Clinical Pathologic Conference

R1 曾敏華 / 洪志興主治醫師

Chief complaint

A 5 year-old boy was admitted because of fever off and on with chillness, cough with whitish sputum and chest pain for 3 days.

Present illness

About 1 week before this episode (10 days before arrival), he suffered from several times of watery diarrhea.

He had chest wall injury while he was playing with his friend.

3 days before admission, fever off and on with chillness, cough with whitish sputum and chest pain were noted.

Present illness

The pattern of chest pain: -No relieve or aggressive factor -No radiating pain. -Did not improved despite of medication. 1 day before admission, dyspnea after climbing

upstairs and palpitation was also noted. He was brought to ER on the day of admission, and

short of breath, pale lips, pale conjuntivae were found and vomiting for 3 times at ER.

Present illness

Throughout the whole course of present illness, he had no skin rash, headache, abdominal discomfort and urinary change.

His appetite and activity were good.

Past,Personal and Family History

Past history -Allergic rhinitis

-Hydronephrosis, left.

-Asthmatic bronchitis

-Urethritis

-Acute gastritis

Past,Personal and Family History

Birth history:

-GA: 38 weeks via C/S, Birth weight: 3700 gm Developmental History: normal No travel history. Family history: Grandfather: had been working at mine,

( 礦物局 ) driver.

: No any family member has the same

symptoms like him.

Physical examination

Vital sign: PR: ↑ 132 /min (65-110), RR: ↑ 36 /min(20-25), BT: 37 ºC

HEENT: Pale conjunctiva/ lip , grade 1/4 of tonsils,

: congested throat Chest wall: A tender mass 2 × 3-cm in size at his fore-

chest wall.

: Coarse breathing sound

Lab. data

0905(admission)B/R: -WBC:14,620 (5,5-15,5 x10 3)

-RBC: 4.72 x10 6 (3.9-5.3 x10 6 ) , Hct:33.4% -Hgb: 11.3 ( 6mo-6 yr: 10.5 - 14) Nelson 17 ed Table 439-1

-MCV: 70.9 (6 mo-6 yr: 70-74) Nelson 17 ed Table 439-1

-MCH: 24 (24-30)

-MCHC: 33.8(31-37)

-PLT: 244 x10 3 (150-450 x10 3 )

-Neut. ↑ 77.1% (54-62%); LYM.:25% (25-33%); Mon.:4.9 %(3-7%); Eos.:0.5 %(1-3%)

Lab. data

0907(hospitalization)B/R: -WBC:10,380 (5,5-15,5 x10 3)

-RBC: 4.65 x10 6 (3.9-5.3 x10 6 ), Hct:33.4% -Hgb: 11.0 ( 6mo-6 yr: 10.5 - 14) Nelson 17 ed Table 439-1

-MCV: 69.7 (6 mo-6 yr: 70-74) Nelson 17 ed Table 439-1

-MCH: 23.7 (24-30)

-MCHC: 34(31-37)

-PLT: 234 x10 3 (150-450 x10 3 )

-Neut. ↑ 65 % (54-62%); LYM.:25% (25-33%); Mon.:8 %(3-7%); Eos.:2 %(1-3%)

Lab. data

Mycoplasma AB: < 40 (-) CRP: ↑2.93 (0.17-1.07) BUN / Cr: 10 / 0.4 CPK: 50 U/L(5-110); CK-MB: 1 U/L: 2 %(1.7-7.9 %) Glucose: 158 mg/dL Na/ K/ Cl: 136/↓3.4/ 100 Total Ca: 9.3

Lab. data

Urine routine:

- pH: 6.0

-Protein: +/-

-RBC: 0-1/ HPF

-WBC: ↑ 5-10/ HPF (Children 0–5/ hpf )

-Spgr: 1.017

Hospital course :

After admission, CxR was taken, and draws blood samples.

He was discharged to home after a series of studies and treatment.

