DOZ047.92: Oesophageal atresia: a multicenter retrospective study on surgical treatment and 1-year follow-up

2019 ◽  
Vol 32 (Supplement_1) ◽  
Author(s):  
C Vercauteren ◽  
H Reusens ◽  
A De Backer ◽  
R Tambucci ◽  
S Van Cauwenberge ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Oesophageal Atresia ◽  
Tube Placement ◽  
First Year ◽  
Paediatric Surgery ◽  
Anastomotic Strictures ◽  
Associated Malformations ◽  
Type C ◽  
First Year Of Life

Abstract Introduction No data are available on surgical management and outcomes of oesophageal atresia (OA) in Belgium. In collaboration with the Belgian Association of Paediatric Surgery (BELAPS) a retrospective study was organized to analyze data from children born with OA, with a 1-year follow-up. Materials and Methods Out of 17 centers in Belgium, 10 participated in this study. A novel checklist to collect data from OA patients born between the 1st of January 2010 and the 1st of January 2015 was created. Gross type A, B, C and D OA were included. Results One hundred children were included. Gross type C OA was seen in 86%. Associated malformations were present in 73%, most frequently cardiac malformations (52%). There was consensus regarding preoperative investigations, anastomotic technique, and transanastomotic tube placement. There was no consensus regarding genetic counseling, placement of a chest tube, and start of enteral feeding. There were six deaths in the first year of life. The majority of patients needed rehospitalization during the first year of life (85.9%) and there was a high occurrence of anastomotic strictures (51.6%). Conclusions This is the first study about management and outcome of OA in Belgium. Although the 1-year survival rate is high (93.8%), these data illustrate that OA has an important morbidity. Multidisciplinary follow-up and prospective studies/registries with longer follow-up are needed.

scholarly journals Congenital Heart Disease and Its Impact on the Development of Anastomotic Strictures after Reconstruction of Esophageal Atresia

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Pernilla Stenström ◽  
Martin Salö ◽  
Magnus Anderberg ◽  
Einar Arnbjörnsson
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Esophageal Atresia ◽  
Congenital Heart ◽  
First Year ◽  
Retrospective Case ◽  
Anastomotic Strictures ◽  
First Year Of Life ◽  
Severe Congenital Heart Disease

Background. The aim was to explore if severe congenital heart disease (CHD) influenced the need for dilatation of anastomotic strictures (AS) after the repair of esophageal atresia (EA). Methods. A retrospective case-control study was conducted examining AS in children with EA and Gross type C. The spectra of CHD and cardiac interventions were reviewed. The frequency of dilatations of AS during the first year following EA reconstruction was compared between children with and without severe CHD requiring cardiac surgery during their first year of life. Endoscopic signs of stricture were an indication for dilatation. Results. Included in the follow-up for AS were 94 patients who had EA reconstructions, of whom 10 (11%) children had severe CHD requiring surgery during the first year including 19 different cardiac interventions. In total, 38 patients needed dilatation of esophageal AS, distributed as six (60%) with severe CHD and 32 (38%) without severe CHD (p=0.31). Conclusion. Severe CHD was present in 11% of children with EA. Esophageal AS developed in 60% children with concomitant CHD, but although high, it did not reach statistical difference from children without CHD (38%).

scholarly journals Anastomotic Strictures after Esophageal Atresia Repair: Timing of Dilatation during the First Two Postoperative Years

2018 ◽  
Vol 04 (02) ◽  
pp. e62-e65 ◽  
Author(s):  
Martin Salö ◽  
Pernilla Stenström ◽  
Magnus Anderberg ◽  
Einar Arnbjörnsson
Keyword(s):  
Esophageal Atresia ◽  
Pediatric Surgery ◽  
First Year ◽  
Esophageal Dilatation ◽  
X Ray ◽  
Anastomotic Strictures ◽  
Tertiary Center ◽  
Type C ◽  
First Year Of Life ◽  
Time Frames

