Dr.Rithwik Sunil, Dr. C. Jayakumar,DrSajith Kesavan ,Dr. Praveena Bhaskaran, Dr.Navya George
AIMS Kochi, Department of Paediatrics
Seven year old boy from Tamil Nadu presented with a 10 day history of a persistent wet cough and very thick tenacious spitted out sputum
Parents also showed the picture of the very thick sputum.In the hospital also he used to spit out sputum but gradually it thinned out .He is a known case Congenital cyanotic heart disease BTS (Blalock-Tausing ) + Confluent plasty and later underwent B/l BDG (Bidirectional Glenn)+ Hilum to Hilum plasty.
Child was initially shown to a local hospital where he was admitted and received IV antibiotics(Ceftriaxone, Amikacin, Augmentin). He was then brought to AIMS in view of persisting symptoms. Here also antibiotics levofloxacin was continued
After five days of admission child developed persistent fever spikes with episodes of desaturation.
No history of contact with people having viral infection, adult Kochs .No history loss of weight, poor growth , loss of appetite or night sweats
At admission Blood pressure (BP) 98/53(mean BP 63), Heart rate (HR) 105, oxygen saturation (SPO2) 93% on room air and respiratory rate of 33 breaths per minute.
The respiratory examination revealed reduced air entry on the right lung zones
Labs
CRP(52mg/l) and neutrophilic leucocytosis(Tc:27k, N:87%)
Gene expert for TB negative
Aspergillosis Galactomannan assay was tested to be negative
Fungal culture was negative
Chest Xray showed patches in Right upper lobe region
HRCT done showed patchy ground glass opacities and ground glass nodules involving all the lobes of both the lungs.
He was getting Management for Pneumonia but the Possibility of Primary ciliary dyskinesia or plastic bronchitis were also considered
Child was then planned for a bronchoscopy to confirm the same.
Bronchoscopy done revealed the presence of large plugs in the bronchus which as then extracted and sent for histopathology
Now the child is transferred back to paediatric cardiology and cardiac catheterisation is planned
Child is still in oxygen support
Antibiotics is escalated
Diagnosis: Plastic Bronchitis
Discussion
Plastic bronchitis is an uncommon condition, but recent evidence suggests that it is underreported as well .Of note, plastic bronchitis prevalence in Fontan surgery patients has been estimated to be as high as 4–14%.
The patients in our cohort can be placed into 2 main categories: (1) those with congenital heart disease, and (2) those with primary pulmonary processes.
Historically, these are the 2 most common diagnostic groups associated with plastic bronchitis. Due to the small number of cases reported, a gender or age predilection was not demonstrated.
This condition can also be seen in asthmatic patients .
While the alarming presentation of large, branching, expectorated casts is pathognomonic, many patients present with less specific symptoms such as dyspnea, cough, and fever.
Severe hypoxia due to airway obstruction can occur either on presentation or in the course of the disease.
• On physical exam, wheezing or decreased breath sounds are commonly observed in symptomatic patients.
• The auscultatory “flag snapping” sign (also called bruit de drapeau) is attributable to a partially obstructing cast moving in a bronchus .
• Radiographic findings are often nonspecific and include atelectasis or infiltrate. Although noninvasive imaging can assist in the diagnosis, a cast specimen for gross and microscopic examination is usually required to confirm the diagnosis. If there is no history of cast expectoration in a patient at risk, a high index of suspicion is appropriate due to the rapid decompensation and even fatal outcome due to acute airway obstruction.
• The use of bronchoscopy for both diagnosis and treatment is important. In situ casts vary in size and can extend throughout the entire tracheobronchial tree.
• Expectorated or extracted cast material is generally beige to white in color and rubbery in consistency
Prognosis: PB is a highly morbid disease with limited treatment options. Bronchoscopy and chest physiotherapy for airway clearance are among the most-utilized therapies. There is high chance of recurrence given the patients cardiac history.
The treatment of plastic bronchitis includes acute therapy to assist the removal and expectoration of bronchial casts and short or long-term treatments that address the hypersecretory process . However, if plastic bronchitis can be a complication of an underlying disease, the underlying condition must be treated to eliminate the formation of bronchial casts.
Take home message:
Chronic Respiratory illness in children with prior history of congenital heart disease and post-surgical correction, one must always keep an open mind for the possibility of plastic bronchitis, even if Xray seems to suggest otherwise and diagnostic bronchoscopy is a good choice
