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Duodenal atresia

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https://www.readbyqxmd.com/read/29851770/total-bile-acid-concentration-in-duodenal-fluid-is-a-useful-preoperative-screening-marker-to-rule-out-biliary-atresia
#1
Tomoya Fukuoka, Kazuhiko Bessho, Makiko Tachibana, Yoshinori Satomura, Akiko Konishi, Kie Yasuda, Takeshi Kimura, Yasuhiro Hasegawa, Takehisa Ueno, Yoko Miyoshi, Keiichi Ozono
OBJECTIVES: Duodenal tube test (DTT) is used as a preoperative screening to rule out biliary atresia (BA). In previous reports, DTT was assessed by the color of the duodenal fluid, but there were no quantitative criteria. The aim of this study was to examine the efficacy of DTT based on the total bile acid (TBA) concentration in duodenal fluid. METHODS: This is a single-center retrospective study of infants with cholestasis who underwent DTT from 2008 to 2016 at the Osaka University Hospital...
May 30, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29715697/epidemiology-and-outcome-of-major-congenital-malformations-in-a-large-german-county
#2
Boris Wittekindt, Rolf Schloesser, Nora Doberschuetz, Emilia Salzmann-Manrique, Jasmin Grossmann, Bjoern Misselwitz, Udo Rolle
INTRODUCTION:  Congenital malformations are associated with substantial neonatal morbidity and mortality. Furthermore, only sparse data are available on the modalities of care provided to and the associated clinical outcomes in affected neonates. In this study, we focused on five malformations that require surgery during the neonatal period: duodenal stenosis and atresia (DA), gastroschisis (GA), omphalocele (OM), congenital diaphragmatic herniation (CDH), and esophageal atresia (EA)...
May 1, 2018: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29707395/fetal-sirenomelia-associated-with-an-abdominal-cyst-originating-from-a-saccular-cloaca
#3
Yui Kinjo, Hitoshi Masamoto, Hayase Nitta, Tadatsugu Kinjo, Tomoko Tamaki, Naoki Yoshimi, Yoichi Aoki
A 40-year-old pregnant woman presented with a fetal abdominal cyst and oligohydramnios. Color Doppler scan revealed a single blood vessel from the fetal aorta into a single umbilical artery. Severe oligohydramnios limited ultrasonographic evaluation of the fetal lower limbs, kidneys, or bladder. The pregnancy was terminated; the fetus showed fused lower limbs, bulging abdomen, and absent external genitalia and was diagnosed with type III sirenomelia. On autopsy, no normal bladder was observed, but duodenal atresia, anorectal atresia, and right renal agenesis were found...
2018: Case Reports in Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29704686/a-novel-nkx3-2-mutation-associated-with-perinatal-lethal-phenotype-of-spondylo-megaepiphyseal-metaphyseal-dysplasia-in-a-neonate
#4
Pelin Ozlem Simsek-Kiper, Can Kosukcu, Ozlem Akgun-Dogan, Rahsan Gocmen, Gulen Eda Utine, Tutku Soyer, Ayse Korkmaz-Toygar, Gen Nishimura, Mehmet Alikasifoglu, Koray Boduroglu
Spondylo-megaepiphyseal-metaphyseal dysplasia (SMMD) is an autosomal recessive skeletal dysplasia, characterized by disproportionate short stature with a short and stiff neck and trunk. SMMD is caused by inactivating mutations in NKX3-2, which encodes a homeobox-containing protein. Because of the rarity of the disorder, the diagnostic feature has not been fully established yet. We describe an affected newborn with dysmorphic facial features and severe short trunk. The patient required immediate intubation at the delivery room and duodenal atresia was detected during his course in neonatal intensive care unit...
