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Query: UMLS:C0042961 (volvulus)
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Our report concerns a child with short bowel syndrome who had 20 cm of small intestine distal to the pylorus (duodenum and jejunum) which remained after subtotal intestinal resection; the resection was necessitated by intrauterine volvulus with intestinal necrosis as a result of gastroschisis. In addition, only 25 cm of the colon remained. Despite continuous enteral nutrition with a semi-elementary diet and conservative therapy, it was not possible to provide within six months at least occasionally half of the caloric intake required on an enteral basis. In such cases treatment is usually discontinued. As final alternative to a small intestine transplantation, we conducted an operation to lengthen the intestine (method according to Bianchi) when the patient was 8 months old. The massively dilatated jejunum section of the intestine was lengthened from 20 cm to 37 cm. Postoperatively enteral caloric intake could be increased from 11 kJ/kg/m to 20 kJ/kg/m. Unfortunately, at 14 months of age, the child died from hepatobiliary complications arising from aggravation of the cholestasis and acidosis. This case shows, nevertheless, that intestinal lengthening had a positive influence on intestinal resorption.
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PMID:Is intestinal lengthening effective in treating extreme short bowel syndrome? 193 44

An operation according to Bianchi in a 2-year-old girl is described and indications as well as technical procedure are discussed. The girl was born with a gastroschisis. There was a jejunal perforation 10 cm below the ligament of Treitz caused by a volvulus. Only 20 cm of the jejunum remained. Moreover, only the left part of the colon was present. Total parenteral nutrition for 2 years was necessary. The principle of the operation is based on a longitudinal division of the remaining bowel and a creation of two separate bowel tubes out of the divided bowel halves, thus effecting an isoperistaltic serial connection by means of two anastomoses. This is technically possible since each half of the bowel wall has its own blood supply. The vessels originating from the mesenterium branch off before they reach the bowel wall so that the mesenteric dissection line can be anastomosed longitudinally with the antimesenteric border. This results in doubling of the bowel length, narrowing of the preoperatively dilated bowel diameter, closer contact of bowel contents with the mucosa, prolonged transit time and a Bacteroides colonization which is reduced by more effective peristalsis. Indications, time of operation and our own experiences are discussed and three cases are described. All children are alive and show marked improvement in nutrition.
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PMID:Surgical techniques in short bowel syndrome. 210 23

While small-bowel transplantation remains an option for the management of short-bowel syndrome (SBS), every effort must be made to optimize the function of the native bowel. This report describes a patient with SBS who dramatically improved after a Bianchi procedure. The patient was born with type IIIb jejunal atresia, complicated by volvulus of the terminal ileum. He was left with 40 cm of small bowel and his ileocecal valve. During the first 20 months of his life, he was always hospitalized except for 3 months. He was on parenteral nutrition (TPN) and suffered multiple episodes of central line sepsis. Massive small-bowel dilatation caused a functional bowel obstruction with bacterial overgrowth, and villous atrophy of the jejunal mucosa was discovered on biopsies done by colonoscopy. In March 1994, we elected to proceed with a Bianchi procedure. Thirty cm of jejunum were divided longitudinally. During the following year, his enteral tolerance steadily improved to the point where TPN could be discontinued. We conclude that pediatric surgeons should not hesitate to use this procedure in the management of SBS.
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PMID:The Bianchi procedure in a patient with jejunal atresia. 949 89

Advances in parenteral nutrition and supportive therapy have led to improvement in survival of babies with short-bowel syndrome. Those whose intestinal mass is very unlikely to be adequate should have surgical therapy as soon as possible, before they develop the complications of long-term parenteral nutrition or significant enteritis. We present a newborn with short-bowel syndrome due to prenatal midgut volvulus. At operation the remaining viable jejunum, 15 cm long, was anastomosed to the cecum. All feeding attempts failed, and the infant suffered from malabsorption. Calories and proteins had to be supplied by intravenous total parenteral nutrition. At 3 months of age there was significant widening of the remaining bowel and Bianchi's bowel-lengthening procedure was performed. The postoperative course was uneventful and there was gradual improvement in intestinal absorptive capacity. The patient was weaned from parenteral nutrition at 3 years of age. Now, 2 years later, she eats a normal diet.
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PMID:[Bowel-lengthening in a newborn with short bowel syndrome]. 1091 85

