Lymphatic Malformation in Newborns as the First Sign of Diffuse Lymphangiomatosis: Successful Treatment with Sirolimus.

Cystic hygroma and lymphangioma, currently defined as 'lymphatic malformations', are developmental abnormalities of the lymphoid system with potential for rapid expansion. The management of these abnormalities depends on the site and extent of the lesion. A different disease is diffuse lymphangiomatosis. It is very rare at birth and its treatment remains controversial. A lymphatic malformation (cystic hygroma) of the neck of a newborn girl as the first sign of diffuse lymphangiomatosis and treatment of the latter condition with sirolimus are presented. A newborn girl with a presumed isolated lymphatic malformation of the neck and significant respiratory involvement was diagnosed via total-body nuclear magnetic resonance imaging to be affected by diffuse lymphangiomatosis; she was treated with sirolimus. The treatment with sirolimus was found to be very effective, with complete resolution of the disease, good tolerability, and no side effects. This report suggests the need to explore the possibility of diffuse lymphangiomatosis in neonates with apparently isolated lymphatic malformations. In the presence of clinical deterioration, sirolimus treatment may achieve excellent results with no adverse events.

Different functional cardiac characteristics observed in term/preterm neonates by echocardiography and tissue doppler imaging.

BACKGROUND AND AIM: To establish, using echocardiography, color-flow Doppler and tissue doppler imaging (TDI), physiological values of systolic/diastolic indexes in healthy term/pre-term newborns, and to identify how different degrees of maturity influence morpho-functional cardiac alterations during the transitional period. STUDY DESIGN AND SUBJECTS: 33 term newborns (M = 19, F = 14; gestational ages: 37th-41st week), and 20 pre-term infants (M = 11, F = 9; gestational ages: 31st-36th week) admitted to our department were studied. All infants underwent to clinical and Doppler ultrasound evaluations, carried out by the third to fourth day. Investigations included: M-mode echocardiography, color-flow Doppler and TDI. OUTCOME MEASURES AND RESULTS: Term and preterm neonates differed for: interventricular septum and left systolic/diastolic ventricle diameters (p<0.01 and <0.05 respectively); left ventricle posterior wall in systole (p<0.01); shortening and ejection fraction (p<0.05). Color-flow Doppler parameters on the tricuspid (peak E, peak A, ratio E/A; p<0.05) and on the mitral (peak E and E/A ratio; p<0.01) significantly differed between the two groups. Significant differences were also present for basal left ventricular lateral wall and right ventricular lateral wall in the Ew (p<0.01 and <0.05 respectively), Sw peak (p<0.01 and <0.05 respectively), and Ew/Aw (p<0.05). The isovolumetric relax time and the E/Ew measured on the medial mitral annulus also demonstrated significant differences (p<0.01) between the two groups. CONCLUSIONS: TDI is feasible in preterm neonates and enables assessment of myocardial velocities. With increasing gestational age, higher myocardial velocities and lower E/E' Πratios were found. TDI addition to standard neonatal echocardiography may provide further important information about cardiac function.

Maternal pulse oximetry perfusion index as a predictor of early adverse respiratory neonatal outcome after elective cesarean delivery.

OBJECTIVE: Evidence suggests increased morbidity, in particular early neonatal respiratory complications, in newborns from elective cesarean section compared with those from vaginal delivery. No reliable maternal predictors of adverse neonatal outcome at elective cesarean section are known. Here, we prospectively tested the hypothesis that a low maternal perfusion index at the baseline phase (i.e., preanesthesia) of the elective cesarean section is a predictor of early adverse neonatal respiratory outcome. DESIGN: Prospective cohort study. SETTING: Operating and delivery rooms of a public health hospital with a tertiary-level neonatal intensive care unit. PATIENTS: Forty-four healthy pregnant women with no known risk factors undergoing elective cesarean section at term gestation. INTERVENTIONS: Elective cesarean section was divided into nine phases. Analysis of pulse oximetry-derived signals (perfusion index, pulse rate, and oximetry) and systolic, diastolic, and differential blood pressure were recorded. Maternal arterial and venous newborn cord blood gas analyses and placental histology were evaluated. MEASUREMENTS AND MAIN RESULTS: Early respiratory complications (transient tachypnea of the newborn, n = 5; respiratory distress syndrome, n = 1) were observed in 13.6% (6 of 44) of the newborns. A maternal perfusion index < or = 1.9 (lower quartile) during the preanesthesia phase of the elective cesarean section was an independent predictor of early adverse neonatal respiratory outcome (odds ratio 68.0, 95% confidence interval 6.02-767.72; p < .0001). CONCLUSIONS: A decreased perfusion index value in the preanesthesia phase of elective cesarean section is a maternal predictor of increased neonatal morbidity and is significantly related to subclinical placental inflammatory disease. These observations suggest the feasibility of a noninvasive pulse oximeter prenatal screening of the high-risk fetus/newborn in elective cesarean section.

Effect of a fermented formula on thymus size and stool pH in healthy term infants.

To investigate the effects of fermented formula (FF) with Bifidobacterium breve C50 and Streptococcus thermophilus 065 on thymus size and stool pH of healthy term infants, ultrasound examinations and evaluations of thymus sizes and thymus indices (TI) and measurements of stool pH were performed in the same 90 term neonates on the 3rd d of life and on the 1st, 2nd, 3rd, and 4th mo of life. Thirty newborns were exclusively breast-fed while the remaining 60 were randomly assigned to receive either a FF or a standard formula (SF). The fecal pH of the breast-fed group was lower than the SF group (p < 0.05), although it was similar to that of the FF group on the third postnatal day, persisting for the entire 4 mo of the study. The difference in TI was statistically significant over repeated measurements among the groups. The FF infants showed a TI similar to the breast-fed newborns. Probiotic fermentation products have effects comparable to those of the bacteria composing the intestinal microflora supporting the idea that intestinal bacterial balance plays an important role in improving host immune responses.