Sudden shortness of breath in a young male!

Clinical scenario:

        A twenty years-old male presented to Emergency room  of our hospital with sudden onset of chest pain of four hours duration. The pain started  on right side to begin with and later became bilateral after 2 hours. The condition was associated with dyspnea and non productive cough.

Systemic review of the patient and his past history was unremarkable.

However , he had been a chronic smoker  40 cigarettes / day for 7 years. He had started smoking at a very young age .

Examination :

        Clinical examination revealed a   distressed patient with tachypnea but no cyanosis.

Pulse was 110/minute, blood pressure was 110/70 mm Hg and his oxygen saturation was 90% on room air.

Neck veins were not engorged

Chest :  Trachea was central

Percussion of both sides revealed no hyperresonance, but there was bilateral distant breath sounds on auscultation with no wheezes.

Rest of the clinical examination was unremarkable .

Evaluation :

        His laboratory findings hemogram , renal and liver function tests were within normal.

Chest radiographs revealed bilateral pneumothorax. High resolution CT chest scan revealed bilateral mid and upper zonal lung cystic lesions with honeycombing which was consistent with the diagnosis of pulmonary Langerhan cell histiocytosis (PLCH), however, other cystic lung lesions were suspected as differential diagnoses (Fig1).

Fig 1 CT scan chest bilateral cystic lesions in chest .

Hospital course :

Oxygen was delivered to the patient through a mask at a rate of 5L/hr. Two intercostal tubes were inserted bilaterally and the patient was prepared for Video-assisted thoracoscopic surgery (VATS) on the right side. 

Thoracoscopic examination showed diffused cystic lesions on the lungs, and then target areas (dominant blebs) were identified at the right upper lobe which was resected using a stapler device with enough amounts of surrounding tissues for histopathological examination. In the first session mechanical pleurodesis was added. 

The pathological report revealed presence of areas of scaring with numerous Langerhans cells (positive for S-100), esinophils, lymphocytes and fibroblasts (Figure 2, 3) and a definitive diagnosis of pulmonary Langerhans cell histiocytosis X was established

Fig 2 Histological specimen showing Langerhans cells  

Fig.3  Langerhan cells positive for S-100 stain 

  A week later, video-assisted thoracoscopic surgery was performed on the left side and the major blebs were also resected with talc pleurodesis as the talc was not available at the first session. The patient had uneventful postoperative course and he was discharged after 7 days. The patient stopped smoking and follow up for more than 5 years revealed no recurrence and both lungs were fully inflated with no difference between both sides.

Teaching message : Cessation of  Smoking helped to prevent recurrence in this patient  . 

Further reading click the Link Langerhan cell histiocytosis

This case was contributed by :

Dr. Majed Al-Mourgi .FRCS,FCCP,FACS

Consultant Thoracic surgeon &

Head of Surgery dept 

College of Medicine Taif University KSA