The Pentalogy of Cantrell (PC) is a rare association of defects involving the lower sternum, abdominal wall, diaphragm, pericardium and heart. We report two rare cases of the PC (variant form), showing fatal progression. Case 1 only survived two hours because of severe cardio-respiratory failure. Physical examination showed midline abdominal and thoracic defects, ectopic heart, pericardial defect, diaphragmatic defect, bilateral undescended testis, scoliosis, and adherence between left upper limb and trunk. In addition, the autopsy revealed diaphragmatic agenesia, intraabdominal testis, bilateral lung hypoplasia and lymphocytic meningitis. Case 2 only survived 15 minutes. In addition to the physical findings, including lower sternal defect, ectopic heart, epigastric omphalocele and scoliosis, the autopsy showed left diaphragmatic agenesia, pericardial agenesia, bilateral lung hypoplasia, deformed rib cage, anterior thoracic myeloschisis, adreno-hepatic fusion, left renal agenesia, meckel diverticulum and multiple accessory spleens. When comparing with other cases of PC, the concurrence of bilateral intraabdominal testis and lymphocytic meningitis in case 1, and adreno-hepatic fusion, anterior myeloschisis, meckel diverticulum, multiple accessory spleens, and renal agenesia in case 2 have not been described previously.
We report a 5-year-old patient with phytobezoar mimicking acute appendicitis preoperatively. During laparotomy, it was detected that terminal ileum was obstructed by several fragments of rubbery material. Bezoar was milked into the large bowel, and phytobezoar including tangerine residues was evacuated via appendix stump because of severe distended cecum, and high risk of the anastomotic leakage and intraperitoneal contamination following enterotomy of the inflamated and ischemic ileum. Postoperative course was uneventful. To date, such a procedure has not been described. We suggest that milking of vegetable fibers into the cecum and then emptying via appendix stump may be an alternative treatment of phytobezoar localizing in terminal ileum.