Background We determined time frames for dilatation of anastomotic strictures (ASs) occurring during the first 2 years after esophageal atresia (EA) repair. Methods A retrospective study was conducted on children with EA (Gross type C) who underwent direct repair between January 2008 and March 2015 at a single tertiary center of pediatric surgery. Endoscopic signs of stricture were indications for dilatation because the endoscopy provides more reliable information than X-ray imagining methods. Results Among our cohort of 49 children with EA, 19 (39%) required at least one esophageal dilatation. All children required initial dilatation within the first year of life and none was older than 1 year during initial dilatation (p < 0.01). A median of three dilatations (range: 1–13) took place per patient, with 87% performed during the first postoperative year. The timing of initial dilatation in the first year (< 6 months, 14/19 [74%] vs. 6–12 months, 5/19 [26%]) was predictive of the need for dilatation beyond the first year (9/14 [64%] vs. 0/5 [0%]; p = 0.03) but not of more numerous dilatations (median, 3 vs. 1; p = 0.07). Conclusion The need for dilatation within 6 months postoperatively predicts the need for dilatation after 1 year, but it does not indicate the number of dilatations that will be needed.

Muenke syndrome: long-term outcome of a syndrome-specific treatment protocol

2019 ◽  
Vol 24 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Bianca K. den Ottelander ◽  
Robbin de Goederen ◽  
Marie-Lise C. van Veelen ◽  
Stephanie D. C. van de Beeten ◽  
Maarten H. Lequin ◽  
...  
Keyword(s):  
Treatment Protocol ◽  
First Year ◽  
Ventricular Size ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Skull Growth ◽  
Muenke Syndrome ◽  
First Year Of Life

OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.

Abnormal Shoulder Girdle Muscle Tone in Premature Infants During Their First 18 Months of Life

PEDIATRICS ◽  
1986 ◽  
Vol 77 (5) ◽  
pp. 664-669
Author(s):  
Michael K. Georgieff ◽  
Judy C. Bernbaum
Keyword(s):  
Premature Infants ◽  
Motor Development ◽  
Muscle Tone ◽  
Birth Asphyxia ◽  
Shoulder Girdle ◽  
First Year ◽  
First Year Of Life ◽  
Girdle Muscle ◽  
Shoulder Girdle Muscle

To document the incidence of and neonatal factors associated with abnormal shoulder girdle muscle tone in premature infants at follow-up, we studied 125 consecutively admitted infants weighing &lt; 1,750 g treated in The Children's Hospital of Philadelphia intensive care nursery and subsequently seen in the Neonatal Follow-up Program up to 18 months of age. Fifty-seven infants (46%) displayed abnormal shoulder girdle muscle tone which presented clinically as scapular retractions. These infants had significantly lower birth weights (P &lt; .001) and gestational age (P &lt; .001) as well as a higher incidence of acute and chronic pulmonary disease (P &lt; 0.01) and CNS insults (P &lt; .05) when compared with infants without scapular retractions. The 57 infants with scapular retractions were further divided into two groups: 42 infants (74%) in whom scapular retractions were associated with generalized mild hypertonicity and 15 infants (26%) in whom scapular retractions compensated for trunk and neck hypotonicity. The infants with scapular retractions and hypotonicity had a significantly higher incidence of neonatal neurologic morbidity including seizures, major resuscitations, and birth asphyxia (P &lt; .01) when compared with the infants with scapular retractions and hypertonicity. Shoulder girdle tone abnormalities in the first year of life inhibit crawling, sitting, and object manipulation and, therefore, may manifest as delays in motor development. Identification of infants with significant neonatal risk factors for tone abnormalities is important to allow for earlier therapeutic intervention.

scholarly journals Safety of potential breast milk exposure to IFN-β or glatiramer acetate

2020 ◽  
Vol 7 (4) ◽  
pp. e757
Author(s):  
Andrea Ines Ciplea ◽  
Annette Langer-Gould ◽  
Anna Stahl ◽  
Sandra Thiel ◽  
Annette Queisser-Wahrendorf ◽  
...  
Keyword(s):  
Breast Milk ◽  
Glatiramer Acetate ◽  
Growth Curves ◽  
Antibiotic Use ◽  
Physical Growth ◽  
First Year ◽  
Motor Delay ◽  
Infant Outcomes ◽  
First Year Of Life

