A successfully operated case of a rare type of incarcerated diaphragmatic hernia / Bochdalek-hernia egy felnottben

The authors present a successfully operated case of a Bochdalek hernia in an adult. This type of diaphragmatic hernia mostly occurs in infants, it is a rarity in adults. A 41-year-old woman was admitted with emergency because of right upper abdominal and dorsal pain. In her previous history, there was not any trauma or known congenital diaphragmatic defect. Her laboratory tests were normal except for anaemia. Chest X-ray was negative, abdominal X-ray showed dilated bowels between the liver and the diaphragm with suspicion of free abdominal air. Chest and abdominal computed tomography scan revealed a right dorsal diaphragmatic hernia with an incarcerated part of small intestine. Physical examination revealed no abdominal tenderness, but she had severe dorsal and upper abdominal pain. She underwent an urgent operation. An abdominal approach (upper horizontal laparotomy) was performed. No hernia sac was present. Approximately 40 cm incarcerated ileum was removed from the thoracic cavity. Due to the immediate expansion of the lungs, thoracostomy tube was not inserted. The diaphragm was sutured, the necrotised small bowel was resected and an end-to-end anastomosis was performed. During the post-operative period, small pneumothorax occurred, but no intervention was needed, it healed spontaneously. The patient was discharged on the 10th day after operation.

Cronkhite­Canada syndrome / Cronkhite-Canada-szindróma.

Összefoglaló. A Cronkhite-Canada-szindróma egy extrém ritka, nem öröklodo, gyomor-bél rendszeri polyposissal, fehérjeveszto enteropathiával és ectodermalis elváltozásokkal járó megbetegedés. A világon eddig összesen körülbelül 500 esetet jegyeztek fel. Az etiológia pontosan nem tisztázott, hátterében elsosorban autoimmun folyamatot feltételeznek. A diagnózis a páciens kórtörténetén, a fizikális vizsgálaton, az endoszkópos képen és a szövettani leleten alapul. A jelen közleményben egy 71 éves férfi beteg esetét mutatjuk be. A klinikai kép és az elvégzett vizsgálatok alapján a tünetek hátterében Cronkhite-Canada-szindrómát igazoltunk, majd a szakirodalomban leggyakrabban alkalmazott kombinált protonpumpagátló, kortikoszteroid és meszalazin adását vezettük be, illetve táplálásterápiát alkalmaztunk. Tudomásunk szerint Cronkhite-Canada-szindrómás beteg esete Magyarországon elsoként kerül ismertetésre. Orv Hetil. 2021; 162(11): 432-438. Summary. Cronkhite-Canada syndrome is an extremely rare, noninherited disease, characterized by gastrointestinal polyposis, protein-losing enteropathy and ectodermal abnormalities. Approximately 500 cases have been reported worldwide. The aetiology is unknown, most probably autoimmune mechanisms may be involved. The diagnosis is based on patient history, physical examination, endoscopic findings and histology. Here we report the case of a 71-year-old male, diagnosed with Cronkhite-Canada syndrome. The treatment consisted of proton-pump inhibitor, corticosteroids, mesalazin and nutritional therapy. To the best of our knowledge, this is the first report of Cronkhite-Canada syndrome in Hungary. Orv Hetil. 2021; 162(11): 432-438.