Problem list

Major problems: Chest pain Short of breath/

dyspnea Chest wall mass Fever, cough Vomiting Diarrhea

Minor problem Pale conjuntivae/ lip Hyperglycemia Pyuria (U/R) ?

Questions?

Correlation between chest injury and chest pain/ chest mass? Chest mass location ? Rib, costochondritic joint, intercostal muscle

or sternum? Chest wall mass: Mobile or immobile? Warm/ heat? fluctuant ? Chest pain: sudden onset or progressive? Needle aspiration (culture)? Mass biopsy ? Viral culture (throat)? CxR ? Patchy infiltration? Hilar lymphoadenopathy? Body weight loss? Night sweating? FHx of TB? Medication Hx? EKG ? Sputum culture?

Chest pain: possible differential diagnosis

Infectious costochondritis: Tietze syndrome Pleurodynia Pneumonia: Pyogenic Empyema Necessitatis Musculoskeletal chest wall pain: Osteomyelitis

Dyspnea: possible mechanism Pediatric Respiratory Medicine P370

↓Pulmonary compliance: Inflammation ↑Airway Resistance:

Airway inflammation & hyperreactivity ↑Sensory Stimuli: Chest wall ↑Chemical Stimuli: Hypoxemia, CO2, Acidosis

Chest wall lesions in childhood

A wide range of pathologies. I. Benign lesions: include a. Lipoma /Lipoblastoma: usually painless, occur in the first 2 years b. Neurofibroma: Painless, associated with neurofibrosis type I c. Lymphangioma: Painless,associated with a variety of other syndromes such as asplenia,Noonan’s syndrome, and tuberculosis.

Chest wall lesions in childhood

d. Haemangioma and AVM

: congenital lesions.

e. Mesenchymal hamartoma

: benign lesion of infancy with most lesions presenting

before 2 years of age.

Chest wall lesions in childhood

II. Malignant lesions: include a. Neuroblastoma: occur before 5 years of age, abdominal mass b. Rhabdomyosarcoma:common found in head/ neck or extremities c. Ewing’s sarcoma, Askin tumour, Primitive neuroectodermal tumours: : all belong to the group of small round blue cell tumor of childhood. : Usually presenting in the second decade of life.

Chest wall lesions in childhood

II. Manifestations of systemic diseases:

e. Leukaemia/ Lymphoma

: always be excluded in a child

: presenting with multiple bone lesions or

bone pain, hematologic finding.

f. Langerhans cell histocytosis

Chest wall lesions in childhood

III. Infections a. Tuberculosis: -TB on the chest wall can occur as an isolated primary infection with no evidence of pulmonary disease - Spread to the chest wall from pulmonary infection may also occur althrough symptoms may be minor - Presentation may be similar to pyogenic abscess or simply an enlarging mass.

Chest wall lesions in childhood

b. Actinomycosis : rare, may mimic TB with pulmonary

infiltration and spread to the chest wall

c. Aspergilosis: in immunocompromised patients, usually

by local invasion from the lung.

d. Progenic abscess

: Staphylococcus, Klebsiella infection

e. Other infection illness: Strongyloidiasis (Strongyloides

stercoralis) 糞小桿線蟲

Chest wall lesions in childhood

IV. Other lesions:

a. Pseudo-lesions: found in cildren of early teenage

years presenting with a hard lump

in costochondral cartilage.

: Usually no specific symptoms.

b. Thoracic cage anomalies,osteochondroma,

hemangiopericytoma

Chest wall mass: possibile DDx

Tuberculosis Actinomycosis Pyogenic abscess Strongyloidiasis (Strongyloides stercoralis)

Chest painInfectious costochondritis:

Tietz syndromePleurodynia Pneumonia- Pyogenic Empyema

NecessitatisOsteomyelitis

Chest massTuberculosisActinomycosisPyogenic abscessStrongyloidiasis

Dyspnea↑Airway Resistance:

Airway inflammation &

hyperreactivity↓Pulmonary compliance:

Inflammation

Differential diagnosis

↑Sensory Stimuli:

Chest wall↑Chemical Stimuli:

Hypoxemia, CO2, Acidosis

1. Tietze syndrome: infectious costochondritis

Inflammation of the costal cartilages (Costochondritis) with a large, tender, fusiform (spindle-shaped), nonsuppurative swelling at the costochondral or chondrosternal junction.

