Oesophageal atresia with very low birth weight: Clinical characteristics and long-term outcome.

AIM OF THE STUDY: An assessment of the clinical data and outcome of patients with oesophageal atresia (OA) with very low birth weight (VLBW) was completed. METHOD: With ethical consent, we reviewed the records of 327 successive patients with OA from 1980 to 2020. Main outcome measures survival and oesophageal repair were compared between patients with VLBW(≤1500 g) and with BW>1500 g. RESULTS: Thirty-four (10%) patients had VLBW. Gross types of OA in VLBW were similar as in other patients: A (15%/7%), B (3%/3%), C (78%/82%), D (3%/4%), E (0%/7%), F (0%/1%) (p = 0.16-0.99). In VLBW the incidence of congenital heart disease (CHD) (47%) and trisomy 13/18 and Cri du Chat (15%) were higher than in BW>1500 g (23% and 1%), (p = 0.001 both). In VLBW one-month mortality was 35% vs 4% in patients with BW>1500 g (p < 0.001), overall mortality 56% and 8% (p < 0.001), respectively. Cause of one-month mortality in VLBW (12 patients) were CHD w/wo chromosomal abnormality (n = 7), cerebral hemorrhage (n = 2), gastric perforation (n = 1), anastomotic leakage (n = 1) and pulmonary hemorrhage (n = 1). Of VLBW patients 79% and of other patients 99% underwent oesophageal repair (p < 0.001). Repair in VLBW patients included early (n = 18) or delayed (n = 5) end-to-end anastomosis and reconstruction (n = 4). Anastomotic complications occurred in 24% of patients with VLBW and in 17% with BW>1500 g, (p = 0.31). From 1980-2000 to 2001-2020 survival in VLBW changed from 11% to 81% (p = 0.002). During 2001 - 2020 all VLBW patients underwent repair. CONCLUSION: OA with VLBW had high incidence of life-threatening associated anomalies and decreased survival. Recently survival and rate of oesophageal repair have improved significantly.

Long-term Outcomes and Health Perceptions in Pediatric-onset Portal Hypertension Complicated by Varices.

OBJECTIVES: Outcomes of pediatric-onset portal hypertension are poorly defined. We aimed to assess population-based long-term outcomes of pediatric-onset portal hypertension complicated by varices. METHODS: All children with esophageal varices (n = 126) were identified from 14,144 single nationwide referral center endoscopy reports during 1987 to 2013, and followed up through national health care and death registers. A questionnaire was sent to survivors (n = 94) of whom 65 (69%) responded. RESULTS: Nineteen underlying disorders included biliary atresia (35%), extrahepatic portal vein obstruction (35%), autosomal recessive polycystic kidney disease (7%), and other disorders (23%). During median follow-up of 15.2 (range 0.5-43.1) years patients underwent median 9 (1-74) upper gastrointestinal endoscopies. Esophageal varices were first observed at a median age of 4.0 (0.3-18.2) years, 112 (89%) patients underwent median 6 (1-56) sclerotherapy/banding sessions, and 61 (48%) experienced median 2 (range 1-20) variceal bleeding episodes. Forty-eight surgical shunt procedures were performed to 41 (36%) patients and 38% underwent liver transplantation. Portal hypertensive biliopathy was diagnosed in 4 patients. Hepatopulmonary syndrome necessitated liver transplantation in 2 patients, hepatic encephalopathy in 2, and hepatorenal syndrome in 1. No patient died of variceal bleeding. Patient-reported perception of health on a scale of 1 to 10 was 9 (range 4-10), and 86% reported no current symptoms attributable to esophageal varices. CONCLUSIONS: Pediatric-onset portal hypertension is a heterogeneous disease with significant long-term morbidity, requiring multimodal approach with considerable resources and continuation of follow-up in adulthood. Although mortality to variceal bleeding was avoided, bleeding episodes recurred also in adulthood, while patient-reported health of long-term survivors was encouraging.

Combined management of perianal rhabdomyosarcoma with chemotherapy, radical surgery, and irradiation: A series of three consecutive children.

