Short-term neurodevelopmental outcome of babies operated on for low-risk esophageal atresia: a pilot study.

Data on the neurodevelopmental outcome of esophageal atresia (EA) survivors are scarce, controversial, and based on small samples. This is an observational prospective longitudinal study on a selected cohort of low-risk EA survivors. We considered a low-risk EA survivor a patient with the following characteristics: gestational age >32 weeks, no long gap, no genetic or chromosomic anomaly associated with neurodevelopmental delay, and no further major surgical congenital anomalies. Infants were evaluated with scales derived from the Bayley Scales of Infant and Toddler Development - 3rd Edition at 6 and 12 months, with a score of 100 considered normal for each scale. Analysis of variance was used to assess differences of cognitive and motor development. Linear regression was used to assess the impact of the following clinical and sociodemographic variables: gender, birthweight, gestational age, length of hospital stay, number of surgeries and number of esophageal dilatations during first hospitalization, days of mechanical ventilation, weight at follow up, number of surgeries and esophageal dilatations at follow up, parental age, educational level, and socioeconomic status. Thirty children form the object of the study. The mean (standard deviation [SD]) cognitive scale's score was 93.7 (7.5) and 98.2 (9.6) at 6 and 12 months, respectively (P < 0.05). The mean (SD) motor scale's score was 97.6 (9.3) and 98.0 (12.1) at 6 and 12 months, respectively (P = n.s.). Children with a body weight <5° percentile at 12 months showed a mean (SD) cognitive score significantly lower when compared with those with a body weight >5° percentile: 88.8 (6.3) and 100.5 (8.9), respectively. At 12 months, children with unemployed mothers had a mean (SD) motor score significantly lower when compared with those in the other socioeconomic classes: 87.7 (9.8) and 100.6 (12.4), respectively. In conclusion, parents of babies operated on for low-risk EA can be reassured about neurodevelopmental outcome at least up to 1 year of age. When offering a multidisciplinary follow-up program, underweight patients should deserve particular attention to promote their quality of life and support their global development.

Antenatal diagnosis of congenital anomaly: a really traumatic experience?

OBJECTIVE: With increasing use of routine prenatal ultrasound, prenatal counseling after diagnosis of congenital malformations is frequently offered to prospective parents. We aimed to assess if the communication of diagnosis of a congenital anomaly in the fetus meets American Psychiatric Association (APA) criteria for trauma in parents. STUDY DESIGN: In the period ranging from 2003 to 2009 a preliminary investigation was conducted with 165 prospective mothers and 91 prospective fathers being interviewed after communication of diagnosis. Analysis of statements was made independently by two psychologists considering the APA definition of trauma. RESULT: A total of 145 mothers and 76 fathers experienced the communication of diagnosis in their fetus as a traumatic event. There was no correlation between type of malformation and trauma nor was there statistical difference between mother and father regarding the stressor. CONCLUSION: Communication of diagnosis of a fetal anomaly can be a traumatic event and should be dealt with consequently. Given the therapeutic value of sharing traumatic experience such practice should be encouraged as part of the consultation process.

Parents' informational needs at the birth of a baby with a surgically correctable anomaly.

Previous studies have assessed informational needs of parents of sick fetuses, neonates and children to identify favourable patterns of physician-parent interaction. The aim of this paper was to assess parents' informational needs before and after the operation when the one affected by a surgically correctable anomaly is a newborn. In the period ranging from 1997 to 2000 all couples with newborns undergoing major surgical procedures at birth, at the Newborn Surgery Unit of the Hospital Bambino Gesù, were surveyed by means of a questionnaire. Thirty couples form the object of the study. The two genders did not show significant difference in any of the considered items. All 30/30 mothers (M; 100%) and 29/30 fathers (F; 97%) had never heard about the anomaly before the diagnosis was established in their baby. All parents (100%) stated that it would be better if the surgeon informed them with written educational material. Principal informational needs before operation regard: the description of the anomaly (M = 10; F = 11) and the prognosis in terms of survival chances of the baby (M = 17; F = 15); after surgery the cause of the anomaly (M = 5; F = 3); the steps of the recovery process (M = 10; F = 12) as well as the quality of their baby's life (M = 9; F = 9). In the immediate perioperative period the surgeon should well address parents' informational needs, which may significantly differ from his communication plan.

Antenatal diagnosis of surgically correctable anomalies: effects of repeated consultations on parental anxiety.

