Necrotizing Enterocolitis Totalis Research Articles

ICG fluorescence angiography is useful in guiding intra-operative surgical decision making in necrotizing enterocolitis

Background: Necrotizing enterocolitis (NEC) is a devastating condition in premature babies and surgical resection of the necrotic bowels is often required. Herewith, we reported the application of Indocyanine green fluorescence angiography (ICG-FA) as a novel approach to assist intra-operative decision.Methods: Between July 2021 and June 2022, ICG-FA was used in 3 patients with severe NEC requiring bowel resection. Their clinical presentations and operative findings were presented.Results: All babies were born at or before 32 weeks of gestation and the body weight at operation ranged from 1.14 to 1.5 kg. NEC developed on day 7 to day 10 of life. In all patients, ICG at a dosage of 0.1 mg/kg was injected intravenously before bowel resection . In case 1,a baby with intra-operative diagnosis of NEC totalis, ICG-FA demonstrated bowel perfusion in 25 cm proximal jejunum that was regarded as necrotic by inspection. Case 2 was a baby with severe inflammation affecting the entire bowel. The indistinguishable boundary between inflamed and ischemic bowel was made clear with ICG-FA and a life-long end colostomy was avoided. In case 3, the decision for primary anastomosis was supported by ICG-FA showing adequate perfusion at the resected ends. In all cases, there was no adverse reactions reported.Conclusion: ICG-FA is helpful in intra-operative decision during surgery for NEC. It is safe and informative regarding the intestinal perfusion. It helps to promote a more precise bowel resection and facilitate primary anastomosis.

Trisomy 18 and necrotizing enterocolitis

Both Trisomy 18 (T18) and Necrotizing enterocolitis totalis (NEC-T) are major causes of morbidity and mortality in neonates. However, to our knowledge there has never been a case of a full-term infant born with both T18 and NEC-T published in the literature. We present a case of NEC totalis in male neonate born at 39 weeks gestation with Trisomy 18. He was diagnosed with T18 in-utero, and after genetic counseling, the mother carried the pregnancy to term. After delivery, the newborn developed signs of respiratory distress, requiring use of continuous positive airway pressure support. On day of life (DOL) 8, multiple bloody bowel movements raised suspicion for NEC and after supplemental investigations prompted a series of exploratory laparotomies that revealed extensive necrotizing enterocolitis involving the entire gastrointestinal tract. On DOL 12, the family opted for palliative care and the patient subsequently expired. To our knowledge, after searching through the English literature, no cases were found showing a neonate with a combination of T18 and NEC-T.

Endoscopy in Infants With Gastrointestinal Bleeding Has Limited Diagnostic or Therapeutic Benefit.

Pediatric gastroenterologists are often consulted to perform diagnostic and therapeutic endoscopy in infants with gastrointestinal bleeding (GIB). The value of endoscopy and risk of complications in this population are not well characterized. We aimed to describe findings and outcomes of infants with GIB who undergo endoscopy. Retrospective, single-center, cohort study of hospitalized infants ≤12 months who underwent esophagogastroduodenoscopy (EGD) and/or colonoscopy/flexible sigmoidoscopy (COL) for GIB. Current procedural technology codes, international classification of diseases codes, and quality control logs identified infants. Fifty-six infants were identified from 2008 to 2019 (51.8% female; mean age 161.6 days). Seven endoscopies identified sources of GIB: gastric ulcers, a duodenal ulcer, gastric angiodysplasia, esophageal varices, and an anastomotic ulcer. Three infants underwent therapeutic interventions of banding/sclerotherapy of esophageal varices and triamcinolone injection of an anastomotic ulcer. Six infants underwent abdominal surgery for GIB or suspected intestinal perforation after endoscopy, where a gastric perforation, jejunal perforation at an anastomotic stricture, necrotizing enterocolitis totalis with perforation, Meckel's diverticulum, and a duodenal ulcer were identified. No source of bleeding was identified surgically in 1 infant with GIB. Respiratory failure, use of vasopressors or octreotide, administration of blood products, and high blood urea nitrogen were associated with increased likelihood of requiring surgery ( P < 0.05 for all). There was limited utility to performing endoscopy in infants ≤12 months old with clinical GIB. Endoscopy in these sick infants carries risk, and 3 infants in this series presented with a gastrointestinal (GI) perforation shortly after the procedure. These limitations and risks should influence clinical decision-making regarding endoscopy in infants with GIB.

