The

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Case Records of the Massachusetts General Hospital

Founded by RichardC. Cabot
EricS. Rosenberg, M.D., Editor
JoAnneO. Shepard, M.D., Associate Editor
SallyH. Ebeling, Assistant Editor

NancyLee Harris, M.D., Editor

AliceM. Cort, M.D., Associate Editor
EmilyK. McDonald, Assistant Editor

Case 25-2015: An 8-Year-Old Girl with a

Chest-Wall Mass and a Pleural Effusion
SamuelM. Moskowitz, M.D., Randheer Shailam, M.D., and EugeneJ. Mark, M.D.

Pr e sen tat ion of C a se

Dr. Jessica M. Rosenthal (Pediatrics): An 8-year-old girl who had required long-term
tracheostomy because of airway stenosis caused by an injury in infancy was admitted
to this hospital because of a chest-wall mass and a large pleural effusion.
The patient was born in China. At 7 months of age, she underwent emergency
tracheostomy after a caustic injury from either ingestion or inhalation. At 2.5 years
of age, she was adopted by an American family and moved to the United States.
Her adoptive parents noted that she had stridor and snoring, and her pediatrician
referred her to an otolaryngologist at the Massachusetts Eye and Ear Infirmary (MEEI)
at 2 years 10 months of age. On examination, the nasopharynx was completely obstructed; there was fusion of the posterior tongue and hard palate and of the hard
palate and epiglottis, with a narrow opening in the oropharynx. The supraglottic passage was normal beyond the stenosis; the subglottic airway was not evaluated.
A tracheostomy was performed, and the left nasal choanal obstruction was
opened with a laser. Repeat bronchoscopy revealed that the larynx and subglottic
trachea were normal. On two occasions, esophagogastroduodenoscopy revealed no
evidence of gastroesophageal reflux. The tracheostomy tube was removed when
the patient was 5.5 years of age; there was a residual trachealcutaneous fistula.
When she was 7.5 years of age (7 months before this admission), another tracheostomy was performed because of worsening oropharyngeal stenosis that precluded intubation and because of concern about possible obstruction.
The patient had been in her usual state of health until approximately 6.5 weeks
before this admission; during a 10-day period, her mother noted recurrent bloodtinged sputum in the tracheostomy tube, and an episode of bleeding at the tracheostomy site occurred at school. The patient was seen in the emergency department
of the MEEI, where a flexible tracheoscopy, which was performed at the bedside
after removal of the tracheostomy tube, revealed no exposed blood vessels or
granulation tissue in the trachea or stoma. A chest radiograph showed consolidation in the right lower lobe, which was thought to be due to aspiration, pneumonia, or atelectasis. Grams staining of the sputum showed abundant neutrophils,
abundant gram-positive cocci in pairs and a few chains, abundant gram-negative
diplococci, a few gram-positive rods, and a few thin gram-negative rods. Culture

From the Departments of Pediatrics

(S.M.M.), Radiology (R.S.), and Patholo
gy (E.J.M.), Massachusetts General Hos
pital; and the Departments of Pediatrics
(S.M.M.), Radiology (R.S.), and Pathology
(E.J.M.), Harvard Medical School both
in Boston.
N Engl J Med 2015;373:657-67.
DOI: 10.1056/NEJMcpc1400836
Copyright 2015 Massachusetts Medical Society.

