2/14/2014 Disclosures Complex and Innovative Cases in General Surgery Marketa Rejtarova, DNP CPNP-AC/PC Christina Allcox, CPNP-PC Learning Objectives Discuss the needs of complex pediatric general surgery patients Describe cutting-edge surgical techniques used in pediatric surgery Describe the need for collaboration among disciplines for care of these complex patients Neither we, nor any member of our immediate families currently have or have had in the past two years a financial interest/arrangement or affiliation with one or more organizations that could be perceived as a real or apparent conflict of interest in the context of the subject of this presentation Inpatient General Surgery PNP Group at Boston Children’s Hospital 14 PNPs (10 FT, 4 PT) Inpatient General Surgery Subspecialities (coordinator, NP(s) and/or advanced RN(s) Bariatric Program Congenital Diaphragmatic Hernia Esophageal Atresia Short Bowel Syndrome/CAIR Transplant Trauma Vascular Anomalies Case #1 N.S. N.S. was born at 38wks via csection to 42yF G2P1->2; IVF pregnancy Followed by MFM at OSH; selfreferred to AFCC at BCH Prenatal imaging: polyhydramnios, absent fluid in stomach, no fluid-filled structure within mediastinum, fetal brain MRI normal, rest of fetal survey fine www.sonoworld.co m LIKELY c/w ESOPHAGEAL ATRESIA 1 2/14/2014 Esophageal Atresia / Tracheoesophageal Fistula EA/TEF con’t www.aafp.org 2 separate issues commonly occurring together The foregut at 19-23days of gestation is a single cell-layer tube Gives rise to esophagus and pharynx Dorsal foregut, primitive esophagus, begins to divide from the ventral trachea at the level of the carina (completed by 26th day of gestation) Disruption in this process TEF Unavailability of tissue d/t rapid growth and elongation of the primitive esophagus and possibly with effect of interruption of the vascular supply EA EA/TEF Types Esophagus Type A often referred to as pure EA TEF type C most common Type E is also called type H Trachea www.learningradiology.com www.aafp.org EA/TEF Workup EA Failure of esophagus to become a continuous tube Separate proximal and distal pouch TEF Abnormal connection between the esophagus and trachea Proximal, distal, or both http://radiographics.highwire.org EA/TEF Con’t Incidence: 1/3,000 - 4,500, slight male predominance, more common in preemies Differential Diagnosis Pneumonia, GER Comorbidities 50% have other anomalies http://commons.wikimedia.org EA/TEF is part of VATER or VACTERL associations Most common are cardiac Prognosis Dependent on associated anomalies EA/TEF Management www.ispub.com History Polyhydramnios, small or empty fetal stomach, and/or presence of a dilated proximal esophageal pouch Findings Pooling of secretions in the upper esophageal pouch Inability to tolerate feeds, coughing, choking Inability to pass a feeding or NG tube* http://en.wikipedia.org Testing/Imaging Plain XR (gasless abdomen or abdominal gas present) Esophagram (blind pouch); Bronchoscopy (fistula) ECHO, renal u/s, spinal u/s, spinal xrays, and other based on clinical findings/suspicion Prior to repair Suction of secretions is critical Parenteral nutrition +/Evaluation of esp. cardiac anomalies http://www.ispub.com http://surgery.med.umich.edu 2 2/14/2014 Case #1 N.S. con’t: After birth Apgars 9/9, unable to pass NG/Repogle left in pouch to LWS, occasional desats Bweight 2.84kg HEENT: AFOS, eyes grossly normal, ears normal shape/position, palate intact, NG in place Resp: Scattered crackles R>L, good air entry CV: S1/S2 audible, no M/R/G, femoral pulse 2+ B/L www.learningradiology.com Abd: Soft, NT, on mases, umbilical stump clamped GU: Normal external, testes descended B/L, anus patent MSK: No limb deformities appreciated, no sacral dimple Case #1 N.S. con’t: Workup/PreFoker Plain XR with gasless abdomen and NG that stops at T2 level, lungs w/some streaky opacities c/w ?TTN Sepsis workup d/t desats (negative) ECHO on DOL #3: normal structure, small PDA, transitional elevated right side pressures DOL #3: DL/bronch, EGD, open GT placement, Intraoperative esophagram, PICC line placement Identification of proximal TEF Renal u/s and spine u/s and x-rays normal No other obvious congenital defects Pouch decompression, nutritional support, pulmonary toilet http://intranet.tdmu.edu.ua EA/TEF Types Esophagus Type A often referred to as pure EA TEF type C most common Type E is also called type H Trachea www.learningradiology.com www.aafp.org EA/TEF Surgical Management A. Gasless abdomen (pure EA and proximal fistula) Gastrostomy Primary repair (short): very rare: +/- ligate and divide fistula Delayed repair (intermediate): +/- Bougie upper pouch, +/Foker process, +/- esophagostomy Staged repair (long): Foker process, +/- esophagostomy, +/replacement/interposition B. Abdominal gas present (distal fistula) Short gap (ligation and division w/primary repair) Intermediate (ligation and division w/primary or delayed repair, GT) emedicine.medscape Wide (rare) (GT, ligation and division w/staged repair) Repairs postponed till baby is medically stable emedicine.medscape. Long-Gap EA Repair: Foker Process EA Team at BCH Foker Process Based on the foundation that esophagus grows under tension in utero Placement of traction sutures at the upper and lower ends of the esophagus and applying tension daily in order to stimulate esophageal tissue growth Stage 1 Stage 2 Stage 3 +/Serial EGDs/dilations www.biomedsearch.com LGEA Foker Process Case #1 N.S. con’t: Foker I DOL #32: Foker I procedure with external traction (gap 7.5cm, lower pouch 2cm) Paralyzed and sedated; on Zosyn Chest tube d/c’d POD #5 NPO, on PN/IL DOL #2: Repair of traction sutures 2/2 looseness DOL #13: Repair of traction sutures 2/2 looseness www.childrenshospital.org http://www1.umn.edu 3 2/14/2014 Case #1 N.S. con’t: Foker II DOL #56: Foker II procedure with primary anastomosis of esophagus emedicine.medscape.com Paralyzed x7 day postop , Versed drip d/c’d POD #17 and morphine drip d/c’d POD #23; continued on Methadone Unasyn till POD #5 Extubated POD #8, issues w/secretions, then weaned to RA Esophagram POD #14 no leak; advanced to full GJT feeds (24 kcal/oz BM 33 cc/hr) Diuretics prn; circumcision on POD #17 Routine x-ray to check bone density POD #10: left humerus and right femur fracture (calcium level ok but PTH high; ergocalciferol started) EA/TEF Complications Anastomotic leak (contained vs. not) Sepsis, mediastinitis d/t leak GER (>50%) Difficulty swallowing Anastomotic strictures Secondary (exogenous) adrenal insufficiency Fistula recurrence Aspiration PNA Plus complicated by associated anomalies http://www.mypacs.net Acute and long-term Case #1 N.S. con’t: After Foker II Transferred to general surgery floor POD #22: 2m immunizations (except Hep B, parents deferred) POD #24: EGD/dil POD# 29: MBS shows aspiration of both thin/honey thick liquids; thus, pacifier dips only and slowly increased POs w/FT POD #30: EGD, bronchoscopy, no laryngeal cleft, no recurrence of TEF, moderate right bronchomalacia POD #37: EGD/dil (stricture 7mm, dilated to 10mm, steroids) POD #43: Follow-up bone x-rays: healed fractures (L humerus, R femur) POD #44: EGD/dil POD #69: EGD/dil On full PO bolus feeds (110cc q3 7x/da) plus breastfeeding POD #71 (HD #121): Discharged locally! Case #1 N.S. con’t: Readmits con’t DOL #169: admission after EGD/dil previous day, w/vomiting Fever upon admission, CXR w/opacity, Zosyn started, bcx negative Progressive resp. distress, O2 started, tx to ICU, CXR w/large PTX, to IR for pigtail chest tube placement/esophagram (large leak) and OR for EGD (two perforations), stent placement and CVL NPO/PN/IL, Zosyn, extubated POD #2 and weaned to RA, GT changed back to GJT for feeds, transferred back to general surgery floor Chest tube d/c’d POD #5, CXRs with enlarging PTX, chest tube replaced Pleural cultures w/mod Pseudomonas and abundant Hemophilus (sensitive to Zosyn) POD #12: Esophagram w/persistent leak, stent d/c’d, clips placed Case #1 N.S. con’t: Readmits DOL #125: s/p EGD/dil with steroid injection: routine observation, d/c’d next day DOL #146: s/p EGD/dil with steroid injection: admission d/t fever in PACU, no recurrence or other issues, d/c’d next day http://en.wikipedia.org http://emedicine.medscape.com Case #1 N.S. con’t: Readmits con’t DOL #169: admission after EGD/dil previous day, w/vomiting (con’t) POD #18: Esophagram without leak, chest tube removed, CXR no PTX/pneumomediastinum POD #23: Transitioned to GT feeds POD #25 & 26: Esophagram w/tight stricture and EGD confirming this, stricture not amenable to dilation POD #31: Esophagram w/persistent tight stricture but allowing passage of clear liquids, allowed to take PO ad lib Developed fevers, vomiting, loose stools (sick contact: gma): sepsis rule out (negative), stool studies/cultures w/+norovirus type 2, supportive therapy 4 2/14/2014 Case #1 N.S. con’t: Readmits con’t DOL #169: admission after EGD/dil previous day, w/vomiting (cont’) POD #37: Esophagram w/increased esophageal stricture POD #47: Taken to OR for esophageal stricture resection, NPO/PN/IL postop, Esophagram POD #10 with patent esophagus, no leak; feeds resumed POD #62 and 15: EGD/dil POD #70 and 23: EGD/dil (upto 12mm, steroid injection), Discharged locally! DOL #302: s/p EGD/dil (upto 12mm), admission for observation d/t “wisp” leak at end of procedure, CXR without PTX or pneumomediastinum, Unasyn x 24 hours, did well, discharged next day For those not as fortunate… Case #1 N.S. con’t: Follow-up Serial EGDs/dilations outpatient Last EGD/dil on DOL #379 (very minimal stricture, upto 12mm, oral budesonide x1 month postop) TYPES OF ESOPHAGEAL REPLACEMENTS Palliation w/esophagostomy and dependence on GJT feeds Advantages AND/OR Esophageal replacement Disadvantages http://courses.md.huji.ac.il Colonic Interposition Jejunal Interpositi on Gastric Transposition (“pull-up”) Readily available Good blood supply Easy to mobilize Adequate length usually attained Adequate length Readily available Caliber similar to esophagus Peristaltic activity Ease of procedure Adequate length usually attained Good blood supply Readily available Easy Blood supply Adequate length usually attained Rapid transit of food Readily available Leaks/strictures Redundancy of graft Slow transit time GER Possible dysphagia Transient diarrhea Precarious blood supply making needed length difficult to attain Possible respiratory problems GER Delayed GE Poor blood supply if need high in neck Transient dysphagia Possibly transient dumping syndrome Leaks/strictur es at cervical anastomosis May not be long enough GER Reversed Gastric Tube Case #2 S.K. 8yM with hx neurofibromatosis type 1 and severe midaortic stenosis (MAS) presents to BCH surgical clinic with associated medically