Ultrasound-Guided Prospective Screening for Spinal Dysraphism in Offspring of Mothers With Pregestational Diabetes: A Pilot Study.

AIM: Determine the utility of prospective spinal ultrasound in infants of mothers with pregestational diabetes (PGDM) for the diagnosis of closed spinal dysraphism (SDs). METHODS: This prospective observational pilot study was completed at a tertiary care center between May 1, 2020 and December 30, 2022. Infants born to mothers with PGDM and with normal spinal physical examinations were included. A total of 25 mother-infant dyads were enrolled in the study and prospectively screened with spinal ultrasound. The study was registered on ClinicalTrials.gov (Identifier-NCT05033275). RESULTS: Twenty-five spinal ultrasounds were performed over the course of this study with three (8%) resulting in abnormal findings that required further imaging. Follow-up with magnetic resonance imaging found one case of tethered cord syndrome. CONCLUSION: Prospective screening in infants of mothers with PGDM found one case of tethered cord syndrome. This finding suggests that risk stratified screening of mothers with diabetes might be a reasonable approach to care.

Diagnostic Utility of Spinal Ultrasounds in Neonates.

OBJECTIVE: Ultrasound (US) is a common imaging modality used to evaluate spinal anomalies in newborns. However, many indications for the use of spinal US are associated with low diagnostic yield. The purpose of this study is to evaluate the indications and the diagnostic utility of spinal USs performed in newborns at our institution. We also review patient presentations for caudal regression syndrome (CRS) that was identified from the USs performed. STUDY DESIGN: This study is a retrospective review of spinal USs performed between January 1, 2006, and December 31, 2021, in newborns at a single institution. Indications for each US and any associated abnormalities were noted. Infants with abnormal USs showing CRS are described with their long-term medical outcomes. RESULTS: A total of 592 USs were performed during the specified time period of which 72 (12%) were abnormal. The presence of a sacral dimple was the most common indication for performing a spinal US, although only 14 (4%) were identified as abnormal. Of these 14, 6 (43%) were further evaluated by spinal magnetic resonance imaging (MRI) at the recommendations of a pediatric radiologist and of these, only 2 (14%) had abnormal MRI findings. The two newborns with abnormal MRI findings had mothers with diabetes mellitus in their pregnancies. Of note, one additional newborn had abnormalities on spinal US that was never confirmed on MRI due to being lost to follow-up. Among the other indications, anorectal anomalies (odds ratio [OR], 7.55; 95% confidence interval [CI], 3.01-18.91), spinal mass (OR, 17.99; 95% CI, 7.86-41.2), and meningocele were most associated with abnormal findings. CONCLUSION: Overall, spinal US has a low diagnostic yield. Sacral dimple was the most common indication for performing a spinal US but had a low yield with few long-term sequelae. Anorectal anomalies had a strong association with abnormal US findings. KEY POINTS: · Sacral dimple is a common indication for spinal ultrasound.. · Spinal ultrasound has low diagnostic yield when performed for sacral dimple.. · Caudal regression syndrome is associated with maternal diabetes and numerous long-term comorbidities..

Diabetic Embryopathies.

Diabetic embryopathy is defined as congenital anomalies that are linked to maternal diabetes. The association between diabetes and fetal, neonatal, and long-term complications is well-established. These complications include organ or structural maldevelopment, fetal growth abnormalities, and learning/psychiatric comorbidities. Recent studies have elucidated the pathophysiology behind these conditions and outlined new management approaches. Caudal regression syndrome, also known as sacral agenesis, is a well-known but less described complication of maternal diabetes. The purpose of this review is to summarize existing research on common neonatal morbidities in infants of mothers with diabetes with a focus on caudal regression syndrome and its long-term associations.

Interval between clinical presentation of necrotizing enterocolitis and bowel perforation in neonates.

OBJECTIVE: To define the interval between clinical presentation of necrotizing enterocolitis (NEC) and bowel perforation in neonates. METHODS: Charts of neonates with discharge diagnosis of NEC (n = 124) from our NICU during 2004-2008 were retrospectively reviewed. Demographic data were collected. Acute episode of NEC was defined as the interval between clinical presentations to resumption of enteral feeds. Neonates are followed, as a standard of care, clinically and radiologically until resumption of enteral feeds at the discretion of the attending clinician. Abdominal radiograph results were reviewed serially to determine the interval between clinical presentation and bowel perforation using pneumoperitoneum as the surrogate radiological marker. Histological report of resected bowel specimens was reviewed for coagulative necrosis as evidence of NEC and to exclude spontaneous intestinal perforation (SIP). Neonates with stage 1 NEC and SIP were excluded from the results. RESULTS: 105 neonates with stage 2 NEC were included in the study. Forty-six needed surgical treatment (group 2) and 59 did not need surgery (group 1). Twenty-six (26/46, 56%) group 2 neonates had bowel perforation and hence required surgery. Pneumoperitoneum was noted at a median interval of 1 day after presentation of symptoms. Twenty neonates in group 2 needed surgery for clinical indications including worsening clinical examination, thrombocytopenia or persistent metabolic acidosis. Fifty-nine neonates (group 1) were treated with bowel rest, antibiotics and parenteral nutrition. Group 2 neonates were significantly more premature, weighed less and had less radiographs than group 1 neonates. Mortality was significantly higher in group 2 compared to group 1. CONCLUSION: Bowel perforation occurs at a median interval of 1 day after clinical presentation of NEC. Neonates not needing surgery for their disease are exposed to significantly more radiographs than those needing surgery. Radiological evaluation can be safely minimized or eliminated after 2 days of presentation.

