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Intestinal Failure: Bianchi, STEP, Transplantation and beyond…
Naira Baregamian, MD
Luca Cicalese, MD
2/24/10
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Goals and Objectives
n E= tiology of intestinal failure in children
n Contemporary medical and surgical = management of intestinal failure
nComprehensi= ve review of surgical approaches to pediatric intestinal failure
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Case Presentation
DM=
nDM, 37 wk GA male who at birth presented with jejunal atresia, no other developmental abnormalities
nUnderwent laparotomy, jejunal resection, with jejunostomy and mucous fistula
<= span style=3D'font-size:88%;text-shadow:auto'>nStarted on TPN and tube feeds
n4 weeks post-op, had contrast abd= ominal study which = did not reveal any evidence of residual intestinal atresia, = and contrast went through ostomy = and mucous fistula without any delay.
nPt underwent reversal of ostomy, without any complications
------=_NextPart_01CAE6B4.FDBC2C70 Content-Location: file:///C:/48C7E2A5/GR022410IntestinalFailure_files/slide0003.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Intestinal Failure
Case Presentation
DM=
nContinued on TPN, while oral feed= s were initiated, and reached a goal shortly after
nPOD14 post-reversal, decreased or= al feeds to= lerance and increased abdominal distention were noted
nBabygram demonstrated a dilated l= oop of jejunum, which persisted despite stopping of oral feeds
nIntestinal plication procedure was planned wi= th placement of DHT, which encompassed invagination of antemesenteric border of jejunal = loop with ru= nning suture
<= span style=3D'font-size:88%;text-shadow:auto'>nPt continued on TPN
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Content-Type: text/html; charset="us-ascii" Intestinal Failure
Case Presentation
DM=
nIntestinal plication and placemen= t of DHT were = unsuccessful, and pt returned to OR for resection of necrotic bowel, removal of DHT and creation of high jejunostomy and mucous fistula
n57cm of non-viable SB was resected
nPt continued on TPN
nOral feeds were re-initiated, cap= tured at proximal jejunostomy and re-fed through a feeding tube passed into the mucous fistula, a= nd tolerated well, began gaining weight rapidly, and weaning TPN down
nDeveloped jaundice  at 6-7 wks of life, intra-op liver biopsy demonstrated cholest= asis with = reversible changes
<= ![if !vml]>
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n6KKYeppY+pp9FWLBQ97GD0zXZaZGpkBIrpbVBGwdeCK1g26DJA5xUkJ4x7U2Y5/KkLFIsjsOKx7 4b87u/WuN1WJBvwK5NhhyPekoph6mv/Z ------=_NextPart_01CAE6B4.FDBC2C70 Content-Location: file:///C:/48C7E2A5/GR022410IntestinalFailure_files/slide0005.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Intestinal Failure
Case Presentation
DM=
nPt went to = OR once more for debridement of wound infection and dehiscence, cultures + E.coli and was treated with appropriate Abx regimen
nAt 3 mo, was taken back to OR for reversal of ostomy
nContinued o= n oral feeds, and weaning of TPN, decreased cholestatic jaundice
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Case Presentation
DM=
nCurrently, continues on TPN & oral feeds combination
nCholestatic jaundice, on Ursodiol
nUS Abd – Hepatosplenomegaly with mild fatty liver
nTriglycerides:  237 (238)
nLFTs:  TP/Alb:  7.6/4.2; AST/ALT:  464/309 (483/319); = <= span style=3D'font-size:88%;text-shadow:auto'>GGT:  260 (232); Alk Phos:  574 (700)
nBili:  1.8/4.3 (1.8/4)
nLipid profile 2/10/10:  Elevated linoleic acid, stearic a= cid, = arachidonic acid, DHA and linolen= ic acid
n
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Case Presentation
DM=
nFuture:
n -Risk of developing intestinal failure/short = gut syndrome sequellae
n -Risk of developing liver fa= ilure if continues <= /span>= on prolonged TPN regimen
n -Quality of life
n -Surgical management 
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Intestinal failure
nDefined as a distinct clinical sy= ndrome characterized by impaired intestinal function related to food digestion, = absorption of fluids, electrolyte= s and nutrients essential to live, grow and develop normally
nThe most common cause is Short Bo= wel Syndrome = (SBS), a problem affecting individuals who had >1/2 of their small intestine removed to = treat conditions such as necrotizing enterocolitis or trauma
nIntestinal failure may also be ca= used by digestive disorders, such as Crohn’s disease or chronic idiopathic intestinal pseudoobstruction synd= rome
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Conditions that cause Intestinal Failure in children include:
<= span style=3D'font-size:88%;text-shadow:auto'>nHirshsprung’s disease/aganglionosis
nDiseases of the intestinal surfac= e, such as congenital villous atrophy or microvillous inclusion disease =
<= span style=3D'font-size:88%;text-shadow:auto'>nGastroschisis
<= span style=3D'font-size:88%;text-shadow:auto'>nIntestinal atresia
<= span style=3D'font-size:88%;text-shadow:auto'>nMidgut volvulus
nMultiple intestinal surgerioes resulting in adhesions and problems with motility and/or absorption
<= span style=3D'font-size:88%;text-shadow:auto'>nNecrotizing enterocolitis
<= span style=3D'font-size:88%;text-shadow:auto'>nPseudoobstruction and other motility diorders
<= span style=3D'font-size:88%;text-shadow:auto'>nShort bowel syndrome
