Exploring the clinical characteristics and prevalence of the annular pancreas: a meta-analysis.

BACKGROUND: The annular pancreas (AP) is a rare gastrointestinal congenital malformation, in which malrotation of the pancreatic ventral bud in the seventh week of embryonic development manifests in a partial or complete ring of tissue around the second part of the duodenum. METHODS: The main online medical databases such as PubMed, ScienceDirect, Wiley online library, Web of Science, and EBSCO discovery service were used to gather all relevant studies on the AP. RESULTS: A total of 12,729,118 patients were analyzed in relation to the prevalence of AP. The pooled prevalence of AP was 0.0045% (95% CI: 0.0021%-0.0077%). The most frequent comorbidity in adults and children was duodenal obstruction, with a pooled prevalence of 24.04% and 52.58%, respectively (95% CI: 6.86%-46.48% and 35.56%-69.31%, respectively). The most frequent operation in adult patients with AP was duodenojejunostomy, with pooled prevalence established at 3.62% (95% CI: 0.00%-10.74%). CONCLUSION: The diagnostic complexity of AP is accentuated by its nonspecific clinical symptoms, making accurate identification reliant on imaging studies. Therefore, having a thorough knowledge of the clinical characteristics of the AP and its associated anomalies becomes paramount when faced with this rare congenital condition.

Intestinal malrotation in extremely premature infants: a potential trap.

INTRODUCTION: Intestinal malrotation is life-threatening and often presents during infancy with bilious vomiting. The prevalence and presentation among extremely premature infants are unknown. METHODS: We retrospectively reviewed all infants born at less than 28 weeks' gestation diagnosed with symptomatic intestinal malrotation in a tertiary neonatal intensive care unit over a 10-year period (2010-2020). RESULTS: Seven of 514 (1.4%) extremely premature infants developed symptomatic intestinal malrotation during this period. All were non-syndromic. In comparison, the prevalence of symptomatic intestinal malrotation in 7382 infants ≥ 28 weeks' gestation admitted during the same period was 0.2%. Intestinal malrotation was confirmed at laparotomy in all extremely premature infants and six of seven had midgut volvulus. All but one presented with marked abdominal distension; none had bilious vomiting and only three had bilious gastric aspirates. A subacute onset with non-specific features such as recurrent apnoea and bradycardia, feed intolerance, and intermittent abdominal distension was common. All infants underwent a Ladd procedure. Two required extensive bowel resection resulting in short gut syndrome and three underwent further surgery for adhesive small bowel obstruction. One patient died at 10 months of age from respiratory failure but the others were well 1-3 years later. CONCLUSIONS: Symptomatic intestinal malrotation in extremely premature infants has a relatively high prevalence. It may present with marked abdominal distension without bilious vomiting, demanding a high index of suspicion. An atypical presentation, potential alternative abdominal pathologies, coexisting comorbidities, and concerns about survival in these fragile babies may deter the surgeon despite the opportunity of a good outcome.

The impact of trisomy 21 on epidemiology, management, and outcomes of congenital duodenal obstruction: a population-based study.

PURPOSE: Congenital duodenal obstruction (CDO) is associated with trisomy 21 (T21), or Down's syndrome, in around a third of infants. The aim of this study was to explore the impact of T21 on the epidemiology, management, and outcomes of infants with CDO. METHODS: Data were prospectively collected from specialist neonatal surgical centres in the United Kingdom over a 12 month period from March 2016 using established population-based methodology for all babies with CDO. Infants with T21 were compared to those without any chromosomal anomaly. RESULTS: Of 102 infants with CDO that underwent operative repair, T21 was present in 33 [32% (95% CI 23-41%)] babies. Cardiac anomalies were more common in those with T21 compared to those without a chromosomal anomaly (91 vs 17%, p < 0.001), whereas associated gastrointestinal anomalies were less common in infants with T21 (3 vs 12%, p = 0.03). Surgical management was not influenced by T21. Time to achieve full enteral feed, need for repeat related surgery, and mortality were similar between groups. Infants with T21 had a longer median initial inpatient stay (23 vs 16.5 days, p = 0.02). CONCLUSIONS: Infants with T21 have a higher incidence of cardiac anomalies and a longer initial inpatient stay; however, it does not change CDO management or outcomes. This information is important for prenatal and postnatal counselling of parents of infants with CDO and T21.