ObjectiveTo determine whether potential breast milk exposure to interferon-beta (IFN-β) or glatiramer acetate (GA) is safe for the infant.MethodsWe identified 74 infants born to 69 women with MS who breastfed under IFN-β (n = 39), GA (n = 34), or both (n = 1). Women had been enrolled into the German Multiple Sclerosis and Pregnancy Registry during pregnancy. Data were obtained from standardized, telephone-administered questionnaires completed by the mother during pregnancy and at 1, 3, 6, and 12 months postpartum and the infant's take-home medical record.ResultsThe median duration of exposed breastfeeding was 8.5 months (wide interquartile range: 4.9–12.7 months). Physical growth curves during the first year of life were consistent with national, sex-specific growth curves. Median body measurements were consistent with national medians. Most children (n = 71, 96%) had normal motor and language development. Gross motor delay was reported in 3 children, of whom 1 remained delayed at last follow-up (3.9 years old) and 2 were normal by 0.9 and 4.1 years old. The proportion of children hospitalized at least once (girls n = 2, 7%, and boys n = 6, 14%) and the proportion of children with at least one episode of systemic antibiotic use during the first year of life (girls n = 7, 23%, and boys n = 8, 18%) are consistent with national averages.ConclusionPotential breast milk exposure to IFN-β or GA did not increase the risk of common adverse infant outcomes in the first year of life. Taken together with the benefits of breastfeeding and low biological plausibility of risk, women with MS who wish to resume IFN-β or GA postpartum can be encouraged to breastfeed.

scholarly journals Outcomes of early repeat sweat testing in infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome (CRMS)/CF screen-positive, inconclusive diagnosis (CFSPID)

2021 ◽  
Author(s):  
vito terlizzi ◽  
Laura Claut ◽  
Carla Colombo ◽  
Antonella Tosco ◽  
Alice Castaldo ◽  
...  
Keyword(s):  
Metabolic Syndrome ◽  
Definitive Diagnosis ◽  
First Year ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Sweat Chloride ◽  
Sweat Testing ◽  
Cf Transmembrane Conductance Regulator ◽  
First Year Of Life

Background: Reaching early and definitive diagnosis in infants with cystic fibrosis (CF) transmembrane conductance regulator-related metabolic syndrome (CRMS)/CF screen-positive, inconclusive diagnosis (CFSPID) is a priority of all CF newborn screening programs. Currently, sweat testing is the gold standard for CF diagnosis or exclusion. We assessed outcomes in a cohort of Italian CRMS/CFSPID infants who underwent repeat sweat testing in the first year of life. Methods: This multicentre, prospective study analysed clinical data and outcomes in CRMS/CFSPID infants born between September 1, 2018 and December 31, 2019, and followed until June 30, 2020. All subjects underwent CF transmembrane conductance regulator (CFTR) gene sequencing and the search for CFTR macrodeletions/macroduplications, and repeat sweat testing in the first year of life. Results: Fifty subjects (median age at end of follow-up, 16 months [range, 7–21 months]) were enrolled. Forty-one (82%) had the first sweat chloride in the intermediate range. During follow up, 150 sweat tests were performed (range, 1–7/infant). After a median follow-up of 8.5 months (range 1–16.2 months), 11 (22%) subjects were definitively diagnosed as follows: CF (n=2 [4%]) at 2 and 5 months, respectively; healthy carrier (n=8 [16%]), at a median age of 4 months (range 2–8 months); and healthy (n=1 [2%]) at 2 months of age. Inconclusive diagnosis remained in 39 (78%) infants. Conclusions: Early repeat sweat testing in the first year of life can shorten the time to definitive diagnosis in screening positive subjects with initial sweat chloride levels in the intermediate range.

