Abstract：
BACKGROUND: Individuals diagnosed with connective tissue disorders (CTD) are known to be predisposed to incisional hernia formation. However, there is a scarcity of data on outcomes for these patients undergoing hernia repair. We sought to describe our outcomes in performing abdominal wall reconstructions in these complex patients.
METHODS: Adult patients with CTD undergoing open, elective, posterior component separation with permanent synthetic mesh at our institution from January 2018 to October 2022 were queried from a prospectively collected database in the Abdominal Core Health Quality Collaborative. We evaluated 30-day wound morbidity, perioperative complications, long-term hernia recurrence, and patient-reported quality of life.
RESULTS: Twelve patients were identified. Connective tissue disorders included Marfan\'s n = 7 (58.3%), Loeys-Dietz syndrome n = 2 (16.7%), Systemic Lupus Erythematosus n = 2 (16.7%), and Scleroderma n = 1 (8.3%). Prior incisions included three midline laparotomies and nine thoracoabdominal, mean hernia width measured 14 cm, and 9 were recurrent hernias. Surgical site occurrences (SSOs) were observed in 25% of cases, and 16.7% necessitated procedural intervention. All twelve patients were available for long-term follow-up, with a mean of 34 (12-62) months. There were no instances of reoperation or mesh excision related to the TAR procedure. One patient developed a recurrence after having his mesh violated for repair of a new visceral aneurysm. Mean HerQLes scores at 1 year were 70 and 89 at ≥ 2 years; Mean scaled PROMIS scores were 30.7 at 1 year and 36.3 at ≥ 2 years.
CONCLUSIONS: Ventral hernia repair with TAR is feasible in patients with connective tissue disorder and can be a suitable alternative in patients with large complex hernias.
METHODS: Adult patients with CTD undergoing open, elective, posterior component separation with permanent synthetic mesh at our institution from January 2018 to October 2022 were queried from a prospectively collected database in the Abdominal Core Health Quality Collaborative. We evaluated 30-day wound morbidity, perioperative complications, long-term hernia recurrence, and patient-reported quality of life.
RESULTS: Twelve patients were identified. Connective tissue disorders included Marfan\'s n = 7 (58.3%), Loeys-Dietz syndrome n = 2 (16.7%), Systemic Lupus Erythematosus n = 2 (16.7%), and Scleroderma n = 1 (8.3%). Prior incisions included three midline laparotomies and nine thoracoabdominal, mean hernia width measured 14 cm, and 9 were recurrent hernias. Surgical site occurrences (SSOs) were observed in 25% of cases, and 16.7% necessitated procedural intervention. All twelve patients were available for long-term follow-up, with a mean of 34 (12-62) months. There were no instances of reoperation or mesh excision related to the TAR procedure. One patient developed a recurrence after having his mesh violated for repair of a new visceral aneurysm. Mean HerQLes scores at 1 year were 70 and 89 at ≥ 2 years; Mean scaled PROMIS scores were 30.7 at 1 year and 36.3 at ≥ 2 years.
CONCLUSIONS: Ventral hernia repair with TAR is feasible in patients with connective tissue disorder and can be a suitable alternative in patients with large complex hernias.
摘要：
背景：已知诊断为结缔组织疾病（CTD）的个体易感切口疝形成。然而,这些接受疝修补术的患者缺乏结局数据.我们试图描述我们在这些复杂患者中进行腹壁重建的结果。
方法：接受开放性CTD的成年患者，选修,我们从2018年1月至2022年10月在我们机构使用永久性合成网进行的后部成分分离从“腹部核心健康质量协作”中前瞻性收集的数据库中进行了查询.我们评估了30天的伤口发病率，围手术期并发症,长期疝复发,和患者报告的生活质量。
结果：确定了12例患者。结缔组织疾病包括Marfann=7(58.3%)，Loeys-Dietz综合征n=2（16.7%），系统性红斑狼疮n=2（16.7%），硬皮病n=1(8.3%)。先前的切口包括三个中线开腹手术和九个胸腹，平均疝宽度14厘米，9例为复发性疝。在25%的病例中观察到手术部位发生(SSO)，16.7%的人需要程序性干预。所有12例患者均可进行长期随访，平均为34（12-62）个月。没有与TAR手术相关的再次手术或网片切除的实例。一名患者因修复新的内脏动脉瘤而破坏网状物而复发。1年时的平均HerQLes评分分别为70分和≥2年时的89分；1年时的平均PROMIS评分为30.7分，≥2年时的平均PROMIS评分为36.3分。
结论：TAR腹侧疝修补术在结缔组织障碍患者中是可行的，在大型复杂疝患者中是一种合适的替代方法。
方法：接受开放性CTD的成年患者，选修,我们从2018年1月至2022年10月在我们机构使用永久性合成网进行的后部成分分离从“腹部核心健康质量协作”中前瞻性收集的数据库中进行了查询.我们评估了30天的伤口发病率，围手术期并发症,长期疝复发,和患者报告的生活质量。
结果：确定了12例患者。结缔组织疾病包括Marfann=7(58.3%)，Loeys-Dietz综合征n=2（16.7%），系统性红斑狼疮n=2（16.7%），硬皮病n=1(8.3%)。先前的切口包括三个中线开腹手术和九个胸腹，平均疝宽度14厘米，9例为复发性疝。在25%的病例中观察到手术部位发生(SSO)，16.7%的人需要程序性干预。所有12例患者均可进行长期随访，平均为34（12-62）个月。没有与TAR手术相关的再次手术或网片切除的实例。一名患者因修复新的内脏动脉瘤而破坏网状物而复发。1年时的平均HerQLes评分分别为70分和≥2年时的89分；1年时的平均PROMIS评分为30.7分，≥2年时的平均PROMIS评分为36.3分。
结论：TAR腹侧疝修补术在结缔组织障碍患者中是可行的，在大型复杂疝患者中是一种合适的替代方法。
