Infants of diabetic mothers. A cohort study.

OBJECTIVE: To determine the outcome of infants born to diabetic mothers at Security Forces Hospital, Riyadh, Saudi Arabia, and compare the complications seen in these infants with infants of non-diabetic mothers. METHODS: This is a concurrent prospective cohort study of a population of newborn infants delivered at Security Forces Hospital, Riyadh, Saudi Arabia for diabetic mothers between January 2011 and November 2011. RESULTS: A total of 601 infants were enrolled in the study consisting of 319 infants of non-diabetic mothers, and 282 infants of diabetic mothers. Infants of diabetic mothers showed significantly higher rates of associated complications and prolonged hospital stay reflected in their admission to the neonatal intensive care when compared with infants of non-diabetic mothers. There was no difference in rate of complications between infants of gestational diabetics and pre-gestational diabetics. CONCLUSION: Our study showed that diabetic pregnancies are associated with an increased incidence of neonatal complications. These seem to be related to the degree of maternal glycemic control. The higher rates of complications among our infants of diabetic mothers, particularly major congenital malformations call for those involved in the care of diabetic mothers to consolidate their efforts to facilitate early booking in specialist clinics.

Sirenomelia and severe caudal regression syndrome.

OBJECTIVE: To describe cases of sirenomelia and severe caudal regression syndrome (CRS), to report the prevalence of sirenomelia, and compare our findings with the literature. METHODS: Retrospective data was retrieved from the medical records of infants with the diagnosis of sirenomelia and CRS and their mothers from 1989 to 2010 (22 years) at the Security Forces Hospital, Riyadh, Saudi Arabia. A perinatologist, neonatologist, pediatric neurologist, and radiologist ascertained the diagnoses. The cases were identified as part of a study of neural tube defects during that period. A literature search was conducted using MEDLINE. RESULTS: During the 22-year study period, the total number of deliveries was 124,933 out of whom, 4 patients with sirenomelia, and 2 patients with severe forms of CRS were identified. All the patients with sirenomelia had single umbilical artery, and none were the infant of a diabetic mother. One patient was a twin, and another was one of triplets. The 2 patients with CRS were sisters, their mother suffered from type II diabetes mellitus and morbid obesity on insulin, and neither of them had a single umbilical artery. Other associated anomalies with sirenomelia included an absent radius, thumb, and index finger in one patient, Potter's syndrome, abnormal ribs, microphthalmia, congenital heart disease, hypoplastic lungs, and diaphragmatic hernia. CONCLUSION: The prevalence of sirenomelia (3.2 per 100,000) is high compared with the international prevalence of one per 100,000. Both cases of CRS were infants of type II diabetic mother with poor control, supporting the strong correlation of CRS and maternal diabetes.

Genetic, chromosomal, and syndromic causes of neural tube defects.

OBJECTIVE: To ascertain the incidence, and describe the various forms of neural tube defects (NTDs) due to genetic, chromosomal, and syndromic causes. METHODS: We carried out a retrospective analysis of data retrieved from the medical records of newborn infants admitted to the Neonatal Intensive Care Unit with NTDs and their mothers spanning 14 years (1996-2009) at the Security Forces Hospital, Riyadh, Saudi Arabia. The cases were ascertained by a perinatologist, neonatologist, geneticist, radiologist, and neurologist. The literature was reviewed via a MEDLINE search. Only liveborn babies were included. Permission from the Educational Committee at the Security Forces Hospital was obtained prior to the collection of data. RESULTS: Out of 103 infants with NTDs admitted during this period, 20 (19.4%) were found to have an underlying genetic syndromic, chromosomal and/or other anomalies. There were 5 cases of Meckel-Gruber syndrome, 2 Joubert syndrome, one Waardenburg syndrome, one Walker-Warburg syndrome, 2 chromosomal disorders, 2 caudal regression, one amniotic band disruption sequence, one associated with omphalocele, one with diaphragmatic hernia, and 4 with multiple congenital anomalies. CONCLUSION: There is a high rate of underlying genetic syndromic and/or chromosomal causes of NTDs in the Saudi Arabian population due to the high consanguinity rate. Identification of such association can lead to more accurate provisions of genetic counseling to the family including preimplantation genetic diagnosis or early termination of pregnancies associated with lethal conditions.

Outcomes of an isolated single umbilical artery in singleton pregnancy: a large study from the Middle East and Gulf region.

OBJECTIVE: To assess the frequency and accuracy of prenatal diagnosis of a single umbilical artery (SUA) and to compare the fetal and neonatal outcome of isolated SUA to that of a normal three-vessel umbilical cord in a population from the Middle East and Gulf region. STUDY DESIGN: Data were collected from 37,500 singleton pregnancies that were scheduled for antenatal care and delivered at Security Forces Hospital in Riyadh, Saudi Arabia, between May 2004 and December 2012. Comparisons between the groups were performed using a chi-square test or a Fisher exact test for the categorical variables, and Student's t test or Wilcoxon's rank-sum test were used for continuous variables. The Kappa statistic was used to study the agreement between the antenatal and final neonatal diagnosis of SUA. A univariable analysis was used to calculate the unadjusted and adjusted ORs and the 95% CIs expressing the relationship of the normal three-vessel umbilical cord, the isolated SUA, and each outcome. RESULTS: A total of 35,249 cases completed the study, including 35,026 cases with normal neonatal three-vessel umbilical cords (Group B). SUA was present in 223 (0.63%) neonates (0.45% isolated SUA (Group A) and 0.18% non-isolated SUA). The sensitivity, specificity, positive predictive value and negative predictive value of using prenatal ultrasound for the diagnosis of SUA were 90.58%, 99.9%, 98.5% and 99.94%, respectively. Neonatal anomaly was present in 2.6% of the fetuses with isolated SUA. A pregnancy with isolated SUA was more likely to be complicated with polyhydramnios (OR 3.32; CI 1.22-9.04), preterm delivery <34 weeks (OR 4.662; CI 2.346-9.195), birth weight <10th percentile (OR 2.1; CI 1.44-2.93), cesarean delivery for fetal distress (OR 2.72; CI 1.53-4.81), perinatal death (OR 3.31; CI 1.34-8.12), admission to NICU (OR 2.71; CI 1.87-3.91), and placental abnormalities (OR 3.25; CI 2.14-4.93; p value 0.0001) compared to a pregnancy with a fetal and neonatal three-vessel cord. CONCLUSION: Isolated SUA is associated with anomalies at birth and with an increased risk of adverse pregnancy outcomes even in the absence of other anomalies. A pregnancy with this complication should receive close fetal monitoring for growth and fetal wellbeing.