Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous disorder. Using a customized targeted exome-sequencing system we identified nonsense mutations in TNXB in a patient who had recurrent gastrointestinal perforation due to tissue fragility. This case highlights the utility of targeted exome sequencing for the diagnosis of congenital diseases showing genetic heterogeneity, and the importance of attention to gastrointestinal perforation in patients with tenascin-X deficient type EDS.
Obturator hernia repair has traditionally been performed via an intra-abdominal approach, although laparoscopy is also emerging as a feasible alternative. On the other hand, the Kugel method is a minimally invasive and effective form of repair of groin hernia, but there have been few reports on its use for an incarcerated obturator hernia. We describe how we used the Kugel method to repair an obturator hernia in two patients. Both patients presented with acute intestinal obstruction, necessitating emergency surgery. Via a preperitoneal approach, the impacted obturator hernia was carefully released and the obturator canal defect was repaired with a Kugel patch. One patient recommenced oral intake on postoperative day (POD) 1, and was discharged on POD 5. The other patient's postoperative course was complicated by ileus, prolonging the hospital stay to 14 days. There has been no sign of recurrent disease for 6 and 21 months, respectively. The Kugel method offers several advantages, such as a short operative time (76-82 min), small scar (3 cm), and early postoperative ambulation (POD 1), thus minimizing the hospital stay. Further study is needed to confirm the usefulness of this procedure for an incarcerated obturator hernia.
An 82-year-old woman with lower abdominal pain was referred to our hospital. Her symptoms and CT suggested strangulated ileus. An emergency laparoscopy-assisted operation was performed. The laparoscopy showed bloody ascites in the cavitas pelvis and strangulated ileum obstruction caused by an intrauterine device (IUD). We resected the annular part of the IUD at outside of the abdomen. Although the strangulation was relieved, the color of the strangulated ileum did not improve. Therefore, we resected the necrotic bowel. The patient had an uneventful postoperative recovery and was discharged on the eighth postoperative day. We suppose the IUD uterine perforation in this patient was caused by uterine atrophy and uterine contraction. A strangulated ileus into an IUD is rare. Older female patients who use IUDs have a risk of acute abdominal pain. Therefore, it is important to interview patients regarding the IUD insertion or removal history.
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