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5/F, Pulmonary artery sling with tracheal ring / recurrent wheezing, cough for 5 years(aggravation; 2 days ago)
Chief complaint
or Title
Pulmonary artery sling with tracheal ring / recurrent wheezing, cough for 5 years(aggravation; 2 days ago)
Difficulty For student/For resident
Seung Kyu Lee, MD, Young Ho Kim, MD, Hae Ran Lee, MD[drran@hallym.or.kr],
Department of Pediatrics, the Kang-Dong Sacred Heart Hospital, College of Medicine, Hallym University. Seoul, Korea
* History
Present illness; She was already diagnosed as a congenital laryngomalacia in an ENT department of other general hospital by laryngoscopy a few years ago. For a few years, she frequently visited to our hospital because of acute bronchiolitis and infantile asthma and was admitted. 2 days ago, dyspnea and cough developed and aggravated, so she visited to the hospital again and was admitted.
Past history
birth history ; IBW- 2.86 kg , normal full-term spontaneous vaginal delivery

Amission history

at age of 6 month  
cough, grunting, dyspnea à admission (acute bronchiloitis)
HD(hospital day) #2 cyanotic change, dyspnea aggravation àmove to ICU, intensive management.
HD #5 fever developed, cough, sputum aggravation à chest CT(HRCT) done
           Chest CT : no specific finding on lung parenchyma ,diffuse narrowing of trachea diameter            
HD #8 general condition improved à move to general ward
HD #15 Discharge
   at age of 10 month
cough, dyspnea, noisy breath sound à admission (acute bronchiloitis)
HD #1 dyspnea aggravation, persistent wheezing and stridor à move to  ICU
HD #5 dyspnea and wheezing decreased, move to general ward, but stridor  persisted      
HD #10 symptoms improved, discharged
OPD(out-patient department) follow-up

at age of 22 month
    dyspnea, cough, sputum àER visit à admission to ICU (infantile asthma)
    management; Nebulizer(salbutamol, budesonide) , IV steroid, IV aminophylline,
O2 inhalation(2L/min)
    HD #4 dyspnea subsided and SaO2 98% at room air à move to general ward
    HD #8  no dyspnea, but stridor persisted
            Oral aminophylline
    HD #11 symptoms improved, discharged
OPD follow-up

   at age of 28 month
    dyspnea à admission
    HD #6 dyspnea improved, but wheezing and stridor persisted
    HD #9 symptoms improved, discharged
OPD follow-up
Vaccination history : all done by schedule
Family history : no remarkable finding
* R.O.S.& P/Ex
Review of system
fever (+), cough/sputum/rhinorrhea (+/+/-) , vomiting/dirrahea (-/-)
dyspnea/cyanosis (+/-) , poor oral intake

Physical examination
Vital sign : PR 128/min   RR 34/min   BT 37.8?
G/A acutely ill-looking appearance, difficulty to breath
HEENT  not anemic conjunctiva , anicteric sclerae
           peritonsillar hypertrophy (++/++) with throat injection
           not dehydrated tongue & oral mucosa
           not palpable neck mass
Chest    regular heart beat without murmur
           coarse breathing sound with wheezing, inspiratory stridor
           symmetric expansion with severe retraction
Abdomen soft and flat , hyperactive bowel sound
Back & Extremities  no specific findings
* Lab

CBCElectrolyteRoutine chemistry
WBC6,510(62.6/25.4)Na148 mEg/LBUN/Cr10.4/0.4 mg/dl
Hb13.2 g/dlK4.6 mEg/LProtein7.5 g/dl
Hematocrit38.4 %Cl106 mEg/LAlbumine5.0 g/dl
Platelet293,000 /uLAST/ALT35/20 IU/L
Ca/P9.7/4.3 mg/dl
* Clinical Course
HD#1; severe dyspnea and cough, SaO2 95%, chest retraction à epinephrine subcutaneous injection, O2 3L inhalation, Salbutamol and budesonide inhalation via nebulizer, Aminophylline I.V. , Steroid (methyl prednisolone) I.V.
HD#6; dyspnea improved ,severe cough and chest wall retraction, wheezing and stridor  
HD #11; cough and wheezing decreased
         Aminophylline I.V à change to  P.O. medication
HD #16; stridor, chest wall retraction persisted
         Chest CT & Bronchoscopy 3D CT done à the left pulmonary artery comes out from an aberrant origination in the right pulmonary artery, and then it goes through   between main trachea and  left bronchus so it compresses air-way. The trachea is narrowed through long segment and it has no membranous portion, so it is called 'tracheal ring'.
HD #17; symptoms improved, discharged
*Her respiratory symptoms has been more improved than before ,so we have not decided to do operation for pulmonary artery sling and tracheal ring. Nowadays we have observed her symptoms.

* If you click the image you able to see original image

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