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of the sputum from the tracheostomy tube grew

methicillin-susceptible Staphylococcus aureus, Moraxella catarrhalis, Haemophilus influenzae, and Streptococcus pneumoniae. The patient remained afebrile,
and no antibiotic agents were administered. She
was admitted overnight for observation and discharged home the next day (5 weeks before this
admission). She had one more episode of bloodtinged sputum thereafter.
During the 2 weeks before this admission, the
oxygen saturation, which was obtained during
continuous overnight monitoring for sleep apnea,
fell to approximately 85% (from the usual level
of approximately 95%) for prolonged periods.
Examination at school revealed decreased breath
sounds at the base of the right lung. One day before this admission, while the patient was being
bathed, her mother noticed that her chest was
asymmetric, with the right nipple appearing lower
than the left, and that blood vessels were prominent on the right side of the chest.
At the pediatricians office the next day, the
patient described a recent episode of discomfort
in her chest that occurred while she was being
hugged. There was no history of respiratory distress, fevers, or cough. On examination, she was
tearful and hesitant. The temperature was 36.1C,
and a tracheostomy tube was in place. The right
lateral chest wall was more prominent than the
left, had no crepitus, and was tender to touch.
Breath sounds were normal on the left side and
diminished on the right. The right upper quadrant of the abdomen was diffusely tender, and the
remainder of the examination was normal. The
patient was referred to another hospital for chest
radiography, which revealed a large pleural effusion on the right side, with underlying pneumonia. The patient was referred to the emergency
department of this hospital.
The patient had sleep apnea and a history of
dental caries that necessitated the extraction of
several deciduous teeth between 5 and 7 years
of age. Immunizations, including the 7-valent
pneumococcal conjugate vaccine (PCV7), were
current; she had reportedly received the bacille
CalmetteGurin (BCG) vaccine at 1 month of
age. A purified protein derivative (PPD) skin test
was negative on her arrival in the United States.
She took no medications and had no known allergies. She lived at home with her adoptive parents

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and siblings; she was physically active, did well in

school, and was able to breathe, speak, and eat
normally, with the tracheostomy tube capped while
she was awake. She lived with two dogs and a cat,
and the mother smoked outside the home; she
had not recently traveled or had contact with ill
persons.
On examination, the temperature was 37.0C,
the blood pressure 102/68 mm Hg, the pulse
135 beats per minute, the respiratory rate 16 to
30 breaths per minute, and the oxygen saturation
95% while the patient was breathing ambient air.
The oropharynx was clear, without erythema or
focal lesions. The neck was supple, and the tracheostomy site was clean and dry, with an underlying small cavity. Lymphadenopathy (with nodes
measuring up to 1 cm in diameter) in the right
anterior cervical chain and a mildly tender right
axillary lymph node (approximately 1 cm in diameter) were present. There were decreased
breath sounds at the base of the right lung.
There was a bulging area (4 cm by 7 cm) on the
right lateral chest wall, which was tender on
palpation and had no fluctuance or overlying skin
changes. The hematocrit, red-cell indexes, and
anion gap were normal, as were blood levels of
calcium, magnesium, glucose, albumin, amylase,
lipase, uric acid, and lactic dehydrogenase and
results of renal- and liver-function tests; other
test results are shown in Table1.
Dr. Randheer Shailam: A frontal chest radiograph
that had been obtained 7 months before this
admission showed a tracheostomy tube, clear
lungs, and no pleural effusion. Frontal, lateral,
and bilateral decubitus chest radiographs that
were obtained on the day of admission (Fig.1)
showed dense air-space opacification in the right
lower lung zone, obscuring the right hemidiaphragm and right cardiac border, as well as a large
pleural effusion on the right side that tracked
along the lateral chest wall, with evidence of loculation. The left lung and pleural space were clear.
Computed tomography (CT) of the chest, performed on the same day after the administration
of contrast material (Fig.2), revealed asymmetry
of the chest wall that was due to multiple heterogeneous soft-tissue masses and thickening of the
soft tissue of the right chest wall. Consolidation
was present in the right lower lobe and portions
of the right middle lobe. Areas of low attenua-

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Case Records of the Massachuset ts Gener al Hospital

tion in the right lower lobe were suggestive of decreased perfusion and possible necrosis. A large
loculated pleural effusion, irregularly shaped parenchymal nodules in the right upper lobe, prominent enhancing ipsilateral axillary lymph nodes,
and subtle periosteal elevation of the medial aspect of the right eighth rib were also present.
Dr. Rosenthal: The patient was admitted to the
pediatric intensive care unit. The temperature rose
to 38.7C, and acetaminophen was administered,
with improvement.
On the second hospital day, a diagnostic procedure was performed.

