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https://www.readbyqxmd.com/read/28289880/pediatric-morgagni-diaphragmatic-hernia-a-descriptive-study
#1
Jamie Golden, Wesley E Barry, Gene Jang, Nam Nguyen, David Bliss
INTRODUCTION: The Morgagni hernia (MH) accounts for 3-4% of congenital diaphragmatic hernias. There is a paucity of data regarding this rare defect. The purpose of this study is to describe the characteristics of children with MH, surgical approaches for repair, and patient outcomes. METHODS: Pediatric patients (ages 0-18) with a MH from 2002 to 2014 at a single, freestanding pediatric hospital were retrospectively reviewed. Patient presentation, demographics, operative methods and findings, and outcomes were evaluated...
March 13, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28271155/not-gastroschisis-or-omphalocele-or-anything-in-between-a-novel-congenital-abdominal-wall-defect
#2
Maija Cheung, Nasser Kakembo, Arlene Muzira, John Sekabira, Doruk Ozgediz
Congenital abdominal wall defects occur when normal embryonic development is interrupted and most commonly results in gastroschisis or omphalocele. Other entities, such as ruptured omphalocele, vanishing gastroschisis, and patent omphalomesenteric ducts with prolapse, have also been described and can create a confusing picture. This case of a newborn with a midline abdominal defect and a mass that was intestine-like and arose from the bowel cannot be classified, and no similar reports were found. This suggests a previously undescribed abdominal wall defect with an aberrant colonic appendage...
March 7, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28248727/interleukin-il-1-4-6-7-8-10-appearance-in-congenital-intra-abdominal-adhesions-in-children-under-1-year-of-age
#3
Anna Junga, Māra Pilmane, Zane Ābola, Olafs Volrāts
Several cytokines have been studied for their potential role in adhesion formation. Regulatory role between the cytokine pathways has not yet to be defined. This study was designed to investigate the relation between proinflammatory and anti-inflammatory cytokines in congenital intra-abdominal adhesions. Tissue samples used for research were obtained from abdominal surgery due to obstructive gut malrotation and several additional pathologies (rectal atresia without perforation, omphalocele). All tissue specimens were stained with hematoxylin and eosin and by immunohistochemistry for interleukin-1 (IL-1), IL-4, IL-6, IL-7, IL-8, and IL-10...
February 28, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28208595/congenital-malformations-in-river-buffalo-bubalus-bubalis
#4
REVIEW
Sara Albarella, Francesca Ciotola, Emanuele D'Anza, Angelo Coletta, Luigi Zicarelli, Vincenzo Peretti
The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival...
February 10, 2017: Animals: An Open Access Journal From MDPI
https://www.readbyqxmd.com/read/28203309/a-recurrent-case-of-pentalogy-of-cantrell-a-rare-case-with-sonological-findings-and-review-of-literature
#5
Srinivas M Naren Satya, Kamala Retnam Mayilvaganan, Ivvala Sai Prathyusha, Munnangi Satya Gautam, Deepthi Raidu, V N Amogh
BACKGROUND: Pentalogy of Cantrell (POC) is an extremely rare and complex congenital anomaly. Ultrasound is a valuable, safe, nonionizing, cost effective, widely available, and easily reproducible imaging tool and is indispensible in the diagnosis of POC. Despite the rarity of POC, it is imperative for a radiologist to be aware of its wide spectrum of presentation on ultrasound in first trimester of gestation. Most reported cases in literature till now have been sporadic. In this paper, we aimed to report for the first time in literature, a recurrent case of POC detected in the first trimester in a mother whose previous pregnancy also was terminated in the second trimester medically due to the ultrasound diagnosis of POC...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28196368/pericardio-amniotic-shunting-for-incomplete-pentalogy-of-cantrell
#6
Alexander C Engels, Anne Debeer, Francesca M Russo, Michael Aertsen, Katleen Aerts, Marc Miserez, Jan Deprest, Liesbeth Lewi, Roland Devlieger
A 27-year-old woman, gravida 2, para 0, presented with an incomplete Pentalogy of Cantrell with an omphalocele, diaphragmatic hernia, and a pericardial defect at 32 weeks' gestation. A large pericardial effusion compressed the lungs and had led to a reduced lung growth with an observed-to-expected total lung volume of 28% as measured by MRI. The effusion disappeared completely after the insertion of a pericardio-amniotic shunt at 33 weeks. After birth, the newborn showed no signs of pulmonary hypoplasia and underwent a surgical correction of the defect...
