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Peer Reviewed

Photoclinic

Langerhans Cell Histiocytosis Presenting as Pneumothorax and Bleeding Gums in a Pediatric Patient

  • Discussion. Spontaneous pneumothorax (SP) is rare and may have nonspecific presentations in children. SP is defined as a rupture of visceral pleura with air accumulation within the pleural space, which can be primary or secondary. Tension pneumothorax (TP) creates increased intrathoracic pressure, leading to mediastinal shift, respiratory distress, hypoxia, and hemodynamic instability, as illustrated in Figure 1.1

    Among the causes of secondary SP are CF, asthma, and interstitial lung disease.1 The prevalence of pediatric primary spontaneous pneumothorax (PSP) is less than 0.01% in the general population,2 and the condition predominates in males, smokers, newborns, and adults.3

    SP usually presents at rest or with minimal exertion with sudden onset of symptoms. Chest radiographs should be obtained in the upright position; inspiratory films are preferable. Chest CT scanning is obtained for chest tube placement and to help determine etiology.4,5

    Pediatric SP has many differential diagnoses including simple viral, bacterial, or fungal infections; CF; LCH; Marfan syndrome; and malignancy.7,10,11 LCH can present in many ways, including SP. Lung findings can be seen in the absence of clinical pulmonary symptoms, so it is vital to be familiar with alternative presentations, such as in bone as illustrated in Figure 3.9,10 If there is enough suspicion to warrant imaging, a CT scan can provide clues to anticipate future episodes of PSP.8 Counseling on long-term management of any lung pathology in children is warranted.6 Smoking cessation, as evidenced among pregnant women with LCH, appears to be a critical factor impacting the course of the disease.6 Follow-up and long-term prognosis of SP is dependent on etiology, and treatments will differ accordingly.

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