Neonatal short bowel syndrome: a cohort study

Background

To date, our knowledge of morbidity and mortality in neonatal short bowel syndrome (SBS) is based on individual case series. Shortcomings of the published literature include long patient recruitment time, selection bias, variable SBS definitions, failure to account for gestational age, and incomplete follow-up. By applying more rigorous methodology, our aim was to determine outcomes of SBS neonates compared with a control group of neonates without SBS.

Methods

A cohort study of all neonates with abdominal pathology requiring laparotomy between January 1, 1997, and December 31, 1998, with observation through July 1, 2001. Short bowel syndrome was defined as patients requiring parenteral nutrition for more than 42 days or residual small bowel length of less than 25% predicted by gestational age. Student's t test, Mann-Whitney U test, and χ² were used where appropriate. Kaplan-Meier curves were used to determine cumulative survival. Covariates important in the development of SBS were examined using forward step-wise logistic regression.

Results

There were 175 patients (with SBS = 40, without SBS = 135) with a mean gestational age of 30.7 ± 4.6 weeks vs 35.9 ± 4.8 weeks, respectively (P < .0005). The patients with SBS suffered significantly more morbidity than the group without SBS in all categories of investigation (surgical complications, septic events, central venous line complications, duration to adaptation and parenteral nutrition independence, cholestasis and liver failure, and duration of hospitalization). The case fatality rate was 37.5% in patients with SBS vs 13.3% in patients without SBS (P = .001). Most of the deaths were caused by liver failure or sepsis and occurred within 1 year from the date of surgery. Presence of an ileostomy (exp(B) = 12.29; P < .0005) and a residual small bowel length less than 50% of the original length (exp(B) = 26.84; P < .0005) were the only 2 variables in a logistic regression analysis found to be independently associated with the development of SBS.

Conclusion

This cohort study clearly illustrates the tremendous morbidity experienced by infants with SBS relative to other surgical neonates. Accurate estimates of the morbidity associated with SBS enables clinicians to appropriately counsel parents, allocate resources and initiate therapeutic trials.     

Comparison of massive vs. repeated resection leading to short bowel syndrome

Short bowel syndrome can result from either a single massive intestinal resection or repeated lesser resections, which might have prognostic implications. The aim of this study was to compare patient populations and outcome of short bowel syndrome caused by massive and repeated resection. The records of 95 adult patients with short bowel syndrome evaluated over a 20-year period were reviewed. Massive resection was performed in 72 patients (76%) and repeated lesser resections in 23 patients (24%). Patients undergoing massive resection were more likely to be more than 70 years of age (26% vs. 9%, P <0.05). Mesenteric vascular disease was more prevalent among patients undergoing massive resection (39% vs. 9%, P <0.05), whereas Crohn’s disease was less prevalent (1% vs. 35%, P <0.05). Distribution of remnant length, presence of the ileocecal junction, and presence of a stoma were similar. Patients undergoing massive resection were more likely to require parenteral nutrition after the first year (56% vs. 23%, P <0.05). Patients with very short remnants (<60 cm) were more likely to receive parenteral nutrition after massive resection (95% vs. 60%, P <0.05). Thirty-day mortality was higher after massive resection (24% vs. 4%, P <0.05). However, those surviving 30 days had similar survival rates at 1 year and 5 years after massive and repeated resections. Patients undergoing massive vs. repeated resections are different with respect to age, underlying condition, and nutritional support needs. These factors may influence overall outcome in short bowel syndrome. The better nutritional prognosis of patients undergoing repeated resection given similar intestinal remnants may be related in part to enhanced intestinal adaptation. Presented at the Fortieth Annual Meeting of The Society for Surgery of the Alimentary Tract, Orlando, Fla., May 16–19, 1999.     

Current short bowel syndrome management: An era of improved outcomes and continued challenges

Prior to the late 1960s, pediatric short bowel syndrome was a frequently fatal disease. Currently, pediatric interdisciplinary bowel rehabilitation centers report very high survival rates. The mortality trends, up-to-date definitions, incidence, causes, and clinical manifestations of short bowel syndrome are reviewed. Emphasis is placed upon the nutritional, medical, and surgical advances that have contributed to the dramatic improvement in outcomes for pediatric short bowel syndrome patients. Recent findings and remaining challenges are highlighted.     

