Spontaneous rupture of the colon sigmoid following spontaneous recurrent pneumothorax in a patient with Marfan syndrome.

Background: Spontaneous ruptures of the colon and rectum are very rare clinical entities and always require laparotomy. In such cases, despite meticulous exploration an underlying pathology cannot be revealed and most reports in the world literature relate spontaneous colon ruptures with unusual connective tissue disorders such as Marfan or Ehlers-Danlos syndrome. Connective tissue diseases affect the structure and the synthesis of elastin and type III collagen, resulting in various systemic disorders. Among the several pulmonary manifestations, spontaneous pneumothorax is observed in about 5% of patients expressing one of the above syndromes. Case Report: In the following report we present a unique case of spontaneous rupture of the sigmoid colon secondary to spontaneous recurrent contralateral pneumothorax. Conclusions: The aim of this presentation is to raise the level of suspicion in every surgeon and gastroenterologist to include connective tissue disorders in their differential diagnosis in any case of spontaneous colon rupture and, if found, to follow further diagnostic procedures in anticipation of any other visceral complications.