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目的 总结6例短肠综合征病人肠管倒置手术失败的教训，强调合理选择手术适应证的重要性和必要性，提出短肠综合征合理的治疗策略，方法 回顾性分析6例短肠综合征病人，均在外院接受肠管倒置手术，除1例为结肠倒置外，其余均为小肠倒置，倒置肠管长度从10～50cm不等，5例小肠倒置手术均在广泛肠切除的同时进行，术后1例出现吻合口瘘，4例出现肠梗阻。结果 1例病人因肝功能衰竭于术后5年死亡，1例病人放弃治疗，1例行肠瘘及倒置小肠切除治愈，2例行倒置肠管切除治愈，1例行倒置肠管复位治愈。治愈病人依靠普通饮食和部分肠内营养支持维持正常营养状况。结论 短肠综合征病人应首选肠康复治疗和小肠移植。只有极少数病人适宜进行肠倒置手术，手术时倒置肠管不应超过10cm，手术时机应在广泛肠切除2年以后进行．手术时应注意倒置肠管的血液供应，避免肠缺血。     

Peritoneal drainage under local anesthesia for perforations from necrotizing enterocolitis

During 1974 and 1975, five newborn infants weighing between 760 and 1600 g developed severe intraabdominal complications of NEC due to necrotic bowel with perforation and peritonitis. Because these tiny neonates were very ill with sepsis and other severe medical problems, no attempt at laparotomy under general anesthesia was carried out. Instead, using local anesthesia, the contaminated peritoneal cavity was drained via a small incision, usually in the right lower quadrant. This permitted evacuation of air, pus, and stool. In all babies there was improvement evident in the abdomen within one wk, although two of the five newborns died from other causes. The three survivors are well and have normally functioning intestinal tracts 1-2 yr following surgery. One of these surviving babies developed a bowel stricture which subsequently required resection. We feel this method is preferable in the handling of the tiny sick neonate with a bowel perforation from NEC.     

A population-based study of the outcome after small bowel atresia/stenosis in New South Wales and the Australian Capital Territory, Australia, 1992-2003

Purpose

The purpose of the study was to describe the incidence, epidemiology, and survival of infants with small bowel atresia/stenosis in New South Wales (NSW) and the Australian Capital Territory (ACT), Australia.

Methods

A population-based cohort study was conducted of infants diagnosed with small bowel atresia/stenosis in NSW and the ACT from 1992 to 2003. Data were obtained from the prospectively collated NSW and ACT Neonatal Intensive Care Units' data collection. Individual risk factors for mortality were assessed using the χ² test.

Results

The incidence of small bowel atresia/stenosis in NSW and the ACT was 2.9 per 10,000 births. Of 299 infants identified with small bowel atresia, 13 were stillborn. Of the 286 live born infants, most (52%) were delivered preterm (<37 weeks' gestation) with an 87% survival, whereas 48% were term with a 98% survival. More than half the infants (54%) had an associated birth defect. The overall mortality was 8%. Prematurity and low birth weight were identified as independent risk factors for mortality (P < .001).

Conclusions

This study of small bowel atresia/stenosis provides population-based outcomes for clinicians and families. It is important to investigate infants with small bowel atresia for associated birth defects. Although the mortality rate has decreased over the last 50 years, it remains substantial at 8% and is higher in premature and low birth weight infants.     

Gastroschisis: primary closure or Silon pouch

There has been a substantial increase in the incidence of gastroschisis in the last few years, and total parenteral nutrition has enabled many of these neonates to survive the long post-operative course. From 1969 to 1976 inclusive, 44 neonates with gastroschisis were treated with either primary closure or the application of a Silon pouch. Fifteen neonates were managed by primary closure. Three of these neonates developed clinical sepsis, but no other intraabdominal complications ensued. There were two deaths (13%) in the early postoperative period, due to CNS hemorrhage and aspiration. This gorup of 15 babies had 17 abdominal operations and the survivors averaged 32 days of parenteral intravenous nutrition before complete oral alimentation could be successfully started. Two ventral hernias required late repair. Twenty-nine neonates were managed primarily with a Silon pouch. Eighteen of these newborns developed sepsis, three Silon sacs had to be removed because of necrotic bowel within, and two other infants developed small bowel fistulae. There were 12 deaths (35%). These 12 infants had prolonged intestinal malfunction that lasted an average of 67 days. This group of 29 babies had 64 abdominal operations and the survivors averaged 46 days of parenteral intravenous nutrition prior to full oral feeds. Four ventral hernias required later repair. The comparison of results in these two groups of newborns reveals a striking difference in their post-operative course and survival. Although the Silon sac has until now become the accepted surgical procedure for gastroschisis, these results conclusively indicate the advantage of primary reduction and closure of the abdominal wall defect.     