BACKGROUND AND AIM: We describe a series of three successive patients with perianal rhabdomyosarcoma (PRMS) from 2014 to 2017 managed with combined chemotherapy, radical surgery, and radiotherapy. METHODS: Ethical consent was obtained. Data including tumor presentation, treatment, and survival was collected from hospital reports. RESULTS: Two girls aged 15 and 16 years (patient #1 and #2) and one boy aged five years (patient #3) were referred because of a suspected perianal abscess. MRI showed large perianal tumors from 7 to 12 cm in diameter that surrounded or infiltrated the anal sphincters and were inconsistent with abscess. Tumor biopsies showed RMS of alveolar (#1 and #2) and embryonal (#3) types. Patient #1 had lymph node and bone metastases, patient #2 lymph node metastases, and patient #3 no metastases. Pretreatment staging, IRS Clinical Group, and Risk Groups were: Stage 4, II, high; Stage 3, GII, intermediate; and Stage 3, I, low, respectively. All underwent colostomy before neoadjuvant chemotherapy (CWS-RMS 2009 program). Neoadjuvant chemotherapy failed to clear the tumors from anal sphincters preventing anus-saving surgery, and all patients underwent abdominoperineal excision. All removed specimens had free margins with negative lymph nodes. After adjuvant chemotherapy and local radiation, the patients were tumor free after 48, 13, and 18 months. CONCLUSION: In PRMS local surgical control required abdominoperineal excision. Confusion between PRMS and abscess may cause unnecessary delay in management. LEVEL OF EVIDENCE: IV (Treatment Study, Case Series with no Comparison Group).

Outcomes of fundoplication in oesophageal atresia associated gastrooesophageal reflux disease.

AIM OF THE STUDY: Conservative management of gastrooesophageal reflux (GORD) in oesophageal atresia (OA) is sometimes inefficient, and fundoplication is required. We assessed the outcomes of fundoplication among OA patients from 1980 to 2016. METHODS: After ethical consent, hospital records of 290 patients, including 22 referred patients, were reviewed. Included were 262 patients with end-to-end repair. Excluded were patients who underwent oesophageal reconstruction (n=23) or no repair (n=5). Primary outcome measures included survival, retaining the native oesophagus, resolution of GGORD symptoms, failure of fundoplication, and long-term endoscopic results. MAIN RESULTS: Gross types of OA in 262 patients were A (n=12), B (n=2), C (n=217), D (n=10), E (n=19), and F (n=2). Eighty-six (33%) patients, type A (n=12, 100%), B (n=2, 100%), C (n=69, 31%), D (n=3, 30%), and F (n=1, 50%), underwent fundoplication at the median age of 5.4 (IQR 3.1-16) months. Main indications included recalcitrant anastomotic stenosis (RAS) in 41 (48%), respiratory symptoms in 16 (19%), and acute life threatening events (ALTE) in 15 (17%) of patients. Associated tracheomalacia in 25 (29%) patients were treated with aortopexy. Median follow-up was 7.5 (IQR 1.8-15) years. RAS resolved in 30 (73%) patients, whereas 11 (27%) with unresolved RAS underwent oesophageal resection (n=8) or replacement (n=3). Six (7%) patients died of heart failure (n=4), bolus impaction (n=1), and ALTE (n=1). Fundoplication failed in 27 (31%) patients, and 13 (15%) underwent redo fundoplication. Fundoplication failure was predicted by long-gap OA RR=3.8 (95%CI=1.1-13), P=0.04. In total GORD associated symptoms persisted in 7 (8%) patients, including one with permanent feeding jejunostomy. Latest endoscopy showed moderate or severe oesophagitis in 7% of fundoplicated and in 3% nonfundoplicated patients and intestinal metaplasia in 3% and 1% (p=0.20-0.29). CONCLUSION: Fundoplication provided a safe and relatively effective control of OA associated symptomatic GORD and oesophagitis. The failure rate of fundoplication was high in those with long-gap OA. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: IV.

Long-term outcomes of oesophageal atresia without or with proximal tracheooesophageal fistula - Gross types A and B.

