterized by signs of worsening respiratory

compromise. Physical examination revealed hepatospleno-

megaly and lymph node enlargement. After a peripheral

node biopsy, chemotherapy was initially performed using

cyclophosphamide, vincristine, and methylprednisolone. On

arrival, the child appeared extremely distressed, gasping,

and cyanotic, and then was immediately intubated and bag

ventilated; a chest x-ray showed a large anterior mediastinal

mass that impaired pulmonary ventilation, mostly on the

right lung (Fig. 1). Pressure-regulated volume-controlled

ventilation was set (tidal volume, 6 mL/kg; rate, 20/min).

Table 1 Clinical presentation and complications during the PICU stay

Case no./year Age Sex Pathological

diagnosis

Cardi

comp

1/1994 4 M T-cell lymphoma Sever

2/1995 14 F T-cell lymphoma Extre

3/1995 1.6 M T-cell lymphoma Mode

4/1998 4 M Pleuropulmonary

blastoma

Sever

5/1999 7 F T-cell lymphoma Extre

6/2002 0.8 M Teratoma Mode

7/2003 1.4 F Anaplastic lymphoma Mode

Abbreviations: PD, peritoneal dialysis; CVVH, continuous veno-venous hemofilt

Given are the arterial blood gas analysis results: pH 7.3;

Pao2, 87; Paco2, 51.5; Sao2, 95; Fio2, 0.7). Instead of a

previously suggested irradiation treatment, a further che-

motherapy approach using daunomycin (60 mg/m2) was

used. On day 2, a clear picture of SVC obstruction appeared,

with a swelling of the upper torso/facial tissues; liver margin

was appreciable at 4 cm. At the echocardiographic examina-

tion, suprahepatic dilated veins and pericardial effusion

were detected; because of the initial heart pump failure, an

inotropic support was added (dopamine, 5 lg/kg per

minute; dobutamine, 7 lg/kg per min). From day 4, the

child progressively improved spontaneous breathing capac-

ity—as revealed by a spirometer—and could be weaned and

extubated uneventfully. Subsequently, he was discharged

from the PICU to the Oncologic Department. After

prolonged chemotherapy treatment, he is at present free

from disease and off-therapy from 1996.

2. A 14-year-old girl was admitted to the PICU on June

1995 because of acute respiratory distress. She complained

respiratory difficulties, progressive fatigue, and weight loss

for 15 days, together with slight elevated body temperature.

A huge mediastinal mass was diagnosed in the emergency

department by a chest x-ray (Fig. 2). On physical examina-

tion, the child had polypnea/orthopnea and labial cyanosis;

because of rapid worsening of patient’s spontaneous

breathing after an initial trial of mask ventilation, endotra-

cheal intubation and positive pressure ventilation were

necessary. A pericardial effusion with signs of cardiac

tamponade needed pericardial drainage. During the clinical

course in PICU, she developed severe hypotension, and

inotropic support was added using a dopamine/dobutamine

association. After a bone marrow aspirate, chemotherapeutic

treatment with cyclophosphamide, vincristine, and methyl-

prednisolone was given. Oliguria (b0.3 mL/kg per hour)

and increasing blood urine nitrogen, creatinine, and uric

acid occurred, necessitating peritoneal dialysis. Fluid

retention and systemic hypertension were controlled,

whereas a moderate reduction in tumor size permitted

of patients described in cases 1 to 7

orespiratory

romise

Mechanical

ventilation (d)

Complications

in the PICU

e 3 Right/left ventricular

pump deficit,

left ventricular diastolic

dysfunction

me 8 Acute renal failure (PD),

cerebral hemorrhage, coma

rate-severe – No complications in PICU

e 5 Respiratory failure,

heart diastolic dysfunction

me 15 Acute renal failure (CVVH),

coma, septic shock

rate-severe 4 Respiratory failure

rate-severe – No complications in PICU

ration.

Fig. 3 Radiological appearance of mediastinal mass on PICU

admission in patient 3. Of interest, the tracheal displacement on the

anteroposterior film is well appreciable.