2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28164002/giant-omphalocele-complicated-by-postoperative-duodenal-obstruction
#7
Sunita Ojha, Shobha Parashar, Dharmil Doshi, Rajiv Kumar Bansal
Omphalocele is a congenital defect in the abdominal wall, usually treated at birth or within 1-2 years of life depending on condition of patient and size and contents of the defect. We repaired a giant omphalocele without mesh in a 9-year-old girl. She developed duodenal obstruction in the postoperative period requiring another laparotomy and duodeno-jejunostomy to bypass obstruction.
January 2017: APSP Journal of Case Reports
https://www.readbyqxmd.com/read/28150314/the-upturned-superior-mesenteric-artery-sign-for-first-trimester-detection-of-congenital-diaphragmatic-hernia-and-omphalocele
#8
Ravi Selvaraj Lakshmy, Joy Agnees, Nity Rose
OBJECTIVES: The aim of this study was to follow the course of the superior mesenteric artery (SMA) in first-trimester fetuses to predict the location of the small bowel. Its abnormal course aids in early detection of congenital diaphragmatic hernia (CDH) and assessment of the contents of omphalocele. METHODS: The SMA can be easily identified in a sagittal section of the fetus by using color Doppler sonography at the 11- to 14-week scan, and normally, it has a downward course caudally to supply the intestines...
March 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28093117/the-biological-prosthesis-is-a-viable-option-for-abdominal-wall-reconstruction-in-pediatric-high-risk-defects
#9
Osnat Zmora, Shannon L Castle, Stephanie Papillon, James E Stein
BACKGROUND: Our aim was to explore the indications for and outcome of biological prostheses to repair high risk abdominal wall defects in children. METHODS: A retrospective chart review was performed of all cases of abdominal wall reconstruction in a single institution between 2007 and 2015. Demographic and clinical variables, technique and complications were described and compared between prosthesis types. RESULTS: A total of 23 patients underwent abdominal wall reconstruction using a biological prosthesis including 17 neonates...
January 6, 2017: American Journal of Surgery
https://www.readbyqxmd.com/read/28079045/hernia-of-the-umbilical-cord-associated-with-a-patent-omphalomesenteric-duct
#10
M Raicevic, I Filipovic, S Sindjic-Antunovic
Congenital hernia of the cord is a different type of ventral abdominal wall defect in which the bowel usually herniates into the base of normally inserted umbilical cord through a patent umbilical ring. It is rare congenital anomaly with incidence of 1 in 5000. Although it was described as a distinct entity since 1920s it is often misdiagnosed as a small omphalocele. We present an unusal case of term male newborn with umbilical cord hernia associated with patent omphalomesenteric duct. The diagnose was made after birth despite antenatal ultrasound scans and it is managed successfully with uneventful recovery...
January 2017: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/28051055/thoracic-heteropagus-conjoined-twins-associated-to-an-omphalocele-report-of-a-case-and-complete-review-of-the-literature
#11
Kotti Ahmed, Ben Dhaou Mahdi, Zitouni Hayet, Louati Hamdi, Jallouli Mohamed, Mhiri Riadh
Heteropagus twins are an extremely rare event with an incidence of 1-2 million live births that represents, sometimes, a real challenge for paediatric surgeons. The majority of cases have complete or partial duplication of the pelvis and/or lower extremities. Thoracic heteropagus conjoined twins is a rare condition in which a grossly defective foetus (the parasite) is attached to the thorax of the main foetus (the autosite). We describe a case of a parasitic heteropagus attached at the chest wall with a rare presentation of giant exomphalos on the autosite...
