Kyphectomy in neonates with meningomyelocele.

PURPOSE: Kyphosis is the most severe spinal deformity associated with meningomyelocele (MMC) and is seen in approximately 15% of neonates. Our purpose is to present our clinical experience, to discuss the technique and deformity correction in kyphectomy in neonates with MMC, and to assess its long-term outcomes. METHOD: In this prospective study, the authors reviewed eight cases submitted to surgery between 2013 and 2015. We evaluated clinical characteristics that were analyzed, as were the operative technique employed, and angle range of the kyphosis deformity postcorrection follow-up. RESULTS: Neonatal kyphectomy was performed of six females and two males. The mean birth weight was 2780 g, and the mean age at the time of surgery was 5.6 days. There were S-shaped type deformity in lumbar region in all neonates. In the correction of the kyphotic deformity, a total vertebrae were removed from four patient, whereas a partial vertebrectomy was done in four. The mean operative time was 116 min. No patients did not require the blood transfusion. There were no serious complications, and wound closure was successful in all patients. The mean follow-up period was 4 years and 3 months (range 36-61 months), except one patient who died 1 week after discharge. The mean preoperative kyphosis of 75.6° (range, 50°-90°) improved at last follow-up to 35° (range 15°-55°). All patients had surgical procedures for hydrocephalus. Three patients had surgery for Chiari type II malformation. The mean hospital stay was 27.7 days. CONCLUSION: Kyphectomy performed at the time of dural sac closure in the neonate is a safe procedure with excellent correction.

Surfactant treatment for neonatal respiratory disorders other than respiratory distress syndrome.

BACKGROUND: It is suggested that there may be expanded use of surfactant replacement for the neonatal diseases such as meconium aspiration syndrome (MAS), pneumonia and possibly bronchopulmonary dysplasia (BPD). OBJECTIVE: To evaluate the characteristics and short-term outcome of the neonates given exogenous surfactant because of the diseases other than respiratory disease syndrome (RDS). METHODS: This retrospective study included 35 neonates admitted to the neonatal intensive care unit from January 2012 to December 2012 for an expanded use of surfactant. Data related to gestational age, birth weight, gender and perinatal risk factors were obtained from the patients' records. The short-term prognosis was also noted. RESULTS: The diagnosis was sepsis in 16 patients, eight MAS, seven transient tachypnea of the newborns (TTN) and four BPD. Mean gestational age was 35.6 ± 4.5 weeks and mean birth weight was 2661 ± 981 g. Of overall cases, 65% were boys and 35% girls. The mortality rate was 17%. Of six fatal cases, three was with BPD, two with sepsis and one with MAS. CONCLUSION: We think that surfactant replacement may be life saver in the neonatal diseases other than RDS such as BPD, MAS and sepsis by rapidly improving oxygenation. Further investigation is necessary to validate the significance of expanded use of surfactant.

Effects of synchronized intermittent mandatory ventilation versus pressure support plus volume guarantee ventilation in the weaning phase of preterm infants*.

OBJECTIVE: To compare the effects and short-term outcomes of pressure support ventilation with volume guarantee versus synchronized intermittent mandatory ventilation in the weaning phase of very low-birth weight infants with respiratory distress syndrome. DESIGN: Randomized controlled prospective study. SETTING: Tertiary care neonatal unit. PATIENTS: A total of 60 premature infants who were less than 33 weeks' gestation and/or less than 1,500 g birth weight and received mechanical ventilation because of respiratory distress syndrome were studied. INTERVENTIONS: All infants were ventilated from the time of admission with synchronized intermittent positive pressure ventilation mode after surfactant treatment for respiratory distress syndrome and then switched to pressure support ventilation with volume guarantee or synchronized intermittent mandatory ventilation mode in the weaning phase. The ventilatory variables and neonatal outcomes were recorded in each group. MEASUREMENTS AND MAIN RESULTS: The mean peak inflation pressure was higher in synchronized intermittent mandatory ventilation group (p < 0.001) and the mean airway pressure was higher in pressure support ventilation with volume guarantee group (p = 0.03), whereas mean tidal volume and respiratory rates were similar in both groups. The prevalence of postextubation atelectasis was higher in synchronized intermittent mandatory ventilation group, but the difference was not statistically significant (p = 0.08). No differences were found in the prevalence of reintubation, patent ductus arteriosus, intraventricular hemorrhage, retinopathy of prematurity, bronchopulmonary dysplasia, and pneumothorax between the groups. CONCLUSIONS: Pressure support ventilation with volume guarantee mode may be a safe and feasible mode during the weaning phase of very low-birth weight infants on mechanical ventilation support for respiratory distress syndrome with respect to reducing the frequency of postextubation atelectasis and using less peak inflation pressure.

Homocysteine levels and its association with intraventricular hemorrhage in preterm infants born to preeclamptic mothers.

OBJECTIVE: To investigate the relation between serum homocysteine levels and intraventricular hemorrhage (IVH) in preterm infants born to preeclamptic mothers. METHOD: This study included 84 preterm infants (42 born to preeclamptic mothers and 42 born to normotensive healthy mothers) who were admitted to Izmir Tepecik Training and Research Hospital Neonatology Clinic on the postnatal first day. The measurement of homocysteine levels in all samples were performed with an Immulite 2000 analyzer, using the chemiluminescence method. Cranial ultrasounds were performed on the fourth day and in the 1 month of age. RESULTS: The mean plasma levels of homocysteine in infants born to preeclamptic mothers and in the control group were 8.2 ± 5.9 µmol/L and 5.3 ± 2.7 µmol/L, respectively. The plasma levels of homocysteine were significantly higher in the study group (p = 0.006). There was no association between the plasma homocysteine levels and IVH or other neonatal complications including necrotizing enterocolitis, retinopathy of prematurity, bronchopulmonary dysplasia and mortality. CONCLUSION: Our data suggest that plasma levels of homocysteine are higher among infants born to preeclamptic mothers, but these high levels are not associated with IVH and other neonatal complications in preterm infants.