refractory renovascular hypertension, failure to thrive from intestinal angina, and claudication symptoms of his lower extremities http://en.wikipedia.org/wiki/Abdominal_aorta 5 2/14/2014 Case #2 S.K. con’t Case #2 S.K.: CTA on 5/28/13 CT scan findings Marked stenosis of the abdominal aorta extending from just above the level of the celiac axis to just below the level of the renal arteries (~3cm) Severe stenosis of celiac artery and complete obliteration of his superior mesenteric artery, as well as stenosis centered around mesenteric vessels Midaortic Stenosis Coarctation of the proximal abdominal aorta (the origin of the renal and visceral arteries) Rare condition and accounts for 0.5-2% of aortic coarctations Can cause renovascular hypertension and ultimately end organ damage Treatment Surgical bypass with artificial or autologous graft Renal autotransplantation Percutaneous transluminal angioplasty with stenting New Approach to MAS Review of MAS for past 30 years (at BCH) Percutaneous intervention acutely brought down BP gradient Surgical technique offered longer period of time from reintervention (Porras etal., 2013) Novel surgical approach to lengthen the aorta Tissue Expander-Stimulated Lengthening of Arteries (TESLA) Developed by Dr. Heung Bae Kim (Kim etal., 2012) Performed in three stages Placement of tissue expander Serial filling of tissue expander Removal of tissue expander, resection of stenotic segment, and primary end-to-end anastomosis Case #2 S.K.: Presenting Symptoms BP maintained on 5mg Amlodipine daily Marked gradient between UE and LE BP (~48 mmHg) Persistent claudication Weight loss Over the next six months, the episodes of severe abdominal pain increased and his weight remained stagnant He was referred to general surgery clinic for further intervention Case #2 S.K.: 1st Stage of TESLA on 5/13/13 Specially made tissue expander (4x10cm, fill volume of 125 mL) with a complete circumferential suture tab and suture tabs coming from each end A “tunnel” is made behind the aorta and the inferior vena cava (IVC) with lumbar veins and arteries incorporated Tissue expander is placed in the retro-aortic space just above the bifurcation Tabs on the expander are placed against the psoas muscle and sutured into the fascia of the muscle Longer sutures are then applied directly to the iliac crest periosteum 6 2/14/2014 Case #2 S.K.: 1st Stage of TESLA on 5/13/13 con’t TESLA Procedure Micro-injection port placed on the right lower costal margin and its tubing tunneled into the abdomen and connected to the tubing from the expander Port is then secured with two silk ties 30 ml of blue-dyed saline is injected into the expander to assess expansion and tension on the blood vessels Barely palpable distal pulse of aorta was noted in OR http://www.nejm.org/doi/full/10.1056/NEJMc1210374#t=article Case #2 S.K.: 2nd Stage of TESLA Routine filling of tissue expander with ultrasound guidance in outpatient surgical clinic SK went to office three times a week for expander filling Unfortunately, tissue expander noted to be shifted to left side which was confirmed on CT scan Able to get to 145 ml of fill volume with noted 3 cm of anterior displacement of aorta, which was felt adequate to proceed with final repair early Case #2 S.K.: 3rd Stage of TESLA Postoperative CTA CTA on POD #9 with good aortic repair with patent vessels Celiac small but patent; left main renal artery tortuous but patent Case #2 S.K.: 3rd Stage of TESLA on 7/3/13 Stenosed aorta from beginning of renal arteries to above the level of celiac artery with noted sharp line of demarcation ~3 cm segment of aorta was resected, noted to contain origins of celiac, SMA and right renal artery After anastomosis secured, surgeons back-bled prior to removing clamps (except aortic clamp), restoring flow to lower limbs, hemostasis was satisfactory Noted excellent pulse in distal aorta The celiac artery, SMA and right renal artery were then each implanted on the newly lengthened aorta via aortic punch Case #2 S.K.: 3rd Stage of TESLA Postoperative Course Complicated by Tachycardia and hypertension, antihypertensives restarted Hematoma on ultrasound Feeding intolerance, PICC placed and PN initiated Able to advance diet, PN stopped and PICC removed Discharged home on POD #15 on Amlodipine 5 mg BID, DAT with nutritional shakes for extra calories Weight on d/c 19.8 kg 7 2/14/2014 Case #2 S.K.: Follow-up Case #2 S.K.: Follow-up CTA 12/3/13 Renal clinic 11/20/13 Gradient between upper and lower BP down to 20 mm Hg Able to titrate down on Amlodipine 2.5 mg BID Weight 21.3 kg Surgical Clinic 12/4/13 BP stable on decreased Amlodipine Only claudication symptoms with feet and ankle pain occurring after prolonged Taekwondo work-out Weight 20.7 kg Case #2 S.K.: Follow-up con’t CT angiogram on 12/3/13 New narrowing of the abdominal aorta at the site of the prior anastomotic cyst The aorta measuring 4mm in diameter vs. 6mm on the last scan Unsure if this is progressive disease or narrowing at the anastomosis but patient asymptomatic Should this become a problem in the future, next step would be to pursue balloon dilation of the stricture Next follow-up with surgery in 6 months with CTA Short Bowel Syndrome / Intestinal Failure Case #3 M.W. HPI: 4 mM (DOL #137) with history of gastroschisis and s/p resection of necrotic bowel w/subsequent short bowel syndrome transferred from OSH due to diagnosis of Short Bowel Syndrome, progressive PN-associated liver disease, and inability to advance enteral feeds Prenatal/Birth Hx: Prenatal diagnosis of gastroschisis. Mom was HSV+; she received Valtrex for viral suppression. Born at 36 weeks via induced vaginal delivery d/t poor fetal growth. Apgar 9/9. //media.thestar.topscms.com Incidence: ~ 0.7-1.1% of live hospitalized births Decreased gastrointestinal mucosal surface area and faster GI transit time Can lead to malabsorption, electrolyte abnormalities, dehydration, and ultimately malnutrition Most common cause of intestinal failure: significant reduction in functional small bowel mass, leading to inadequate digestion and absorption, with subsequent growth failure Functional definition: PN dependence for >3 months http://www.pediatricsconsultant360.com/ 8 2/14/2014 SBS Causes What Patients Can You Encounter //emedicine.medscape.com //emedicine.medscape.com Neonates adolescents (adults) Diagnoses Necrotizing enterocolitis Intestinal atresia(s) Gastroschisis Malrotation with midgut volvulus Total intestinal aganglionosis/HD w/SB involvement Trauma, vascular injury IBD, tumors, radiation enteritis, intestinal pseudoobstruction, enteropathies, motility disorders Combination of above SB loss vs. mucosal enteropathy/motility d/o vs. combination as causes of intestinal failure Initial hospitalization versus readmissions Second opinion http://bestpractice.bmj.com //surgery.med.umich.edu /img.medscape.com http://bms.brown.edu /emedicine.medscape.com www.pbase.com emedicine.medscape.com Case #3 M.W. con’t: PMH/PSH Ventilator Dependence, CLD, s/p tracheostomy (DOL #123) Hypertension, renal u/s c/w renal medical disease, received amlodipine (DOL #77-127) Gastroschisis: Placement of silo (DOL #1), return of bowel loops into silo d/t compartment syndrome (DOL #2), daily reductions of bowel, second stage of gastroschisis closure w/vicryl mesh (DOL #14), washout w/replacement of vicryl mesh (DOL # 20), mesh removal and final closure of gastroschisis (DOL #54) Bowel perforation: Ex-lap, repair of enterotomy (DOL #1) http://www.surgical-tutor.org.uk /emedicine.medscape.com /emedicine.medscape.com http://www.adhb.govt.nz Case #3 M.W. con’t: PMH/PSH con’t Bowel necrosis; Enterocutaneous fistula; Short bowel syndrome: Ex-lap, bowel resection with 25cm of jejunum and 15cm of colon left, repair of jejunocutaneous fistula, jejunocolonic anastomosis DOL #54) Gastrostomy placement (DOL #54) Oral aversion GERD, s/p Nissen fundoplication (DOL #89) http://www.pocketnurse.com Failure to advance enteral feeds, on trophic feeds only TPN dependency, progressive PNALD Hx Tracheal and urine +candida, treated w/Fluconazole, then peritoneal cx +candida, changed to Micafungin and Amphotericin B (dx’d DOL #12 and then DOL #20) This image cannot currently be display ed. http://www.mercedsunstar.com Case #3 M.W. con’t: PMH/PSH con’t Hx abdominal wall celullitis, concern for sepsis, treated w/Vanco and Zosyn (dx’d DOL #50) Bacterial overgrowth, started on Flagyl (DOL #82) Hx Enterococcal bacteremia, urine +serratia and pseudomonas, tracheal aspirate +serratia (thought to be colonization) (dx’d DOL #118) , treated with Zosyn Abdominal wall cellulitis and concern for sepsis (dx’d DOL #134), treated with Vanco and Zosyn CVL placement (DOL #1), CVL leak w/subsequent removal (DOL #80) and PICC placement, CVL replacement (DOL # 89) SBS: Major Predictors at Play for Ultimate Enteral Feeding Tolerance Length and portion of small bowel resected Presence or absence of ileocecal valve (or terminal ileum?) +/- Colon resection Adaptive and functional capacity of the remaining bowel Health of other organs that assist in digestion and absorption, PNALD/IFALD Bacterial overgrowth 9 2/14/2014 Bowel Length • Normal SB length • 217 ± 24 cm 27-35 weeks gestation • 304 ± 44 cm > 35 weeks gestation • At term, mean length is reported 250-300 cm • Another 2-3m addition to adulthood • Normal LB length • 30-40 cm at birth • Growing to 1.5 – 2 m in adulthood • Loss of intestinal length reduces exposure to nutrients to brush-border hydrolytic enzymes as well as pancreatic and biliary secretions; thus, limits digestion and absorption Bowel Conservation Limiting as much bowel loss as possible Utilization of a “second-look” operation for bowel of questionable viability Utilization of temporary closure to avoid risk of inducing abdominal compartment syndrome In case of intestinal atresia with pre-existing bowel dilation and limited distal small intestine (usually <35cm), a primary STEP bowel lengthening procedure may be done Re-establishment of bowel continuity via stoma takedown is associated with more rapid weaning from PN withfriendship.com Bowel Length and Area Resected Nutrient Absorption Sites To avoid life-long dependence on TPN, one needs 10-35 cm SB with intact ICV (or terminal ileum) 35-60 cm SB without ICV www.ivanhoe.com Nutrient Loss Based on Portion Resected Duodenum: iron, calcium, magnesium, zinc Jejunum: cholecystokinin w/decreased biliary and pancreatic secretions (malabsorption), motilin function (dysmotility) Ileum: fat absorption, ADEK Distal Ileum: bile acid reabsorption, vitamin B12 with intrinsic factor absorption Colon: delays in bowel adaptation, loss of energy from absorption of short-chain fatty acids, fluid and electrolyte imbalance