Principles and Practice of Pediatric Infectious Diseases, 2nd ed

Etiology:

-Exact cause unclear

-Most often idiopathic

-Associated with excessive exercise, chest injury or a preceding viral respiratory illness (postinfectious)

1. Tietze syndrome

Epidemiology -Generally occurs in late teens to early twenties, but has been

described in young children. S/s: sharp pain in anterior chest wall, may radiate to back or upper abdomen. : varies in intensity and quality. PE: Tender by palpation at local site or movements of the arm or shoulder. :May show evidence of tachypnea, tachycardia or systolic HTN. : Distortion of local soft tissue.

1. Tietze syndrome

Diagnostic workup -Made with historical and physical clues. -Biopsy: increased cartilage and lack of inflammatory fibrosis. Management: NSAIDs, Injections into the painful area with triamcinolone

are sometimes curative.Question: Fever ? preceding viral respiratory illness?

2. Pleurodynia Nelson 17ed p1045, Principles and Practice of Pediatric Infectious Diseases, 2nd ed P1183

Etiologic agents are most frequently coxsackieviruses B3 and B5, as well as coxsackieviruses B1 and B2 and echoviruses 1 and 6. Nelson 17ed p1045

Sex: Males are affected more than females. Half of these patients are younger than 5 years, and 30% are younger than 1 year.

Coxsackievirus B usually causes an asymptomatic or brief upper respiratory tract or gastroenteric infection. Nelson 17ed p1045

The virus has an incubation time of 1 week in the GI tract and subsequently involves the target organs through hematogenous dissemination.

2. Pleurodynia Nelson 17ed p1045, Principles and Practice of Pediatric Infectious Diseases, 2nd ed P1183

Prodromal symptoms such as malaise, myalgias, headache and diarrhea are followed by sudden onset of fever and pain.

Principles and Practice of Pediatric Infectious Diseases, 2nd ed P1183

Involvement of the intercostal and other muscles of respiration results in enhanced pain with deep breathing and a sensation of “pleuritic” pain that can result in splinting of the chest and rapid, shallow breathing.

Principles and Practice of Pediatric Infectious Diseases, 2nd ed P1183

Pain, typically located in the chest or upper abdomen, and is spasmodic and can be excruciatingly severe. Nelson 17ed p1045

During spasms, respirations are usually rapid, shallow, and grunting, pale, sweaty, and shock-like appearance.Nelson 17ed p1045

2. Pleurodynia Nelson 17ed p1045, Principles and Practice of Pediatric Infectious Diseases, 2nd ed P1183

Causes necrosis of the striated intercostal muscles in patients with pleurodynia.

Muscle tenderness can be detected by direct palpation; less commonly, localized swelling is observed ( Sylvest, 1934 )

Associated symptoms related to the viral infection may include the following:

-Upper respiratory tract symptoms, including sore throat, rhinitis,

and cough. -Constitutional symptoms, including headaches (50%), fever, and malaise

2. Pleurodynia Nelson 17ed p1045, Principles and Practice of Pediatric Infectious Diseases, 2nd ed P1183

-GI symptoms, including nausea, vomiting, diarrhea (50%), and in children, abdominal pain (usually in the epigastric area)

Diagnosis: -Viral cultures(throat) isolation of coxsackievirus B in cell cultures . -RT-PCR(throat) -Histologic Findings: Necrosis of the striated intercostal muscles is visible . -Serum creatine kinase is usually elevated because of muscle necrosis. -CxR: reveals clear lung fields; less than 10% of patients have a pleural friction

rub or pleural fluid evident on chest radiographs.

