pediatric case chest-wall mass and a pleural effusion

8/18/2019 Pediatric case Chest-Wall Mass and a Pleural Effusion

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n engl j med 373;7 nejm.org August 13, 2015 657

T h e n e w e n g l a n d j o u r n a l o f medicine

Presentation of Case

Dr. Jessica M. Rosenthal (Pediatrics): An 8-year-old girl who had required long-termtracheostomy because of airway stenosis caused by an injury in infancy was admittedto 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 emergencytracheostomy after a caustic injury from either ingestion or inhalation. At 2.5 yearsof 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 pediatricianreferred 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 ob-structed; 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 pas-sage was normal beyond the stenosis; the subglottic airway was not evaluated.

A tracheostomy was performed, and the left nasal choanal obstruction wasopened with a laser. Repeat bronchoscopy revealed that the larynx and subglottictrachea were normal. On two occasions, esophagogastroduodenoscopy revealed noevidence of gastroesophageal reflux. The tracheostomy tube was removed whenthe patient was 5.5 years of age; there was a residual tracheal–cutaneous fistula.When she was 7.5 years of age (7 months before this admission), another trache-ostomy was performed because of worsening oropharyngeal stenosis that pre-cluded intubation and because of concern about possible obstruction.

The patient had been in her usual state of health until approximately 6.5 weeksbefore this admission; during a 10-day period, her mother noted recurrent blood-tinged sputum in the tracheostomy tube, and an episode of bleeding at the trache-ostomy site occurred at school. The patient was seen in the emergency departmentof the MEEI, where a flexible tracheoscopy, which was performed at the bedsideafter removal of the tracheostomy tube, revealed no exposed blood vessels orgranulation tissue in the trachea or stoma. A chest radiograph showed consolida-tion in the right lower lobe, which was thought to be due to aspiration, pneumo-nia, or atelectasis. Gram’s staining of the sputum showed abundant neutrophils,abundant gram-positive cocci in pairs and a few chains, abundant gram-negativediplococci, 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 — bothin Boston.

N Engl J Med 2015;373:657-67.

DOI: 10.1056/NEJMcpc1400836

Copyright © 2015 Massachusetts Medical Society.

Founded by Richard C. CabotEric S. Rosenberg, M.D., Editor Nancy Lee Harris, M.D., Editor Jo-Anne O. Shepard, M.D., Associate Editor Alice M. Cort, M.D., Associate Editor Sally H. Ebeling, Assistant Editor Emily K. McDonald, Assistant Editor

Case 25-2015: An 8-Year-Old Girl with aChest-Wall Mass and a Pleural Effusion

Samuel M. Moskowitz, M.D., Randheer Shailam, M.D., and Eugene J. Mark, M.D.

Case Records of the Massachusetts General Hospital

The New England Journal of Medicine

Downloaded from nejm.org by BEATRIZ AYALA ROBLES on March 5, 2016. For personal use only. No other uses without permission.

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n engl j med 373;7 nejm.org August 13, 2015658


of the sputum from the tracheostomy tube grewmethicillin-susceptible Staphylococcus aureus , Morax-ella catarrhalis , Haemophilus influenzae , and Strepto-coccus pneumoniae . The patient remained afebrile,and no antibiotic agents were administered. She was admitted overnight for observation and dis-charged home the next day (5 weeks before this

admission). She had one more episode of blood-tinged sputum thereafter.

During the 2 weeks before this admission, theoxygen saturation, which was obtained duringcontinuous overnight monitoring for sleep apnea,fell to approximately 85% (from the usual levelof approximately 95%) for prolonged periods.Examination at school revealed decreased breathsounds at the base of the right lung. One day be-fore this admission, while the patient was beingbathed, her mother noticed that her chest wasasymmetric, with the right nipple appearing lowerthan the left, and that blood vessels were prom-inent on the right side of the chest.

