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PubMed1992

Short-bowel syndrome: a collective review.

Neonates can now be expected to survive with very short lengths of bowel because of advances in pediatric neonatal care and in parenteral and enteral nutrition. Most pediatric surgeons have only a few patients with this problem under their care, so individual experience is limited. This collective survey, carried out by postal questionnaires to pediatric surgeons in the British Association of Paediatric Surgeons in the United Kingdom with an interest in the gastrointestinal tract, documents current techniques of management, the complications encountered, and reviews the clinical and economic consequences of prolonged total parenteral nutrition. We conclude that within the limitations of resources and our understanding of prognosis, neonates, especially if less than 35 weeks' gestation, with remaining jejunoileal segment of greater than 20 cm with an intact ileocecal valve (ICV) or greater than 30 cm without an ICV, should be considered salvageable.

Enteral Nutrition↗
PubMed1993

Gastroschisis: an 18-year review.

From 1972 to 1990, 69 cases of gastroschisis were treated at Akron Children's Hospital Medical Center. Eighty-one percent of these patients underwent primary closure of their abdominal wall defect. Thirteen of 69 patients (19%) required Silastic silos with final closure in an average of 7.8 days. There was no sex predilection, the average birth weight was 2,473 g, and the mean gestational age was 36.3 weeks. Twenty-six percent had associated anomalies, the majority were intestinal atresia, volvulus, and/or undescended testicles. Seventy-seven percent of the infants were delivered vaginally. Fourteen children were delivered via cesarean section. Seven cesarean sections were done solely for prenatal ultrasonic identification of an abdominal wall defect. There was no improvement in hospital stay, complications, days until enteral feeds were tolerated, days intubated, or number of surgical procedures in this group. In 14 patients, mesh sheeting (Marlex, Silastic) was used in the final closure. Sixty-four percent of these incurred wound breakdown necessitating removal of the mesh. This compares with a 3.2% wound breakdown in the nonmesh group. The average hospital stay was 43.9 days and the average time to enteral feeds 20.2 days. Sixty-four percent of the patients required postoperative intubation for an average of 5.5 days. The overall mortality rate was 4.3%. The present data do not support gastroschisis alone as an indication for cesarean section. The data indicate that mesh be avoided in the final closure if possible and support a favorable prognosis for most babies.

Abdominal Muscles↗
PubMed1993

Adaptation in short-bowel syndrome: reassessing old limits.

The improving survival of patients with severe short-bowel syndrome along with the advent of successful intestinal transplantation have accentuated the need to answer two questions. (1) Is there an intestinal length below which adaptation to full enteral nutrition can not be expected to occur? (2) How much time is necessary to complete intestinal adaptation? We reviewed the outcome of 21 infants with less than 50 cm of small intestine to answer these questions. Patients were divided into three groups based on intestinal length, regardless of ileocecal valve status: group I, < 10 cm (n = 3); group II, 10 to 30 cm (n = 11); and group III, 30 to 50 cm (n = 7). Data were collected to assess survival, incidence of adaptation, time to adaptation, and causes of mortality. Infants in group I did not achieve intestinal adaptation to full enteral nutrition. One survived and 2 died, one from varicella pneumonia and the other after intestinal transplantation. Eight of the 11 (73%) patients in group II survived and 5 of 8 (63%) survivors achieved full intestinal adaptation after a mean interval of 320 days (range, 148 to 506 days) on parenteral nutrition. Six of the seven patients (86%) in group III survived and all survivors (100%) achieved complete enteral adaptation after an average of 376 days (range, 58 to 727 days). The overall survival was 71% (15/21), but survival in patients with > 10 cm was 78%.(ABSTRACT TRUNCATED AT 250 WORDS)

Catheterization, Central Venous↗
PubMed2005

Neonatal short bowel syndrome: a cohort study.