2. Pleurodynia Nelson 17ed p1045, Principles and Practice of Pediatric Infectious Diseases, 2nd ed P1183

Illness usually lasts 3–6 days, but can last up to a couple of weeks.

Illness may occasionally be associated with meningitis, orchitis, myocarditis, or pericarditis.

Management: -Analgesics and limitation of physical activity are helpful in

reducing pain, which is generally most severe on presentation and then progressively diminishes during the course of illness (which is typically 4 to 6 days but which may last as long as 3 weeks) ( Warin et al., 1953)

Question: Serum creatine kinase within normal range

3. Tuberculosis Principles and Practice of Pediatric Infectious Diseases, 2nd ed

I. Bony tuberculosis Osteomyelitis is otherwise most frequently found in the

metaphysis of long bones, although the ribs, pelvis and skull may be infected.

In children in countries in which TB is prevalent, osteomyelitis is a significant cause of crippling deformity.

Trauma does not predispose to tuberculous osteomyelitis

3. Tuberculosis Principles and Practice of Pediatric Infectious Diseases, 2nd ed

Bony TB may be accompanied by sinus tracts or soft tissue masses.

Diagnosis:

-May be difficult because lesions can appear osteolytic or sclerotic on radiography.

-Biopsy : acid-fast bacilli (AFB) stains, culture or PCR

3. Tuberculosis Principles and Practice of Pediatric Infectious Diseases, 2nd ed

II. Tuberculous abscesses:  May form in most soft tissues, including muscle . This is usually secondary to contiguous spread of infection but

may follow hematogenous dissemination. The classic abscess site is in the psoas muscle, and such an

abscess can occur with or without localizing signs Cough, diarrhea and vomiting can be happened, but rare and

chronic when the pulmonary and gastointestinal were involved. Question: Contact history? insidious enlarging mass? Chronic steroid usage? Body weight loss? Night weating?

4. Actinomycosis Nelson Textbook of Pediatrics, 17th

Actinomyces israelii , a slow-growing, gram-positive bacteria that are part of the normal oral flora in humans.

Infection caused by these bacteria is termed actinomycosis, which is a chronic, granulomatous, suppurative disease characterized by peripheral spread with extension to contiguous tissue in the formation of numerous draining sinus tracts.

7% of the patients <10 yr of age. The youngest patient in this series was 28 days old.

4. Actinomycosis Nelson Textbook of Pediatrics, 17th

Actinomycosis is usually not an opportunistic infection, disease has been described in patients receiving corticosteroids and those with leukemia, renal failure, congenital immunodeficiency diseases, and HIV infection

three important sites of Actinomyces infection in order of frequency are cervicofacial, abdominal, and thoracic

Pulmonary actinomycosis may present as fever, productive cough, chest pain, and weight loss.

Infection frequently dissects along tissue planes and may extend through the chest wall or diaphragm, producing numerous sinuses.

4. Actinomycosis Nelson Textbook of Pediatrics, 17th

Diagnosis : confirmed by examining purulent sinus tract drainage for sulfur granules, and by appropriate cultures.

The differential diagnosis of pulmonary actinomycosis includes lung abscess and tuberculosis.

Tx: mainstay of treatment for actinomycosis is prolonged antibiotic therapy (Penicillin) and an appropriate surgical approach to sinus tracts and abscesses.

4. Actinomycosis Nelson Textbook of Pediatrics, 17th

Question: Chronic process, underlying

immunocompromise status; diarrhea? Vomiting?

5. Pneumonia- Pyogenic Empyema Necessitatis

Pyogenic Empyema Necessitatis:

A rare complication of empyema; is a collection of inflammatory tissue that usually extends directly from the pleural cavity into the thoracic wall forming a mass in the extrapleural soft tissues.

The hallmark of empyema necessitatis is a mass The most common etiologies are tuberculosis and

actinomycosis.