Management strategies and treatment results of pediatric choledochal malformations in the Nordic countries.

BACKGROUND: Incidence and long-term outcomes of choledochal malformations (CMs) in children remain unclear. METHODS: Clinical characteristics, operative details, complications, and follow-up data were collected from eight pediatric surgical centers in Sweden, Norway, Denmark, and Finland, which also answered a questionnaire addressing management practices. RESULTS: During 2000-2017, 126 pediatric CMs were diagnosed, corresponding an incidence of 1:37,400. Diagnostic, treatment, and follow-up practices varied markedly. Of patients with complete clinical data (n = 119), 85% and 11% had type I and IV CMs and were managed by open hepaticojejunostomy at median age of 2.5 (interquartile range 0.46-5.8) years. Associated malformations were more common in fusiform and type IV (23%) than cystic CMs (8%, p = 0.043). Pancreaticobiliary maljunction was more frequently confirmed in patients presenting with pancreatitis (26% vs. 7%, p = 0.005) and with fusiform CMs (56% vs. 25%, p = 0.001). Cholangitis/pancreatitis episodes, occurring in 12% during postoperative follow-up of 4.0 (2.0-7.9) years, associated with longer surveillance (OR 1.32, 95% CI 1.13-1.54, p < 0.001). However, only two thirds of centers continued follow-up until adulthood. No malignancies were reported. CONCLUSIONS: CM incidence was higher than traditionally reported among Western populations. Although open hepaticojejunostomy carries good short-term outcomes, long-term morbidity is noteworthy. Standardized evidence-based management strategies and long-term follow-up are encouraged.

Incidence of gastro-intestinal anomalies and surgical outcome of fetuses diagnosed with echogenic bowel and bowel dilatation.

BACKGROUND: We aimed to evaluate the incidence of gastro-intestinal (GI) anomalies and surgical outcome in fetuses diagnosed with either echogenic bowel (EB) or EB plus bowel dilatation (BD) but no associated chromosomal, DNA and/or additional structural defects. METHODS: A 10-year (2008-2018) retrospective review was performed on all fetuses diagnosed with EB and EB+BD (RES-18-0000-072Q). Results are reported as number of cases (%) and mean ±SD. Fisher's exact test, Mann-Whitney U test and logistic regression were used to identify differences between groups and predisposing factors for gastro-intestinal anomalies. RESULTS: We identified 41 fetuses with EB and 14 fetuses with EB+BD. Post-natal surgical intervention was required in no patient of the EB group and in 7/14 (50%) of the EB+BD group, p<0.001. The risk of having a GI anomaly was higher in the EB+BD group (RR 42.0 [2.5-691.6]; p=0.009). Advanced maternal age (p=0.04), ascites (p=0.006) and polyhydramnios (p=0.007) were associated with a higher incidence of GI pathology. CONCLUSIONS: In fetuses with no associated chromosomal, DNA and/or additional structural defects, the finding of EB+BD is associated with 50% incidence of GI anomalies at birth. Advanced maternal age, ascites and polyhydramnios are also associated with higher incidence of GI pathology at birth.

Incidence, spectrum and outcome of congenital anomalies seen in a neonatal intensive care unit in Southern Nigeria.