Table 1. Laboratory Data.*

Reference Range,
Age-Adjusted

On Admission,
This Hospital

11.515.5

11.2

450013,500

11,500

Neutrophils

3359

73

Lymphocytes

3350

20

Monocytes

411

08

150,000450,000

891,000

Sodium (mmol/liter)

135145

134

Potassium (mmol/liter)

3.44.8

3.8

Chloride (mmol/liter)

100108

96

Carbon dioxide (mmol/liter)

23.031.9

25.1

Total

6.08.3

8.5

Globulin

2.34.1

5.0

4.55.5

4.0

<8.0 for inflammation

217.2

Variable
Hemoglobin (g/dl)
White-cell count (per

mm3)

Differential count (%)

Eosinophils
3)

Platelet count (per mm

Differ en t i a l Di agnosis
Dr. Samuel M. Moskowitz: This 8-year-old girl had
had a caustic exposure during infancy that resulted
in upper-airway obstruction and tracheostomy
dependence. I suspect that her airway injury may
have caused mild dysphagia, with an increased risk
of tracheal aspiration. She had a history of poor
oral hygiene. Her immunizations included PCV7
and BCG, and a PPD skin test was negative after
she immigrated to the United States. Six weeks
before this admission, bacterial tracheitis with S.
aureus, S. pneumoniae, and H. influenzae developed,
and focal opacification of the right lower lobe
was noted. Bacterial tracheobronchitis and pneumonia are common in children who have undergone tracheostomy, with an annual incidence of
up to 88%.1 The tracheitis abated spontaneously,
without antibiotic treatment, whereas the lobar
process progressed and was accompanied by hypoxemia, chest tenderness, decreased breath
sounds, and pleural effusion but no fever.
On presentation, the patient had tachypnea,
prominence and tenderness of the right lateral
chest wall, and a tender right axillary lymph node.
Laboratory test results showed elevated levels of
acute-phase reactants, such as thrombocytosis
and hyperglobulinemia. Imaging studies of the
chest revealed opacification in the right lower
lung zone, a loculated effusion, and an irregular
chest-wall mass, with regional lymph-node enhancement and minimal bony changes.
The chest-wall mass is the most worrisome
clinical feature of this childs presentation. An
acquired chest-wall deformity or mass can originate in bone or soft tissue; the differential diag-

Protein (g/dl)

Phosphorus (mg/dl)
C-reactive protein (mg/liter)

* To convert the values for phosphorus to millimoles per liter, multiply by
0.3229.
Reference values are affected by many variables, including the patient popula
tion and the laboratory methods used. The ranges used at Massachusetts
General Hospital are age-adjusted for patients who are not pregnant and do
not have medical conditions that could affect the results. They may therefore
not be appropriate for all patients.

nosis includes trauma, neoplasm, inflammatory

and infectious processes, or a combination of
these causes (Table2). Before the results of imaging studies are available to rule in or rule out
specific causes, the mass could represent a herniated right lung, a benign or malignant tumor,
a sterile collection of inflammatory cells, or an
abscess.
Chest-Wall Trauma

Traumatic disruption of the skeletal components of the chest wall can result in flail chest,
with paradoxical chest-wall motion and compromised gas exchange; these findings were
not present in this child. Lung herniation is an
uncommon chest-wall deformity that causes

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*
*

Figure 1. Chest Radiographs.