5. Pneumonia- Pyogenic Empyema Necessitatis

1-year-old boy with Streptococcus pneumoniae empyema necessitatis and review previously reported . Pediatr Infect Dis J - 01-FEB-2004; 23(2): 177-9

Symptoms: as the underlying disease, fever, chest pain, chest wall mass,cough and short of breath may be occur. Pediatr Infect Dis J - 01-FEB-2004; 23(2): 177-9

The mass might be painful and fluctuant.

5. Pneumonia- Pyogenic Empyema Necessitatis

Diagnosis: -Chest radiograph: Pleural effusion, with thickening of the pleura at the involved site, may be an enlargement of the overlying ribs with a thick, calcified pleural rind. -CT scan: may show a thick-walled, possibly well-encapsulated, pleural mass that protrudes through the chest wall.(Virtually diagnostic of empyema necessitatis)

5. Pneumonia- Pyogenic Empyema Necessitatis

Questions: Rare chronic complication of pneumonia,

decreased breath sounds, dull percussion on

physical examination, vomiting, diarrhea?

6. Pyogenic abscess

Pyogenic abscesses involving Staphylococcus or Klebsiella infection of the chest wall may also occur and may be due to central line or catheter-related sepsis in immunocompromise.

Large multifocal lesions may develop . Question: diarrhea, vomiting? Cough? No predisposing

factor? Not multifocal mass?

7. Osteomyelitis: Rib or sternum Nelson 17ed P2297

Most cmmon in young children, more common in boy than girl(2:1)

The majority of infection in otherwise healthy children are hematogeneous origin.

Long bone is principlly involved in osteomyelitis (2.9% in chest bones)

Blunt trauma, bacteremia, illness, malnutrition, immune system deficiency have been described as predisposing conditions.

7. Osteomyelitis Etiology: S. aureus is the most frequently isolated

pathogen excluding patients with hemoglobinopathies, such as SCD, where Salmonella sp. is more likely .

Primary viral infections of bone are exceedingly rare, but accompanied viral syndrome, suggesting immune-mediated pathogenesis. Nelson 17ed P2298

- Extremely rarely, the viruses which cause chickenpox and smallpox have been found to cause a viral osteomyelitis.

7. Osteomyelitis

Clinical features may including chills, fever,chest pain,tenderness,swelling and erythema over the involved bone. Scand J Infect Dis 1999 ;31: 98–100.

Insidious onset of nonspecific symptoms associated with gradual painful swelling over the bone area.

Pediatr Infect Dis J 2001; 20: 547–50

7. Osteomyelitis

Diagosis:

-CBC: leukocytosis with left shift, and a thrombocytosis may be present.Pediatric Clinics of North America 43: 933-947, 1996

-ESR and CRP are the nonspecific sign of inflammation.

-Culture taken from the bone, either surgically or by needle aspiation.

-CT or Three phases bone scan Question: More insidious process? Chickenpox? Smallpox?

8. Strongyloidiasis (Strongyloides stercoralis)

8. Strongyloidosis (Strongyloides stercoralis)

Pulmonary disease secondary to larval migration through the lung rarely occurs and

may resemble Löffler's syndrome (cough, wheezing, shortness of breath, and transient pulmonary infiltrates

accompanied by eosinophilia). Nelson 17ed P1160

Clinical manifestations of strongyloidiasis in southern Taiwan. J Microbiol Immunol Infect - 01-MAR-2002; 35(1): 29-36

The most frequent clinical findings were diarrhea (74%), fever (70%), abdominal pain (59%), cough (37%), dyspnea (33%), and constipation (26%).

The common initial laboratory abnormalities were leukocytosis (81%), anemia (67%), liver function impairment (52%), and eosinophilia (44%).

Most of the patients had comorbid conditions, including malnutrition in 20 (74%), corticosteroid dependence in 15 (55%), chronic obstructive pulmonary disease in 9 (33%), chronic liver disease or cirrhosis in 8 (30%), and peptic ulcer disease in 7 (26%).