BACKGROUND: To date, our knowledge of morbidity and mortality in neonatal short bowel syndrome (SBS) is based on individual case series. Shortcomings of the published literature include long patient recruitment time, selection bias, variable SBS definitions, failure to account for gestational age, and incomplete follow-up. By applying more rigorous methodology, our aim was to determine outcomes of SBS neonates compared with a control group of neonates without SBS. METHODS: A cohort study of all neonates with abdominal pathology requiring laparotomy between January 1, 1997, and December 31, 1998, with observation through July 1, 2001. Short bowel syndrome was defined as patients requiring parenteral nutrition for more than 42 days or residual small bowel length of less than 25% predicted by gestational age. Student's t test, Mann-Whitney U test, and chi2 were used where appropriate. Kaplan-Meier curves were used to determine cumulative survival. Covariates important in the development of SBS were examined using forward step-wise logistic regression. RESULTS: There were 175 patients (with SBS = 40, without SBS = 135) with a mean gestational age of 30.7 +/- 4.6 weeks vs 35.9 +/- 4.8 weeks, respectively (P < .0005). The patients with SBS suffered significantly more morbidity than the group without SBS in all categories of investigation (surgical complications, septic events, central venous line complications, duration to adaptation and parenteral nutrition independence, cholestasis and liver failure, and duration of hospitalization). The case fatality rate was 37.5% in patients with SBS vs 13.3% in patients without SBS (P = .001). Most of the deaths were caused by liver failure or sepsis and occurred within 1 year from the date of surgery. Presence of an ileostomy (exp(B) = 12.29; P < .0005) and a residual small bowel length less than 50% of the original length (exp(B) = 26.84; P < .0005) were the only 2 variables in a logistic regression analysis found to be independently associated with the development of SBS. CONCLUSION: This cohort study clearly illustrates the tremendous morbidity experienced by infants with SBS relative to other surgical neonates. Accurate estimates of the morbidity associated with SBS enables clinicians to appropriately counsel parents, allocate resources and initiate therapeutic trials.

Cholestasis↗
PubMed2005

A randomized controlled trial of elective preterm delivery of fetuses with gastroschisis.

BACKGROUND: Elective preterm delivery of the fetus with gastroschisis may help to limit injury to the extruded fetal gut and thus promote faster recovery of neonatal gut function and earlier hospital discharge. This hypothesis has not previously been tested in a prospective randomized controlled trial. METHODS: Between May 1995 and September 1999, all women referred to a single tertiary center before 34 weeks' gestation with a sonographically diagnosed fetal gastroschisis were invited to participate in a randomized controlled trial. Eligible patients were randomized to elective delivery at 36 weeks or to await the onset of spontaneous labor. The method of delivery was not prescribed by the trial. Primary outcome measures in the neonate were the time taken to tolerate full enteral feeding (150 mL/kg per day) and duration of hospital stay. RESULTS: Of 44 eligible women, 42 were randomized, 21 to elective delivery and 21 to await spontaneous labor. There were 20 liveborn infants in each group. Four babies in the elective group and 4 in the spontaneous group delivered before 36 weeks' gestation but were included in the analysis on an intention-to-treat basis. Mean gestational age at delivery was 35.8 weeks in the elective group and 36.7 weeks in the spontaneous group. Primary closure of the gastroschisis was achieved in a similar proportion (80%-85%) of infants in both groups. Two babies in the elective group died from short gut complications. In the survivors, there was a trend in favor of a shorter median time to achieve full enteral feeding (30.5 vs 37.5 days) and a shorter median duration of hospital stay (47.5 vs 53 days) in the elective group, but this was not statistically significant. These findings remained unaltered when the data were reanalyzed after (a) excluding infants with intestinal atresia or (b) excluding infants born before 36 weeks' gestation. CONCLUSIONS: Although limited by the small number of patients, this randomized controlled trial demonstrates no significant benefit from elective preterm delivery of fetuses with gastroschisis.