www.columbiasurgery.org www.cmaj.ca Area Resected and Transit Time Normal intestinal transit time Rapid in jejunum Slow in distal ileum (ileal brake) Small bowel resection Transit time decreases Ileal resection reduces transit time more than duodenal/jejunal resection Gastric emptying is also more rapid with ileal resection Colon resection shortens transit time further Presence or Absence of Ileocecal valve ICV regulates the flow of enteric contents from SB to LB Absence of ICV (or terminal ileum?) Reduction of GI transit time Increased loss of fluid and nutrients Colonic bacteria can contaminate and colonize SB causing inflammatory response that damages the bowel mucosa that leads to further malabsorption (bacterial overgrowth) Bile salts and B12 may be deconjugated by the bacteria 10 2/14/2014 Adaptive and Functional Capacity of the Remaining Intestine: Key Points www.norathomas.com Ability to compensate (area of rxn, other trophic stimuli) Ileum more able to adapt and compensate for proximal bowel loss Intestinal adaptation Gross anatomic and histological changes, lengthening of villi, increased intestinal absorptive surface area, and improved absorptive function Digestive enzymes are decreased (functional immaturity); thus Functional improvement does not immediately follow increase in absorptive surface area The younger the patient, the bigger the advantage d/t opportunity for further growth Enteral nutrition as a stimulant of mucosal growth Case #3 M.W. con’t: Hospital Course at BCH: 4m-8m (DOL #137-255) Neurological Prolonged narcotic wean Dandy-Walker Malformation Deformational plagiocephaly (fitted for helmet) http://www.medgadget.com Respiratory Weaned off ventilator (DOL #141) to CPAP then trach collar 28% FiO2 Failed trach capping trial (DOL #176) Periodic increase in secretions requiring optimization of pulm regimen and/or antibiotic coverage DL/bronch and kenalog injection of tracheal granuloma (DOL #137) DL/bronch, excision of suprastomal granulation tissue , baloon dilation of subglottis, tracheostomy downsized from 4.0 Neo to 3.5 Pedi Shiley (DOL #167) Case #3 M.W. con’t: Hospital Course at BCH: 4m-8m (DOL #137-255) con’t FEN/GI con’t Slow feed advancement, immodium initiated, developed bilious vomiting and high stool output on multiple occasions, back down to trophic feeds only Only PO stim d/t marked oral aversion UGI/SBFT: No obstruction ; transit ~15 min (DOL #145) Contrast enema: Caliber change between markedly dilated small bowel (likely SB) and nondilated distal colon, no obvious stricture (DOL #161) Rectal suction biopsy to r/o Hischsprung’s disease: + ganglion cells (DOL #188) UGI/SBFT: consistent with Hx SBS, no obstruction, transit ~40 min (DOL #247) Citrulline Level Reflection of functional absorptive capacity Non-structural amino acid primarily synthesized in intestinal mucosa reflecting mucosal mass Highly positively correlated with intestinal length and ability to wean from PN Persistent level <12 µmol/L, patients usually unable to wean from PN Wikipedia.org Case #3 M.W. con’t: Hospital Course at BCH: 4m-8m (DOL #137-255) con’t CV EKG (DOL #137) showed RV hypertrophy suspicious for Pulmonary hypertension, ECHO (DOL #249) was of poor quality but overall normal ventricular function, Pulm HTN not excluded by cardiac service, planned f/u FEN/GI Continued on TPN (cycled to 14 hours prior to dispo), started on Ursodiol Initiated on Omegaven for PNALD (BILIs DOL #137 7.9/5.3, 1month later 1.2/0.7, then normalized and wnl since) Continued on maximal PPI therapy Citrulline level on admit = 10 µmol/L, on discharge = 23 /santinosmiles.wordpress.com Case #3 M.W. con’t: Hospital Course at BCH: 4m-8m (DOL #137-255) con’t GU Circumcision (DOL #167) Heme Non-obstructive clot in RIJ noted on routine Doppler u/s, probably d/t prior PICC line, chronic in nature, not treated per hematology ID Abdominal wall cellulitis and concern for sepsis on admission, completed Vanco/Zosyn course (dx’d DOL #137) Increased secretions thought to be d/t viral infection (dx’d DOL #195) Increased secretions 2/2 ?Serratia and Pseudomonas colonization, received Bactrim IV course and Tobramycin nebulizer treatment (dx’d DOL #244) TLD PICC line removal, placement of a tunneled CVL (DOL #167) /surgery.med.umich.edu http://abbottnutrition.com 11 2/14/2014 Special Aspects of Fluid and Nutritional Therapy in SBS Patients SBS Medical Management www.peteducation.com • Goal • To maintain normal growth, promote intestinal adaptation, and avoid complications associated with intestinal resection and PN • Fluid, Electrolytes, and PN • Large fluid losses common • Usually start w/standard PN solution for age and titration based on fluid/electrolyte balance after such losses stabilize • TPN indicated until SB growth and adaption permit growth on enteral nutrition • Increased needs • Enteral Feedings • After postoperative ileus resolves and fluid/electrolyte status stabilizes Parenteral nutrition Enteral nutrition Prokinetic agents Controlling stool output Hormonal therapy http://www.shortgutsupport.com Parenteral Nutrition SBS Enteral Nutrition http://www.kendallhq.com ttp://nursingcourses.wordpress.com Life-saving therapy in children with intestinal failure Goal is to provide adequate caloric intake, macronutrients and micronutrients to optimize growth and development Tailored to meet specific individual requirements Lipids seems to be the major factor in PNALD/IFALD: lipid-limited and/or fish-oil based formulas are being investigated as to their effects on prevention and/or reversal of PNALD/IFALD Reduced intestinal absorptive capacity leads to low bicarbonate level and low sodium level Risk factors of PNALD/IFALD, sepsis, mucosal atrophy, and bacterial overgrowth SBS Enteral Nutrition con’t Breastmilk Immunologic and anti-infective factors, growth factors, nucleotides, glutamine, other amino acids aiding in intestinal adaptation Other formulas (somewhat controversial re ideal formula) (Neocate/Elecare) Consider age, functional anatomy and capacity of digestion and absorption Complex nutrients tend to stimulate intestinal adaptation more effectively; however, limited mucosal surface area leads to lactose, protein, and long-chain fatty acid malabsorption Thus, protein hydrolysate formula or amino acid-based formula that is lactose free and has MCT is used to facilitate absorption Protein is generally tolerated well; fat high caloric density/neurodevelopment/less adverse effect than carbohydrates Initiation of enteral nutrition is the most important factor as it obviates IFALD and catheter-associated bloodstream infections (CABSI) Continuous feeds provide constant saturation of carrier transport proteins NG, GT to start, later +/- GJT Trophic feeds as soon as feasible Advancing of feeds based on stool output, gastric residuals, and s/s malabsorption As feeds advance, PN is weaned Initiation of small amounts of PO feedings asap BID-TID to promote sucking/swallowing and prevent oral aversion Bolus feeds, once tolerating full continuous feeds Usually start with 1hr worth bolus and transition slowly to Q3-4 hrs Nutrient Deficiency in SBS Most SBS patients have absent or compromised terminal ileum While on PN, these get repleted IV Regular assessment of vitamin/trace element levels (serum vitamin A, 25-OH vitamin D3, vitamin E, Zinc, vitamin B-12, and PT/INR as a reflection of vitamin K) http://www.faqs.org 12 2/14/2014 SBS Feeding Advancement Principles SBS Feeding Advancement Principles con’t Quantify feeding intolerance primarily by stool/ostomy output and secondarily by reducing substances/gastric aspirates/dehydration s/o < 10 stools/day or 2cc/kg/hr (advance by 10-20 cc/kg/d) s/o 10-12 stools/day or 2-3 cc/kg/hr (no change) s/o > 12 stools/day or >3cc/kg/hr (reduce or hold feeds) RS < 1% (advance feeds per s/o) RS = 1% (no change) RS > 1% (reduce rate or hold feeds) Gastric aspirate < 4x previous hour’s infusion (advance feeds) Gastric aspirate > 4x previous hour’s infusion (reduce or hold) No s/s dehydration (advance feeds per s/o) Present s/s dehydration (reduce or hold) Causes of Increased Gastric Output and/or Vomiting in SBS Patients Underlying dysmotility in a subset of SBS patients Ileus Stricture, adhesions/SBO Acute infection (GI or non-GI) Bacterial overgrowth http://radiographics.rsna.org http://radiographics.rsna.org http://radiographics.rsna.org ispub.com Tolerance assessment no more than twice per 24hrs and no more than 1 advancement in 24hrs Ultimate goals (patient-dependent) ~100-140 kcals/kg/d If ostomy/stool output prevents advancement at 20 kcal/oz for 7 days, then increase kcal density Weaning of PN simultaneously as advancing feeds Prokinetic Agents in SBS Relatively frequent in patients with gastroschisis Aside from true intestinal pseudoobstruction, motility issues tend to improve with time Erythromycin: improves gastric emptying and antroduodenal coordination; azithromycin also possible (Octreotide: may accentuate bowel ischemia) (Metoclopromide: can induce tardive dyskinesia; black-box warning) (Domperidone: treatment of gastroparesis; only under investigational FDA use; can induce cardiac arrhythmias) Cisapride: promotes motility in SB and the stomach; under investigational FDA protocol; occasional use http://www.ajronline.org Causes of Increased Stool/Ostomy Output in SBS Patients Hormonal Therapy 2 cc/kg/hr http://www.gaumard.com Baseline stool output in SBS patients Higher due to limited absorptive surface Continuous feeds tend to cause less diarrhea in SBS patients Worsening stool output Reaching or going over the maximum present absorptive capacity Quantify feeding intolerance primarily by stool/ostomy output and secondarily by reducing substances/gastric aspirates/dehydration Small bowel bacterial overgrowth History of recurrent abdominal distention, foul-smelling stools, and flatulence Acute change in stool output, associated s/s, +/- ABX use, ID exposure If no mechanical or infectious etiology exists, loperamide can be used to decrease stool/ostomy output; stomal refeeding also a strategy in patient with a long mucous fistula Hormonal manipulation in attempt to improve bowel adaptation Glucagon-like peptide 2 (GLP-2) Acts on bowel itself and leads to marked bowel adaptation esp. in porcine models Long-acting GLP-2 analogue (teduglutide) Can improve water and to a lesser degree nutrient absorption (studied in adults with SBS) Trials are ongoing Theoretical concerns about induction of GI malignancy exist http://www.nature.com 13 2/14/2014 Case #3 M.W. con’t: Readmits apps.childrenshospital.org 1/13-2/4/2011 (DOL# 314-336) Short Bowel Syndrome, Inability to Advance Feeds, Bowel Dilation DOL#315: DL/bronch (ORL) and ex-lap, LOA, STEP procedure (88cm SB