Gambarara M, Ferretti F, Bagolan P, Papadatou B, Rivosecchi M, Lucchetti M C, Nahom A, Castro M
Ospedale Pediatrico Bambino Gesù, Roma, Italia.
Eur J Pediatr Surg. 1999 Aug;9(4):267-70. doi: 10.1055/s-2008-1072261.
Short-bowel syndrome (SBS) either in adults or in children is considered as an indication to small-bowel transplantation (SBTx), particularly in its most severe form with a residual bowel length below 20 cm. Among factors likely to worsen the prognosis, more recent reports also indicate the number of surgical interventions, early onset sepsis and early development of liver disease. We report six cases of ultra-short-bowel syndrome followed from birth to verify the importance of various prognostic factors. In our case series, the male sex is predominating (5:1). Intestinal resection was indicated in 3 patients for multiple intestinal atresias, in 2 for volvulus and in 1 for necrotizing enterocolitis. The length of intestine remaining was invariably less than 20 cm and 2 patients had a preserved ileocecal valve. In most cases, more than 50% of the colon remained. The number of abdominal operations ranged from 1 to 4. In almost all cases (5 of 6), sepsis and hepatopathy developed early. Our experience suggests that rather than depending on the length of intestine remaining or the presence of the ileocecal valve, the prognosis of patients with the extreme-short-bowel syndrome depends on recurrent neonatal onset sepsis and early onset liver impairment. In addition, our case review shows that the extreme-short-bowel syndrome is not necessarily an indication for bowel transplantation.
成人或儿童的短肠综合征（SBS）被视为小肠移植（SBTx）的指征，尤其是在其最严重的形式中，即残余肠长度低于20厘米时。在可能使预后恶化的因素中，最近的报告还指出了手术干预的次数、早期败血症的发作以及肝病的早期发展。我们报告了6例从出生起就进行随访的超短肠综合征病例，以验证各种预后因素的重要性。在我们的病例系列中，男性占主导（5:1）。3例患者因多处肠闭锁接受肠切除，2例因肠扭转，1例因坏死性小肠结肠炎。剩余肠长度始终小于20厘米，2例患者保留了回盲瓣。在大多数情况下，超过50%的结肠保留。腹部手术次数从1次到4次不等。几乎所有病例（6例中的5例）都早期出现了败血症和肝病。我们的经验表明，极短肠综合征患者的预后并非取决于剩余肠长度或回盲瓣的存在，而是取决于新生儿期反复发作的败血症和早期出现的肝功能损害。此外，我们的病例回顾表明，极短肠综合征不一定是肠移植的指征。