BACKGROUND: Congenital anomalies (CAs) refer to defects that are present in a newborn but occurred during intrauterine life. They can be due to genetic, modifiable environmental or multifactorial causes. There was no prior report of their burden in our state. AIMS: This study aims to describe the incidence, spectrum, predisposing factors and outcome of CAs in our setting. METHODS: It was a total population study of all neonates with major birth defects admitted into the unit during the study period. Their clinical-demographic features, diagnoses and outcome were entered into an excel sheet. Clinical detection of birth defects was based on standard diagnostic criteria. The data were analysed using IBM SPSS Statistics for Windows, Version 20.0. Armonk, NY: IBM Corp. Patterns and outcome of birth defects were presented as proportions. Selected characteristics were tested for possible association with birth defect using Fisher's exact test. The level of significance was set at P < 0.05. RESULTS: The incidence of major CAs was 4.3/1000 live births. Female neonates were more affected (59.0%). Participants' mean gestational age was 37.7 ± 3.3 weeks. Central nervous system anomalies were the most common (38.5%) birth defects. These were followed by musculoskeletal, body wall and digestive system anomalies: 28.2%, 23.1% and 10.3%, respectively. One-third (33.3%) of the infants had multiple anomalies. Nearly three quarters of them (74.0%) were referred, 18.0% died while 5.0% were discharged alive. CONCLUSION: A wide range of CAs occur in our setting with dire consequences. Provision of relevant specialised multidisciplinary care is desirable. Furthermore, pubic enlightenment on its modifiable possible causes can reduce the burden.

Enteric duplication in children.

INTRODUCTION: Enteric duplication is a congenital anomaly with varied clinical presentation that requires surgical resection for definitive treatment. This had been approached with laparotomy for resection, but has changed with minimally invasive technique. The purpose of our study was to determine the demographics, natural history, operative interventions, and outcomes of pediatric enteric duplication cysts in a contemporary cohort. METHODS: With IRB approval, we performed a retrospective chart review of all patients less than 18 years old treated for enteric duplication between January 2006 and August 2016. Demographics, patient presentation, operative technique, intraoperative findings, hospital course, and follow-up were evaluated. Descriptive statistical analysis was performed; all medians were reported with interquartile range (IQR). RESULTS: Thirty-five patients underwent surgery for enteric duplication, with a median age at surgery of 7 months (2.5-54). Median weight was 7.2 kg (6-20). Most common patient presentations included prenatal diagnosis 37% (n = 13). Thirty-four patients (97%) had their cyst approached via minimally invasive technique (thoracoscopy or laparoscopy) with only three (8%) requiring conversion to an open operation. Median operative time was 85 min (54-133) with 27 (77%) patients requiring bowel resection. Median length of bowel resected was 4.5 cm (3-7). Most common site of duplication was ileocecal (n = 15, 42%). Postoperative median hospital length of stay was 3 days (2-5) and median number of days to regular diet was 3 (1-4). No patients required re-operation during their hospital stay. Median follow-up was 25 days (20-38). CONCLUSION: In our series, most enteric duplication cysts were diagnosed prenatally. These can be managed via minimally invasive technique with minimal short-term complications, even in neonates and infants.

Screening and Treatment of Intestinal Rotational Abnormalities in Heterotaxy: A Systematic Review and Meta-Analysis.

OBJECTIVE: To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities (IRAs) in asymptomatic patients with heterotaxy. STUDY DESIGN: PubMed, Embase, and Cinahl were searched electronically to determine the overall incidence of IRAs in heterotaxy; the detection rate of IRAs associated with screening; the incidence of midgut volvulus in patients without screening; and the incidence of morbidity and mortality after prophylactic and emergency Ladd procedures. Relevant data were computed with a meta-analysis of proportions. Between-study heterogeneity was assessed with the I(2) statistic. RESULTS: From 276 papers identified, 24 studies with a total of 1433 patients with heterotaxy were included for systematic review. No randomized study was identified. True incidence of IRA in heterotaxy could not be ascertained through meta-analysis. In patients who underwent screening, the incidence of IRA was 58%. Acute midgut volvulus occurred in 5.8% of those who did not undergo screening. Postoperative mortality after Ladd procedure mainly was associated with cardiac insufficiency, and overall it was significantly greater in the emergency group compared with the prophylactic group (18% vs 5.6%). The complication rate also was greater in case of emergency vs prophylactic abdominal surgery (27% vs 16%); adhesional small bowel obstruction was the most common complication overall (6%). CONCLUSION: The screen-detected incidences of IRA and acute midgut volvulus were significantly greater in heterotaxy than the normal population. Prophylactic Ladd procedure was associated with less morbidity and mortality compared with emergency surgery. A long-term prospective randomized trial is needed to define the indication for screening and prophylactic treatment of IRA in heterotaxy.