PURPOSE: Because of an extended gap between esophageal pouches a variety of methods are employed to treat oesophageal atresia (OA) without (type A) or with (type B) proximal tracheooesophageal fistula. This retrospective observational study describes their single centre long-term outcomes from 1947 to 2014. METHODS: Of 693 patients treated for OA 68 (9.7%) had type A (n=58, 8.3%) or B (n=10, 1.4%). Hospital records were reviewed. Main outcome measures were survival and oral intake. RESULTS: Nine (13%) patients had early and 10 (15%) delayed primary anastomosis, 30 (44%) underwent reconstruction including colonic interposition (n=13), reversed gastric tube (n=11) and jejunum interposition (n=6), whereas19 (28%) had died without a definite repair. Median follow up was 35 (interquartile range, 7.4-40) years. Thirty-one (63%) of 49 patients with definitive repair survived long term. Survival was 22% for early and 80% for delayed primary anastomosis, 57% for colon interposition, 82% for gastric tube and 84% for jejunum interposition. Gastrooesophageal reflux was most common after gastric tube (80%), dysphagia after colon interposition (50%), and 3 (60%) of 5 survivors with jejunum interposition had permanent feeding ostomy because of neurological disorder. Endoscopic follow-up disclosed no oesophageal cancer or dysplasia. Repair in the most recent patients from 1985 to 2014 (n=14) included delayed primary anastomosis (n=7), jejunum interposition (n=6) and gastric tube (n=1) with 93% long-term survival. CONCLUSION: Morbidity among long-term survivors of type A or B OA is high. With modern management survival is, however, excellent and patients without neurological disorder achieve full oral intake either after primary anastomosis or reconstruction. LEVELS OF EVIDENCE: IV.

Duodenal Disaccharidase Activities During and After Weaning off Parenteral Nutrition in Pediatric Intestinal Failure.

OBJECTIVES: Data on factors affecting absorptive function in children with intestinal failure (IF) are sparse. We evaluated duodenal disaccharidase activities and inflammation in relation to parenteral nutrition (PN) and intestinal resection in pediatric onset IF. METHODS: Disaccharidase (maltase, sucrase, and lactase) activities and histologic inflammation were evaluated from duodenal biopsies in 58 patients during PN (n = 23) or full enteral nutrition (n = 40) and in 43 matched controls. The first and the last postresection biopsies were analyzed separately after 4.3 (1.2-9.7) years and 6.5 (2.3-12.4) years, respectively. RESULTS: During PN, maltase and sucrase activities were 1.6-fold lower and mucosal inflammation more frequent (22% vs 3%) when compared to matched controls (P < 0.05 for both). In patients on full enteral nutrition, activities of maltase and sucrase were significantly higher than that in patients receiving PN and comparable to those of matched controls. Postresection time correlated positively (r = 0.448 and r = 0.369) and percentage length of the remaining small intestine inversely (r = -0.337 and r = -0.407) with maltase and sucrase activity in patients on full enteral nutrition (P < 0.05 for all), whereas proportional length of remaining colon correlated positively with maltase and lactase activity (r = 0.424-0.544, P < 0.05) in patients receiving PN. CONCLUSIONS: In children with IF, PN dependency associated with decreased duodenal maltase and sucrase activities and mucosal inflammation, which may disturb intestinal absorptive function. Localization and extent of intestinal resection and post-resection time correlated with duodenal disaccharidase activities.

Endoscopic Surveillance After Repair of Oesophageal Atresia: Longitudinal Study in 209 Patients.

AIM: After repair of oesophageal atresia (OA), the need for endoscopic follow-up (EFU) remains unclear. To end this, we assessed the trends of oesophageal mucosal changes in successive follow-up biopsies. METHODS: EFU records of 264 patients including histological grades of oesophagitis (from 0 to III), gastric (GM) or intestinal (IM) metaplasia and dysplasia (mild to severe) at 1, 3, 5 10, 15, and >15 years after repair of OA were reviewed. RESULTS: Included were 209 patients with 616 biopsies. A total of 60 patients had undergone antireflux surgery and 24 had long-gap OA (LG). Median follow-up was 12 (range 1-17) years with 3 (1-6) endoscopies per patient. Highest grade of oesophagitis was Gr 0 (no oesophagitis) in 47%, Gr I in 37%, and Gr II or III in 16%. Metaplasia, GM (n = 31), IM (n = 4), occurred in 17% of patients and reached 15% prevalence by 15 years. Dysplasia and cancer were not found. From 1 to 15 years after repair grade of histological oesophagitis often fluctuated between Gr 0 and Gr I, but further progression was unlikely, hazard ratio = 0.2-3.4 (95% confidence interval 0.0-29), P = 0.06-0.87. LG and antireflux surgery predicted early detection of metaplasia (P < 0.001). Only 9% of patients with metaplasia and 32% with Gr II oesophagitis were symptomatic. A total of 6 (3%) patients had a symptomatic anastomotic stenosis at 1 year. CONCLUSIONS: EFU revealed frequent oesophagitis and metaplasia, but no dysplasia or cancer. Routine endoscopic surveillance had limited benefit and seems unnecessary during childhood after repair of OA.