OBJECTIVE: To assess whether or not a correlation exists between antenatal consultations and parental anxiety. STUDY DESIGN: In total, 31 couples undergoing prenatal consultation after a diagnosis of a surgically correctable anomaly were asked to fill in a questionnaire (Spielberger State-Trait Anxiety Inventory) measuring anxiety levels (AL) both after the first consultation and at birth. Prenatal counselling was done by a perinatal team including paediatric surgeon, obstetrician, and psychologist. RESULTS: Malformations were: eight diaphragmatic hernias, 10 intestinal atresias, four abdominal masses, nine abdominal wall defects. Each fetus was subsequently followed up at regular intervals from diagnosis to birth. AL at birth were then compared with the number of antenatal consultations. A negative correlation (r=-0.688, p<0.001) was found between the number of consultations and the level of anxiety at birth. Patients having at least two consultations had significantly lower anxiety levels at birth. CONCLUSION: Irrespective of the type of malformation, there is evidence that having more than one antenatal consultation may significantly reduce AL at birth. Therefore, early antenatal diagnosis should be encouraged in order to increase as much as possible chances of repeated consultations for the prospective parents.

The impact of cystic fibrosis on neonatal intestinal obstruction: the need for prenatal/neonatal screening.

To determine the incidence of cystic fibrosis (CF) in neonates with intestinal obstruction (NIO) secondary to meconium ileus (MI), jejunoileal atresia (JA), meconium plug syndrome (MPS), volvulus (V), and meconium peritonitis (MP) and analyze the correlation of ultrasonographic (US) signs with CF in NIO with a prenatal diagnosis of intestinal anomaly, a prospective analysis of different types of NIO from 1990 to 1998 was undertaken. Immunoreactive trypsin measurement, genetic studies, and sweat tests were performed to confirm or rule out CF. Cases with prenatal diagnosis were analyzed for gestational age, dilated bowel, ascites, hyperechoic bowel, and calcifications. Of 80 neonates, 19 (24%) had CF: 2/33 (6%) JA, 6/14 (43%) MPS, 1/14 (7.1%) MP, 10/10 (100%) MI, and 0/9 V. Thirty (37.5%) had a prenatal diagnosis of an intestinal anomaly. The overall incidence of CF in NIO with a prenatal diagnosis of intestinal anomaly was 4/30 (13%), or 333 times the estimated risk of CF in the general population. A hyperechoic pattern with dilated bowel was associated with higher specificity for CF: 3/3 cases (100%), followed by hyperechoic bowel with ascites: 3/4 cases (75%). All babies with any type of NIO should thus be screened for CF. Prenatal screening for CF should be indicated in all pregnancies with US patterns of specific intestinal disorders.

Cystic anomalies of biliary tree in the fetus: is it possible to make a more specific prenatal diagnosis?

BACKGROUND/PURPOSE: Biliary tree cystic malformations (BCM) occur rarely. Current progress in ultrasonography makes it possible to identify them prenatally. The aim of this study was to analyze retrospectively prenatal ultrasound patterns of 5 consecutive subhepatic cysts in an effort to differentiate the BCM before birth and onset of symptoms. METHODS: The prenatal ultrasound patterns of 5 consecutive BCM were correlated with the postnatal diagnosis. RESULTS: Case 1 showed a hyperechogenic cyst that underwent enlargement during fetal life; the diagnosis at birth was an obstructed choledochal cyst (CC). In cases 2 and 3, an anechoic cyst with small, stable dimensions during pregnancy was identified; a biliary atresia (BA) type III-C was confirmed at birth. A slightly hyperechogenic cyst with no increase in size at repeated prenatal scans, was observed in cases 4 and 5; a CC and a cystic lesion at the confluence of the hepatic ducts (without obstruction) were diagnosed postnatally, respectively. CONCLUSIONS: The ultrasound scan pattern and size allow to make a prenatal differential diagnosis of BCM. Anechoic, small cyst in the hepatic hilum is highly suspicious for BA. Large, echoic or enlarging cyst is strongly suggestive of "obstructed" CC. Echoic, small and stable cyst is more suggestive of "unobstructed" CC.

Multidisciplinary management of fetal surgical anomalies: the impact on maternal anxiety.

AIM: To assess the impact on maternal anxiety of a multidisciplinary approach in prenatal management of fetal surgical anomalies. DESIGN: A case-control study was undertaken: Group A (cases) consisted of 16 couples receiving a prenatal multidisciplinary counselling, Group B (controls) was represented by 16 couples, who received diagnosis of the fetal anomaly and the relevant counselling by an obstetrician only. The fetuses were affected by the following gastrointestinal anomalies: gastroschisis, omphalocele, intestinal atresia. METHODS: The Italian version of the Spielberger State-Trait Anxiety Inventory was utilized to assess maternal anxiety. In Group A maternal anxiety level was assessed after the first antenatal counselling and at birth, whereas in Group B only at birth. RESULTS: At birth, Group A presented STAI-S scores significantly lower than after the first antenatal consultation with the team (Mean +/- SD = 39.87 +/- 6.46 versus 68.93 +/- 5.81; p < 0.01). At the end of the first day spent with the baby in the Neonatal Surgery Unit, Group A presented STAI-S scores significantly lower than Group B (Mean +/- SD = 39.87 +/- 6.46 versus 70.62 +/- 4.12; p < 0.01). CONCLUSIONS: This study provides evidence of the positive impact on maternal anxiety of a multidisciplinary approach in prenatal management of fetal surgical anomalies.