Outcome of fetal gastro-intestinal cysts: a systematic review and meta-analysis.

OBJECTIVES: To explore the outcome of fetal gastrointestinal (GI) cysts and to ascertain the detection rate of prenatal ultrasound in identifying these anomalies. METHODS: Medline and Embase databases were searched. The outcomes explored were: resolution of the cyst, additional GI anomalies detected only at birth, clinical symptoms, need for surgery, post-surgical complications and diagnostic accuracy. Meta-analyses of proportions and hierarchical summary receiver operating characteristics (HSROC) model were used to analyse the data. RESULTS: Ten studies were included; 27.0% (95% CI 2.6-64.4) of the cysts resolved either pre or post-natally. Additional GI anomalies were detected in 6.0% (95% CI 1.1-14.7). Clinical symptoms occurred in 31.1% (95% CI 14.9-50.2), while 50.6% (95% CI 10.0-90.8) had surgery. Post-surgical complications occurred in 6.1% (95% CI 0.2-19.6). Overall detection rate of prenatal ultrasound in correctly identifying GI cysts was good (sensitivity: 94.5%, 95% CI: 39.1-99.8; specificity: 97.7%, 95% CI 89.9-99.5). CONCLUSION: GI cysts are usually benign. Clinical symptoms occur in approximately one third of children. About one third of the cysts resolves, while the rate of complications after surgery is low. Prenatal ultrasound has an overall good diagnostic accuracy in identifying these anomalies. © 2016 John Wiley & Sons, Ltd.

Open versus laparoscopic approach for intestinal malrotation in infants and children: a systematic review and meta-analysis.

PURPOSE: Although the principles of the Ladd's procedure for intestinal malrotation in children have remained unchanged since its first description, in the era of minimally invasive surgery it is controversial whether laparoscopy is advantageous over open surgery. The aim of our study was to determine whether the surgical approach for the treatment of malrotation had an impact on patient outcome. METHODS: Using a defined strategy (PubMed, Cochrane, Embase and Web of Science MeSH headings), two investigators independently searched for studies comparing open versus laparoscopic Ladd's procedure in children. Case reports and opinion articles were excluded. Outcome measures included age at operation, time to full enteral feeding, length of hospital stay, and post-operative complications. Maneuvers were compared using Fisher's exact test and meta-analysis was conducted using RevMan 5.3. Data are expressed as mean ± SD. RESULTS: Of 308 abstracts screened, 49 full-text articles were analyzed and nine (all retrospective) met our search criteria. Selected articles included 1003 patients, of whom 744 (74 %) underwent open surgery and 259 (26 %) laparoscopy. Patients who had open surgery were younger (0.9 ± 1.2 years) than those who underwent laparoscopy (2.6 ± 3 years; p < 0.0001). Laparoscopy was converted to open Ladd's in 25.3 % patients. Laparoscopy was associated with faster full enteral feeding (1.5 ± 0.3 days) in comparison to open surgery (4.6 ± 0.1 days, p < 0.0001). Length of hospital stay was shorter in the laparoscopic group (5.9 ± 4.3 days) than in the open group (11.2 ± 6.7 days; p < 0.0001). Open surgery was associated with higher overall post-operative complication rate (21 %) than laparoscopy (8 %; p < 0.0001). Although there was no difference in the prevalence of post-operative bowel obstruction (open, n = 10 %; laparoscopy, n = 0 % p = 0.07), post-operative volvulus was more frequent in the laparoscopy group (3.5 %) than in the open group (1.4 %, p = 0.04). CONCLUSION: Comparative but non-randomized studies indicate that laparoscopic Ladd's procedure is not commonly performed in young children. Although one third of laparoscopic procedures is converted to open surgery, laparoscopy is associated with shorter time to full enteral feeds and length of hospital stay. However, laparoscopic Ladd's procedure seems to have higher incidence of post-operative volvulus. Prospective randomized studies with long follow-up are needed to confirm present outcome data and determine the safety and effectiveness of the laparoscopic approach.