A population-based, complete follow-up of 146 consecutive patients after transanal mucosectomy for Hirschsprung disease.

OBJECTIVE: The objective of the study is to define the population-based bowel functional outcomes and enterocolitis following transanal endorectal pull-through (TEPT) in patients with Hirschsprung disease (HD) treated at our institution between 1986 and 2011. METHODS: 146 consecutive patients who had undergone primary surgical treatment for HD were included. The median follow-up time was 15 (3-33) years. The clinical details and prevalence of enterocolitis were evaluated in all patients, and bowel function in patients >3 years of age with functional intestino-anal continuity. RESULTS: No patients were lost to follow-up. Overall survival was 98%. The level of disease was rectosigmoid in 83%, long segment in 7%, total colonic in 4%, and extending up to the small bowel in 6%. 29% had an associated syndrome. 22% had a preoperative stoma. Operations included TEPT (89%), proctocolectomy with ileoanal anastomosis in 9%, and 3% had a permanent endostomy owing to small intestinal aganglionosis. One patient underwent intestinal transplantation. At the latest follow-up, 42% had occasional soiling, 12% had frequent soiling and 46% had no soiling. Constipation occurred in 9%. An associated syndrome was the only predictor for soiling or constipation (OR 4.3, 95% CI 1.5-12). 44% developed recurrent postoperative enterocolitis, which was predicted by extended aganglionosis (OR 6.9, 95% CI 2.4-20) and syndromatic disease (OR 2.4, 95% CI 1.2-5.0). CONCLUSION: The major functional sequelae following TEPT were recurrent enterocolitis and fecal soiling, which was mostly occasional. An associated syndrome was a predictor of a reduced bowel functional outcome, and alongside extended aganglionosis were significant risk factors for recurrent postoperative enterocolitis.

Revisional surgery for recurrent tracheoesophageal fistula and anastomotic complications after repair of esophageal atresia in 258 infants.

AIM: We assessed the occurrence and outcome of major reoperations following repair of esophageal atresia with or without tracheoesophageal fistula (TOF). Major outcome measures were survival, preservation of native esophagus, and long-term esophageal function. METHODS: Hospital charts of 258 consecutive patients treated for esophageal atresia from 1980 to 2013 were reviewed. RESULTS: Forty-two (16%) patients required a total of 57 reoperations after primary repair (n=37) or esophageal reconstruction (n=5). The indications were anastomotic leakage (n=17), anastomotic rupture after endoscopic dilatation (n=5), recurrent tracheoesophageal fistula (TOF) (n=12), undiagnosed proximal TOF (n=3), recalcitrant anastomotic stricture (n=11, primary anastomosis 9, reconstruction 2), undetected proximal fistula (n=3), and inadvertently perforated jejunal graft (n=1). Anastomotic leakage and rupture after dilatation were treated with rethoracotomy and suture and recurrent or undetected TOF by open repair. Strictures not manageable with repeated dilatations were resected and esophageal ends reanastomosed (n=10) or bridged with jejunum graft (n=1). Five (12%) patients required further reoperations, two after recurrent TEF (reocclusion n=1, reconstruction with gastric tube n=1), two after stricture operations (re-resection n=1, resuture after leakage n=1), and one after recurrent dilatation-related rupture. Mortality was 4/42 (10%). Two patients died of recurred leakage or TOF and two of unrelated cause. Of 38 survivors, 35 retained their native or initially reconstructed esophagus, and 3 had secondary reconstruction. After a median follow-up of 23 (range 0.6-32) years, 35 (95%) patients have acceptable esophageal function. Three patients remained dependent on gastrostomy feedings. CONCLUSION: Anastomotic and TOF complications required a substantial number of reoperations, including esophageal reconstructions. Over 90% of the patients survived with a functioning native or reconstructed esophagus.

Rectopexy for paediatric rectal prolapse: good outcomes but not without postoperative problems.