October 2016: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/28050387/alobar-holoprosencephaly-associated-with-meningomyelocoele-and-omphalocoele-an-unusual-coexistence
#12
Tejaswini Priyadarshan Waghmare, Pragati Aditya Sathe, Naina Atul Goel, Bhuvaneshwari Mahendra Kandalkar
Holoprosencephaly is a rare congenital disorder which results from failure of cleavage or incomplete differentiation of the forebrain structures at various levels or to various degrees. Depending on the degree of involvement, it is classified into 4 types: Alobar, Semilobar, Lobar and Middle interhemispheric fusion variant. A male child was born to 28-year-old female at 34 weeks of gestation. The mother on antenatal follow-up was detected to have a fetus with multiple congenital anomalies on Ultrasonography (USG) done at 34weeks of gestation...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28040136/clinical-findings-and-molecular-cytogenetic-study-of-de-novo-pure-chromosome-9p-deletion-pre-and-postnatal-diagnosis
#13
Qiao-Fang Hou, Dong Wu, Yan Chu, Shi-Xiu Liao
OBJECTIVE: The aim of this report is to describe the phenotype-genotype correlation of chromosome 9p deletion syndrome cases, particularly the prenatal cases. MATERIALS AND METHODS: A 30-year-old woman was referred to a hospital at 19(+1) weeks of gestation because of omphalocele detected in the fetus. The conventional karyotyping analysis and array comparative genomic hybridization (aCGH) were utilized for the prenatal diagnosis and genetic counseling in the fetus...
December 2016: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27957373/a-rare-case-report-of-thoracic-ectopia-cordis-an-obstetrician-s-point-of-view-in-multidisciplinary-approach
#14
Diana Ramasauskaite, Vilija Snieckuviene, Viktorija Zitkute, Ramune Vankeviciene, Dalia Lauzikiene, Grazina Drasutiene
Ectopia cordis is a rare congenital anomaly associated with the heart positioned outside of the thoracic cavity either partially or completely. It can be associated with other congenital abnormalities. Overall, the prognosis for infants with ectopia cordis is very poor but depends greatly on the type and severity of ectopia cordis and intracardiac and associated malformations. We present one case of a fetus with prenatally diagnosed thoracic ectopia cordis with intracardiac defects and omphalocele, all the abnormalities seen in pentalogy of Cantrell except a diaphragmatic defect...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27878457/poorly-understood-and-often-miscategorized-congenital-umbilical-cord-hernia-an-alternative-repair-method
#15
E İnce, A Temiz, S S Ezer, H Ö Gezer, A Hiçsönmez
PURPOSE: Umbilical cord hernia is poorly understood and often miscategorized as "omphalocele minor". Careless clamping of the cord leads to iatrogenic gut injury in the situation of umbilical cord hernia. This study aimed to determine the characteristics and outcomes of umbilical cord hernias. We also highlight an alternative repair method for umbilical cord hernias. METHODS: We recorded 15 cases of umbilical cord hernias over 10 years. The patients' data were retrospectively reviewed, and preoperative preparation of the newborn, gestational age, birth weight, other associated malformations, surgical technique used, enteral nutrition, and length of hospitalization were recorded...
November 22, 2016: Hernia: the Journal of Hernias and Abdominal Wall Surgery
https://www.readbyqxmd.com/read/27859469/fraser-syndrome-features-suggestive-of-prenatal-diagnosis-in-a-review-of-38-cases
#16
Aude Tessier, Mélie Sarreau, Fanny Pelluard, Gwenaelle André, Sophie Blesson, Martine Bucourt, Pierre Dechelotte, Laurence Faivre, Thierry Frébourg, Alice Goldenberg, Valérie Goua, Corinne Jeanne-Pasquier, Fabien Guimiot, Annie Laquerriere, Nicole Laurent, Mathilde Lefebvre, Philippe Loget, Martine Maréchaud, Charlotte Mechler, Marie-Josée Perez, Jean Christophe Sabourin, Alain Verloes, Sophie Patrier, Anne-Marie Guerrot
OBJECTIVE: Fraser syndrome (FS) is a rare malformation recessive disorder. Major criteria are cryptophtalmos, syndactyly, respiratory, genital and urinary tract anomalies. Few prenatal presentations have been reported. METHOD: We analyzed the prenatal and postnatal fetal phenotype in 38 cases of FS, including 25 pregnancy termination cases, 8 intra-uterine death cases and 4 cases that died after birth. RESULTS: Including both prenatal and postnatal fetal phenotypic evaluation, all cases presented dysmorphic features with nose and ear dysplasia...
