Early Enteral Nutrition Following Gastrointestinal Surgery in Children: A Systematic Review of the Literature.

OBJECTIVE: To investigate the safety and effectiveness of early enteral feeding versus traditional enteral feeding after gastrointestinal (GI) anastomosis in children in the pediatric literature. SUMMARY OF BACKGROUND DATA: Prolonged postoperative fasting has been the traditional model of care following pediatric GI surgery. In contrast, early feeding has become well established in the adult population, where meta-analyses have shown early introduction of enteral feeds to be beneficial to hospital stay and patient outcomes. METHODS: Comprehensive literature searches of the English literature search (PubMed, Ovid, Embase databases) from inception to present according to the PRISMA guidelines. Included studies were assessed according to the MINORS criteria. Outcomes for time to first feed and full feeds, and discharge, and risk of major complications were synthesized. RESULTS: Ten studies comprising 451 patients were included in the analysis. All studies aimed at investigating the safety of early feeding in pediatric GI surgery, with or without a fast-track program. Only 4 studies compared the study group to a control group in which patients were fed in a traditional way (traditional feeding).Most studies defined early feeding as feeds commenced ≤24 hours postoperatively (range 2-72 hours). Mean time to first feed was significantly lower in the early feeding group, but not significantly lower for the mean time to full feeds and mean hospital stay. Bowel obstruction and anastomotic breakdown were classed as major complications. There was no significant difference in their occurrence in both groups. CONCLUSIONS: Although the studies identified are few and heterogeneous, they demonstrate that there is no clear advantage of keeping children "nil by mouth" and no clear disadvantage of providing early enteral nutrition following elective gastrointestinal surgery. Larger randomized controlled trials are required to assess the true impact on postoperative complications, health care associated costs, and to investigate patient-reported outcome measures.

Is Screening of Intestinal Foregut Anatomy in Heterotaxy Patients Really Necessary?: A Systematic Review in Search of the Evidence.

OBJECTIVE: (1) Is screening of intestinal rotational anatomy obligatory in "asymptomatic" patients with heterotaxy? (2) Does detection of an anomaly warrant surgical correction? SUMMARY OF BACKGROUND DATA: Heterotaxy is an abnormal arrangement of thoraco-abdominal viscera across a left-to-right axis. Intestinal rotational anomalies are frequent among patients with heterotaxy, but debate exists as to whether they are benign in nature, requiring careful observation alone, or if surgical correction is warranted to prevent obstruction or midgut volvulus. METHODS: A systematic review [according to PRISMA guidelines] was conducted using CINAHL, EMBASE, Medline, and Cochrane Databases. Article quality was assessed using MINORS criteria. Conference proceedings and unpublished data were screened additionally. RESULTS: Nineteen studies met the eligibility criteria but reporting was adequate for 9. All were observational studies. These included a total of 414 patients managed expectantly, that is, "asymptomatic patients" in whom no intestinal rotation screening was undertaken (group A), 191 cases in whom screening was performed routinely (group B), and 92 patients considered "symptomatic" of potential rotational anomalies and therefore underwent imaging or laparotomy (group C). In group A, 1 patient developed symptoms attributable to malrotation in whom laparotomy confirmed the diagnosis (0.24%). Among groups B and C, 151 had Ladd's operations (53%) and 14 cases of malrotation with obstruction or volvulus were described (4.9%), of which 2 "symptomatic patients" died before laparotomy. Overall surgical complication rate was 17% with 30-day mortality rate of 2.6% to 4.6%. CONCLUSION: The evidence base for screening "asymptomatic" patients is weak especially considering the life-limiting comorbidities.

Current Surgical Management of Pediatric Idiopathic Constipation: A Systematic Review of Published Studies.