Adult↗
PubMed2006

Outcome of gastroschisis: a 20-year case review of infants with gastroschisis born in Galveston, Texas.

BACKGROUND/PURPOSE: In the past decade, the preferred method of closure of gastroschisis at our institution has been staged reduction using a silo with repair on an elective basis (SR) rather than primary surgical closure (PC). We performed a 20-year case review of infants with gastroschisis at a university hospital to compare these shifts in management and to determine factors affecting outcome. METHODS: Seventy-two cases were reviewed from 1983 to 2003. Times to first and full feeds were outcome variables for statistical analysis. RESULTS: The prevalence of gastroschisis increased from 0.03% to 0.1% since 1983. Patients had low birth weights (mean = 2294 g) and were borderline premature (mean = 35.8 weeks). Only 3% of the infants were African American. There was a high rate of cesarean deliveries (57%). Ten patients (15%) had gastroschisis complicated by liver herniation, intestinal atresia(s), and/or necrosis/perforation. Most patients were managed by SR (67%). Eight percent of the infants died, 9% developed necrotizing enterocolitis, and 50% had other gastrointestinal complications. Twenty-seven percent of the infants managed with SR did not need initial mechanical ventilation. However, the patients who underwent SR were ventilated longer after birth as compared with those who underwent PC (P < .08). Infants with a complicated gastroschisis had significantly longer times to first and full feeds (P < .001). Patients managed with SR took significantly longer to reach full feeds (P = .001), and there was a trend of starting feeds later (P = .06). When patients with a complicated gastroschisis were excluded, the differences between the SR and PC groups were even greater (P = .01; P < .001). CONCLUSIONS: In our patient population, the prevalence of gastroschisis increased by more than 400% since 1983. The defect was rare in African-American infants. Management by SR was associated with longer ventilation times and longer times to first and full feeds for both uncomplicated and complicated gastroschisis cases.

Digestive System Surgical Procedures↗
PubMed2006

Cow's milk protein allergy presenting with Hirschsprung's disease-mimicking symptoms.

BACKGROUND: Pediatric surgeons often encounter neonates who present with Hirschsprung's disease (HD)-like symptoms and plain x-ray findings, but respond well to conservative treatment. During our investigation of the etiology of this condition, which we named "benign transient nonorganic ileus of neonates" (BTNIN), we noticed that BTNIN included cases of cow's milk allergy (CMA). Therefore, a prospective study of the identity of BTNIN and CMA was conducted. METHODS: Cow's milk allergy was diagnosed when a baby showed HD-like symptoms after oral feeding, and a drug-induced lymphocyte stimulation test was positive for cow's milk with a titer of more than 300%. MATERIALS: Of 38 neonates with suspected HD, a surgical disorder was excluded by plain x-ray in 9, intestinal atresia was diagnosed in 3, and the remaining 26 were enrolled in this study. RESULTS: Of 26 cases, 9 were diagnosed as HD by manometric studies and 17 as CMA. Thirteen of 17 CMA cases had been fed with breast milk and 4 with formula milk. CONCLUSION: The proportion of CMA in the cases presenting with HD-like symptoms in the neonatal period is much higher than what we expected, and most cases of BTNIN are caused by CMA. If HD is ruled out, CMA should be considered.

Diagnosis, Differential↗
PubMed2006

The acute abdomen in the newborn.

The acute abdomen in the newborn provides challenging problems from many aspects, not only with regard to diagnosis, resuscitation and treatment, but also now with prenatal management. Most conditions are uncommon and treatment in specialist centres enables concentration of appropriate resources and expertise. Co-morbidity is common, particularly in the preterm or low birth weight infant. A multi-disciplinary team of surgeons, anaesthetists, neonatologists, radiologists, cardiologists, obstetricians, nurses, physiotherapists and other health professionals experienced in dealing with extremely small infants will provide the best outcome. The infant should be resuscitated and, as soon as conditions permit, transferred to a specialist surgical centre with intravenous fluids, gastric tube drainage and circulatory, respiratory and general support as needed. This involves close liaison within healthcare networks and readily available patient transfer facilities. Surgery itself should be carried out in a theatre fully equipped for neonatal surgery. A gentle touch is essential because of the fragility of the tissues, and painstaking care should be taken with blood loss.