Radiographs of the chest were obtained on admission. Frontal and lateral chest radiographs (Panels A and B, re
spectively) show airspace opacification in the right lower lung zone (asterisks) and a large right pleural effusion
(arrows). Decubitus chest radiographs (Panel C [right side down] and Panel D [left side down]) show that pleural
fluid on the right side is not freely layering (arrows).

dyspnea, pain, and a bulge that varies with the

respiratory cycle. It usually results from trauma
but can also be congenital, arise spontaneously
after a coughing paroxysm, or occur in patients
with neoplastic, inflammatory, or infectious disorders of the chest wall.2 In this child, examination and imaging studies did not reveal the
characteristic bulge of a herniated lung. With-

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out a history of recent trauma, the diagnoses of

hematoma and ruptured hemidiaphragm are also
unlikely.
Benign and Malignant Tumors
of the Chest Wall

Various benign and malignant tumors can arise

from the bones of the chest wall (Table 2). How-

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Case Records of the Massachuset ts Gener al Hospital

*
*

Figure 2. CT Scans of the Chest.

CT scans of the chest were obtained on admission, after the intravenous administration of contrast material. Panel
A shows asymmetry of the chest wall, thickening of the soft tissue of the right chest wall (arrowheads), and multiple
heterogeneous softtissue masses (arrows). Panels B and C show irregularly shaped parenchymal nodules in the
right upper lobe (arrows). Panel D shows consolidation in the right lower lobe (arrow) and portions of the right
middle lobe, areas of low attenuation in the right lower lobe that suggest decreased perfusion and possible necrosis
(arrowheads), and a large loculated pleural effusion (asterisks). Panel E also shows the large loculated pleural effu
sion (asterisks), as well as a prominent enhancing axillary lymph node (arrow). Panel F shows subtle periosteal ele
vation of the medial aspect of the right eighth rib (arrow).

ever, in this case, chest CT revealed only a minimal rib lesion, and thus a bony tumor is unlikely.
Some benign tumors that occur in children, such
as lymphangioma (cystic hygroma) or hemangioma, arise in the soft tissue of the chest wall and
are generally confined to it, without the pleural
and pulmonary components that were seen in

n engl j med 373;7

this case. Other benign tumors originating in soft

tissue, such as lipoma, are more common in
adults than in children. Peripheral-nerve tumors
can be associated with effusion and pleural nodules but would be more likely to occur in a patient
with known neurofibromatosis. Desmoid tumor,
a histologically benign fibromatosis that can be

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Table 2. Differential Diagnosis of Acquired Chest-Wall Deformity or Mass in a Child.

Diagnosis

Originates in or Affects Bone

Trauma

Originates in or Affects Soft Tissue

Flail chest

Lung herniation, hematoma, ruptured hemidiaphragm

Benign tumor

Eosinophilic granuloma (Langerhans-cell histiocyto

sis), osteochondroma (exostosis), chondroma,
chondroblastoma, fibrous dysplasia, osteoid
osteoma, osteoblastoma, mesenchymal ham
artoma

Lymphangioma (cystic hygroma), hemangioma, periph

eral-nerve tumor (neurofibroma, plexiform neurofi
broma, schwannoma), fibroma, fibromatosis (des
moid tumor), lipoma, benign cystic mesothelioma,
inflammatory myofibroblastic tumor

Malignant neoplasm

Osteosarcoma of the rib, chondrosarcoma of the rib,

primitive neuroectodermal tumor (Ewings sar
coma, Askins tumor), multiple myeloma, metas
tasis

Rhabdomyosarcoma, other sarcomas (neurofibrosarco

ma, fibrosarcoma, leiomyosarcoma, angiosarcoma,
liposarcoma), pleuropulmonary blastoma, lympho
ma, malignant fibrous histiocytoma, metastasis

Inflammatory process

Hyperostosis, SAPHO syndrome (synovitis, acne,

pustulosis, hyperostosis, osteitis), chronic recur
rent multifocal osteomyelitis (nonpurulent in
flammation of bone), Friedrichs disease (aseptic
necrosis of the sternoclavicular joint)

