Surgical management of a cecal adenocarcinoma in a young man affected by fibrodysplasia ossificasn progressiva (FOP)

The video shows the surgical treatment of a peculiar case recently observed. A 36 years old man affected by Fibrodysplasia Ossificans Progressiva (FOP) was discovered a cecal tumour. FOP is a severely disabling inherited disorder of connective tissue characterized by congenital bone malformation associated to spontaneous or trauma induced progressive ossification of striated muscles and soft tissues including tendons, ligaments and skeletal muscle [1]. At the time of observation no case of abdominal surgery had been reported in fellows affected by FOP, so that great concern was raised about the correct management. His breathing was mainly depending on the diaphragm motility as all the ribs joints were ossified. Ossification of the right shoulder and elbow forced the right arm to hang over the right abdomen precluding the possibility of a laparotomy. For this reason as well as to avoid worsening breathing dynamics, we opted for laparoscopy. General anaesthesia had already been described with no particular shrewdness if not for the need of a nasotracheal intubation under bronchoscopy [2,3]. All trocars where positioned on the abdominal midline, entailing some technical difficulties in achieving proper tissue retraction. After completing dissection a 6 cm laparotomy was created joining existing trocar incisions. Right hemicolectomy was completed with an ileotransverse latero-lateral stapled anastomosis. Recovery was uneventful. Three months after surgery the patient is disease free, fully recovered to his preexisting clinical conditions. Surgical scars have consolidated, no additional heterotopic bone appeared so far. The description of this first case of major abdominal surgery in a fellow with an advanced stage of FOP allows to conclude that major abdominal surgery, with minor expedients added to a sufficient experience in minimally invasive techniques, is not contraindicated. References 1. Kaplan FS, Le Merrer M, Glaser DL, Pignolo RJ et al. Fibrodysplasia ossificans progressive. Best Practice & Research Clinical Rheumatology, 2008; 22:191-205 2. Tumololo M, Moscatelli A, Soilvestri G. Anaesthetic management of a child with fibrodysplasia ossificans progressiva. Br J Anaesth, 2006; 97: 701-3. 3. Vashisht R, Prosser D. Anesthesia in a child with fibrodysplasia ossificans progressiva. Pediatric Anesthesi, 2006; 16: 684-688.