At the pediatrician’s office the next day, thepatient described a recent episode of discomfortin her chest that occurred while she was beinghugged. There was no history of respiratory dis-tress, fevers, or cough. On examination, she wastearful and hesitant. The temperature was 36.1°C,and a tracheostomy tube was in place. The rightlateral chest wall was more prominent than theleft, had no crepitus, and was tender to touch.

Breath sounds were normal on the left side anddiminished on the right. The right upper quad-rant of the abdomen was diffusely tender, and theremainder of the examination was normal. Thepatient was referred to another hospital for chestradiography, which revealed a large pleural effu-sion on the right side, with underlying pneumo-nia. The patient was referred to the emergencydepartment of this hospital.

The patient had sleep apnea and a history ofdental caries that necessitated the extraction ofseveral deciduous teeth between 5 and 7 years

of age. Immunizations, including the 7-valentpneumococcal conjugate vaccine (PCV7), werecurrent; she had reportedly received the bacilleCalmette–Guérin (BCG) vaccine at 1 month ofage. A purified protein derivative (PPD) skin test was negative on her arrival in the United States.She took no medications and had no known aller-gies. She lived at home with her adoptive parents

and siblings; she was physically active, did well inschool, and was able to breathe, speak, and eatnormally, with the tracheostomy tube capped whileshe was awake. She lived with two dogs and a cat,and the mother smoked outside the home; shehad not recently traveled or had contact with illpersons.

On examination, the temperature was 37.0°C,the blood pressure 102/68 mm Hg, the pulse135 beats per minute, the respiratory rate 16 to30 breaths per minute, and the oxygen saturation95% while the patient was breathing ambient air.The oropharynx was clear, without erythema orfocal lesions. The neck was supple, and the tra-cheostomy site was clean and dry, with an under-lying small cavity. Lymphadenopathy (with nodesmeasuring up to 1 cm in diameter) in the rightanterior cervical chain and a mildly tender rightaxillary lymph node (approximately 1 cm in di-ameter) were present. There were decreasedbreath sounds at the base of the right lung.There was a bulging area (4 cm by 7 cm) on theright lateral chest wall, which was tender onpalpation and had no fluctuance or overlying skinchanges. The hematocrit, red-cell indexes, andanion gap were normal, as were blood levels ofcalcium, magnesium, glucose, albumin, amylase,lipase, uric acid, and lactic dehydrogenase andresults of renal- and liver-function tests; othertest results are shown in Table 1.

Dr. Randheer Shailam: A frontal chest radiographthat had been obtained 7 months before thisadmission showed a tracheostomy tube, clearlungs, 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 rightlower lung zone, obscuring the right hemidia-phragm and right cardiac border, as well as a largepleural effusion on the right side that trackedalong the lateral chest wall, with evidence of locu-lation. The left lung and pleural space were clear.

Computed tomography (CT) of the chest, per-formed on the same day after the administrationof contrast material (Fig. 2), revealed asymmetryof the chest wall that was due to multiple hetero-geneous soft-tissue masses and thickening of thesoft tissue of the right chest wall. Consolidation was present in the right lower lobe and portionsof the right middle lobe. Areas of low attenua-





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tion in the right lower lobe were suggestive of de-creased perfusion and possible necrosis. A largeloculated pleural effusion, irregularly shaped pa-renchymal nodules in the right upper lobe, promi-nent enhancing ipsilateral axillary lymph nodes,and subtle periosteal elevation of the medial as-pect of the right eighth rib were also present.

Dr. Rosenthal: The patient was admitted to thepediatric intensive care unit. The temperature roseto 38.7°C, and acetaminophen was administered, with improvement.

On the second hospital day, a diagnostic pro-cedure was performed.

Differential Diagnosis

Dr. Samuel M. Moskowitz: This 8-year-old girl hadhad a caustic exposure during infancy that resultedin upper-airway obstruction and tracheostomydependence. I suspect that her airway injury mayhave caused mild dysphagia, with an increased riskof tracheal aspiration. She had a history of poororal hygiene. Her immunizations included PCV7and BCG, and a PPD skin test was negative aftershe immigrated to the United States. Six weeksbefore 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 pneu-monia are common in children who have under-

gone tracheostomy, with an annual incidence ofup to 88%.1 The tracheitis abated spontaneously, without antibiotic treatment, whereas the lobarprocess progressed and was accompanied by hy-poxemia, chest tenderness, decreased breathsounds, and pleural effusion but no fever.