Fig. 1 Radiological appearance of mediastinal mass on PICU

admission in patient 1.

M. Piastra et al.78

tracheal extubation on day 8. Chemotherapeutic toxicity

occurred (thrombocytopenia; nadir value, 18000/lL on

day 14). The patient was discharged from the PICU on day

10 and readmitted on day 13 because of sudden onset of

deep coma. Right anisocoria was present, and a cerebral

computed tomography (CT) scan showed a subdural

hemorrhage in the posterior cranial fossa and a triventricular

hydrocephalus. A surgical drainage was performed and

antiedema therapy was started. Hypercellularity in cerebro-

spinal fluid examination was found (955 cells/lL, primarily

blasts) and intrathecal methothrexate was given. No further

improvement of the clinical status was obtained: the

patient’s condition gradually deteriorated and she died

7 days later.

3. A 20-month-old boy with signs of SVCS and

respiratory compromise was admitted on November 1995.

The clinical history began 2 weeks before having signs of

intermittent wheezing and deteriorated after 48 hours from

admission, showing the picture of SVCS. On chest x-rays,

an anterior mediastinal mass was diagnosed, causing

compression and right displacement of the trachea (Fig. 3).

The child appeared polypneic (respiratory rate N45/min) and

tachycardic; labial cyanosis was present on room air; Sao2

more than 88% to 89% could only be achieved by mask

Fig. 2 Radiological appearance of mediastinal mass on PICU

admission in patient 2.

oxygen therapy (Fio2 60%) and subsequent continuous

positive airway pressure +4 to 6 cm H2O administration via

a tight-fitting face mask. Spontaneous breathing improved

by turning the child in a semiprone position. A chest CT scan

showed an infiltrating mass constricting the pulmonary

artery trunk and posteriorly displacing the trachea and

mainstem bronchi. A bone marrow tap showed lymphoma-

tous infiltration (immature T-cell lymphoma). Before

definitive histology, a chemotherapeutic treatment was

started using cyclophosphamide (300 mg/m2) and methyl-

prednisolone (60 mg/m2). In the following 24 to 36 hours, a

dramatic improvement of spontaneous breathing capacity

was obtained, and the child was discharged to the oncologic

ward to continue chemotherapy in an aseptic environment.

Control chest x-rays showed a visible mass reduction and

pulmonary ventilation improvement. The child is now

disease free and off-therapy from August 1998.

4. A 3-year-old child came to our PICU in June 1998

because of impending respiratory failure, with a 1-month

history of cough, dyspnea, andwheezing that is misdiagnosed

as asthma. On admission, chest radiograms and CT scan

showed an enormous mass that caused an extreme tracheal

Fig. 4 Radiological appearance of mediastinal mass on PICU

admission in patient 4. Of interest, the tracheal displacement on the

anteroposterior film is well appreciable.

Fig. 5 a, Radiological appearance of mediastinal mass on PICU

admission in patient 5. b, The postintensive chest film has been

added, showing a remarkable narrowing of the tracheal lumen.

Life-threatening presentation of mediastinal neoplasms: report on 7 consecutive pediatric patients 79

distortion, and a right shift was evidenced (Fig. 4). Due to the

severity of clinical presentation and the poor general status,

chemotherapy was immediately started using cyclophospha-

mide 1 g/m2 intravenously, even in the absence of a

histological confirmation. In the following hours, the child

developed acidosis and hypercapnia as respiratory muscles

became seriously fatigued. Arterial blood gas analysis

worsened further (pH 7.04; Pao2, 62 mm Hg; Paco2, 73.5

mm Hg, HCO3, 19.2 mEq/L; base excess (BE), �13; Sao2,

89%-90%; Fio2, 1) and the child needed tracheal intubation

and mechanical ventilation. Initial parameters were peak

inspiratory pressure (PIP), 35 mm Hg; positive end-

expiratory pressure, 5; risk ratio, 24; inspiratory time, 35%;

Fio2, 1. A pulmonary biopsy was performed and histology

showed a mesenchymal neoplasm with ovoid cells.