OBJECTIVE: Surgery for pediatric idiopathic constipation (IC) is undertaken after failure of bowel management programs. Decisions are influenced by rectal biopsy, transit studies, megacolon/megarectum, degree of soiling/incontinence, and anorectal manometry profile(s). A systematic review of ALL published studies critically evaluates outcomes of surgery for IC. METHODS: MEDLINE (PubMed), Google Scholar, and EMBase were searched for English-language articles only. Studies included (1) peer-review publications with 3 or more patients, and (2) clinical outcomes defined by authors. RESULTS: Forty-five reports (1157 patients) met full inclusion criteria. Only 2 papers were randomized controlled trials. Many had small patient numbers (median n = 16; range: 3-114). Twenty-three described heterogenous populations with variant pathology. Follow-up was short (median = 1.5 years: range: 3 months-14 years). The antegrade continence enema operation (ACE)-[open/laparoscopic assisted, cecostomy, or "left sided" ACE]-was judged as successful in 82% of cases, although high morbidity and reoperations were reported. Colon resection and pull through operations had "good" outcome(s) in 79% of children with 17% reporting significant morbidity and a 10% incidence of revisional surgery. Anal dilatation did not improve outcome(s). Botulinum toxin injection scored equally effective compared to internal sphincter myectomy in short-term follow-up. Permanent colostomy was considered successful in 86% of refractory IC cases. CONCLUSIONS: Surgical management and outcome(s) for pediatric IC are based on low-quality evidence. No single operation was considered "best practice." This study crucially highlights that surgeons must develop better care strategies.

Heterotaxy and intestinal rotation anomalies: 20 years experience at a UK regional paediatric surgery centre.

BACKGROUND: The association of heterotaxy with intestinal rotation anomalies is well described. However debate exists with regard optimal management notably should 'asymptomatic' bowel rotation anomalies undergo operation? The present study therefore sought to determine: (1) the risk(s) of volvulus in patients diagnosed with heterotaxy and (2) define morbidity associated with operation for 'asymptomatic' anomalies in a fragile patient cohort with co-existent congenital heart disease. METHODS: Medical case record reviews of ALL heterotaxy patients born during January 1993-December 2013 and attending a UK paediatric centre were analyzed. RESULTS: Of a total of 92 patients, 16 (17.4%) cases underwent foregut imaging studies. Three examinations were performed in 'symptomatic' patients. Twelve studies reported 'abnormal anatomy' with only five patients undergoing surgical correction. No complication(s) were recorded after Ladd's operation to correct defects. A single fatality occurred within 30 days postoperatively from cardiac failure. In 87 patients in whom Ladd's operation was not undertaken, no single patient developed intestinal volvulus (median length of follow-up 27.2 months, total 446.1 person years). CONCLUSION: This study strongly supports a 'watchful waiting' policy for heterotaxy patients. Many children who ultimately die from heart disease may avoid unnecessary abdominal surgery.

Routine evaluation of vesico-ureteric reflux in children with anorectal malformation does not reduce the rate of urinary tract infection.

OBJECTIVE: Children with anorectal malformations (ARM) have a high rate of renal anomalies and increased risk of urinary tract infection (UTI). We aimed to determine whether using routine Micturating Cystourethrogram (MCUG) to detect VUR is effective in reducing the incidence of UTI or renal scarring in children with ARM. METHODS: A retrospective study of consecutive children diagnosed with ARM in two centres with a minimum of 3 years follow-up was performed, excluding those with cloaca or an MCUG prior to ARM repair. Univariate and multivariate logistic regression analysis was used to determine variables which were associated with VUR, UTI and renal scarring. Associations are described as Odd's Ratio (OR), 95% Confidence Interval. Significance was taken as p<0.05. RESULTS: 344 children were included with a median age of 8 years (IQR 5-11 years). 150 (44%) were female. 89 (26%) had renal anomalies and 101 (29%) had spine anomalies. 148 patients had routine MCUG and VUR was found in 62 (42%) of these children. Univariate analysis did not correlate any of the assessed variables with VUR or renal scarring. However, abnormal renal ultrasound - OR 6.18 (95% CI 2.99-13.07, p 0.0001) was associated with UTI whilst abnormal spine - OR 0.27 (95% CI 0.10-0.62, p 0.009), low ARM - OR 0.30 (CI 0.14-0.63, p 0.006) and intermediate ARM - OR 0.35 (CI 0.17-0.70, p 0.01) were associated with a reduced risk of UTI. On multivariate analysis, only abnormal renal USS retained a significant association with UTI (p<0.0001). CONCLUSIONS: VUR is common in patients with ARM. Children with an abnormal R-USS are at increased risk of UTI. Performing routine MCUG does not reduce the risk of UTI in children with ARM.

Variations in the Detection of Anorectal Anomalies at Birth among European Cities.