Small Bowell Malignant Melanoma-Report of Three Cases and Review of Literature

Malignant melanomas have a predilection to metastasize to the small bowel. Three patients with malignant melanoma involving the small bowel are reported. Two patients were operated on for small bowel obstruction and the third for gastrointestinal bleeding with anemia. Two patients remained well 6 month and 2 years, respectively, after surgery. One patient died of metastatic cerebral melanoma 6 months postoperatively.

One should suspect small bowel metastasis in every patient with malignant melanoma in his past medical history, who presents with recent changes in bowel habits, intestinal obstruction or gastrointestinal bleeding. Preoperative assessment can only raise the suspicion, even with advanced imaging methods: capsule endoscopy, enteroscopy, CT or PET-CT. The only therapeutic procedure is surgical resection, offering both short term survival as well as an improvement in the quality of life. Although prognosis is dismal there are factors associated with prolonged survival: complete surgical resection with no residual primary or metastatic tumor, so-called primary small bowel tumors in patients aged more then 60 years, LDH < 200 U/L, lack of tumor spread in mesenteric lymph nodes.     

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.     

Lethal short-bowel syndrome

Infants with short-bowel syndrome are difficult to manage. Despite supportive measures with parenteral nutrition and surgery to lengthen remaining bowel or increase functional absorptive surface area, the outcome for many of these infants is poor. We have reviewed a series of seven infants diagnosed with severe short bowel. Causes included volvulus (3), multiple atresias (2), and total intestinal aganglionosis (2). Survival time ranged from 15 days to 8 months. During the hospital course, each infant underwent one to three operative procedures to diagnose and manage the short bowel and all received total parenteral nutrition (TPN) ranging from 10 days to 6 months. One infant died of liver failure and two others developed significant liver dysfunction secondary to TPN. Most infants remained hospitalized until their death. Death occurred at an average of 9 weeks following the diagnosis of short-bowel syndrome. This review suggests that infants with less than 6 cm of small bowel beyond the Ligament of Treitz will inevitably die of their disease or treatment complications. Until bowel transplant becomes a viable alternative, operative intervention and nutritional support may prolong survival but will not change the outcome of these infants and will only contribute to additional morbidity. A decision to withhold further therapy would be reasonable at the time the diagnosis is established.     

Gastroschisis, atresia, dysmotility: surgical treatment strategies for a distinct clinical entity

Purpose

Intestinal atresia (IA) occurs in 10% to 20% of infants born with gastroschisis (GS). We describe a distinct subset of these children with severe dysmotility, without mechanical intestinal obstruction, and with adequate intestinal length for enteral nutrition and propose possible treatment strategies.

Methods

A total of 177 patients with GS managed at a single institution between 1993 and 2007 were retrospectively reviewed.

Results

Twenty-one (12%) patients had IA. Six (29%) did well, with gradual progression to full diet; 1 died at birth because of complete midgut infarction; 1 died of necrotizing enterocolitis; 4 with short bowel syndrome (SBS) (small bowel length, 30-41 cm) died of liver failure because of total parenteral nutrition (3) or sepsis (1). Nine (43%) were felt to have gastroschisis/atresia/dysmotility, defined as adequate small bowel length for survival as measured at time of laparotomy (mean, 146 cm; range, 66-233 cm), massive intestinal dilatation, and stasis. Of 9 patients with gastroschisis/atresia/dysmotility, 5 (56%) survived. All had surgery to “rescue” their dysfunction intestine, at mean age 128 days (range, 52-271 days): 4 had tapering enteroplasties of mean 37 cm (range, 5-115 cm)—all 4 survived; 3 had diverting stomas created for intestinal decompression, followed by stoma closure—1 survived; 1 nonsurvivor had a Kimura “patch”; 1 nonsurvivor underwent redo anastomosis. Survivors weaned off total parenteral nutrition at mean age 331 days after rescue surgery (range, 42-814 days).

