Núñez R, Cabrera R, Agulla E, Moreno C, Serrano A, Ortiz C, Blesa E
Servicio de Cirugía Pediátrica, Hospital Universitario Materno-Infantil Infanta Cristina, Badajoz.
Cir Pediatr. 2001 Apr;14(2):76-81.
During 1994 through 1999, we have treated five patients (3 boys, 2 girls) with total colonic aganglionosis (TCA) and ileal involvement. In three of them we performed a diverting ileostomy in the neonatal period and at the age of four and five months respectively in the remaining two patients, due to intestinal obstruction. In these two last patients a diagnosis of Hirschsprung's disease was made by anorectal manometry and rectal biopsies in the neonatal period. During laparotomy, a cutaneous ileostomy was created in all patients at the distal end of normal ileum, which was 30 to 110 cm (mean = 71 +/- 37 cm) from the ileocecal valve. After operation, a short bowel syndrome developed in three patients causing fluid and nutritional problems that required prolonged total parenteral nutrition (TPN). The definitive operative repair is performed at 4.5 to 14 months (mean = 8 +/- 3.7 months) by a 12 to 20 cm side-to-side ileocolostomy created between the ileum and aganglionic ascending color (Boley procedure) and ileorectal primary anastomosis (Rehbein procedure) using a circular stapler. Rectal dilatation, irrigation of the colon with saline, loperamide hydrochloride and resincholestyramine were begun in all patients postoperatively. Oral antibiotic prophylaxis, was given to the three patients who suffered from SBS. Oral feedings with semielemental diet were tolerated once stools were semiformed and TPN was discontinued at 8 to 34 days (mean = 21 +/- 1.7 days). postoperatively. After the definitive operation, enterocolitis developed in two patients, requiring one of them a short hospitalization during the episode. A possible explanation for the low incidence of enterocolitis in this series is the systematic postoperative use of irrigations of the colon with saline in all patients. These five patients have been followed-up for growth, development, bowel habit and continence. Follow-up has ranged from 15 to 62 months (mean = 32.2 +/- 19.2 months). Presently, all patients in this series have full enteral feeding and one to three bowel movements per day, with formed or semiformed stools. No patients is incontinent of stool. The patient's body weights (74 to 93%) and heights (89 to 92%) for their age were below average (four patients) or within normal range (one patient). Hemoglobin levels are within normal range in all patients. One patient has iron deficiency and another one has serum ferritin concentration below normal. Three patients have folic acid deficiency. Vitamin B12 absorption is normal in all patients. Although we can not conclude this is a better procedure than others, with is use we have obtained satisfactory results, with an excellent survival, scanty morbidity, a rapid return of bowel function and continence, and an acceptable physical development. During long-term follow-up, patients mus be evaluated for iron and folic acid deficiency.
1994年至1999年期间,我们治疗了5例患有全结肠无神经节症(TCA)并累及回肠的患者(3名男孩,2名女孩)。其中3例患者在新生儿期行转流性回肠造口术,其余2例患者分别在4个月和5个月时因肠梗阻行该手术。在这最后2例患者中,新生儿期通过肛门直肠测压和直肠活检确诊为先天性巨结肠。剖腹手术时,在所有患者正常回肠远端距回盲瓣30至110厘米(平均 = 71 ± 37厘米)处做皮肤回肠造口术。术后,3例患者出现短肠综合征,导致液体和营养问题,需要长期全胃肠外营养(TPN)。在4.5至14个月(平均 = 8 ± 3.7个月)时进行确定性手术修复,采用12至20厘米的回肠与无神经节的升结肠侧侧吻合术(Boley手术),并使用圆形吻合器进行回直肠一期吻合术(Rehbein手术)。所有患者术后均开始进行直肠扩张、用盐水冲洗结肠、使用盐酸洛哌丁胺和消胆胺。3例患有短肠综合征的患者接受口服抗生素预防。一旦粪便成形且TPN在术后8至34天(平均 = 21 ± 1.7天)停用,患者即可耐受半要素饮食的口服喂养。确定性手术后,2例患者发生小肠结肠炎,其中1例在此期间需要短期住院治疗。本系列小肠结肠炎发病率低的一个可能原因是所有患者术后均系统地使用盐水冲洗结肠。对这5例患者进行了生长、发育、排便习惯和控便情况的随访。随访时间为15至62个月(平均 = 32.2 ± 19.2个月)。目前,本系列所有患者均完全经口喂养,每天排便1至3次,粪便成形或半成形。无患者大便失禁。患者的体重(74%至93%)和身高(89%至92%)低于同龄人平均水平(4例患者)或在正常范围内(1例患者)。所有患者血红蛋白水平均在正常范围内。1例患者缺铁,另1例患者血清铁蛋白浓度低于正常。3例患者有叶酸缺乏。所有患者维生素B12吸收正常。虽然我们不能得出这一手术比其他手术更好的结论,但使用该手术我们获得了满意的结果,包括良好的生存率、低发病率、肠道功能和控便功能快速恢复以及可接受的身体发育。在长期随访中,必须对患者进行缺铁和叶酸缺乏的评估。