A 14-year-old female with a 1- week history of intermittent right-sided chest and back pain and shortness of breath presents to the Emergency Department. She describes the pain as dull in quality, diffuse but non-radiating, and lasts approximately 1 hour before subsiding. The pain is pleuritic in nature and the only alleviating factor identifiable is ibuprofen. The shortness of breath is not related to any exertion or activity and was constant.
There is no past medical history of a specific or relevant medical problem and the patient specifically denies a history of asthma, pneumonia, gastro-esophageal reflux, or broncho-pulmonary dysplasia. The patient was a full term baby born via spontaneous vaginal delivery without any complications. Menarche occurred at 12 years of age. Cycles are regular, occurring every 31 days and lasting for 4 days.
The patient received all of her recommended immunizations for age. She lives with her mother, father, and brother in a house. There are no pets or smokers in the home. There is no history of recent sick contacts, no recent illnesses, and no recent travel. She denies sexual activity and denies alcohol, tobacco, or other illicit substance use.
Family history is significant for asthma in her maternal grandfather but negative specifically for tuberculosis, Connective tissue disease, pneumothorax, or Sarcoidosis.
Vitals signs are within normal limits. She is breathing at a rate of 20 breaths per minute and oxygen saturation is 100% on room air. Height and weight reveal a girl at the 90th and 10th percentiles respectively.
Physical exam reveals an alert, awake female who appears her stated age and is in mild distress. All physical exam findings are within normal limits with the exception of the respiratory exam, which reveals diminished breath sounds in the right upper lung field, and the musculoskeletal exam that reveals a mild scoliosis and a Marfanoid body habitus. A complete blood count and basic metabolic panel are within normal limits. A chest x-ray demonstrates a 2.3cm apical pneumothorax in the right hemithorax.
This was the patient's first of several presentations. During a 2-year period she developed 9 pneumothoraces both in the right and left hemithorax, all confirmed radiographically. After the first 5 were treated primarily with a non-rebreather mask and symptom management, she had a bilateral video-assisted thoracoscopic surgery (VATS) and a blebectomy of the most highly affected areas. The VATS did not reveal any lesions suggesting a definitive pathology for the pneumothoraces. For 9 months following this procedure, she continued to have recurrent pneumothoraces that occurred approximately every 3 months.
Testing for cystic fibrosis, alpha-1 antitrypsin deficiency, and Fibrillin-1 (to evaluate for Marfan's and adolescent idiopathic scoliosis) was negative. A CT-Chest w/o contrast (see Fig. 1) performed on her 7th presentation displayed a left-sided 2.1cm pneumothorax but did not demonstrate any evidence of blebs or bronchiectasis.
After her 9th presentation, the obstetrics and gynecology team was consulted. The pneumothoraces always presented within+-two days of her menses. She was placed on a trial of continuous combined oral contraceptive pills (COCs), specifically 90 mcg levonorgestrel and 20 mcg ethinyl estradiol tablets (Amethyst ). She achieved complete resolution of her symptoms after this and was thus clinically diagnosed with catamenial pneumothorax. Three years after starting the COC she has remained in total remission and has not experienced another episode of pneumothorax.
Non-contrast CT scan demonstrating one of many right-sided pneumothoraxes in our patient. No bronchiectasis or blebs present on CT.