Abdomen, Acute↗
PubMed2006

Midgut abnormalities.

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Diagnostic Techniques, Digestive System↗
PubMed2003

Transumbilical laparotomy in infants: a novel approach for a wide variety of surgical disease.

BACKGROUND/PURPOSE: Laparoscopy has kindled an interest in minimally invasive surgery in infants. To combine improved cosmesis with the advantages of open exposure, we adopted the transumbilical approach for laparotomy in babies up to 26 months of age. METHODS: Only hemodynamically stable babies were considered candidates. The umbilicus was incised nearly circumferentially, and the peritoneum was entered in the midline. The procedure was completed in the standard, open fashion by positioning the incision over the area of interest, and the periumbilical wound was closed subcuticularly. RESULTS: Since 1998, 42 infants have undergone 44 transumbilical laparotomies, with only one conversion to a traditional incision (operating time, 35 to 202 minutes). Twenty-nine newborns were treated transumbilically for duodenal atresia/stenosis/web (n = 11), malrotation (n = 7), intestinal atresia/stricture (n = 5), and other pathology (n = 7). Thirteen older infants (1 to 9 months) were treated transumbilically for intussusception (n = 4), malrotation (n = 4), and other pathology (n = 5). Three toddlers (13 to 24 months) also underwent successful transumbilical laparotomy. There were 2 wound infections and one anastomotic leak. CONCLUSIONS: These results suggest that a transumbilical approach is feasible, safe, and cosmetically superior to the open operation for a broad spectrum of surgical procedures in infants, and it may also be useful in older children.

Child, Preschool↗
PubMed2003

Serum growth factors and growth indices pre- and post-pediatric intestinal transplantation.

BACKGROUND/PURPOSE: Although intestinal transplantation (ITx) has succeeded in liberating children with intestinal failure from total parenteral nutrition (TPN), positive growth has yet to be achieved in the majority of patients. This investigation aims to evaluate levels of serum growth factors as they relate to growth parameters and nutritional outcomes. METHODS: Serum measures of insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP-3) that had been obtained before and after transplantation were reviewed (with Institutional Review Board approval) in a subset of pediatric ITx recipients. Z-scores for weight and height were calculated at transplant and biannually thereafter for 2 years. RESULTS: Five children received a small bowel/liver transplant between August 1996 and March 2000 (median age, 1.3 years). Before transplantation, levels of IGF-1 and IGFBP-3 were low in 60% and 67% of patients, respectively. Posttransplant levels of these growth factors were within normal limits or elevated in all but 2 patients (IGFBP-3 only). A positive trend in z-scores was observed in just one of 5 patients for weight and in 2 of 5 for height/length during the follow-up period. Of the 3 patients who experienced negative linear growth velocity over time, 2 had low pretransplant levels of both IGF-1 and IGFBP-3. All patients were weaned from TPN within 3 months after transplant. CONCLUSIONS: Pretransplant levels of growth mediators may be predictive factors in children who will require an intensive regimen of nutritional rehabilitation posttransplant to promote the growth process. Absorption studies may aid in determining the appropriate nutrient substrates for the post-ITx population.

Anthropometry↗
PubMed1986

Imperforate anus in females: frequency of genital tract involvement, incidence of associated anomalies, and functional outcome.