April 25, 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29670869/pathogenetic-and-prognostic-factors-for-neonatal-gastric-perforation-personal-experience-and-systematic-review-of-the-literature
#5
Chiara Iacusso, Alessandro Boscarelli, Fabio Fusaro, Pietro Bagolan, Francesco Morini
Introduction: Neonatal gastric perforation (NGP) is a rare entity. Our aim was to report our experience and review the recent literature to characterize NGP, describe associated factors, and define prognostic factors. Materials and methods: Retrospective review of all consecutive patients with NGP treated between June 2009 and December 2017 in a third level pediatric hospital. In addition, a systematic review of Medline and Scopus database was performed using a defined strategy...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29666202/nutritional-role-of-amniotic-fluid-clues-from-infants-with-congenital-obstruction-of-the-digestive-tract
#6
Nigel J Hall, Melanie Drewett, David Burge
AIMS: To investigate the role played by amniotic fluid in late fetal nutrition by analysis of infants born with digestive tract atresia. METHODS: Birth weight (BW), gestational age and gender of infants born with oesophageal (OA), duodenal (DA), jejunal (JA) and ileal atresia (IA) were recorded and BW Z-scores compared. Infants with incomplete obstruction (stenosis), chromosomal or syndromic conditions and multiple congenital malformations were excluded. Term infants admitted with suspected postnatal intestinal obstruction in whom no congenital malformation was found were used as a control group...
April 17, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29641367/the-case-for-simulation-based-mastery-learning-education-courses-for-practicing-surgeons
#7
Lauren M Baumann, Katherine A Barsness
INTRODUCTION: Pediatric surgeons rely on simulation courses to develop skills for safe minimally invasive repair of complex congenital anomalies. The majority of minimally invasive surgery (MIS) training courses occur during short "exposure courses" at annual conferences. Little data are available to support the benefit of these courses relative to the safe implementation of new skills. The purpose of this article is to determine the impact of an exposure course for advanced neonatal MIS on self-perceived comfort levels with independent performance of advanced MISs...
March 12, 2018: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/29621304/trends-in-congenital-anomalies-in-europe-from-1980-to-2012
#8
Joan K Morris, Anna L Springett, Ruth Greenlees, Maria Loane, Marie-Claude Addor, Larraitz Arriola, Ingeborg Barisic, Jorieke E H Bergman, Melinda Csaky-Szunyogh, Carlos Dias, Elizabeth S Draper, Ester Garne, Miriam Gatt, Babak Khoshnood, Kari Klungsoyr, Catherine Lynch, Robert McDonnell, Vera Nelen, Amanda J Neville, Mary O'Mahony, Anna Pierini, Annette Queisser-Luft, Hanitra Randrianaivo, Judith Rankin, Anke Rissmann, Jennifer Kurinczuk, David Tucker, Christine Verellen-Dumoulin, Diana Wellesley, Helen Dolk
BACKGROUND: Surveillance of congenital anomalies is important to identify potential teratogens. METHODS: This study analysed the prevalence of 61 congenital anomaly subgroups (excluding chromosomal) in 25 population-based EUROCAT registries (1980-2012). Live births, fetal deaths and terminations of pregnancy for fetal anomaly were analysed with multilevel random-effects Poisson regression models. RESULTS: Seventeen anomaly subgroups had statistically significant trends from 2003-2012; 12 increasing and 5 decreasing...
2018: PloS One
https://www.readbyqxmd.com/read/29609227/-characteristics-and-prognosis-of-interrupted-inferior-vena-cava-with-azygous-continuation
#9
Q Xu, H X Sun, J S Xie, J L Wang, Q Y Pei, X H Zhang
Objective: To explore the clinical characteristics of interrupted of the inferior vena cava with azygous continuation and the prognosis. Methods: Retrospective analysis of 21 fetuses diagnosed with interrupted inferior vena cava with azygous continuation among 28 567 pregnant women who underwent routine ultrasound scan. The clinical data, ultrasonographic features, genetic information and prognosis were collected. Results: Interrupted of the inferior vena cava with azygous continuation occurred in 21(0.07%, 21/28 567) of 28 567 patients...
