اقاي دکترسيد احمد طباطبائي
فوق تخصص ريه عضو هيئت علمي

به اتفاق اعضای هیئت علمی بيمارستان

كودكان مفيد

تشخيص:

Post Operative

TEF

تشخيص هاي افتراقي:

خانم دكتر الهه موحدي

رزيدنت بيمارستان مفيد

Question remind unclear about case

Trend of growth?

Recurrent  infectious (pneumonia or AOM or sinusitis,…)?

Symptoms after vaccination?

Hx of diseases in other sibling

Leg pain?

Hx of trauma?

Night sweating, exertional dyspnea, cough

Cyanosis, Clubbing?

Parental relative?

Drug Hx?

Diff- U/A-BS-LFT

EOSINOPHILIC LUNG DISEASES
(PIE)

 Early description :

   pulmonary symptoms or CXR abnormalities and

   PB eosinophilia

 After 1970 description :

characterized  by  increase  in  BAL eosinophil number but not necessarily blood eosinophils

Some affect the airways predominantly,          some  affect  the  lung  parenchyma,  and some affect  predominantly  the  lung  vasculature.

From subtle symptoms and are self-limited to  respiratory failure.

Subtype:

Group I, Löffler syndrome or simple pulmonary eosinophilia

 Group II, prolonged pulmonary eosinophilia

 Group  III,  Weingarten’s  syndrome  or tropical  eosinophilia

Group  IV,  pulmonary  eosinophilia  with asthma

 Group V, polyarteritis nodosa

Parasitic  infection (Ascaris) & drug reactions are currently an important etiology

No cause in 1/3 of cases

Mild febrile illness with myalgias ,nonproductive cough, and dyspnea.

No abnormalities on physical examination, but sometimes a few crackles or wheezes  are  heard

Parasites That Cause
Eosinophilic Lung Disease

Ancylostoma sp.

Ascaris sp.

Echinococcus sp.

Schistosoma sp.

Strongyloides stercoralis

Toxocara sp.

Trichinella spiralis

Wuchereria bancrofti

Patients with Ascaris infection are usually febrile with a nonproductive  cough  and  chest  pain. In  severe  cases  hemoptysis occurs. 

CXR: abnormalities  are usually bilateral and peripheral, pleural  based

Parasites  and  ova  are sometimes  found  in  the stool after the resolution of the  pul. illness

larvae can be isolated from gastric aspirates or sputum

Weingarten = tropical  eosinophilia =

severe  spasmodic cough,  massive peripheral  eosinophilia,  diffuse  mottling  in  both  lungs

endemic  in  India, Sri Lanka  due to  Wuchereria  bancrofti 

DRUG-INDUCED

 Cough,  dyspnea,  and  fever

 Histologically : interstitial edema with a lymphocytic and eosinophilic infiltrate, (alveoli contain  eosinophils  and  histiocytes)

CXR show  interstitial or alveolar infiltrates  and Kerley B lines

Skin testing  with either patch  or prick  tests  is  usually  negative

ACUTE EOSINOPHILIC PNEUMONIA

Idiopathic

febrile  illness  of  1  to 5  days + myalgias,  pleuritic chest pain, and severe hypoxemia

basilar or diffuse crackles

 CXR: diffuse alveolar infiltrates involving all  lobes

Small to moderate-sized pleural effusions are common

CHRONIC EOSINOPHILIC PNEUMONIA

middle-aged women

fever,  night  sweats,  weight  loss

cough,  dyspnea,  and  wheezing (asthma)

lymphadenopathy  and hepatomegaly

Pb & BAL eosinophil

ESR & IgE  is elevated

CXR; bilateral, peripheral infiltrates negative image of pulmonary edema =dignostic

CT : peripheral airspace disease  and  may  show hilar lymphadenopathy

ALLERGIC ANGIITIS AND GRANULOMATOSIS

allergic rhinitis and asthma for years

peripheral eosinophilia with values up to 80%

small and medium-sized arteries and veins.

 sinusitis, rhinitis, and nasal polyps

 GI:abdominal pain, diarrhea, bleeding, and obstruction;

cardiovascular: pericarditis, and heart failure.