Adriamycin, 20-mg intravenous bolus as a single dose,

was added to his therapy. Bone marrow aspirate resulted

negative. The child was then paralyzed and underwent

controlled one-lung ventilation with permissive hypercap-

nia. After 5 days, the functional obstruction was gradually

removed, thus, allowing spontaneous breathing restoration

and tracheal extubation. A control CT scan confirmed a mass

volume reduction; day 9 from admission, the child could be

discharged to the pediatric oncology division. Definitive

histological diagnosis revealed a rare pleuropulmonary

blastoma. On October 1998, the child underwent left lower

lobectomy; off-therapy from May 1999, he had no evidence

of disease.

5. A 7-year-old girl was admitted to the PICU of Catholic

University in September 1999 on emergency basis because

of sudden onset of cardiorespiratory failure. Previous

clinical history was unremarkable, except for the presence

of cervical vein engorgement 48 hours before the admission.

The patient suddenly awoke from night sleep having severe

asphyxia and hypotension, and then precipitated into a deep

coma. After initial resuscitation, arterial blood gases showed

severe mixed acidosis (pH 6.7; Pco2, 158 mm Hg; BE,

�15; HCO3, 20). On arrival to the PICU, an extreme picture

of cardiac tamponade and respiratory failure was diagnosed.

A very high resistance to pulmonary inflation was encoun-

tered, due in part to the presence of cardiogenic pulmonary

edema: hand ventilation was performed intermittently to

supply inflation pressures in excess of 60 to 70 cm H2O: the

patient tolerated the highest airway pressures poorly and

became hypotensive, needing intravenous colloid intake and

elevated dopamine/dobutamine inotropic support. Maximal

steroid therapy was started on admission to the PICU. A CT

scan confirmed the chest x-ray findings, showing an

enormous mediastinal mass inglobating the heart and the

great vessels, and no pericardial fluid was detected (Fig. 5a).

In spite of the maximal inotropic support, the patient

remained severely hypotensive (arterial pressure, 60/40 mm

Hg) and a further epinephrine infusion needed to be titrated

up to 0.4 lg/kg per minute. At 10 to 12 hours from the

admission, hemodynamic status progressively improved, and

blood pressure was stabilized (N90/50 mm Hg). Chemother-

apy was started on presumptive basis (suspected diagnosis of

lymphoblastic lymphoma) after a percutaneous biopsy was

performed. In the following days, oliguria/anuria with

hyperuricemia (uric acid level, 12 mg/dL before chemother-

apy) developed, and continuous venovenous hemodiafiltra-

tion via an indwelling femoral double-lumen catheter was

started to replace renal function and to control fluid/

electrolyte balance. In spite of the aggressive chemotherapy,

the mass showed minimal or absent volume reduction, and

the patient still needed high inflation pressures (in excess of

40 cm H2O) to obtain a satisfying pulmonary ventilation.

Hematologic toxicity also developed. Moderate improve-

ment of airway patency was achieved by a radiotherapeutic

treatment on the mediastinum in a divided dose of 150 cGy

for 4 days, and the trachea could be extubated on day 18

from admission. Previously, attempts to withdraw endo-

tracheal (ET) tube were unsuccessful, and a tracheal tube was

still necessary to maintain airway patency. On control chest

radiogram in absence of endotracheal tube, narrowing and

displacement of the trachea resulted well appreciable

(Fig. 5b). Humidified and heated oxygen and aerosolized

epinephrine and steroids were given to minimize mucosal

edema and made spontaneous breathing easier. The girl was

transferred to the oncologic ward 3 weeks after PICU

admission. One week later, she was readmitted to the PICU

Fig. 7 Radiological appearance of mediastinal mass on PICU

admission in patient 7.

M. Piastra et al.80

after a trial of chemotherapy because of respiratory failure,

then she died on the 30th hospital day in septic shock.

Postmortem examination confirmed a T-cell lineage lym-

phoblastic lymphoma.

