scholarly journals Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity

Author(s):  
Stéphanie Cupers ◽  
Christine Van Linthout ◽  
Brigitte Desprechins ◽  
Léon Rausin ◽  
Martine Demarche ◽  
...  

Heterotaxy syndrome is a situs anomaly that comprises a large spectrum of cardiac and extracardiac malformations. Its association with intestinal malrotation is frequent. This later might be asymptomatic or manifest by signs of abdominal discomfort or of intestinal obstruction. We report on the case of a full term, small for gestational age neonate in whom heterotaxy syndrome with partial situs inversus abdominalis, intestinal malrotation, polysplenia and vena azygos continuity was diagnosed at prenatal echography. Due to the high risk of volvulus carried by the malrotation, Ladd’s procedure was performed at the age of two months. Follow-up examination at the age of four years shows excellent post-operative result and normal development of the child.

2020 ◽  
Vol 25 (Supplement_2) ◽  
pp. e10-e10
Author(s):  
Maad Saleem ◽  
Lamia Hayawi ◽  
Nick Barrowman ◽  
Nadya BenFadel ◽  
Jana Feberova ◽  
...  

Abstract Background Lower gestational age or birth weight and higher rate of neurodevelopmental impairment have been commonly linked to higher need for developmental resources in premature infants. Existing evidence on need for developmental support in premature infants is limited in description of the needs, timing and method of assessment. Objectives To identify predictors for the needs for developmental resources among high-risk groups of infants born less than 29 weeks or with a birth weight (BW) less than 1250 grams. Secondarily, to compare the need of referrals to developmental resources and the rate of neurodevelopmental impairment defined as cerebral palsy, global developmental delay, blindness and deafness for this high risk population. Design/Methods We conducted a retrospective chart review of premature infants < 29+0 weeks GA or BW < 1250 grams born between January 2005 and December 31st 2014, who had at least one visit at the neonatal follow up clinic. Univariate and multivariate logistic regression analyses were conducted to examine potential predictors for referral to developmental resources. Results The study included 687 infants. Within this high risk population, 579 (85.0%) of infants were referred, of these 153 (26.4%) had one referral, 132 (22.8%) had 2 and 294 (50.8%) had 3 or more referral/s to developmental resources. Most frequent referrals were for speech therapy (339, 50.0%) physiotherapy (319, 46.8%) occupational therapy (262, 38.3%) and infant development program (232, 34.1%). The rates of referral to developmental resources decrease with increasing gestational age. Multivariate logistic regressions showed that gestational age (OR: 1.19, 95% CI: 1.05 - 1.35), birth weight (OR: 0.87, 95% CI: 0.77 - 0.99), female gender (OR: 0.70, 95% CI: 0.49 - 0.99), intraventricular hemorrhage grades III-IV (OR: 3.02, 95% CI: 1.28 - 7.16), and days on mechanical ventilator (OR:1.03, 95% CI: 1.01 - 1.05) were predictors for 2 or more referrals to developmental resources. Cerebral palsy was present in 4.4 % of the study population, deafness in 4% and blindness in 0.6%. The rate of referral to developmental resources was 54 (98.2%) among infants with neurodevelopmental impairment (NDI) compared to 522 (83.9%) in infants without NDI (p value= 0.01). Conclusion There is substantial need for developmental support among high-risk premature infants. Infants without neurodevelopmental impairments still need significant developmental resources to achieve their outcome.


2018 ◽  
Vol 5 (6) ◽  
pp. 2304
Author(s):  
Yogesh N. Parikh ◽  
Priya M. Patel ◽  
Palak T. Hapani

Background: Retinopathy of prematurity is a vaso-proliferative disorder of retina in premature babies. The objective of this study was to study retinopathy of prematurity in premature babies and to study associated risk factors.Methods: Study was a prospective study was conducted among infants in high risk follow up clinic of our department KT Children Hospital, tertiary teaching hospital attached to Government medical college, Rajkot. Sample size of study was conducted on 100 high risk neonates who were brought to high risk follow up clinic at K T children hospital, Rajkot.Results: Out of studied 100 infant, 40% developed ROP, 12% developed stage 1 ROP, 13% developed stage 2 ROP,15% developed stage 3 ROP. Out of 100 cases 40% were ROP positive.15% occurred between 28-30 weeks of gestation. In 31-33 weeks of gestation 82.5%. In 34-36 weeks of gestation 2.5%. 15% of cases between 740-1000 gm of birth weight developed ROP.67.5% between 1000-1500 gm of birth weight developed ROP.15% between 1500-2000 gm of birth weight developed ROP.2.5% between 1500-2000 gm of birth weight developed ROP. 70 newborns received oxygen, 95% of newborns developed ROP. 5% newborns who have not received oxygen developed ROP. In present study there is association of ROP with oxygen therapy. 40 cases had apnea. Out of 40 cases 55% had ROP. Association between apnea and ROP. 21 received blood products.57% of them had ROP. Association between blood transfusion and ROP. 20 received ventilation. 75% of them had ROP. Association between ventilation and ROP. Out of 40 positive newborns there 17 were male and 23 were female. No difference in distribution of ROP in male and female sex. Significant relationship between gestational age, O2 therapy, mechanical ventilation, apnea and blood products. Insignificant relationship between sexes.Conclusions: This study has identified ROP is found to be associated with gestational age, o2 therapy, mechanical ventilation, apnea and blood products.


