Familial Thoracic Endometriosis Syndrome: Case Report

Background: The presence of functional endometrial tissue in the tracheobronchial tree, pleural, and lung is normally referred to as thoracic endometriosis (TE) or extra genital endometriosis. The commonest extragenital site of endometriosis is within the thoracic cavity. The association of catamenial pneumothorax, catamenial haemothorax, catamenial haemoptysis and pulmonary nodules is referred to as thoracic endometriosis syndrome. The same mechanism of inheritance of genital endometriosis apply to extragenital endometriosis. Case Summaries: Case1. We present EA, a 26-year-old chef/baker who presented to our unit on account of recurrent cyclical right sided chest pain and difficulty in breathing, recurrent haemoptysis and cyclical abdominal pain and swelling with multiple tender umbilical nodules. On examination she was found to be in respiratory distress but not cyanosed with SPO2 of 97-99% on room air. Examination of the chest revealed a right sided chest fullness and reduced movement with breathing. Percussion note was stony dull and absent of breath sound on right hemithorax. Abdominal examination revealed a moderately distended abdomen with positive fluid thrill. Both Pleurocentesis and paracentesis abdominis yielded a haemorrhagic fluid acellular and pleural and lung biopsy demonstrated endometrial stroma and gland. Imaging examination by way of chest X-ray showed a homogenous opacity of the right hemithorax, pulmonary nodule following drainage. A diagnosis of Thoracic Endometriosis Syndrome. Had VAT with lung and pleural biopsy, pleural abrasion and a thoracostomy tube drainage. Case 2. We present JA a 29-year-old lady, a filling station attendant who presented to our unit with a history of gradual onset of right sided chest pain, progressive difficulty in breathing and non-productive cough, and abdominal pain and swelling. On examination she was found to be in respiratory distress but not cyanosed with SPO2 of 98% on room air. Examination of the chest revealed a right apical flattening, reduced ipsilateral chest movement with breathing. Percussion note was stony dull and absent air entry on the ipsilateral hemithorax. Abdominal examination showed a mildly distended abdomen with a positive shifting dullness. Both Pleurocentesis and paracentesis abdominis yielded a haemorrhagic fluid acellular and pleural and lung biopsy demonstrated endometrial stroma and gland. Imaging examination by way of chest X-ray showed a homogenous opacity of the right hemithorax. A diagnosis of Familial Thoracic Endometriosis Syndrome. Had USS guided lung and pleural biopsy and a thoracostomy tube drainage. She had chemical pleurodesis with tetracycline. Conclusion: Familial thoracic endometriosis syndrome is the diagnosis in a lady of reproductive age whose one of the siblings had earlier present with cyclic or a cyclical recurrent right sided chest pain, cyclical or a cyclical dyspnoea, haemoptysis, cyclical or a cyclical abdominal swelling and peritonitis.