Feasibility and Added Value of Fetal DTI Tractography in the Evaluation of an Isolated Short Corpus Callosum: Preliminary Results.

BACKGROUND AND PURPOSE: Prognosis of isolated short corpus callosum is challenging. Our aim was to assess whether fetal DTI tractography can distinguish callosal dysplasia from variants of normal callosal development in fetuses with an isolated short corpus callosum. MATERIALS AND METHODS: This was a retrospective study of 37 cases referred for fetal DTI at 30.4 weeks (range, 25-34 weeks) because of an isolated short corpus callosum less than the 5th percentile by sonography at 26 weeks (range, 22-31 weeks). Tractography quality, the presence of Probst bundles, dysmorphic frontal horns, callosal length (internal cranial occipitofrontal dimension/length of the corpus callosum ratio), and callosal thickness were assessed. Cytogenetic data and neurodevelopmental follow-up were systematically reviewed. RESULTS: Thirty-three of 37 fetal DTIs distinguished the 2 groups: those with Probst bundles (Probst bundles+) in 13/33 cases (40%) and without Probst bundles (Probst bundles-) in 20/33 cases (60%). Internal cranial occipitofrontal dimension/length of the corpus callosum was significantly higher in Probst bundles+ than in Probst bundles-, with a threshold value determined at 3.75 for a sensitivity of 92% (95% CI, 77%-100%) and specificity of 85% (95% CI, 63%-100%). Callosal lipomas (4/4) were all in the Probst bundles- group. More genetic anomalies were found in the Probst bundles+ than in Probst bundles- group (23% versus 10%, P = .08). CONCLUSIONS: Fetal DTI, combined with anatomic, cytogenetic, and clinical characteristics could suggest the possibility of classifying an isolated short corpus callosum as callosal dysplasia and a variant of normal callosal development.

Accuracy of prenatal ultrasound screening to identify fetuses infected by cytomegalovirus which will develop severe long-term sequelae.

OBJECTIVES: To compare the ability of detailed routine ultrasound examination, performed without knowledge of maternal serology and fetal status, with that of targeted prenatal imaging performed in prenatal diagnostic units in cases of known fetal infection to identify cytomegalovirus (CMV)-infected fetuses that will develop long-term sequelae. METHODS: All prenatal imaging reports were collected for 255 children with congenital CMV in a registered cohort between 2013 and 2017 (NCT01923636). All women had undergone detailed routine fetal ultrasound examination at 20-24 and 30-34 weeks as part of routine antenatal care. All cases of known fetal CMV infection had also undergone targeted prenatal ultrasound examination. Postnatal structured follow-up for up to 48 months of age involved clinical, audiological and neurological assessment, including Brunet-Lezine scoring. Long-term sequelae (> 12 months) were considered to be mild in cases with isolated unilateral hearing loss and/or vestibular disorders, and severe in cases with bilateral hearing loss and/or neurological sequelae. All imaging reports were analyzed retrospectively with the knowledge of congenital CMV infection, searching for reference to findings that were, or could have been, related to fetal infection. Findings were analyzed in relation to whether the cases were diagnosed with CMV in utero or only postnatally. RESULTS: There were 237 children with complete follow-up data (> 12 months), for a median of 24 (range, 12-48) months. Of these, 30% (71/237) were diagnosed with CMV prenatally and 70% (166/237) were diagnosed within 3 weeks after birth. 72.5% (29/40) of children with long-term sequelae, including 74% (14/19) with severe long-term sequelae, were not identified in the prenatal period. Among those diagnosed prenatally, the sensitivity of prenatal imaging for predicting long-term sequelae and severe long-term sequelae was 91% and 100%, respectively, while, in the group diagnosed only postnatally, non-specific infection-related ultrasound findings had been reported without raising suspicion in 48% of cases with long-term sequelae and 64% of those with severe long-term sequelae. CONCLUSIONS: Routine detailed ultrasound examination in pregnancy is not an appropriate screening tool for congenital CMV infection that leads to long-term sequelae, in contrast with the high performance of targeted prenatal imaging in known cases of fetal infection. The non-specific nature of ultrasound features of CMV and their evolution, and a lack of awareness of caregivers about congenital CMV, are likely explanations. Awareness of the sonologist regarding congenital CMV and knowledge of the maternal serological status in the first trimester seem key to the performance of prenatal ultrasound. Copyright © 2020 ISUOG. Published by John Wiley & Sons Ltd.