On presentation, the patient had tachypnea,prominence and tenderness of the right lateralchest wall, and a tender right axillary lymph node.Laboratory test results showed elevated levels ofacute-phase reactants, such as thrombocytosisand hyperglobulinemia. Imaging studies of the

chest revealed opacification in the right lowerlung zone, a loculated effusion, and an irregularchest-wall mass, with regional lymph-node en-hancement and minimal bony changes.

The chest-wall mass is the most worrisomeclinical feature of this child’s presentation. Anacquired chest-wall deformity or mass can origi-nate in bone or soft tissue; the differential diag-

nosis includes trauma, neoplasm, inflammatoryand infectious processes, or a combination ofthese causes (Table 2). Before the results of im-aging studies are available to rule in or rule outspecific causes, the mass could represent a her-niated right lung, a benign or malignant tumor,a sterile collection of inflammatory cells, or anabscess.

Chest-Wall Trauma

Traumatic disruption of the skeletal compo-nents of the chest wall can result in flail chest, with paradoxical chest-wall motion and com-promised gas exchange; these findings werenot present in this child. Lung herniation is anuncommon chest-wall deformity that causes

VariableReference Range,

Age-Adjusted†On Admission,This Hospital

Hemoglobin (g/dl) 11.5–15.5 11.2

White-cell count (per mm3) 4500–13,500 11,500

Differential count (%)

Neutrophils 33–59 73

Lymphocytes 33–50 20

Monocytes 4–11 5

Eosinophils 0–8 2

Platelet count (per mm3) 150,000–450,000 891,000

Sodium (mmol/liter) 135–145 134

Potassium (mmol/liter) 3.4–4.8 3.8

Chloride (mmol/liter) 100–108 96

Carbon dioxide (mmol/liter) 23.0–31.9 25.1

Protein (g/dl)

Total 6.0–8.3 8.5

Globulin 2.3–4.1 5.0

Phosphorus (mg/dl) 4.5–5.5 4.0

C-reactive protein (mg/liter)


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dyspnea, pain, and a bulge that varies with therespiratory cycle. It usually results from traumabut can also be congenital, arise spontaneouslyafter a coughing paroxysm, or occur in patients with neoplastic, inflammatory, or infectious dis-orders of the chest wall.2 In this child, exami-nation and imaging studies did not reveal thecharacteristic bulge of a herniated lung. With-

out a history of recent trauma, the diagnoses ofhematoma and ruptured hemidiaphragm are alsounlikely.

Benign and Malignant Tumors

of the Chest Wall

Various benign and malignant tumors can arisefrom the bones of the chest wall (Table 2). How-

Figure 1. Chest Radiographs.

Radiographs of the chest were obtained on admission. Frontal and lateral chest radiographs (Panels A and B, re-spectively) show air-space 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 pleuralfluid on the right side is not freely layering (arrows).

A

*

B

DC

*





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ever, in this case, chest CT revealed only a mini-mal rib lesion, and thus a bony tumor is unlikely.Some benign tumors that occur in children, suchas lymphangioma (cystic hygroma) or hemangio-ma, arise in the soft tissue of the chest wall andare generally confined to it, without the pleuraland pulmonary components that were seen in

this case. Other benign tumors originating in softtissue, such as lipoma, are more common inadults than in children. Peripheral-nerve tumorscan be associated with effusion and pleural nod-ules but would be more likely to occur in a patient with known neurofibromatosis. Desmoid tumor,a histologically benign fibromatosis that can be

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 multipleheterogeneous soft-tissue 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).

A B

DC

FE

*

*

*

*





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locally invasive, is more likely to occur in theshoulder region than in the anterolateral chest.3 Benign mesothelioma is rare in children andusually confined to the pleural space.