Low-fat diet impairs postresection intestinal adaptation in a rat model of short bowel syndrome

Background: Low-fat diets (LFD) are utilized frequently in patients with short bowel syndrome (SBS). The purpose of this study was to investigate the effects of LFD on intestinal adaptation, enterocyte proliferation, and enterocyte cell death in a rat model of SBS.Methods: Adult male Sprague-Dawley rats were divided into 3 experimental groups: Sham-NC rats underwent bowel transection and reanastomosis and were fed normal chow (NC), SBS-NC rats underwent 75% small bowel resection and were fed NC, and SBS-rats were fed a low-fat diet (SBS-LFD). Parameters of intestinal adaptation, enterocyte proliferation, and enterocyte apoptosis were determined on day 14 after operation.Results: SBS-NC rats showed a significant increase (v Sham-NC) in jejunal and ileal bowel and mucosal weight, mucosal DNA and protein, villus height, and crypt depth. A significant 67% increase in crypt cell proliferation rate and 265% increase in villus enterocyte apoptosis was seen in the ileum of SBS-NC rats compared with control animals (P < .05). SBS-LFD animals showed lower ileal mucosal weight (29%; P < .05), jejunal crypt depth (20%; P < .05), and ileal villus height (21%; P < .05). A significant decrease in villus apoptosis in jejunum (74%; P < .05) and ileum (67%; P < .05) and a decrease in cell proliferation in ileum (35%; P < .05) was seen also after exposure to LFD compared with SBS-NC.Conclusions: In a rat model of SBS, early LFD appears to inhibit parameters of intestinal adaptation. A possible mechanisms for this effect may be decreased cell proliferation. Decreased enterocyte loss via apoptosis, found in this study, may reflect a reduced number of enterocyte.     

Improved survival in a multidisciplinary short bowel syndrome program

Purpose

Pediatric short bowel syndrome (SBS) remains a management challenge with significant mortality. In 1999, we initiated a multidisciplinary pediatric intestinal rehabilitation program. The purpose of this study was to determine if the multidisciplinary approach was associated with improved survival in this patient population.

Methods

The Center for Advanced Intestinal Rehabilitation includes dedicated staff in surgery, gastroenterology, nutrition, pharmacy, nursing, and social work. We reviewed the medical records of all inpatients and outpatients with severe SBS treated from 1999 to 2006. These patients were compared to a historical control group of 30 consecutive patients with severe SBS who were treated between 1986 and 1998.

Results

Fifty-four patients with severe SBS managed by the multidisciplinary program were identified. Median follow-up was 403 days. The mean residual small intestinal length was 70 ± 36 vs 83 ± 67 cm in the historical controls (P = NS). Mean peak direct bilirubin was 8.1 ± 7.9 vs 9.0 ± 7.4 mg/dL in controls (P = NS). Full enteral nutrition was achieved in 36 (67%) of 54 patients with severe SBS vs 20 (67%) of 30 patients in the control group (P = NS). The overall survival rate, however, was 89% (48/54), which is significantly higher than in the historical controls (70%, 21/30; P < .05).

Conclusions

A multidisciplinary approach to intestinal rehabilitation allows for fully integrated care of inpatients and outpatients with SBS by fostering coordination of surgical, medical, and nutritional management. Our experience with 2 comparable cohorts demonstrates that this multidisciplinary approach is associated with improved survival.     

短肠综合征病人的营养支持及肠道代偿治疗

目的探讨短肠综合征病人的营养支持以及肠道代偿、康复治疗体会。方法回顾性分析1986~2005年复旦大学附属中山医院64例短肠综合征病人治疗过程及随访情况,其中26例联合应用生长激素[GH,每天(0.10±0.06)mg/kg]和谷氨酰胺[GLN,每天(0.30±0.17)g/kg]进行肠道促代偿治疗。结果64例病人中死亡6例,存活58例,存活时间3个月至19年,平均(6.6±9.4)年。9例长期接受家庭肠外营养(HPN),13例接受部分PN或肠内营养(EN)支持,完全摆脱PN者36例,平均摆脱PN的时间为(8.6±14.2)个月。26例接受GH GLN治疗的SBS病人,其中9例(34.6%)治疗后近期内完全摆脱PN;8例(30.8%)经治疗后明显减少了PN用量[从每周需要PN支持(6.5±1.0)d下降至(4.2±1.0)d,每周PN需要量从(13.6±5.2)L降至(8.2±3.3)L];9例(34.6%)在治疗后仍依赖PN支持。64例共发生286次各种并发症。结论经过适宜的营养支持和肠道促代偿治疗,大多数短肠综合征病人的残留肠道可充分代偿,完全摆脱PN或减少PN用量,长期健康存活。     