Current surgical management of intestinal rotational abnormalities.

PURPOSE OF REVIEW: At present, there is a debate as to the management of malrotation in pediatric patients. This review highlights recent literature including the role of laparoscopy, and the management of asymptomatic patients with and without congenital cardiac disease. RECENT FINDINGS: Symptomatic patients are still recommended to undergo open Ladd's procedure. Laparoscopic Ladd's procedure in asymptomatic patients confers a shorter time to tolerating feeds, shorter length of stay, and a decreased rate of bowel obstruction and other complications. It may, however, carry a higher risk of postoperative volvulus. Diagnostic laparoscopy is recommended in asymptomatic patients in whom the diagnosis is uncertain on upper gastrointestinal imaging. Asymptomatic patients with congenital heart disease may be managed with initial observation or operation, based upon individualized risk benefit assessment. If an elective Ladd's procedure is performed, it should take place after palliative cardiac operations have resulted in stable cardiac function. Screening upper gastrointestinal studies in heterotaxy patients may be unnecessary as the reported rates of volvulus in recent studies are low. SUMMARY: Laparoscopic Ladd's procedure may be an acceptable alternative to an open procedure for asymptomatic patients. Observation of asymptomatic patients with congenital cardiac disease is a reasonable alternative in selected patients.

Anatomic variants of the pancreatic duct and their clinical relevance: an MR-guided study in the general population.

OBJECTIVES: To investigate the frequency of pancreatic duct (PD) variants and their effect on pancreatic exocrine function in a population-based study using non-invasive secretin-stimulated magnetic resonance cholangiopancreatography (sMRCP). METHODS: Nine hundred and ninety-five volunteers, 457 women and 538 men, aged 51.9 ± 13.4 years, underwent navigator-triggered, T2-weighted, 3D turbo spin echo MRCP on a 1.5 T system after 1 unit/kg secretin administration. Two readers evaluated images for PD variants. Pancreatic exocrine function and morphological signs of chronic pancreatitis such as abnormalities of the main PD, side branch dilatation, and pancreatic cysts were evaluated and related to PD variants using a Kruskal-Wallis test and post hoc analysis. RESULTS: Of all sMRCP, 93.2% were of diagnostic quality. Interobserver reliability for detection of PD variants was found to be kappa 0.752 (95 %CI, 0.733 - 0.771). Normal PD variants were observed in 90.4% (n = 838/927). Variants of pancreas divisum was identified in 9.6% (n = 89/927). Abnormalities of the main PD, side branch dilatation, and pancreatic cysts were observed in 2.4%, 16.6%, and 27.7%, respectively, and were not significantly different between pancreas divisum and non-divisum group (P = 0.122; P = 0.152; P = 0.741). There was no association between PD variants and pancreatic exocrine function (P = 0.367). CONCLUSION: PD variants including pancreas divisum are not associated with morphological signs of chronic pancreatitis or restriction of pancreatic exocrine function. KEY POINTS: MRCP allows the evaluation of pancreatic duct variants and morphological change. Pancreatic duct variants are not associated with morphological signs of chronic pancreatitis. Pancreas divisum is not accompanied by restriction of pancreatic exocrine function. Pancreatic duct variants including pancreas divisum are limited in their clinical relevance.

Pancreas divisum: a common developmental variant that deserves attention in preclinical medical education.