Sebaceous cyst

Infectious process

Osteomyelitis (tuberculosis, actinomycosis), septic

arthritis

locally invasive, is more likely to occur in the

shoulder region than in the anterolateral chest.3
Benign mesothelioma is rare in children and
usually confined to the pleural space.
Patients with an inflammatory myofibroblastic tumor can present with a soft-tissue mass of
the chest wall. It arises from lung parenchyma and
can extend into the chest wall, with localized mass
effect.4 These lesions, which are the most common lung tumors in children, are typically benign
but can occasionally undergo malignant transformation. They are typically associated with dyspnea, cough, hemoptysis, and fever. Laboratory
evaluation may reveal iron-deficiency anemia,
thrombocytosis, and hypergammaglobulinemia.
However, chest-wall invasion is inconsistent and
generally occurs no sooner than 12 to 18 months
after the onset of symptoms, pleural effusions are
uncommon, and lymphadenopathy is rare. Associated chest CT reveals a varying nonspecific pattern, with heterogeneous enhancement and attenuation.5 The laboratory test results and imaging
findings in this patient were suggestive of an inflammatory myofibroblastic tumor, but her course
was unusually rapid for this lesion and she did not
have several of the usual signs and symptoms.
This child did not have persistent systemic
symptoms suggestive of a malignant tumor; how-

662

Abscess, phlegmon, cellulitis, empyema necessitatis

ever, because of the consequences of missing a

malignant neoplasm, these lesions deserve consideration. In children, rhabdomyosarcoma is the
most common malignant tumor arising in the
soft tissue of the chest wall, but the associated
chest CT would typically reveal a discrete mass,4
which is unlike the finding in this child. Other
soft-tissue sarcomas are relatively rare in children.
Pleuropulmonary blastoma arises from the pleura or lung and can invade the chest wall, but it
usually occurs in children 5 years of age or younger. Malignant fibrous histiocytoma is usually
confined to the chest wall and is more common
in elderly persons than in children.
Lymphomas in the thorax typically arise in
the mediastinum and can extend into the anterior
chest wall, and thus a locally invasive lymphoma
needs to be considered in this case. Patients with
thoracic lymphoma typically present with chest
pain, dyspnea, and lymphadenopathy, sometimes
without fever. Early-stage Hodgkins or non-Hodgkins lymphoma that arises in the mediastinum
can invade the lung parenchyma and chest wall,
but this invasion usually is contiguous with a
tumor in the anterior mediastinum and does not
involve the lower lateral chest (the location of
the chest-wall lesion seen in this child).6,7 In addition, lymphomatous chest-wall invasion usu-

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Case Records of the Massachuset ts Gener al Hospital

ally develops more slowly than did the process

in this child.
In summary, a neoplastic process cannot be
ruled out in this case, but an inflammatory or
infectious process is more likely.
Inflammatory and Infectious Processes
of the Chest Wall

Primary inflammation of bone, such as that seen

with hyperostosis or chronic recurrent multifocal
osteomyelitis, can cause a chest-wall mass. Various pathogens can cause osteomyelitis or septic
arthritis with localized chest-wall swelling and
induration. However, the localization and imaging findings in this child were not consistent
with an osseous lesion.
Patients with a sebaceous cyst can present
with a soft-tissue mass, but it would remain
confined to the chest wall and would not invade
the pleura or lung. Soft-tissue infections of the
chest wall can be manifested by a solitary abscess or cellulitis, with associated inflammation
of adjacent tissues. However, several findings in
this child were consistent with an infection originating in the chest cavity and extending into the
chest wall.
Empyema Necessitatis