2019 ◽  
Vol 6 (2) ◽  
pp. 640
Author(s):  
Ameer Khan ◽  
Anil Galwa

Background: Infants born <30 weeks’ gestation is at increased risk of long-term neurodevelopmental problems compared with term born peers. The predictive value of neuro behavioral examinations at term equivalent age in very preterm infants has been reported for subsequent impairment. Therefore, this study aims to attempt to study incidence of neurological abnormality in high risk neonates in our neonatal intensive care unit.Methods: A prospective cohort observational study was conducted at centre for comprehensive child health, division of neonatology, Pushpanjali Crosslay hospital (Max super speciality hospital, Vaishali), Vaishali, Ghaziabad, Uttar Pradesh, India. Duration of study was October 2014 to September 2015. Seventy-five high risk neonates fulfilling the inclusion criteria admitted at NICU were recruited after taking informed consent from mothers /fathers.Results: Total 75 high risk newborns were studied. Their clinical data was noted, neurodevelopmental, neurosensory examination done at 40 weeks of completed gestational age and also at 3 months on follow up. The data was collected according to pre-decided proforma. Neuro developmental assessment was done by updated Amiel-Tison manual of neurological examination. On follow up at 3 months all the newborns who were normal (54) at 40 weeks were found to be normal (100%) and also all those who were definite abnormal (4) at 40 weeks were found to be definite abnormal (100%). But out of the 17 who were moderately abnormal at 40 weeks, 13 (76.5%) have become normal and 4 (23,5%) remained moderately abnormal and none progressed to definite abnormality.Conclusions: There is lack of awareness regarding dog bite and its management among the rural population.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
María Ángeles Sánchez-Durán ◽  
María Teresa Higueras ◽  
Cecilia Halajdian-Madrid ◽  
Mayte Avilés García ◽  
Andrea Bernabeu-García ◽  
...  

Abstract Background The aims of this study were to determine the prevalence of the different anti-erythrocytic alloantibodies, to describe pregnancy outcomes according to a low-risk and high-risk classification for fetal anemia and to determine the factors that influence adverse perinatal outcomes. Methods This retrospective observational study included women referred to our center following the identification of maternal anti-erythrocytic alloantibodies between 2002 and 2017. Pregnancies were classified as high risk for fetal anemia in cases with clinically significant antibodies, no fetal-maternal compatibility and titers ≥1:16 or any titration in cases of Kell system incompatibility. In high-risk pregnancies, maternal antibody titration and the fetal middle cerebral artery peak systolic velocity (MCA-PSV) were monitored. Low-risk pregnancies underwent routine pregnancy follow-up. Results Maternal antibodies were found in 337 pregnancies, and 259 (76.9%) of these antibodies were clinically significant. The most frequent antibodies were anti-D (53%) and anti-K (19%). One hundred forty-three pregnancies were classified as low risk for fetal anemia, 65 (25%) cases were classified as no fetal-maternal incompatibility, 78 had clinically nonsignificant antibodies, 4 (2.8%) resulted in first-trimester pregnancy loss, and 139 (97.2%) resulted in livebirths. Of the 194 high-risk pregnancies, 38 had titers < 1:16 (resulting in 38 livebirths), and 156 had titers ≥1:16 or anti-K antibodies. In the last group, 6 cases miscarried before 18 weeks, 93 had a MCA-PSV < 1.5 multiples of the median (MoM), resulting in 3 perinatal deaths that were unrelated to fetal anemia, one termination and 89 livebirths; and 57 had a MCA-PSV > 1.5 MoM, resulting in 3 intrauterine deaths, 6 terminations and 48 livebirths. Ninety-two intrauterine transfusions were performed in 45 fetuses (87% anti-D). Adverse outcomes were related to a MCA-PSV > 1.5 MoM (p < 0.001), hydrops (p < 0.001) and early gestational age at first transfusion (p = 0.029) Conclusion Anti-D remains the most common antibody in fetuses requiring intrauterine transfusion. A low or high-risk classification for fetal anemia based on the type of antibody, paternal phenotype and fetal antigen allows follow-up of the pregnancy accordingly, with good perinatal outcomes in the low-risk group. In the high-risk group, adverse perinatal outcomes are related to high MCA-PSV, hydrops and early gestational age at first transfusion.


2020 ◽  
Vol 39 (1) ◽  
Author(s):  
Antonio Valvano ◽  
Giorgio Bosso ◽  
Valentina Apuzzi ◽  
Valentina Mercurio ◽  
Valeria Di Simone ◽  
...  

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