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Interesting Cases - Paediatrics

 

Pediatric staff team with survived extremely low birth weight neonate (less than 600 grams)

 

10 year girl with Dicky Devidoff Mason Syndrome cared in pediatric ward.

 

Pediatric staff at a press conference with a rare case of syngnathia.

 

           BROCHIOLITIS OBLITERANS ORGANISING PNEUMONIA WITH PNEUMOMEDIASTINUM.

Dr Chandana D R., Dr Vinay Patil., Dr Sanjay D., Dr Chandrashekar Gouli.,Dr Kalappanavar N K. 

Case report

              An  apparently healthy 5 month old Kavya, female child, presented to us with cough for the past 1 month which was initially associated with low grade fever and running nose. Fever and running nose subsided with treatment in 3-4 days. But cough was persisting, spasmodic in nature, non-pertusoid, no post-tussive vomiting, no diurinal or no postural variation, no aggravating or relieving factors.

            Mother noticed hurried breathing from last 1 week which was progressively increasing for which child was hospitalized in local hospital for 4days. As no clinical improvement was observed, child was referred here for further management.

There is no history suggestive of persistent fever, recurrent aspiration, infection at other sites, poor feeding, lethargy, convulsions, bluish discolouration of the body. 

Past history, Family history, Birth history, Immunization history & Developmental history were normal.                          

PHYSICAL EXAMINATION: 5 month old female child, conscious, irritable and tachypneic(RR 80/min) with HR 140/min, , tempt – 98.8F, CFT –2sec. SPO2 - 75% in room air and 92 – 95% with 6L of headbox O2.

ANTROPOMETRY revealed Wt- 4.5 Kg /7kg (64%), Lt– 60 cm /64 cm (bw 3rd & 25th centile) (as per WHO charts), HC – 40/42cm (Normal), US:LS –1.6:1(Normal), CC – 37cms.  Impression: failure to thrive. 

Pallor present, There was no icterus, cyanosis, clubbing, lymphadenopathy, edema or neurocutaneous markers. Other examination findings were normal.                           

  SYSTEMIC EXAMINATION

RESPIRATORY SYSTEM:

  Bilaterally symmetrical chest, abdominothoracic breathing, tachyapnea was seen with flaring of alae nasi, intercostal & subcostal retractions.                                       

Trachea central, apex beat at left 3rd intercostals space lateral to midclavicular line, palpable crepitations in bilateral axillary and infra-axillary areas. (suggestive of  subcutaneous emphysema).

On percussion resonant  all over lung fields with liver dullness in right 5th intercostals space. On auscultation, Bilateral air entry equal, vesicular breath sounds with prolonged expiration, fine crackles heard all over lung  fields along with the expiratory rhonchi.   

Other systems were normal

 Following PROVISIONAL DIAGNOSIS were considered :         

                    persistant pneumonia-Secondary to ?Community acquired pneumonia, Partially treated pneumonia, Congenital lung malformations,  Immunodeficiency, GERD, Aspiration pneumonia,  Atypical infections, Tuberculosis, Interstitial lung diseases were considred.

INVESTIGATIONS:

CBC :Hb-9.5gm/dl  WBC-13,700/cumm,HCT-31.2%, PLT-3.04/cumm       N -57%    L-37.2%      PS- normocytic normochromic anaemia.

CHESTX-RAY –   b/l patchy homogenous opacities with pneumomediastinum

BLOODCULTURE: Pseudomonas sensitive to levoflox, oflox, gatiflox, piperacilline  tazobactum, imipenam.

Urine, seum electrolytes, ABG, renal and liver function tests were normal.  HIV ELISA : nonreactive

IMMUNOLOGICAL WORKUP: ANA, Anti Ds DNA ab, Anti Sm ab, Anti centromere ab, Anti Scl 70 ab were all negative.

BRONCOSCOPY :

Upper airways,medium sized bronchi upto terminal bronchi are normal. No luminal or extraluminal obstruction seen. Bronchoalveolar lavage sent for culture and sensitivity.

CT SCAN :Diffuse areas of consolidation & ground glass poacities involving both the lungs with relative sparing of anterior segment of both  lower lobes s/o cryptogenic pneumonia/ BOOP.

 

LUNG BIOPSY :  granulation tissue plugs with in the smaller airways which extend into alveolar ducts & alveoli S/O BOOP.     

COURSE IN THE HOSPITAL :

                                                 At first ceftrioxone & cloxacillin  was started with o2 therapy. Subsequently based on x – ray, azithromycin was added to cover atypical organisms. Later based on blood culture report piperacillin-tazobactum was added. But child did not show improvemnent in 1st 5 days of hospital stay, on 6th day child was ventilated for desaturation, and worsening respiratory distress.  Meanwhile CT- thorax was done , which guided for lung biopsy. Lung biopsy confirmed the diagnosis of BOOP. For which corticosteroids was started. After which decrease in ventilator pressure requirements was noted and ABG also showed improvement. Currently child is still on ventilator.

 

BRONCHILITIS OBLITERNS ORGANIZING PNEUMONIA

                                               ( BOOP) :

                          

                   Also called as cryptogenic organizing pneumonia, one of the forms of interstitial lung diseases, rare in children. Overall incidence in general population is 0.01%.

Etiology : is unknown , thought to be precipitated by

viruses -adenovirus, influenza , measles .

 Bacteria - pertussis , legionella  .

Atypical organisms - mycoplasma .

other causes – JRA , SLE , scleroderma , steven Johnson syndrome. Toxic gases – NO2 , NH3 .

 post organ transplantation - lung , bone marrow.                       PATHOLOGY: Initial insult-inflamation results in obliteration of airway lumen which then extends into alveolar spaces. Unresolved inflammation, exudates persists and results in fibrosis.

C/F : cough, fever, dyspnoea, tachypnoea,cyanosis, hypoxia, anaemia

INVESTIGATIONS : routine blood investigations, chest x-ray, HRCT, pulmonary function tests, BAL, lung biopsy.

TREATMENT : No specific treatment . steroids- oral prednisolone, iv methylprednisolone for 1 year. Newer drugs- immunomodulators methotrexate, cyclosporine, tacrolimus, sirolimus, infliximab can be tried.

PROGNOSIS : frequently relapsing disease, some patient rapidly detoriate, depends on the underlying systemic cause. Idiopathic form shows best response upto 60%.

 

 

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