[Retrospective diagnosis of congenital CMV infection in DBS from Guthrie cards: French experience]. / Le diagnostic rétrospectif de l'infection congénitale à cytomégalovirus sur le sang séché des cartes de Guthrie: l'expérience en France.

Systematic screening for cytomegalovirus congenital infection is not performed in France. For children with hearing loss or other neurological CMV compatible symptoms, retrospective diagnosis is possible by PCR detection of CMV DNA in dried blood spot of neonatal Guthrie cards. We report here the results obtained with this technique in the French national reference laboratory for cytomegalovirus.

The value of middle cerebral artery peak systolic velocity in the diagnosis of fetal anemia after intrauterine death of one monochorionic twin.

OBJECTIVE: The purpose of this study was to assess the value of the fetal middle cerebral artery peak systolic velocity in the prediction of anemia within 24 hours of the death of one monochorionic twin in twin-to-twin-transfusion syndrome and to establish the correlation between middle cerebral artery peak systolic velocity and hemoglobin concentration in fetuses who are at risk for acute anemia. STUDY DESIGN: Doppler examination of the middle cerebral artery peak systolic velocity was performed in 20 monochorionic survivors of pregnancies that were complicated by twin-to-twin-transfusion syndrome that occurred between 20 and 34 weeks of gestation. Doppler examination was performed before cordocentesis and after intrauterine transfusion when appropriate. Both hemoglobin concentration and middle cerebral artery peak systolic velocity were expressed in multiples of the median. Severe anemia was defined as hemoglobin concentration of <0.55 multiples of the median, and we used the cutoff point of 1.50 times the median values at any gestational age to calculate the sensitivity and specificity of middle cerebral artery peak systolic velocity in detecting moderate or severe anemia. RESULTS: Fetal anemia was confirmed in 10 of 20 fetuses. We performed seven intrauterine transfusions. The sensitivity and specificity of middle cerebral artery peak systolic velocity in the prediction of severe fetal anemia were of 90%, with a false-negative rate of 10%. The correlation between peak systolic velocity and hemoglobin concentration both before and after transfusion was evaluated by regression analysis and was strongly significant. CONCLUSION: In fetuses who are at risk of acute anemia, the measurement of middle cerebral artery peak systolic velocity was found to be a reliable noninvasive diagnostic tool and may be helpful in counseling and planning invasive assessment.

[Use of a polyethylene bag: a way to improve the thermal environment of the premature newborn at the delivery room]. / Utilisation d'un sac en polyéthylène: un moyen d'améliorer l'environnement thermique du prématuré en salle de naissance.

BACKGROUND: Early interventions, such as occlusive wrapping of very low birth weight infants at delivery reduce postnatal temperature fall. This new intervention was implemented in our hospital on January 2000. The aim of this study was to investigate retrospectively the effect of polyethylene wrap, applied immediately at birth, on thermoregulation. PATIENTS AND METHODS: Matched pair analysis was conducted for 60 infants delivered inborn at less than 33 weeks' gestation and 60 premature infants who were born during the second half of 1999 fulfilling the same criteria. The only difference in the management (medical and environmental) was wrapping with a polyethylene bag in the delivery room. Rectal temperature and other vital parameters were taken, after removal of wraps, on admission to NICU. RESULTS: The perinatal characteristics of both groups were comparable. Use of wrapping resulted in a significantly higher admission rectal temperature (difference in means = 0.8 degree C, p < 0.0001), this difference was also significant in infants < 30 weeks. The incidence of hypothermia (< 35.5 degrees C) was less frequent in infants enclosed in plastic bags (8.3% vs 55%). No side effects (skin burns, infection or hyperthermia) were attributable to the intervention. The heart rate was higher in the wrapping group (163 +/- 16 vs 150 +/- 17 b/min, p < 0.01), as well as the capillary glycemia (62 +/- 26 vs 45 +/- 30 mg/dl, p < 0.01). There was no significant difference on arterial pressure. CONCLUSION: Occlusive wrapping with a polyethylene bag at birth prevented low rectal temperature in premature infants in the immediate postnatal period. This method is easy, practical and effective, and does not interfere with current practice for resuscitation.