March 25, 2018: Zhonghua Fu Chan Ke za Zhi
https://www.readbyqxmd.com/read/29479560/atypical-perioperative-management-for-duodenal-obstruction-in-an-infant-with-heterotaxy-syndrome-a-case-report
#10
Hirofumi Obinata, Shinichi Nishibe, Yoko Ishihara
Background: Heterotaxy syndrome (HS) is characterized by a wide variety of cardiac and extra-cardiac malformations, including pulmonary valve stenosis, interruption of the inferior vena cava, total anomalous pulmonary venous connection (TAPVC), asplenia, polysplenia, intestinal malrotation, and preduodenal portal vein (PDPV). We report the case of a heterotaxic infant with an infracardiac TAPVC and preduodenal portal vein who experienced repetitive hemodynamic instability during urgent laparotomy for duodenal obstruction...
2018: JA Clinical Reports
https://www.readbyqxmd.com/read/29351932/megaduodenum-in-a-59-year-old-man-a-very-late-postoperative-complication-after-duodenal-atresia
#11
Jessica Rueff, Oliver Söllner, Markus Zuber, Benjamin Weixler
Intestinal malformations are common defects of the newborn, treated in experienced centres. Reports on long-term follow-up and associated complications are scarce, possibly leading to misinterpretation of clinical signs and symptoms in adulthood. To prevent treatment errors, it is important that physicians are aware of long-term complications of intestinal malformations.
January 18, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29317564/pdx1-gene-mutation-with-permanent-neonatal-diabetes-mellitus-with-annular-pancreas-duodenal-atresia-hypoplastic-gall-bladder-and-exocrine-pancreatic-insufficiency
#12
Abhishek Kulkarni, Varun K Sharma, Fazal Nabi
BACKGROUND: Neonatal diabetes mellitus is a rare condition. CASE CHARACTERISTICS: A small for gestational age male, presented with neonatal onset diabetes mellitus, duodenal atresia, annular pancreas and gall bladder hypoplasia. OBSERVATION: Observation: A novel homozygous mutation p.K163R (c.488A>G) in the PDX1 gene was found. Parents were heterozygous for the same. MESSAGE: This case highlights the importance of establishing the genetic diagnosis in all cases of neonatal diabetes mellitus...
December 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/29049228/the-efficiency-of-sonography-in-diagnosing-volvulus-in-neonates-with-suspected-intestinal-malrotation
#13
Wenhua Zhang, Hongjun Sun, Fangqiong Luo
This study is to prospectively evaluate the efficiency of sonography for volvulus diagnosis in neonates with clinically suspected intestinal malrotation.A total of 83 patients with suspected intestinal malrotation who underwent detailed abdominal sonography and upper gastrointestinal contrast study were included. Malrotation was characterized by inversion of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) in sonographic examination. The "whirlpool sign" of Color Doppler Sonography was recognized as a characteristic for malrotation with volvulus...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29030699/prematurity-and-biliary-atresia-a-30-year-observational-study
#14
Natalie Durkin, Maesha Deheragoda, Mark Davenport
AIM OF STUDY: The diagnosis of biliary atresia (BA) remains challenging and delay can lead to significant morbidity with time to surgery a key factor in determining outcome. Prematurity may impact on outcome potentially delaying diagnosis. We sought to assess whether the premature BA infants (PBA) have a delayed time to surgery and as such, worse outcomes? METHODS: Review of a single-centre prospectively maintained database. Prematurity was defined as delivery < 37/40 gestation...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29018788/transition-from-laparotomy-to-laparoscopic-repair-of-congenital-duodenal-obstruction-in-neonates-our-early-experience
#15
Min Jeng Cho, Dae Yeon Kim, Seong Chul Kim, Jung Man Namgoong
BACKGROUND: The aim of this report was to review our early experience of the last 7 years with repairs of congenital duodenal obstruction (CDO) to determine the efficacy and outcomes of laparoscopic repairs compared to laparotomy. METHODS: A retrospective review was conducted on all neonate (<30 days) with CDO between 2009 and 2015. Patients with duodenal atresia, stenosis, web, and annular pancreas were included. Patients with only malrotation or delayed presentation were excluded...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28919320/is-selective-echocardiography-in-duodenal-atresia-the-future-standard-of-care
#16
Abid Khan, Sharman Tan Tanny, Elizabeth J Perkins, Rodney W Hunt, John M Hutson, Sebastian K King, Bryn Jones, Warwick J Teague
BACKGROUND: Duodenal atresia (DA) is associated with cardiac defects that may have perioperative care implications. Standard preoperative care includes echocardiography to identify such cardiac defects, but this dogma has been challenged. We aimed to assess selective and selective strategies for preoperative echocardiography in DA patients. METHODS: Single-center retrospective review of neonates with DA over a 16-year period was performed. Data included preoperative cardiovascular and respiratory examination, chest x-ray, and echocardiography...
