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Intestinal atresia

Prasanta Kumar Tripathy, Banoj Kumar Ray, Hiranya Kishore Mohanty
We report an extremely rare finding 'congenital absence of jejunum and ileum' during explorative laparotomy of a 16-day-old female neonate. The dilated duodenum was terminating blindly, and the next segment of intestine was a peanut-sized cecum followed by microcolon. On an extensive survey of literature this type of intestinal atresia is not reported in living babies.
July 2017: African Journal of Paediatric Surgery: AJPS
Damian Dylkowski, Sumit Dave, J Andrew McClure, Blayne Welk, Jennifer Winick-Ng, Sarah Jones
BACKGROUND/PURPOSE: This study was designed to determine the volume, postoperative surgical outcomes and, if possible, the relationship between outcome and institutional / surgeon volume in neonates undergoing repair of esophageal atresia with tracheoesophageal fistula (EA-TEF) over the last 20years in Ontario. METHODS: Using administrative databases, a population based cohort study of patients undergoing EA-TEF repair in Ontario between 1993 and 2012 was conducted...
February 8, 2018: Journal of Pediatric Surgery
Eishin Nakamura, Kouki Samejima, Natsuko Takayanagi, Yoshihisa Ono, Shigetaka Matsunaga, Tomonori Nagai, Yasushi Takai, Masahiro Saitoh, Kazunori Baba, Hiroyuki Seki
AIM: Umbilical cord ulceration (UCU) is a disease in which an ulcer forms in the umbilical cord in the pregnant uterus and is accompanied by hemorrhaging from the same site. UCU occurs in fetuses with congenital upper-intestinal atresia (CUIA); however, its onset mechanism remains unclear. Here, we report our investigation of cases of UCU in our hospital. METHODS: Among the 9825 deliveries performed between 2007 and 2016 at this hospital, 20 fetuses were diagnosed with CUIA, 4 (20%) of which had UCU...
March 8, 2018: Journal of Obstetrics and Gynaecology Research
Jim C H Wilde, Ana M Calinescu, Barbara E Wildhaber
Biliary atresia (BA) and congenital choledochal malformations (CCM) are rare. Both pathologies must (BA) or may (CCM) be operated during the neonatal period or early infancy. We briefly describe the classical operative techniques for both pathologies, followed by the most common and severe perioperative complications encountered during and up to 30 days after surgery in children operated for BA and CCM early in life. For patients with BA, intestinal complications represent the most common and hazardous perioperative surgical complications...
March 6, 2018: European Journal of Pediatric Surgery
Brenna S Fullerton, Cristine S Velazco, Charles R Hong, Alexandra N Carey, Tom Jaksic
PURPOSE: Severe combined immunodeficiency (SCID) screening by T-cell receptor excision circles (TREC) has been part of Massachusetts routine newborn screening since 2009. Tetratricopeptide repeat domain 7A gene (TTC7A) mutations responsible for hereditary multiple intestinal atresia with combined immunodeficiency (MIA-CID) were also recently identified. We reviewed newborn SCID screening among infants with intestinal failure and correlated results with patient characteristics and outcomes...
January 2018: JPEN. Journal of Parenteral and Enteral Nutrition
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 Clin Rep
Maria Chiara De Nardo, Catherine Douch, Louis Yee, Deena Patel
The familial occurrence of multiple-level intestinal atresia in newborn is extremely uncommon, with very sporadic similar cases reported in the literature. We present a case of multiple intestinal atresia in two consecutive siblings who successfully underwent surgical repair.
February 5, 2018: BMJ Case Reports
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
Charles R Hong, David Zurakowski, Brenna S Fullerton, Katelyn Ariagno, Tom Jaksic, Nilesh M Mehta
BACKGROUND: We aimed to describe nutrient intake and growth in infants with gastroschisis and identify factors associated with impaired growth. METHODS: Retrospective study of neonates who underwent gastroschisis repair from 2010 to 2015. Nutrient intake and weight-for-age z scores (WAZ) were recorded. RESULTS: Data from 60 eligible infants with median (Q1, Q3) gestational age of 36 weeks (35, 37) and birth weight 2418 g (2098, 2665) were analyzed...