6. A 10-month-old child was referred to the ED from a

peripheral hospital with a 7-month history of recurrent upper

respiratory infections. Wheezing signs were reported inter-

mittently. Two weeks before the admission, the patient had a

high fever and productive coughing. An antibiotic treatment

(10 days intramuscular ceftazidime) obtained only a brief

interval of apyrexia. Chest x-ray revealed a massive

opacification of the right lung; the midline resulted shifted

to the left. A right posterior basal indistinct image was

present. The chest CT scan showed a 10-cm-diameter mass,

arising from the anterior mediastinum. Solid, liquid, and fat

areas were present, together with calcifications. A mediasti-

nal teratoma was hypothesized, extending mostly on the right

hemithorax and causing compression of the homolateral

parenchyma and right mainstem bronchus. Signs of air

trapping were well appreciable peripherally. The echocardio-

gram demonstrated a compression on the right atrial wall. On

admission to PICU, the child was distressed, polypneic, and

peripheral oxygen saturation on Fio2 70% was 89% to 90%.

Metallic, repetitive coughing attacks were present. An

emergency surgical removal of the mass was undertaken.

After surgery, the child was paralyzed and mechanically

ventilated for 72 hours. Aspiration chest drainages promoted

right lung reexpansion. The child was extubated and could

resume spontaneous breathing. Histology confirmed amature

teratoma (Fig. 6).

7. A 17-month-old child came to the ED for cough and

increasing dyspnea with a 2-week history of upper airway

infections. A chest x-ray documented a mediastinal enlarge-

ment and right pleural effusion (Fig. 7). A bidimensional

echocardiography revealed a moderate cardiac effusion and

a 6-cm diameter mass anterior to the pulmonary artery,

expanding to the right with a moderate infundibulum

compression. On physical examination, enlarged supra-

Fig. 6 Radiological appearance of mediastinal mass on PICU

admission in patient 6.

clavicular nodes and a reduced air entry mostly in the right

hemithorax were evidenced. The child’s clinical conditions

suddenly deteriorated, dyspnea worsened, and high oxygen

support (Fio2, 0.70) was necessary to achieve an 88% to

90% peripheral oxygen saturation. Node biopsy, bone

marrow aspiration, and biopsy were performed in emergen-

cy; sedation/analgesia was provided by propofol/fentanyl

intravenous administration, avoiding muscle relaxants and

tracheal intubation. On these bases, steroids were started

without a definitive diagnosis. Oxygen supplementation

through Venturi mask and intermittent continuous positive

airway pressure were adopted to allow an acceptable arterial

gas analysis; circulatory status was stabilized with colloid

supplementation and low-dose dopamine infusion. Clinical

conditions improved in the following 24 to 48 hours,

permitting oxygen support decrease. A total body CT scan

was performed, revealing a jugular and supraclavear node

enlargement (2-cm diameter). An anterior mediastinal mass

with a dysomogeneous enhancement (5 cm diameter) was

evidenced, compressing the vena cava and the right atrium

and ventricle, and inglobating the epiaortic vessels and

trachea. Pulmonary parenchyma resulted uninjured. No

abdominal anomalies were recorded. Node histology

revealed an anaplastic non-Hodgkin’s lymphoma (CD30+)

and a chemotherapeutic treatment was started.

3. Discussion

Rapidly evolving symptoms of respiratory compromise

from an anterior mediastinal neoplasm represent true

emergencies that mandate prompt treatment in an ICU

setting. Large mediastinal masses may result in life-

threatening events such as upper airway obstruction, cardiac

or pulmonary artery compression, and acute pulmonary

edema. Airway obstruction can arise unexpectedly at any

time, even after the trachea has been secured through

intubation. In addition, circulatory compromise becomes

clinically apparent when compression of the great vessels

Life-threatening presentation of mediastinal neoplasms: report on 7 consecutive pediatric patients 81

occurs despite airway patency. Airway obstruction and

cardiovascular compression are the cornerstone of the

clinical picture of these patients: as tumors increase in size,

the trachea and the mainstem bronchi as well as the major

vessels may be exposed to an increasingly positive pressure.

When the transmural pressure of the airway/SVC exceeds

the elastic recoil of the wall of these structures, extrinsic

compression develops. Once these complications occur, the

death rate appears to be high.