[Jargon of the neonatal intensive care unit]. / Le jargon de la réanimation néonatale.

Jargon, the specialized vocabulary and idioms, is frequently used by people of the same work or profession. The neonatal intensive care unit (NICU) makes no exception to this. As a matter of fact, NICU is one place where jargon is constantly developing in parallel with the evolution of techniques and treatments. The use of jargon within the NICU is very practical for those who work in these units. However, this jargon is frequently used by neonatologists in medical reports or other kinds of communication with unspecialized physicians. Even if part of the specialized vocabulary can be decoded by physicians not working in the NICU, they do not always know the exact place that these techniques or treatments have in the management of their patients. The aim of this article is to describe the most frequent jargon terms used in the French NICU and to give up-to-date information on the importance of the techniques or treatments that they describe.

Prenatal diagnosis of a subdural hematoma associated with reverse flow in the middle cerebral artery: case report and literature review.

To our knowledge this is the first reported case of a subdural hematoma, related to fetal autoimmune thrombocytopenia, diagnosed prenatally by ultrasound. The underlying etiology was hidden maternal autoimmune thrombocytopenia. This disease, which is as serious as alloimmune thrombocytopenia, causes severe fetal thrombocytopenia, which carries with it a high risk of intracranial hemorrhage and recurrence in subsequent pregnancies. Only 14 cases of fetal subdural hematomas have been reported in the literature. The etiologies of these cases were principally traumatic, or due to disorders of hemostasis. Their prognoses were generally poor, with 50% resulting in fetal death in utero and the remaining 50% demonstrating post-natal sequelae. The presence of reverse flow in the middle cerebral artery is rare and its association with a subdural hematoma unusual. Abnormalities found on cerebral Doppler studies indicate an adaptive response to a fetal condition for which the prognosis is then very poor. Monitoring fetuses at risk for hemorrhage by ultrasound imaging and Doppler studies enables us to detect indicators of a worsening prognosis before the ultrasound appearance of morphological hemorrhage. The development of intracranial hemorrhage raises difficult management issues during the index pregnancy and in subsequent pregnancies.

Randomised trial of analgesic effects of sucrose, glucose, and pacifiers in term neonates.

OBJECTIVES: To assess and compare the analgesic effects of orally administered glucose and sucrose and pacifiers. To determine the synergistic analgesic effect of sucrose and pacifiers. DESIGN: Randomised prospective study with validated behavioural acute pain rating scale. SETTING: Maternity ward. PARTICIPANTS: 150 term newborns undergoing venepuncture randomly assigned to one of six treatment groups: no treatment; placebo (2 ml sterile water); 2 ml 30% glucose; 2 ml 30% sucrose; a pacifier; and 2 ml 30% sucrose followed by a pacifier. RESULTS: Median (interquartile) pain scores during venepuncture were 7 (5-10) for no treatment; 7 (6-10) for placebo (sterile water); 5 (3-7) for 30% glucose; 5 (2-8) for 30% sucrose; 2 (1-4) for pacifier; and 1 (1-2) for 30% sucrose plus pacifier. Mann-Whitney U test P values for comparisons of 30% glucose, 30% sucrose, pacifier, and 30% sucrose plus pacifier versus placebo (sterile water) were 0.005, 0.01, <0.0001, and <0.0001, respectively. Differences between group median pain scores for these comparisons were 2 (95% confidence interval 1 to 4), 2 (0 to 4), 5 (4 to 7), and 6 (5 to 8), respectively. P values for comparisons of 30% glucose, 30% sucrose, and 30% sucrose plus pacifier versus pacifier were 0.0001, 0.001, and 0.06, respectively. Differences between group medians for these comparisons were 3 (2 to 5), 3 (1 to 5), and 1 (0 to 2), respectively. CONCLUSION: The analgesic effects of concentrated sucrose and glucose and pacifiers are clinically apparent in newborns, pacifiers being more effective than sweet solutions. The association of sucrose and pacifier showed a trend towards lower scores compared with pacifiers alone. These simple and safe interventions should be widely used for minor procedures in neonates.