Conclusions

More than one third of patients with GS/IA appear to have significant intestinal dysmotility without true SBS nor obstruction. Successful treatment of these infants may be achieved with the use of tapering enteroplasty and/or temporary diverting stomas.     

Does the colon play a role in intestinal adaptation in infants with short bowel syndrome? A multiple variable analysis

Purpose

We sought to examine in a multiple variable model the impact of residual colonic length on time to intestinal adaptation in a cohort of infants with short bowel syndrome.

Method

Infants with a surgical diagnosis of short bowel syndrome who underwent operation 90 days or younger were included in this analysis. Univariate Cox proportional hazards models for time to full-enteral feeds were developed. Predictors significant at the .2 level were entered into a stepwise multiple variable Cox proportional hazards model.

Results

A total of 106 infants were included in the cohort (70 adapted). Predictors meeting the criteria for the multiple variable model were as follows: multidisciplinary management (P = .045), Serial Transverse Enteroplasty Procedure (P = .057), percent small bowel (P < .001), percent large bowel (P < .001), preserved ileocecal valve (P = .001), number of septic (P < .001), and central line complications (P < .001). The final model included the following: multidisciplinary management (hazard ratio [HR], 1.932; 95% confidence interval [CI], 1.137-3.281), percent small bowel (HR, 1.028; 95% CI, 1.02-1.04), and septic events (HR, 0.695; 95% CI, 0.6-0.805).

Conclusions

The colon does not seem to play a significant role in intestinal adaptation. However, in addition to highlighting the importance of residual small bowel length, our model highlights the benefit of multidisciplinary intestinal rehabilitation and reduction of septic complications in achieving intestinal adaptation.     

Peritoneal drainage as definitive treatment for neonates with isolated intestinal perforation

BACKGROUND/PURPOSE: To better define the indications for peritoneal drainage (PD) in premature babies with intestinal perforation, the authors reviewed their experience with this procedure in a tertiary neonatal intensive care setting. METHODS: The charts of all neonates who underwent PD as initial treatment for intestinal perforation between 1996 and 1999 were reviewed. Those patients with pneumatosis intestinalis on abdominal radiograph had perforated necrotizing enterocolitis (NEC) diagnosed; whereas, those infants with no pneumatosis had isolated intestinal perforation diagnosed. The clinical characteristics and outcomes of these 2 groups were compared. RESULTS: Twenty-one premature neonates had primary PD between 1996 and 1999, 10 for isolated intestinal perforation and 11 for perforated NEC. Patients with isolated intestinal perforation had lower birth weights (708 v 949 g; P < .05), were less likely to have started feedings (30% v 91%, P < .05), and the perforation developed at an earlier age (10.6 v 28.0 d, P < .05) compared with the patients who had perforated NEC. Only 2 of 10 infants with isolated perforation required subsequent laparotomy (at 10 weeks for stricture and 12 weeks for a persistent fistula). For these patients, the long-term survival rate was 90%. In contrast, 8 of 11 infants with perforated NEC required laparotomy, and although the 30-day survival rate was 64%, the long-term survival rate was only 27%. CONCLUSIONS: Peritoneal drainage provides successful and definitive treatment for most premature babies with isolated intestinal perforation. For neonates with perforation caused by NEC, peritoneal drainage may provide temporary stabilization, but most of these infants require subsequent laparotomy, and few survive.     

An experimental evaluation of segmental reversal after massive small bowel resection.

The technique of reversal of a distal segment of residual small intestine was applied to the management of massive resection. A controlled evaluation was carried out in dogs into the effects of segmental reversal on the proximal remnant after the distal 75 per cent of the small bowel had been resected. The variables studied were survival, body weight, glyceryl tri(palmitate-1-14C) absorption and mouth-to-caecum transit time. Animals subjected to resection alone had accelerated intestinal transit, poor absorption, serious weight loss and a high early mortality. It was clearly demonstrated that segmental reversal after resection produced marked and progressive delay in transit time from mouth to caecum, sustained improvement in fat absorption and body weight and enhanced survival.     