Patients with an inf lammatory myofibroblas-tic tumor can present with a soft-tissue mass ofthe chest wall. It arises from lung parenchyma andcan extend into the chest wall, with localized masseffect.4 These lesions, which are the most com-

mon lung tumors in children, are typically benignbut can occasionally undergo malignant trans-formation. They are typically associated with dys-pnea, cough, hemoptysis, and fever. Laboratoryevaluation may reveal iron-deficiency anemia,thrombocytosis, and hypergammaglobulinemia.However, chest-wall invasion is inconsistent andgenerally occurs no sooner than 12 to 18 monthsafter the onset of symptoms, pleural effusions areuncommon, and lymphadenopathy is rare. Asso-ciated chest CT reveals a varying nonspecific pat-tern, with heterogeneous enhancement and at-

tenuation.5

The laboratory test results and imagingfindings in this patient were suggestive of an in-flammatory myofibroblastic tumor, but her course was unusually rapid for this lesion and she did nothave several of the usual signs and symptoms.

This child did not have persistent systemicsymptoms suggestive of a malignant tumor; how-

ever, because of the consequences of missing amalignant neoplasm, these lesions deserve con-sideration. In children, rhabdomyosarcoma is themost common malignant tumor arising in thesoft tissue of the chest wall, but the associatedchest CT would typically reveal a discrete mass,4 which is unlike the finding in this child. Othersoft-tissue sarcomas are relatively rare in children.Pleuropulmonary blastoma arises from the pleu-

ra or lung and can invade the chest wall, but itusually occurs in children 5 years of age or young-er. Malignant fibrous histiocytoma is usuallyconfined to the chest wall and is more commonin elderly persons than in children.

Lymphomas in the thorax typically arise inthe mediastinum and can extend into the anteriorchest wall, and thus a locally invasive lymphomaneeds to be considered in this case. Patients withthoracic lymphoma typically present with chestpain, dyspnea, and lymphadenopathy, sometimes without fever. Early-stage Hodgkin’s or non-Hodg-

kin’s lymphoma that arises in the mediastinumcan invade the lung parenchyma and chest wall,but this invasion usually is contiguous with atumor in the anterior mediastinum and does notinvolve the lower lateral chest (the location ofthe chest-wall lesion seen in this child).6,7 In ad-dition, lymphomatous chest-wall invasion usu-

Diagnosis Originates in or Affects Bone Originates in or Affects Soft Tissue

Trauma 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 (Ewing’s sar-coma, Askin’s 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), Friedrich’s disease (asepticnecrosis of the sternoclavicular joint)

Sebaceous cyst

Infectious process Osteomyelitis (tuberculosis, actinomycosis), septicarthritis

Abscess, phlegmon, cellulitis, empyema necessitatis

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





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ally develops more slowly than did the processin this child.

In summary, a neoplastic process cannot beruled out in this case, but an inflammatory orinfectious 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 multifocalosteomyelitis, can cause a chest-wall mass. Vari-ous pathogens can cause osteomyelitis or septicarthritis with localized chest-wall swelling andinduration. However, the localization and imag-ing 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 remainconfined to the chest wall and would not invadethe pleura or lung. Soft-tissue infections of thechest wall can be manifested by a solitary ab-scess or cellulitis, with associated inflammationof adjacent tissues. However, several findings inthis child were consistent with an infection origi-nating in the chest cavity and extending into thechest wall.

Empyema Necessitatis

The clinical and radiographic features seen inthis case are most consistent with empyema ne-

cessitatis, a process characterized by extension ofpleural empyema into the chest wall. This condi-tion was first described by Gullan de Baillon in1640. Empyema necessitatis typically developsover a period of 4 to 8 weeks and is associated with pain, swelling, and lymphadenopathy of theanterolateral chest, often without fever; thesefeatures were seen in this case. It can also resultin a pleurocutaneous or bronchopleurocutaneousfistula, which was not seen in this case. RenéLaennec succinctly described its pathogenesis in1819 as follows: “When, as the result of chronic

pleurisy, a gangrenous eschar forms on the pleura,it sometimes happens that the effusion infiltratesthrough the intercostal muscles at this point, andcomes to form under the skin an abscess whosenatural or artificial opening provides, in a fewrare cases, a healing of the empyema. These spe-cies of abscess, known since the origin of the

art, have been observed from time to time by thesurgeons, and their opening constitutes what iscommonly called the empyema of necessity. Thiscase is also very rare; my friend Mr. Recamiertold me that he has observed twice; I have en-countered it but once.”8