Features of DiGeorge syndrome and CHARGE association in five patients.

We report on five patients presenting with features of two congenital disorders, DiGeorge syndrome (DGS) and CHARGE association. CHARGE association is usually sporadic and its origin is as yet unknown. Conversely, more than 90% of DGS patients are monosomic for the 22q11.2 chromosomal region. In each of the five patients, both cytogenetic and molecular analysis for the 22q11.2 region were normal. In view of the broad clinical spectrum and the likely genetic heterogeneity of both disorders, these cases are consistent with the extended phenotype of either DGS without 22q11.2 deletion or CHARGE association, especially as several features of CHARGE association have been reported in rare patients with 22q11.2 deletion association phenotypes. On the other hand, these could be novel cases of an independent association involving a complex defect of neural crest cells originating from the pharyngeal pouches.

[Water intoxication following preparation for barium enema]. / Intoxication par l'eau après une préparation pour un lavement baryté.

BACKGROUND: Induction of water intoxication from tap water enemas was reported a few years ago. Its treatment is still debated. CASE REPORT: A 4 1/2 year-old boy was admitted because he suffered from coma grade I. A barium enema had been prescribed for fecal incontinence and the patient had been given orally about 4 liters of water during the 24 hours preceding this investigation. Blood examination showed;: Na 122 mEq/l; K 3 mEq/l; Cl 87 mEq/l. Brain CT scan was normal. The patient was placed under restriction of fluid and was given i.v. 5.8% NaCl solution (2 mM/kg) for 3 hours. Convulsions appeared despite this treatment requiring intubation and ventilation plus increasing doses of NaCl: 20% solution (2 mM/kg) for 30 minutes followed by 2 mM/kg for 3 hours, associated with mannitol and furosemide infusion. CONCLUSION: Use of hypertonic saline solutions in the treatment of water intoxication is discussed. Acute hyponatremia must be rapidly corrected using hypertonic saline solution plus restriction of fluid and diuretic.

[Isolated fetal tachycardia, a diagnostic event of Basedow's disease. Apropos of a case]. / Tachycardie foetale isolée, circonstance de découverte d'une maladie de basedow. A propos d'une observation.

Two of thousand pregnancies are complicated by a Graves' disease. The circumstances of diagnosis are usually maternal disorders (tachycardia, exophthalmia, loss of weight...), also fetal disorders (tachycardia, intra-uterine growth retardation, stillborn...). The discovery of fetal tachycardia (160 beats per minute), and maternal tachycardia (120 beats per minute) associated with high blood pressure, allowed us to suspect, in this case, a Graves' disease, responsible of fetal hyperthyroidism by autoantibodies crossing the placenta. The measuring of T3, T4, TSH and autoantibodies confirmed the diagnosis. The drug of choice is the PTU (propylthiouracil). It prevents synthesis of thyroid hormones and inhibits peripheral deiodination of T4 to T3. It treats simultaneously mother and fetus; the surveillance must allow us to adjust treatment to avoid fetal hypothyroidism, maternal thyrotoxicosis of peripartum and neonatal thyrotoxicosis.

Intravenous nicardipine in hypertensive children.

Fourteen hypertensive patients hospitalized in a paediatric intensive care unit were studied to evaluate safety and hypotensive efficacy of intravenous nicardipine. Systolic and diastolic blood pressure significantly decreased 1 h after the beginning of the treatment (1 microgram/kg per minute). Mean decrease in systolic blood pressure during the first 24 h was between 9.9% and 13.4% of the initial value. Mean lowering of diastolic blood pressure was between 16.7% and 25.6%. Nicardipine did not significantly affect heart rate with dose of 1 microgram/kg per minute. No clinical side-effects were observed. Nicardipine could be a first line drug for the treatment of hypertension in paediatric intensive care units.