to 115cm SB), revision of jejunocolic anastomosis, liver bx (mild portal inflammation and fibrosis), and GT ex-change (GNS) NPO/TPN/OM, tx’d from ICU POD #3, UGI/SBFT POD #10 no leak/obstruction (30 min transit), GT clamped and feeds started, advanced to 10cc/hr, back on Imodium, PN cycled to 12 hours Attempted trach capping trial but d/t increased WOB additional attempts postponed till later 4/7-4/15/2011 (DOL #398-406) ~13 month Respiratory distress/sepsis Vent support, Vanco/Zosyn (Redman’s w/Vanco), tx’d from ICU HD #4 Bcx +SNA, resp cx +E-coli, +paraflu, Zosyn x7d, Vanco x10d Citrulline level = 15 µmol/L Case #3 M.W. con’t: Readmits con’t 6/14-6/17/2011 (DOL #466-469) ~15 month Fever of unknown origin, sepsis workup negative 6/28-7/11/2011 (DOL #480-493) DL/bronch, trach decannulation, O2 wean 8/2-8/6/2011 (DOL #515-519) Fever, Klebsiella line infection (Zosyn x14 days) http://www.saferhealthcarenow.ca Con’t Elecare 20kcal/oz at 30cc/hr MBS 8/4/2011 cleared him for purees 8/15-8/17/2011 (DOL #528-530) Fever of unknown origin, sepsis workup negative 8/24-9/1/2011 (DOL #537-545) Fever, increased stool output, sepsis workup negative, feeds restarted (upto 32cc/hr) Citrulline level = 39 µmol/L This image cannot currently be display ed. Case #3 M.W. con’t: Readmits con’t 11/7-11/17/2011 (DOL #612-622) Fever, rigors, increased resp distress, still on Zosyn HD #2, CVL removed in OR, changed to Meropenem d/t multiple infections, Bcx + Klebsiella, sensitive to Unasyn (total x14 days) HD #7, CVL placement and ECHO (worsening PVS and RV hypertrophy), need for continued oxygen therapy, plan for repeat ECHO 12/21-12/27/2011 (DOL #656-662) ~21 month Bloody stools, sepsis workup negative, feeding intolerance, cont w/heme positive stools, restarted on bacterial overgrowth regimen, advanced back to 32cc/hr 1/4-1/4/2012 (DOL #670) ?Dehydration, labs wnl, tolerating feeds Case #3 M.W. con’t: Readmits con’t 5/9-5/27/2011 (DOL #430-448) Transfer from OSH after 12hr PN volume given over 1hr w/subsequent seizures and pulm edema, CVL damaged/removed, bcx +SNA, no more s/z activity, required PICU stay, now in need of a new CVL Bradycardia, EKG RV hypertrophy HD #7 Insertion of cuffed CVL; sedated ECHO (RV hypertrophy, and PVS) HD #8 Lung perfusion scan w/decresed oxygenation 37% L vs. 63% R HD #10 CTA w/mild/mod PVS left, mild PVS right lower PV HD #14 Cardiac cath w/RVP 2/3 systemic sensitive to oxygen therapy RV hypertrophy felt to be RLT to CLD and not PVS, plan to keep on oxygen to keep pressures low, cont w/stable sinus brady (50’s to 80’s) Intolerance of feed advancement, stool cx negative, finally able to adv to ½ rate (8cc/hr) Vancomycin x 14d for SNA line infection Case #3 M.W. con’t: Readmits con’t 9/20-10/1/2011 (DOL #564-575) ~18 month Fever, vomiting, loose stools, sepsis workup negative, stool cultures negative Feeding intolerance, UGI/SBFT w/interval increase in bowel dilatation, slower transit time, restarted feeds and advanced to 20cc/hr 10/13-11/3/2011 (DOL #587-608) Watery diarrhea despite stopping feeds, then low grade fever Bowel rest, stool cultures negative, UGI/SBFT unchanged (moderate bowel dilatation and transit time), no improvement EGD/C-scope on HD #6 w/+bacterial overgrowth (E-coli, Strep viridans, other GPC, and Peptostreptococcus species), started on Cipro alternating w/ Flagyl each 1wk/month, feeds slowly restarted to 20cc/hr, cont PN/OM CVL broke, repaired at bedside, then Bcx +Pseudomonas aeruginosa, Zosyn x14 days Case #3 M.W. con’t: Readmits con’t 1/11-1/20/2012 (DOL #677-686) Fever, vomiting, dark stools, +sick contact (gastro)http://microblog.me.uk/268 Septic workup negative, bowel rest, stool cx w/+cdiff, IV Flagyl and enteral Vanco, then monotherapy w/enteral Vanco (total x14d), feeds restarted upto 28cc/hr, imodium restarted 2/27-3/15/2012 (DOL #724-741) ~2 years Fever, lethargy, Bcx +GNR, Zosyn/Gent, transferred from OSH Vanco/Zosyn, Bcx +Klebsiella, changed to Cefepime (x14 d total), cdiff negative Inability to restart feeds, bowel rest, then slowly upto 20cc/hr w/imodium 3/19-3/27/2012 Fever, fatigue, septic workup negative, slowly advanced back to 20cc/hr 14 2/14/2014 Case #3 M.W. con’t: Readmits con’t 4/11-4/30/2012 Bilious vomiting, watery stools, low grade fever, Flagyl started empirically outpatient Septic workup negative, bowel rest, GT changed to GJT 4/13/2012 Stool +cdiff, started enteral Vanco (x14 days) EGD/c-scope 4/18/2012 w/cx Klebsiella resistant to Cipro, changed to Gentamicin for bacterial overgrowth (together w/Flagyl) Citrulline level = 21 µmol/L Barium enema 4/25/2012 w/mildly dilated colon and SB W/many bumps, slowly advanced to 10cc/hr via JT 5/20-5/25/2012 Lethargy, low grade fever, vomiting Bcx +SNA, V/Mero changed to Cefazolin (x14 days), initially NPO advanced to 10cc/hr via JT w/imodium Case #3 M.W. con’t: Readmits con’t 7/25-7/28/2012 Admission for EGD/c-scope/manometry testing by GI (normal, tortuous colon, normal fasting/medication challenge manometry) 8/1-8/6/2012 Fever, lethargy, septic workup negative, higher stool output/imodium restarted, GT clamped 8/7-8/21/2012 “Bubble” in CVL, brought to OSH where leukocytosis WBC 18.3, hypotensive 50’s, bilious emesis x1, transferred to BCH Vanco/Mero rule out, bcx NGTD, line repaired at bedside UGI/SBFT 8/9/2012 w/no concerns Exchange of GT to GJT in IR 8/10/2012, advanced to 7cc/hr via JT CVL leak again, to OR for CVL replacement over guidewire 8/12/2012 GJT exchange to skin-level GJT 8/14/2013, feeds increased to 15cc/hr over 20 hours Case #3 M.W. con’t: Readmits con’t 6/9-6/18/2012 ~2 ¼ years Feculent output from GT, vomiting KUB nonobstructive, fluid resuscitated, bowel rest, bcx NGTD, slowly restarted GJT feeds (10cc/hr) Note 6/8-6/25: Transplant team consultation for isolated intestine transplant, not meeting criteria (multiple bouts of CVL/enteral infections, recent conversion to GJT, tolerating some JT feeds currently, need to give more time to assess) 6/27-7/9/2012 Feculent emesis, increased GT output KUB nonobstructive, bowel rest, Bcx NGTD GJT exchanged in IR, then tip too proximal again, GJT exchange again No stool x 48 hours, glycerin w/+stool, unable to restart feeds, stool output increased (imodium restarted), GJT changed back to MICKey for comfort measures under his vest and kept to gravity Plan for manometry testing outpatient Case #3 M.W. con’t: Readmits con’t 9/7-9/15/2012 ~ 2 ½ years Decreased stool output, brown GT output, low grade fever Septic workup negative, stooling back at baseline, JT feed restarted Sinus bradycardia into 50’s asleep, sedated ECHO 9/12 w/mild pulm hypertension, lung perfusion scan 9/12 improved (L43%/R57%), EKG w/right axis deviation and RV hypertrophy, recs to continue supplemental O2 9/30-10/8/2012 URI, started Amox by PCP, then desats, CXR at OSH w/PNA, changed to Azithromycin, coming for eval CXR without consolidation, septic rule out negative, though cont w/desats into 80’s (upto 2L O2), increased neb therapy, cards/pulm rec 14 day Cefepime and cont O2 No change to JT feeds (Elec JR at 15cc/hr) Citrulline level = 20 µmol/L Wikipedia.org Case #3 M.W. con’t: Readmits con’t 12/18-12/23/2012 ~2 ¾ years URI s/s, wheezing, fever, +sick contact (URI) CXR w/perihilar interstitial markings, septic workup negative, supportive therapy (O2, nebs added 3% NS nebs, viral DFA/flu neg) Tolerating feeds at 26cc/hr 12/28-12/31/2012 Increased O2 requirement and PNA on CXR at OSH V/Z started, bcx NGTD, changed to Augmentin for PNA (x10 days), supportive therapy Attempt at unsedated ECHO to eval RVP but unable 1/13-1/17/2013 Broken CVL, clamped, line replaced over guidewire in OR 1/14 Septic workup negative, due for GJT exchange (done), feeds upto 27cc/hr Case #3 M.W. con’t: Readmits con’t 1/21-2/7/2013 Bilious vomiting, worsened, lethargy Aggressive IVF resuscitation, still oliguric, abdominal pain, inconsolable, HD #2 taken to OR for Ex-lap w/LOA but no focal point found, ARBF postop, then JT restarted and advanced to 5cc/hr Left hip cellulitis, u/s negative for joint involvement/collections, received 7d Vanco 3/12-3/15/2013 ~3 years Line repaired x2 at OSH, no ABX given, presents w/fever and irritability Vanco/Zosyn, developed hives w/Zosyn, got Epinephrine/Benadryl, changed to Meropenem, d/c’d w/negative rule out Found to have swelling/erythema R submandibular neck w/enlarged lymph nodes (largest 1.6cm on u/s, inflammation), afebrile, throat wnl, CRP 6.3, completed 7d Ancef for infected lymph node Feeds increased to 18cc/hr x 20 hours This image cannot currently be display ed. http://learnpediatrics.com 15 2/14/2014 Case #3 M.W. con’t: Readmits con’t Case #3 M.W. con’t: Readmits con’t 4/6-4/13/2013 Vomiting, becoming feculent, increased GT output Septic rule out negative, bowel rest, then slow restarting of JT feeds upto 26cc/hr, GT clamped Citrulline level = 26 µmol/L 5/2-5/9/2013 Fever, septic workup negative Underwent EGD, DL/bronch on 5/6 as previously scheduled (reassuring findings) 5/12-6/13/2013 Fever, Bcx +GNR at OSH, transferred for further care Vanco/Mero, bcx + Enterococcus and Enterobacter, changed to V/Cefepime, completed 14d ABX, test of cure Bcx + w/Enterococcus on 6/3/2013, to OR for CVL removal, ECHO without signs of vegetation, changed to Vanco/Mero, then Vanco only x 14 days since 1st negative, PICC placed in meantime for TPN/OM, feeds upto 15cc/hr SBS Complications 7/17-7/22/2013 Fever, fatigue, increased stools Septic workup negative, JT feeds restarted http://drhem.com 7/28-8/10/2013 Bilious vomiting, septic workup negative, unable to restart JT feeds w/stable plain films and no obstruction/stable bowel dilatation on UGI/SBFT 8/1/2013 8/25-8/29/2013 ~3 ½ years Fever, fussy, not tolerating feeds, ?viral enteritis Septic workup negative, able to restart feeds (upto 8cc/hr) PNALD / IFALD /1.bp.blogspot.com Intestinal failure associated liver disease (IFALD) / PN-associated liver disease (PNALD): cholesterol gallstones, cholestasis, steatorrheic liver disease, portal fibrosis, cirrhosis, liver failure Catheter-associated bloodstream infection (CABSI) Vein thrombosis, loss of access Hyperacid secretions leading to impairment in carbohydrate and protein digestion and absorption, fat lipolysis and then to diarrhea Vitamin/mineral deficiencies (esp. once off PN) Bacterial overgrowth Oxalate renal stones Major cause of morbidity/death in children with SBS Pathophysiology is unclear but vegetable (soy) oil-based lipid emulsions (VBLEs) are associated with liver injury Accumulation of potent pro-inflammatory mediators derived from omega-6 fatty acids Dysfunctional bile homeostasis due to high levels of phytosterols PN dependence and DBILI persistently >2 mg/dL (34 µmol/L) not due to other diseases Prevention is key