短肠综合征时结肠的代偿研究

目的　观察及评价短肠大鼠结肠代偿性增生及结肠对营养物质吸收的促进作用。　方法　制作切除（80～85）%的超短肠大鼠模型,用百普素(Pepti-2000)做肠内营养（EN）治疗,观察全身营养状况和结肠形态学的改变,并在术后第21天用木糖和15N-甘氨酸混合液对带血管蒂的结肠进行封闭式灌注,观察结肠对水、碳水化合物和氨基酸的吸收情况。　结果　EN组于术后第21天净氮平衡与对照组（CONT）无差异,体重仅比术前减轻(10±18)g。结肠壁明显增厚,皱襞增大增粗,结肠壁的厚度、粘膜厚度、皱襞高度和皱襞表面积与CONT组相比差异具有非常显著性意义（P＜0.01）。EN组与CONT组相比DNA指数1.21±0.11vs.1.01±0.15(P＜0.05),S期细胞百分比（52.6±5.5）%vs.（42.9±4.1）%（P＜0.05）。连续循环灌注3h之后EN组对水、木糖和氨基酸的吸收明显高于CONT组（P＜0.05）。　结论　大鼠结肠在短肠综合征时发生了明显的形态和功能上的代偿。早期适当的肠内营养不但可使超短肠大鼠获得足够营养支持,并且能够促进短肠大鼠结肠代偿。     

短肠综合征的肠内营养支持

目的探讨短肠综合征患者肠内营养支持的临床意义、疗效及注意事项。方法回顾性总结1999至2005年收治的40例短肠综合征患者的临床资料。所有患者均存活至今,并随访2年以上。统计分析其肠内营养用量、费用、脱离肠外营养时间及目前营养状况。结果40例患者平均残存小肠（50．8±29．4）cm,脱离肠外营养平均时间为（29．1±9．2）个月。肠内营养用量为（3284．0±1408．8）kJ／d,其费用显著低于肠外营养（P〈0．01）。目前本组患者平均体质指数为（17．8±3．2）kg,／m^2,血红蛋白（113．3±14．8）g／L,血清白蛋白（35．0±4．1）g／L。平均大便次数为（3．4±1．7）次／d,平均大便量为（720．2±350．3）ml／d。结论肠内营养对于维持短肠综合征患者营养状况、减少并发症具有重要意义,但在具体实施时需掌握方法。     

Serial transverse enteroplasty for short bowel syndrome: a case report

The patient is a 2-year-old boy born with gastroschisis and midgut volvulus that left him dependent on total parenteral nutrition (TPN). At 11 months of age, a Bianchi procedure was performed increasing the total length of bowel from 72 cm to 130 cm. Although he appeared to have sufficient bowel length, he continued to have malabsorption and could only tolerate 10% of his caloric requirement enterally. A barium study found significant dilatation of the lengthened small bowel. At 23 months, we performed a novel bowel lengthening procedure that we have reported previously in an animal model. The serial transverse enteroplasty (STEP) operation increased the 83 cm of dilated and previously lengthened bowel to 147 cm, making the total small bowel length 200 cm. The patient tolerated the procedure well and began to have semisolid bowel movements. Small intestinal absorptive capacity measured by D-xylose absorption showed a substantial increase from 5 to 12 mg/dL (normal range, >20), implying improved but not completely normal small bowel function. This case shows that the STEP procedure increases intestinal length, can be used after a prior Bianchi, and may result in improved intestinal absorptive capacity. The STEP procedure should be considered a surgical option for children with short bowel syndrome.     

短肠综合征患者饮食行为特征及管理的质性研究

目的 深入了解短肠综合征患者饮食行为特征及自我饮食管理方面的体验,以进一步指导和干预患者饮食管理,提高饮食管理依从性.方法 运用质性研究中的现象学方法 ,对15例短肠综合征患者进行面对面、半结构式访谈,采用Colaizzi现象学资料分析法分析访谈资料.结果 短肠综合征患者的饮食行为特征及管理中主要存在4个方面的体验:日...     

Two-hit rat model of short bowel syndrome and sepsis: independent of total parenteral nutrition, short bowel syndrome is proinflammatory and injurious to the liver

Introduction

Infants with short bowel syndrome (SBS) are at a high risk for infectious complications and liver failure. We hypothesized that SBS, independent of total parenteral nutrition, is a proinflammatory state that is magnified by sepsis.

Methods

Sprague-Dawley rats were divided into 2 groups: sham laparotomy (SH, n = 10) or 75% small bowel resection (n = 10). After 14 days, each group underwent a second sham laparotomy (SH/SH and SBS/SH) or cecal ligation and puncture, followed 16 hours later by cecal excision and peritoneal washout (SH/sepsis and SBS/sepsis). Animals were killed 56 hours later.