PURPOSE: Rectopexy is a simple treatment of persisting complete rectal prolapse (RP) or related functional disorders in children. The results of rectopexy have been encouraging with few complications. We describe the postoperative complications and outcome of rectopexy in our institution from 2002 to 2013. METHODS: Ethical committee accepted the study. Hospital records of 27 successive patients (16 males), median age 7.2 (range 2.8-17) years, who underwent rectopexy (25 laparoscopic, 2 open) were reviewed. Indication for rectopexy included RP (n = 24), solitary rectal ulcer with enterocele (n = 2) and rectocele (n = 1). Nine patients (39 %) were healthy. In the remaining 14 patients, RP was secondary to anorectal malformation (n = 2), bladder exstrophy (n = 1), sacrococcygeal teratoma (n = 1) and myelomeningocele (n = 1) or associated with mental retardation (n = 8) and Asperger's syndrome (n = 1). Five (18 %) patients had constipation. Unexpected postoperative events and complications were rated by Clavien-Dindo classification (Grades I-V). RESULTS: Seventeen (61 %) patients had postoperative complications (Grade I n = 5, II n = 2 and III n = 7). Readmission was required in 11 (41 %) and reoperation, endoscopy or other surgical procedure in 9 (33 %) patients. Complications included severe faecal obstruction (n = 2), constipation (n = 3), faecal soiling (n = 1) urinary retention (n = 2), enuresis (n = 1), infection (n = 2), residual mucosal prolapse (n = 5), discomfort at defecation (n = 1) and recurrent RP (n = 2). Reoperations included sigmoid resection with re-rectopexy (n = 1), resection of mucosal prolapse (n = 1), suprapubic urinary catheter (n = 2), evacuation of faecal impaction (n = 2), colonoscopy (n = 3), appendicostomy for antegrade continence enema (n = 1). Mental retardation or behavioural disorder increased the risk of postoperative faecal obstruction and constipation RR = 84 (95 % CI 4.3-1600), p = 0.0035. After median follow-up of 4.1 (range 0.6-11) years RP or related condition was cured in 26 patients. Constipation and faecal soiling require management in a total of seven patients. CONCLUSIONS: Long-term results of rectopexy were good. Postoperative complications from mild to moderate grade were unexpectedly frequent. Preoperative neurobehavioural disorder and constipation increase the risk of postoperative problems and should be mentioned in patient counselling.

Persistent abnormal liver fibrosis after weaning off parenteral nutrition in pediatric intestinal failure.

UNLABELLED: The aim of this study was to evaluate the long-term effects of pediatric intestinal failure (IF) on liver histology. Altogether, 38 IF patients (median age: 7.2 years; range, 0.2-27) underwent liver biopsy, gastroscopy, abdominal ultrasound, and laboratory tests. Sixteen patients were on parenteral nutrition (PN) after 74 PN months (range, 2.5-204). Twenty-two had weaned off PN 8.8 years (range, 0.3-27) earlier, after 35 PN months (range, 0.7-250). Fifteen transplant donor livers served as controls. Abnormal liver histology was found in 94% of patients on PN and 77% of patients weaned off PN (P = 0.370). During PN, liver histology weighted with cholestasis (38% of patients on PN versus 0% of patients weaned off PN; P = 0.003) and portal inflammation (38% versus 9%; P = 0.050) were found. Fibrosis (88% versus 64%; P = 0.143; Metavir stage: 1.6 [range, 0-4] versus 1.1 [range, 0-2]; P = 0.089) and steatosis (50% versus 45%; P = 1.000) were equally common during and after weaning off PN. Plasma alanine aminotransferase (78 U/L [range, 19-204] versus 34 [range, 9-129]; P = 0.009) and conjugated bilirubin (43 µmol/L [range, 1-215] versus 4 [range, 1-23]; P = 0.037) were significantly higher during than after weaning off PN. Esophageal varices were encountered in 1 patient after weaning off PN. Metavir stage was associated with small bowel length (r = -0.486; P = 0.002) and number of septic episodes (r = 0.480; P = 0.002). In a multivariate analysis, age-adjusted small bowel length (ß = -0.533; P = 0.001), portal inflammation (ß = 0.291; P = 0.030), and absence of an ileocecal valve (ß = 0.267; P = 0.048) were predictive for fibrosis stage. CONCLUSION: Despite resolution of cholestasis and portal inflammation, significant liver fibrosis and steatosis persist after weaning off PN. Extensive small intestinal resection was the major predictor for liver fibrosis stage.