<= span style=3D'font-size:88%;text-shadow:auto;visibility:hidden'>n<= span style=3D'font-size:88%;text-shadow:auto;mso-special-format:lastCR;display:= none'>
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Short Bowel Syndrome
nMajor features of SBS are dehydra= tion secondary to diarrhea, malabsorption of macro- and micronutrients, = malnutrition and failure to thriv= e
nResection of certain regions of intestine can lead to permanent loss of specialized absorptive functions
nAfter resection, the residual sma= ll bowel undergoes intestinal adaptation, which is the gut’s attempt to optimize its absorptive capacity&= #13;
nThe adaptation process may take s= everal months or = years to complete, and infants may be totally or partially = dependent on TPN
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Short Bowel Syndrome
nResidual intestinal length and ability to adapt= and become TPN-independent are poorly understood = association
nPatient characteristics such as the region of b= owel = resected, functional capacity of the remaining = intestine, presence or absence of the ileocecal= = valve, length of residual colon, underlying diagnosis, gestational age probably all contrib= ute = to the problem of determining the minimum small bowel length required to avoid SBS=
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Intestinal Failure Management
nMedical
n - TPN
nSurgical
n - Bowel lenghtening procedures
n - Intestinal transplantation (alone v. = multivisceral)
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nManagement = of SBS/IF has to be undertaken by a multidisciplinary team including gastroenterology, surgery, and nutrition
nThe final therapeutic goal is favoring intestinal adaptation by the bowel so that the patient can= be weaned from parenteral nutrition
Intestinal Failure Management
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Management of Intestinal Failure
nBy definition, therapy for intest= inal failure relies on parenteral nutrition, usually in conjunction with oral nutritional support
nSome patients may survive indefin= itely on TPN and oral nutritional support, others suffer multiple complications, such as recurrent infection, dehydration, vascular thrombosis, or progressive liver disease that may result in <= /span>= death
nA small number of patients with t= hese complications may be rescued by small bowel transplantation, which in itself is = associated with numerous potential complications and requirement for life-long immunosuppression
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TPN protocol for SBS
nGlc-polymer= based oral rehydration solutions (ORS)
nElectrolyte replacement/polymer
nBlood Glc, TG monitoring
nFluid management
nH2 bolckers/PPIs first 6 months
nDiarrhea control
nOral calcium supplements
nVitamin/Mineral supplements <= /div>
n
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<= span style=3D'text-shadow:auto'>nHigh complex carbohydrate diet
<= span style=3D'text-shadow:auto'>nCycle TPN &= #13;
<= span style=3D'text-shadow:auto'>nCatheter ca= re teaching
<= span style=3D'text-shadow:auto'>nTPN weaning=
<= span style=3D'text-shadow:auto'>nPartial TPN supprt with eaning
<= span style=3D'text-shadow:auto'>nLow oxalate diet
<= span style=3D'text-shadow:auto'>nMedium-chain triglycerides (MCT) as part of diet
nDiagnosis and treatment of TPN complications, <= /span>including catheter-related complications=
TPN protocol for SBS cont’d
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Medical therapy
nIntestinal adaptation is the goal= of initiating this protocol
nAnimal models show adaptation as luminal dilation with thickening and lengthening of the GI tract = developing in association with hyperplasia of the crypt villous axis; most noticeable in residual ileum
nThese responses lead to an increa= se in cells of the proliferate zone of the villi, causing villous hyperplasia and a parallel increase in the production of brush border enzymes
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<= ![if !vml]> <= ![if !vml]>
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Mechanisms of adaptation
nMultiple mechanisms and mediators= have been proposed for the adaptation response, but no single factor adequately explains all th= e changes that occur in intestinal structure and function
<= span style=3D'font-size:75%;text-shadow:auto'>nThere is a role for luminal nutrients, GI secretions, and humoral factors
nHormones, growth factors, and cyt= okines have been proposed to be important in adaptation because their levels are increas= ed in the serum and tissues after massive small bowel resection, and exogenous administration of these substance= s has been demonstrated to enhance various components of the adaptation response (e.g. IGF-= 1, glucagons, = peptide-YY, EGF)
nThe strongest stimulus for the adaptation is the presence of enteral nutrition within the lumen. Lumin= al nutrients have both a direct and indirect effect on adaptation. Nutrients promote release= of hormones and secretions as well as stimulate peristalsis and splanchnic blood flow =
<= span style=3D'font-size:75%;text-shadow:auto;visibility:hidden'>n<= span style=3D'font-size:75%;text-shadow:auto;display:none'> <= /div>
<= span style=3D'font-size:75%;text-shadow:auto;visibility:hidden'>n<= span style=3D'font-size:75%;text-shadow:auto;mso-special-format:lastCR;display:= none'>