LONGITUDINAL EEG-CLINICAL CORRELATIONS IN CHILDREN FROM BIRTH TO 4 YEARS OF AGE

PEDIATRICS ◽  
1968 ◽  
Vol 41 (5) ◽  
pp. 945-954
Author(s):  
Fernando Torres ◽  
Michael E. Blaw
Keyword(s):  
Statistical Significance ◽  
Newborn Infants ◽  
Normal Subjects ◽  
First Year ◽  
Advanced Stages ◽  
The Difference ◽  
Focal Abnormality ◽  
First Year Of Life ◽  
Single Focus

One hundred-thirty children who had an EEG during their first days of life and who were registered in a clinical longitudinal study were followed with concurrent clinical and EEG examinations every 4 months for the first year of life and at 2, 3, and 4 years of age. Thirty children had EEG characteristics which are frequently considered abnormal in their neonatal record. Twenty-three children had clinical abnormalities during the 4-year period covered by the study. There was no significant correlation between a single EEG and clinical abnormalities at any age. Newborn infants with more than one focal abnormality in their EEG presented clinical abnormalities more frequently than those with a single focus. The difference, however, did not attain statistical significance. Children with an abnormal EEG at birth and an additional abnormal record later, had a higher incidence of clinical abnormalities than those with only an abnormal neonatal EEG. However, this finding is of questionable significance because the children who had clinical abnormalities had a larger number of EEG's than the normal subjects. It is expected that continued follow-up of these children at more advanced stages of their development may give a positive EEG-clinical correlation which was not found in this study.

Effectiveness of Frenotomy in Ankyloglossia Treatment Between 0-1 Years Old

2021 ◽  
Author(s):  
Fatih Akova
Keyword(s):  
First Year ◽  
Bleeding Control ◽  
Old Patients ◽  
Feeding Function ◽  
Group 2 ◽  
Hospital Pediatric ◽  
First Year Of Life ◽  
Results Of Surgery ◽  
Group 1

Objective: The aim of this study is to present the frenotomy technique in cases of ankyloglossia (tongue-tie), which is diagnosed and treated in the first year of life. Patients were operated by a single surgeon. Method: Cases of ankyloglossia operated at Biruni University Faculty of Medicine Hospital Pediatric Surgery Clinic Between 2016-2020 were evaluated retrospectively in terms of clinical complaints, age, type of ankyloglossia, surgical technique, indications and results of surgery. Results: Frenotomy was performed in 56 patients including 47 boys (84%) and 9 girls (16%), between the ages of 0-1. Average age of the patients was 93 days (1-360). Patients were divided into two groups as Group 1 (n: 40: 0-90 days old) and Group 2 (n: 16: 90-360 days old). Patients were admitted with complaints of having difficulty in sucking the mother’s breast, not being able to take their tongue out, feeding with a bottle, pain at the nipple and not being able to grasp the breast. No additional intervention was required for bleeding in Group 1, and in 12 (75%) patients in Group 2 bleeding control was achieved using bipolar cautery. During follow-up, significant improvement was obtained in all patients who had difficulty in sucking and gripping the nipple. Improvement was observed in 15 of 25 patients with nipple pain. Conclusion: Frenotomy is an easily applied surgical procedure with minimal complications. Additional application may be required for bleeding control in infants older than 3 months. It should be considered that the probability of recurrence may depend on the type, intervention used and thickness of the frenulum, and phrenotomy may not be sufficient. The improvement in breast feeding function of Frenotomy may provide a significant improvement in the complaints of nipple pain, and may contribute to the emotional attachment between the mother and her baby. Randomized controlled trials are required to determine the effects of phrenotomy.

Adaptive capabilities of newborns depending on the conditions of intrauterine development

1982 ◽  
Vol 63 (4) ◽  
pp. 5-7
Author(s):  
E. A. Efimova ◽  
S. L. Nesterov ◽  
N. L. Yashina ◽  
T. K. Shakurova ◽  
V. A. Novikova ◽  
...  
Keyword(s):  
Blood Cells ◽  
Neonatal Period ◽  
Preventive Therapy ◽  
First Year ◽  
Children At Risk ◽  
Intrauterine Development ◽  
Adaptive Capabilities ◽  
First Year Of Life ◽  
Adaptive Reactions

Clinical, cytochemical, and rheological features of the neonatal period were studied in 460 children from mothers suffering from rheumatism or having suffered late toxicosis of pregnant women. On the basis of impaired adaptive reactions, changes in the enzymatic spectrum of blood cells and disorders of microcirculatory mechanisms, children at risk were identified. 50 of them underwent preventive therapy in the neonatal period. The follow-up indicates a lower infectious index during the first year of life in children who received preventive therapy.

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