The patient in Laennec’s case is said to have

been a 12-year-old boy. In 1869, William Mooredescribed empyema necessitatis in a 10-year-oldgirl.9 The publication of only a few relevant casereports since the 19th century indicates that thiscondition remains extremely rare in children;nonetheless, I believe that this child had empy-ema necessitatis.

Empyema necessitatis was more common inthe era before antibiotics. At that time, it was as-sociated with an overall mortality of approximately66%; the most common pathogens were Myco-bacterium tuberculosis (mortality, 87%) and S. pneu-moniae (mortality, 28%). Since antibiotics have beenin use, cases of empyema necessitatis are rarelyfatal, and actinomyces species are a more commoncause than is S. pneumoniae . Less common patho-gens include S. aureus , S. milleri, Fusobacterium nuclea-tum, M. avium, M. intracellulare , Burkholderia cepacia,blastomyces species, and Nocardia asteroides .10

Given this child’s history of BCG immuniza-tion and the negative PPD skin test, tuberculosisis 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 inapproximately 50% of children with uncompli-cated empyema, and other pathogens are muchless common in these patients.11 However, thepatient’s history of pneumococcal vaccinationmakes this pathogen unlikely. Other aerobicbacteria in her tracheal secretions could causepneumonia1; of these, only S. aureus has beenreported in patients with empyema necessitatis.Both S. pneumoniae and S. aureus would be expectedto cause fever, which was not seen in this case.

Poor oral hygiene promotes growth of bacteria

that can cause pneumonia,12

including aerobessuch as S. pneumoniae but also anaerobes that can-not be detected on a routine culture of trachealsecretions. Chief among anaerobes that couldcause empyema necessitatis is A. israelii. Additionalfactors that increase the likelihood of pulmonaryactinomycosis in this case include the risk of aspi-





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ration, subacute course, and absence of fever.Pulmonary actinomycosis is often polymicrobi-al, with gram-positive bacteria or anaerobes ascommon coisolates,13 and its parenchymal com-ponent may cavitate. Small-to-medium-sized pleu-ral effusions may occur, and chest-wall invasionmay result in early rib erosion, which was seen

in this child. I suspect that an actinomyces spe-cies was her primary pathogen.

If a neoplasm were likely in this case, I mightsuggest the use of positron-emission tomogra-phy (PET) or magnetic resonance imaging (MRI)to delineate the tumor burden or ultrasound-guided or CT-guided percutaneous aspiration toobtain a tissue sample. In cases of infection orinflammation that is confined to the pleural andparenchymal compartments, my preferred ap-proach would be video-assisted thoracoscopicsurgery to facilitate pleural drainage and decor-tication and to obtain a lung-biopsy specimen,as indicated. For this chest-wall lesion, I wouldfavor exploratory thoracotomy to obtain a biopsyspecimen and to permit open débridement orresection.

Dr. Nancy Lee Harris (Pathology): Dr. Ryan, would you tell us the thinking of the clinicalteam and describe the diagnostic procedure?

Dr. Daniel P. Ryan (Pediatric Surgery): We were very concerned that the multifocal nodular pleu-ral lesions and the large lump on her chest wall

— which was not fluctuant or warm and did nothave typical signs of infection — represented aneoplastic process. We brought her to the oper-ating room to perform a biopsy of the mass andto place a thoracostomy tube to drain the pleuralfluid. We made a small incision over the chest- wall mass. There was no subcutaneous edema,and the mass itself was somewhat gray in ap-pearance. We performed an incisional biopsy, which released yellow pus that contained small, white, solid granules (≤1 mm in size). Then, weinserted a drain in the mass. We located the

pleural effusion and placed a chest tube, whichdrained clear fluid. At that point, we decided notto perform thoracoscopy.