Clinical literature indicates that pancreas divisum (PD) is present in 3-22% of the population and may be associated with an increased risk of pancreatitis. PD is a developmental variant wherein the duct systems derived from the dorsal and ventral pancreatic buds are not fused. Hence secretions from the head, neck, body, and tail, which develop from the dorsal bud, must pass through the minor duodenal papilla. The smaller uncinate process, derived from the ventral bud, drains through the major duodenal papilla. The purpose of this study was: (1) to do a cadaveric dissection to confirm whether PD is common in donors who had not been selected because they had pancreatitis and (2) to determine the frequency of PD descriptions in anatomy, embryology, pathology, and surgery books in our libraries. For our anatomical study, pancreata of eight human donors were dissected. Dye was injected into the ducts so that any communications between main and accessory ducts could be easily located. For our literature review, 22 anatomy, 14 embryology, 11 pathology, and 26 surgery books were examined for mention of PD. PD was unambiguously identified in two donor cadavers. However, only 14% of the anatomy plus embryology books compared to 70% of the surgery plus pathology books describe PD. Cadaveric dissection confirms that PD is indeed prevalent. The prevalence of PD with its increased risk of pancreatitis merits inclusion of this topic in textbooks of anatomy and embryology.

Investigation of the retrorenal colon frequency using computed tomography in patients with advanced scoliosis.

PURPOSE: The aim of this study is to investigate the frequency of retrorenal colon in patients with advanced scoliosis. MATERIALS AND METHODS: The existence of retrorenal colon was retrospectively investigated in 550 patients with vertebral scoliosis who had undergone abdominal CT scans at our institution between January 2008 and March 2012. The investigation was also carried out on a control group of 200 patients without scoliosis. RESULTS: Among the 550 patients with scoliosis, 100 patients had advanced scoliosis necessitating treatment. Among these 100 patients with advanced scoliosis, retrorenal colon was detected in a total of 25 patients (25 %). The variation was observed on the right side in eight patients (two males, six females) (8 %), on the left side in 15 patients (five males, ten females) (15 %), and bilaterally in two patients (both females) (2 %). In the control group consisting of 200 individuals, retrorenal colon was detected in seven subjects (3.5 %), among which six were on the left and one was on the right. The difference between the incidence of retrorenal colon observed in the patients with advanced scoliosis and those without scoliosis was found to be statistically significant (p < 0.001). CONCLUSION: Since the frequency of retrorenal colon in patients with advanced scoliosis is significantly higher than the control group without scoliosis, the risk of experiencing complications during renal interventions including renal biopsy is also higher. Therefore, these patients should undergo a detailed CT examination before these procedures, and renal interventions should be planned according to findings.

Prevalence of Mental Health Disorders in Individuals With Gastrointestinal Congenital Surgical Anomalies: A Systematic Review and Meta-analysis.

PURPOSE: Studies examining functional outcomes and health-related quality of life for patients with congenital surgical anomalies (CSA) are increasingly common. However, the prevalence of mental health disorders in this population has not been determined. The purpose of this review is to summarize the reported prevalence of mental health disorders in children born with gastrointestinal CSA. METHODS: A systematic review of the literature was conducted on Medline (OVID), EMBASE (OVID), CINAHL (EbscoHost), and Scopus to identify studies reporting mental health diagnoses in children with a gastrointestinal CSA. A meta-analysis of the prevalence of anxiety disorders and depressive disorders was completed. RESULTS: Of 2546 manuscripts, seven met the inclusion criteria. All included manuscripts looked at patients with anorectal malformation (ARM) and/or Hirschsprung disease (HD). No studies assessed other gastrointestinal CSAs. A total of 183 psychiatric diagnoses were reported in 1167 patients. A pooled analysis of ARM patients revealed a 14.0% prevalence of anxiety disorders (N = 208; CI95 = 4.4-23.4%) and an 18.75% prevalence of depressive disorders (N = 48; CI95 = 7.7-29.8%). A pooled analysis of HD patients revealed a 15.8% prevalence of anxiety disorders (N = 19; CI95 = 0.6-32.2%) and a 4.75% prevalence of depressive disorders (N = 758; CI95 = 3.23-6.26%). CONCLUSIONS: There is a paucity of evidence on mental health outcomes of individuals born with gastrointestinal CSA. However, the available evidence suggested a high prevalence of mental health diagnoses in individuals born with ARM or HD with 1 in 7 having an anxiety disorder. Additionally, 1 in 5 ARM patients and 1 in 20 HD patients were diagnosed with a depressive disorder. TYPE OF STUDY: Systematic review and meta-analysis. LEVEL OF EVIDENCE: IV.