Renal

CNS

CXR: patchy and transient infiltration

Eosinophilic pleural effusions and hilar adenopathy

IDIOPATHIC HYPEREOSINOPHILIC SYNDROME

elevated eosinophila for >6 months,

or for <6 months with evidence of organ (heart) damage

most common in adults 20 to 50 years - male

ABPA

 1% to 2% of asthmatics and 7% to 9% of cystic fibrosis

Minimal Diagnostic Criteria:

Acute or subacute clinical deterioration

Total  serum  IgE >500 IU/mL

 Immediate  cutaneous  reactivity  or RAST

(a)IgG  antibody  to A. fumigatus; or (b) new or recent abnormalities on CXR or chest  CT that  have  not cleared with antibiotics and standard physiotherapy

Hypersensitivity Pneumonitis

HP = extrinsic allergic alveolitis

recurrent inhalation of organic antigens 

pet birds – molds (Aspergillus, Penicillium)

 mean  age=10 y

Male

 flu-like syndrome  with  fever,  chills,  cough,  myalgias,  and  malaise

 leukocytosis  with neutrophilia,  elevated  CRP & ESR

 ill appearing  child with  dyspnea  and  basilar  crackles

CXR : bilateral lower lobes reticulonodular  infiltrates

CT: ground-glass appearance and centrilobular nodules

 Acute eosinophilic pneumonia, P. carinii pneumonia, and some drug induced lung diseases have Eosinophils in the BAL fluid

Acute  & chronic  eosinophilic  pneumonia,  ILD,  and  tropical eosinophilia usually lead to a restrictive defect

Immunologic:

+ve: Weigh + Aspergillosis

CGD

Hyper IGE (skin-face-teeth-AOM-pneumonia-staph pneumatoceles-mucocutaneous candidiasis)

Rheumatology:

+ve: Weigh + kidney

PAN (heart-joints-skin-GI-Renal-eye lung-CNS-PNS)

JRA

 SLE

DDx lung consolidation + lymphangectasis may include :

Obstractions

Infections

Aspergillosis (invasive/infection or allergic)

Ascariasis

Hydatid cyst

TB

Blastomycosis

Cryptococcosis

IDDM

CGD

ABPA (CF)

Lymphoma

TB

PAN-JRA

HIV

TEST

BS

NBT or DHR àCGD

SCT

IgE - Prick test –

PFT

Lung Biopsy

خانم دكتر مينا دادخواه

رزيدنت بيمارستان لقمان

به نام خداوند بخشنده و مهربان

P.I

Female /6 y.o

Wheezing/cough (righte now)/Fever

Cough and wheezing from neonate period

Congenital esophageal atresia

primary repair of a congenital EA w/o TEF on the first day of her life.

recurrent  pneumonia and wheezy episodes

barium swallow:NL

unresponsiveness to anti-asthmatic and anti-biotics and anti-reflux treatment

Weight loss

No allergy

CXR: straggly &bilateral , brief grand glass appearance

CT-SCAN: segmental collapse and alveolar infiltation in lower parts

Bilateral diffuse nodular opacities and tree in bud pattern accompanied by mosaic attenuation due to air trapping.

Cylindrical bronchiectasis of lower lobes

Dilatation of esophagus and hiatal hernia

VIRTUAL BRONCHOSCOPY:NL

PPD:NEG/BK:NEG

Sweat test:Nl

Immunologic test: Nl

Lab data:

Wbc:24300/neut:90%,lymph:10%

Mcv:50.9/RBC:5,600,000

Plt:605000

Bun:7/cr:0.7

Bronchiectasis
Bronchiectasis, a disease characterized by

irreversible abnormal dilatation and anatomic distortion of the bronchial tree, likely

represents a common end stage of a number of nonspecific and unrelated antecedent events.

patients presented with cough, tachypnea, wheezing, sputum production and failure to thrive. Clubbing was found in (33%) of the patients. Cyanosis and oxygen requirement were reported in  (23%) of the patients. Hemoptysis was reported.

Bronchiectasis DDx

Pulmonary

Asthma

Kartagener

Foreign body aspiration

ABPA

Tuberculosis

Bronchogenic cyst

Prematurity

Cystic fibrosis

TEF repair

second congenital fistula

Reccurent fistula

Most infants with EA with TEF have proximal atresia with distal TEF. They are easily diagnosed in the first days of life with apparent symptoms and findings and undergo surgical repair. Since two fistulas with EA occur very infrequently, proximal fistula may be overlooked during primary surgical repair. An undiagnosed proximal fistula shares the same clinical features with an isolated H-type fistula, which is usually diagnosed later, even in adulthood.

Although symptoms of an undiagnosed proximal fistula may include choking, coughing and recurrent respiratory tract infections, there may be long and asymptomatic intervals, which may be explained by the nature of the fistula. The contractions of the muscle wall of the fistula or the oblique direction of the fistula tract from the trachea to esophagus may protect the airway from aspiration of foods during swallowing.

Recurrent respiratory tract infections and wheezing are commonly reported in infants after primary repair, but become less frequent over time. Bronchiectasis is a very unusual complication.

