Prenatal diagnosis of a retroperitoneal lymphangioma: a case and review.

Prenatal sonographic findings of lymphangiomas characteristically appear as thin-walled, multiseptate hypoechogenic masses. In our case, a retroperitoneal hypoechogenic mass was detected at 26 weeks of gestation by sonography. Serial sonographic examinations demonstrated multiple septations at 29 weeks of gestation, which in retrospect was a classical finding of retroperitoneal lymphangioma. MRI at 38 weeks of gestation revealed a multilocular mass, which was homogeneous, low on T1-weighted and high on T2-weighted images, in the left retroperitoneal cavity. These findings were compatible with the diagnosis of a lymphangioma. This case shows the change in characteristic imaging features, from a unilocular to multilocular pattern, of a lymphangioma with regard to gestational age. It is important to observe the size and extension of such tumors in order to determine the prospect for neonatal prognosis, as well as to make decisions on the delivery timing and style.

The lung-to-thorax transverse area ratio at term and near term correlates with survival in isolated congenital diaphragmatic hernia.

OBJECTIVE: The purpose of this study was to determine how well liver position, the lung area-to-head circumference (L/H) ratio, and the lung-to-thorax transverse area (L/T) ratio predicted the need for extra-corporeal membrane oxygenation (ECMO) and survival in fetuses with isolated congenital diaphragmatic hernia (CDH). METHODS: Antenatal records of 25 fetuses with isolated left-sided CDH who were born by cesarean delivery under fetal stabilization at this institution were reviewed. The latest determinations of the L/H and L/T ratios before birth (between 34 and 38 weeks' gestation) were compared on the basis of the cutoff points for mortality: less than 1.0 versus 1.0 or greater for the L/H ratio and 0.08 or less versus greater than 0.08 for the L/T ratio. Outcome measures assessed were survival (discharge to home) and the need for ECMO. RESULTS: Overall survival was 64% (16/25). Postnatal survival in fetuses with an L/T ratio of 0.08 or less was statistically lower than in those with an L/T ratio of greater than 0.08 (33% versus 81%; P = .0308). The percentage requiring ECMO in the group with an L/T ratio of 0.08 or less was also higher than that of the group with an L/T ratio of greater than 0.08, but the difference was not statistically significant (67% versus 25%; P = .0872). Neither the L/H ratio nor herniation of the fetal liver into the chest affected survival or the need for ECMO. CONCLUSIONS: In fetuses with isolated CDH at term or near term, the L/T ratio may be a better predictor of outcome than the L/H ratio or liver herniation.

Increased inflammatory markers are associated with early periventricular leukomalacia.

The aim of the study was to investigate whether inflammatory markers are associated with the occurrence of periventricular leukomalacia (PVL). Superoxide (O(2) (-)) production of neutrophils and plasma antioxidative superoxide dismutase (SOD) activity in umbilical cord blood were studied. Participants were preterm infants with early PVL (n=6; three males, three females; mean birthweight 1458g [SD 517], range 620-2040g; mean gestational age 29.8wks [SD 2.9], range 27-34wks); and preterm control infants without PVL (n=10; five males, five females; mean birthweight 1838g [SD 664], range 925-2748g; mean gestational age 30.6wks [SD 3.1], range 26-34wks). In addition, pro-inflammatory cytokine levels were measured in the umbilical cord blood. N-formyl-methionyl-leucyl-phenylalanine-induced O(2) (-) production by neutrophils in infants with early PVL was significantly higher than that in the control group. In contrast, there was no significant difference in concentrations of copper/zinc-SOD and SOD activity between groups. Concentrations of interleukin (IL)-1beta and tumour necrosis factor-alpha (but not IL-6, IL-8, or granulocyte-colony stimulating factor) were significantly higher in infants with early PVL than in control infants. The excess O(2) (-) produced by activated neutrophils with increased pro-inflammatory cytokine production could play a role in the molecular cascade leading to white matter damage in PVL.

The influence of thermal environment on pulmonary hemodynamic acclimation to extrauterine life in normal full-term neonates.

OBJECTIVE: To assess chronological changes of pulmonary blood flow in response to ambient temperature load in normal full-term neonates. METHODS: Group I (n=8) neonates were maintained at 25-26 degrees C for the first 24 h after birth, with Group II (n=7) at 32-33 degrees C. Left pulmonary artery flow volume (Vp) and ductus arteriosus diameter were measured at 2, 4, 6, 12, and 24 h using Doppler ultrasound. Core and peripheral temperatures, as a marker for cold stress, were also evaluated over the same time frame. RESULTS: For Group I, Vp was steady for the first 6 h after birth before gradually decreasing. In contrast, Vp for Group II significantly decreased from 2-6 h, without later changes. At 6 h after birth in Group II, mean values of both Vp and ductus arteriosus diameter were significantly lower, whereas mean value of peripheral temperature was significantly higher than values in Group I (P<0.05). CONCLUSION: Pulmonary blood flow in neonates placed at neutral ambient temperature stabilizes earlier than that of neonates placed at room temperatures. Changes of peripheral flow in response to ambient temperature load may be associated with decreased pulmonary blood flow through a left-to-right ductal shunt.

Pleural fluid/serum immunoglobulin ratio is a diagnostic marker for congenital chylothorax in utero.

OBJECTIVE: To distinguish congenital chylothorax from other causes of hydrothorax in utero. METHODS: Serum and pleural fluid samples from 8 fetuses with congenital chylothorax and 5 control fetuses with other causes of hydrothorax were tested for total protein, albumin, IgG, IgA, and IgM. RESULTS: Fetuses with congenital chylothorax had higher levels of IgG in pleural fluid, but not the other four proteins, than control fetuses (P<0.05). There were no significant differences in serum proteins among fetuses. When we examined pleural fluid to serum ratios, the IgG ratio in fetuses with congenital chylothorax was significantly higher than that of control fetuses (P<0.05). The IgG ratio in chylothorax was greater than 0.6 regardless of lymphocyte count. CONCLUSION: Pleural fluid/serum IgG ratio may be a diagnostic marker for congenital chylothorax in utero.

Molecular characterization of del(8)(p23.1p23.1) in a case of congenital diaphragmatic hernia.

A 36-week-old fetus was referred to the medical center because of his cystic mass and fluid in left thoracic cavity, and was delivered by cesarean section to manage neonatal problems at 37 weeks of gestation. Emergent surgical repair of the left diaphragmatic hernia was performed, but severe hypoxia persisted, and he expired on the following day. Chromosome analysis of cultured amniotic fluid cells indicated 46,XY,del(8)(p23.1p23.1). This is the fourth case of 8p23.1 deletion associated with diaphragmatic hernia. Microarray comparative genomic hybridization analysis using DNA of cultured amniotic fluid cells showed that six clones were deleted, which were mapped to the region between two low copy repeats (LCRs) at 8p23.1 previously described. Microsatellite analysis revealed that the deletion was of paternal origin, and his parents did not carry 8p23.1 polymorphic inversion. These data strongly suggested that the 8p23.1 interstitial deletion should have arisen through a different mechanism from that of inv dup del(8p) whose structural abnormality is always of maternal origin and accompanies heterozygous 8p23.1 polymorphic inversion in mother.