Anomalies associated with anorectal malformations.

BACKGROUND: The purpose of the paper is to review the incidence of associated congenital anomalies that are encountered in patients presenting anorectal malformations and compare these results with those previously published. MATERIAL AND METHODS: A number of 50 cases with ARM from our institution were reviewed (from 2005 to 2012) and information was collected on patient demographics, type of ARM and associated congenital anomalies, the latter being then categorized according to organ systems. RESULTS: Out of 50 newborns, 28 were males and 22 females (1.27:1). 34 (68%) had at least one associated abnormality. The majority of patients (40%) had imperforated anus without fistula. The most frequent seen anomalies were gastrointestinal (36%), urogenital (24%) and cardiovascular (16%). CONCLUSIONS: More than half of the children included in our series have other associated abnormalities. We found gastrointestinal anomalies to be the most common associated congenital defects in our patients. A higher incidence of this type of anomalies was encountered in newborns with persistent cloacal anomaly. The rectovestibular fistula group was most likely to present cardiac abnormalities. The incidence of genitourinary anomalies in the perineal fistula group is higher than the one described in other studies.

Outcomes After Ladd Procedures for Intestinal Malrotation in Newborns with Heterotaxy Syndrome.

PURPOSE: Intestinal malrotation may be asymptomatic in patients with heterotaxy syndrome (HS), and whether these newborns benefit from prophylactic Ladd procedures is unknown. This study sought to uncover nationwide outcomes of newborns with HS receiving Ladd procedures. METHODS: Newborns with malrotation were identified from the Nationwide Readmission Database (2010-2014) and stratified into those with and without HS utilizing ICD-9CM codes for situs inversus (759.3), asplenia or polysplenia (759.0), and/or dextrocardia (746.87). Outcomes were analyzed using standard statistical tests. RESULTS: 4797 newborns with malrotation were identified, of which 16% had HS. Ladd procedures were performed in 70% overall and more common in those without heterotaxy (73% vs. 56% HS). Ladd procedures in newborns with heterotaxy were associated with higher complications compared to those without HS including surgical site reopening (8% vs. 1%), sepsis (9% vs. 2%), infections (19% vs. 11%), venous thrombosis (9% vs. 1%), and prolonged mechanical ventilation (39% vs. 22%), all p < 0.001. HS newborns were less frequently readmitted with bowel obstructions (0% vs. 4% without HS, p < 0.001) with no readmissions for volvulus in either group. CONCLUSION: Ladd procedures in newborns with heterotaxy were associated with increased complications and cost without differences in rates of volvulus and bowel obstruction on readmission. TYPE OF STUDY: Retrospective Comparative. LEVEL OF EVIDENCE: III.

Congenital non-central nervous system malformations in cerebral palsy: a distinct subset?

AIM: The aim of this article was to identify and contrast the subset of children with cerebral palsy (CP) and non-central nervous system (CNS) congenital malformations with children with CP but no coexisting non-CNS congenital malformations. METHOD: A population-based regional comprehensive CP registry was used to identify children with CP who had non-CNS congenital malformations (n = 34; 19 males, 15 females; 22 classified as Gross Motor Function Classification System [GMFCS] levels I-III, 12 as GMFCS level IV or V). Their clinical features were then compared with other children with CP without non-CNS congenital malformations (n = 207; 115 males, 92 females; 138 classified as GMFCS levels I-III, 69 as GMFCS level IV or V). RESULTS: Children with CP and non-CNS congenital malformations did not differ from those without in terms of neurological subtype distribution or functional severity, as measured by the GMFCS. Also, there was no association with previous maternal infections (i.e. toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus 2 [TORCH]), maternal fever, use of illicit substances, asphyxia, neonatal encephalopathy, intraventricular haemorrhage, or septicaemia. The incidence of comorbidities such as convulsions, communication difficulties, gavage feeding, cortical blindness, and auditory impairment was not higher in this subgroup. INTERPRETATION: The incidence of congenital non-CNS malformations among children with CP is appreciable. Children with these non-CNS malformations do not appear to differ from other children with CP regarding neurological subtype, functional severity, and comorbidities, or maternal or obstetrical factors. Thus, the specific presence of a non-CNS congenital malformation does not appear to assist the practitioner in the management or understanding of a child's CP.