1424. Short-Course Antibiotic Therapy for Urinary Tract Infection (UTI) in the NICU: It’s Safe and Effective!

Abstract Background Antibiotic overuse in the neonatal intensive care unit (NICU) has been associated with adverse patient outcomes such as necrotizing enterocolitis (NEC), late-onset sepsis, invasive candidiasis, bronchopulmonary dysplasia, neurodevelopmental impairment, and death. On 11/2020 at Nationwide Children’s Hospital, Columbus, OH, the Neonatal Antimicrobial Stewardship Program Committee recommended 5 days of antibiotic therapy with a subsequent antibiotic “timeout” for uncomplicated UTI without bacteremia as part of ongoing efforts to reduce antibiotic exposure among high risk infants in 7 Level 2/3/4 NICUs. Methods Retrospective review of all infants who received antimicrobial therapy for UTI after implementation of a UTI Diagnosis and Management Protocol. Twice weekly NEO-ASP meetings provided prospective audit and feedback to neonatologists on appropriate antibiotic therapy and duration. Pertinent clinical, laboratory, and outcome data were obtained from the infants’ electronic health record. Safety measure evaluated include recurrence of infection with the same previously identified pathogen in the fourteen days after discontinuation of antibiotic therapy and mortality. Results Since implementation of the UTI protocol, 28 infants (median gestational age, 28 wk [IQR, 26-30 wk; median birth weight, 1203 g [IQR, 731-1801 g]) received antimicrobial therapy for treatment of a UTI at a median age of 50 days (IQR, 28-69 days). The most frequent pathogens were E. coli (n=8), K. pneumoniae (n=5), and Enterobacter spp. (n=4). Median duration of antibiotic therapy was 6 calendar days (IQR, 5-6 days). Only 1 (4%) infant had antibiotic therapy restarted within 14 days of discontinuation of initial therapy; infant had fever but blood, urine, and CSF cultures were sterile and antibiotic therapy was discontinued after 48 hours. One (4%) infant (23 wk gestational age) died at 6 weeks of age from NEC totalis not related to the previous UTI. 22 (81%) infants have been discharged home while 6 remain in the NICU. Conclusion Our preliminary data suggests that short course antibiotic therapy (with a timeout) < ![if !supportAnnotations] >[SP1]< ![endif] > for uncomplicated UTIs in the NICU is an effective and safe therapy that ultimately may lead to less antibiotic exposure among high risk infants. Disclosures All Authors: No reported disclosures

Necrotizing enterocolitis totalis complicates an infantile presentation of ARL6IP1-related spastic paraplegia 61

Spastic paraplegia 61 is a rare, complicated form of hereditary spastic paraplegia characterized by diffuse sensory and motor polyneuropathy. Knowledge about the clinical manifestations of disease in patients with this genetic condition is limited. We describe a case of a 4-month-old infant with spastic paraplegia 61 (SPG61) who developed severe bowel inflammation consistent with necrotizing enterocolitis totalis. To our knowledge, this is the first reported case of necrotizing enterocolitis in a patient with this genetic condition.

Commentary on ethical dilemmas in the management of infants with necrotizing enterocolitis totalis

This is a commentary on the manuscript titled “Ethical Dilemmas in the Management of Infants with Necrotizing Enterocolitis Totalis”.

Commentary on ethical dilemmas in the management of infants with enterocolitis totalis

This is a Commentary on the manuscript titled “Ethical Dilemmas in the Management of Infants with Necrotizing Enterocolitis Totalis” by Pennington E, Javid P, Mueller C, et al.

Characteristics and outcomes of a multi-institution cohort of infants with necrotizing enterocolitis totalis.