Modern outcomes of oesophageal atresia: single centre experience over the last twenty years.

AIM OF THE STUDY: The aim of this study was assessment of the contemporary outcomes of oesophageal atresia (OA) in a national paediatric surgical centre. PATIENTS AND METHODS: A review of the hospital records of all patients who underwent repair of oesophageal atresia (OA) in our institute between 1991 and 2011 was performed. RESULTS: The study included 130 consecutive infants with OA: types A (n=4, 3%), B (n=2, 2%), C (n=110, 85%), D (n=5, 4%), and E (n=9, 7%). Median follow-up was 8.8 (range 0.1-21) years. Twenty-nine (22%) infants had cardiac and 76 (58%) other anomalies, and seventeen (13%) had a long-gap OA. The final repair was primary (n =113, 87%) or delayed (n=3) end-to-end anastomosis, oesophageal replacement (n=8) (6%) with gastric tube (n=4) or with jejunum interposition (n=4), and closure of the trachea-oesophageal fistula (Type E, n=9). Oesophageal continuity was achieved in all patients. Overall mortality was 3/130 (2%) and caused by gastric perforation (n=1), prolonged apnoeic spell (n =1), and food asphyxiation (n=1). Oral feeds were achieved in 121 (94%) children. Eight (6%) children remain dependent on feeding ostomy. Long-gap OA was a major predictor of post-repair complications. CONCLUSION: The modern outcome for infants with OA is characterized by an extremely low hospital mortality and satisfactory oesophageal function, enabling full oral feeds in the vast majority of children.

Surgical treatment and outcomes of severe pediatric intestinal motility disorders requiring parenteral nutrition.

AIM: The aim of this study was to characterize outcomes of children with severe intestinal motility disorders (IMD) requiring parenteral nutrition (PN). METHODS: Twenty consecutive children with primary IMD requiring long-term PN between 1984 and 2010 were included. Median (interquartile range) follow-up was 13.1 (5.2-20.1) years. Treatment, PN dependence, growth, nutritional status, liver function, and survival were assessed. RESULTS: Underlying etiology included chronic intestinal pseudo obstruction (CIPO; n=8) and Hirschsprung disease with extensive aganglionosis (n=12). CIPO and aganglionosis patients had 100 (86-100%) and 29 (19-40%) of age-adjusted small bowel length remaining, respectively. In order to facilitate enteral tolerance and avoid PN-associated liver disease, short aganglionic segment (40 cm) was left in situ in four of five cases, with aganglionosis extending to duodenojejunal flexure combined with Ziegler myectomy-myotomy in two. Six of seven children with aganglionosis extending into mid small intestine underwent staged jejunoanal pull-through. Feeding/venting gastrostomies (n=13) or jejunostomies were commonly employed. Probability of PN dependence owing to IMD was markedly increased in relation to short bowel syndrome (70 versus 19% at 5 years, P<0.0001). Two (10%) patients developed end-stage liver disease. A total of 11 (55%) patients (5 CIPO and 6 aganglionosis) weaned off PN after 8.2 years (1.8-17 years), including two patients after intestinal transplantation (ITx). Two children died before ITx-era giving overall survival of 90%. Survivors had well-preserved liver function, growth, and nutritional status. CONCLUSIONS: Despite high PN dependence, long-term survival is achievable in the majority of children with IMD requiring PN. A wide repertory of surgical options including ITx is required for optimal outcomes.

Long-term controlled outcomes after autologous intestinal reconstruction surgery in treatment of severe short bowel syndrome.

