Real-Time Ultrasound Guidance for Umbilical Venous Cannulation in Neonates With Congenital Heart Disease.

OBJECTIVES: Umbilical venous cannulation is the favored approach to perinatal central access worldwide but has a failure rate of 25-50% and the insertion technique has not evolved in decades. Improving the success of this procedure would have broad implications, particularly where peripherally inserted central catheters are not easily obtained and in neonates with congenital heart disease, in whom umbilical access facilitates administration of inotropes and blood products while sparing vessels essential for later cardiac interventions. We sought to use real-time, point-of-care ultrasound to achieve central umbilical venous access in patients for whom conventional, blind placement techniques had failed. DESIGN: Multicenter case series, March 2019-May 2021. SETTING: Cardiac and neonatal ICUs at three tertiary care children's hospitals. PATIENTS: We identified 32 neonates with congenital heart disease, who had failed umbilical venous cannulation using traditional, blind techniques. INTERVENTIONS: Real-time ultrasound guidance and liver pressure were used to replace malpositioned catheters and achieve successful placement at the inferior cavoatrial junction. MEASUREMENTS AND MAIN RESULTS: In 32 patients with failed prior umbilical venous catheter placement, real-time ultrasound guidance was used to successfully "rescue" the line and achieve central position in 23 (72%). Twenty of 25 attempts (80%) performed in the first 48 hours of life were successful, and three of seven attempts (43%) performed later. Twenty-four patients (75%) were on prostaglandin infusion at the time of the procedure. We did not identify an association between patient weight or gestational age and successful placement. CONCLUSIONS: Ultrasound guidance has become standard of care for percutaneous central venous access but is a new and emerging technique for umbilical vessel catheterization. In this early experience, we report that point-of-care ultrasound, together with liver pressure, can be used to markedly improve success of placement. This represents a significant advance in this core neonatal procedure.

Thrombocytosis in an infant with a TRPV4 mutation: a case report.

Mutations in the calcium channel gene Transient Receptor Potential cation channel subfamily V member 4 (TRPV4) cause autosomal dominant skeletal dysplasia, with phenotypes ranging from mild to perinatal lethality. A recent report detailed enhanced proplatelet formation and increased murine platelet count in the context of TRPV4 activation. No prior reports have described platelet count abnormalities in human TRPV4 disease. Here, we report a case of prolonged thrombocytosis in the context of TRPV4-associated metatropic dysplasia that was lethal in the infantile period.

Do infants in the neonatal intensive care unit diagnosed with urinary tract infection need a routine voiding cystourethrogram?

OBJECTIVE: To determine if infants diagnosed with urinary tract infection (UTI) in the neonatal intensive care unit (NICU) require a routine voiding cystourethrogram (VCUG). STUDY DESIGN: Retrospective data analysis from three centers for infants admitted to the NICU born between 2000 and 2013 and diagnosed with UTI. RESULTS: One hundred twenty-six infants from three centers were diagnosed with UTI during their hospitalization. Renal ultrasound (RUS) was performed in 115 infants (91.2%), of which 69 (60%) were abnormal. Mild to moderate hydronephrosis or pelviectasis were the most common abnormalities identified (n = 34, 30%). There were 14 infants (12%) with severe abnormalities on RUS. VCUG was performed in 71 infants (56%), of which 3 (4%) were interpreted as abnormal with grade 2 vesicoureteral reflux (VUR) or less (two infants were with normal RUS and one infant was with abnormal RUS). CONCLUSIONS: More than 50% of infants with a UTI had an abnormal RUS but severe abnormalities were found only in 11% of infants. Only 4% of infants with UTI had VUR; none of these infants had severe VUR on VCUG. A routine VCUG after UTI in the NICU has a low yield and may be reserved for infants with severe or persistent abnormalities on RUS.

Partial trisomy 19p13.3 and partial monosomy 1p36.3: Clinical report and a literature review.

We report on a 15-month-old girl with a deletion of the distal short arm of chromosome 1p36.3, partial trisomy of the short arm of chromosome 19p13.3, growth and developmental delay, and multiple anomalies including microcephaly, bifrontal prominence, obtuse frontonasal angle, short columella, hypertelorism, sacral dimples, and a bicuspid pulmonary valve. Based on our FISH mapping studies, we estimate the size of the trisomic region of 19p.13.3 to be approximately 3.17 Mb, and the region of monosomy for 1p36.3 as 1.3 Mb. This is the first report of a patient with partial trisomy 19p13.3 and partial monosomy p36.3.

Calcified meconium balls in a newborn: an unusual case with imperforate anus, rectourinary fistula, colpocephaly, and agenesis of corpus callosum.

Calcified intraluminal meconium is a rare finding in newborn infants. It is often associated with communication between the urinary and gastrointestinal tracts. Intra-abdominal calcifications are unusual radiographic findings in the newborn and can easily be misinterpreted as meconium peritonitis. We report on a newborn infant with anorectal malformation, meconium balls, intraluminal calcifications, colpocephaly, and agenesis of the corpus callosum, a rare association.