Push enteral nutrition Avoid overfeeding, use lower lipids or Omegaven as indicated Cycle PN off for at least 2-6 hours/day Ursodiol to help body digest fats Aggressively prevent and treat infections http://blog.bluechipcommunication.com.au Fish oil-based Lipid Emulsion (FOLE) vs. Lower VBLE/Combination Catheter-Associated Bloodstream Infections (CABSI) http://www.drug3k.com FOLE – Omegaven (only under FDA for compassionate use; 10% lipid emulsion) Rich in omega-3 fatty acids, low in omega-6 fatty acids, no phytosterols Significant decrease in TBILI, DBILI, TG, CRP, cholesterol, LDL, and trend towards increase in HDL Reversal of PNALD; potentially also prevents PNALD (studies underway) Risks Essential fatty acid deficiency, but no evidence to date Longterm outcomes are investigated VBLE – High in omega-6 fatty acids, contain phytosterols Lower VBLE (Lipid restriction to 0.51g/kg/d; 20% Intralipid emulsion) Possible reversal of PNALD Caloric requirements must be met by glucose Risk of essential fatty acid deficiency (documented cases esp. w/0.5g/kg/d) Longterm neurodevelopmental outcomes are unknown Combination emulsions Even small amount of VBLE leads to some liver injury/cholestasis Need for CVL for PN administration http://topnews.ae Aseptic technique Effort to avoid ligating the vessel (esp. large vessel so can be reused in the future thus prevent “loss of access”) Line care (various protocols) Any suspicion, s/s fever, lethargy, irritability, ileus blood culture High incidence of CABSI d/t enteric organisms, broad spectrum ABX If fungal infection or evidence of hemodynamic instability, CVL removal Ethanol locks: promising role in preventing CABSI MORE STUDIES NEEDED / SOME ARE UNDERWAY /santinosmiles.wordpress.com 16 2/14/2014 Bacterial Overgrowth Complicated SBS: Options http://en.wikipedia.org • In upto 60% patients with SBS • Bowel dysmotility and dilation offer ideal environment for abnormal bacterial growth • Leads to: abdominal pain, worsening motility, mucosal ulceration with bleeding, deconjugation of toxic byproducts such as D-lactic acid • Also thought to potentiate translocation and hence septicemia • Treatment is largely empiric • Possible to obtain duodenal culture aspirate to guide therapy esp. in challenging cases • Enteral ABX x 7days followed by a period of no ABX (anaerobic and GNR coverage) • Bowel tapering if medical management becomes refractory • Probiotics are NOT recommended in PN-dependent SBS patients SBS Surgical Options Especially in those who are extremely difficult or impossible to wean from parenteral nutrition Very short remaining small bowel segment (esp <35 cm), IFALD, CABSI, marked dysmotility, and bacterial overgrowth Enteral feedings Wean and cycle TPN as much as possible Omegaven and/or 1g/kg Intralipid Meticulous line care, bowel prophylaxis Surgical options www.baxa.com STEP Procedure Nontransplant operations Intestinal lengthening procedures: Bianchi, STEP, other Intestinal tapering for dilated dysfunctional bowel segments Stricturoplasty Creation of intestinal valves or reversed bowel segments for patients with rapid intestinal transit times Multivisceral or intestinal transplantation surgery.med.umich.edu https://gi.jhsps.org First reported in 2003 (initial idea by Dr. H.B. Kim) apps.childrenshospital.org Technically straightforward zig-zag lengthening and tapering of intestine To increase intestinal length and/or taper dilated proximal bowel in SBS patients and ameliorate bacterial overgrowth International STEP Data Registry (incepted 2004) In 2010, >110 patients Overall survival rate 89% ~50% of patients with refractory IF to maximal medical management were converted to full enteral feeds Median time to enteral autonomy ~21 months 5 patients eventually required SB transplant www.pediatricsurgerymd.org Case #3 M.W. con’t: Follow-up Last CAIR Clinic Visit 10/21/2013 Slowly advanced on JT feeds outpatient upto 18cc/hr x 20 hours/d Stage 1 babyfood via GT 3x/day Mom happy w/developmental progress, speech PN/OM providing 66 kcal/kg/d and Elecare JR giving 25 kcal/kg/d A/AE: Vanco (Redman’s), Zosyn (hives) Meds: ETOH locks 0.5mL IV 3x/wk, Albuterol prn, Atrovent 2puffs BID, Flovent 2puffs BID, Ursodiol 150mg JT BID, Imodium 6mg JT BID, Ferrous Sulfate 30mg JT daily, Cholecalciferol 4,000 units JT daily, Gentamicin 29mg BID x 7 days via JT every other week, Miconazole ointment prn topically for fungal rash PE: Unremarkable (CVL and GJT in place/intact) Plan: Transition to Compleat Pediatric feeds, increasing PN calories for weight/growth, d/c cholecalciferol d/t high vit D level, set up due for cardiology follow-up Case #3 M.W. con’t: Yet Another Re-admission http://www.bioridgepharma.com 1/9-1/13/2014 ~ 3 ¾ years Transfer from OSH d/t presentation after anaphylactic reaction to Pediatric Compleat (hives, face/tongue swelling, resp. s/s) S/s resolved, transitioned back to Elecare 30kcal/oz via JT without issues (at 34cc/hr x 20 hours/day), PN over 14 hours Allergy team c/s (RAST testing for major components in Pedi Compleat and other major food allergens), follow-up arranged Routine GJT ex-change in IR F/u liver ultrasound (slight prominence of the CBD, small gallbladder with likely sludge, and abnormally small caliber of the visualized portion of the hepatic IVC (all similar to prior imaging), Doppler deferred by radiology d/t normal on prior imaging) 17 2/14/2014 Case #4 N.N. Now 21 year old male s/p isolated intestine and renal transplant ~1 year ago for short bowel syndrome secondary to ischemic injury from mesenteric venous thrombosis after laparoscopic appendectomy for perforated appendicitis Case #4 N.N. con’t: PMH/PSH At age 15 yr, presented to OSH on 2/26/08 with ruptured appendicitis Laparoscopic appendectomy with drainage of abdominal abscess Developed mesenteric venous thrombosis OR on POD #2 for Exploratory laparotomy (Ex-lap), small bowel resection of 10 cm of gangrenous bowel OR on POD #3 for Ex-lap, second look with further small bowel resection of 15cm d/t necrosis OR on POD #6 for Ex-lap with anastomosis of small bowel and abdominal closure http://photos1.blogger.com/img/210/3870/640/DSCN0665.jpg Mesenteric Venous Thrombosis Rare condition (1 in 5,000 to 15,000 admissions) Accounts for 6 to 9 % of acute mesenteric ischemia Mesenteric arterial thrombosis is most common cause of ischemia Predisposing factors Prothrombotic states, vessel wall injury, and venous stasis Factors causing vessel wall injury include inflammatory causes like appendicitis, peritonitis, IBD, and intra-abdominal surgeries http://en.wikipedia.org/ Mesenteric Venous Thrombosis con’t http://www.najms.org/old/resources/full+text+329-332+Postoperative+mesenteric+venous+thrombosis.htm http://drkeyurbhatt.blogspot.com/2011/ 03/case-acute-mesenteric-venousthrombosis.htm Case #4 N.N. con’t: PMH/PSH Remained in the ICU intubated Developed hemoperitoneum and returned to the OR on 3/8/07 (POD #10 from appendectomy) for Ex-lap with lysis of adhesions and abdominal washout Continued with bloody stools and pressor requirement Colonoscopy performed 3/13/08 (POD 15) secondary to rectal bleeding with grossly normal mucosal surface; rectal biopsy with ulcerated mucosa EGD with gastric ulcer Tagged RBC scan without bleeding source Abdominal CTs noted pan-colitis and no evidence for abscess formation 18 2/14/2014 Case #4 N.N. con’t: ROS upon transfer Case #4 N.N. con’t: ROS upon transfer Pulmonary Intubated from 2/26-3/8/08 and 3/10-3/15/08 Cardiovascular Briefly on 3 pressors while in septic shock Renal Developed acute renal failure secondary to ATN Initially on CRRT, transitioned to hemodialysis three times a week Oliguric, on fluid restriction. Hematology Family history of hypercoaguable state (father's side) Work-up ongoing at time of transfer ID Persistent fevers despite broad-spectrum antibiotics, blood cultures negative Multiple CT scans of abdomen without evidence of abscess http://crashingpatient.com/wpcontent/images/part1/cuffed%20dialy sis%20cath.jpg Case #4 N.N. con’t: Transfer to BCH Transferred to ICP at BCH in 4/4/08 for further care He was followed by GI service closely Initial CT scan Diffuse mucosal enhancement throughout the entire colon and rectum, most pronounced in the ascending colon, transverse colon, and descending colon, and least pronounced in the rectosigmoid Moderate to large amount of diffuse ascites Liver, gallbladder, pancreas, and spleen are within normal limits Noted relatively diminished enhancement of the kidneys Follow up scans on 4/30, 5/21, and 6/13 continued to show pancolitis Case #4 N.N. con’t: Workup at BCH con’t Cardiovascular Non-sustained ventricular tachycardia 5/08; then hemodynamically stable; evaluated by Cardiology ECHO (4/16, 5/9, 5/12, 5/30) showed normal anatomy, no vegetation, low normal LV function, and trace pericardial effusion EKG showed incomplete bundle branch block, normal QTc, no evidence of atrial or ventricular hypertrophy Had episode of chest pain with transient elevation in troponins, leading to gated cardiac CTA for coronary arteries and function on 5/12; this showed normal anatomy and coronary function. He was started on Metoprolol XL No arrhythmias since 5/31/08 EKG and signal-averaged EKG before discharge with Q waves consistent with old ischemia ECHO on 7/15/08 done because of a new gallop; no major changes from before. Case #4 N.N. con’t: Workup at BCH Neurological NN suffered from chronic abdominal pain, with limited response to narcotics TENS unit used with success A trial of Neurontin proved to have no added benefit Pulmonary Stable on room air during hospitalization LLL pneumonia, s/p treatment Bilateral effusions identified on CT on 3/27 and 4/9/08. Case #4 N.N. con’t: Workup at BCH con’t GI/FEN Feeding intolerance due to abdominal pain and nausea Continued on full parenteral nutrition with resulting cholestasis with max indirect/direct bilirubin of 4/2.5; bilirubin on discharge normalized to 0.4/0.2 Electrolytes fairly stable and controlled through dialysis Patient continued on calcitriol for vitamin D supplementation, Ursodiol for cholestasis Colonoscopy on 4/23/08 showed severe segmental inflammation characterized by congestion, erythema, friability and confluent ulcerations in the entire colon 19 2/14/2014 Case #4 N.N. con’t: Workup at BCH con’t GI/FEN con’t CT angiography on 4/30/08 showed decreased mucosal enhancement of colon, dilated small bowel, bleeding in left colon and normal vessels Abdominal US on 4/24/08 showed normal portal venous pressure Endoscopy 5/29/08 showed diffuse moderately erythematous, edematous and friable mucosa with no bleeding found in the entire stomach Sigmoidoscopy on 5/29 /09 showed patchy areas of severely friable, erythematous, edematous