Asthma is reported in nearly one-fourth of children with repaired EA and TEF. In our patient, late onset of recurrent bouts of pneumonia and wheezing resulting in bronchiectasis, unresponsiveness to anti-asthmatic treatment, incompatible with the diagnosis of asthma raised the possibility of a recurrent TEF or a second congenital fistula.

In the pre-operative period, although standard barium swallow failed to show the fistula, in the prone position confirmed the diagnosis. The presence of a TEF can also be demonstrated by three-dimensional CT scanning and virtual bronchoscopy. A dynamic radionuclide gastroesophageal scintigraphy with technetium- 99m showed a fistulous opening between the trachea and the esophagus .

Cardiac diseases:

 Dextrocardia 

Congestive heart failure

Ventricular septal defect

Atrial septal defect

Pulmonary hypertension

Mitrale valve prolapse

Skeletal: 

Pectus excavatum

Scoliosis  Absent ribs  Marfan's syndrome

Immunodefficiency:

Hypogammaglobulinemia

SCIDS

Human immunodeficiency virus

Hyper IgE1

IgG subclass defficiency

Hyper IgM

Whiscott Aldrich syndrome

Common variable-Hypogammaglobulinemia

T-cell deficiency

Barre lymphocyte syndrome

Central nervous system disease: 

Cerebral palsy/ seizure disorder

Apnea  Craniosynostosis 

Cutis laxa/ developmental delay  Down syndrome/seizure  Fatty acid oxidation defect 

Other disease associations: 

Neuroblastoma 

Antithrombin III defficiency 

Corrosive ingestion  Liver cirrhosis  Ethmoid mucocele 

Discussion

All patients with confirmed bronchiectasis had the following tests done:

Respiratory cultures from sputum or from nasopharyngeal aspirates if unable to produce sputum for culture and sensitivity; PPD skin test; sputum for acid fast bacilli (AFB) stain and AFB culture, or gastric aspirates instead of sputum   for children who are unable to produce sputum.

(PPD:NEG/BK:NEG)

barium swallow to rule out vascular ring or tracheoesophageal fistula (TEF):

barium swallow:NL

A dynamic radionuclide gastroesophageal scintigraphy with technetium- 99m  canshowed a fistulous opening between the trachea and the esophagus.

sinus CT for those who presented with persistent rhinorrhea for

more than 3 months.

For patients who had family history of bronchiectasis, nasal brush by specialist (ENT) or biopsy of airway endothelium for electron microscopy to rule out immotile cilia syndrome was done.

Pulmonary function test was done for patients >5 years of age and able to comprehend to test maneuvers.

Associated diseases were investigated according to presenting symptoms or type of referrals. (For example, magnetic resonance or CT brain was done in patients with cerebral palsy or central nervous system abnormalities.

Sweat test: This test is done to see if your child has cystic fibrosis

Chest x-ray: A chest x-ray be used to check heart, lungs, and chest wall.

Echocardiography:

Heart disease

Result

1)second congenital fistula

2)Cystic fibrosis

3)Congenital lung disease

4)Esophageal atresia repair

5)Human immunodeficiency virus(HIV)

6)Immotile cilia syndrome

7)Tuberclusis

خانم دكتر مريم مخملباف

رزيدنت بيمارستان شهدا

بسم الله الرحمن الرحیم 

Problem list:

Cough and wheeze from neonatal

Increase in respiratory problem from 4 m ago

Hx of atresia and GER surgery

frequent pul infection

FTT

CT FINDINGS :

Segmental lower lobe collapse with cylindrical bronchectasia

Tree in bud

Neg important:

sweat test neg (1 time), smear TB neg,PPD neg, clubbing neg, immunologig? Neg

Approach to brochectasia with anatomic anomaly

Missing points:

ESR, HB, Alb ?

ABG?

Growth Index (Cilliary diskinesia)

Survey of GERD and barium meal after surjery

Immunodeficiency tests?

IgE , Prick  tese? (sensitivity to Aspergillus)

BALL?

Stool Exam (Fatty stool)

Sweet  test (one time only?)

Background problem

CF

GERD

Asthma

Cilliary diskinesia

TEF 

(Other anatomiCdefects such as: Sequestration,

broncogenic cyst , Pulmonary agenesis)

Immunodeficiency

Acquired bronchial obstruction:(Middle lobe syndrome, CGD, granulomatose)

alpha-1 antitrypsin

9.

CF

Respiratory complications:

Recurrent bronchitis and pneumonia

Atelectasis

Bronchiectasis

Gastrointestinal complications:

Esophageal atresi

CT: Segmental lower lobe collapse with cylindrical bronchectasia

Tree in bud

Dx:

1)Sweat test

False-negative :hypoproteinemic edema

2) Mutation analysis

3)72-hour fecal fat measurement

GERD:

Complications: failure to thrive, or chronic/recurrent respiratory tract disease and anemia.