The clinical and radiographic features seen in

this case are most consistent with empyema necessitatis, a process characterized by extension of
pleural empyema into the chest wall. This condition was first described by Gullan de Baillon in
1640. Empyema necessitatis typically develops
over a period of 4 to 8 weeks and is associated
with pain, swelling, and lymphadenopathy of the
anterolateral chest, often without fever; these
features were seen in this case. It can also result
in a pleurocutaneous or bronchopleurocutaneous
fistula, which was not seen in this case. Ren
Laennec succinctly described its pathogenesis in
1819 as follows: When, as the result of chronic
pleurisy, a gangrenous eschar forms on the pleura,
it sometimes happens that the effusion infiltrates
through the intercostal muscles at this point, and
comes to form under the skin an abscess whose
natural or artificial opening provides, in a few
rare cases, a healing of the empyema. These species of abscess, known since the origin of the

art, have been observed from time to time by the

surgeons, and their opening constitutes what is
commonly called the empyema of necessity. This
case is also very rare; my friend Mr. Recamier
told me that he has observed twice; I have encountered it but once.8
The patient in Laennecs case is said to have
been a 12-year-old boy. In 1869, William Moore
described empyema necessitatis in a 10-year-old
girl.9 The publication of only a few relevant case
reports since the 19th century indicates that this
condition remains extremely rare in children;
nonetheless, I believe that this child had empyema necessitatis.
Empyema necessitatis was more common in
the era before antibiotics. At that time, it was associated with an overall mortality of approximately
66%; the most common pathogens were Mycobacterium tuberculosis (mortality, 87%) and S. pneumoniae (mortality, 28%). Since antibiotics have been
in use, cases of empyema necessitatis are rarely
fatal, and actinomyces species are a more common
cause than is S. pneumoniae. Less common pathogens include S. aureus, S. milleri, Fusobacterium nucleatum, M. avium, M. intracellulare, Burkholderia cepacia,
blastomyces species, and Nocardia asteroides.10
Given this childs history of BCG immunization and the negative PPD skin test, tuberculosis
is unlikely. Because a culture of tracheal secretions
was positive for S. pneumoniae, it would seem to be
a prime suspect; it is found in pleural fluid in
approximately 50% of children with uncomplicated empyema, and other pathogens are much
less common in these patients.11 However, the
patients history of pneumococcal vaccination
makes this pathogen unlikely. Other aerobic
bacteria in her tracheal secretions could cause
pneumonia1; of these, only S. aureus has been
reported in patients with empyema necessitatis.
Both S. pneumoniae and S. aureus would be expected
to cause fever, which was not seen in this case.
Poor oral hygiene promotes growth of bacteria
that can cause pneumonia,12 including aerobes
such as S. pneumoniae but also anaerobes that cannot be detected on a routine culture of tracheal
secretions. Chief among anaerobes that could
cause empyema necessitatis is A. israelii. Additional
factors that increase the likelihood of pulmonary
actinomycosis in this case include the risk of aspi-

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ration, subacute course, and absence of fever.

Pulmonary actinomycosis is often polymicrobial, with gram-positive bacteria or anaerobes as
common coisolates,13 and its parenchymal component may cavitate. Small-to-medium-sized pleural effusions may occur, and chest-wall invasion
may result in early rib erosion, which was seen
in this child. I suspect that an actinomyces species was her primary pathogen.
If a neoplasm were likely in this case, I might
suggest the use of positron-emission tomography (PET) or magnetic resonance imaging (MRI)
to delineate the tumor burden or ultrasoundguided or CT-guided percutaneous aspiration to
obtain a tissue sample. In cases of infection or
inflammation that is confined to the pleural and
parenchymal compartments, my preferred approach would be video-assisted thoracoscopic
surgery to facilitate pleural drainage and decortication and to obtain a lung-biopsy specimen,
as indicated. For this chest-wall lesion, I would
favor exploratory thoracotomy to obtain a biopsy
specimen and to permit open dbridement or
resection.
Dr. Nancy Lee Harris (Pathology): Dr. Ryan,
would you tell us the thinking of the clinical
team and describe the diagnostic procedure?
Dr. Daniel P. Ryan (Pediatric Surgery): We were
very concerned that the multifocal nodular pleural lesions and the large lump on her chest wall
which was not fluctuant or warm and did not
have typical signs of infection represented a
neoplastic process. We brought her to the operating room to perform a biopsy of the mass and
to place a thoracostomy tube to drain the pleural
fluid. We made a small incision over the chestwall mass. There was no subcutaneous edema,
and the mass itself was somewhat gray in appearance. We performed an incisional biopsy,
which released yellow pus that contained small,
white, solid granules (1 mm in size). Then, we
inserted a drain in the mass. We located the
pleural effusion and placed a chest tube, which
drained clear fluid. At that point, we decided not
to perform thoracoscopy.