Role of mini-invasive surgery in the treatment of enteric duplications in paediatric age: a survey of 15 years.

BACKGROUND: Gastrointestinal tract duplications (ATD) are rare malformations that occur with an incidence of 1 in 4000-5000 live births, with heterogeneous clinical pictures in relation to the different location and size. The purpose of this study was to analyze the role of minimally invasive surgery in the management of ATDs, through a critical analysys of 15 years of experience of the Department of Paediatric Surgery, University of Bologna. METHODS: The medical records of 22 children diagnosed with ATDs were retrospectively reviewed (January 1995-August 2010). The study analyses: clinical presentation, preoperative diagnosis, site, anatomic type, treatment, and outcome. RESULTS: Children were 16 males and 6 females, with age ranged from 1 day to 10 years. 20 ATDs (91%) were cystic type, while 2 cases (9%) were tubular one. During the first period of our experience, 10 (45.5%) cases were approached with an open surgery. Then subsequent 8 (36.4%) cases were treated with a diagnostic laparoscopy. This approach permitted also to perform a minilaparotomy, close to the site of the malformation, with a short length of the scar. In 2 cases (9%), we realized an ileal resection with end-to-end anastomosis with a trans-umbilical video-assisted procedure. In 2 cases (9%), we performed a complete removal of the lesions, after complete ligation of the vascular pedicle through a laparoscopic approach. 10 cases (45.5%) were located in the ileum, 6 cases (27.3%) were esophageal duplication, 3 cases (13.6%) were gastric duplications, 2 cases (9%) were located in the colon-rectum. The postoperative course was uneventful in all the cases. DISCUSSIONS AND CONCLUSIONS: this study shows how, in the management of intestinal duplications, a mininvasive approach, is increasingly taking the field, along with increasing the "learning curve" with laparoscopy. In experienced hands, the laparoscopic approach allows an accurate definition of the exact site of duplication and a miniinvasive treatment with similar principles of open techniques.

Should all babies with oesophageal atresia have routine screening for midgut malrotation anomalies? A systematic review in search of evidence.

Background/Purpose Oesophageal Atresia (OA) is associated with co-existent anomalies. There is a controversy of literature pertaining to the risk (s) of intestinal malrotation. In order to guide management we critically evaluate the incidence of IM anomalies in OA newborns. Design MEDLINE and EMBASE databases were searched using keywords "(O)Esophageal Atresia and Malrotation/Associated Abnormalities/Associated Anomalies". Full texts of articles were screened if manuscripts exclusively reported patients with OA malrotation and/or associated anomalies. Larger case series (> 10patients) were included if abstract (s) showed that associated anomalies were systematically assessed. Full eligibility criteria required at least one case of malrotation in an OA index case. Data were collected on article type, number of patients and method (s) of diagnosis. Results 632 abstracts were screened of which 158 papers were analysed based on inclusion criteria-30 manuscripts documented the incidence (%) of malrotation. Incidence rate (s) were 0.5-13%. Malrotation was observed to have a higher incidence (10-44%) in OA babies with other gastrointestinal anomalies (VACTERL). Conclusion Newborns with OA appear to be at a higher risk (%) of having intestinal malrotation anomalies than healthy babies. Prospective studies are required to accurately quantify and define the ' true incidence ' of this association. Given the potential lethal consequences of midgut volvulus screening may be justified in OA babies. Consensus guidelines (DELPHI) exploring surgeons attitudes with regards management of ' asymptomatic malrotation ' disorders in OA newborns may further guide best practice.