AIM: The aim of this study was to analyze outcomes of severe short bowel syndrome (SBS) treated with autologous intestinal reconstruction (AIR) surgery to facilitate independence of parenteral nutrition (PN). METHODS: PN dependence, growth, nutritional status, liver function, and survival were comparatively assessed in SBS children treated with (n=10) or without (n=18) AIR surgery. RESULTS: Median follow-up was 9.2 (4.9-14) years. Residual small intestinal length was 28 cm at birth. A total of 13 AIR procedures were performed at median age of 16 (11-41) months: serial transverse enteroplasty (STEP; n=8), longitudinal lengthening and tailoring (LILT; n=2), and tapering enteroplasty (n=3). Following STEP and LILT, length of the remaining small intestine increased by 19 (15-38) cm or 43% (38%-64%). Two children required repeat STEP due to recurrence of symptoms and bowel re-dilatation. Median duration of PN was 12 (11-17) months before and 14 (0-19) months after AIR. Eight children remain off PN 6.9 (3.6-9.7) years after surgery, and one with ultra short bowel (12 cm) receives 12% of energy as PN - all with reassuring growth and nutritional status and normal liver function. Actuarial PN dependence, including those who died on PN, was similar (P=0.19) with or without AIR surgery being 30% and 20% at four years, respectively. Complications of AIR surgery (lymphocele, bowel obstruction, and staple line bleeding) resolved without operative interventions. Survival was 90% with and 83% without AIR procedures. CONCLUSIONS: AIR surgery can provide long-term survival, independence of PN, and satisfactory general health in selected children with severe SBS.

Assessment and treatment of gastroesophageal reflux in healthy infants with apneic episodes: a retrospective analysis of 87 consecutive patients.

This retrospective study sought to assess whether gastroesophageal reflux (GER) is associated with recurrent infant apneic episodes (AEs) and whether its treatment prevents AEs. Symptoms, diagnostic measures, and treatment of GER in 87 infants admitted for AEs were recorded. The effect of GER on recurrent AEs and survival were assessed. Esophageal pH monitoring was done to 58/87 (67%) patients, of whom 53/58 (91%) had a pathological finding; 48 patients had treatment for GER (medical 43%/49%; surgical 5%/6%) with continuing AEs during hospitalization (25%/29% patients) as the main indication. Follow-up (65 patients) disclosed recurrent AEs in 12 (18%) patients (no treatment 4/21, medical 8/39, surgical 0/5, P = NS). All 87 patients survived. Recurrent AEs after discharge was predicted by AEs during hospitalization but not by pathological GER. AEs observed during hospitalization predicted postdischarge AE recurrence. Of GER treatment modalities, only surgery prevented recurrent AEs.

Effects of long-term parenteral nutrition on serum lipids, plant sterols, cholesterol metabolism, and liver histology in pediatric intestinal failure.

BACKGROUND AND OBJECTIVE: Plant sterols (PS) in parenteral nutrition (PN) may contribute to intestinal failure-associated liver disease. We investigated interrelations between serum PS, liver function and histology, cholesterol metabolism, and characteristics of PN. PATIENTS AND METHODS: Eleven patients with intestinal failure (mean age 6.3 years) receiving long-term PN were studied prospectively (mean 254 days) and underwent repeated measurements of serum lipids, noncholesterol sterols, including PS, and liver enzymes. PS contents of PN were analyzed. Liver biopsy was obtained in 8 patients. Twenty healthy children (mean age 5.7 years) served as controls. RESULTS: Median percentage of parenteral energy of total daily energy (PN%) was 48%, including 0.9 g · kg(-1) · day(-1) of lipids. Respective amounts of PN sitosterol, campesterol, avenasterol, and stigmasterol were 683, 71, 57, and 45 µg · kg(-1) · day(-1). Median serum concentrations of sitosterol (48 vs 7.5 µmol/L, P < 0.001), avenasterol (2.9 vs 1.9, P < 0.01), stigmasterol (1.9 vs 1.2, P < 0.005), but not that of campesterol (9.8 vs 12, P = 0.22), were increased among patients in relation to controls, and correlated with PN% (r = 0.81-0.88, P < 0.005), but not with PN fat. Serum cholesterol precursors were higher in patients than in controls. Serum liver enzymes remained close to normal range. Glutamyl transferase correlated with serum PS (r = 0.61-0.62, P < 0.05). Liver fibrosis in 5 patients reflected increased serum PS (r = 0.55-0.60, P = 0.16-0.12). CONCLUSIONS: Serum PS moderately increase during olive oil-based PN, and correlate positively with PN% and glutamyl transferase. Despite well-preserved liver function, histology often revealed significant liver damage.

Surrogate markers of cholesterol metabolism in children with native liver after successful portoenterostomy for biliary atresia.