INTRODUCTION: The diagnosis of anorectal malformations (ARMs) is made at birth by perineal examination of the newborn, yet small series reported late diagnosis in almost 13%. No large series to date have looked into the magnitude of missed ARM cases in the neonatal period across Europe. This study aimed to define the rate of missed ARM at birth across four United Kingdom and European Union centers. MATERIALS AND METHODS: All ARM cases treated at two United Kingdom tertiary centers in the past 15 years were compared with two tertiary European centers. Demographic and relevant clinical data were collected. Late diagnosis was defined as any diagnosis made after discharge from the birth unit. Factors associated with late diagnosis were explored with descriptive statistics. RESULTS: Across the four centers, 117/1,350, 8.7% were sent home from the birth unit without recognizing the anorectal anomaly. Missed cases showed a slight female predominance (1.3:1), and the majority (113/117, 96.5%) were of the low anomaly with a fistula to the perineum. The rate of missed ARM cases was significantly higher in the United Kingdom centers combined (74/415, 17.8%) compared with those in the European Union (43/935, 4.6%) (p < 0.00001), and this was independent of individual center and year of birth. CONCLUSION: Significant variation exists between the United Kingdom and other European countries in the detection of ARM at birth. We recommend raising the awareness of accurate perineal examination at the time of newborn physical examination. We feel this highlights an urgent need for a national initiative to assess and address the timely diagnosis of ARM in the United Kingdom.

Traumatic pseudoaneurysm of the popliteal artery after blunt trauma: case report and a review of the literature.

Pseudoaneurysms of the popliteal artery following trauma are rare lesions. We present 3 cases of pseudoaneurysms of the popliteal artery (4, 20, and 45 years old respectively) with a delayed presentation, following blunt trauma, presenting over a 3-year period, and a short review of the relevant literature. A delayed pattern of presentation, over 2 years after blunt popliteal trauma, was observed in all patients. They presented with a painful pulsatile mass in the popliteal fossa, and their peripheral pulses were normal. Orthopedic and rheumatologic evaluation findings were negative. Diagnostic evaluation included triplex, arteriography, and computed tomography angiography. A posterior popliteal approach was used, and after aneurysmal excision, reversed great saphenous vein and lesser saphenous vein (1 patient) grafts were used. After 2 to 4 years of follow-up, all grafts remain patent and the patients have fully functional limbs. A literature search revealed 70 cases of pseudoaneurysms of the popliteal artery reported in civilian settings. These lesions should be repaired shortly because their complications (rupture, thromboembolic episodes) carry a high risk for limb dysfunction and amputation. Penetrating or blunt trauma is the main causative factor (62.5%), but iatrogenic trauma accounts for an increasing number of reports (37.5%). Strict follow-up of popliteal trauma is essential. A high level of suspicion and awareness can lead to early diagnosis and treatment of pseudoaneurysms of the popliteal artery and prevent the serious complications associated with these lesions.

Kabuki Syndrome and Anorectal Malformations: Implications for Diagnosis and Treatment.

Kabuki syndrome (KS) is a rare genetic condition characterized by a distinctive facies, intellectual disability, growth delay, and a variety of skeletal, visceral, and other anomalies, including anorectal malformations (ARMs). We present two cases of female patients with KS, diagnosed and successfully managed at our institution, one with a perineal fistula and one with a rectovestibular fistula. Our report, along with a literature review, shows that the syndrome is usually associated with "low" anomalies, with a potential for a good prognosis. Management of the anorectal anomaly in patients with KS is not essentially different from that in other nonsyndromic patients, taking into account the frequent association of the syndrome with serious congenital heart disease, which might affect the decision-making and timing of the stages of anorectal reconstruction. The frequent occurrence of learning and feeding difficulties makes establishment of toilet training and bowel management rather more challenging, requiring the expertise of a multidisciplinary team. The finding of ARMs in female patients with other characteristics of KS, although inconstant, could support the clinical suspicion for the syndrome until genetic confirmation is available, and should alert the physician for the potential of severe cardiac defects.

Inflammatory fibroid polyp: a rare benign tumor of the alimentary tract in children presenting as intussusception-case report and review of literature.