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Mechanisms of adaptation
nNutrition composition is also important, with complex nutrients initiating a stronger adaptation response. Complex nutri= ents require great= er metabolic effort for digestion and absorption; therefore, the compensatory respo= nse is maximize. Fats, especially long-chained, unsaturated lipids, are the most trophic of the = macronutrients
nIn the USA, an estimated 20,000 patients with intestinal disease receive home parenteral nutrition at an annual cost of <= /span>approximately $150,000 per person= (this translates into $3 billion annually in USA)
nIf therapy designed to enhance adaptation allows discontinuation of TPN in just 10% of patients with SBS, a minimum of $300,000,000 could be saved
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Intestinal Adaptation Process
nDiarrheal stage (1-2 weeks) <= /div>
nIntestinal adaptation stage <= /div>
nResolution stage
n =
nInitiation of enteral feeding can be attempted once a child has stabilized from the presentation
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The use of TPN in SBS
nThe use of TPN in SBS can produce gallstones and liver disease
nGallstones form due to interruption of the = enterohepatic circulation of bile acids
nLiver disease (steatosis, cholestasis, cirrhosi= s) is one of the lonf term sequellae of TPN, with = 50% having grade 2 fibrosis at > 5yrs
nWith the failure of medical management, = surgical intervention must be considered=
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Surgical Management
nMethods of surgical intervention generally fall into 3 categories
n 1. Increasing transit time
n 2. Increasing the bowel length
n 3. Intestinal transplantation
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Increasing transit time
nMethods available are reversed segments, artificial valve creation, stricturoplasty, recirculating loops, retrograde electronic paci= ng and intrinsic myenteric denervation
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Increasing the bowel length
nMethods are those devised by Bianchi  in 1980, = and more recently the development of Serial = Transverse Enteroplasty (STEP)
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Intestinal transplantation
nWhen intestinal failure is considered permanent= = and not amenable to surgical correction and = administration of TPN is resulting in life-threatening complications, then intestinal transplantation with or without a liver is the = only = remaining option
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Reversed Segments
<= span style=3D'font-size:75%;text-shadow:auto'>nFirst described by Mall in 1896 in dogs, and were ultimately fatal
<= span style=3D'font-size:75%;text-shadow:auto'>nFirst human operation of this kins was reported by Gibson in 1962
nOptimum length of reversed segmen= t is 10cm in adult and 3cm in infants, if possible, distal small bowel is resected, reversed and reinseted
nThe idea is that chyme passes dow= n the small intestine due to the peristaltic nature of the gut, then when it encounters a reverse segment, its transit is momentarily opposed and pools of chyme are encouraged to form, hence increas= ing time for absorption
nThis procedure doe offer a short-= term advantage, but its long-term value remains unclear
nNeonates and infants are less fav= orable candidates for reversed SB segments because of their potential for intestinal growt= h (lengthening). Growth of a revers= ed segment may ultimately produce complete bowel obstruction
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Intestinal tapering and plication
nBowel dilatation slows intestinal transit and increa= ses = mucosal absorptive area. At a poi= nt, however, this normal adaptative mechanism becomes pathologic. = Dilated segments become dysmotile= , and fecal stasis, bacterial overgrowth, and malabsorption ensues
nFunctional dysmotile segments should be streamlined = by tapering or plication to improve peristalsis and decrease bacterial overgrowth
nStreamlining has the advantage of preserving bowel <= /span>length compared with resection of dilated segments
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nTapering enteroplasty involves pa= rtial resection of the antemesenteric border of the dilated loop
nThe blood supply arising from the mesenteric side is not disturbed
<= span style=3D'font-size:88%;text-shadow:auto'>nThe disadvantage: some absorptive area is lost <= /span>
<= span style=3D'font-size:88%;text-shadow:auto'>nLow risk of post-op leaks from suture line
nTo ensure an adequate and uniform lumen, an a= ppropriately-sized Foley catheter or chest tube can be inserted into the bowel lumen through a small enterotomy at the beginning of the dilated segment
Intestinal tapering and plication
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Plication
nPlication a= lso streamlines  the bowel, but n= o = bowel wall is resected
nThe dilated= bowel wall is inverted into the lumen, and the serosal surfaces are umbricated
nMucosal sur= face area for absorption is maintained and motility improved with plication
nComplicatio= ns: bowel obstruction, suture line failure
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Intestinal tapering and plication
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Artificial valves
nIn 1981 Ricotta described a method of constructing an artific= ial ileocecal valve.