Of 162 female patients with imperforate anus, 21% had a noncommunicating and 79% a communicating anomaly of the rectum or anus. Associated anatomical abnormalities were found in the lower urinary tract (15%), upper urinary tract (25%), lower genital tract (27%), upper genital tract (35%), and additional organ systems (51%). Death occurred in 26 patients and in 19, this was attributed to the associated abnormalities. Functional outcome was assessed in those patients 13 years of age or older. Bowel function was normal or near normal in 85%, as was urinary and renal function. In 44% of patients evaluated, there was persistent vaginal abnormality or scarring and in 25%, this was severe enough to require future surgery.

Abnormalities, Multiple↗
PubMed1988

Maternal serum alpha-fetoprotein is a marker for fetal anomalies in pediatric surgery.

Maternal serum alpha-fetoprotein (MS-AFP) screening has become part of routine obstetric care. Although elevated MS-AFP was originally associated with neural tube defects (NTD), it is also able to detect several fetal anomalies of interest to a pediatric surgeon, ie, ventral abdominal wall defects, intestinal atresias, and sacrococcygeal teratomas. Previously, decreased MS-AFP had only been associated with fetal trisomies, but not surgically correctable lesions. In the present study, we review our recent experience with both elevated and decreased MS-AFP as a marker to detect fetal anomalies of concern to the pediatric surgeon. Forty-one fetal anomalies were associated with 333 pregnancies referred for follow-up after abnormal MS-AFP screening results from November 1985 through November 1986. One hundred ninety-six were elevated and 139 were decreased. In most cases, evaluation included counseling, repeat MS-AFP, level II ultrasound, and amniocentesis. This revealed elevated MS-AFP to be associated with 32 (16.3%) anomalies (2 NTD, 5 anencephalics, 5 ventral abdominal wall defects, 1 stage IV-S neuroblastoma, 1 renal anomaly, 1 ventriculomegaly, 15 fetal demises, and 2 fetal-maternal bleeds). Decrease in MS-AFP was associated with nine (6.4%) anomalies (2 congenital diaphragmatic hernias, 3 Down's syndrome, 1 Turner's syndrome, 2 duodenal atresias, and 1 choroid plexus cyst). In this study, MS-AFP detected several fetal anomalies known to be associated with abnormal MS-AFP and three anomalies not previously described (congenital diaphragmatic hernia, neuroblastoma, and choroid plexus cyst). Elevated as well as decreased MS-AFP are significant and should be pursued by a full prenatal evaluation.(ABSTRACT TRUNCATED AT 250 WORDS)

Algorithms↗
PubMed1989

Microcolon in the absence of small bowel obstruction in the newborn.

Microcolon is a manifestation of intestinal obstruction in utero, and in the overwhelming majority of cases it accompanies low small bowel obstruction. Two cases of congenital abdominal masses, an ileal duplication, and a cystic mesenchymal hamartoma of the liver caused microcolon although the bowel was intrinsically normal. Bowel compression in utero may also lead to neonatal microcolon.

Adult↗
PubMed1997

Is nasogastric tube decompression necessary after major abdominal surgery in children?

Nasogastric (NG) decompression has traditionally been used after major abdominal surgery in pediatric patients. This study was designed to determine if NG tubes could be routinely omitted in pediatric patients undergoing major abdominal procedures. Between January 1993 and December 1995, 83 patients had follow-up prospectively without NG decompression after a variety of major abdominal surgeries. NG tubes were inserted for persistent vomiting or abdominal distension. Exclusion criteria included bowel obstruction, intestinal atresia, and perforation of the stomach or duodenum. Ages ranged from 13 days to 22 years. Seventy-four patients (89%) were treated successfully without postoperative NG decompression. There were no cases of pneumonia, wound dehiscence, anastomotic leak, or delay in return of gastrointestinal function. Nine patients required NG tubes for persistent vomiting or abdominal distension. An anastomotic leak developed in one patient after endorectal pull-through. NG decompression is unnecessary after most major abdominal operations in pediatric patients. The endorectal pull-through may represent a group of patients that benefit from routine decompression.

Adolescent↗