CT:

Segmental lower lobe collapse

cylindrical bronchectasia

Tree in bud

Complications of fundoplication include paraesophageal hernia, and wrap failure with recurrent GER

GERD contributes significantly to the respiratory disease (reactive airway disease) that often complicates EA and TEF and also worsens the frequent anastomotic strictures after repair of EA.

ASTHMA:

بهبود مختصر با برونکودیلاتورها:

افزایش حساسیت راه های هوایی

Cilliary diskinesia:

CT: Segmental lower lobe collapse

cylindrical bronchectasia

Tree in bud

TEF:Complications of surgery include anastomotic leak, re-fistulization, and anastomotic stricture.

Immunodeficiency: CGD, Combined

( only resiratory problems, No diarrhea, No mouth Trust, No abNL reaction to vaccina)

Acquired bronchial obstruction

Acute problems:

1.BOOP

2.ABAPA

3.TB

4.Infections: pertusis , measles

5. bronchiolitis obliterans

Idiopathic 25% chronic bac bronchitis

BOOP:

adenovirus, Mycoplasma, measles, legionella, influenza, pertussis, TB

CT:

Segmental lower lobe collapse

bronchectasia

Tree in bud  and mosaic pattern

In intact lung or  lung withbackground problems

ABAPA:

CF and Asthma

CT: Segmental lower lobe collapse

cylindrical bronchectasia

Tree in bud

TB

Congenital tuberculosis is rare because the most common result of female genital tract tuberculosis is infertility.

PPD :negative, BK : negative

GASTRIC WASHING?

خانم دكتر سولماز حسني

رزيدنت بيمارستان امام حسين

بسمه تعالی
 هوالشافی

Radiologic Finding      

CXR : straggly ,  Bilateral  , Infiltration  ,grand glass

CT: -segmental collapse -Tree in bud                                                                                                              -Cylindrical  bronchietasis  -Dilation of esophagus and hiatal hernia

Bronchoscopy :   NL       Mass: negative     Stenosis:  negative    PPD: negative          Sputum: negative       Allergy: negative                   

DH: Anti reflux , Anti Biotic , Bronchodilator

Wt=14.80 kg    B.WT=2.350kg   HC=33.5 cm

Problem list

A 6 years  old girls presented with fever

Cough from birth day

Weight  : loss

Confined cycle of infection

Reflux  è operation in 1.5 years ago

CF :negative 

Immunodeficiency state: negative

EA=operation

Spo2: low(in birth day)

  Ph/E

Chest:

- inter costal refraction  -Expiration phase in long more than inspiration phase

Lung auscultation: 

- Generalized expiratory wheezing & bilateral crackles                                      

Lab Data

Wbc =24300   Neut=90%   lymph=10%

RBC = 5.6 

MCV= 50

PH = 605000

Bun=7

Cr=0.7

 DD

Complication of surgery :

Anastomotic  leak   -refistulization

-Anastomotic stricture

GERD complicates

Non-CF  related Bronchictasis

.Bronchopulmonary sequestration (intralobar)

.Congenital anatomic defects:

-Williams- Campbell syndrome

-Mounier kuhn syndrome

.Acquired bronchial obstruction:

-middle lobe syndrome

Foreign body aspiration

.Abnormal secretion clearance

.Neuromuscular Weakness

.Primary ciliary dyskenesia (PCD)

Non-CF  related Bronchictasis

Immunodeficiency :

-HIV

-Agammaglobulinemia

-SCID

-Digeorge  syndrome

-CGD

Non-CF  related Bronchictasis

Infection:

-Persistent bacterial bronchitis

-Pertussis and measles

-Mycoplasma pneumonia

-Adenovirus infections

-Mycobactrial infection

.Other disorder:

-Allergic bronchopulmonary aspergillosis

-Chronic aspiration of gastric contents

Chronic aspiration

Videofluoroscopy

Esophageal  PH Monitoring

Nuclear scintigraphy

Imaging and bronchoscopy

Neurologic dysfunction

Missed point
Bal ?

Immunologic test ?

 اهداي جوايز كتاب رباعيات حكيم عمر خيام به 4 رزيدنت

اقاي دكتر صدر

فلوشيپ ريه

اقاي دكتر مصلحي

فلوشيپ ريه

اقاي دكتر غفاري پور

فلوشيپ ريه

اقاي دكتر جفرودي

عضو هيئت علمي بيمارستان شهدا

خانم دكتر منصوري

فوق تخصص ايمونولوژي

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