PURPOSE: Cholestasis gradually ensues after portoenterostomy for biliary atresia (BA) and may deteriorate liver function. Cholesterol metabolism and its relationships with serum markers of liver function were evaluated in children living with native liver after successful portoenterostomy for BA. SUBJECTS AND METHODS: Serum lipids, noncholesterol sterol ratios to cholesterol, that is, surrogate markers of cholesterol metabolism, and liver function were cross-sectionally studied in 17 consecutive children after successful (postoperative bilirubin <20 micromol/L) portoenterostomy for BA with native liver and a mean age of 5.2 years. The results were compared with healthy age-matched controls. RESULTS: Mean serum total and low-density lipoprotein cholesterol and campesterol ratio were 18%, 43%, and 26% less than those of controls, respectively (P < .01 for all). Despite low serum cholesterol and campesterol (marker of cholesterol absorption) levels, serum lathosterol (marker of cholesterol synthesis) was decreased by 34% (P < .0001) from control levels and reflected serum prealbumin (r = 0.666) and cholestanol (r = -0.515, P < .05 for both). Cholestanol, twice higher than those of controls (P < .0001), reflected abnormally high serum alkaline phosphatase, glutamyl transferase, and bile acids (r = 0.558-0.711, P < .05). Serum campesterol was inversely related with lathosterol (r = -0.238, P < .05) in controls, but not in patients (r = -0.039). CONCLUSION: Children living with native liver after portoenterostomy for BA are inclined to low serum concentration and absorption of cholesterol. Cholesterol homeostasis was disturbed so that low cholesterol absorption was not associated with compensatory increase in cholesterol synthesis that decreased together with worsening of cholestasis.

Cholesterol metabolism in pediatric short bowel syndrome after weaning off parenteral nutrition.

BACKGROUND: Small intestine essentially regulates cholesterol homeostasis. AIMS: To evaluate cholesterol metabolism in short bowel syndrome (SBS). METHODS: Cholesterol precursors (e.g., cholestenol, desmosterol and lathosterol) and plant sterols (campesterol and sitosterol), respective markers of cholesterol synthesis and absorption, were determined in SBS patients (n=12) an average of 31 months after weaning off parenteral nutrition and in age-matched controls (n=80). RESULTS: Among patients, serum cholesterol precursor sterol to cholesterol ratios were 2-10 times higher (P<0.0001 for each). Those without any remaining ileum had 1.2-2.8 times higher precursor sterol to cholesterol ratios than those with an ileal remnant (P<0.05 for each). Serum cholesterol concentration, campesterol/cholesterol and campesterol/sitosterol were 34-39% lower (P<0.05 for each) in relation to controls. Bile acid absorption was markedly impaired (2.4 (0.2-3.2)%). Plant sterol ratios reflected the absolute length of remaining jejunum (r=0.625-0.663), and precursor sterol ratios inversely that of ileum (r=-0.589 to 0.750, P<0.05 for all). CONCLUSION: After weaning off parenteral nutrition, patients with pediatric onset SBS continue to have marked intestinal malabsorption of bile acids and moderate cholesterol malabsorption resulting in decreased serum cholesterol despite a marked compensatory increase in cholesterol synthesis.

Outcomes of intestinal failure--a comparison between children with short bowel and dysmotile intestine.

BACKGROUND: We investigated whether mortality, intestinal adaptation, and liver function differ between intestinal failure (IF) patients with either short bowel (SB) or bowel dysmotility (DM). PATIENTS AND METHODS: Twenty-six consecutive patients with SB (n = 20) or DM (n = 6) treated between 2000 and 2007 were retrospectively assessed. Intestinal failure was defined as less than 25% of age-adjusted small intestinal length or dependence on parenteral nutrition (PN) more than 6 months. RESULTS: Median age-adjusted small intestinal length (17% vs 45%) and gestational age (35 vs 40 weeks) were (P < .05) shorter, whereas proportion of the remaining colon (86% vs 0%) was (P < .05) higher in the SB group relative to the DM group. Overall survival was 92%. Median peak serum bilirubin (80 vs 25 micromol/L) and rate of cholestasis (11/20 vs 0/6) were higher (P < .05) in the SB group. Short bowel rather than DM as an etiology of IF predicted weaning off PN (RR, 39.3; 95% confidence interval [CI], 1.43-526; P < .01) and development of cholestasis (risk ration [RR], 18.3; 95% CI, 0.658-127; P < .05). Three SB children developed liver failure and two died, whereas neither of these occurred in the DM group. CONCLUSIONS: Children with SB are more likely to wean off PN but more prone to cholestatic liver disease than those with DM as an etiology of IF.