短肠综合征患者血清瓜氨酸水平与小肠吸收面积和功能的相关性

目的研究短肠综合征患者血清瓜氨酸水平的变化及其与肠道面积及吸收功能的相关性。方法采用高效液相色谱法测定22例短肠患者（短肠组）和33例健康人（对照组）血清瓜氨酸水平。短肠患者残存小肠长度及直径采用X线造影检测，并测定短肠患者尿D-木糖排泄率和肠道蛋白吸收度。分析短肠患者血清瓜氨酸与残存小肠长度、面积、蛋白及D-木糖吸收的相关性。6例行肠康复治疗的患者测定康复治疗前后瓜氨酸、D-木糖及蛋白吸收水平的变化。结果短肠组血清瓜氨酸水平显著低于健康对照组[（5．94±2．65）比（16．87±5．97）μmol／L，P〈0．01]。短肠组患者血清瓜氨酸水平与残存小肠长度（r=0.82）及表面积（r=0.86）呈正相关，与尿D-木糖排泄（r=0.56）及肠道蛋白吸收（r=0.48）也呈正相关。6例行肠康复治疗的患者治疗后血清瓜氨酸水平、蛋白及D-木糖吸收均显著增加，但3者增加百分比之间并无相关。结论血清瓜氨酸水平与短肠患者的小肠吸收面积和吸收功能呈正相关，能反映短肠患者小肠功能和衰竭程度，是康复疗效的良好指标。     

The 2 STEP: an approach to repeating a serial transverse enteroplasty

Purpose

The serial transverse enteroplasty procedure (STEP) procedure is a successful and safe approach to lengthen small bowel in patients with short bowel syndrome (SBS). However, postlengthening dilatation may occur, which can lead to bacterial overgrowth and malabsorption. We addressed this problem by reperforming the STEP in 2 patients.

Methods

Two infants underwent the STEP procedure at 3 days and at 4 months of life for SBS secondary to intestinal atresia and gastroschisis. The patients' small bowel lengths were 20 and 32 cm before and 25 and 52 cm after the initial STEP. Nine and 8.5 months afterward, the patients developed dilatation of the small bowel and feeding intolerance. A second STEP procedure was undertaken with additional transverse firings of staplers between previously lengthened segments and tapering of redundant blind-ending portions of bowel.

Results

At operation, the bowel lengths were 45 and 62 cm. The second STEP left the patients with 61 and 73 cm of small bowel with a normalization of intestinal diameter. The first patient is doing well 5 months after surgery, and the second child tolerated increased enteral intake but died 1 year later from total parenteral nutrition-related liver failure.

Conclusions

This is the first report of a successful application of a second STEP procedure to further lengthen small bowel in SBS patients. Reapplication of the procedure requires careful stapling and a removal of small blind-ending segments to avoid further stasis.     

Prevention and reversal of intestinal failure-associated liver disease in premature infants with short bowel syndrome using intravenous fish oil in combination with omega-6/9 lipid emulsions

Although premature infants with short bowel syndrome are at the highest risk of developing intestinal failure-associated liver disease (IFALD), they have great capacity for intestinal growth and adaptation if IFALD can be prevented. Conventional soybean oil-based intravenous lipid emulsions have been associated with IFALD. This study presents data on 5 premature neonates with short bowel syndrome treated with a combination of parenteral fish oil- and olive/soybean-based lipid emulsion for periods ranging between 7 and 17 months. Despite an enteral tolerance of less than 50% in 4 of these patients during their first year of life, direct bilirubin levels normalized while on this combination of ClinOleic (Baxter, Maurepas, France)/Omegaven (Fresenius Kabi, Bad Homburg, Germany) at a 1:1 ratio. None of our patients developed irreversible IFALD even though all of them were premature, had undergone multiple major surgical procedures, and had experienced several episodes of sepsis. Thus far, we have not seen any adverse effects of this mixed lipid emulsion in these preterm infants. All 5 patients are growing and developing well and have normal liver function.     

Human milk fortifier: An occult cause of bowel obstruction in extremely premature neonates

Background

Human milk fortifier (HMF) is used in neonatal units throughout North America to facilitate growth of preterm infants. Little data is available on the gastrointestinal side effects and potential adverse events. The purpose of this paper was to present a series of infants presenting with bowel obstruction associated with HMF.

Methods

Cases of HMF obstruction were collected between January 2010 and December 2012. Charts were reviewed and relevant data was collected.

Results

During the study period, 7 premature infants presented with bowel obstruction secondary to intestinal concretions of HMF. All babies were premature with gestational ages from 25 to 27 weeks. Birth weight was less than 1000 grams in all patients. Patients presented with feeding intolerance, bilious aspirates, abdominal distension, and obstipation. Four of the patients presented with acute deterioration and required urgent surgical intervention.