mucosa with no bleeding UGI on 6/2/08 showed terminal ileum narrowing in caliber with a loss of the normal mucosal fold pattern, no discrete stricture identified Case #4 N.N. con’t: Workup at BCH con’t Hematology Completed hypercoagulation workup without any inherited coagulopathy or risk factors Treated with subcutaneous heparin and transitioned to oral warfarin starting 6/27/08 Immunology/Rheumatology Consulted for hypogammaglobulinemia Low vaccine titers, borderline low memory B cells (CD 27+), and low T and B cells, and poor T cell response to tetanus antigen Received IVIG therapy x2 for low levels, thought to have CVID, work-up ongoing Consulted due to concerns for vasculitis; autoimmune workup negative Case #4 N.N. con’t: ICP Discharge Discharged home on 7/26/08 with plan to return for OR in August for exploratory laparotomy for persistent nausea and feeding intolerance Parenteral nutrition (PN) cycled over 12 hours with restricted clears diet by mouth Discharge meds Metoprolol, Prednisone 1 mg BID, Omeprazole, Zofran, Ursodiol, Warfarin, Levothyroxine, Somatropin daily, Calcitriol, Nephrocap PRN meds of Oxycodone, Promethazine, Nystatin powder, hydrolatum Case #4 N.N. con’t: Workup at BCH con’t Renal Hemodialysis catheter changed on admission 3x/wk HD schedule for several months Erythropoeitin w/HD 2L/day fluid restriction Evaluation for kidney transplant initiated Endocrine http://clinicindelhi.com/hemodialysis/ Hypothyroidism, likely from Hashimoto thyroiditis (Anti-TPO elevated at 15.4 and Anti-thyroglobulin <20 on 5/24/08) Started on Synthroid Growth hormone started Case #4 N.N. con’t: Workup at BCH con’t Infectious Disease Blood cultures remained negative No evidence of abscess formation on multiple scans (4/23, 4/30, 5/21, 6/13/08) CMV studies 5/23/08: shell antigen negative, IgG positive, IgM negative, PCR negative C. diff infection (positive on 4/13/08), treated with metronidazole, and has remained negative thereafter VRE swab positive 5/26, 6/3, and 6/6/08, all other cultures negative Case #4 N.N. con’t: Readmit 8/20/08 Pre-operative UGI on 8/14/08 Distal jejunum and ileum markedly abnormal, featureless with multiple strictures 20 2/14/2014 Case #4 N.N. con’t: Readmit 8/20/08 Planned Ex-lap surgery 8/20/08 Two small bowel strictures, one 112 cm from Ligament of Treitz and another 136 cm from Ligament of Treitz, at sites of prior anastomoses Other two anastamoses noted to be wide open and slightly dilated Extensive lysis of adhesions Stricturoplasty x 2 5 cm Rxn of terminal ileum ICV preserved End-ileostomy creation http://medicaldictionary.thefreedictionary.com/ Case #4 N.N. con’t: Readmit 8/20/08 con’t Hyperglycemia requiring sliding scale insulin, stopped upon discharge home HPA axis was evaluated per endocrine AM cortisol 29.7 (high, but patient on prednisone), DHEAS low at 28.4 (adrenals suppressed), ACTH 23 (normal) On 9/22/08, repeat AM cortisol was 42; high-dose Dexamethasone suppression test deferred Requires stress dose steroids for procedures per endocrine Discharged home on 9/23/08 on PN and Peptamen Jr. 1.5 @ 20 ml/hr x 12 hr nightly via GT, diet as tolerated by mouth; new medication of sliding scale insulin for BG > 250 Case #4 N.N. con’t: Readmit 11/8/08 OR on 11/8/08 Ileoscopy with the GI team that showed two strictures, consistent with pre-operative contrast study, and normal looking bowel proximally Ex-lap with tedious lysis of inflamed adhesions A large diverticulum was noted at one of the stricturoplasty sites just proximal to his ostomy, with the second stricture just proximal to the stricturoplasty at the presumed anastamosis Noted 60 cm abnormal bowel with flattening of normal folds; left in place with potential for absorptive capacity; mesentery very edematous Resection of 25 cm of strictured small bowel with new endileostomy creation At end of case, 118 cm of small bowel remaining Case #4 N.N. con’t: Readmit 8/20/08 con’t Planned Ex-lap surgery 8/20/08 con’t Stamm gastrostomy tube placement and needle liver biopsy 144 cm of small bowel from Ligament of Treitz to ileostomy at end of case Pressor requirement from POD #1-POD #2, given stress dose steroids Wound dehiscence, abdominal CT on 9/5/08 showed large midline abscess underlying surgical scar. Abscess drained by IR with drain placement, drain removed 9/9/08. Treated with Vancomycin and Zosyn x 7 days. Liver biopsy showed mild steatosis, no fibrosis Continued hemodialysis 3x/wk and IVIG infusions q 2 weeks Case #4 N.N. con’t: Readmit 9/27/08 Abdominal pain, vomiting and increased ostomy output CT with IV and PO contrast with no evidence of vascular compromise or obstruction, but did show persistent anterior abdominal abscesses and lesion concerning for a second stricture proximal to the ostomy site Contrast study with ileal stricture in the region of the ileostomy Treated with IV antibiotics for persistent abscess and discharged home on Moxifloxacin on 9/30/08 His nausea and abdominal pain persisted Seen in surgical clinic outpatient and the plan was made to return to the OR for full intraoperative enteroscopy to determine what portions of bowel were functional and possible further bowel resection Case #4 N.N. con’t: Readmit 11/8/08 con’t Restarted enteral feeds on POD #4 and advanced slowly to rate of 20ml/hr Post-op course c/b mild wound infection, treated effectively with antibiotics Discharged home on 11/28/08 on PN and enteral feeds of Peptamen 1.0 @ 20 ml/hr x 8 hr nightly via GT Referred to the CAIR program (BILIs slightly bumped to 0.8/0.5; abd u/s 11/7/08 w/enlarged echogenic liver, diffuse cortical thinning of the kidneys) CAIR managed his parenteral nutrition and enteral feeding regimen outpatient Per CAIR team started on pancreatic enzymes for malabsorption 21 2/14/2014 Case #4 N.N. con’t: Readmits 4/09 Progressive renal failure http://www.scielo.br/scielo.php ?pid=S180759322005000100008&script=s Left arm AV fistula graft on 4/9/09 ci_arttext Complicated by stenosis related to an old IV site Revision on 6/25/09 http://westcoastvascular.c om/DialysisAccessCenter.h Started aspirin to maintain patency tml Readmit 4/15-4/29/09 for fever CT scan with abdominal wall collection near ostomy, surgery consulted but did not feel it needed intervention Stomagram 4/21 showed no fistula, possible descending colon stenosis Discharged home on 6 weeks of IV antibiotics (original cultures not drawn from HD line) with plan for colonic manometry Case #4 N.N. con’t: Readmit 3/19/10 con’t Final pathology Resected terminal ileum and strictured splenic flexure segment c/w remote ischemia Multiple samples of mesentery and intestine without evidence of persistent vasculopathy Post-op period unremarkable except for excessive ostomy output, for which he was started on Loperamide Discharged home on combination enteral feeds and PN on 3/26/10, BILIs normalized 0.4/0.2 Case #4 N.N. con’t: Transplant Evaluation Transplant referral 7/29/10 from CAIR clinic Due to intestinal failure, dependence on parenteral nutrition, and complication risk related to immunosupression associated with isolated kidney transplant, combined small bowel and kidney transplant was advised by the transplant team LFTs remained stable outside of acute infections; thus liver transplant was not pursued N.N. with his family open to suggestion for transplant but wanted to continued to continue to try to purse increasing enteral feeds while weaning parenteral nutrition for the time being Case #4 N.N. con’t: Readmits 3/9/10 and 3/19/10 Readmit 3/09-3/12/10 for abdominal pain CT scan of the abdomen revealed an ileostomy stricture, a colonic stricture at the splenic flexure, and a possible small bowel to colon fistula Plan for OR with GI to scope and general surgery for Ex-lap OR 3/19/10 Colonoscopy with patchy area of moderately erythematous mucosa up to the splenic flexure with blind end there Extensive lysis of adhesions Blind loop obstruction of the colon caused by the long segment splenic flexure stricture splenic flexure stricture, resected No connection between the small and large bowel was found Ileum remnant scarred down so ileocecectomy was performed He then had a jejunostomy creation and right colon mucous fistula creation Case #4 N.N. con’t: 2010 Persistent abdominal pain Abdominal US on 5/6/10 showed no fluid collection or hernia, did note interval slight worsening in appearance of the echogenic kidney with parenchymal thinning and loss of corticomedullar differentiation Readmitted 5/13/10-5/14/10 to GI service with bloody ostomy output Coagulation panel normal, other labs stable, KUB unchanged Discharged home with plan for scopes by GI team 5/21/10 endoscopy unremarkable, ileoscopy with mild erythema Abdominal CT on 5/26/10 showed no bowel loop dilatation or air fluid levels seen to suggest obstruction, did note atrophic kidneys Case #4 N.N. con’t: 2011-2012 Maintained on full PN Worsening kidney disease, HD progressed to 6x/wk Metoprolol stopped by cardiology outpatient; started on Midodrine for hypotension Several central line infections, required CVL removal and replacement 1/11/11 and 1/14/11 Readmit 3/18/11- 4/13/11 for partial small bowel obstruction, ultimately requiring ileostomy revision in situ on 3/25/11 CT in ED on 3/18/11 showed small atrophic kidneys with progression of multiple hypoattenuating lesions 22 2/14/2014 Indications for Intestinal Transplant Intestinal failure Short bowel syndrome is the most common reason for intestinal transplantation in children Every effort is made to optimize enteral nutrition and decrease the need for parenteral nutrition in patients with SBS to promote bowel rehabilitation and because of the risk of liver damage Unfortunately, some patients are not able to progress on enteral feeds and become dependent on full PN with resulting PNALD necessitating liver transplant as well Congenital malformations, infections of the gastrointestinal tract, absorptive impairment There are three types of transplant for SBS Intestinal Transplant Isolated Small Intestine Intestinal Transplant Combined Liver/Intestine Multivisceral http://transplant.surgery.ucsf.edu Case #4 N.N. con’t: Decision to Evaluate & Transplant Listing UNOS (United Network for Organ Sharing) National transplant waiting list In order to: Confirm transplant is the best treatment Determine the urgency for transplant Assure a good organ match Decision to pursue transplant option in May of 2012 Listed for a combined kidney and intestine transplant on 6/21/12 UNOS: Listing Criteria UNOS http://www.uofmmedicalcenter.org Transplant Evaluation http://www.theliverfoundationforkids.org Small Bowel Transplant http://www.uofmmedicalcenter.org Labs/Imaging Blood type, HLA typing CBC w/diff, coags Chem 10, LFTs w/GGTP +/Titers CMV, EBV, HHV6, HSV I/II, VZV HIV, Hepatitis A, B, C, HLA Toxoplasmosis +/- Tb, MMR IgG, RPR, Ammonia CXR, KUB, Abd u/s w/Doppler GI Endoscopy (upper and lower) Upper GI series and barium enema Abdominal CT &/or CTA abd/pelvis +/- Motility studies +/- Liver biopsy Other (hypercoagulability workup w/hx mesenteric venous thrombosis) Consults Surgery, Tx Coordinator Hepatology/GI; Renal/Urology; Dialysis Nurse Anesthesia Infectious Disease Nutrition Transplant pharmacy Social work Child life specialist Psychiatry Physical therapy Dentist Financial coordinator +/- Other comorbidity dependent Difficult organ to transplant because of its immunologic properties Approximately 80% of immune cells normally reside in the gut After transplant the immune cells are repopulated with recipient cells However the genotype of the epithelium remains that of the donor, making the graft immunogenic and chimeric Gut barrier can breached by ischemia or reperfusion injury, recipient immune cells or impaired microbial control mechanism causing inflammation and tissue damage, increasing chance of infection http://tidsskriftet.no/article/2243910/en_GB 23 2/14/2014 Small Bowel Transplant con’t Kidney Transplant A multivariate analysis of cases within the last 5 years revealed that transplantation of patients waiting at home, recipient age, antibody induction immune suppression, and center experience with at least 10 cases were associated with improved patient survival (Grant etal., 2003) Most common etiology Congenital renal and urologic anomalies Renal allograft and patient survival rates have increased in the past few years with advancements in immunosuppressive therapy Living donor transplants have increased graft survival as compared to deceased donor renal transplants Important part of pre-transplant evaluation is detection of anti-Human Leukocyte Antigen (HLA) antibodies to the donor http://www.multivu.com/mnr/52073 http://emedicine.medscape.com/ http://emedicine.medscape.com/ Case #4 N.N. con’t: BIG DAY Called in for a deceased donor kidney and intestinal transplant on 1/13/13 Admitted to general surgery floor preop Medication consideration, fever/sick, NPO Possibility of Tx cancellation Donor CMV positive, recipient negative Donor EBV negative, recipient positive Transplant Preop Orderset: Kidney Specific: Deceased Donor Steroid Avoidance Protocol Campath in OR for deceased donor, night before for living donor Same dosing as for intestinal transplant, 0.3mg/kg IV <66kg or 20mg IV >66kg Premeds for Campath Methylprednisolone dosing based on BSA: <1.67m2 IV 300 mg/m2, >/= 1.67m2 IV 500 mg Tylenol and Benadryl Vaclganciclovir on POD -1, <15kg: PO 7.5 mg/kg, >/= 15kg: PO 250 mg/m2 Zofran prn N/V, Meperidine prn shivering http://www.webmd.com/ http://www.surgical-tutor.org.uk/ http://bobsnewheart.files.wordpress.com/ Transplant Preop Orderset: Intestine Specific Admit, +/- PIV, full labs, T&S, ABORh, blood on hold (PRBC, PLT, FFP), CXR, notify anesthesia/blood bank/radiology, GI fellow Repeat serologies only if negative in the past 2 months ABX on-call to OR Campath 0.3mg/kg IV <66kg or 20mg IV >66kg Premedications for Campath (GIVEN IN OR), frequent VS Tylenol and Benadryl 30-60 minutes prior Methylprednisolone 1mg/kg IV <50kg or 50mg IV >50kg 30-60 minutes prior Zofran prn N/V; Meperidine prn shivering Procurement of donor organ and enterectomy (+/- LOA) Cross-clamping (ischemia times!) Graft implantation: vascular then bowel anastomoses To ICU postop Case #4 N.N. con’t: BIG DAY con’t Extensive lysis of adhesions was performed first. Completion enterectomy (small segment at ligament of Treitz was left for anastomosis) Remaining colon was preserved (RUQ mucous fistula in place) Mobilization of infrarenal aorta and vena cava for anastomosis Small bowel graft was procured at the same time Donor vessels were used to create two small grafts coming off the infrarenal aorta and vena cava Patient was fully heparinized Intestinal graft was then brought up to the table 24 2/14/2014 Case #4 N.N. con’t: BIG DAY con’t: Small Intestine Tx Donor superior mesenteric artery (SMA) was anastomosed end-toside to the infrarenal aorta via an iliac artery donor graft Donor superior mesenteric vein (SMV) was anastomosed end-toside to the infrarenal vena cava via a donor innominate vein graft Arterial flow was restored to the graft Several bleeding points in the upper mesentery of the graft, which were controlled The entire graft appeared well perfused Donor proximal jejunum to the native jejunum end-to-end anastomosis Gastrojejunostomy tube passed well past this anastomosis while being secured at the anastomosis Case #4 N.N. con’t: BIG DAY con’t: Kidney Tx LLQ prepped and draped Incision was made in the left iliac fossa through the external oblique and internal oblique and into the retroperitoneum Due to multiple prior surgeries, the retroperitoneal space was slightly difficult to get into, and surgeons did make a small hole in the peritoneum Donor kidney was a left kidney with single artery, single vein, and single ureter Surgeons mobilized the external artery and vein of the recipient The donor renal vein anastomosed end-to-side to the recipient external iliac vein The donor renal artery was then anastomosed end-to-side to the recipient external iliac artery Transplant Postop Orderset: Intestine Specific To ICU postop GJT to gravity, +/- NGT to LWS, JP x2 to bulb, Foley to gravity, stoma to gravity +/- CVP monitor, SCDs >25kg, +/- Heparin gtt IVF +/- replacements, ABX periop Morphine/Tylenol prn, PPI ppx Frequent VS, labs q6 x3d, then q12 x2d, then qday Abd u/s w/Doppler & CXR postop, +/- UGI plan Immunossupression postop Infection prophylaxis Methylprednisolone taper • Periop ABX Prograf when able • Fungal (Nystatin) Cellcept • PCP (Atovaquone) Ganciclovir • CMV (Ganciclovir, Case #4 N.N. con’t: BIG DAY con’t: Small Intestine Tx con’t Distal graft ileum brought through the prior right lower quadrant ileostomy site Abdomen thoroughly irrigated with warm saline solution Final inspection for hemostasis Two Jackson-Pratt drains placed through right upper quadrant incisions (at right retroperitoneum and near the jejunal anastomosis on the left) Abdomen was then closed Case #4 N.N. con’t: BIG DAY con’t: Kidney Tx con’t Small blood flush of the kidney prior to releasing the renal vein clamp The renal vein anastomosis was secured and the kidney reperfused nicely Surgeons then filled the bladder via the Foley that had been placed previously Bladder was dissected with exposing a site on the lateral and anterior portion for the ureteral anastomosis The ureter was cut to the appropriate length and spatulated, creating a diamond-shaped opening Ureter then anastomosed to the bladder mucosa opening The muscle was then closed over creating a long submucosal tunnel to prevent reflux Transplant Postop Orderset: Kidney Specific Foley catheter x 5 days, JP x 1 Morphine prn, IV PPI IVF for insensible losses 1:1 replacements with ½ NS for UOP and JP OP until tolerating fluids PO Immunosuppression: Methylprednisolone until Prograf therapeutic then tapered, Cellcept, Prograf Infection ppx: Atovaquone (PCP), Ganciclovir then Valganciclovir (CMV) Frequent VS; labs post-op: q6hr x 48hr, q12hr x 48hr then daily US with Doppler in PACU, Mag 3 scan on POD 1 Valganciclovir) • +/- HSV, VZV (Acyclovir) 25 2/14/2014 Tacrolimus Mainstay drug for intestinal and kidney transplants Retrospective reviews (Fishbein etal., 2009; Grant etal., 2003) Have shown increased graft survival rate over time, especially with the use of Tacrolimus, which inhibits signal-1 activation of T lymphocytes through the inhibition of calcineurin One-year patient survival rates of 81% are being achieved using antibody-based pretreatment/induction therapy and tacrolimus-based maintenance immunosuppression This is comparable to survival rate for liver transplants Most common complication of Tacrolimus is renal damage (Grant etal., 2003) Transplant Complications Surgical complications Graft rejection Acute Chronic GVHD Infection PTLD Graft dysfunction h//www.medscape.com/viewarticle/436543_11 http://emedicine.medscape.com/article/1013915treatment#a17 Case #4 N.N. con’t: Post-Transplant POD #0-2 Initially hypotensive; on pressors through the eve of POD #1 Resumed parenteral nutrition on POD #1 Ostomy with green liquid output On IVF for insensible losses and 1:1 replacement of UOP and JP output Made good urine post-op with serial labs showing slow decline of BUN and creatinine Mag 3 scan on POD #2 showed fair uptake of kidney, no obstruction, or extravasation Continued Methylprednisone, Prograf and Cellcept started on the eve of POD #0 Ganciclovir started on POD #0 Transferred to floor on eve of POD #2 Left arm edema on POD #2; non-occlusive thrombus in left basilic vein; started on prophylactic lovenox per hematology team Case #4 N.N. con’t: Immediately Post-Transplant Tolerated transplantation procedure well Extubated at the end of the case Immediate post-op ultrasounds Renal Patent vessels with good flows and low resistive waveforms, no fluid collections Abdominal Limited 2/2 bandage Visualized portions of the abdominal aorta and IVC patent with good arterial and venous waveforms The infrarenal anastomoses could not be visualized No free fluid. Case #4 N.N. con’t: Post-Transplant POD #2-10 Ileal pluck biopsies performed frequently post transplant, all negative for rejection immediately post-op Weaned off steroids by POD #10 with therapeutic Prograf level He continued Cellcept and Ganciclovir per protocol Started on continuous Pedialyte via JT on POD #3, slowly advanced over the next day to goal volume and transitioned to formula After reaching goal feed volume via JT, he had acute increase in his Gtube output, thus his enteral feeds were suspended for several days UGI series on 1/22/13 showed patency of the graft with mild narrowing An abdominal ultrasound that same day without intra-abdominal fluid collections 26 2/14/2014 Case #4 N.N. con’t: 2-5 Weeks PostTransplant FEN/GI/Surgery Gtube output slowly decreased; enteral feeds were reinitiated Persistent nausea with increasing enteral feeds; abdominal US on 2/2/13 showed increasing fluid collection CT on 2/3/13 with large interloop abscess in right hemiabdomen adjacent to distal ileum and ileostomy. Blood supply to intestinal graft patent with no signs of poor perfused bowel. Pigtail catheter placed by IR on 2/3/13; a lot of pain with the tube and it was removed on 2/5 as the drainage was low and collection too thick to pass through the catheter Feeds advanced to goal thereafter His goal minimum fluid intake of 4.5 liters/day Of note, PN was discontinued on 2/17/13 Case #4 N.N. con’t: 2-5 Weeks PostTransplant