December 2016: Prenatal Diagnosis
https://www.readbyqxmd.com/read/27847120/the-risk-of-midgut-volvulus-in-patients-with-abdominal-wall-defects-a-multi-institutional-study
#17
MULTICENTER STUDY
Jason A Fawley, Abdelhafeez H Abdelhafeez, Jessica A Schultz, Allison Ertl, Laura D Cassidy, Shawn St Peter, Amy J Wagner
BACKGROUND: The management of malrotation in patients with congenital abdominal wall defects has varied among surgeons. We were interested in investigating the risk of midgut volvulus in patients with gastroschisis and omphalocele to help determine if these patients may benefit from undergoing a Ladd procedure. METHODS: A retrospective chart review was performed for all patients managed at three institutions born between 1/1/2000 and 12/31/2008 with a diagnosis of gastroschisis or omphalocele...
January 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27834001/thorax-to-head-ratio-and-defect-diameter-to-head-ratio-in-giant-omphaloceles-as-predictor-for-fetal-outcome
#18
Nina Diemon, Katrin Funke, Mareike Möllers, Kerstin Hammer, Johannes Steinhard, Cristina Sauerland, Volker Müller, Walter Klockenbusch, Ralf Schmitz
PURPOSE: To investigate the relationship between the thorax diameter and defect diameter of giant omphaloceles as a predictor for fetal outcome. METHODS: In a retrospective study, 17 fetuses with isolated giant omphaloceles were included for evaluation. The anterior-posterior thorax diameter and the defect diameter were measured from ultrasound images. For analysis, the thorax-to-head ratio (T/HC), the defect diameter-to-head ratio (DD/HC), and the quotient of the defect diameter and the thorax diameter (DD/T) were calculated...
February 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/27820755/the-impact-of-breast-milk-respiratory-insufficiency-and-gerd-on-enteral-feeding-in-infants-with-omphalocele
#19
Shelly Haug, Shawn St Peter, Sandhya Ramlogan, Donna Goff, Donna Thorpe, Andrew Hopper, Joanne Baerg
OBJECTIVES: The aim of this study was to document the process of achieving full enteral feeding in infants with omphalocele and to identify factors that affect feeding success. METHODS: After Institutional Review Board approval (5100169), 123 infants with omphalocele, born between 1993 and 2011 were reviewed. Mortalities were excluded. All survivors had complete follow-up. Variables suspected to impact enteral feeding in infants with non-giant versus giant omphalocele were compared...
November 5, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27748998/lung-development-requires-an-active-erk-mapk-pathway-in-the-lung-mesenchyme
#20
Olivier Boucherat, Kim Landry-Truchon, Rifdat Aoidi, Nicolas Houde, Valérie Nadeau, Jean Charron, Lucie Jeannotte
BACKGROUND: Reciprocal epithelial-mesenchymal communications are critical throughout lung development, dictating branching morphogenesis and cell specification. Numerous signaling molecules are involved in these interactions, but the way epithelial-mesenchymal crosstalk is coordinated remains unclear. The ERK/MAPK pathway transduces several important signals in lung formation. Epithelial inactivation of both Mek genes, encoding ERK/MAPK kinases, causes lung agenesis and death. Conversely, Mek mutation in mesenchyme results in lung hypoplasia, trachea cartilage malformations, kyphosis, omphalocele, and death...
January 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
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