of

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Dr . S a muel M. Moskow i t zs
Di agnosis
Empyema necessitatis, with pneumonia of the
right lower lobe and empyema of the right pleural
cavity caused by actinomyces species.

Pathol o gic a l Discussion

Dr. Eugene J. Mark: The diagnostic procedure was

a biopsy of the soft-tissue mass of the chest wall.
Histopathological examination revealed an abscess with purulent inflammation (Fig.3A), surrounded by granulomatous inflammation and
fibrosis (Fig.3B). Staining with hematoxylin and
eosin revealed a sulfur granule in the abscess
(Fig.3A). Filamentous structures were visible at
the periphery of the sulfur granule; on silver
staining, the filamentous structures could be
seen both creating the sulfur granule (Fig.3C)
and extending as bacillary organisms from the
periphery of the sulfur granule (Fig.3D). These
morphologic findings are typical of actinomyces
species.
Both actinomyces species and nocardia species are filamentous bacteria, with branching
segments that often vary as positive or negative
on Grams staining. Actinomyces are found in
the sulfur granules and not in the surrounding
inflammation.
Cultures of the tissue and abscess specimens
grew A. israelii and Aggregatibacter (formerly Actinobacillus) actinomycetemcomitans (Fig.3E and 3F).
Aggregatibacter is a gram-negative facultative
nonmotile rod that is frequently isolated together
with actinomyces. It is an oral commensal organism often found in association with localized
aggressive periodontitis.14-16
It is not clear to me in this case whether the
pleuropulmonary infection is due to aspiration
from the oral cavity or whether the trachea or
the soft tissue around the trachea is infected
with actinomyces. The closest analogy that I
have found is involvement of tissue around the
innominate artery that results in a pseudoaneurysm.17
Dr. Harris: Dr. Pasternack, would you tell us how
Cl inic a l Di agnosis
you treated the patient and how she is doing?
Possible malignant tumor of the chest wall or
Dr. Mark Pasternack (Pediatric Infectious Displeura.
eases): This patient had a classic presentation of

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Figure 3. Chest-WallBiopsy Specimen.

Hematoxylin and eosin staining shows purulent and histiocytic inflammation in adipose tissue and a sulfur granule
(dark blue) amid the inflammation (Panel A); there is fibrosis in the adipose tissue that resulted from the histiocytic
inflammation (Panel B). Methenamine silver staining of the tissue sample shows the filamentous forms of the or
ganism creating a relatively solid nodule (the sulfur granule) (Panel C); the filamentous forms also can be seen ex
tending from the periphery of the nodule (Panel D). Grams staining of smears of the cultures shows grampositive
rods, which were identified as Actinomyces israelii (Panel E), and gramnegative rods identified as Aggregatibacter
actinomycetemcomitans (Panel F). (Panels E and F are courtesy of Dr. JiYeon Kim [Pathology].)

thoracic actinomycosis. The findings in the soft of acute infection, and radiologic evidence of an
tissue of the chest wall, indolent clinical pro- apparent thoracic mass led to the initial considgression, absence of fever, signs and symptoms eration of a malignant process. The diagnosis of