con’t Transplant Pluck biopsy on 2/4/13 with moderate rejection; treated with IV pulse steroids 10mg/kg for 3 days, then slowly tapered over next two weeks Repeat pluck biopsy on 2/20/13 with no evidence of rejection Transitioned from IV to oral steroids on 2/20/13 with plan for taper Continued on cellcept and prograf Infectious Disease Transitioned to valganciclovir and started on Mepron for prophylaxis On discharge CMV and EBV PCR were negative Case #4 N.N. con’t: Readmission 3/43/26/13 (~2m post tx) Concern for infected bilateral middle finger infection (blisters) WBC 3, ANC 3.22 Area unroofed and dressed by plastic surgery I&D on 3/4 and 3/6; dressing changes; wicks removed on 3/7 Wound cultures with abundant staph aureus HSV, VZV DFA, culture and PCR all negative Initially Vancomycin and Zosyn then switched to Cefazolin and IV Acyclovir per ID recs Transitioned to oral Keflex and then switched to Bactrim; stopped on 3/18/13 per ID recs Case #4 N.N. con’t: 2-5 Weeks PostTransplant con’t Renal Adequate urine output Serum BUN and creatinine trending down (43/1.2) Required Amlodipine daily for hypertension/nephroprotection Endocrine Developed hyperglycemia in the post-operative period Requiring insulin (Lantus and Humalog) Continued on Synthroid Hematology Received 1 week of Lovenox for LUE clot, stopped per hematology service recommendations Required PRBC transfusion during the stay Case #4 N.N. con’t: Discharge PostTransplant Discharged home on 2/22/13, POD #40 Allowed regular diet by mouth with feeds of Peptamen 1.0 at 50cc/hr x 10 hrs plus free water Discharge meds: Prograf, Cellcept, Prednisone, Amlodipine, Protonix, Baking Soda, Loperamide, Magnesium Oxide, ADEK, Humalog sliding scale and carb coverage, Lantus, Levothyroxine, Nystatin, Atovaquone, Valganciclovir Enteral feeds weaned to off outpatient secondary to his advancement of diet and ability to meet goal kcal and fluid requirements all by mouth Case #4 N.N. con’t: Readmission 3/43/26/13 (~2m post tx) con’t Course complicated by secretory diarrhea, stool culture +Norovirus Received extra fluid of ~ 5 liters daily to compensate He started on enteral IgG for norovirus on 3/13 for a 3 day course Intestinal biopsies on 3/8, 3/13, and 3/20 were negative for rejection; continued on steroid taper Bedside scope on 3/21 appeared normal on exam but biopsies showed focal mild rejection in the setting of norovirus Course also complicated by fall on left knee, which became swollen with a mobile knee cap and an effusion on XR. Ortho was consulted and recommended a patellar brace and outpatient follow up. 27 2/14/2014 Case #4 N.N. con’t: Readmission 4/12-4/14/13 (~ 3m post tx) Admit for fever and sepsis rule-out Also note recent history of outpatient decrease in immunosuppression secondary to +BK virus Low WBC 4 and electrolytes with mild dehydration Blood cultures ultimately negative and empiric antibiotics stopped Bedside pluck biopsy of his ostomy showed > 6 apoptotic bodies but no inflammation CMV and EBV titers negative, repeat BK PCR pending on discharge Prograf dosage increased on discharge Note weaned off insulin upon admission, none required in-house Case #4 N.N. con’t: Readmission 4/22-5/21/13 (~ 3-4m post tx) con’t Incisional hernia and abscess formation at his midline abdominal incision; I&D with packing done Culture with yeast and staph non-aureus; started on Fluconazole and Keflex. Pluck biopsy on 5/20 showed continued improvement with areas of ulceration mixed with healthy regenerating mucosa Final ileoscopy on 5/21 with no areas of ulceration but 1 area with white fibrinous exudate; overall marked improvement BK virus decreased to 365,615 High blood sugars in-house requiring restarting of sliding scale insulin Discharged home on new immunosuppression of Leflunomide, Keflex and Fluconazole to stop on 5/31/13, and sliding scale Humalog prn Case #4 N.N. con’t: Readmission 4/22-5/21/13 (~ 3-4m post tx) Admit for fever and friability of stoma Intestinal biopsy positive for severe rejection Started on pulse steroids, Zosyn and Valcyte http://emedicine.medscape.com/arti cle/1013915-treatment#a17 Renal biopsy consistent with mild rejection and evidence of BK virus (1.2 million on 5/14); started on Leflunomide; Cellcept dose was decreased by half and discontinued on 5/17 Serial intestinal biopsies next few days showing gradual improvement of rejection on steroid treatment; thus the Zosyn and Valcyte were stopped, he was restarted on oral Valgancyclovir Scope on 5/16 showed healing ulcers and areas without rejection; steroids transitioned to oral Case #4 N.N. con’t: Readmission 6/36/4/13 (~5m post tx) Increased ostomy output Biopsy showed 8-10 apoptotic bodies with concerns for rejection Bedside ileoscopy showed resolving ulcerations, and appeared similar in appearance to his last ileoscopy on 5/21/13 BK virus was resulted at 37, 786 Remained stable in-house, decision made to discharge home and follow-up on biopsies outpatient Biopsies did show ongoing rejection, for which he continued enteral steroids and increased dose of Leflunomide Repeat scope 6/24/13 showed active CMV, and was treated with BID Valcyte 900mg http://emedicine.medscape.com/ Case #4 N.N. con’t: Readmission 7/99/7/13 (~6-8m post tx) Low grade fever, decreased hunger and film over ostomy; admit sepsis rule out Ileoscopy with a couple of areas of ulceration, biopsies were sent Labs with WBC low at 0.6 with ANC 440, stable liver function tests and creatinine High dose Methylprednisone and empiric antibiotics started; ultimately cultures negative so antibiotics stopped Repeat scope on 7/15 with continued rejection so pulse steroids continued Hospital course c/b RLE then LUE swelling and pain; u/s negative Midline abdominal incision w/purulent drainage; culture positive for candida (started on Fluconazole); wound con’t open; VAC applied and ultimately discontinued on 8/1; persistent fluid collection on u/s 8/3; bedside I&D (fascia intact) Case #4 N.N. con’t: Readmission 7/99/7/13 (~6-8m post tx) con’t Ileoscopies on HD #21, 27, and 30 (7/31, 8/6 and 8/9) with signs of rejection found on 8/6; EBV and CMV PCR were negative CMV stains of the ileal biopsies were positive and he was started on IV ganciclovir Per ID team, persistent CMV disease indicating resistance to Ganciclovir; started on Cytogam and on renally dosed Foscarnet Adenovirus PCR also returned positive (73k copies) Abdominal midline wound required VAC reapplication on 8/9 Fluconazole was changed to Micafungin per ID recs Received GCSF therapy for neutropenia until his ANC normalized 28 2/14/2014 Case #4 N.N. con’t: Readmission 7/99/7/13 (~6-8m post tx) con’t VAC changes in OR until VAC discontinued on 8/16 and wet to dry dressings instituted RLE swelling and pain persisted, repeated US negative for DVT MRI on 8/11 showed extensive subcutaneous/intramuscular edema, bilateral lower extremity bone infarcts, and bilateral knee effusions Orthopedics team was consulted, and saw no fluid collection amenable to tapping He continued on Micafungin for abdominal wound, Vancomycin and Meropenem for lower extremity cellulitis, and Foscarnat and Cytogam for CMV. Valganciclovir was stopped. Case #4 N.N. con’t: Readmission 10/18-12/30/13 (~ 9-11m post tx) Increasing abdominal pain Abdominal US showed multiple fluid collections in the SQ tissue in the anterior abdominal wall at the level of the wound packing Started on Zosyn HD #1: Aspiration of one collection in IR with culture ultimately yielding Candida Albicans Ileoscopy and biopsies on HD #1 unremarkable and biopsies with no rejection I&D of midline abdominal wound abscess on 10/21; VAC applied Culture grew out candida (sent to an outside laboratory for further sensitivity testing; sensitive to Micafungin) Over the next couple of weeks, multiple OR wound washouts and VAC changes. Case #4 N.N. con’t: Readmission 10/1812/30/13 (~ 9-11m post tx) con’t Finally, VAC changes were done at bedside with premedication. Hospital course c/b hypertension, for which his Amlodipine was increased and he was started on hydrochlorothiazide Serum labs showed stable BUN and creatinine GT site continued to leak; definitive GT closure on 12/10 with an endoscopy and VAC dressing change Overall improvement with ongoing ulceration of the stomach; ileostomy biopsies showed: CMV in the gastric antrum only VAC placed at old gastrostomy site Abdominal site additional purulent fluid; culture positive for candida albicans; Micafungin was restarted Case #4 N.N. con’t: Readmission 7/99/7/13 (~6-8m post tx) con’t Bedside scope on 8/13 and 8/19 showed apoptosis and CMV but overall improvement; steroids began to wean slowly Viral studies on 8/19 with negative DFA and serum CMV, inconclusive stool CMV, BK virus 1.5 million copies, Adenovirus 76K in the blood. Viral studies on 8/27 showed: CMV and adeno PCRs negative, BK virus 739,097 copies; viral shell for CMV and wound fungal culture negative Vancomycin and Micafungin stopped on 8/30 Meropenem stopped on 9/3 with resolution in RLE swelling Bedside scope on 9/3 grossly improved with the ulcer @ 15cm appearing much smaller. Biopsies were negative for rejection, showed an improvement in the inflammation, and CMV; adenovirus was negative Case #4 N.N. con’t: Readmission 10/1812/30/13 (~ 9-11m post tx) con’t Wound culture from 11/7 returned negative for candida and micafungin was stopped Later wound culture showed GNR; started on Zosyn on 11/13 Serum PCR positive for BK virus, CMV, and EBV; CMV also positive from stains on intestinal biopsies, including his native stomach; thus, he was started on foscarnet Zosyn course x 2 weeks and samples were sent for CMV genotype testing per ID recs (resistance to Foscarnet; stopped on 11/21) Enrolled in Maribavir study at BWH to treat his CMV per ID team Repeat CMV PCR on 11/29 and 12/5 negative; BK virus positive on 12/4 at 97, 000 Repeat scope 11/18 with persistent non-bleeding ulcers in gastric antrum, but overall mucosa looked better Case #4 N.N. con’t: 12/30/13 Discharge and Follow-up Outpatient After Finally discharged home with double wound VAC in place Discharge meds: Leflunomide, Prednisone, Maribavir, Valganciclovir, Atovaquone, Valtrex, Nystatin, Micafungin, Amlodipine, Hydrochlorothiazide, Pantoprazole, Loperamide, Levothyroxine, Humalog sliding scale, Lantus, Vitamins, Potassium Phosphate, Magnesium Oxide, and Zinc Sulfate VAC changes outpatient once weekly coordinated with his Maribavir appointment He will continue on Maribavir as prophylaxis per ID team at BCH Seen in clinic 1/13/14 and prednisone decreased Clinic 1/21/14 with no significant changes 29 2/14/2014 References Thank You! www.istockphoto.com Have fun in Boston! 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