ObjectiveTo characterize the presentation, management and outcomes of infants with necrotizing enterocolitis totalis (tNEC) vs surgical non-totalis NEC (sNEC).Study designThis retrospective study identified infants undergoing surgery for NEC through The Children’s Hospitals Neonatal Database. Demographic, surgical and mortality characteristics were compared.ResultsOf 1059 infants, 161 (15.2%) had tNEC. Perinatal characteristics did not differ. tNEC infants were older and were less likely to have pneumoperitoneum at referral (5.6% vs 13.1%, p < 0.001) or intestinal perforation at surgery (38.5% vs 66.7%, p < 0.001). Infants with tNEC were more acidotic preoperatively (7.1, [IQR 7, 7.3] vs 7.3, [IQR 7.2, 7.4], p < 0.001). Mortality was 96.9% for tNEC and 26.5% for sNEC (p < 0.001). tNEC cases varied by center, accounting for 0–43% of all surgical NEC cases.ConclusionsMortality is high for tNEC infants, who present at older age, with greater illness severity but are less likely to have intestinal perforation than sNEC infants.

Long-term outcomes of severe surgical necrotizing enterocolitis

PurposeWe sought to describe long-term outcomes of infants with severe surgical necrotizing enterocolitis (NEC). MethodsData were collected on infants with surgical NEC (2009–2018). Severe surgical NEC was defined by extensive bowel loss with residual bowel length <30 cm, and “NEC totalis” was identified per operative report. Post-operative management and long-term outcomes were assessed, including enteral autonomy, severe neurodevelopmental disability, and educational attainment. ResultsOf 268 infants with surgical NEC, 41 (15%) had severe surgical NEC, and 14/41 were identified as “NEC totalis”. Zero severe NEC vs. 8 (57%) “NEC totalis” patients were placed on comfort measures following initial surgery (p < 0.001). Twenty-five patients (93%) with severe NEC survived vs 3/6 with “NEC totalis” (p < 0.001). The 28 survivors (68%) were followed for a median (IQR) duration of 8(4,10) years. Nine (32%) with severe NEC were weaned from parenteral nutrition. Eight (29%) had ≥1 marker for severe neurodevelopmental disability, and 11/16(69%) (7–16 years) were attending school at last follow-up. ConclusionLong-term survival is excellent following initial discharge, and achievement of enteral autonomy is feasible in patients with severe surgical NEC. The majority of patients who survive do not have severe neurodevelopmental disability and participate in school. Given current survivals and outcomes, focus on measured residual bowel length may be more appropriate than the subjective term “NEC-totalis.” Type of StudyPrognosis Study. Level of EvidenceLevel II.

Variable management strategies for NEC totalis: a national survey.

Necrotizing enterocolitis (NEC) is a serious disease linked to prematurity. A variant, NEC totalis, is associated with nearly 100% mortality. There is wide variation in counseling practices for NEC totalis. Our objectives are to determine what treatment options, if any, are offered to families, and which factors influence these decisions. An anonymous survey was distributed to members of the AAP Sections on Neonatal-Perinatal Medicine and Pediatric Surgery. Data were analyzed utilizing chi-square tests and Spearman correlations, where applicable. In the setting of NEC totalis, 90% of the 378 respondents viewed offering life-sustaining interventions (LSI) as ethically permissible and 87% felt that transfer to another center willing to provide LSI should be considered; however, only 43% reported offering LSI to families. Management of NEC totalis remains challenging and significant practice variability persists. Most respondents do not offer ongoing medical/surgical management, despite believing it is an ethically permissible option.