nOne method is creating a valve similar in structure to intussusception, but on a smaller= scale
nValves have been shown to increase transit time but not to = result in any increases in absorp= tive surface, functional absorptive capacity or body weight
n
<= ![if !vml]>
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4+mRgcidDay6aOiYmUtUXSNOQMok7gakEDlc1swr/1HNDiu83vzN4BzO4jzO5FzO5nzO6JzO6uwR AQEAOw== ------=_NextPart_01CAE6B4.FDBC2C70 Content-Location: file:///C:/48C7E2A5/GR022410IntestinalFailure_files/slide0031.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Intestinal Failure
Colonic Interposition
nThe main purpose remains the slow= ing of the intestinal transit time, as the colonic motility is slower than that of small bow= el, hence = reversal of the bowel segment is not necessary
nFirst applied by Trinkle and Brya= nt in 1967 in an infant with SBS and midgut volvulus
nReversal of colonic interposition= has also been performed (ACI, antiperistaltic colonic interposition)
nA colonic segment is isolated (8-= 24cm, ?optimal length) and separated, then anastamosed between the jejunum and ileum, = continuity of the colon is re-established
nThe colonic segment can be anasta= mosed in iso- or anti-peristaltic direction
nLower risk of bowel obstruction, however, longer segments are associated with D-lactic acidosis due to lactobacillus overgrowth
------=_NextPart_01CAE6B4.FDBC2C70 Content-Location: file:///C:/48C7E2A5/GR022410IntestinalFailure_files/slide0034.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Intestinal Failure
Reversed Electrical Intestinal Pa= cing
<= span style=3D'font-size:75%;text-shadow:auto'>nHyperperistalsis is common in SBS =
nOverriding the intrinsic intestin= al pacemaker should slow intestinal transit in SBS patients to optimize water and nutrient absorption
nThe normal intestinal pacemaker is located in the foregut within the duodenum. The electrical wave is propagated toward the i= leum to = generate peristalsis
nCanine experiments have demonstra= ted better absorption , but manipulation of the duodenal pacemaker requires duodenal= resection
nMost recently, intestinal interlu= minal electrodes have been placed experimentally within the jejunum to slow intestinal wav= es down. It = may be helpful in SBS patients with primary motility problems,but <= span style=3D'position:absolute;top:85.5%;left:9.73%;width:87.82%;height:5.25%'= >it has not been fully evaluated in human trials
------=_NextPart_01CAE6B4.FDBC2C70 Content-Location: file:///C:/48C7E2A5/GR022410IntestinalFailure_files/slide0032.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Intestinal Failure
Bianchi or Longitudinal Intestinal Lengthening Technique (LILT)
nFirst described in 1980 by Bianch= i in porcine = model
nThe procedure is based on SB dual= blood supply that e= nters the bowel wall within two layers of the mesentery
nThe bifurcated blood supply enter= s each hemicircumfer= ence of the bowel wall. The two leaves of the mesentery are separated on the mesenteric surface of the bowel&#= 13;
nThe bowel is divided longitudinal= ly between two layers so that each parallel lumen has its own = single source blood supply
nTwo newly created bowel segments = are then = anastamosed end-to-end, creating one intestinal segment that is twice as long and half the = diameter of the original <= /span>
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