January 8, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
S Suchitha, B N Kumarguru, Sunila, G V Manjunath
Intramural calcification in intestinal atresia is a rare type of intra-abdominal calcification. The exact etiology of intramural calcification remains obscure. A 1-day-old newborn male baby presented with signs of intestinal obstruction and was diagnosed to have jejunal atresia. The newborn underwent laparotomy with resection of atretic and dilated part of the small bowel. Histology of atretic part of jejunum and adjacent area revealed intramural calcification with extensive foreign-body giant cell reaction...
October 2017: International Journal of Applied and Basic Medical Research
Samira Spineli-Silva, Luciana M Bispo, Vera L Gil-da-Silva-Lopes, Társis P Vieira
Craniofacial Microsomia (CFM) also known as Oculo-auriculo-vertebral Spectrum (OAVS) or Goldenhar Syndrome, presents wide phenotypic and etiological heterogeneity. It affects mainly the structures originated from the first and second pharyngeal arches. In addition, other major anomalies may also be found, including congenital heart diseases. In this study, we report a patient with distal deletion in the 22q11.2 region and a phenotype which resembles CFM. The proband is a girl, who presented bilateral preauricular tags, left auditory canal stenosis, - malar hypoplasia, cleft lip and palate, mild asymmetry of soft tissue in face, congenital heart disease, intestinal atresia, annular pancreas and hydronephrosis...
December 27, 2017: European Journal of Medical Genetics
Antti I Koivusalo, Risto J Rintala, Mikko P Pakarinen
AIM OF THE STUDY: Conservative management of gastrooesophageal reflux (GORD) in oesophageal atresia (OA) is sometimes inefficient, and fundoplication is required. We assessed the outcomes of fundoplication among OA patients from 1980 to 2016. METHODS: After ethical consent, hospital records of 290 patients, including 22 referred patients, were reviewed. Included were 262 patients with end-to-end repair. Excluded were patients who underwent oesophageal reconstruction (n=23) or no repair (n=5)...
February 2018: Journal of Pediatric Surgery
Anastasia Mentessidou, Ioanna Loukou, Georgios Kampouroglou, Anastasia Livani, Ioannis Georgopoulos, Petros Mirilas
BACKGROUND/PURPOSE: In the few studies on intestinal complications and growth of cystic fibrosis (CF) patients with a history of meconium ileus (MI), operated MI has not been investigated separately. We aimed to investigate the incidence of long-term intestinal obstruction sequelae [constipation, distal intestinal obstruction syndrome (DIOS)] and growth in CF patients operated for MI. METHODS: Retrospective study (1989-2016) including operative diagnoses and procedures, constipation and DIOS events, yearly Body Mass Index (BMI) measurements...
November 15, 2017: Journal of Pediatric Surgery
Ayman Goneidy, Evelyn Geok Peng Ong
BACKGROUND: Biliary atresia (BA) is typically treated by Kasai portoenterostomy (KPE), and there is a relationship between age at surgery and outcome. We hypothesize that previous abdominal surgery (PAS) for associated congenital intestinal conditions could be used to identify BA earlier, perhaps improving prognosis. METHODS: A retrospective case note review was performed of all BA patients at a single centre from 1999 to 2016. Demographics and clinical outcome data were collected...
February 2018: Journal of Pediatric Surgery
Mustefa Mohammed, Tadesse Amezene, Moges Tamirat
Background: Failure to pass meconium by a full-term neonate within the first 24 hours should raise a suspicion of bowel obstruction. The objective of this study was to determine pattern of presentation, diagnosis and outcome of management of intestinal obstruction in the early neonatal period in the Neonatal Care Unit of Tikur Anbessa Specialized Hospital in Addis Ababa, Ethiopia. Methods: Retrospective chart review of admitted cases from January 2011 to December 2013 was done...