Inflammatory fibroid polyp (IFP) represents a rare cause of gastrointestinal polypoid disease in childhood. Τhe lesion has been described by various names beyond the currently accepted term, including "Vanek's tumour," eosinophilic or submucosal granuloma, gastric fibroma with eosinophilic infiltration, inflammatory pseudotumor, and hemangiopericytoma. The etiopathogenesis and origin of the mesenchymal spindle-shaped cells that comprise the polyp remains enigmatic. Recent studies have shown familial occurrence, expression of platelet-derived growth factor receptor (PDGFRA) and oncogenic PDGFRA mutations in the majority of lesions, suggestive of a neoplastic nature. We present a rare case of a 10-year-old boy with an IFP of the terminal ileum, who presented acutely with intussusception and was treated with a right hemicolectomy. Postoperative course was uneventful and the patient has been asymptomatic during follow-up. Histopathology and immunohistochemical analysis excluded inflammatory myofibroblastic tumor (negative for Alk1, desmin, smooth muscle actin [SMA]), gastrointerstinal stromal tumors (GIST) (negative for CD117) and schwannoma (negative for S100). The lesion was positive for CD34 and faintly for vimentin. Despite the classification of IFPs as a mesenchymal benign neoplasm, in the vast majority of cases, surgical excision alone was curative, and no reports exist of a malignant transformation. A cautious approach with periodic surveillance of the affected children seems reasonable though.

Hemophagocytic lymphohistiocytosis mimicking surgical symptoms and complications: lessons learned from four cases.

OBJECTIVE: Hemophagocytic lymphohistiocytosis (HLH) is a severe immunological disorder that leads to a massive inflammatory reaction that may prove rapidly fatal. We show that HLH may present by masquerading as surgical disease or as a postoperative complication leading to delays in diagnosis and treatment. STUDY DESIGN: A case series of four children with acute surgical presentation and prolonged unexplained postoperative sepsis, who were diagnosed with HLH. RESULTS: Four children with different clinical presentations (1. neonatal abdominal distension, 2. ileostomy closure and Hirschsprung's disease, 3. iatrogenic sigmoid perforation and Crohn's disease, and 4. streptococcal toxic shock syndrome with primary peritonitis) were diagnosed with HLH at our regional pediatric surgical centre in the last two years. All developed signs of prolonged postoperative sepsis with hepatosplenomegaly and pancytopenia, requiring intensive care support. In the absence of explanation for their symptoms and deteriorating clinical condition, a total of six 'negative' exploratory laparotomies were performed. Eventually, HLH was diagnosed with bone marrow aspiration after an average of 23 days (range 17-40), following the finding of significantly elevated ferritin (up to 293150 ng/ml) and triglyceride levels. All children improved with initiation of high-dose steroid treatment followed by etoposide and cyclosporin. CONCLUSION: HLH may rarely present with symptoms and signs of surgical disease or complicate post-operative recovery. This diagnosis should be considered in children with unexplained prolonged fever, hepatosplenomegaly and pancytopenia, especially if associated with high ferritin levels. HLH can prove rapidly fatal without appropriate treatment.

Colon atresia and frontal encephalocele: a rare association.

The association of colonic atresia with craniofacial anomalies has been well described and probably represents a malformative event that occurs in the early embryonal period. We present a case of an infant with colonic atresia and a frontal encephalocele and believe this to be a newly reported association. We review possible pathogenic mechanisms.

Pseudoaneurysm of the popliteal artery complicated by peroneal mononeuropathy in a 4-year-old child: report of a case.

Pseudoaneurysms of the popliteal artery (PPA) rarely occur in children. In fact, we found only 10 cases reported in the medical literature. We report the case of a 4-year-old boy who presented with a painful palpable mass in the right popliteal fossa. He also had mild, painless right foot-drop and difficulty toe-walking on the same side. The diagnosis of a PPA was based on the findings of triplex ultrasound and computed tomographic-angiography. We attributed the cause of the lesion to blunt trauma, which he had suffered 2 years earlier. Thorough preoperative evaluation excluded the possibility of a self-immune process or a bone tumor in the region. Neurological examination demonstrated a mild, isolated, peripheral mononeuropathy of the right peroneal nerve. Thus, we performed surgical repair using an autologous reversed great saphenous vein graft. The patient had an uneventful postoperative course and his peripheral neuropathy and foot-drop resolved completely within 1 month after surgery. Now, after 3 years of follow-up, the patient has a patent graft and a fully functioning limb. PPAs are rare, especially in children, and trauma is the predominating underlying cause. PPAs should be treated immediately after diagnosis because their complications are associated with high rates of functional impairment and even limb loss.