Pini Prato Alessio, Gentilino Valerio, Giunta Camilla, Avanzini Stefano, Mattioli Girolamo, Parodi Stefano, Martucciello Giuseppe, Jasonni Vincenzo
Department of Pediatric Surgery, IRCCS G. Gaslini Institute, Genoa 16147, Italy.
J Pediatr Surg. 2008 Apr;43(4):612-9. doi: 10.1016/j.jpedsurg.2007.10.007.
Long-term results after surgical treatment of Hirschsprung disease (HSCR) are not always as good as a surgeon may perceive. Several studies have tried to correlate preoperative features to the surgical outcome of HSCR, but none came to definitive conclusions. Our study is aimed at identifying risk factors of poorer long-term outcome after surgery for HSCR.
One hundred two patients with HSCR were included. Eighty had rectosigmoid aganglionosis and 22 had total colonic aganglionosis (TCSA). Preoperative variables were sex, associated anomalies, delayed meconium passage, preoperative enterocolitis, preoperative bowel obstruction, age at surgery, and number of pull-through procedures performed. Outcome measures were surgical complications, postoperative enterocolitis, perineal excoriations, constipation, continence, psychological self acceptance, and patients' perspectives. These were evaluated with regard to preoperative features and length of aganglionosis.
Results of patients with TCSA proved to be significantly worse than those of patients with classic HSCR (constipation and poor continence excluded). Male patients with TCSA proved to have a significantly higher incidence of complications (100% vs 38.5%) and poor psychological self acceptance (100% vs 46.2%). Patients with associated anomalies (central nervous system) experienced a significantly higher incidence of postoperative constipation (43% vs 13%). Patients who described failure or delayed meconium passage complained of significantly worse continence (28.3% vs 7.1% of fair to poor continence). Preoperative enterocolitis proved to be significantly correlated to postoperative enterocolitis (16.4% vs 37.1%) and perineal excoriations (13.7% vs 30.9%). Patients with preoperative intestinal obstructions complained of significantly worse psychological self acceptance (37.8% vs 12.5%).
Length of aganglionosis has the greatest impact on overall surgical outcome of HSCR. Other minor risk factors have been identified, namely, male sex, associated central nervous system anomalies, failure to pass meconium, and preoperative enterocolitis occurrences. Age at surgery and redo procedures proved not to significantly interfere with the outcome. Our study provides comprehensive and useful data to inform parents and families of a baby with HSCR with regard to expected results and long-term outcomes of surgery basing on preoperative features. Further studies on larger series are strongly recommended.
先天性巨结肠症（HSCR）手术治疗的长期效果并不总是如外科医生所认为的那样理想。多项研究试图将术前特征与HSCR的手术结果相关联，但均未得出明确结论。我们的研究旨在确定HSCR手术后长期效果较差的风险因素。
纳入102例HSCR患者。其中80例为直肠乙状结肠无神经节细胞症，22例为全结肠无神经节细胞症（TCSA）。术前变量包括性别、相关畸形、胎粪排出延迟、术前小肠结肠炎、术前肠梗阻、手术年龄以及进行的拖出术次数。观察指标包括手术并发症、术后小肠结肠炎、会阴擦伤、便秘、控便能力、心理自我接受程度以及患者的看法。根据术前特征和无神经节细胞症的长度对这些指标进行评估。
TCSA患者的结果明显比经典HSCR患者差（不包括便秘和控便能力差）。TCSA男性患者的并发症发生率（100%对38.5%）和心理自我接受程度差的发生率（100%对46.2%）明显更高。伴有相关畸形（中枢神经系统）的患者术后便秘发生率明显更高（43%对13%）。描述胎粪排出失败或延迟的患者控便能力明显更差（大便失禁至排便不畅者中分别为28.3%对7.1%）。术前小肠结肠炎与术后小肠结肠炎（16.4%对37.1%）和会阴擦伤（13.7%对30.9%）明显相关。术前肠梗阻的患者心理自我接受程度明显更差（37.8%对12.5%）。
无神经节细胞症的长度对HSCR的总体手术结果影响最大。已确定其他一些次要风险因素，即男性、伴有中枢神经系统畸形、胎粪排出失败以及术前小肠结肠炎的发生。手术年龄和再次手术未被证明会对结果产生显著干扰。我们的研究提供了全面且有用的数据，以便根据术前特征向患有HSCR的婴儿的父母和家庭告知手术的预期结果和长期效果。强烈建议对更大样本量进行进一步研究。