Results

The SBS rats had higher serum levels of interleukin (IL) 6 vs SH (355 ± 99 vs 104 ± 71 pg/mL, P < .05). Liver injury scores were higher in SBS/sepsis compared with SBS/SH animals (3.7 ± 0.7 vs 1.9 ± 0.3, P < .05). Hepatic messenger RNA levels of IL-6 (12.8-fold change [FC]) and tumor necrosis factor α (5.65 FC) were elevated in SBS vs SH rats; and IL-6 (114 FC), tumor necrosis factor α  (3.87 FC), and Toll-like receptor 4 (7.65 FC) were increased in SBS/sepsis compared with SH/sepsis animals.

Conclusion

Our results suggest that SBS, independent of total parenteral nutrition, is a proinflammatory state and that sepsis induces an exaggerated proinflammatory cytokine response that may play an important role in liver damage and may be mediated by Toll-like receptor 4.     

Neonatal short bowel syndrome outcomes after the establishment of the first Canadian multidisciplinary intestinal rehabilitation program: preliminary experience

Background

A multidisciplinary intestinal rehabilitation team has been in place at our institution for 3 years. Our goal was to compare the preliminary outcomes of neonates with short bowel syndrome before with those after the establishment of our formalized intestinal failure program (Group for the Improvement of Intestinal Function and Treatment [GIFT]).

Methods

We conducted a retrospective analysis of our intestinal failure registry comparing preGIFT (1997-1999) with GIFT (2003-2005) outcomes.

Results

Fifty-four patients (mean gestational age = 33.5 weeks) were included in the GIFT cohort, and 40 patients (mean gestational age = 30.7 weeks) formed the preGIFT cohort. Overall mortality rates (33.3% vs 37.5%, P = .84) were equivalent in the 2 cohorts, although fewer patients died of liver failure after the establishment of the GIFT. Among those with liver failure, the mortality in the preGIFT group was 9/10 as compared with that of 6/13 in the GIFT group (P = .03). The decrease in liver-related deaths was partly attributable to earlier referral for and increased survival to transplant (4 for the GIFT group vs 0 for the preGIFT group).

Conclusions

Analysis of the preliminary outcomes of the GIFT program suggests that the natural history of neonatal short bowel syndrome remains unaltered to date despite a coordinated approach to care. However, improved communication and integration with the transplant service have resulted in earlier assessment, increased rates of transplantation, and decreased mortality from liver failure.     

短肠综合征肠道再手术分析

目的 分析手术后短肠综合征（SBS）病人再手术,降低SBS再手术率。方法 回顾性总结2001年1月至2010年12月南京军区南京总医院解放军普通外科研究所收治的短肠综合征病人198例,其中因肠道原因而再次手术治疗病人76例（男59例,女17例）,年龄5~62（37.98±13.75）岁,残存小肠长度0~150（72.53±41.83）cm。结果 76例SBS接受再次手术124（1.63±0.4）例次,再次手术以肠造口还纳（43例次）、腹腔感染引流（28例次）及病变肠袢切除吻合（肠梗阻13例次、肠瘘12例次,蛋白质丢失病7例次、肠穿孔/肠坏死5例次）为主。SBS病人因肠道原因再手术率为38.4%。结论 SBS病人因肠道原因再手术率较高,选择合理的首次手术方式,有助于降低再手术率。     

短肠综合征并肠外瘘的诊治(附32例分析)

目的研究短肠综合征合并肠外瘘的诊断与治疗方法的特点与规律。方法1995~2005年共收治剩余小肠<100 cm的肠外瘘病人32例,就肠外瘘发生原因、部位、治疗方法和肠康复方法进行分析。结果治愈20例,死亡6例,6例好转后中断治疗出院。病人残存的肠管平均(58.03±28.30)cm。28例接受了肠内营养,其中9例未进行肠康复治疗,肠管平均长度为(52.8±31.5)cm,肠内营养平均恢复时间为(129.6±89.8)d;19例接受了肠康复治疗,肠管平均长度(64.1±19.2)cm,肠内营养平均恢复时间为(61.8±54.0)d。结论短肠综合征合并肠外瘘的主要疾病为肠扭转,部位多在吻合口,原因是坏死范围较大,切除界限不易判断。早期使用生长抑素有减少肠液分泌的作用,还可减少短肠综合征急性期的腹泻症状。后期使用生长激素有促进肠外瘘自愈和肠康复的双重作用。     

The use of oral tacrolimus in a case of short bowel syndrome

Achieving adequate oral immunosuppression in a renal transplant patient who develops short bowel syndrome provides a significant challenge. We report a case where oral tacrolimus has been used to provide immunosuppression in an established renal transplant patient who developed short bowel syndrome secondary to mesenteric infarction. After reviewing the literature, we conclude that tacrolimus can be used as first-line immunosuppression in patients with short bowel syndrome.     