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The

n e w e ng l a n d j o u r na l

infection was established only at the time of

surgical exploration.
Dr. Rosenthal and I were consulted postoperatively, after the Grams stain of the chest-wall
abscess revealed pleomorphic gram-positive rods.
In many cases of actinomycosis, cultures remain
negative, perhaps because of previous administration of antibiotics or exposure of the specimen to air. However, in this case, the culture
grew not only A. israelii but also the fastidious oral
gram-negative pathogen A. actinomycetemcomitans.
Since the child was known to have virulent
pathogens in preoperative respiratory secretions,
including methicillin-susceptible S. aureus and
Pseudomonas aeruginosa (in a culture that was obtained on the day of admission), we initiated
single-agent parenteral antibiotic therapy with
imipenem. A peripherally inserted central catheter was placed for the administration of intravenous antibiotic therapy at home. After nearly
5 weeks of therapy, acute fevers developed, with
temperatures exceeding 40C. The patient was
readmitted to this hospital. Blood cultures were
negative, and follow-up CT showed resolution of
the chest-wall lesions, with residual pleural thickening and improving consolidation in the right
lower lobe. Her fever persisted despite discontinuation of imipenem and initiation of ceftriaxone
therapy. She defervesced after her therapy was
transitioned to oral doxycycline and she underwent removal of the catheter.
The patient had a favorable response to the
doxycycline therapy and received a 1-year course
of therapy. Three years later, she is currently well,
and she and her family are considering reconstructive surgery that might permit removal of the
tracheostomy tube.
Dr. Harris: Are there questions or comments
for any of our discussants? Dr. Moskowitz, do you
have any comments?
Dr. Moskowitz: I was concerned that there was
a polymicrobial component to this infection, but
References
1. Brook I. Bacterial colonization, tracheobronchitis, and pneumonia following tracheostomy and long-term intubation in pediatric patients. Chest 1979;76:
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666

of

m e dic i n e

I thought the discussion had gone far enough

with actinomyces and elected not to delve into
six-syllable coinfectants.
A Physician: Because of the upper-airway abnormalities, is this patient at risk for a recurrence of this disease?
Dr. Pasternack: This is clearly a very rare complication of tracheostomy, and one would have to
think, as Dr. Moskowitz has suggested, that aspiration led to this complication. The patient certainly might be at risk for reaspiration.
Dr. Harris: In retrospect, do you think that the
episodes of bleeding that occurred 6 weeks before admission, with the finding of consolidation
in the right lower lobe on imaging studies, represented the beginning of this process?
Dr. Pasternack: I suspect that this was the case.
I reviewed the films from the admission at MEEI,
and the possibility that this was atelectasis of the
right lower lobe was reasonable. The upper-airway symptoms improved, so the imaging finding
in the lung was thought to not be related and
was not evaluated at follow-up.

A nat omic a l Di agnosis

Infection of the chest wall (empyema necessitatis)
with Actinomyces israelii and Aggregatibacter actinomycetemcomitans.
This case was discussed at Pediatric Grand Rounds.
Dr. Moskowitz reports receiving fees for serving on advisory
boards from Vertex Pharmaceuticals; consulting fees from
EnBiotix, MCIC Vermont, Kala Pharmaceuticals, Pfizer, and
Cowen Group; and grant support from Gilead Sciences, Novartis, Rempex Pharmaceuticals, Kala Pharmaceuticals, Vertex
Pharmaceuticals, and Savara Pharmaceuticals; as well as holding a pending patent for intermediate-metabolism products
that potentiate aminoglycoside antibiotics in bacterial infections (U.S. application number, 61,871,554). Dr. Mark reports
providing expert testimony in litigation regarding asbestos
exposure. No other potential conflict of interest relevant to
this article was reported.
Disclosure forms provided by the authors are available with
the full text of this article at NEJM.org.
We thank Drs. Bernard Kinane, Mark Pasternack, and Jessica
Rosenthal for assistance with preparing the case history.

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