Necrotizing enterocolitis totalis: High mortality in the absence of an aggressive surgical approach

BackgroundNecrotizing enterocolitis is the leading case of gastrointestinal-related morbidity in premature infants. Necrotizing enterocolitis totalis is an aggressive form of necrotizing enterocolitis, which has traditionally been managed with comfort care. Recent advances in management of short bowel syndrome have resulted in some reported long-term survival. MethodsUsing the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, studies that reported outcomes in children with necrotizing enterocolitis totalis were identified. The definition of necrotizing enterocolitis totalis was captured along with length of follow-up, patient demographics, and outcomes. ResultsA total of 766 articles were screened, of which 166 were selected for full article review. Of these, 32 articles included data on 414 patients with necrotizing enterocolitis totalis. In the majority of studies (52%), necrotizing enterocolitis totalis was not defined. Aggressive surgical therapy (defined as bowel resection or fecal diversion) was undertaken in 32 patients (7.7%), with a mortality rate of 68.8%. In contrast, nonaggressive surgical therapy was undertaken in 382 patients (92.3%), and the mortality in these patients was 95%. Long-term outcomes for necrotizing enterocolitis totalis survivors, such as length of time on parenteral nutrition, progression to liver and/or small bowel transplant, and quality of life, were not reported. ConclusionWe found that there is no accepted definition of necrotizing enterocolitis totalis. Aggressive surgical therapy is rarely pursued, which likely drives the overall high mortality rate. This study underscores the importance of standardizing the definition of necrotizing enterocolitis totalis and capturing short and long-term outcomes prospectively. With more aggressive surgical therapy, more infants are likely to survive this abdominal catastrophe, which was once thought to be uniformly fatal.

Necrotizing Enterocolitis Totalis: Changing the Conversation

Introduction: Necrotizing enterocolitis (NEC) is a major source of morbidity and mortality amongst neonates. Many patients are successfully managed medically while others require surgical intervention. For some, the bowel injury can be devastating resulting in significant morbidity and mortality. Approximately 10% of patients will develop NEC totalis (NEC-T), which is a rapidly progressive form of NEC involving most if not the entire bowel. Historically, NEC-T has been considered a “lethal” disease with nearly 100% mortality, and thus it has been common practice for medical providers to offer only comfort …

Short-term outcomes for preterm infants with surgical necrotizing enterocolitis.

To characterize the population and short-term outcomes in preterm infants with surgical necrotizing enterocolitis (NEC). Preterm infants with surgical NEC were identified from 27 hospitals over 3 years using the Children's Hospitals Neonatal Database; infants with gastroschisis, volvulus, major congenital heart disease or surgical NEC that resolved prior to referral were excluded. Patient characteristics and pre-discharge morbidities were stratified by gestational age (<28 vs 28(0/7) to 36(6/7) weeks' gestation). Of the 753 eligible infants, 60% were born at <28 weeks' gestation. The median age at referral was 14 days; only 2 infants were inborn. Male gender (61%) was overrepresented, whereas antenatal steroid exposure was low (46%). Although only 11% had NEC totalis, hospital mortality (<28 weeks' gestation: 41%; 28(0/7) to 36(6/7) weeks' gestation: 32%, P=0.02), short bowel syndrome (SBS)/intestinal failure (IF) (20% vs 26%, P=0.06) and the composite of mortality or SBS/IF (50% vs 49%, P=0.7) were prevalent. Also, white matter injury (11.7% vs 6.6%, P=0.02) and grade 3 to 4 intraventricular hemorrhages (23% vs 2.7%, P<0.01) were commonly diagnosed. After referral, the median length of hospitalization was longer for survivors (106 days; interquartile range (IQR) 79, 152) relative to non-survivors (2 days; IQR 1,17; P<0.001). These survivors were prescribed parenteral nutrition infrequently after hospital discharge (<28 weeks': 5.2%; 28(0/7) to 36(6/7) weeks': 9.9%, P=0.048). After referral for surgical NEC, the short-term outcomes are grave, particularly for infants born <28 weeks' gestation. Although analyses to predict outcomes are urgently needed, these data suggest that affected infants are at a high risk for lengthy hospitalizations and adverse medical and neuro-developmental abnormalities.