Clinical Diagnosis

Possible malignant tumor of the chest wall orpleura.

Dr. Sa muel M. Moskowitz’s

Diagnosis

Empyema necessitatis, with pneumonia of theright lower lobe and empyema of the right pleuralcavity caused by actinomyces species.

Pathological Discussion

Dr. Eugene J. Mark: The diagnostic procedure wasa biopsy of the soft-tissue mass of the chest wall.Histopathological examination revealed an ab-scess with purulent inflammation (Fig. 3A), sur-rounded by granulomatous inflammation andfibrosis (Fig. 3B). Staining with hematoxylin andeosin revealed a sulfur granule in the abscess(Fig. 3A). Filamentous structures were visible atthe periphery of the sulfur granule; on silverstaining, the filamentous structures could beseen both creating the sulfur granule (Fig. 3C)and extending as bacillary organisms from theperiphery of the sulfur granule (Fig. 3D). Thesemorphologic f indings are typical of actinomycesspecies.

Both actinomyces species and nocardia spe-cies are filamentous bacteria, with branchingsegments that often vary as positive or negativeon Gram’s staining. Actinomyces are found inthe sulfur granules and not in the surroundinginflammation.

Cultures of the tissue and abscess specimensgrew A. israelii and Aggregatibacter (formerly Acti-nobacillus ) actinomycetemcomitans (Fig. 3E and 3F).Aggregatibacter is a gram-negative facultativenonmotile rod that is frequently isolated together with actinomyces. It is an oral commensal organ-ism often found in association with localizedaggressive periodontitis.14-16

It is not clear to me in this case whether thepleuropulmonary infection is due to aspirationfrom the oral cavity or whether the trachea orthe soft tissue around the trachea is infected

with actinomyces. The closest analogy that Ihave found is involvement of tissue around theinnominate artery that results in a pseudoaneu-rysm.17

Dr. Harris: Dr. Pasternack, would you tell us how you treated the patient and how she is doing?

Dr. Mark Pasternack (Pediatric Infectious Dis-eases): This patient had a classic presentation of





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thoracic actinomycosis. The findings in the softtissue of the chest wall, indolent clinical pro-gression, absence of fever, signs and symptoms

of acute infection, and radiologic evidence of anapparent thoracic mass led to the initial consid-eration of a malignant process. The diagnosis of

Figure 3. Chest-Wall–Biopsy 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 histiocyticinflammation (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). Gram’s staining of smears of the cultures shows gram-positive

rods, which were identified as Actinomyces is raelii (Panel E), and gram-negative rods identified as Aggregatibacter

actinomycetemcomitans (Panel F). (Panels E and F are courtesy of Dr. JiYeon Kim [Pathology].)

A B

DC

FE





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infection was established only at the time ofsurgical exploration.

Dr. Rosenthal and I were consulted postop-eratively, after the Gram’s stain of the chest-wallabscess revealed pleomorphic gram-positive rods.In many cases of actinomycosis, cultures remainnegative, perhaps because of previous adminis-

tration of antibiotics or exposure of the speci-men to air. However, in this case, the culturegrew not only A. israelii but also the fastidious oralgram-negative pathogen A. actinomycetemcomitans .

Since the child was known to have virulentpathogens in preoperative respiratory secretions,including methicillin-susceptible S. aureus andPseudomonas aeruginosa (in a culture that was ob-tained on the day of admission), we initiatedsingle-agent parenteral antibiotic therapy withimipenem. A peripherally inserted central cath-eter was placed for the administration of intravenous antibiotic therapy at home. After nearly5 weeks of therapy, acute fevers developed, withtemperatures exceeding 40°C. The patient wasreadmitted to this hospital. Blood cultures werenegative, and follow-up CT showed resolution ofthe chest-wall lesions, with residual pleural thick-ening and improving consolidation in the rightlower lobe. Her fever persisted despite discontinu-ation of imipenem and initiation of ceftriaxonetherapy. She defervesced after her therapy wastransitioned to oral doxycycline and she under-

went removal of the catheter.The patient had a favorable response to thedoxycycline therapy and received a 1-year courseof therapy. Three years later, she is currently well,and she and her family are considering recon-structive surgery that might permit removal of thetracheostomy tube.