Central nervous system involvement of epstein barr virus associated lymphoproliferative disorder in a child with acute lymphoblastic leukemia: successful treatment with rituximab and interferon-alpha.

UNLABELLED: Central nervous system (CNS) involvement of Epstein-Barr virus (EBV)-associated lymphoproliferative disease is a rare and serious complication in children with leukemia. Although rituximab therapy seems to be promising in these cases, persistent hypogammaglobulinemia may appear after treatment due to complete depletion of normal B lymphocytes in the peripheral blood. Here we report isolated CNS involvement of EBV-associated lymphoproliferative disorder in a 4-year-old boy with acute leukemia. The patient was treated with rituximab and interferon alpha; however, persistent hypogammaglobulinemia developed as a complication. Given the rarity of the complication in children receiving these agents, our experience with such a case may be helpful to others. CONFLICT OF INTEREST: None declared.

Mutation of the proton-coupled folate transporter gene (PCFT-SLC46A1) in Turkish siblings with hereditary folate malabsorption.

Hereditary folate malabsorption (HFM) is a rare autosomal recessive disorder characterized by systemic and central nervous system folate deficiency. Turkish siblings are reported with the clinical syndrome of HFM, homozygous for deletion of 2 bases (c.204_205 delCC) within the first exon of the proton-coupled folate transporter (PCFT) gene, causing a frameshift. Low blood and cerebrospinal fluid folate levels were detected at ages 3.5 and 1 month. Treatment with parenteral 5-formyltetrahydrofolate resulted in normal development now at ages 3 and 1 year. Extending current knowledge on the phenotypic manifestations of HFM and the PCFT mutation spectrum will provide opportunities to define possible genotype-phenotype correlations and clarify the basis for the phenotypic variability that is characteristic of this disorder.

Chorioangiomatosis presenting with severe anemia and heart failure in a newborn.

Although chorioangiomas are the most common placental tumors, multiple chorioangiomas (or chorioangiomatosis) are extremely rare. We report a female newborn at 37 weeks of gestation presenting with severe anemia, thrombocytopenia, heart failure, and intrauterine growth retardation. The pathological examination of the placenta revealed chorioangiomatosis explaining the pathophysiology of the symptoms presented. The patient was treated successfully with erythrocyte and thrombocyte transfusions and was discharged after full recovery. We conclude that evaluating placental lesions may be of great value in explaining severe problems during the fetal and the neonatal period and thus prevents performing unnecessary clinical investigations.

Effect of retinoic acid on oxygen-induced lung injury in the newborn rat.

Oxygen-induced lung injury is believed to lead to the development of bronchopulmonary dysplasia (BPD). To determine whether retinoic acid (RA) treatment prevents the development of BPD by minimizing lung injury, we investigated the effect of RA on the histopathologic characteristics of oxygen-induced lung injury in a newborn rat model. Eighteen rat pups were divided into three groups: room air-exposed control group (n=5), oxygen-exposed placebo group (n=7), and RA-treated oxygen-exposed group (n=6). Measurement of alveolar area, quantitation of secondary crest formation, microvessel count, evaluation of alveolar septal fibrosis, and smooth muscle actin (SMA) immunostaining were performed to assess oxygen-induced changes in lung morphology. Treatment of oxygen-exposed animals with RA resulted in a significant increase in mean alveolar area; however, it had no effect on the number of secondary crests and microvessel count. The degree of fibrosis and SMA expression showed a significant decrease in RA-treated animals. We conclude that RA treatment improves alveolar structure and decreases fibrosis in the newborn rat with oxygen-induced lung injury. Extrapolating these findings to humans, we speculate that similar treatment with RA may reduce lung injury in preterm infants at risk for BPD.

Isolated retrovesical cyst hydatid in a child: an unusual presentation of echinococcosis.

Echinococcosis is the most widespread, serious human cestode infection in the world. Isolated retrovesical cyst hydatid is extremely rare in children and mostly causes symptoms because of its pressure on adjacent organs. We report on the case of an 8-year-old girl with an isolated retrovesical cyst hydatid. Final diagnosis was established after histological examination, whereas imaging techniques failed to establish the diagnosis.

Polycythemia vera in a 12-year-old girl: a case report.

A case of a 12-year-old girl presenting with headache and splenomegaly and fulfilling the diagnostic criteria of polycythemia vera is reported. Her peripheral blood values were as follows: hemoglobin 18 g/dL, red blood cells 7,000,000/mm3, while blood cells 22,000/mm, and platelets 1,248,000/mm3. Phlebotomy was performed initially but was ineffective. Afterward 100 mg/kg per day aspirin and 30/mg/kg per day hydroxyurea were given. The patient has been asypmtomatic for 1 year and her recent hemoglobin level is 15.5 g/dL, platelet count 922,000/mm3, and white blood cell 12,800/mm3. Polycythemia vera is an extremely uncommon disease in childhood and for this reason its treatment is not well established.