July 2017: Ethiopian Journal of Health Sciences
João Farela Neves, Isabel Afonso, Luis Borrego, Catarina Martins, Ana Isabel Cordeiro, Conceição Neves, Caroline Lacoste, Catherine Badens, Alexandre Fabre
Tricho-hepato-enteric syndrome (SD/THE) and Multiple intestinal atresia with combined immune deficiency (MIA-CID) are autosomal recessive disorders that present immunological and gastrointestinal features. There are two different phenotypes of patients with TTC7A mutations: the severe form, caused by null mutations and leading to the classical MIA-CID; and the mild form, caused by missense mutations and leading to predominant features of VEO-IBD, less severe immunological involvement and hair abnormalities...
November 23, 2017: European Journal of Medical Genetics
Don K Nakayama
Sir Denis John Wolko Browne (1892-1967), while not the first in the British Isles to devote his entire surgical practice to pediatric surgery, is accepted as "the father of pediatric surgery in the United Kingdom." He made contributions to operations as varied as tonsillectomy, pyloromyotomy, and hypospadias repair, and provided fundamental insights into the proper treatment of club foot, congenital dislocation of the hip, and cryptorchidism. He introduced the transverse laparotomy incision, primary repair of congenital intestinal obstruction, and the end-to-back anastomosis for intestinal atresia, techniques so commonly used that it is difficult to think of pediatric surgical operations done any differently...
October 26, 2017: Journal of Pediatric Surgery
Xiaofang Wang, Yifan Shi, Shi Zeng, Jiawei Zhou, Jia Zhou, Hongxia Yuan, Lin Wang, Weiyuan Shi, Qichang Zhou
BACKGROUND: To investigate the differences in cardiovascular disease, extracardiac anomalies and outcomes between fetuses with levocardia and dextrocardia. METHODS: Clinical demographics, prenatal features, postnatal characteristics and the outcomes of fetuses with levocardia or dextrocardia were recorded and analyzed. RESULTS: Sixty-five fetuses with dextrocardia and thirty-eight fetuses with levocardia were enrolled. Right ventricle outlet obstruction, atrioventricular septal defect and intestinal malrotation were common in both groups...
November 23, 2017: BMC Pregnancy and Childbirth
Philip J Lupo, Jennifer L Isenburg, Jason L Salemi, Cara T Mai, Rebecca F Liberman, Mark A Canfield, Glenn Copeland, Sarah Haight, Sanjiv Harpavat, Adrienne T Hoyt, Cynthia A Moore, Wendy N Nembhard, Hoang N Nguyen, Rachel E Rutkowski, Amy Steele, C J Alverson, Erin B Stallings, Russell S Kirby
BACKGROUND: Gastrointestinal defects are a phenotypically and etiologically diverse group of malformations. Despite their combined prevalence and clinical impact, little is known about the epidemiology of these birth defects. Therefore, the objective of the 2017 National Birth Defects Prevention Network (NBDPN) data brief was to better describe the occurrence of gastrointestinal defects. METHODS: As part of the 2017 NBDPN annual report, 28 state programs provided additional data on gastrointestinal defects for the period 2010-2014...
November 1, 2017: Birth Defects Research
Quentin Ballouhey, Laurence Richard, Laurent Fourcade, Ines Ben Rhaiem, Jean Michel Vallat, Franck Sturtz, Sylvie Bourthoumieu
INTRODUCTION: Intestinal atresia, a rare congenital condition, is often associated with intestinal motility disorders despite adequate neonatal surgery. Previous studies have focused on changes in the enteric nervous system (ENS). We hypothesized that other components of the digestive tract could be involved in this condition. MATERIAL AND METHODS: In a rat model of surgically-induced intestinal obstruction, a transcriptome analysis was performed to measure the global gene expression...
2017: PloS One
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