Comparison of short-term outcomes of late preterm singletons and multiple births: an institutional experience.

We compare 4 short-term outcomes--namely admission to special care nursery (SCN), length of stay (LOS), age at full feeds (AFF) and respiratory morbidity/need for ventilation--in 1015 late preterm singletons and 366 twins and triplets born at our institution over a 4-year period. Birth weight (BW) and gestational age (GA) rather than plurality of birth determined need for admission to SCN, LOS, AFF, and need for respiratory support. When matched for GA, compared to singletons, twins and triplets needed less admission to SCN and respiratory support at 36 weeks, whereas at 34 weeks, they had longer LOS and took longer to get to full feeds. We conclude that the outcomes of interest are affected by GA and BW rather than plurality.

Short-term outcomes of late preterms: an institutional experience.

This article describes the short-term outcomes of late preterm neonates born between 34 and 36 (6/7) weeks gestational age (GA) in a 4-year period. A total of 1381/ 20554 (6.7%) births were between 34 and 36 weeks of GA and were predominantly Caucasian (87%). In all, 697/1381 (51%) were admitted to nursery whereas 684/1381 (49%) remained with their mothers. Of the babies born at 34, 35, and 36 weeks GA, 97%, 53%, and 32%, respectively, required admission and of these 30%, 33%, and 23%, respectively, required respiratory support. Air leaks developed in <4% infants. Median length of stay and age at full enteral feeds were 11, 6, and 4 days and 10, 6, and 3 days for 34, 35, and 36 weeks GA, respectively. It is concluded that late preterms have significant morbidity in the neonatal period.

Outcomes of exomphalos: an institutional experience.

OBJECTIVE: Abdominal wall defects, particularly exomphalos, in newborn infants are associated with significant morbidity and mortality. The objective of the present study was to review the outcomes of neonates with exomphalos in our neonatal intensive care unit during the last 12 years. STUDY DESIGN: In this retrospective study 52 neonates with exomphalos were identified from 1996 to 2007. Exomphalos were stratified by the type of defect [exomphalos minor versus major (major defined as defect size more than 5 cm and/or liver in the sac)]. Clinical data, demographic data, and outcome measures of mortality, length of stay (LOS), duration of mechanical ventilation and age at full enteral feeds were studied. Associated anomalies were compared between the two groups. RESULTS: Of the 52 neonates, 1 was transferred back to referring hospital after surgical repair of the defect and was not analyzed. Exomphalos minor accounted for 24 cases and exomphalos major in 27 cases. Mortality was higher in infants with exomphalos major (n = 9, 33%) compared to infants with exomphalos minor (n = 2, 8%). The median LOS (10 vs. 47 days, P = 0.023), median age at full enteral feeds (5 vs. 23 days, P = 0.004) and median duration of mechanical ventilation (7 vs. 23 days, P = 0.001) were shorter for exomphalos minor compared to exomphalos major. Bacteremia was present in 4 (15%) of neonates with exomphalos major. Syndromic associations were present in 8 neonates (33%) with exomphalos minor compared to 2 neonates (7%) with exomphalos major. Beckwith Wiedemann syndrome was most frequently noted in neonates with syndromic exomphalos minor. Trisomy 13 was the only chromosomal abnormality in the entire cohort (1/51 = 2%) and was seen in a single neonate (1/27 = 3.7%) with exomphalos major. Non-syndromic anomalies were seen in 12 (50%) and 14 neonates (52%) with the minor and major defects, respectively. Only four neonates with exomphalos minor (16%) and ten neonates with exomphalos major (37%) had no associated anomalies. Pulmonary hypoplasia and pulmonary hypertension were identified on either lung biopsy or autopsy (n = 5) as causes of mortality. CONCLUSION: Neonates with exomphalos minor have better survival, decreased LOS, time to full enteral feeds and shorter duration of mechanical ventilation. Syndromic associations were more common in exomphalos minor. Respiratory failure was the major cause of mortality in infants with exomphalos major.

Outcomes in neonatal gastroschisis: an institutional experience.

We describe outcomes of 70 infants with gastroschisis admitted to our neonatal intensive care unit between 2001 and 2005. Demographic data and outcome measures including discharge or death, simple versus complex (intestinal atresia, perforation, bowel necrosis, or volvulus), length of stay, mechanical ventilation and total parenteral nutrition, commencement of enteral feeds and age at reaching full feeds, and number of episodes of bacteremia were evaluated. In our study, 72% of the patients (n = 58) were simple cases, 28% were complex, and 44% had a positive blood culture. Median length of stay was 43 and 116 days for simple and complex cases, respectively. Median age at commencement of enteral feeds was 19 and 44 days for simple and complex cases, respectively. Six (14%) infants with simple and 8 (50%) with complex gastroschisis required an assisted feeding device (gastrostomy) tube before discharge. We concluded that the type of gastroschisis (simple versus complex) is an important determinant of outcome, including time to start and get to full feeds, duration of parenteral nutrition, length of stay, and survival. Although it is not always possible to determine whether the defect is simple or complex by antenatal scans, this information will be valuable to families with infants with gastroschisis and should be made available as soon as possible.