Evaluation of intestinal absorption after longitudinal intestinal lengthening for short bowel syndrome

Purpose

The aim of this study was to answer if the longitudinal intestinal lengthening and tailoring (LILT) by Bianchi, modified by Aigrain, can allow the child to be weaned from parenteral nutrition (PN) and if the length of the bowel after the procedure can influence the results of the absorption test such as Schilling or d-xylose test.

Patients and Methods

We reviewed the files of 7 children who have had LILT from 1980 to 2003. We performed to explore 2 intestinal function tests: the d-xylose and the Schilling tests. Both were performed early (during the first year after the procedure) and late (during the second year) after the LILT. We used the χ² and Bartlett's correlation tests for statistical analysis.

Results

There were 6 boys and 1 girl. The surgical indication was short bowel syndrome with parenteral nutrition owing to multiple intestinal atresia (2 cases), severe necrotizing enterocolitis with volvulus (1 case), necrotizing enterocolitis (1 case), intestinal atresia with gastroschisis (2 cases), and volvulus owing to malrotation (1 case). The length of the bowel was significantly different before and after LILT (P < .0001). After LILT, the length of the bowel was significantly correlated with the percentage of PN on energy at 6 months (P = .02) and at 12 months (P = .001). Moreover, the length of the bowel after the procedure was significantly correlated with the results of the d-xylose test during the first year (P = .002) but not with the results after the second year. The length after lengthening influenced neither the results of the Schilling test during the first nor those of the second year after. Four patients were weaned from the PN 21 months in average after the LILT (57%); 1 was not because we had only a 2-month follow-up. The average follow-up was 111 (5 months; range, 4- 206).

Conclusion

Longitudinal intestinal lengthening and tailoring for short bowel syndrome is a good option to allow children to be weaned from the PN. The length of the bowel after the procedure can influence the absorption test such as d-xylose during the first postoperative year but not during the second and does not influence the Schilling test. We think it is not necessary to perform these tests during the follow-up of these patients.     

短肠综合征康复治疗的实验与临床研究

目的 研究生长激素对大部小肠切除后残存小肠粘膜增殖活性的影响;评价短肠康复治疗的临床疗效。方法 利用病理图象分析、流式细胞分析、免疫组化法和ＲＴ－ＰＣＲ法观察比较对照组（假手术组）、短肠组（８０％小肠切除）和生长激素组（８０％小肠切除加１Ｕ．ｋｇ＾－１．ｄ＾－１生长激素皮下注射２８ｄ）ＳＤ大鼠小肠粘膜的增殖状况。观察肠康复治疗（肌注生长激素８～１２Ｕ．ｄ＾－１加静脉滴注加谷氨酰胺０．６ｇ．ｋｇ＾－     

Mesenteric thrombosis causing short bowel syndrome in nephrotic syndrome

Nephrotic patients are at risk of developing venous and arterial thrombotic complications. Pulmonary embolism due to affected deep leg veins is by far the most common event. Renal or cerebral vein thromboses have been described. Thrombosis of arterial vessels is less frequent. Mesenteric infarction is a rare but severe complication in patients with nephrotic syndrome (NS). We report a 7-year-old boy with a steroid-dependent (SD) NS and a homozygous mutation of methylenetetrahydrofolate reductase, increasing the risk of thromboembolic events. He developed a thrombosis of his superior mesenteric artery during his ninth relapse, which was responsible for a necrosis of 240 cm of his small bowel, necessitating resection of necrotic parts and double external ostomy diversion. Remission was achieved with pulse prednisolone therapy. Corticoids were reduced over 4 months progressively. Oral cyclosporin A (CyA) was initiated for long-term treatment. Due to a short bowel syndrome with severe malabsorption, even oral administration of 22.5 mg/kg per day CyA did not lead to sufficient plasma levels. Intravenous cyclophosphamide pulse therapy over 6 months led to a complete remission. No relapse occurred over a period of more than 5 months after the last cyclophosphamide pulse. Anticoagulation and screening for increased susceptibility for thrombotic events are necessary in every nephrotic patient. Intravenous cyclophosphamide pulse therapy is a useful alternative in SDNS with impaired intestinal absorption of applied immunosuppressive drugs.