8. Strongyloidosis (Strongyloides stercoralis)

Question:, no pruritus, absence of anemia, no

peripheral hypereosinophilia, abdominal pain

(common), chest pain (rare)

Final diagnosis

1. Pleurodynia (Dx. Procedure:viral culture, histiologic examination by biopy)

2. Tietze syndrome (Dx. Procedure: Biopsy; PE: Location)

3. Primary viral infectious osteomyelitis. (Dx. Procedure: aspiration culture/ virus isolation, CT, bone scan)

4.Tuberculous abscesses (Dx. Procedure: AFS, PCR or culture)

1. Chest pain

Tender mass on fore-chest. Erythematous change ? Warm sensation? Precipitating factors: trauma. Acute respiratory

infection (cough, fever).

exrcise? Weight lifting ?foreign body ingestion?caustic

ingestion? Underlying illness: Kawasaki disease, CHD?

Family Hx of cardiac disease? Peptic ulcer disease?

or rheumatoid disease?

1. Chest pain Cardiac involvement: - Palpitation, dyspnea after climbing upstair -Risk factor: * FHx of myocardial infarction or hypercholesterolemia (-) * CHD (-)or acquired heart disease * Syncope (-) * Pain with exercise (-) * Hx of Kawasaki disease(-)

1. Chest pain

Cardiac involvement:

-Physical finding:

Heart murmur(-), Persistent tachycardia(-),

increased intensity of S2 (-), gallop rhythm(-),

friction rub(-), or decreased femoral pulses(-).

1. Chest pain

Cardiac involvement: -Examination:

*EKG: Ventricular hypertrophy? ST-Twave change?

Arrhythmia?

* CxR: cardiomyopathy?

1. Chest pain

Cardiac involvement: possible idebtify:

-Coronary artery abnormalities

-Arrhythmia

-Cardiomyopathy

-Myocarditis/ pericarditis

-Outflow obstructions

-Mitral valve prolapse

1. Chest pain

Pulmonary involvement: -Pain of muscle strain (related to cough) -Pleural irritation or pleurisy -Diaphragmatic irritation (pneumonia, bronchitis) -Asthma -Thoracic tumor -Foreign body aspiration -Pulmonary embolism -Mediastinal causes: pneumomediastinum, mediastinitis, or tumor

1. Chest pain

Pulmonary involvement:

-S/s: cough, fever, short of breath.

-Exam: CxR?SaO2

1. Chest pain

Musculoskeletal involvement:

Tender on palpation and Hx of chest injury

-Muscle overuse (-)

-Trauma (+) involving the pectoral, upper back or shoulder muscle.

1. Chest pain

Musculoskeletal involvement: possible identify:

-Muscle sprain.

-Costochondritis

-Slipping rib syndrome

-Xiphoid process

-Tietze syndrome

1. Chest pain

Gastrointestinal involvement:

Watery diarrhea, Vomiting

-Endoscopy ?

-Upper GI series ?

-pH probe study or trial of antacids ?

1. Chest pain

Gastrointestinal involvement: possible identify:

-Peptic ulcer disease

-Esophagitis, esophageal spasm, foreign body in esophagus

-gastritis

-Caustic ingestizxons.

-Others: pancreatitis, cholecystitis, subdiapragmatic abscess

1. Chest pain

Other cause of chest pain: -Hyperventilation or stress -Lightheadness, dizziness, paresthesia,, chest pain -High anxiety, psychopathologic process -Thelarche -Gynecomastia -rare: herpes zoster infection, nephrolithiasis, fibrocystic

disease, adenocarcinoma of breast , chest syndrome of sickle cell disease

cold” chest wall mass may present a diagnostic challenge. The differential diagnosis of such a chest wall mass would include actinomyces, cold abscess due to Mycobacterium tuberculosis, or possibly malignancy.