“Unpredictable Air: Recurrent Spontaneous Pneumothorax”

Dr.Ghaniya,Dr.Greeshma(Intensivist), Dr.Sajith Kesavan(Paed pulmonologist and Intensivist),Dr.C.Jayakumar
AIMS Kochi 
Kerala
India 

A twelve-year-old female child presented with a history of cough accompanied two episodes of hemoptysis lasting for one day.

Medical History:
The patient, the first child of non-consanguineous marriage, who has had an uneventful perinatal history . At five months developed respiratory lower tract infections necessitating antibiotic therapy .In between there is no history of IP care 
At nine years old, she was admitted due to cough and respiratory distress and chest X-ray revealed a left pneumothorax, for which an intercostal drain (ICD) was inserted. Despite treatment with high-flow humidified nasal cannula (HHHNC) support and intravenous antibiotics, the pneumothorax persisted. 
Subsequent imaging showed a persistent left pneumothorax and spiking fever. 
CT scan revealed mild to moderate left pneumothorax with a small fluid level suggestive of hydropneumothorax. After unsuccessful pigtail insertion, the patient underwent ICD placement. Immunodeficiency workup yielded normal results, and subsequent X-rays showed resolving pneumothorax.

At ten years old, she again presented with left-sided chest pain, and imaging revealed loculated pleural collection with fluid and air pockets. 
This necessitated left thoracotomic decortication with abrasion pleurodesis, after which the patient remained asymptomatic for nearly a year.

Current Presentation:
Upon examination, the child exhibited tachypnea with mild desaturation, maintaining oxygen saturation with 5L O2 via a nasal mask for one day. Other vitals were within normal limits.
Auxology is normal No marfanoid features
Investigations:
Labs 
Elevated total leukocyte count and CRP. Imaging studies including chest X-ray, CT pulmonary angiogram, and ultrasound chest showed significant findings consistent with hydropneumothorax, loculated pleural collection, and mediastinal involvement.

Biopsy Findings:
Pleural and lung biopsies revealed inflammatory changes, hemorrhage, and congestion, with negative immunohistochemistry for malignancy and no evidence of Kochs Gene expert ultra and biopsy 

Management:
Thoracoscopy revealed left hemi-pneumothorax with collapsed upper lobe, prompting open decortication and ICD insertion. The patient received antibiotic therapy and was referred for gynecological evaluation for suspected pleural endometriosis.Hematology work up for coagulation studies were within normal limits.

Plan : To send WES , Sweat chloride , Alpha 1 anti-trypsin
Discussion:
The case underscores the challenge of managing recurrent spontaneous pneumothorax in pediatric patients and highlights potential etiologies including infectious, connective tissue, and malignant.

Airway disease : 
Emphysema 
Cystic fibrosis 
Severe Asthma
Infectious lung disease : 
PCJ 
TB
Necrotising pneumonia
Interstitial lung disease 
Idiopathic pulmonary fibrosis, 
Sarcoidosis
histiocytosis X
Rheumatoid arthritis
Scleroderma
Ankylosing spondylitis
Marfan’s syndrome 
Ehler’s Danlos syndrome
Lung cancer 
sarcoma.

Take Home Message :
Recurrent spontaneous pneumothorax in pediatric patients warrants comprehensive evaluation and multidisciplinary management to address underlying causes and prevent recurrence.

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