Dr. Harris: Are there questions or commentsfor any of our discussants? Dr. Moskowitz, do youhave any comments?

Dr. Moskowitz: I was concerned that there wasa polymicrobial component to this infection, but

I thought the discussion had gone far enough with actinomyces and elected not to delve intosix-syllable coinfectants.

A Physician: Because of the upper-airway ab-normalities, is this patient at risk for a recur-rence of this disease?

Dr. Pasternack: This is clearly a very rare com-

plication of tracheostomy, and one would have tothink, as Dr. Moskowitz has suggested, that as-piration led to this complication. The patient cer-tainly might be at risk for reaspiration.

Dr. Harris: In retrospect, do you think that theepisodes of bleeding that occurred 6 weeks be-fore admission, with the finding of consolidationin the right lower lobe on imaging studies, rep-resented 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 theright lower lobe was reasonable. The upper-airway symptoms improved, so the imaging findingin the lung was thought to not be related and was not evaluated at follow-up.

Anatomical Diagnosis

Infection of the chest wall (empyema necessitatis) with Actinomyces israelii and Aggregatibacter actino-mycetemcomitans .

This case was discussed at Pediatric Grand Rounds.Dr. Moskowitz reports receiving fees for serving on advisory

boards from Vertex Pharmaceuticals; consulting fees fromEnBiotix, MCIC Vermont, Kala Pharmaceuticals, Pfizer, andCowen Group; and grant support from Gilead Sciences, Novar-tis, Rempex Pharmaceuticals, Kala Pharmaceuticals, VertexPharmaceuticals, and Savara Pharmaceuticals; as well as hold-ing a pending patent for intermediate-metabolism productsthat potentiate aminoglycoside antibiotics in bacterial infec-tions (U.S. application number, 61,871,554). Dr. Mark reportsproviding expert testimony in litigation regarding asbestosexposure. No other potential conflict of interest relevant tothis article was reported.

Disclosure forms provided by the authors are available withthe full text of this article at NEJM.org.

We thank Drs. Bernard Kinane, Mark Pasternack, and JessicaRosenthal for assistance with preparing the case history.

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open technique. Interact CardiovascThorac Surg 2008;7:506-7.3. O’Sullivan P, O’Dwyer H, Flint J,Munk PL, Muller N. Soft tissue tumoursand mass-like lesions of the chest wall: apictorial review of CT and MR findings.Br J Radiol 2007;80:574-80.4. Baez JC, Lee EY, Restrepo R, Eisenberg

RL. Chest wall lesions in children. AJR Am J Roentgenol 2013;200:W402-W419.5. Narla LD, Newman B, SpottswoodSS, Narla S, Kolli R. Inflammatorypseudotumor. Radiographics 2003;23:719-29.6. Hodgson DC, Tsang RW, Pintilie M,et al. Impact of chest wall and lung inva-





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ated with the highly leukotoxic clone of Actinobacillus actinomyce temcomit ans . J DentRes 2001;80:1580-3.15. Slots J. Update on Actinobacillus actino-mycetemcomitans and Porphyromonas gingi-valis in human periodontal disease. J IntAcad Periodontol 1999;1:121-6.16. Slots J, Ting M. Actinobacillus actino-mycetemcomitans and Porphyromonas gingi-

valis in human periodontal disease: oc-currence and treatment. Periodontol2000 1999;20:82-121.17. Wani NA, Rawa IA, Pala NA, Kosar T.Pseudoaneurysm of internal mammaryartery caused by pulmonary actinomyco-sis. Br J Radiol 2010;83(995):e235-e238.Copyright © 2015 Massachusetts Medical Society.

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