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.
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PMID:Serial transverse enteroplasty for short bowel syndrome: a case report. 1466 85

In cases with short bowel syndrome (SBS), intestinal adaptation often requires a long time. In addition, the quality of life in SBS is not especially good due to some complications, including growth impairment, severe diarrhea, complications of parenteral nutrition (PN), and so on. We herein report an adolescent boy with SBS secondary to midgut volvulus. He suffered mainly from both severe high output syndrome, which caused a large amount of enteric fluid to flow out from the jejunostomy, and growth impairment, although PN continued after the occurrence of SBS. As a result, he thereafter could not continue his daily school life. Therefore, he was introduced to our department at 6 months after the operation. A closure of the jejunostomy combined with longitudinal intestinal lengthening using Bianchi's procedure was performed. Thereafter, the above-described symptoms disappeared. He was discharged and thereafter was again able to attend school with an improvement in his growth within 2 months after the second operation. Home PN was discontinued at 15 months after the second operation. Based on our experience, in cases of SBS without intestinal functional disorder, an early closure of the jejunostomy combined with Bianchi's procedure might therefore possibly lead to an early improvement in the complications related to SBS.
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PMID:Improvement in the quality of life using both Bianchi's procedure and the closure of a jejunostomy in a case with short bowel syndrome. 1702 35

There are several reasons for the possible development of a short bowel syndrome, which, however, occurs only rarely. The main causes consist of extended intestinal resections in cases of congenital anomalies (e.g., gastroschisis, intestinal atresia or dysplasia) or ischaemic lesions due to a volvulus. In addition, an intestinal stoma at a more upper segment of the GI tract can result in the functional manifestation of a short bowel syndrome. The differentiation between temporary and persisting types is essential for initiation of an adequate treatment. Loss or exclusion of organic resorption area at the inner surface of the (small) intestine can be associated with numerous pathological consequences requiring treatment. As a principle consideration from the paediatric point of view, the potential of intestinal adaptation needs to be assessed. Basic conservative treatment options are parenteral and enteral nutrition regimens, in particular, to prevent complications (such as D-lactate acidosis). The main surgical approaches are the procedures called LILT (longitudinal intestinal lengthening and tailoring) according to Bianchi and STEP (serial transverse enteroplasty). The technique to create intestinal segments of antiperistalsis has been abandoned. Because of the encouraging results of intestinal transplantation, this novel treatment option has gained greater attention over the past few years and is now also an option for paediatric patients. The limiting factor and thus major complication is the central venous catheter for long-term treatment. Catheter-related complications are still the main reason for a considerable mortality in these children.
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PMID:[Age-dependent visceral medicine: paediatric visceral medicine - visceral medical paediatrics - considerations on the short bowel syndrome in childhood]. 2323 40

The goal of any treatment programme for short bowel syndrome SBS is to achieve nutritional enteral autonomy. This must begin with conservation of as much bowel as possible from the time of first presentation. Frequent causes of the short bowel syndrome are intestinal atresia, necrotizing enterocolitis, midgut volvulus, extended intestinal aganglionosis, 'vanished gut' often associated with gastroschisis and occasionally catastrophic trauma. Atresia is more amenable to successful surgery than other causes, except when associated with gastroschisis. Intrinsic dysmotility has a poor prognosis. Intestinal lengthening procedures are only indicated if there is sufficient bowel dilatation. Extended intestinal aganglionosis is rarely amenable to any form of non-transplant surgery. Options available are to conserve bowel, close stomas early (use all available bowel to the maximum or even re-feed stoma effluent into the distal unused bowel), release adhesions causing obstruction, resect strictures, taper or excise localized dilatations and finally address dilated bowel with lengthening and tailoring operations. These procedures aim to improve effective peristalsis, thereby reducing bacterial overgrowth and improving nutrient contact with enteral mucosa to maximize absorption and intestinal adaptation. The Bianchi longitudinal splicing operation and the serial transverse enteroplasty operations have stood the test of time in providing considerable improvement in enteral nutritional autonomy in around 60% of cases. In SBS without dilatation attempts at 'mechanically' delaying transit (nipple valves, reversed bowel segments, colon interposition) have had inconsistent outcomes. Growing neomucosa and lengthening bowel by longitudinal stretch are still experimental.
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PMID:Non-transplant surgery for short bowel syndrome. 2398 89