In children, most anterior mediastinal tumors are

malignant (mostly lymphomas) [4]. Their clinical presenta-

tion may be critical and the additive effects of general

anesthetics or positioning during diagnostic procedures may

contribute to worsen the airway obstruction leading to fatal

cardiorespiratory failure. Even in large malignant lympho-

mas, a prompt chemotherapy and/or radiotherapy may lead

to effective compression relief [5]. On the other hand, an

early treatment—designed to cause a rapid shrinking of the

tumor—can make definitive diagnosis impossible: if the

patient is in critical condition, however, the treatment should

not be delayed until definitive histological diagnosis is

achieved [5-7].

In literature, few cases have been described of really

critical presentation, that is, children that needed immediate

resuscitation, airway patency restoration, and intensive care

approach. Regarding this presentation, 2 children have been

reported to be admitted in near asphyxiation; they were

resuscitated with endobronchial intubation, but both patients

eventually died in the ICU from sepsis and massive

hemorrhage, respectively [8,9]. Jeng et al [10] reported 7

pediatric SVCS over a 12-year period, of whom 2 cases

received immediate resuscitation upon arrival and died

within 2 hours. Freud et al [2] recently reported 5 children

among a mixed series requiring emergency respiratory

support on admission to PICU; all were affected by a

malignant lymphoma. On the other hand, several case reports

exist of patients who developed airway and/or circulatory

complications when an elective anesthesiologic procedure

was performed. A review of 163 consecutive patients

younger than 18 years at Memorial Sloan Kettering Cancer

Center has been published in 1990 [11]. An anterior

mediastinal mass was a component of their disease; only 9/

44 patients that required anesthesia (20%) were symptomatic

preoperatively: SVCS was present in 5/44 (about 1l%).

Admission plain chest radiographs may not reveal the

presence of airway compromise; all the authors underline

the risks of managing such patients, also in elective

procedures [12], because cardiorespiratory complications

may occur abruptly, often not related to mild preoperative

respiratory symptoms and roentgenographic evidence [13].

The severity of pulmonary symptoms—although sugges-

tive—is not a reliable indicator of the degree of tracheo-

bronchial compromise [14]. Also, after a successful

resuscitative approach, the clinical picture may worsen

because of nonrespiratory complications: tumor lysis syn-

drome may be expected as a direct consequence of the

chemotherapy-induced tumor shrinking, necessitating emer-

gency extrarenal depuration, mostly by continuous venove-

nous hemofiltration or hemodiafiltration [15]. Preventive

hemofiltration/hemodiafiltration is rarely applicable to

patients with sudden onset of neoplastic SVCS.

Overall, some specific points are to be emphasized:

1. All efforts should be directed toward patient stabilization

in the emergency department and intensive care setting.

The best available cardiovascular monitoring should be

placed as soon as possible. Spontaneous breathing

should be maintained—if clinically acceptable—to

avoid the negative effects of deep sedation and muscle

paralysis [16]. Anxiety treatment with midazolam is

always desirable. Pressure support ventilation—even

noninvasively—may result more tolerated.

2. Therapeutic approach would be best guided by tissue

diagnosis, revealing the tumor nature, but diagnostic

procedures are often poorly tolerated, time-consuming,

and may lead to clinical deterioration. The least invasive

bedside procedures should be performed under local

anesthesia, avoiding the additive risks of general

anesthesia. Recently, criteria on the basis of peak

expiratory flow rate and tracheal cross-sectional area

have been proposed to identify high-risk patients

[17,18].

3. Severely symptomatic patients, with unsatisfying hemo-

dynamic and/or ventilatory stabilization, will require

empiric pretreatment of the mass before definitive

diagnosis. Therapeutic strategy will be guided by the

clinical/radiological findings.

Cases of emergency onset represent a serious challenge

for both intensivists and pediatric oncologists, and an

aggressive therapeutic approach is necessary to achieve the

best clinical result: give the patient a chance to benefit from

specific therapy, once the diagnosis has been fully estab-

lished. The initial presence of a threatening SVCS associated

with a critical clinical picture does not preclude both PICU

discharge and long-term survival in this patient group.

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