Conclusions

HMF is an important source of nutritional support in infants, which is felt to be safe. We present a series of infants where its use has resulted in significant complications. HMF should be used with caution in infants, especially those with a history of necrotizing enterocolitis. Further research should examine the calcium, protein, and fatty acid concentration tolerable in the gastrointestinal tract of infants.     

Degree of bowel distension on plain-radiographs— A surgical-radiological study of new criteria in mechanical intestinal obstruction

Three hundred sixty clinical cases which presented with or without mechanical intestinal obstruction were evaluated in order to discover reliable quantitative and qualitative criteria for pre-surgical x-ray diagnoses. It was discovered that small bowel distension of above 1.0 (ratio) together with obvious gas fluid level is usually indicative of small bowel obstruction, while large bowel distension of above 1.5 (ratio) together with obvious gas fluid level is usually indicative of large bowel obstruction. Subsequent surgery confirmed that there exists a correlation between the degree of small bowel distension seen on radiographs prior to surgery and the actual small bowel distension secondary to small bowel obstruction measured during surgery. The present results may be diagnostically useful in the radiological differentiation of mechanical intestinal obstructions from other abdominal conditions.     

Adverse effects of liver dysfunction and portal hypertension on intestinal adaptation in short bowel syndrome in children

BACKGROUND: The effects of liver dysfunction and portal hypertension on intestinal adaptation in short bowel syndrome are generally unknown. The presence of these disorders may adversely affect the ability to wean these patients from parenteral nutrition. METHODS: Forty-two infants with short bowel syndrome were placed in one of three Child's classifications, depending on serum bilirubin, prothrombin time, ascites, albumin, and liver biopsy, and compared for time to diet tolerance, time required for parenteral nutrition, and survival. A subgroup of these patients also underwent portal pressure measurement, which was combined with liver biopsy results to compare three groups for the same parameters. RESULTS: Survival was Child's class A 100%, B 84%, C 61%, while time to feeding tolerance was A 16.3 days, B 20.0 days, C 28 days, and total parenteral nutrition time was A 80.0 days, B 98.0 days, C 100.0 days. In the groups that underwent portal pressure measurement, the survival was group I (normal biopsy and pressure) 100%, group II (abnormal biopsy and normal pressure) 90%, group III (abnormal biopsy and pressure) 66%, while time to feeding tolerance was I 15.0 days, II 18.0 days, III 24.0 days, and total parenteral nutrition time was I 72.0 days, II 94.0 days, III 184.0 days. CONCLUSIONS: Cholestatic liver disease, especially associated with portal hypertension adversely affects bowel adaptation in short bowel syndrome.     

Intestinal adaptation in short bowel syndrome

Regaining enteral autonomy after extensive small bowel resection is dependent on intestinal adaptation. This adaptational process is characterized by hyperplastic growth of the remaining gut, which is accompanied by both an increase of cell division at the level of the crypt cells and by an increased rate of programmed cell death (apoptosis). Apart from the absorptive function, the small bowel also has a barrier function and plays an important role in interorgan metabolism. Also, these functions are greatly affected by a massive intestinal resection and subsequent recovery by intestinal adaptation. This review aims to give an overview of the debilitating effects of massive intestinal resection on gut function and subsequently discusses intestinal adaptation and possible factors stimulating adaptation.     

Malignant tumours of the small intestine

Adenocarcinoma, neuroendocrine tumours, sarcomas and lymphomas are the four most common malignant tumours arising in the small intestine, although over forty different histological subtypes are described. Collectively these account for only 2% of cancers of the digestive system. The incidence of small bowel cancer has increased in recent decades with a four-fold increase in carcinoid tumours. Risk factors for small bowel tumours include coeliac disease, inflammatory bowel disease and a number of genetic abnormalities. The non-specific nature of their symptoms and the difficulty in visualising these tumours with normal endoscopic techniques often results in late diagnosis. Furthermore the paucity of literature on this topic has made it difficult to standardise management. There has however been marked improvement in imaging methods resulting in earlier diagnosis in many cases. As expected, early detection of localised, well differentiated tumours followed by surgical resection with negative margins offers the best chance of long term survival. Better adjuvant treatment, notably for gastrointestinal stromal tumours, has improved 5-year survival rates significantly. Development of surveillance guidelines for at risk populations may be a valuable way of improving early diagnosis of this challenging group of conditions.