December 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28880692/clinical-outcome-of-pregnancies-with-the-prenatal-double-bubble-sign-a-five-year-experience-from-one-single-centre-in-mainland-china
#17
Yu Yang, Ping He, Dong-Zhi Li
The aim of this study was to describe the risk of aneuploidy, associated structural anomalies and clinical outcome in pregnancies with the prenatal double bubble sign. A retrospective study on ultrasound reports and pregnancy outcomes was performed in 71 foetuses with double bubble sign, who were examined at the Guangzhou Women and Children Medical Center during a five-year period. Forty-nine patients had the regular prenatal care since first trimester; of these, 20 had the sonographic sign before 24 weeks and the remaining 29 had the sonographic diagnosis after 24 weeks...
February 2018: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28876575/neonatal-surgery-a-study-of-two-years-at-nelson-mandela-academic-hospital-mthatha-eastern-cape
#18
A Delgado, A Cejas, D Bangasa
BACKGROUND: Advances in diagnostic techniques and perioperative care have greatly improved the outcome of neonatal surgery. Despite this, disparity still exists in the outcome of neonatal surgery between developed and developing countries. METHOD: We performed a prospective study of neonates admitted and treated due to surgical congenital diseases and other conditions in our hospital from April 2015 to April 2017. RESULTS: There were 19 (28,7%) females and 47 (70,3%) males in this group...
September 2017: South African Journal of Surgery. Suid-Afrikaanse Tydskrif Vir Chirurgie
https://www.readbyqxmd.com/read/28855988/intestinal-atresia-following-chemotherapy-presenting-as-superior-mesenteric-artery-syndrome-a-case-report
#19
Xing Wang, Jian-Zhong Li, Ying-Hui Yang, Xiao-Li Huang, Yu Wang, Bin Wu
Multi-agent chemotherapy is recognized as the most common and effective treatment for Burkitt lymphoma, and intestinal mucosal injury is a common gastrointestinal complication following intensive chemotherapy. The aim of the present study was to describe a case of non-Hodgkin lymphoma with intestinal obstruction after chemotherapy in a young adult. The patient presented with aggravated vomiting during the second session of chemotherapy, which was initially attributed to superior mesenteric artery syndrome. However, following surgical intervention, the symptom was proven to be due to extreme intestinal stenosis in the ascending part of the duodenum...
October 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28770132/congenital-duodenal-obstruction-situs-inversus-totalis-and-gastric-perforation-in-a-neonate
#20
Rajat Piplani, Samir K Acharya, Deepak Bagga
We report a rare case of incomplete congenital duodenal obstruction (Type 1 duodenal atresia) in association with situs inversus totalis presenting with gastric perforation in a neonate. The infantogram was suggestive of perforation with air under diaphragm along with dextrocardia. On exploration, a pin point perforation at fundus near lesser curvature along with situs inversus was noted. Primary closure of gastric perforation was done. Patient was then discharged on full breast feeds but was readmitted with intolerance to feeds and recurrent bilious vomiting...
April 2017: Journal of Neonatal Surgery
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