Hakalmaz Ali Ekber, Topuzlu Tekant Gonca
Department of Pediatric Surgery, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Turk Arch Pediatr. 2023 Nov;58(6):572-579. doi: 10.5152/TurkArchPediatr.2023.23090.
Anorectal malformation is a disease with different subtypes and anatomical and functional multisystemic involvement that requires a unique approach in each age group. Anomalies associated with vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities (VACTERL) association require detailed investigation and management. Beginning from the neonatal period, treatment is carried out with different surgical procedures. The clinical course of these patients may be associated with medical problems, accompanying congenital anomalies, perioperative management, or late sequelae. Constipation and fecal-urinary incontinence are the most common problems encountered in long-term follow-up. Renal failure is the most important cause of long-term mortality. In addition, these patients need to be under control until adulthood due to cardiological, spinal, genital, gynecological, and endocrine problems. In this follow-up, many pediatric disciplines such as neonatal intensive care, cardiology, nephrology, gastroenterology, and endocrinology cooperate with pediatric surgeons and pediatric urologists.
肛门直肠畸形是一种具有不同亚型且涉及解剖和功能多系统的疾病,在每个年龄组都需要独特的治疗方法。与脊柱缺陷、肛门闭锁、心脏缺陷、气管食管瘘、肾脏异常和肢体异常(VACTERL)相关的畸形需要详细的检查和管理。从新生儿期开始,采用不同的外科手术进行治疗。这些患者的临床病程可能与医疗问题、伴随的先天性畸形、围手术期管理或晚期后遗症有关。便秘和粪尿失禁是长期随访中最常见的问题。肾衰竭是长期死亡的最重要原因。此外,由于心脏、脊柱、生殖、妇科和内分泌问题,这些患者在成年前都需要接受监测。在这种随访中,新生儿重症监护、心脏病学、肾脏病学、胃肠病学和内分泌学等许多儿科专业与小儿外科医生和小儿泌尿外科医生合作。