A novel scoring system to predict the development of necrotizing enterocolitis totalis in premature infants

Background/PurposeNecrotizing enterocolitis totalis (NEC-totalis) is the severest form of NEC, with mortality rate of almost 100% even in the busiest neonatal centers. Despite such a prognosis, its risk factors remain elusive. We seek to identify clinical and laboratory parameters that differentiate NEC-totalis from NEC, and to use these factors to develop a scoring system to identify patients at risk for NEC-totalis upon presentation. MethodNEC patients were identified from our electronic medical record using ICD9 code. Diagnosis of NEC-totalis was based on operative and autopsy reports. Patients were divided into 2 groups: NEC-but-no-totalis and NEC-totalis. Clinical/laboratory data were obtained for each group. T-test, multivariate logistic regression and backward stepwise regression analysis were performed to identify risk factors for NEC-totalis and these risk factors were formulated into a “Totalis Score.” ResultAmong 157 NEC patients, 13 had NEC-totalis. NEC-totalis patients, compared to NEC alone, had fewer platelets, older age at diagnosis of NEC and greater phosphorus and creatinine levels. A 0-5 point scale “Totalis Score” based on these risk factors had sensitivity of 92% and a specificity of 78% for the diagnosis of NEC-totalis. ConclusionLow platelet, high phosphorus, high creatinine and older age at diagnosis of NEC were associated with a greater risk of developing NEC-totalis.

Cytokine Levels in the Preterm Infant with Neonatal Intestinal Injury

The purpose of this study is to characterize the cytokine response of preterm newborns with surgical necrotizing enterocolitis (NEC) or spontaneous intestinal perforation (SIP) before surgical treatment and to relate these finding to intestinal disease (NEC vs. SIP). The study was a 14-month prospective, cohort study of neonates undergoing surgery or drainage for NEC or SIP or surgical ligation of patent ductus arteriosus (PDA). Multiplex cytokine detection technology was used to analyze six inflammatory markers: interleukin-2, interleukin-6 (IL-6), interleukin-8 (IL-8), interleukin-1 β (IL-1β), interferon-gamma, and tumor necrosis factor-α (TNF-α). Patients with NEC had much higher median preoperative levels of IL-6 (NEC: 8,381 pg/mL; SIP: 36 pg/mL; PDA: 25 pg/mL, p < 0.001), IL-8 (NEC: 18,438 pg/mL; SIP: 2,473 pg/mL; PDA: 1,110 pg/mL, p = 0.001), TNF-α (NEC: 161 pg/mL; SIP: 77 pg/mL; PDA: 71 pg/mL, p < 0.001), and IL-1β (NEC: 85 pg/mL; SIP: 31 pg/mL; PDA: 24 pg/mL, p = 0.001). Patients with NEC totalis (NEC-totalis had the highest levels of IL-8 and were significantly different from infants with limited NEC (28,141 vs. 11,429 pg/mL, p = 0.03). Surgical NEC is a profoundly more proinflammatory disease than SIP. The cytokine profiles of patients with SIP are closer to those of a nonseptic surgical neonate.

What Role Does Laparoscopy Play in the Diagnosis and Immediate Treatment of Infants with Necrotizing Enterocolitis?

Laparotomy is the preferred option for the surgical treatment of necrotizing enterocolitis (NEC); however, laparoscopy may offer benefit, particularly when it is uncertain if the operation is necessary. Our aim was to review the current evidence for the use of diagnostic and therapeutic laparoscopy in NEC. A systematic review of published literature (1990-July 2012) found in MEDLINE, EMBASE, the Cochrane Library Controlled Trials Register, and the Cumulative Index to Nursing and Allied Health reporting the use of laparoscopy for management of acute NEC was undertaken. Seven studies (six case series and one case report) reporting the use of laparoscopy in 44 infants (weighing 0.5-2.9 kg) with NEC were identified and included for review. There were no randomized controlled trials. Laparoscopic techniques differed among the studies, with standard laparoscopy (gas) used in 28 (64%) cases, fluorescein-aided assessment in 8 cases (18%), and gasless laparoscopy in 8 cases. Eight (18%) infants did not require further surgery following laparoscopy: 4 (9%) had no evidence of NEC, 2 (5%) had no evidence of perforation and/or intestinal gangrene, and 2 (5%) had NEC totalis precluding further surgery. In the remaining 36 (86%) infants in whom further procedures were required following laparoscopy, 9 had placement of a peritoneal drain, 20 had a stoma, and 7 had intestinal anastomosis. Perforation was detected in 25 (57%) infants; however, among the 19 infants who had no detectable perforation at initial laparoscopy, 1 infant (5%) subsequently required laparotomy for a missed perforation. Six (14%) infants died: 2 had care withdrawn due to pan-intestinal NEC, 2 died of ongoing NEC, and 2 of recurrent NEC following recovery from the acute episode. Laparoscopy is a useful tool in the armamentarium of surgical management of NEC, with one-fifth of patients not requiring further surgery. Laparoscopy can be useful in selected babies with suspected NEC to avoid unnecessary laparotomy and minimize the trauma of surgery.

The Clip and Drop Back Technique in the Management of Multifocal Necrotizing Enterocolitis: a Single Centre Experience

The surgical management of multifocal necrotizing enterocolitis (NEC) remains a major challenge. The "clip-and-drop" strategy with a second-look laparotomy permits re-assessment of bowel viability after optimization, thus offering the potential of both improving survival and conserving bowel length. This study reviews the outcome of this strategy in a single regional center. Since 2000, NEC patients undergoing emergency laparotomy selectively underwent a "clip-and-drop" operation if there was peri-operative instability and/or multifocal disease with uncertain bowel viability. Bowel with full thickness gangrene was resected and bowel-ends were temporarily tied-off; a second-look definitive procedure was performed when the patient had stabilized. For this review, in-hospital and follow-up records were studied retrospectively for demographics, 30-day mortality and long-term outcome. Between 2000 and 2010, 16 patients underwent a "clip-and-drop" operation. The mean post-conception age was 32.8 weeks (27.7-41.7 weeks) with a median body weight of 1.4 kg (0.76-4.4 kg) at first operation. Preoperative radiograph showed free gas in 43.8% and portal venous gas in 37.5% of patients. 2 patients did not survive to the second laparotomy. 14 patients received a second laparotomy, after a mean of 51 h (35-74 h). 2 patients were found to suffer from NEC totalis on the second laparotomy and died without further procedures. All other patients (n = 12) had stoma formation. 1 patient died 4 days after stoma formation. The 30-day mortality for NEC with the "clip-and-drop" strategy was 31.6% (5/16). Among the 11 survivors, 1 died from liver failure complicated by short bowel syndrome at 5 months post operation, 2 others died from respiratory complications of prematurity despite adequate gastrointestinal function. The median follow-up time for the 8 long-term survivors was 45 months (7-129 months). Their median time to achieving full feeds was 41 days (range 21-105 days) after the second operation. The "clip-and-drop" strategy, when used in selected patients with multifocal NEC, may help bowel conservation in survivors.

Risk factors for necrotizing enterocolitis totalis: a case–control study

The objective of this study was to compare demographic and clinical events in three groups of preterm neonates: those with necrotizing enterocolitis totalis (NEC-T), those with NEC non-totalis (NEC non-T) and in preterm patients without NEC. This retrospective case-control study was conducted at Yale New Haven Children's Hospital using patient data from January 1991 to December 2007. Study patients were less than 36 weeks of gestational age (GA) at birth, without gastrointestinal (GI) malformations. Cases (NEC-T) were diagnosed at operation or at autopsy with observation of >80% necrosis of the GI tract. Two control groups were assigned: Group 1 or NEC non-T and Group II or Non-NEC. Two to four controls per case were matched to cases by GA at birth±2 weeks. Demographic and clinical data for the day of diagnosis and retrospectively up to 7 days preceding diagnosis were recorded for those with NEC-T and NEC. Group II controls were matched for date of birth and day of life, in addition to GA at birth. A total of 14 075 patients were admitted to the Newborn Special Care Unit during the study interval. Overall 328 patients (2.3%) developed NEC≥Bell's Stage II; 39 patients met inclusion criteria for NEC-T case status; 148 NEC non-T and 110 non-NEC controls were assigned. In the comparison of NEC T and NEC non-T neonates, use of breast milk was associated with decreased risk of NEC-T, adjusted odds ratio (OR)=0.26, 95% confidence interval (CI) of OR=0.08-0.085, P=0.03. When NEC T and non-NEC patients were compared, having reached full-enteral feeds before the date of diagnosis of the matched case (adjusted OR=28.5, 95% CI of OR=2.7-299, P=0.005) and use of breast milk (adjusted OR=0.09, 95% CI of OR=0.02-0.56, P=0.01) were significantly different between the two groups. Breast milk usage was significantly associated with decreased occurrence of NEC-T in our comparison of NEC-T, NEC non-T and non-NEC patients. Although there were some differences, the majority of demographic and clinical variables assessed were not shown to be significantly different between cases and controls. This highlights the need for more biological data in assessing risk of developing NEC-T.

Portal venous air: The poor prognosis persists

Background/Purpose: The prognostic importance of portal vein air (PVA) in babies with necrotizing enterocolitis (NEC) has been controversial. This study compares the outcome in babies with NEC and PVA treated surgically versus those with medical management. Methods: Forty neonates in the neonatal intensive care unit (NICU; 1995 through 1999) had (PVA) during their hospitalization. Babies were analyzed for gestational age (GA), birth weight (BW), and survival after operative versus medical management. Results: The average GA was 26 weeks, average BW was 1,173 g. Twenty-three patients (57.5%) tolerated full feedings and 8 (20%) partial feedings at diagnosis. All 40 babies required intubation at birth with 23 (57.5%) requiring reintubation with onset of PVA. In all cases, PVA was present within 24 hours of onset of abdominal distension, feeding intolerance, or heme-positive stools. Two cases of PVA “resolved” only to recur later in the patients' courses. Thirty-two patients (80%) manifested pneumatosis intestinalis on abdominal radiographs, and 8 (20%) had perforations. Acidosis was present in 25 (63%) patients, and vasopressor support (dopamine) was required in 15 (38%), with 2 patients requiring support only preoperatively. Initial management consisted of bowel rest, fluid resuscitation, orogastric decompression, and broad-spectrum antibiotics. Operation was performed in 31 (78%). Seventeen underwent resection with ostomy formation with 6 deaths and 11 survivors. Four underwent resection using the clip and drop back method, with one death and 3 requiring an ostomy at second look laparotomy. Ten had NEC totalis and closure of the abdomen only. Overall operative mortality rate was 17 of 31 (54%). Nine seemingly stable patients were treated nonoperatively. Six had progressed disease and died before salvage laparotomy could be performed, whereas 3 (33%) survived without further therapy. Conclusions: PVA has been a relative indication for operation. This view has been challenged by the survival of some patients without laparotomy. Although nonoperative therapy seems appealing in hemodynamically stable patients without acidosis, our data confirm the poor prognosis of infants with PVA and NEC. J Pediatr Surg 36:1143-1145. Copyright © 2001 by W.B. Saunders Company.

Significance of portal vein air in necrotizing entercolitis: Analysis of 53 cases

Fifty-three of 177 infants with necrotizing enterocolitis (NEC) developed portal vein air (PVA). These infants were evaluated for prenatal, perinatal, therapeutic, clinical, laboratory, radiographic, and operative factors as they related to infants with and without PVA. A significantly higher incidence of PVA was seen in infants with NEC who weighed less than 2400 g (P less than 0.025). The use of Vitamin E (P less than 0.01), aminophylline, and high-density premature formula (P less than 0.001) was associated with an increased incidence of PVA. NEC totalis was seen in 55% of the infants with NEC and PVA (P less than 0.001). The presence of PVA was associated with a 58% mortality rate (P less than 0.05). Infants with NEC and PVA operated on prior to 1983 had a 71% mortality. Since 1983, seven of nine infants with NEC and PVA survived early operation using PVA as an indication for surgical intervention. In infants at risk for this highly lethal form of NEC, the avoidance of enteral feedings or altered dietary intake, cautious placement of arterial catheters and the judicious application of pharmacologic agents (eg, Vitamin E, aminophylline) is of importance in regard to prevention. These observations suggest that PVA is a sign of advanced disease and that these high-risk patients should be considered candidates for early surgical intervention.